First Aid for the USMLE Step 2 CK, Eleventh Edition [11 ed.] 9781264856510, 1264856512, 9781264855100, 1264855109

Table of contents :
COVER
GUIDE TO EFFICIENT EXAM PREPARATION
CARDIOVASCULAR
DERMATOLOGY
ENDOCRINOLOGY
EPIDEMIOLOGY
HEALTH SYSTEMS SCIENCE
GASTROINTESTINAL
HEMATOLOGY
MUSCULOSKELETAL
NEUROLOGY
OBSTETRICS
GYNECOLOGY
PEDIATRICS
PSYCHIATRY
PULMONARY
RENAL/GENITOURINARY
MULTISYSTEM
RAPID REVIEW
TOP-RATED REVIEW
RESOURCES
ABBREVIATIONS AND SYMBOLS
COMMON LABORATORY VALUES
INDEX

Citation preview

A STUDENT-TO­ STUDENT GUIDE Completely revised and expanded for the new USMLE® Step 2 CK Case vignettes test your application of knowledge Updated key facts and mnemonics reinforce key information Rapid Review section for last minute cramming Hundreds of new and revised color clinical images and illustrations

Mc

Graw Hill

TAO LE ■ VIKAS BHUSHAN ■ MONA ASCHA ABHISHEK BHARDWAJ ■ MARINA BOUSHRA ■ DANIEL GRIFFIN

FIRST AID��:.

USMLE Step 2 CK Eleventh Edition TAO LE, MD, MHS Founder, ScholarRx Associate Clinical Professor, Department of Medicine University of Louisville School of Medicine

VIKAS BHUSHAN, MD Founder, First Aid for the USMLE Step 1 Boracay, Philippines

MONA ASCHA, MD Fellow, Department of Plastic Surgery Johns Hopkins University

ABHISHEK BHARDWAJ, MD Pulmonologist and lntensivist Coast Pulmonary Associates, Orange County, California Assistant Clinical Professor, Health Sciences University of California, Riverside

MARINA BOUSHRA, MD Emergency Medicine and Critical Care Medicine Cleveland Clinic Foundation, Cleveland, Ohio

DANIEL GRIFFIN, DO lntensivist, Saint Francis Medical Center

CAROLINE COLEMAN, MD Resident, Department of Medicine Emory University School of Medicine

STEPHANIE JONES, PhD Emory University Laney Graduate School

KIMBERLY KALLIANOS, MD Assistant Professor, Department of Radiology and Biomedical Imaging University of California, San Francisco School of Medicine

New York

Chicago Milan

San Francisco Athens London Madrid Mexico City New Delhi Singapore Sydney Toronto

Copyright© 2023, 2019, 2016, 2012, 2010, 2007, by Tao Le. All rights reserved. Except as permitted under the United States Copyright Act of1976, no part ofthis publication may be reproduced or distributed in any form or by any means, or stored in a database or retrieval system, without the prior written permission ofthe publisher. ISBN: 978-1-26-485651-0 MHID: 1-26-485651-2 The material in this eBook also appears in the print version ofthis title: ISBN: 978-1-26-485510-0, MHID: 1-26-485510-9. eBook conversion by codeMantra Version 1.0 All trademarks are trademarks oftheir respective owners. Rather than put a trademark symbol after every occurrence ofa trademarked name, we use names in an editorial fashion only, and to the benefit ofthe trademark owner, with no intention ofinfringement ofthe trademark. Where such designa­ tions appear in this book, they have been printed with initial caps. McGraw Hill eBooks are available at special quantity discounts to use as premiums and sales promotions or for use in corporate training programs. To contact a representative, please visit the Contact Us page at www.mhprofessional.com. McGraw Hill, the McGraw Hill logo, Practice Makes Perfect, and related trade dress are trademarks or registered trademarks of McGraw Hill and/ or its affiliates in the United States and other countries and may not be used without written permission. All other trademarks are the property oftheir respective owners. McGraw Hill is not associated with any product or vendor mentioned in this book. NOTICE

Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. The authors and the publisher ofthis work have checked with sources believed to be reliable in their efforts to provide information that is com­ plete and generally in accord with the standards accepted at the time of publication. However, in view ofthe possibility ofhuman error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication ofthis work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work. Readers are encouraged to confirm the information contained herein with other sources. For example and in particular, readers are advised to check the product information sheet included in the package ofeach drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is ofparticular importance in connection with new or infrequently used drugs. TERMS OF USE This is a copyrighted work and McGraw-Hill Education and its licensors reserve all rights in and to the work. Use ofthis work is subject to these terms. Except as permitted under the Copyright Act of1976 and the right to store and retrieve one copy ofthe work, you may not decompile, disassemble, reverse engineer, reproduce, modify, create derivative works based upon, transmit, distribute, disseminate, sell, publish or sublicense the work or any part ofit without McGraw-Hill Education's prior consent. You may use the work for your own noncommercial and personal use; any other use ofthe work is strictly prohibited. Your right to use the work may be terminated ifyou fail to comply with these terms. THE WORK IS PROVIDED "AS IS." McGRAW-HILL EDUCATION AND ITS LICENSORS MAKE NO GUARANTEES OR WARRANTIES AS TO THE ACCURACY, ADEQUACY OR COMPLETENESS OF OR RESULTS TO BE OBTAINED FROM USING THE WORK, INCLUD­ ING ANY INFORMATION THAT CAN BE ACCESSED THROUGH THE WORK VIA HY PERLINK OR OTHERWISE, AND EXPRESSLY DISCLAIM ANY WARRANTY, EXPRESS OR IMPLIED, INCLUDING BUT NOT LIMITED TO IMPLIED WARRANTIES OF MERCHANT­ ABILITY OR FITNESS FOR A PARTICULAR PURPOSE. McGraw-Hill Education and its licensors do not warrant or guarantee that the functions contained in the work will meet your requirements or that its operation will be uninterrupted or error free. Neither McGraw-Hill Education nor its licensors shall be liable to you or anyone else for any inaccuracy, error or omission, regardless of cause, in the work or for any damages resulting therefrom. McGraw-Hill Education has no responsibility for the content ofany information accessed through the work. Under no circumstances shall McGraw-Hill Education and/or its licensors be liable for any indirect, incidental, special, punitive, consequential or similar damages that result from the use ofor inability to use the work, even ifany ofthem has been advised ofthe possibility ofsuch damages. This limitation ofliability shall apply to any claim or cause whatsoever whether such claim or cause arises in contract, tort or otherwise.

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DEDICATION To the contributors to this and past editions, who took time to share their knowledge, insight, and humor for the benefit of students and physicians everywhere.

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Contents Contributing Authors ............................... vi Faculty Reviewers .................................. viii Preface.............................................. ix Acknowledgments...................................x How to Contribute.................................. xi How to Use This Book.............................. xii

Pediatrics ......................................... 509

SECTION 1: GUIDE TO EFFICIENT EXAM PREPARATION ................................. 2

Rapid Review ..................................... 765

Introduction ........................................ 2 USMLE Step 2 CK-Computer-Based Testing Basics.. 2 Defining Your Goal................................. 10 Study Resources ................................... 12 Test-Day Checklist.................................. 13 Testing Agencies................................... 14

SECTION 2: DATABA SE OF HIGH-YIELD FACTS ... 15 How to Use the Database .......................... 16 Cardiovascular ..................................... 17 Dermatology....................................... 87 Endocrinology .................................... 123 Epidemiology..................................... 157 Health Systems Science ........................... 177 Gastrointestinal................................... 197 Hematology/Oncology............................269 Musculoskeletal................................... 313 Neurology ........................................ 351 Obstetrics.........................................427 Gynecology.......................................467

Psychiatry......................................... 585 Pulmonary........................................ 625 Renal/Genitourinary............................... 675 Multisystem....................................... 717

SECTION 3: TOP-RATED REVIEW RESOURCES ... 791 How to Use the Database .........................792 Comprehensive................................... 794 Question Banks ................................... 794 Internal Medicine, Emergency Medicine, Family Medicine................................ 795 Neurology ........................................ 795 OB/GYN........................................... 795 Pediatrics ......................................... 796 Psychiatry......................................... 796 Surgery ........................................... 796 Commercial Review Courses ......................797 Appendix I: Acronyms and Abbreviations.......... 799 Appendix II: Common Laboratory Values .......... 807 Index .............................................809 About the Authors ................................ 842

CONTRIBUTING AUTHORS Manik Aggarwal, MD, MBBS Fellow, Gastroenterology and Hepatology Mayo Clinic, Rochester, Minnesota

Waneeza Mughees, MD Resident, Obstetrics and Gynecology Drexel University College of Medicine, Philadelphia

John Carl Barba II, MPH MD Candidate Ohio State University College of Medicine

Christian Cuvillier Padilla, MD Chief Resident, Internal Medicine Cleveland Clinic and Cleveland Veterans Affairs Medical Center, Cleveland, Ohio

Anup Chalise, MBBS, MS Nepal Paola Ghanem, MD Physician, Department of Internal Medicine Johns Hopkins University Myles Mowery, DO, MBA Resident, Diagnostic and Interventional Radiology Spectrum Health/Michigan State University Grand Rapids, Michigan

Vivek Podder, MBBS Intern BIRDEM General Hospital, Bangladesh Vikram Shee, MBBS, MSc Physician Case Manager Teladoc Health, Inc. Collin Andrew Weintraub, MD Resident, General Surgery State University of New York Upstate Medical University

IMAGE AND ILLUSTRATION TEAM Yoree Grace Chung MD/PhD Candidate Emory University School of Medicine

Victor Jose Martinez Leon, MD Resident, Internal Medicine Einstein Medical Center, Philadelphia

Nikitha Crasta, MBBS Mangalore, India

Angel Xiao, MD, MSE Resident, Orthopedic Surgery University of California-San Francisco

Sean Evans, MD Resident, Internal Medicine Emory University School of Medicine

vii

ASSOCIATE AUTHORS Aparna Savitri Bhat, MD Fellow, Pulmonary and Critical Care Medicine Cleveland Clinic, Cleveland, Ohio Naveena Daram, MD Resident, Obstetrics and Gynecology Wright State University lfrah Fatima, MBBS Resident, Internal Medicine University of Missouri-Kansas City School of Medicine Lindsay Friedman, MD, MS Resident, General Surgery Rush University Medical Center Luise Josefine Froessl, MD Resident Universite d'Aix-Marseille, Faculte de Medecine lzhan Hamza, MBBS Resident University of Texas Medical Branch, Internal Medicine

Abdul Rehman Mustafa, MBBS Alfaisal University College of Medicine, Saudi Arabia Aaron Panicker, MD Resident, Emergency Medicine University of Florida College of Medicine Austin Patrick, DO Resident Franciscan Health Olympia Fields Faateh Ahmad Rauf, MBBS CMH Lahore Medical College and Institute of Dentistry, Pakistan Jaimie Rogner, MD, MPH Resident, Internal Medicine/Pediatrics University of Rochester Medical Center/Strong Memorial Hospital Kyle Robert Wagner MD Candidate University of Illinois College of Medicine, Peoria

viii

FACULTY REVIEWERS Brooks D. Cash, MD Professor, Department of Medicine Chief, Gastroenterology, Hepatology, and Nutrition University of Texas Health Science Center at Houston

Soroush Rais-Bahrami, MD Professor of Urology and Radiology and Interim Chair, Department of Urology University of Alabama at Birmingham School of Medicine

Dimitri Cassimatis, MD Associate Professor, Department of Medicine Emory University School of Medicine

Keisha Ray, PhD Assistant Professor, McGovern Center for Humanities and Ethics University of Texas Health Science Center at Houston

Bradley Cole, MD Assistant Professor of Neurology Loma Linda University School of Medicine

Rachel Marie E. Salas, MD, MEd Professor, Neurology and Nursing Johns Hopkins Medicine

Meredith K. Greer, MD Assistant Professor, Department of Medicine Emory University School of Medicine

Sarah Schimansky, MB BCh BAO Resident, Department of Ophthalmology Bristol Eye Hospital

Jennifer 0. Howell, MD Associate Professor, Department of Obstetrics and Gynecology Duke University Medical Center

Shireen Madani Sims, MD Associate Professor and Vice Chair for Education, Obstetrics and Gynecology University of Florida College of Medicine

Matthew Kraybill, PhD Clinical Neuropsychologist Cottage Health, Santa Barbara, California Patrick M. Lank, MD, MS Assistant Professor, Department of Emergency Medicine Northwestern University, Feinberg School of Medicine Kachiu C. Lee, MD, MPH Assistant Professor (Adjunct), Department of Dermatology Lewis Katz School of Medicine at Temple University Carl Marfurt, PhD Professor Emeritus, Department of Anatomy, Cell Biology and Physiology Indiana University School of Medicine-Northwest, Gary Peter Marks, MD, PhD Center for Biologics Evaluation and Research US Food and Drug Administration Kristen L. Pagel, MD, MPH Assistant Professor, Department of Psychiatry University of Utah School of Medicine Mahesh Patel, MD Professor, Department of Medicine University of Illinois at Chicago College of Medicine Diane Payne, MD, MPT Orthopedic, Hand and Microvascular Surgery OrthoAtlanta Newnan, Georgia

Nathan Wm. Skelley, MD Associate Professor, Medical Director of Orthopaedic Surgery Sanford Health-University of South Dakota School of Medicine Matthew Sochat, MD Physician, Hematology/Oncology Southeastern Medical Oncology Center Lorrel Toft, MD Associate Professor of Cardiology, Department of Medicine University of Nevada, Reno School of Medicine Tisha Wang, MD Professor of Clinical Medicine, Department of Medicine David Geffen School of Medicine at UCLA Sylvia Wassertheil-Smoller, PhD Professor Emerita, Department of Epidemiology and Population Health Albert Einstein College of Medicine Adam Weinstein, MD Associate Professor of Medical Sciences and Pediatrics Frank H. Netter MD School of Medicine at Quinnipiac University James S. Yeh, MD, MPH Department of Medicine Massachusetts General Hospital, Harvard Medical School Kristal Young, MD Clinical Instructor, Department of Cardiology Huntington Hospital, Pasadena, California

Preface With the 11th edition of First Aid for the USMLE Step 2 CK, we continue our commitment to pro­ viding students with the most high-yield and up-to-date preparation guide for the USMLE Step 2 CK exam. Preparation for and performance on the Step 2 CK exam are more important than ever with the transition of the Step 1 exam to a pass/fail scoring system in 2022. With this in mind, we have greatly expanded the content and depth for the 11th edition. This revision includes: ■ ■ ■ ■

■ ■ ■ ■ ■

Over 200 additional pages of content incorporating the most current evidence-based reviews and rec­ ommendations to help students on the Step 2 CK exam and in clinical practice. 163 new and revised diagrams and illustrations, including more than 40 new diagnostic and manage­ ment algorithms, to further drive home the next best diagnostic and management options. 140 new and revised photos/images to help visualize various disorders, descriptive findings, and clini­ cal content tie-ins. Extensive text revisions, new mnemonics, and clarifications curated by a team of 26 medical student and resident physician authors who excelled on their USMLE exams and verified by a team of expert faculty advisors and nationally recognized USMLE instructors. Continued focus on clinical presentation and the best initial step in diagnosis and management, mirroring the content outline and blueprint of Step 2 CK Vignette-style fl.ash cards embedded in the margins to reinforce key concepts. Heavily updated and revised Rapid Review section for last-minute preparation. Revised rating of current high-yield review resources, with clear explanations of their relevance to Step 2 CK exam review. Improved organization and integrations of text, illustrations, clinical images, tables, and algorithms throughout for focused review of high-yield topics.

The 11th edition of First Aid for the USMLE Step 2 CK truly is a completely revised, in-depth, student­ to-student guide for preparation for the Step 2 CK exam. The 11th edition would not have been pos­ sible without the help from hundreds of students and faculty members who contributed their feedback and suggestions. We invite students and faculty to continue sharing their thoughts and ideas to help us improve First Aid for the USMLE Step 2 CK (see How to Contribute, p. xi). Tao Le

Louisville

Vikas Bhushan Boracay

Mona Ascha Baltimore

Abhishek Bhardwaj

Orange County

Marina Boushra Cleveland

Daniel Griffin

Cape Girardeau, Missouri

Caroline Coleman Atlanta

Stephanie Jones

Tumwater, Washington

Kimberly Kallianos

San Francisco

Acknowledgments This has been a collaborative project from the start. We gratefully acknowledge the thoughtful comments, correc­ tions, and advice of the many medical students, international medical graduates, and faculty who have supported the authors in the continuing development of First Aid for the USMLE Step 2 CK. Thanks to our publisher, McGraw Hill, for the valuable assistance of its staff, including Bob Boehringer, Christina Thomas, and Jeffrey Herzich. For outstanding editorial work, we thank Megan Chandler and Lisa Nahach. We are also grateful to our medical illustrator, Rachael Williams, and illustration manager, Susan Mazik. For administra­ tive support we thank Miranda Carter, Katherine Knight, and Louise Petersen. A special thanks to CW, Inc. for remarkable production work. For contributions and corrections, we thank Ataa Ahmed, Arjun Basnet, Jeffrey Beach, Monica I. Burgos Claudio, Fiorella B. Castillo, Mallory Castillo, Christian Casteel, Anthony Chung, Jonathan Daou, Karanpal Dhaliwal, Fadi Dib, Celia Escamilla, Arber Frakulli, Mohan Bharadwaj Gudivada, Jacqueline Hairston, Nathaniel Hayward, Lydia Kaotzani, Panagiotis Kaparaliotis, Alex Lu, Juliana Maya, Austin McCullough, Nupur Mishra, Mounika Mukherjee Peethala, Majd Oteibi, Shannon D. Powell, Aubtin Saedi, Tanjot Saini, Angelica Maria Sanchez Ruiz, Charles Sanky, Maida Sarfraz Chaudhry, Neetu Scariya, Ryan Schusler, Tarif Shaaban, Tomonari Shimoda, Biraj Singh Karki, Colton Southall, Charles Starling, Ari Stone, Erica Stratton, Jennifer Tram, Nicholas Ting, Vivekanand Tiwari, Sheela Vaswani, and Earl Vialpando. Tao Le

Louisville

Vikas Bhushan Boracay

Mona Ascha Baltimore

Abhishek Bhardwaj

Orange County

Marina Boushra Cleveland

Daniel Griffin

Cape Girardeau, Missouri

Caroline Coleman Atlanta

Stephanie Jones

Tumwater, Washington

Kimberly Kallianos

San Francisco

How to Contribute In our effort to continue to produce a high-yield review source for the Step 2 CK exam, we invite you to submit any suggestions or corrections. We also offer paid internships in medical education and publishing ranging from three months to one year (see below for details). Please send us your suggestions for the following: ■ ■ ■

Study and test-taking strategies for the Step 2 CK exam New high-yield facts, mnemonics, diagrams, and illustrations Low-yield topics to remove

For each entry incorporated into the next edition, you will receive up to a $20 gift certificate to Amazon as well as personal acknowledgment in the next edition. Diagrams, tables, partial entries, updates, corrections, and study hints are also appreciated, and significant contributions will be compensated at the discretion of the authors. Also let us know about material in this edition that you feel is low yield and should be deleted. The preferred way to submit entries, suggestions, or corrections is via our blog: www.firstaidteam.com We are also reachable by e-mail at [email protected].

NOTE TO CONTRIBUTORS All entries become property of the authors and are subject to editing and reviewing. Please verify all data and spell­ ings carefully. If similar or duplicate entries are received, only the first entry received will be used. Include a refer­ ence to a standard textbook to facilitate verification of the fact. Please follow the style, punctuation, and format of this edition if possible.

INTERNSHIP OPPORTUNITIES The author team is pleased to offer part-time and full-time paid internships in medical education and publishing to motivated physicians. Internships may range from three months (eg, a summer) up to a full year. Participants will have an opportunity to author, edit, and earn academic credit on a wide variety of projects, including the popular First Aid series. Writing/editing experience, familiarity with Microsoft Word and Google Docs, and illustration skills are highly desired. For more information, e-mail a resume or a short description of your experience along with a cover letter to [email protected].

xii

How to Use This Book We have made many improvements and added several new features to this edition of First Aid for the USMLE Step 2 CK. In particular, we have added more than two hundred pages of content and hundreds of new illustrations and images throughout the text to facilitate studying. We encourage you to read all aspects of the text to learn the ma­ terial in context. We have also included comments in the margins and vignette questions to periodically test your knowledge of key concepts. These questions are located in the lower corner of certain pages. To prevent peeking at the answers, you'll find the answer on the back of the same page in the lower corner. These questions are not always representative of test questions. To practice for the exam and simulate the actual test day, you can use the USMLE-Rx Step 2 CK Qmax question test bank (www.usmle-rx.com). If you are constantly on the move, use the USMLE-Rx Step 2 CK app. The question bank and this text are more than enough to allow many students to ace the exam. Good luck!

GUIDE TO EFFICIENT EXAM PREPARATION 11 12

Introduction

2

USMLE Step 2 CK-Computer-Based Testing Basics How W1LL THE CBT BE STRUCTURED?

2 2 2

TESTING CONDITIONS: WHAT WILL THE (BT BE LIKE?

3

CLINICAL REVIEW BOOKS

WHAT DOES THE (BT FORMAT MEAN FOR ME?

3

TEST BANKS

12 12 12 12

How Do I REGISTER TOTAKE THE EXAMINATION?

4

TEXTS AND NOTES

13

WHAT IF I NEED TO RESCHEDULE THE EXAMINATION?

5

(OMMERCIAL (OURSES

13

NBME/USMLE PUBLICATIONS

13

WHO (AN REGISTER FOR THE EXAM?

WHAT ABOUT TIME?

6

SECURITY MEASURES

6

IF I LEAVE DURING THE EXAMINATION, WHAT HAPPENS TO MY SCORE?

6

WHAT TYPES OF QUESTIONS ARE ASKED?

6

How LONG W1LL I HAVE TO WAIT BEFORE I GET MY SCORES?

8

How ARE THE SCORES REPORTED?

9

Defining Your Goal

WHEN TOTAKE THE EXAM How W1LL THE STEP 2 CK SCORE AFFECT MY MATCH?

Study Resources QUALITY (ONSIDERATIONS

Test-Day Checklist THINGS TO BRING WITH You TOTHE EXAM

Testing Agencies

13 13

14

10

1

2

SECTION 1

GUIDE TO EF FICIENT EXAM PREPARATION

INTRODUCTION

0--n

KEY FACT

The goal of the Step 2 CK is to apply your knowledge of medical facts to clinical scenarios that you may encounter as a resident.

The United States Medical Licensing Examination (USMLE) Step 2 allows you to pull together your clinical experience on the wards with the numerous "factoids" and classical disease presentations that you have memorized over the years. Where Step 1 stresses basic disease mechanisms and principles, Step 2 places more emphasis on clinical diagnosis and management, disease pathogenesis, and preventive medicine. Previously, the Step 2 examination consisted of the Step 2 Clinical Knowledge examination (Step 2 CK), and the Step 2 Clinical Skills examination (Step 2 CS). However, recent changes have removed the Step 2 CS exam as a requirement for ECFMG certification after the onset of the pandemic, and this change has been recorded as perma­ nent by the ECFMG. The USMLE Step 2 CK is the second of three examinations that you must pass to become a licensed physician in the United States. The computerized Step 2 CK is a 1-day (9-hour) multiple-choice examination.

USM LE STEP 2 CK-COMPUTER-BASED TESTING BASICS WHO CAN REGISTER FOR THE EXAM? The eligibility requirement for USMLE Step 2 CK exam is same as that of USMLE Step 1 and can be taken either before or after the Step 1 exam. This means that you should be: Officially enrolled in, or be a graduate of, a US or Canadian medical school leading to the MD degree (LCME accredited), or Officially enrolled in, or be a graduate of, a US medical school leading to the DO degree (COCA accredited), or Officially enrolled in, or be a graduate of, a medical school outside the US and Canada and listed in the World Directory of Medical Schools as meet­ ing ECFMG eligibility requirements and meet other ECFMG criteria. These criteria should be met at the time of application and on the test day.

HOW WILL THE CBT BE STRUCTURED?

0-,r KEY FACT

Sometimes the answer to the previous question in a sequential question set is provided to you once you lock your answer. Do not be disheartened if you got it wrong. Simply understand that you now have an opportunity to get at least one answer correct in the sequence.

The Step 2 CK exam is a computer-based test (CBT) administered by Prometric, Inc. It is a 1-day examination with a maximum of 318 items divided into eight I-hour blocks that are administered during a single 9-hour testing session. The number of items in a block are displayed at the beginning of each block. This number may vary from block to block but will not exceed 40 items per block. Two question styles predominate throughout. The most common format is the single one-best-answer question. This is the traditional multiple-choice format in which you are tasked with selecting the "most correct" answer. Sequential item sets comprises the second question style. These are sets of multiple-choice questions that are related and must all be answered in sequence without skipping a question in the set. As you answer questions in a set, the previous answers become locked and cannot be changed. These are the only questions on the USMLE examination that are locked in such a way. There are no more than five sequential item sets within each USMLE Step 2 CK exam. During the time allotted for each block in the USMLE Step 2 CK exam, you can answer test questions in any order and can also review responses and change your answers (except for responses within the sequential item sets

GUIDE TO E F FICIENT EXAM PREPARATION SECTION 1 described earlier). However, under no circumstances can you return to previ­ ous blocks and change your answers. Once you have finished a block, you must click on a screen icon to continue to the next block. Time not used dur­ ing a testing block will be added to your overall break time (45 minutes total at start of exam), but it cannot be used to complete other testing blocks. Also note that a short tutorial (shorter than the one available at the USMLE web­ site) is present at the start of the exam, which if you choose to skip, can add 15 minutes to your total break time.

0-rr

KEY FACT

0-rr

KEY FACT

Expect to spend up to 9 hours at the test center.

TESTING CONDITIONS: WHAT WILL THE CBT BE LIKE? Even if you are familiar with CBT and the Prometric test centers, you should still access the latest practice software from the USMLE Web site (http://www. usmle.org) and try out prior to the examination. For security reasons, you are not allowed to bring personal equipment (except those needed for medical reasons and soft-foam earplugs as detailed later) into the testing area - which means that writing implements, outerwear, watches (even analog), cellular telephones, and electronic paging devices are all prohibited. Food and beverages are prohibited as well. The proctor will assign you a small locker to store your belongings and any food you bring for the day. You will also be given two 8 cm x 11 cm laminated writing surfaces, pens, and erasers for note taking and for recording your test Candidate Identifi­ cation Number (CIN). You must return these materials after the examination. Note that you are not allowed to write on these until you enter the CIN number in the computer. Testing centers are monitored by audio and video surveillance equipment, and minimum of 2 surveillance rounds by the exam monitor per hour. Each time you enter the testing room, you will have to undergo a screen­ ing process to ensure that you are not bringing in personal items. You should become familiar with a typical question screen. A window to the left displays all the questions in the block and shows you the unanswered questions (marked with an "i"). Some questions will contain figures, color illustrations, audio, or video adjacent to the question. Although the contrast and brightness of the screen can be adjusted, there are no other ways to manipulate the picture (eg, zooming or panning). Larger images are accessed with an "exhibit" button. You can also call up a window displaying normal lab values. You may mark questions to review at a later time by clicking the check mark at the top of the screen. The annotation feature functions like the provided dry-erase sheets and allows you to jot down notes during the exami­ nation. Play with the highlighting/strike-out and annotation features with the vignettes and multiple answers. You should also do a few practice blocks to determine which tools will help you process questions more efficiently and accurately. If you find that you are not using the marking, annotation, or highlighting tools, then key­ board shortcuts can be quicker than using a mouse. Headphones are pro­ vided for listening to audio and blocking outside noise. Alternatively, you can bring soft earplugs to block excess noise. These earplugs must be examined by Prometric staff before you can take them into the testing area.

WHAT DOES THE CBT FORMAT MEAN FOR ME? The CBT format is the same format as that used on the USMLE Step 1. If you are uncomfortable with this testing format, spend some time playing with a Windows-based system and pointing and clicking icons or buttons with a mouse. The USMLE also offers students an opportunity to take a simulated test, or practice session, at a Prometric center. The session is divided into three

Keyboard shortcuts: ■ A-E-Letter choices. ■ Enter or space bar-Move to the next question. ■ Esc-Exit pop-up Lab and Exhibit windows. ■ Alt-T-Countdown and time­ elapsed clocks for current session and overall test.

3

4

SECTION 1

GUIDE TO E F FICIENT EXAM PREPARATION I-hour blocks of up to 50 questions each. The approximately 127 Step 2 CK sample test items that are available on the USMLE Web site (http://www. usmle.org) are the same as those used at CBT practice sessions. No new items are presented. The cost is about $7 5 for US and Canadian students but is higher for international students. Students receive a printed percent-correct score after completing the session. No explanations of questions are provided. You may register for a practice session online at http://www.usmle.org. The National Board of Medical Examiners (NBME) provides another option for students to assess their Step 2 CK knowledge with the Comprehen­ sive Clinical Science Self-Assessment (CCSSA) test. This test is available on the NBME Web site for $60, which will display at the end of the exam all of the questions that you answered incorrectly. The current versions of the test also have answer explanations. The content of the CCSSA items resembles that of the USMLE Step 2 CK After you complete the CCSSA, you will be given a performance profile indicating your strengths and weaknesses. This feedback is intended for use as a study tool only and is not necessarily an indi­ cator of Step 2 CK performance. For more information on the CC SSA exami­ nation, visit the NBME's Web site at http://www.nbme.org, and click on the link for "Students and Residents."

HOW DO I REGISTER TO TAKE THE EXAMINATION? Information on the Step 2 CK exam's format, content, and registration require­ ments are found on the USMLE Web site. To register for the examination, students/graduates of accredited schools in the United States and Canada can apply online at the NBME Web site (http://www.nbme.org), whereas students/ graduates of non-US/Canadian schools should apply through the Educational Commission for Foreign Medical Graduates (ECFMG) (https://iwa2.ecfmg. org). A printable version of the application is also available on these sites. The preliminary registration process for the USMLE Step 2 CK exam is as follows: Complete a registration form and send your examination fees to the NBME (online) for students in US/Canada medical schools, and to the ECFMG (online) for international medical students. The fees payable are outlined in Table 1. 1. ■ Select a 3-month block in which you wish to be tested (eg, June/July/August). ■ Attach a passport-type photo to your completed application form. ■ Complete a Certification of Identification and Authorization form. This form must be signed by an official at your medical school such as from the registrar's office (if you are a student) or a notary public (if you have gradu­ ated) to verify your identity. It is valid for 5 years, allowing you to use only your USMLE identification number for future transactions. Send your certified application form to the NBME for processing. Appli­ cations may be submitted more than 6 months before the test date, but examinees will not receive their scheduling permits until 6 months prior to the eligibility period. ■ The NBME will process your application within 4-6 weeks and will send you a slip of paper that will serve as your scheduling permit. ■ Once you have received your scheduling permit, decide when and where you would like to take the examination. For a list of Prometric locations nearest you, visit https://www.prometric.com. ■ Call Prometric's toll-free number or visit https://www.prometric.com to arrange a time to take the examination. The Step 2 CK is offered on a year-round basis except for the first 2 weeks in January. For the most up-to-date information on available testing days at your preferred testing location, refer to http://www.usmle.org. ■

GUIDE TO EF FICIENT EXAM PREPARATION SECTION 1 TA B L E 1 . 1

5

Exam Fees for the USMLE Step 2 CK

FEE PAYA BLE TO NBME (US AND CA NADA SCHOOLS O N LY)

FEE PAYA BLE TO ECFMG (ALL OTH ER SCHOOLS)

Exam fee

$645

$985

Scheduling charge

None

$21 0

Eligibility period extension

$70

$ 1 00

Changing testing region

$90

$90

Requesting exam recheck

$80

$80

The scheduling permit you receive from the NBME will contain the fol­ lowing important information: Your USMLE identification number. The eligibility period during which you may take the examination. Your "scheduling number," which you will need to make your examina­ tion appointment with Prometric. ■ Your CIN, which you must enter at your Prometric workstation in order to access the examination.

■ ■ ■

Prometric has no access to the codes and will not be able to supply these numbers, so do not lose your permit! You will not be allowed to take the Step 2 CK unless you present your permit along with an unexpired, government­ issued photo identification that contains your signature (eg, driver's license, passport). Make sure the name on your photo ID exactly matches the name that appears on your scheduling permit.

WHAT IF I NEED TO RESCHEDULE THE EXAMINATION? You can change your date and/or center within your 3-month period by con­ tacting Prometric if space is available. When you reschedule, a fee may apply (Table 1.2). If you need to reschedule outside your initial 3-month period, you can apply for a single 3-month extension (eg, April/May/June can be extended through July/August/September) after your eligibility period has begun. For other rescheduling needs, you must submit a new application along with another application fee.

TA B L E 1 .2

Rescheduling Fees Payable to Prometric for USMLE Step 2 CK (1 Jan, 2022)

RESCH EDULING TIME BEFORE EXAM DATE

FEES FOR THE US A N D CA NADA TESTI N G REGION

FEES FOR TESTI NG REGIONS OTHER THAN THE US AND CANADA

46 days or more

No fee

No fee

Between 3 1 and 45 days

$35

$35

Between 6 days and 30 days

$ 1 00

$ 1 00

Less than 5 days

$ 1 44

$369

0-n

KEY FACT

Because the Step 2 CK examination is scheduled on a "first-come, first­ served" basis, you should be sure to call Prometric as soon as you receive your scheduling permit.

6

SECTION 1

GUIDE TO E F FICIENT EXAM PREPARATION WHAT ABOUT TIME? Time is of special interest on the CBT examination. The following is a break­ down of the examination schedule: Tutorial 1 -hour q uestion blocks (40 q uestions per block) Break time (includes time for lunch) Total test time

1 5 minutes 8 hours 45 minutes 9 hours

The computer will keep track of how much time has elapsed during the examination. However, the computer will show you only how much time you have remaining in a block. Therefore, it is up to you to determine if you are pacing yourself properly. The computer will not warn you if you are spending more than the 45 minutes allotted for break time. The break time includes not only the usual concept of a break - when you leave the testing area - but also the time it takes for you to make the transition to the next block, such as entering your CIN or even taking a quick stretch. If you do exceed the 45-minute break time, the time to complete the last block of the test will be reduced. How­ ever, you can elect not to use all of your break time, or you can gain extra break time either by skipping the tutorial or by finishing a block ahead of the allotted time.

SECURITY MEASURES Smile! The USMLE uses a check-in/check-out process that includes elec­ tronic capture of your fingerprints and photograph. Fingerprints from a finger on each hand will be used for this process. These measures are intended to increase security by preventing fraud, thereby safeguarding the integrity of the examination. These procedures also decrease the amount of time needed to check in and out of the examination throughout the day, thereby maximizing your break time. However, you still need to sign out and sign in with the Test Center Log when exiting and entering the testing area.

IF I LEAVE DURING THE EXAMINATION, WHAT HAPPENS TO MY SCORE? You are considered to have started the examination once you have entered your CIN onto the computer screen, but to receive an official score, you must finish the entire examination. This means that you must start and either finish or run out of time for each block of the examination. If you do not complete all of the question blocks, your examination will be documented on your USMLE score transcript as an incomplete attempt, but no actual score will be reported. The examination ends when all blocks have been completed or time has expired. As you leave the testing center, you will receive a written test comple­ tion notice to document your completion of the examination.

WHAT TYPES OF QUESTIONS ARE ASKED? The Step 2 CK is an integrated examination that tests understanding of nor­ mal conditions, disease categories, and physician tasks. Almost all questions on the examination are case based. Some questions will involve interpreting a study or drug advertisement. A substantial amount of extraneous information

GUIDE TO E F FICIENT EXAM PREPARATION SECTION 1

may be given, or a clinical scenario may be followed by a question that could be answered without actually requiring that you read the case. It is your job to determine which information is superfluous and which is pertinent to the case at hand. Content areas include internal medicine, OB/GYN, pediatrics, preventive services, psychiatry, surgery, and other areas relevant to the provi­ sion of care under supervision (see Tables 1.3, 1.4, and 1. 5). Most questions on the examination have a single best-answer format. The part of the vignette that actually asks the question - the stem - is usually found at the end of the scenario and generally relates to the physician task. From student experience, there are a few stems that are consistently addressed throughout the examination: What is the most likely diagnosis? (40%) Which of the following is the most appropriate initial step in manage­ ment? (20%) ■ Which of the following is the most appropriate next step in management? (20%)

■ ■

TA B L E 1 .4

Exam Content Specification per System

SYSTEM

RANGE, %

General Principles Of Fou ndational Science'

2-4

I m m une System

3-5

Blood & Lymphoreticular System

4-6

Behavioral Health

6-8

Nervous System & Special Senses

6-8

Musculoskeletal System/Skin & Subcutaneous Tissue

6-1 0

Cardiovascular System

8-1 0

Respiratory System

7-9

Gastrointestinal System

7-9

Renal & Urinary System & Male Reproductive

4-6

Pregnancy, Childbirth & The Puerperium

4-6

Female Reproductive System & Breast

4-6

Endocrine System

4-6

Multisystem Processes & Disorders

4-6

Biostatistics & Epidemiology/Population Health/I nterpretation

3-5

Of Medical Literatu re Social Sciences: Legal/Ethical Issues & Professionalism/ Systems-Based Practice & Patient Safety

1 0-1 5

Percentages are subject to change at any time. 'The Step 2 CK General Principles category includes normal and abnormal processes that are not limited to specific organ systems.

7

TA B L E 1 .3 Exam Content Specification per Discipline

COMPETENCY

RANGE, %

Medicine

50-60

Surgery

25-30

Pediatrics

20-25

Obstetrics & Gynecology

1 0-20

Psychiatry

1 0-1 5

8

SECTION 1

GUIDE TO E F FICIENT EXAM PREPARATION TAB L E 1 .s

Exam Content Specification per Physician Tasks/Competencies

COMPETENCY

RANGE, %

Medical Knowledge: Applying Foundational Science Concepts Patient Care: History And Physical Exam Patient Care: Laboratory/Diagnostic Studies

1 3-1 7

Patient Care: Diagnosis

1 6-20

Patient Care: Prognosis/Outcome

5-9

Patient Care: Health Maintenance/Disease Prevention

8-1 2

Patient Care: Pharmacotherapy

8-1 2

Patient Care: Clinical I nterventions

6-1 0

Patient Care: Mixed Management

1 2-1 6

Practice-Based Learning & I mprovement

3-5

Professionalism

5-7

Systems-Based Practice & Patient Safety

5-7

Percentages a re subject to change at any time. "Test items that assess patient care competencies may also assess knowledge of underlying foundational science concepts. bTest items that assess history and physical exam competencies are covered in Step 1 and Step 3 examinations.

■ ■ ■

Which of the following is the most likely cause of. . . ? ( 5 %) Which of the following is the most likely pathogen . . . ? (3 %) Which of the following would most likely prevent. . . ? (2%) Other (10%)

Additional examination tips are as follows: Note the age and race of the patient in each clinical scenario. When eth­ nicity is given, it is often relevant. Know these well (see high-yield facts), especially for more common diagnoses. ■ Be able to recognize key facts that distinguish major diagnoses. ■ Questions often describe clinical findings rather than naming eponyms (eg, they cite "audible hip click" instead of "positive Ortolani sign"). Questions about acute patient management (eg, trauma) in an emergency setting are common. The cruel reality of the Step 2 CK examination is that no matter how much you study, there will still be questions you will not be able to answer with confidence. If you recognize that a question cannot be solved in a rea­ sonable amount of time, make an educated guess and move on; you will not be penalized for guessing. Also bear in mind that some of the USMLE ques­ tions are "experimental" and will not count toward your score.

HOW LONG WILL I HAVE TO WAIT BEFORE I G ET MY SCORES? The USMLE reports scores 3-4 weeks after the examinee's test date. During peak periods, however, as many as 6 weeks may pass before reports are scored.

GUIDE TO E F FICIENT EXAM PREPARATION SECTION 1

This usually includes scheduled delays after the first two weeks of the year in January when the scores may get delayed up to March. Official information concerning the time required for score reporting is posted on the USMLE Web site, http://www.usmle.org and recent changes may need to be checked every testing session.

HOW ARE THE SCORES REPORTED? Like the Step 1 score report, your Step 2 CK report includes your pass/fail status, a numeric score, and a performance profile organized by discipline and disease process (see Fig. 1. 1). The score is a 3-digit scaled score based on a predefined proficiency standard. The current required passing score is 2 14. This score requires answering 60-70% of questions correctly. Any adjustments in the required passing score will be available on the USMLE Web site.

US·MLE

United States Med ical Licensi ng Examination ®

Step 2 CK Score Report

United States Medical Licensing Examfoation

FOR EXAMINEE USE ONLY. THIRD-PARTY USERS OF USM LE SCORES SHOULD RELY SOLELY ON OFFICIAL TRANSCRI PTS RECEIVED DIRECTLY FROM THE EXAMINEE'S USMLE REGISTRATION ENTITY.

NAM E : Smith, John USMLE ID: 1-234-567-8

'---------' ®

TEST DATE: M a rch 3 1, 2022

Your Performance

Test Result

Test Score

262

PASS

Your Performance Compared to Other Examinees

The chart below represents the distribution of scores for examinees from US and Canadian medical schools taking Step 2 CK for the first time between July 1, 2017 and June 30, 2018. Reported scores range from 1-300 with a mean of 244 and a standard deviation of 17.

Your Score

I

Minimum

I Passing Score

SlSS

156-170

171-185

186-200

201 15

216-230

231-245

246-260

261-275

>276

If you tested repeatedly under the same conditions on a different set of items covering the same content, without learning or forgetting, your score would fall within one standard error of the estimate (SEE) of your current score two-thirds of the time. The SEE on this exam is 8 points. Your score +/- SEE: 254 - 270

F I G U R E 1 - 1 . Sample score report-front page.

9

10

SECTION 1

GUIDE TO E F FICIENT EXAM PREPARATION

U nited States Medical Licensing Examination

Step 2 CK Score Report Supplementa l I nformation : U ndersta nding the Content Areas The information below is a visual representation of the content weighting on this examination that may be informative in guiding remediation. Descriptions of the topics covered in these content areas, as well as other topics covered on USM LE Step 2 CK, can be found in the information materials on the USMLE website (https://www.usmle.org). Please use the contact form on the USMLE website (https://www.usmle.org/contact/) if you have additional questions. Physician Task PC: Diagnosis PC: Pharmacotherapy, Interventions & Management MK: Applying Foundational Science Concepts PC: Health Maint, Prevention & Surveillance

( % Items Per Test) (40 - 50%) (22 - 27%) (12 - 16%) (7 - 11%)

Abbreviations: M K, Medical Knowledge; PC, Patient Care. System cardiovascular System Gastrointestinal System Respiratory System Behavioral Health Musculoskeletal Sys/Skin & Subcutaneous Tissue Nervous System & Special Senses Multisystem Processes & Disorders Endocrine System Female Reproductive & Breast Pregnancy, Childbirth & the Puerperium Renal & Urinary System & Male Reproductive Blood & Lymphoreticular System Immune System

(% Items Per Test) (8 - 12%) (7 - 11%) (7 - 11%) (6 - 10%) (6 - 10%) (6 - 10%) (S - 9%) (4 - 8%) (4 - 8%) (4 - 8%) (4 - 8%) (4 - 7%) (4 - 6%)

Discipline Medicine Surgery Pediatrics Obstetrics & Gynecology Psychiatry

(% Items Per Test) (50 - 60%) (2S - 30%) (20 - 25%) (10 - 20%) (10 - 15%)

FIGURE 1 - 1 .

Sample score report-back page.

DEFINING YOUR GOAL Step 2 CK scores are becoming increasingly used for residency selection. The amount of time spent in preparation for this examination varies widely among medical students. Possible goals include the following: ■ Beating the mean. This signifies an ability to integrate your clinical and factual knowledge to an extent that is superior to that of your peers (around 247 for recent examination administrations). Others redefine this goal as achieving a score 1 standard deviation above the mean (usually in the range of 250-260). Highly competitive residency programs may use your Step 1 and Step 2 scores (if available) as a screening tool or as a selec­ tion requirement. International medical graduates should aim to beat the mean, as USMLE scores are likely to be a selection factor even for less competitive US residency programs. ■ Acing the exam. Perhaps you are one of those individuals for whom noth­ ing less than the best will do - and for whom excelling on standardized examinations is a source of pride and satisfaction.

GUIDE TO E F FICIENT EXAM PREPARATION SECTION 1

■ Evaluating your clinical knowledge. In many ways, this goal should serve as the ultimate rationale for taking the Step 2 CK, as it is technically the reason the examination was initially designed. The case-based nature of the Step 2 CK differs significantly from the more fact-based Step 1 exami­ nation in that it more thoroughly assesses your ability to recognize classic clinical presentations, deal with emergent situations, and follow the step­ by-step thought processes involved in the treatment of particular diseases. ■ Preparing for internship. Studying for the USMLE Step 2 CK is an excel­ lent way to review and consolidate all of the information you have learned in preparation for internship. Matching statistics, including examination scores related to various special­ ties, are available at the National Resident Matching Program Web site at https://www.nrmp.org under "Data and Reports."

WHEN TO TAKE THE EXAM The second most important thing to do in your exam preparation is to decide when to take the examination. With the CBT, you now have a wide variety of options regarding when to take the Step 2 CK Here are a few factors to consider: The nature of your objectives, as defined earlier. The specialty to which you are applying. An increasing number of resi­ dency programs are viewing the Step 2 CK as an integral part of the resi­ dency application process. Several research publications demonstrate the increasing importance placed on this examination by residency directors. Some programs are now requiring the Step 2 CK score in order to rank candidates for a residency position. It is therefore in the best interest of candidates to have this examination done in time for scores to be available for the residency application. Taking the examination in June or July ensures that scores will be available for the Match period that begins in September. Some programs, however, will accept scores after the applica­ tion process starts. Check with programs in your desired specialty to deter­ mine when to take the exam. ■ Prerequisite to graduation. If passing the USMLE Step 2 CK is a prereq­ uisite to graduation at your medical school, you will need to take the examination in the fall or winter at the latest. ■ Proximity to clerkships. Many students feel that the core clerkship mate­ rial is fresher in their minds early in the fourth year, making a good argu­ ment for taking the Step 2 CK earlier in the fall. ■ The nature of your schedule. ■ Considerations for MD/PhD students. The dates of passing the Step 1, Step 2, and Step 3 examinations should occur within a 7-year period. However, the typical pathway for MD/PhD students consists of 2-3 years of preclinical (and sometimes clinical) work in medical school, 3-4 years of graduate work with research, and finally returning to medical school for clinical work. MD/PhD students typically exceed the 7-year limit. Depending on the state in which licensure is sought, such students may need to petition their licensure body for an exception to this rule. ■ Considerations for International Medical Graduates. A passing score on the Step 2 CK is required to qualify for ECFMG certification which is necessary to match. It is generally recommended to take the Step 2 CK early enough to obtain ECFMG certification before interview season is completed, ideally even before applications are reviewed by programs. ■ ■

0-,,. KEY FACT The Step 2 CK is a n opport u n ity to conso l idate you r c l i n ica l knowledge and prepa re for i ntern s h i p.

11

12

SECTION 1

GUIDE TO EF FICIENT EXAM PREPARATION HOW WILL THE STEP 2 CK SCORE AF FECT MY MATCH? Since Step 1 is now being reported as pass or fail, it is expected that the Step 2 CK score may take on more importance. Programs receive hundreds if not thousands of applications yearly, and they rely on certain objective metrics to select applicants to interview. Having a competitive Step 2 CK score will not guarantee a match at a top choice program but it will strengthen your applica­ tion. It is one of many elements that programs will consider.

STU DY RESO U RCES QUALITY CONSIDERATIONS Although an ever-increasing number of USMLE Step 2 CK review books and software packages are available on the market, the quality of these materials is highly variable (see Section 3). Some common problems include the following: Some review books are too detailed to be reviewed in a reasonable amount of time or cover subtopics that are not emphasized on the examination (eg, a 400-page anesthesiology book). ■ Many sample question books have not been updated to reflect current trends on the Step 2 CK. ■ Many sample question books use poorly written questions, contain factual errors in their explanations, give overly detailed explanations, or offer no explanations at all. ■ Software for boards review is of highly variable quality, may be difficult to install, and may be fraught with bugs.

0-,,. KEY FACT

The best review book for you reflects the way you like to learn. If a given review book is not working for you, stop using it no matter how highly rated it may be.

CLINICAL REVIEW BOOKS Many review books are available, so you must decide which ones to buy by carefully evaluating their relative merits. Toward this goal, you should com­ pare different opinions from other medical students; read the reviews and rat­ ings in Section 3 of this guide, and examine the various books closely in the bookstore. Do not worry about finding the "perfect" book, as many subjects simply do not have one. There are two types of review books: those that are stand-alone titles and those that are part of a series. Books in a series generally have the same style, and you must decide if that style is helpful for you and optimal for a given subject.

TEST BANKS A test bank can serve multiple functions, including the following: Provide information about strengths and weaknesses in your fund of knowledge. Add variety to your study schedule. ■ Serve as the main form of study. ■ Improve test-taking skills. ■ Familiarize examinees with the style of the USMLE Step 2 CK examination. Students report that some test banks have questions that are, on average, shorter and less clinically oriented than those on the current Step 2 CK exam. Step 2 CK questions demand fast reading skills and the application of clinical

GUIDE TO EF FICIENT EXAM PREPARATION SECTION 1 facts in a problem-solving format. Approach sample examinations critically, and do not waste time with low-quality test bank questions until you have exhausted better sources. After you have taken a practice test, try to identify concepts and areas of weakness, not just the facts that you missed. Use this experience to motivate your study and to prioritize the areas in which you need the most work. Ana­ lyze the pattern of your responses to questions to determine if you have made systematic errors in answering questions. Common mistakes include reading too much into the question, second-guessing your initial impression, and mis­ interpreting the question.

TEXTS AND NOTES Most textbooks are too detailed for high-yield boards review and should be avoided. When using texts or notes, engage in active learning by making tables, diagrams, new mnemonics, and conceptual associations whenever pos­ sible. If you already have your own mnemonics, do not bother trying to mem­ orize someone else's. Textbooks are useful; however, they are best used to supplement incomplete or unclear material.

COMMERCIAL COURSES Commercial preparation courses can be helpful for some students, as they offer an effective way to organize study material. However, multiweek courses are costly and require significant time commitment, leaving limited time for independent study. Also note that some commercial courses are designed for first-time test takers, students who are repeating the examination, or interna­ tional medical graduates.

NBME/USMLE PUBLICATIONS We strongly encourage students to use the free materials provided by the test­ ing agencies and to study the following NBME publications: ■

■

■

USMLE Step 2 Clinical Knowledge (CK): Content Description and General Infonnation. This publication provides you with nuts-and-bolts details about the examination (included on the Web site http://www. usmle.org; free to all examinees). USMLE Step 2 Clinical Knowledge (CK): Sample Test Questions. This is a PDF version of the test questions and test content also found at http:// www.usmle.org under "Prepare for your exam". USMLE Web site (http://www.usmle.org). In addition to allowing you to become familiar with the CBT format, the sample items on the USMLE Web site provide the only questions that are available directly from the test makers. Student feedback varies as to the similarity of these questions to those on the actual exam, but they are nonetheless worthwhile to know.

TEST-DAY CH ECKLIST THINGS TO BRING WITH YOU TO THE EXAM ■

Be sure to bring your scheduling permit as a hard copy and a photo ID with signature. (You will not be admitted to the examination if you fail to bring your permit, and Prometric will charge a rescheduling fee.)

0-n

KEY FACT

13

Use test banks to identify concepts and areas of weakness, not just facts that you missed.

14

SECTION 1

GUIDE TO E F FICIENT EXAM PREPARATION ■ ■ ■ ■ ■ ■

■

Remember to bring lunch, snacks (for a little "sugar rush" on breaks), and fluids (including a caffeine-containing drink if needed). Bring clothes to layer to accommodate temperature variations at the testing center. Earplugs will be provided at the Prometric center. Remove all jewelry (eg, earrings, necklaces) before entering the testing center. Bring acetaminophen/ibuprofen, in case you develop a headache during the exam. Check the USMLE Web site (http://www.usmle.org/test-accommodations/ PIEs.html) for the personal item exception list to see if a medical device or personal item that you need is allowed into the testing facility without sub­ mitting a special request. ■ If you have a medical condition that requires use of an item NOT on the above list, contact the NBME personal item exception (PIE) coor­ dinator at [email protected] or (215) 590-9700 for addi­ tional information on how to request a personal item exception. If you need test accommodation for one of the following reasons: assis­ tance with keyboard tasks, audio rendition, extended testing time, addi­ tional break time, you need to fill in a request by going to the website https://www.usmle.org/step-exams/test-accommodations

TESTI N G AG ENCIES National Board of Medical Examiners (NBME) Department of Licensing Examination Services 3 7 50 Market Street Philadelphia, PA 19104-3102 Customer Service: (215) 590-9700, Front Desk: (21 5) 590-9 500 Fax: (215) 590-9460 http://www.nbme.org/contact/ e-mail: [email protected] USMLE Secretariat 3 7 50 Market Street Philadelphia, PA 19104-3190 (215) 590-9700 Fax: (215) 590-9460 http://www.usmle.org e-mail: [email protected] Educational Commission for Foreign Medical Graduates (ECFMG) 3624 Market Street Philadelphia, PA 19104-268 5 (215) 386-5900 Fax: (215) 386-9196 http://www.ecfmg.org/contact.html e-mail: [email protected] Federation of State Medical Boards (FSMB) 400 Fuller Wiser Road, Suite 300 Euless, TX 76039 (817) 868-4041 Fax: (817) 868-4098 http://www.fsmb.org/contact-us e-mail: [email protected]

DATABASE OF HIGH -YIELD FACTS Cardiovascular

Obstetrics

Endocrinology

Pediatrics

Dermatology

Gynecology

Epidemiology

Psychiatry

Gastrointestinal

Renal/Genitourinary

Health Systems Science Hematology

Musculoskeletal Neurology

Pulmonary

Multi system

Rapid Review

15

HOW TO USE THE DATABASE The 11th edition of First Aid for the USMLE Step 2 CK contains a revised and expanded database of clinical material that student authors and faculty have identified as high yield for boards review. We have organized informa­ tion according to subject matter, whether medical specialty (eg, Cardiovascu­ lar, Renal) or high-yield topic (eg, Health Systems Science). Each subject then branches out into smaller subsections of related facts. Individual facts appear in a logical fashion, from basic definitions and epi­ demiology to history/physical exam, diagnosis, and treatment. Lists, mnemon­ ics, pull quotes, vignette flash cards, and tables help the reader form key associations. In addition, we have interspersed color and black-and-white pho­ tos throughout the text. At the end of Section 2, we also feature a Rapid Review chapter consisting of key facts and classic associations that can be studied a day or two before the exam. The content contained herein is useful primarily for the purpose of reviewing material already learned. The information presented is not ideal for learning complex or highly conceptual material for the first time. The Database of High-Yield Facts is not meant to be comprehensive. Use it to complement your core study material, not as your primary study source. We have condensed and edited the facts and notes to emphasize essential material. Work with the material, add your own notes and mnemonics, and recognize that not all memory techniques work for all students. We update material to keep current with new trends in boards content, as well as to expand our database of high-yield information. However, we must note that our database inevitably does not include many other high-yield entries and topics. We actively encourage medical students and faculty to submit entries and mnemonics so that we may enhance the database for future students. We also solicit recommendations for additional study tools that may be useful in pre­ paring for the exam, such as diagrams, charts, and computer-based tutorials (see How to Contribute, p. xi).

DISCLAIMER The entries in this section reflect student opinions of what is high yield. Owing to the diverse sources of material, we have made no attempt to trace or reference origins of individual entries. We have regarded mnemonics as essentially in the public domain. We will gladly correct errors and omissions if brought to the attention of the authors, either through the publisher or directly by email.

CARDIOVASCU LAR Electrocardiogram

18

Dyslipidemia

55

Cardiac Physical Exam

22

Hypertension

57

Arrhythmias

25

BRADYARRHYTHMIAS A N D CONDUCTION ABNORMALITIES

TACHYARRHYTHMIAS

25 25

Cardiac Life Support Basics

34

Congestive Heart Failure

34

CLASSIFICATION SYSTOLIC DYSFUNCTION/HEART FAILURE WITH REDUCED EJECTION FRACTION HEART FAILURE WITH PRESERVED EJECTION FRACTION

Cardiomyopathy DILATED CARDIOMYOPATHY HYPERTROPHIC CARDIOMYOPATHY ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA RESTRICTIVE CARDIOMYOPATHY

34 35 39

40 41 41 42 43

SECONDARY CARDIOMYOPATHY

44

OTHER CARDIOMYOPATHIES

46

Coronary Artery Disease ANGINA PECTORIS PRINZMETAL (VARIANT) ANGINA

Acute Coronary Syndromes UNSTABLE ANGINA/NON-ST-SEGMENT ELEVATION MYOCARDIAL INFARCTION ST-SEGMENT ELEVATION MYOCARDIAL INFARCTION CAROTID ARTERY STENOSIS

47

47 49

PRIMARY (ESSENTIA L) HYPERTENSION SECONDARY HYPERTENSION HYPERTENSIVE EMERGENCY/URGENCY

Pericardia! Disease ACUTE PERICARDITIS CONSTRICTIVE PERICARDITIS PERICARDIAL EFFUSION

CARDIAC TAMPONADE

57 63 63

64 64 65 66 67

Endocarditis

67

Valvular Heart Disease

72

Vascular Diseases

75

AORTIC ANEURYSM AORTIC DISSECTION DEEP VENOUS THROMBOSIS POSTTHROMBOTIC ( POSTPHLEBITIC) SYNDROME PERIPHERAL ARTERIAL DISEASE LYMPHEDEMA

Syncope

75

76 78 80 81 82

82

49

49 51 55

17

18

H I G H -Y I E L D FACTS I N

CARDIOVASCULAR

ELECTROCARDIOGRAM

0-n

KEY FACT

*Heart rate = 300/number of large boxes between two consecutive QRS complexes. *Presuming ECG recorded at usual speed (25 mm/sec), where each large box = 200 msec and each small box = 40 msec.

An ECG provides an assessment of the electrical activity of the heart. The heart rate, rhythm, axis, intervals, ischemia, and chamber enlargement can be evaluated (Fig. 2.1-1).

Rate Normal adult heart rate (HR) is 60-100 beats/min (bpm). HR 100 bpm is tachycardia. Common causes of sinus bradycardia are physical fitness, sick sinus syndrome, drugs, vasovagal attacks, acute myocardial infarction (Ml), and i intracranial pressure. Com­ mon causes of sinus tachycardia are anxiety, anemia, pain, fever, sepsis, congestive heart failure (CHF), pulmonary embolism, hypovolemia, thyro­ toxicosis, carbon dioxide (CO2 ) retention, and sympathomimetics. Rhythm Sinus rhythm: Normal rhythm that originates from the sinus node. It is char­ acterized by a P wave (upright in leads II, III, and aVF; inverted in lead aVR) preceding every QRS complex and a QRS complex following every P wave. Sinus arrhythmia is a sinus rhythm originating from the sinoatrial (SA) node with cyclical beat-to-beat variation (> 120 milliseconds [ msec]) in the P-P interval and a constant P-R interval, which results in an irregular ventricular rate. It is common in young adults and is considered a normal variant. Axis

The QRS axis represents the direction in which the mean QRS current flows. It can be determined by examining the QRS in leads I, II, and aVF (see Table 2.1-1 and Fig. 2.1-2).

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Normal electrocardiogram from a healthy subject. (Reproduced with permission from

I I

CARDIOVASCULAR TA B L E 2 . 1 - 1 .

Axis Deviation by ECG Findi ngs

DEGREES

Mean left ventricular (LV) depolarization vector (red arrow) toward

Normal axis

leads I, 11, and aVF, resu lti ng in u pwa rd deflection (eg, positive ORS) in these leads

LV vector toward lead I (+ve ORS), away from lead aVF (-ve ORS)

Left axis deviation

Seen in ventricular tachycardia, inferior Ml, LV hypertrophy, left a nterior hemiblock

LV vector toward lead aVF (+ve ORS), away from lead I I (-ve ORS)

Right axis deviation

Seen in right ventricular hypertrophy, a nterolatera l Ml, left posterior hemi block (also consider p u l monary embolism)

LV vector opposing lead a VF and lead I I (both -ve ORS deflections)

Extreme axis

Some common causes include misplaced limb leads (reversa l of rig ht and left), ventricular rhythms, and ventricular paci ng

-90

1 80

°

°

//J ► ° + 1 20

aVF T + 90

F I G U R E 2 . 1 -2. USM LE-Rx.com.)

°

ECG axis interpretation. QRS axis and frontal leads. (Reproduced with permission from

H I G H -YI E L D FACTS I N

19

20

H I G H -YI ELD FACTS I N

0-,,. KEV FACT Smith-Modified Sgarbossa Criteria are used to diagnose Ml in the presence of LBBB should be suspected in a patient with LBBB and the fol lowi ng ECG findings: ■ Concordant ST elevation (STE) 2: 1 m m in 2: 1 lead ■ Concordant ST depression 2: 1 m m in 2: 1 lead ofV 1 -V3 ■ Excessive discordant STE in 2: 1 lead with 2: 1 mm STE, where excessive discordance is defi ned as STE to the maximum QRS amplitude ratio 2:25%

CARDIOVASCULAR

I nterva ls

■ PR interval: Normally 120 to 200 msec (3-5 small boxes). ■ Prolonged = delayed atrioventricular (AV) conduction (eg, first-degree heart block). ■ Short = fast AV conduction down accessory pathway (eg, WolffParkinson-White [WPW] syndrome). QRS interval: Normally 10 mm Hg with _ _ msp1rahon): Cardiac tamponade; pericardia! constriction; also seen in obstructive lung diseases (eg, severe asthma), tension pneumothorax, and foreign body in airway. ■ Pulsus alternans (alternating weak and strong pulses): Cardiomyopathy; impaired left ventricular systolic function (LVF). Poor prognosis. ■ Pulsus p arvus et tardus (weak and delayed pulse): Aortic stenosis. ■ Jerky: Hypertrophic obstructive cardiomyopathy (HOCM). ■ Pulsus bisferiens (bifid pulse/"twice beating"): Aortic regurgitation; combined aortic stenosis and aortic regurgitation, HOCM.

CARDIOVASCULAR

ARRHYTHMIAS BRADYARRHYTHMIAS AND CONDUCTION ABNORMALITIES

H I G H -Y I E L D FACTS I N

25

tnu

rJ Slo: pathway

AV � ode

Fast pathway

[lJ

If

AV �

node �

His B u ndle

Table 2.1-3 outlines the etiologies, clinical presentation, and treatment of common bradyarrhythmias and conduction abnormalities.

TACHYARRHYTHMIAS Tables 2.1-4 and 2.1-5 outline the etiologies, clinical presentation, and treat­ ment of common supraventricular and ventricular tachyarrhythmias.

Mechanism of Tachya rrhythmias The primary mechanisms of tachyarrhythmias include abnormal automatic­ ity, triggered activity, and re-entry. Abnormal automaticity: Myocardial tissue that does not normally pace the heart (ie, no automaticity) may develop automaticity due to pathologic mechanisms (eg, ischemia, metabolic disturbances). Examples include accelerated idioventricular rhythm or multifocal atrial tachycardia. ■ Tri ggered activity: Due to oscillations ( called afterdepolarizations) in the membrane potential of cardiomyocytes that occur during or immedi­ ately after an action potential. If afterdepolarizations cross a critical threshold, a new action potential is generated. Examples include torsade de pointes. ■ Reentry: Most common cause of tachyarrhythmias. Two distinct pathways connect to form a circuit. Conduction down this circuit could sustain a fixed and stable wavefront, which depolarizes the myocardium (Fig. 2.19). Examples include AVRT and AVNRT.

■

Atrial Fibril lation Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia. It results in disorganized atrial electrical activity and an irregularly irregular ven­ tricular rhythm. This causes increased rates of stroke, heart failure, and mor­ tality. See Table 2.1-4 for a summary. Risk Factors

■ AF is associated with increased age, hypertension, valvular disease, heart failure (HF), CAD, lung disease (PE, chronic lung disease, obstructive sleep apnea). ■ Other reversible causes such as hyperthyroidism, electrolyte derangements, and substance use (caffeine or drugs) should be excluded on initial evaluation. Pathophysiology

Often triggered by irregularly depolarizing cells near the ostia of the pul­ monary veins that result in disorganized atrial electrical activity. ■ This results in an irregularly irregular pattern of ventricular contractions. Chronic AF may results in structural changes to the atrium.

■

Classification

■ AF may be valvular (associated with mitral stenosis or prosthetic valve) or nonvalvular. ■ It may be new-onset/recurrent, paroxysmal (self-limited), persistent (> 7 days), long-standing (> 12 months), or permanent (not looking to restore sinus rhythm).

Normal conduction

Premature beat initiates re-entrant circuit

f

node

[!I �_n :� e f.

Conduction retrograde up the "fast" pathway

AV

I!] �

circuit

Reentry

ilU Re-entry circuit established

F I G U R E 2 . 1 -9. Mechan ism of re-entry at the AV node. The AV node has a

slow-conducting pathway with a short refractory period and a fast-conducting pathway with a long refractory period. During normal sinus rhythm, impulses are conducted down both pathways simultaneously but only transmitted to the His bundle down the fast pathway (myocardium is refractory once the slow impulse arrives) (A). A premature atrial contraction could arrive when the fast pathway is still refractory but the slow pathway is able to conduct (B). When the impulse from the slow pathway reaches the His bundle, the fast pathway is no longer refractory, thus allowing ret­ rograde conduction up the fast pathway (C). This impulse may continuously cycle through the fast and slow pathways, resulting in a reentry tachycardia, ie, AVNRT (D). Other reentrant tachycar­ dias may share a similar mechanism. ERP: Effective refractory period. (Repro­ d uced with permission from USMLE-RX.com.)

26

H I G H -YI ELD FACTS I N

TA B L E 2 . 1 -3.

CARDIOVASCULAR

Bradyarrhythm ias and Conduction Abnormal ities

TYPE/ETIOLOGY

SIGNS/SYMPTOMS

Sinus bradycardia

ECG findings: Sinus rhythm. Ventricular rate 200 msec

First-degree AV block

PR,

'-----'

PR,

'-----'

PR,

PR,

Can occur in normal individua ls; associated with i vagal tone, 13-blocker, or CCB

Asymptomatic

Second-degree AV block (Mobitz type 1/Wenckebach)

ECG findings: Progressive PR lengthening u nti l a

use

None necessary

d ropped beat occu rs (arrow); the PR interva l then resets

PR,


3 to 4 mm in diameter (a nor­ mal pericardia) space has 30-50 mL of fluid, and this space is :;;2 mm in diam­ eter). (B) Echocardiography showing pericardia) effusion (red arrows) . (Image A ada pted with perm ission from Bogaert J, Francone

M. Card iovascular mag netic resona nce i n pericardia! diseases. J Cardiovasc Magn Reson. 2009; 1 1 [1 ] : 1 4. doi: 1 0. 1 1 86/1 53 2-429X-1 1 - 1 4. Image B ada pted with permission from YousufT, Kramer J, Kopiec A, et al. A rare case of ca rd iac tamponade induced by chronic rheumatoid arthritis. J C/in Med Res. 201 5;7[9] :720-723. doi: 1 0. 1 47 40/jocm r2226w.)

A hypera ldoste ro n i s m wo rkup with seru m a l doste rone and ren i n l evel s is an a p p ro p riate next best d iag nostic ste p.

■ ■

Idiopathic Infectious (most common infection, likely etiology Coxsackie B virus, Staphylococcus, Streptococcus, tuberculosis [TB]) Connective tissue disorder (ie, systemic lupus erythematosus [SLE], rheu­ matoid arthritis, Goodpasture syndrome) Post-MI (either within days after MI or as a delayed phenomenon, ,e, Dressler syndrome) Uremia Neoplasms Drugs Radiation Trauma or open heart surgery (Fig. 2.1-24).

Acute pericarditis can compromise cardiac output via tamponade (extrava­ sation of large amounts of fluid secondary to pericarditis) or constrictive peri­ carditis (chronic pericarditis).

History/PE ■ Presentation: Sharp pleuritic chest pain, dyspnea, cough, and fever. ■ Key feature: Chest pain tends to worsen in the supine position and with inspiration. Classically, patient is seen sitting up (pain improves in prone position) and bending forward. ■ Exam: May reveal a pericardia! friction rub. Elevated JVP, tachycardia, muffled S 1 and S2 , and pulsus paradoxus (,J, in SBP > 10 mm Hg on inspi­ ration) can be present with pericardia! tamponade. Kussmaul sign can be present with constrictive pericarditis. Diagnosis ■ ECG: ■ Stage 1: Diffuse ST-segment elevation (concave or saddle shaped) and PR-segment depressions. ■ Stage 2: Normalization of ST segment and PR-segment changes. ■ Stage 3: Development of diffuse T-wave inversions (Fig. 2.1-25). Typi­ cally seen after ST segment becomes iso-electric. ■ Stage 4: Normalization of ECG. ■

CXR: Cardiomegaly may indicate a pericardia! effusion.

CARDIOVASCULAR

H I G H -YI E L D FACTS I N

65

0-,,. KEV FACT

Pericardia! calcification seen on CXR strongly suggests constrictive pericarditis due to chronic fi brosis and calcification of the pericardium.

F I G U R E 2 . 1 -25. Acute pericarditis. Diffuse ST-segment elevations in multiple leads not con­ sistent with any discrete coronary vascular territory and PR-segment depressions. (Reproduced with perm ission from USM LE-Rx.com.)

■ Blood tests: Full blood count (FBC), ESR, urea and electrolytes (U&Es), cardiac enzymes (troponin may be raised), viral serology, and if indicated, autoantibodies, fungal precipitins, and thyroid function tests (TFTs). ■ Echo: Pericardia! thickening or effusion may be evident.

Treatment Goal of treatment is pain relief, resolution of inflammation, and prevention of recurrence. ■

■ ■

■

■

Activity restriction: Patients should be instructed to restrict strenuous phys­ ical activity until symptoms have resolved and biomarkers have normalized. NSAIDs + colchicine: For patients with idiopathic or viral pericarditis. Glucocorticoids should be used for initial treatment of acute pericarditis only in patients with contraindications to NSAIDs or for specific indica­ tions (SLE, renal failure). Avoid corticosteroids within a few days after Ml, as they can predispose to ventricular wall rupture. For patients in whom etiology for pericarditis is known, specific treatment for the underlying cause is indicated (dialysis in uremia, ASA for post-MI pericar­ ditis, corticosteroids/immunosuppression for SLE-related pericarditis). When acute symptoms are resolved, NSAIDs are tapered weekly to reduce recurrence rate, and colchicine is used for a total duration of 3 months.

CONSTRICTIVE PERICARDITIS Constrictive pericarditis is the result of scarring and consequent loss of the normal elasticity of the pericardia! sac. Typically, it is chronic in nature.

History/PE ■ Presents with symptoms suggesting fluid overload (edema of lower limbs and ascites) and low cardiac output (fatiguability and dyspnea on exertion). ■ Clinical exam: JVD, hepatosplenomegaly, Kussmaul sign, and pericardia! knock (produced from heart hitting the calcified pericardium in diastole).

0-,,. KEV FACT

ST-segment elevations in pericarditis are differentiated from Ml in that they are not localized to one region of the heart; widespread ST-segment elevations are seen.

0-,,. KEV FACT

Consider uremic pericarditis in a patient with end-stage renal disease and azotemia. It is characterized by prominent fi brin deposition but no epicardial inflammation; thus classical ECG changes (diffuse ST elevation, PR depression) are not seen. Treatment is with hemodialysis.

66

H I G H -YI E L D FACTS I N

CARDIOVASCULAR

Diagnosis

■ ■ ■

Echocardiography: Should see septa] bounce, respiratory septa] shift, peri­ cardia] thickening, and a decrease in ventricular filling flow during inspiration. CXR: May show calcifications around the cardiac silhouette. CT scan shows thickened pericardium. ECG may show low-voltage complexes.

Treatment

■ ■ ■

Diuretics to control edema and ascites. Medical management with NSAIDs and colchicine if features are mild. Pericardia} stripping (ie, surgical removal of the pericardium) is the definitive management. Performed if patient fails medical management or if he or she has severe symptoms.

PERICARDIAL EF FUSION Pericardia] effusion is characterized by an increased amount of fluid in the pericardia] cavity. Many patients with pericardia] effusion are asymptomatic, and the effusion is detected incidentally. Common causes include idiopathic (most common), malignancy, infections, autoimmune disease, hypothyroid­ ism, ascending aortic dissection, anticoagulants, and medication. In countries where TB is endemic, more than 60% of effusions are due to TB. History/PE

■ ■

F I G U R E 2 . 1 -26.

Pericardia! effusion.

Water bottle-shaped heart seen on CXR with pericardia) effusion. (Reproduced with permission from Chen MY et al. Basic Radiology, 2nd ed. New York, NY: McGraw-H ill; 201 1 .)

■ ■

Diagnosis

■ ■ ■

F I G U R E 2.1 -21. Electrical alternans, where voltage of QRS changes with each beat. If seen, diagnostic of peri­ cardia! effusion. (Modified with perm ission from Maharaj SS, Chang SM. Cardiac tamponade as the initial presentation of systemic lupus erythe­ matosus: A case report and review of the literature. Pediatr Rheumatol Online J. 20 1 5; 1 3:9. doi: 1 0. 1 1 86/ s 1 2969-0 1 5-0005-0.)

Patient may present with signs suggestive of acute pericarditis. Important to obtain history for recent illnesses, malignancy, history of TB and vaccination status, autoimmune disorders, history of CKD or renal failure, or history of CHF, hypothyroidism, or liver disease. Physical exam may show muffled/distant heart sounds. Ewart sign/dullness to percussion at the base of the left inferior scapular border in conjunction with tubular breath sounds and egophony.

CXR may show an enlarged, globular, water bottle-shaped heart with a large effusion (Fig. 2.1-26). Cardiac echo may show increased fluid in pericardia] space. On CT imag­ ing, pericardia] effusion is diagnosed when the pericardia] space is > 3 to 4 mm in diameter (a normal pericardia] space has 30-50 mL of fluid and is equal to or less than 2 mm in diameter). If present on ECG, electrical alternans (due to swinging motion of heart in fluid filled cavity) is diagnostic of a large pericardia] effusion (Fig. 2.1-27).

Treatment

■ ■

Treatment focused on the cause of the disease. In patients with pericardia] effusion of unknown cause and elevated inflammatory markers, empiric treatment for pericarditis is reasonable. If cancer or bacterial infection is suspected, pericardiocentesis with or without a pericardia] biopsy is recommended.

CARDIOVASCULAR

■

■

H I G H -YI E L D FACTS I N

67

If idiopathic effusions are present for more than 3 months, pericardia! drainage should be considered, as the likelihood to convert into cardiac tamponade is high in these individuals. In patients with symptoms suggestive of cardiac tamponade, immediate pericardia! drainage is advised (Cardiac Tamponade section to follow).

CARDIAC TAMPONADE Excess fluid in the pericardia! sac i the intrapericardial pressure, leading to compromised ventricular filling and .J, cardiac output. The rate of fluid for­ mation is more important than the size of the effusion. Risk factors include pericarditis, malignancy, SLE, TB, and trauma (commonly stab wounds medial to the left nipple).

History/PE ■ Presents with fatigue, dyspnea, anxiety, tachycardia, and tachypnea that can rapidly progress to shock and death. ■ Exam of a patient with acute tamponade may reveal Beck triad (hypoten­ sion, distant or muffled S 1 and S2 heart sounds, and JVD), a narrow pulse pressure, and pulsus paradoxus. ■ Lung fields are clear on exam (high yield and frequently tested in CK). Diagnosis ■ Echo is diagnostic and shows right atrial and right ventricular diastolic col­ lapse and echo-free zone around the heart. ■ Right heart catheterization will show equalization of all the pressures (RA, RV, pulmonary capillary wedge pressure [PCWP]) in the heart during diastole. Treatment ■ Aggressive volume expansion with IV fluids. ■ Urgent pericardiocentesis (aspirate will be nonclotting blood) with a peri­ cardia! drain. Send fluid to lab analysis to determine etiology. ■ If significant drainage continues for more than 3 to 4 days or if effusions are recurrent, a pericardia! window should be considered.

ENDOCARDITIS Endocarditis is inflammation of the endocardium, the inner layer of the heart overlying the valves. It has noninfective (NBTE) and infective (bacteria/fungi) causes. Infective endocarditis (IE) is the consequence of bacteremia (dental procedures, injections, surgery) and usually causes tricuspid, mitral, and aor­ tic valve lesions, resulting in regurgitation. Acute IE occurs over hours to days, whereas subacute IE progresses over weeks to months. Causes are dis­ cussed in Table 2.1-24.

0-,,. KEY FACT Beck triad ca n diag nose acute cardiac ta m ponade: ■ JVO ■ H ypotension ■ D i stant hea rt sounds

68

H I G H -YI ELD FACTS I N

TA B L E 2 . 1 -24.

CARDIOVASCULAR

Etiologies of Endocarditis

NATIVE VALVE

P ROSTH ETIC VALVE (HIGHEST RISK)

IVDUS

NON- BACTERIAL THROM BOTIC EN DOCARDITIS (N BTE)

Viridans streptococci (most com mon,

> S aureus)

>60 days surgery:

Late-onset causes

(Streptococci)

Enterococci Streptococci Fungus (Candida, Asper­

gillus [may see in H IV/ AIDS patient))

Pseudomonas

Hypoxia

Hypercoagulability

Ca rcinomatosis

Key terms of NBTE:

Ca ncer/i l l nesses ➔ Mara ntic N BTE

Lupus ➔ verrucous or Li bman-Sacks N BTE (both sides of valve')

GU procedure)

Culture 0 organisms, Coxiella bur­

Key presentation/finding:

netii (fa rm exposu re), Bartone/la

Asym ptomatic, found on a utopsy. Symp­

quintana/henselae (lice/cat), Cuti­ bacterium acnes Bruce/la (fa rm),

tomatic ➔ sterile lesions of thrombus + immune complexes on left-sided

va lves (most com mon) embolize ➔

Mycobacteria (aerosolized particles),

systemic circulation.

Tropheryma whipplei (farm exposu re) HACEK (no longer culture [-) causes of IE with modern culture tech niques)

I m m u n e complexes

Treatment: Anticoagu lation

• N BTE and infective ca uses of endoca rditis usually infect and cause lesions on one side of the va lve.

Select organisms from Table 2.1-24: Staphylococcus aureus: Gram/catalase/coagulase EB cocci ➔ Use of IV drugs may result in cocci from skin being introduced into veins ➔ high virulence ➔ attacks normal right-sided heart valves (tricuspid valve ➔ tri­ cuspid regurgitation). Most common cause of acute IE in all patients (fatal without treatment in 6 weeks). Infects any heart valve (Fig. 2. l -28A). ■ Staphylococcus epidennidis: Gram/catalase EB and coagulase 8 cocci of skin flora. Low virulence ➔ usually need prosthetic heart valves to cause IE. Viridans streptococci (S mitis, S mutans, S sanguinis): Gram EB and cata­ lase 8 cocci of oral flora. Most common cause of subacute endocarditis. Dental procedure ➔ bacteremia ➔ low virulence ➔ Viridans produce dextrans ➔ dextrans adhere to fibrin on damaged valve ➔ IE. Streptococcus gallolyticus (S bovis type 1) and Clostridium septicum are gram EB cocci and rods, respectively, normally found in the GI tract. If either one causes IE, a colonoscopy is indicated because both are associ­ ated with colonic pathology, especially colon cancer. ■ Enterococci: Gram EB cocci normally found in the GI tract. After GI or genitourinary (GU) procedures (abdominal surgery, urinary catheter, transurethral resection of prostate for benign prostatic hypertrophy [BPH]) ➔ subacute endocarditis in older men.

CARDIOVASCULAR

H I G H -Y I E L D FACTS I N

69

■ HACEK: Haemophilus species (subsequently called Aggregatibacter aphrophilus + Aggregatibacter paraphrophilus); Actinobacillus actinomy­ cetemcomitans (subsequently called Aggregatibacter actinomycetemcomi­ tans); Cardiobacterium hominis; Eikenella corrodens; and Kingella kingae. Normal flora of oropharyngeal region that typically cause subacute IE associated with poor dental hygiene/procedures. No longer considered important causes of culture 8 IE with modern culture techniques. ■ Candida + Aspergillus fumigatus: Most common causes of fungal endo­ carditis typically in immunocompromised patients (HIV/AIDS, trans­ plant), IV drug use (IVDUs), long-term IV catheter use. ■ Culture negative IE: Most common manifestation of chronic Q fever (Coxiella bumetii) . Bartonella quintana Gram 8 rod ➔ lice spread ➔ individuals with poor hygeine.

History/PE ■ IE should be suspected in any patient with unexplained fever(s) :±: bacte­ remia and a new regurgitant heart murmur of the tricuspid, mitral, or aor­ tic valve. Fulminant HF due to severe regurgitant lesions may also occur. ■ Septic emboli: Coronary artery (Ml), brain (stroke), spinal cord (paraly­ sis), eye (blindness), extremities (septic arthritis), splenic/renal infarctions, PE (from tricuspid lesions), mycotic aneurysm, abscesses, Janeway lesions (painless erythematous microabscesses on palms/soles). ■ Immune phenomena: Eyes (Roth spots = oval red retinal lesions with clear/pale center; see Fig. 2.1-28B), glomerulus (signs/symptoms of glo­ merulonephritis), Osler nodes (painful/raised lesions on digits/feet; "Ouchler nodes," see Fig. 2. l -28C), splinter hemorrhages (vasculitis underneath fingernails that follows direction of growth, see Fig. 2.1-280), rheumatoid factor. ■ Paravalvular abscess: Persistent fever + bacteremia despite treatment ➔ paravalvular abscess formation. Notably, paravalvular abscess of aortic valve ➔ new-onset AV block on ECG.

F I G U R E 2 . 1 -2s.

l,)Q MNEMONIC Presentation of Infective EndocarditisFROM JANE with • Fever Roth spots Osler nodes (painful, "Ouchler nodes") Murmur Janeway lesions ("Mary Jane is painless") Anemia of chronic disease Nail hemorrhage Emboli

Features of infective endocarditis. (A) Large vegetations due to Haemophilus parainfl,uenzae; (B) Roth spots on the retina;

Osler nodes; (D) splinter hemorrhages.

(C)

(Image A adapted with permission from The US Department of Health and H u m a n Services and Dr. Edwin P. Ewing, Jr. Image C Ya ng

ML, Chen YH, Lin WR, et al. Case report: infective endocarditis caused by Brevundimonas vesicularis. BMC Infect Dis. 2006;6: 1 79. DOI: 1 0. 1 1 86/ 1 47 1 -2334-6- 1 79. Images B and D reprod uced

with permission from USM LE-Rx.com.)

H I G H -Y I E L D FACTS I N

70

CARDIOVASCULAR

Diagnosis ■ ■ ■

■

(t MNEMONIC Duke Criteria Mnemonic: "BE Duke tivor" Step 1: ■ Capital letters = Major Criteria. ■ Lowercase letters = minor criteria. ■ The number/type of letters in each word then represents a diagnostic category: ■ Two Major Criteria (BE). ■ One Major + three minor (Duke) or five minor criteria (fivor)

Step 2

■ BE: (Bacteremia [blood cultures] + Endocardial involvement [echo or murmur]) = Major Criteria ■ Duke (diagnostic category, name of criteria) ■ fivor: (fever, immune phenomena, vascular phenomena, organism cultures not meeting major criteria, risk factors) = minor criteria

TA B L E 2 . 1 -2s.

■

CBC with leukocytosis and left shift,i ESR and C-reactive protein (CRP) may be seen. Duke clinical criteria (see mnemonic and Table 2.1-25). Two major, one major + three minor, or five minor criteria must be present to diagnose IE. Best initial test: Blood culture (9 5 %-99% sensitive). Best imaging test: TEE (9 5 % sensitive/specific) > TTE (60% sensitive, 95 %-100% specific) in diagnosing endocarditis. Most patients should first get a TIE as initial screening test. ECG not routinely used. If treatment failures, aortic paravalvular abscess ➔ new AV block.

Treatment

■ General guidelines: At least two, preferably three, sets of blood cultures should be drawn prior to initiating antibiotics, generally even in acutely ill patients. If empiric antibiotics are given for native or prosthetic valve endo­ carditis, the best initial treatment is vancomycin :±: gentamicin or another antibiotic. Blood culture results are used to tailor therapy. Antibiotics are delivered parenterally for 4 to 6 weeks for left-sided lesions and 2 weeks for right-sided lesions, and are targeted toward the specific microorganism (Table 2.1-26). ■ Surgical considerations: Early surgical consultation + intervention is con­ sidered with ■ Valvular damage ➔ acute HF ■ Fungal IE ■ Left-sided IE with highly resistant microbes (eg, methicillin-resistant S aureus [MRSA]) ■ Persistent fever or bacteremia 5 to 7 days post-antibiotic treatment ■ Prosthetic valves ■ Antibiotics cannot penetrate large vegetations: Vegetation > 15 mm or vegetation > 10 mm + systemic emboli ■ Perivalvular extension (eg, paravalvular abscess: aortic paravalvular abscess ➔ new-onset AV block), pseudoaneurysm, or fistula formation

Duke Clinical Criteria for the Diagnosis of I nfective Endocarditis

CRITERIA

COM PON ENTS

Major

1 . Bacteremia (one of the following): two separate EB blood cultures of typical I E organisms (Staphylococcus, Strep­ tococcus, enterococci, HACEK) or persistently EB blood cultures (at least two samples drawn > 1 2 hours apart for typica l IE organisms or th ree or majority of >4 separate blood cultures for typical skin contaminant organisms with the first and last drawn at least 1 hour apart) or single EB blood culture or phase lgG a ntibody titer for Coxi­

MNEMONIC BE

ella burnetii. 2. Endocardial involvement (one of the following): echocardiogram evidence of vegetation, abscess, valve perfo­ ration, or prosthetic dehiscence or new valvu lar regu rgitation murmur M inor

1. Fever 2:38°C ( 1 00.4° F) 2. Immune phenomena: g lomerulonephritis, Osler nodes, Roth spots, rheu matoid factor 3. Vascular phenomena: septic arterial/pulmonary emboli, mycotic a neurysm, intracranial/conjunctival hemor­ rhages, Janeway lesions 4. Organism cu lture not meeting major criteria or serologic evidence of active infection with I E organism 5. Risk factors: abnormal risk of bacteremia (IVDUs) or abnormal heart (prosthetic valve or lesion with significant regurgitation)

fivor

CARDIOVASCULAR TA B L E 2 . 1 -26.

Targeted Treatment Regimens for Infective Endocarditis

BLOOD C ULTURE EB FOR . . .

POSSI BLE TREATM ENT REG I M E N S

Methicillin-susceptible staphylo­

Oxacillin, nafcillin, or cefazolin ➔ native valve

cocci (coagulase 0 or (±))

Oxacillin or nafcillin :t gentamicin, rifampin ➔ prosthetic valve

Methicillin-resistant staphylococci (coagulase 0 or (±))

Vancomycin ➔ native valve Vancomycin :t gentamicin, rifampin ➔ prosthetic valve

Fungus

Amphotericin + valve replacement

Enterococcus spp.

E faecalis (usually penicillin sensitive) ➔ ampicillin + gentamicin E faecium (usually penicillin resistant) ➔ vancomycin + gentamicin

Viridans streptococci, S.

Ceftriaxone :t gentamicin

gallolyticus HACEK

First line is ceftriaxone

BLOOD C ULTURE 8 FOR . . . Coxiella, Bartone/la

Ceftriaxone

Prophylaxis Prophylactic antibiotics may be indicated to prevent IE in certain patients at high risk of poor outcomes. These are discussed in Table 2.1-27.

TA B L E 2 . 1 -21.

Indications for Endocarditis Antibiotic Prophylaxis

MUST HAVE O N E QUALIFYI N G CA RDIAC I N DICATION

A N D MUST HAVE ONE QUALIFYING PROCEDURE I N D I CATION

Prosthetic heart valve(s) History of infective endocarditis (scarring i vulnerability) Congenital heart disease (unrepaired cya­

Dental ➔ bleeding (even cleanings) Respiratory ➔ bleeding (biopsy/incision) Skin/musculoskeletal tissue ➔ bleeding (biopsy/incision)

notic CHD or repaired with prosthetic material in last 6 months) Cardiac transplant with valvulopathy

Cardiac surgery with prosthetic material

If patient has one qualifying cardiac + one qualifying procedure indication ➔ antibiotic prophylaxis is recommended 30 to 60 minutes prior to indicated procedure (eg, amoxicillin is first-line for dental procedure; al lergy ➔ macrolide, cephalexin, or doxycycline). Prophylaxis is NOT recommended for native mitral stenosis/mitral valve prolapse or routine GI endoscopy or GU cystoscopy.

H I G H -YI E L D FACTS I N

71

72

H I G H -YI ELD FACTS I N

VALVULAR HEART DISEASE

0-,,. KEY FACT

Mitral regurgitation classically occurs secondary to posteromedial papillary muscle rupture 2 to 7 days after a posterior descending coronary artery Ml due to its singular blood supply. Conversely, anterolateral papillary muscle rupture is 6 to 1 2 times less likely due to its dual blood supply from the left anterior descending + left circumflex arteries.

0-,,. KEY FACT

Many cardiovascular conditions have an increased risk during pregnancy. For example, pregnancy is not advised with severe obstructive valvular lesions (mitral stenosis, aortic stenosis), symptomatic HF with EF 60 years of age

Asymptomatic: Years despite severe stenosis

Severity categorized based on

Sym ptomatic: Angina, Syncope, CHF (ASC), in order of worsening prognosis without treatment

pressure gradient across aortic valve or valve area into mild (1 5-25 mm Hg, >1 .5 cm'), mod­

Bicuspid AS

Murmur: Harsh systolic crescendo-decrescendo murmur, heard best at the second right inter­ costal space, radiates to carotids, paradoxical splitting due to delayed LV outflow

erate (25-40 mm Hg, 1 .0-1 .S cm'), severe (>40 mm Hg,

Congenital, Tu rner disease Dystrophic calcification of a bicuspid valve replacing

increase afterload Tricuspid Regurgitation (TR)

Tricuspid endocarditis (IV drug use) Ebstein anomaly (con­

genital downward displacement of tricuspid valve ➔ RV); carcinoid syndrome (see later), lu pus, myxomatous degeneration Normal: Up to 70% of

adults have physi­ ologic TR; majority are

Signs/Symptoms

Diagnosis

Majority asymptomatic ➔ symptoms manifest

TIE is best initial test; can assess

with pulmonary HTN➔ right HF and may include ascites, pulsatile/enlarged liver, edema, prominent V waves + rapid y descent in jugular venous pulse, JVD; right atrial dilatation ➔ AF Murmur: Sound ofTR murmur = seconddegree MR murmur (holosystolic) but is at lower-left sternal border Increase murmur = i venous return via inha lation (like sipping a straw) + leg raise

pulmonary pressures via TR velocity Treatment

Treat u nderlying cause Diuretics for congestion Surgery for severe + symptomatic TR without pulmonary HTN

asymptomatic RV dilation: LV failure (most

common), RV Ml, inferior wall Ml, pulmonary HTN ➔ cor pulmonale. Right-sided valve disease: Murmur-like left-sided counterpart with different listening area. Tricuspid/pulmonic stenosis/regurgitation is caused by carcinoid heart disease (tumor releases serotonin ➔ neutralized in lungs = only right-sided valves coated/fi brosed). TR: classically endocarditis in IVDU, RV enlargement. PR classica lly i n TOF patient years after repair.

CARDIOVASCULAR

H I G H -YI E L D FACTS I N

75

VASCULAR DISEASES AORTIC ANEURYSM Greater than 50% dilation of all three layers of the aortic wall. Aortic aneu­ rysms are most commonly associated with atherosclerosis. Most are abdomi­ nal, and >90% originate below the renal arteries. ■ Etiologies: Degeneration (atherosclerosis, fibromuscular dysplasia), infec­ tion (syphilis), trauma, inflammation (Takayasu), connective tissue dis­ eases (Marfans, Ehlers-Danlos syndrome), and congenital (Turner syndrome, tuberous sclerosis) ■ Ascending aortic aneurysm - think cystic medial necrosis or connec­ tive tissue disease ■ Descending aortic aneurysm - think atherosclerosis ■

Complications: Rupture, thrombosis, embolism, fistulae, pressure on sur­ rounding structures

History/PE

■

Usually asymptomatic and discovered incidentally on exam or radiologic study. It may cause mild abdominal or back pain. Less frequently, those with symptomatic but unruptured aneurysms may present with signs of limb ischemia (acute or chronic) or systemic symptoms (fever, malaise). ■ Exam can demonstrate a pulsatile abdominal mass or abdominal bruits. ■ Risk factors include HTN, high cholesterol, other vascular disease, a EB family history, smoking (strongest predictor of rupture), gender (males > females), and age. ■ Ruptured aneurysm leads to hypotension and severe, tearing abdominal pain that radiates to the back, iliac fossae, or groin and syncope.

0-,r KEY FACT AAA is genera l l y defi ned as abdo m i n a l aortic d i l ation i n ad u lts > 3 . 0 cm.

Diagnosis

■

Screening: All men 65 to 7 5 years of age with a history of smoking are rec­ ommended for a one-time screening by ultrasound for AAA (Fig. 2.1-29). Figure 2.1-30 suggests an algorithm for the diagnosis and initial manage­ ment of AAA. ■ Abdominal ultrasound is used for diagnosis or to follow the course of an aneurysm over time.

I

F I G U R E 2 . 1 -29. Abdominal aortic aneurysm. (A) Ultrasound image of an AM (Ao, Aorta). (B) Transaxial image from a contrast-enhanced CT showing an aneurysm with extensive mural thrombus (arrowhead) . (A reproduced with permission from Tinti nalli JE et al. Tintinalli's Emergency Medicine: A Com­

prehensive Study Guide 6th ed. New York, NY: McGraw-Hill; 2004. B reprod uced with permission from Doherty GM. Current Diagnosis & Treatment: Surgery 1 3th ed. New York, NY: McGraw-Hill; 201 0.)

A 70-yea r-old m a n with HTN presents for a ro ut i n e a ppoi ntment. He q u it smoki n g 20 years ago but has a 20-pack-yea r h i story. What scree n i ng, if any, is i n d icated ?

76

H I G H -YI ELD FACTS I N

CARDIOVASCULAR Suspected symptomatic abdominal aortic aneurysm (AAA) Abdom inal, fla nk, or back pain, pu lsatile abdominal mass, +/- fla n k ecchymosis

!

Assess hemodynamic sta bi lity Stable

CT abdomen

Un ruptured

Cardiovascular evaluation U rgent endovascula r or surgical repa i r if the lesion is >5.5 cm

F I G u RE 2. 1 -30.

0-rr KEY FACT Size of AAA determines treatment: ■ 5 cm ➔ surgical correction

0-rr KEY FACT Rapidly expanding aortic aneurysms are defi ned as >5 m m increase in size in 6 months or > 1 0 mm increase in size in 1 2 months.

0-rr KEY FACT Aortic a neurysm is most often associated with atherosclerosis, whereas aortic dissection is commonly linked to HTN.

The U n ited States Preventive Services Task Force (USPSTF) g u idel i nes recommend one-time screen i ng for AAA by u ltrasound in men 65 to 75 years of age who have ever smoked.

Ruptured

Unstable

H i story of AAA

Yes

No

U ltrasound abdomen

AAA present

Emergent endovascular or surgical AAA repa i r

AAA absent

Eva luate cause of sym pto ms

Diagnosis of suspected abdominal aortic aneurysm (AAA). (Reproduced with per­

mission from USMLE-Rx.com.)

■ CT with contrast or magnetic resonance angiography (MRA) may be use­ ful to determine the precise anatomy.

Treatment ■ In asymptomatic patients, monitoring is appropriate for lesions Surgical or endovascular correction is indicated if the lesion is 2': 5. 5 cm (abdominal), >6 cm (thoracic), or smaller but rapidly enlarging (watch for bowel ischemia and infarction). ■ Emergent surgery for symptomatic or ruptured aneurysms.

AORTIC DISSECTION A transverse tear in the intima of a vessel that results in blood entering the media, creating a false lumen and leading to a hematoma that propagates lon­ gitudinally. Most commonly secondary to HTN, but also due to blunt chest trauma. The most common sites of origin are above the aortic valve and distal to the left subclavian artery. Most often occurs at 40 to 60 years of age, with a greater frequency in males than in females.

H istory/PE ■ History: HTN, Marfan syndrome, mitral valve prolapse, trauma ■ Presentation: Sudden tearing/ripping pain in the anterior chest (ascend­ ing) with or without radiation to the back (descending), typically between the scapulae PE: ■ Patients are typically hypertensive. If hypotensive, consider pericardia! tamponade, hypovolemia from blood loss, or other cardiopulmonary etiologies.

H I G H -YI E L D FACTS I N

CARDIOVASCULAR Asymmetric pulses and BP measurements or acute limb ischemia. A murmur of aortic regurgitation may be heard if the aortic valve 1s involved with a proximal dissection. ■ Neurologic deficits, such as paraplegia, may be seen if the aortic arch or spinal arteries are involved. ■ Anuria may be seen if renal arteries are involved. ■ Signs of pericarditis or pericardia! tamponade may be seen. ■

Diagnosis ■ ■ ■

■ ■ ■

Aortic dissection suspected based on history and physical exam findings. Best initial test for hemodynamically stable patients: CT angiography. MRA can be used if contrast CT is contraindicated. TEE. Visualization of an intimal flap as well as a false lumen is diagnostic. It may also be used to visualize details of the proximal aorta and coronary vessels and can also evaluate for pericardia! effusion. The Stanford system classifies any dissection proximal to the left subcla­ vian artery as type A and all others as type B (Fig. 2.1-31). See diagnostic algorithm in Fig. 2.1-32. Type A (~70%) is the most common and involves the ascending aorta, irrespective of the site of the tear. Type B does not involve the ascending aorta.

Stanford Classification

0-n

KEY FACT

Ascending aortic dissections are surgical emergencies; descending dissections are still emergencies but can often be treated medically.

Suspected aortic dissection

j

I nitial eva luation: h istory, physical exam, labs, ECG, CXR suggestive of aortic dissection

Ascending

No

Eva luate for other diag noses and treat appropriately

F I G U R E 2 . 1 -3 1 .

Stanford classification of aortic dissec­

tion. Type A involves the ascending aorta and may progress

to involve the arch and thoracoabdominal aorta. Type B involves the descending thoracic or thoracoabdominal aorta distal to the left subclavian artery without involvement of the ascending aorta. (Reproduced with permission from USMLE-Rx.com.)

I

Yes

Determine appropriate diag nostic imaging Hemodynamically

Hemodynamically

unstable

stable

CTA If contrast CT contraindicated, MRA

TEE If not available, CTA

Diagnosis confirmed

Control heart rate and blood pressure with (3 - blocker Surgical consult Type A

Emergency surgery

F I G U R E 2 . 1 -32.

I

Type B

Evidence of ischemia No

Continue medical management

Yes

U rgent surgery

Diagnosis of suspected aortic dissection.

(Reproduced with permission from USM LE-Rx.com.)

77

78

H I G H -YI ELD FACTS I N

CARDIOVASCULAR Treatment ■ BP control: Important to monitor and medically manage BP and heart rate as necessary. Avoid thrombolytics. Begin IV 13-blockers (eg, IV labet­ alol) before starting vasodilators (nitroprusside) to prevent reflex tachycar­ dia. BP goals in the management of aortic dissection aim to decrease shear stress on the dissection, but maintain adequate organ perfusion with sys­ tolic blood pressure of 100 to 120 mm Hg. Heart rate goal is 15 seconds is seen in severe ischemia. ■ Acute ischemia: May be due to thrombosis in situ (most common), emboli (usually of cardiac origin), graft/angioplasty occlusion, or trauma. Acute occlu­ sions commonly occur at bifurcations distal to the last palpable pulse (see mnemonic for signs and symptoms). May also be secondary to cholesterol atheroembolism ("blue toe syn­ drome"), which is characterized by blue toes, livedo reticularis, and renal failure (often secondary to catheterization). ■

Chronic ischemia: Lack of blood perfusion leads to muscle atrophy, pal­ lor, loss of sweat and sebaceous glands, cyanosis, hair loss, and gangrene/ necrosis.

Diagnosis ■ Identify cardiovascular risk factors, especially smoking, diabetes, HTN, and hyperlipidemia. ■ Best initial test: Ankle-brachia! index (ABI) test (1-1.4 is normal); can provide objective evidence of atherosclerosis ( :s0.9 is highly sensitive and specific for PAD, rest pain usually occurs with an ABI 90% of brachia! readings. ■ Most accurate test: Angiography (invasive); computed tomography angi­ ography (CTA) with runoff (noninvasive); often not necessary unless revas­ cularization is indicated. Treatment ■ Treat acute symptomatic ischemia with heparin and prompt revascularization. ■ Smoking cessation (vital); optimally treat HTN, hyperlipidemia, and diabetes. ■ Educate regarding careful hygiene and foot care. Exercise helps develop collateral circulation.

0-n

KEY FACT

Critical limb ischemia (chronic limb­ threatening ischemia): Presence of

PAD in combination with pain at rest, gangrene, or a lower limb ulceration > 2 weeks duration

:0-

MNEMONIC

The 6 Ps of acute ischemia­

Pai n Pallor Paralysis Pulse deficit Paresthesias Poikilothermia

0-n

KEY FACT

0-n

KEY FACT

Rest pain seen with an ABI 3 seconds) (continues)

CARDIOVASCULAR TA B L E 2 . 1 -29.

H I G H -YI E L D FACTS I N

85

Selected Syncope Syndromes (contin ued)

PATIENT PRESENTATION

MOST LI KELY DIAGNOSIS

WORKUP

MANAGEMENT

Orthostatic chal lenge: Change i n BP from supine to erect posture can be evaluated in several ways:

■ Education and reassura nce ■ Expa nsion of extracel lular vol u me: Maintain hydration, avoid extreme heat, increased sodium i ntake

■ Active standing test ■ Head-up tilt-table test

■ Stop or reduce antihypertensive treatments ■ Counterpressure maneuvers when

O RTHOSTATIC SYNCOPE Syncope on change in body postu re with history of drug use that is a ssoci­ ated with orthostasis (eg, alcohol, vasodilators, diuretics, phenothiazine,

Volume depletion

antidepressants) Syncope with volume loss such as hem­ orrhage, diarrhea, vomiting Syncope with neurologic diseases such as pure autonomic failure, m u ltiple

Primary autonomic failure

system atrophy, Parkinson disease, or dementia with Lewy bodies

Syncope with history of diabetes, amyloidosis, spinal cord injuries,

Secondary auto­ nomic failure

autoimmune autonomic neuropathy, paraneoplastic autonomic neurop­ athy, kidney failure

BP monitoring (ABPM) may allow assessment of BP with changes in postures or on performing maneuvers Autonomic function: To detect autonomic failure as cause: Valsalva maneuver Deep breathing test: Reduced variability in H R may suggest autonomic dysfunction ■ 24-hour ABPM

A young woman with history of tachy­ cardia on assuming upright posture,

Postural tachy­ cardia syndrome

mental clouding, chronic fatigue, and other systemic symptoms pres­ ents with syncope on change in body posture; there is no orthostatic

(POTS) Note: Most patients with POTS present without

hypotension

■ 24-hour ambulatory

syncope

■ Exclude cardiomyopathy or pheochromocytoma ECG, 24-hour Holter monitoring

warning symptoms are present ■ Compression stockings or abdominal binders (to increase venous return) ■ Head-up tilt sleeping > 1 O degrees (prevents nocturnal polyuria, maintains better distribution of body fluids, and ameliorates nocturnal hypertension) Medications: Midodrine (fi rst line in chronic a utonomic failure, increases BP), fludrocortisone (expands volume), 13-blockers, pyridostigmine, clonidine

I n addition to treatments mentioned earlier, consider exercise reconditioning

86

H I G H -YI ELD FACTS I N

CARDIOVASCULAR NOTES

DERMATOLOGY Layers of the Skin

88

Allergic and Immune-Mediated Skin Disorders

89

LICHEN PLANUS

1 14

ROSACEA

1 14

PITYRIASIS ROSEA

115

VITILIGO

115

EYELID LESIONS

1 16

HYPERSENSITIVITY REACTIONS

89

ATOPIC DERMATITIS (ECZEMA)

90

CONTACT DERMATITIS

91

SEBORRHEIC DERMATITIS

91

PSORIASIS

92

URTICARIA (HIVES)

93

DRUG ERUPTION

94

ERYTHEMA MULTIFORME

95

STEVENS-JOHNSON SYNDROME/TOXIC EPIDERMAL NECROLYSIS

95

ERYTHEMA NODOSUM

96

BuLLOUS PEMPHIGOID/PEMPHIGUS VuLGARIS

96

Neoplasms of the Skin

1 17

NUMMULAR ECZEMA

98

SEBORRHEIC KERA TOSIS

117

PYODERMA GANGRENOSUM

98

ACTINIC KERATOSIS

1 18

98

CUTANEOUS SQUAMOUS CELL CARCINOMA

118

Infectious Disease Manifestations of the Skin VIRAL DISEASES

98

BACTERIAL INFECTIONS

103

FUNGAL INFECTIONS

108

PARASITIC INFECTIONS

1 10

EPIDERMAL INCLUSION CYSTS

1 16

DERMATOFIBROMA

1 16

HIDRADENITIS SuPPURATIVA

1 16

lcHTHYOSIS VULGARIS

117

AGE-RELATED SKIN CHANGES

117

SuN PROTECTION

117

SUNBURN

117

BASAL CELL CARCINOMA

119

MELANOMA

119

KAPOSI SARCOMA

121

MYcos1s FuNGOIDES (CUTANEOUS T-CELL LYMPHOMA)

121

CHERRY ANGIOMAS (HEMANGIOMAS)

122

1 12

INFANTILE HEMANGIOMAS

122

DECUBITUS ULCERS

112

PYOGENIC GRANULOMA

122

GANGRENE

1 13

NECROBIOSIS LIPOIDICA

122

lschemic Skin Disorders

Miscellaneous Skin Disorders

1 14

STASIS DERMATITIS

1 14

ACANTHOSIS N1GRICANS

1 14

87

H I G H -YI E L D FACTS I N

88

D E R M ATO LOGY

LAYERS OF THE SKIN The skin is the largest organ in the human body. It provides a barrier and immunologic protection against the environment; regulates body tempera­ ture, fluids, and electrolytes; and allows for touch and sensation. Table 2.2-1 outlines common terminology related to the skin. TA B L E 2.2- 1 .

Dermatologic Macroscopic Terms

LESION

CHA RACTERISTICS

EXAM PLES

Macule

Flat lesion < 1 cm

Freckle, labial macule (see I mage A)

Patch

Flat lesion � 1 cm

Salmon patch (see I mage B)

Papule

Elevated palpable lesion < 1 cm

Mole (nevus; see I mage C), acne

Plaque

Elevated lesion � 1 cm

Psoriasis (see I mage D)

Vesicle

Fluid-containing blister < 1 cm

Chickenpox (varicella), shingles (zoster; see I mage E)

Bulla

Fluid-containing blister � 1 cm

Bullous pemphigoid (see I mage F)

Cyst

Epithelium-lined sac containing material or fluid

Pilar cyst (follicular cyst on scalp)

Pustule

Vesicle containing pus

Pustular psoriasis (see I mage G)

Wheal

Transient edematous papule or plaque

Hives (urticaria; see I mage H)

Scale

Flaking off of stratum corneum

Psoriasis (see I mage I)

Crust

Exudate of dried serum, blood, and/or pus

I mpetigo (see I mage J)

U lcer

Defect extending through the epidermis and upper dermis

Diabetic foot ulcer

Lichenification

Hypertrophy and thickening of the epidermis with accentuation of normal skin markings

Chronic scratching (pruritic scabies, eczema)

I mages reproduced with permission from Dr. Richard Usatine.

DERMATOLOGY

H I G H -YI E L D FACTS I N

89

ALLERGIC AND IMMUNE-MEDIATED SKIN DISORDERS HYPERSENSITIVITY REACTIONS Figure 2.2-1 illustrates the algorithm for a skin rash workup. Table 2.2-2 out­ lines information regarding the four types of hypersensitivity reactions. TA B L E 2 . 2 - 2 .

Types and Mechanisms of Hypersensitivity Reactions

DESCRIPTION

MECHA N I SM

COMMENTS

EXAMP LES

Antigen cross-links preformed

Fi rst and Fast (like

Anaphylaxis (bee sti ng, food

TYPE ! Anaphylactic and atopic Mast cell or basophil

Fe receptor

surface-bound lgE on mast

cells and basophils, triggeri ng the release of vasoactive ami nes like h ista m ine

anaphylaxis)

Types I, 11, and Ill a re all antibody med iated

allergy), asthma, u rtica ria,

u rticaria! d rug reactions, loca l whea l a n d fla re

Reaction develops ra pidly as a result of preformed anti body TYPE I I Cytotoxic

lgM and lgG bind to a ntigen

on a n "enemy" cell, leading

to lysis by complement or phagocytosis

Cy-2-toxic

Anti body and comple­

ment lead to formation of the mem brane attack complex (MAC)

Autoi m m u n e hemolytic

anemia, erythroblastosis

feta lis, Goodpasture syn­ d rome, rheumatic fever

@ = complement

TYPE I l l I m m une complex

Antigen-a ntibody com plexes fix

complement, which attracts

polymorphonuclea r neutro­ phils (PMNs; PMNs release lysosomal enzymes)

I magine a n i m m u n e

complex as th ree things

stuck together: a ntigenanti body-com plement Includes many glomerulonephritides and vascul itides

Polyarteritis nodosa, i m m u n e

com plex g lomerulonephritis, systemic l u pus erythematosus (SLE), rheu matoid arthritis

TYPE IV Delayed (cel l-mediated) type

Sensitized T lymphocytes

encou nter a ntigen and then

release lym phokines (leading

to macrophage activation)

Fou rth and final

(last)-delayed

Cell med iated, not a nti body

Tuberculosis (TB) skin tests, tra nsplant rejection, contact dermatitis

mediated; therefore it is not transferable by serum

APC, Antigen-presenting cell; Th cells, T-helper cells. Modified with permission from Le T et al. First Aid for the USMLE Step 1 20 15. New York, NY: McGraw-Hill Education; 201 5.

90

H I G H -YI E L D FACTS I N

Purulent blisters

Pustular

Erythematous papules and vesicles

Ato pic dermatitis {eczema)

D ERMATOLOGY

Nonpurulent blisters

Skin rash

-_,,. -., Scaly erythematous papules and plaques

Vesiculobullous

Well-defined erythematous papules and vesicles

Contact dermatitis

Eczematous

Hyperpigmented edema + venous ulcer usually on medial ankle

Stasis dermatitis

Sharply demarcated scaly papules and plaques

Pa pulosqua mous

Seborrheic dermatitis

Infants

Diaper rash {red and yellow scales, blisters and erosions) ± cradle cap {sca ling and crusti n g on sca lp)

Children and adults

Red, sca ly, thin plaques on face, mid-chest ± scalp

F I G U R E 2,2- 1 . Algorithm for eczematous skin rash workup. Management depends on the appearance of the rash and the age group affected. Eczematous lesions require further workup to determine the type of dermatitis. (Reproduced with perm ission from USMLE­ Rx.com.)

Cm

KEY FACT

Long-term use of immunomodulating medications (particularly TN F-a inhibitors) may i the risk for developing lymphoma.

Cm

KEY FACT

Erythema toxicum neonatorum typically begins 1 to 3 days after delivery and presents with red papules, pustules, and/or vesicles with surrounding erythematous halos. i eosinophils are present in the pustules or vesicles. This benign eruption usually resolves in 1 to 2 weeks with no treatment.

F I G U R E 2.2-2. Atopic dermatitis. Lichen­ ification, excoriations, and ill-defined, scaly erythematous plaques are character­ istic. (Reproduced with permission from Tinti nalli J E

et a l . Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7th ed. New York, NY: McGraw-Hill; 201 1 .)

ATOPIC D ERMATITIS (ECZEMA) A chronic inflammatory dermatitis that classically manifests in infancy and persists into adulthood. It is characterized by epidermal barrier dysfunction (multifactorial; however, likely due to filaggrin deficiency), causing sensitiza­ tion, which in turn leads to inflammation, pruritus, and ultimately lichenifi­ cation (Fig. 2.2-2). History/PE

Look for a family history of asthma, eczema, and allergic rhinitis ("atopic triad"), as well as food allergies. ■ Patients are at i risk for secondary bacterial (Staphylococcus aureus or Streptococcus pyogenes) and viral (herpes simplex virus or molluscum) infection due to constant waxing and waning cycles of pruritus and excoriation. ■ Triggers include climate, food, skin irritants, and allergens. ■ Manifestations by age group: ■ Infants (Fig. 2.2-3): Erythematous, edematous, weeping, pruritic vesi­ cles, papules, and plaques on the face, scalp, and extensor surfaces of the extremities. The diaper area is often spared. ■ Children: Dry, scaly, pruritic, excoriated vesicles, papules, and plaques in the flexural areas and neck. ■ Adults: Lichenification and dry, fissured skin in a flexural distribution. Often, there is hand, wrist, neck involvement. ■

Diagnosis

Characteristic exam findings and history are sufficient. Excluding contact der­ matitis by history and anatomic distribution is important. Potassium hydrox­ ide (KOH) prep can help distinguish chronic eczema from tinea. Mild peripheral eosinophilia and i IgE may be seen but have no diagnostic value.

DERMATOLOGY

H I G H -YI E L D FACTS I N

91

Treatment

The prima ry goal of therapy is to break the itch-scratch cycle with agents targeted at inflammation, pruritus, and xerosis (dry skin). ■ Topical corticosteroids are first-line therapy for flares, but atrophy, telangi­ ectasias, and rebound flares can occur with prolonged use. Topical calci­ neurin inhibitors (eg, tacrolimus) are useful as steroid-sparing agents for moderate to severe eczema for patients > 2 years of age. ■ H 1 -blockers may be used for relief of pruritus. A first-generation H 1 -blocker (eg, hydroxyzine) would be appropriate for nighttime use. ■ Aggressive use of emollients, avoidance of harsh soaps, and limiting hot showers after resolution of acute flares will prevent future episodes. Con­ sider phototherapy and dupilumab treatment. ■

CONTACT DERMATITIS

F I G U R E 2.2-3.

Atopic dermatitis in a n infant. Characteristic involvement o f the

face and cheeks, which is not commonly seen in adults. (Reproduced with permission

from Dr. Richard U satine.)

A type IV hypersensitivity reaction that results from contact with an allergen to which the patient has previously been exposed and sensitized such as nickel, poison ivy, perfumes/deodorants, and neomycin. More common in adults. History/PE

Presents with pruritus and an eczematous rash, with the distribution of the rash often mimicking the contact event (Fig. 2.2-4). Characteristic distri­ butions are seen where makeup, clothing, perfume, nickel jewelry, and plants come into contact with the skin. ■ Often described as a "linear" or "angular" rash. It can spread over the body via transfer of allergen by the hands or via circulating T lymphocytes. ■ Frequently implicated allergens: Poison ivy, poison oak, nickel, topical over-the-counter antibiotics, cosmetics, and latex. ■

Diagnosis

Characteristic exam findings and history are sufficient. Excluding atopic der­ matitis (eczema) is important. Patch testing can be used to establish the caus­ ative allergen after the acute-phase eruption has been treated. Treatment

The best initial treatment involves topical corticosteroids and allergen avoid­ ance. In severe cases, a systemic corticosteroid may be needed.

SEBORRHEIC DERMATITIS A common chronic inflammatory skin disease that may be caused by a reac­ tion to Malassezia furfur, a generally harmless yeast found in sebum and hair follicles. It has a predilection for areas with sebaceous glands such as the eye­ brows, nasolabial folds, and posterior ears.

F I G U R E 2.2-4.

Contact dermatitis.

Shown are erythematous papules and vesicles with serous weeping localized to areas of contact with the offending agent. (Reproduced with permission from H u rwitz RM. Pathology of the Skin. Atlas of Clinical-Pathological

Correlation, 2nd ed. Stamford, CT: Appleton & La nge; 1 998.)

0-,,. KEY FACT Patch testi ng resu lts a re affected by topica l steroids and ca lcineurin i n h i bitors but n ot by antih ista m i n es, beca use type IV hypersensitivity reactions a re not h ista m i n e med iated.

History/PE

Rash presentation varies with age: Infants: Severe, red diaper rash with yellow scale, erosions, and blisters. Scal­ ing and crusting ("cradle cap") may be seen on the scalp (see Fig. 2.2-5A). ■ Children/adults: Ill-defined red, scaly, thin plaques are seen around the ears, eyebrows, nasolabial fold, midchest, and scalp (see Fig. 2.2-5B). ■ Patients with HIV/AIDS, psychotic disorders, and Parkinson disease can develop severe, widespread seborrheic dermatitis. ■

A 23-yea r-old wom a n is seen for a n itchy, l i near ras h o n h e r right leg. She retu rned from a ca mping trip 4 days ago and d e n ies u s i n g any new ma ke u p, cloth ing, or j ewe l ry. What features of this presentation favor a contact derm atitis?

92

H I G H -YI E L D FACTS I N

D ERMATOLOGY

FIGURE 2.2-s. Seborrheic dermatitis. (A) Seborrheic dermatitis (cradle cap) in an infant. Note the yellow, scaly crust present on the infant's scalp with an area of erosion. (B) Photo-exacerbated seborrheic dermatitis, affecting the face only at sites of predilection for the seborrheic eruption. (A reproduced with permission from Tintinalli JE et al. Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7th ed. New York, NY: McGraw-Hill; 201 1 . B reproduced with permission from Gold-smith LA et al. Fitzpatrick's Dermatology in General Medi· cine, 8th ed. New York, NY: McGraw-Hill; 201 2.)

Diagnosis Characteristic exam findings and history are sufficient. Can be confused with atopic dermatitis, contact dermatitis, tinea, or psoriasis. Treatment Treat adults with ketoconazole, selenium sulfide, or zinc pyrithione sham­ poos for the scalp and topical antifungals (ketoconazole cream) and/or topical corticosteroids for other areas. Cradle cap often resolves with routine bathing and application of emollients in infants.

PSORIASIS A T-cell-mediated inflammatory dermatosis characterized by well-demarcated, erythematous plaques with silvery scales (Fig. 2.2-6A) due to dermal inflamma­ tion and epidermal hyperplasia. Psoriasis can begin at any age.

The asym metric i nvo lvem ent of the rash, its linear a rra ngement (possi bly fro m contact with a p l a nt d u ri n g the ca m p i n g trip), and the time fro m expos u re to ras h presentation a l l point t o contact dermatitis.

History/PE ■ Lesions are classically found on the extensor surfaces, including the elbows and knees. Scalp and lumbosacral regions are often involved. Nails are frequently affected with pitting, "oil spots," and onycholysis (lifting of the nail plate, see Fig. 2.2-6B). ■ Lesions initially appear small but may become confluent and can be pro­ voked by local irritation or trauma (Koebner phenomenon). Some medi­ cations such as 13-blockers, lithium, and angiotensin-converting enzyme (ACE) inhibitors can worsen psoriatic lesions. ■ Up to 30% develop psoriatic arthritis (affecting small joints of the hands and feet).

DERMATOLOGY

H I G H -YI E L D FACTS I N

93

F I G U R E 2.2-6. Psoriasis. (A) Skin changes. The classic sharply demarcated plaques with silvery scales are commonly located on the extensor surfaces (eg, elbows, knees). (B) Nail changes. Note the pitting, onycholysis, and "oil spots." (A reproduced with permission from Wolff K et al. Fitzpatrick's Color

Atlas and Synopsis of Clinical Dermatology, 7th ed. New York, NY: McGraw-Hill; 201 3. B reprod uced with permission from Hu rwitz RM. Pathology of the Skin. Atlas of Clinical-Pathological Correlation, 2nd ed. Stamford, CT: Appleton & Lange; 1 998.)

Diagnosis ■ Characteristic exam findings and history are sufficient. Classical pre­ sentation: Auspitz sign (pinpoint bleeding when scale is scraped) over­ lying well-demarcated, erythematous plaques with silvery "micaceous" scale. ■ Perform a biopsy if diagnosis is uncertain. Histology shows a thickened epidermis, elongated rete ridges, an absent granular cell layer, preservation of nuclei in the stratum corneum (parakeratosis), and a sterile neutro­ philic infiltrate in the stratum corneum (Munro microabscesses). Treatment ■ Local disease: Manage with topical steroids, calcipotriene (vitamin D derivative), and retinoids such as tazarotene or acitretin (vitamin A derivative). ■ Severe disease or presence of psoriatic arthritis: Methotrexate or anti­ tumor necrosis factor (TNF) biologics (etanercept, infliximab, adalim­ umab). Other agents such as ustekinumab (anti-interleukin [IL]-12/23), secukinumab (anti-IL l 7), and ultraviolet (UV) light therapy can be used for extensive skin involvement, except in immunosuppressed patients who can develop skin cancer from UV light. ■ Before starting methotrexate or anti-TNF biologics, patients should, at a minimum, get a complete blood count (CBC), comprehensive metabolic panel (CMP), hepatitis panel, and testing for TB (purified protein deriva­ tive [PPD] or interferon gamma release assay [IGRA]).

URTICARIA (HIVES) Results from the release of histamine and prostaglandins from mast cells in a type I hypersensitivity response. Sharply demarcated edematous plaques with surrounding erythema ("wheal and flare") are seen, with each lesion lasting 6 weeks).

0-,,. KEY FACT If a ras h i nvolves the extensor su rfaces, t h i n k psoriasis. If a ras h i nvolves the flexor s u rfaces, th i n k atopic dermatitis.

0-,,. KEY FACT "Sa usage dig its" a n d pencil-in-c u p x-ray fi nd i n g s a re suggestive of psoriatic a rth ritis.

94

H I G H -YI E L D FACTS I N

D ERMATOLOGY

History/PE ■ Urticaria lesions (wheals) are erythematous or white transient papules or plaques representing dermal edema. Lesions may be widespread. ■ In severe allergic reactions, extracutaneous manifestations can include tongue swelling, angioedema (deep, diffuse swelling often around the eyes and mouth; Fig. 2.2-7), asthma, gastrointestinal (GI) symptoms, joint swelling, and fever. ■ Acute urticaria is a response to some often-unidentified trigger: food, drug, virus, insect bite, or physical stimulus (cold, heat, sun). Chronic urticaria is usually idiopathic. F I G U R E 2.2-1. Urticaria (hives) and angioedema. This patient has urticaria

occurring on the face, neck, and shoul­ ders with orbital angioedema. (Reproduced

with perm ission from Goldsmith LA et al. Fitzpatrick's Dermatology in General Medicine, 8th ed. New York, NY: McGraw-Hill; 201 2.)

0-n

Diag nosis Characteristic exam findings and history are sufficient. Positive dermatogra­ phism (formation of wheals where the skin is stroked) may help. If in doubt, drawing a serum tryptase (co-released with histamine from mast cells) can help clinch the diagnosis. It can often be difficult to determine the cause of urticaria. Treatment Treat urticaria with systemic antihistamines. Anaphylaxis (rare) requires intra­ muscular epinephrine, antihistamines, IV fluids, and airway support.

KEY FACT

Patients with d ru g eru ptions often have peri phera l eosinop h i l ia and eos i n o p h i l s on histopathology.

DRUG ERUPTION Drug eruptions can range from a mild morbilliform rash (most common; Fig. 2.2-8) to the rare but life-threatening toxic epidermal necrolysis (TEN). Main­ tain a high suspicion for a cutaneous drug reaction in patients who are hospi­ talized and develop rashes. Drugs can cause all four types of hypersensitivity reactions (Table 2.2-2), and the same drug may cause different types of reac­ tions in different persons.

History/PE ■ Non-anaphylactoid eruptions usually occur 7 to 14 days after exposure: If a patient reacts within 1 to 2 days of starting a new drug, it is probably not the causative agent. Eruptions are generally widespread, relatively symmetric, and pruritic. ■ Most disappear within 1 to 2 weeks following removal of the offending agent. ■ Extreme complications of drug eruptions include erythroderma, drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens­ Johnson syndrome (SJS), and TEN. Diag nosis Characteristic exam findings and history are sufficient. Excluding other causes is important, including viral exanthema, graft-versus-host disease, and autoim­ mune dermatoses. A skin biopsy may be helpful if the diagnosis is not clear. F I G U R E 2.2-s. Morbilliform rash. Morbil­ liform rash following drug administra­ tion. (Reproduced with perm ission from Longo DL et al. Harrison's Principles of Internal Medicine, 1 8th ed. New York, NY: McGraw-H ill; 201 1 .)

Treatment Discontinue the offending agent; treat symptoms with antihistamines and top­ ical steroids to relieve pruritus. In severe cases, systemic steroids and/or IV immunoglobulin (IVIG) may be used.

DERMATOLOGY

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95

ERYTHEMA MULTIFORME Erythema multiforme (EM) is a cutaneous reaction pattern with classic targe­ toid lesions (Fig. 2.2-9) that has many triggers and is often recurrent. Herpes simplex is the most common agent.

History/PE ■ Initially, lesions start as erythematous, dusky macules that develop into the characteristic target lesion that commonly affects the palms and soles. The target lesions are described as a central, dusky blister surrounded by a pale edematous ring with a peripheral halo of erythema. The palms, soles, and lips are often affected. ■ EM minor is uncomplicated and localized to the skin. ■ EM major involves mucous membranes. It is a distinct entity from SJS, and there is no risk for progression to TEN. ■ May have systemic symptoms, including fever, myalgias, arthralgias, and headache. Diag nosis Characteristic exam findings and history are sufficient. As opposed to SJS or TEN, in EM the Nikolsky sign is 8. Treatment ■ Symptomatic treatment is all that is necessary; systemic corticoste­ roids are of no benefit. ■ EM minor can be managed supportively; EM major should be treated as burns.

STEVENS- JOHNSON SYNDROME/TOXIC EPIDERMAL NECROLYSIS SJS and TEN constitute two different points on the spectrum of life-threatening exfoliative mucocutaneous diseases that are often caused by a drug-induced immunologic reaction. The epidermal separation of SJS involves 30% of BSA. Mucosa] involvement is present in >90% of cases of SJS/TEN.

F I G U R E 2.2-9.

Erythema multiforme.

(Reproduced with permission from Dr. Richard Usatine.)

0-,,. KEY FACT EM is often triggered by infections such as HSV or mycoplasma. SJS and TEN are typica l ly caused by d rugs. Both are type IV hypersensitivity reactions.

0-,,. KEY FACT A d ifferential d iagnosis should always incl ude SJS and TEN if a EB N i kolsky sign is present.

History/PE ■ Exam reveals severe mucosa] erosions with widespread erythematous, dusky red or purpuric macules, or atypical targetoid lesions (Fig. 2.2-10). The epidermal lesions often become confluent and show a EB Nikolsky sign (separa­ tion of the superficial skin layers with slight rubbing) and epidermal detachment. ■ Mucous membranes (eyes, mouth, and genitals) often become eroded and hemorrhagic. ■ Associated with first-time exposure to drugs: sulfonamides, penicillin, sei­ zure medications (phenytoin, carbamazepine), quinolones, cephalospo­ rins, steroids, nonsteroidal anti-inflammatory drugs (NSAIDs). F I G U R E 2.2-1 o. Toxic epidermal necroly­ sis. Note the diffuse erythematous bullae

and areas of sloughing secondary to the full-thickness necrosis of the epidermis.

(Reproduced with perm ission from Tintinalli JE et al. Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7th ed. New York, NY: McGraw-H ill; 201 1 .)

96

H I G H -YI E L D FACTS I N

0-,r KEY FACT Do not confuse SJS and TEN with SSSS. SSSS is usua l ly seen i n c h i l d ren 60 years of age

Usually 40-60 yea rs of age

Associated medication

Genera l ly idiopathic

ACE i n h ibitors, penici llami ne, phenobarbital,

Mortality

Rare

Possible

Diag nosis

Tense bul lae on the tru n k a re ind icative of bullous

Flaccid/un roofed bullae and erosions on the extrem­

triggers

penicillin

pemphigoid

Most accurate test: skin biopsy with d i rect i m m unofluo­ rescence enzyme-l i n ked i m m u nosorbent assay (ELISA)

Treatment

ities and m ucous mem branes a re indicative of pemphigus vulgaris

High-does steroids + i m m u nomod u latory therapy

Topica l: Hig h-potency corticosteroids Systemic: Corticosteroid, doxycycline

I mages reprod uced with permission from Wolff K, Johnson RA. Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology, 6th ed. New York, NY: McGraw-Hill; 2009. Normal

Pemphigus vulgaris l

Epidermis

Bullous pemphigoid �

Disrupted desmosomes � I ntact � desmosomes

lgG antibodies

.. .. .

hemidesmosomes

. .

Disrupted hemidesmosomes

F I G U R E 2.2- 1 2. Blistering dermatosis. Compare and contrast the layers of the epidermis in normal skin with the blistering dermatosis. Pemphigus vulgaris involves disruption of desmo­ somes and shows separation of the stratum spinosum from the stratum basale, causing a "row of tombstones appearance . " Bullous pemphigoid involves separation of the epidermis from the dermis due to disruption of hemidesmosomes. (Reproduced with permission from USMLE-Rx.com.)

A 28-yea r-old Black wo m a n presents to the physicia n fo r a new-on set, pa i n fu l ras h . She noticed the erythemato u s n od u les on both l owe r legs 3 d ays ago. She ha s a h i story of uveitis. What is t h e n ext best step to ide ntify the u nd e rlyi n g ca use of this ra sh7

98

H I G H -YI E L D FACTS I N

D ERMATOLOGY NUMMULAR ECZEMA Chronic relapsing-remitting, pruritic, coin-shaped, scaly plaques (Fig. 2.2-13) most commonly found on the extremities (nummun is Latin for coin). While the pathogenesis is unclear, it is thought to be associated with xerosis and decreased skin lipids. Diagnosis is clinical, and treatment includes use of emollients and avoidance of harsh soaps to prevent d ry skin and use of topical glucocorticoids.

F I G U R E 2.2- 1 3.

PYODERMA GANGRENOSUM Nummular eczema.

(Reproduced with permission from Bissek AC, Tabah EN, Kouotou E, et al. The spectrum of skin diseases in a rural setting in Cameroon (sub-Saharan Africa). BMC Dermatol. 201 2;1 2:7. doi: 1 0. 1 1 86/1 471 -S94S- 1 2-7.)

Neutrophilic dermatosis associated with inflammatory bowel disease, rheuma­ toid arthritis, and underlying malignancy. Presents as papules or pustules that rapidly progress to a painful ulcer with a violaceous border and purulent base (Fig. 2.2-14). Demonstrates pathergy (formation of ulcers at sites of injury). Diagnosis depends on recognizing the clinical presentation and excluding other causes. Treatment involves local or systemic glucocorticoids.

INFECTIOUS DISEASE MANIFESTATIONS OF THE SKIN VIRAL DISEASES Human Herpesvi ruses F I G U R E 2.2- 1 4.

Pyoderma gangreno­

sum. (Reproduced with permission from Fonder MA, Cummins DL, Ehst BD, et al. Adalimumab therapy for recalcitrant pyoderma gangrenosum. J Burns Wounds. 2006;S:e8.)

0-rr

KEY FACT

Dermatitis herpetiformis (DH) has vesicles a n d erosions l i ke herpes but is NOT caused by HSV. DH consists of sym metric, bilatera l pru ritic papu les, vesicles, b u l lae, and erosions on the el bows (Fig. 2.2- 1 5), knees, b uttocks, neck, and sca l p, a n d it is associated with celiac d i sease ( 1 5%-25%). Treat with da psone and a g l uten-free d iet.

X-ray of the c hest (CX R) to look for bi late ra l hilar aden opathy, which i s sugg estive o f sa rcoidosis. Erythema nodos u m is the most co m mo n nonspecific cutaneous m a n ifestation of sarcoidosis, afte r cutaneous sa rcoidosis.

The human herpesviruses (HHVs) are a group of DNA viruses that result in lifelong latent infection and are usually transmitted via physical contact (Table 2.2-4). Herpes S i m plex

Painful, recurrent vesicular eruption of the mucocutaneous surfaces due to infection with HSV. Both serotypes can affect both genital and extragenital regions. The virus spreads through epidermal cells, fusing them into giant cells. The local host inflammatory response causes erythema and swelling. History/PE ■ The initial infection is by direct contact with oral or genital fluids; after the primary episode, the virus remains dormant in local nerve ganglia: HSV-1 in the trigeminal ganglia and HSV-2 in sacral ganglia. First epi­ sodes are generally longer and more severe than recurrences. ■ Onset is preceded by prodromal tingling, burning, or pain but can also present with lymphadenopathy, fever, discomfort, malaise, and edema of involved tissue. Recurrences are limited to mucocutaneous areas innervated by the involved nerve: ■ Recurrent oral herpes (HSV- 1 ) : The common "cold sore," or herpes labialis, which presents as a cluster of crusted vesicles on an erythema­ tous base (Fig. 2.2-16A). Often triggered by stress, sunlight, or infections. ■ Recurrent genital herpes (HSV-2): Unilateral and characterized by a cluster of ulcers on an erythematous base, but with less pain and sys­ temic involvement than the primary infection.

D E RMATO LOGY

TA B L E 2.2-4.

H I G H -YI E L D FACTS I N

99

Human Herpesviruses

VI RUS

HISTORY AND MAN I F ESTATIONS

DIAGNOSIS

MANAGEMENT

HHV- 1 and

H HV-1 : Transmitted via respi ratory/ora l

Cli nical d iagnosis should be con­

Lesions and other man ifestations

H HV-2 (herpes

sim plex

viruses)

secretions. Usually causes oral vesicles and u lcers. Causes tem pora l lobe encephal itis.

H HV-2: Transmitted via sexual contact. Usually manifests as genita l lesions.

Causes viral meningitis.

H HV-3

(varicella­

zoster virus [VZV])

The only herpesvirus with ai rborne transmission.

fi rmed by laboratory testing

with acyclovir, va lacyclovi r,

sam ples can be sent for viral

clovir for severe infections i n

reaction (PCR).

cases o f centra l nervous system

u n roofed and swabbed­

cultu re or polymerase chain

VZV lesions can be swa bbed

and sent for PCR for defin itive

Chicken pox (primary i nfection) character­

diagnosis. Samples from other

distribution. Severe cases can develop

(e.g., cerebrospinal fl uid [CSF]]

Shing les (reactivation) presents with vesicu­

bronchoalveolar lavage [BAL]

ized by pruritic vesicles with centrifugal

pneumonia, hepatitis, and encephalitis.

lar rash that is pai nful and appears in

dermatomal distribution. Can man ifest

as Ramsay H unt synd rome (external ear

can be treated or suppressed

if possible. Vesicles can be

suspected sites of i nfection

i n suspected CNS i nfection, or

fl uid i n suspected pneumonia) can also be sent for PCR.

or famciclovir. Choose IV acy­

immunocompromised, or i n (CNS) i nfection.

Contact and airborne precautions for hospita lized patients with

VZV i nfection or disseminated zoster.

Treatment with acyclovir/valacy­ clovir within 72 hours of rash

onset significantly decreases the intensity and duration of pain

associated with lesions. IV acy­

clovir should be used for severe

infections. Vaccines are available

lesions, facial palsy, hearing loss).

to prevent primary infection and reactivation.

H HV-4 (Epstein­ Barr virus [EBV])

Transmitted via respi ratory secretions and saliva.

Main cause of infectious monon ucleosis (exudative pharyngitis, fever, fatig ue, hepatitis, splenomega ly).

Associated with development of Burkitt and Hodgkin lymphomas, B- and T-cell lym­

phomas, nasopharyngeal carcinoma, and

I nfectious monon ucleosis can be diagnosed by identifying het­

e rophile antibodies (Monospot

test) or lgM agai nst the EBV vira l capsid antigen.

Blood smear can show atypical lymphocytes.

posttransplant lymphoproliferative disease. H HV-5 (cyto­

megalovirus [CMV])

Transmitted via saliva, sexual contact, blood transfusions, and organ transplants.

Can cause CMV mononucleosis (similar

Treatment is mostly supportive.

Patients should avoid contact

sports for 4 weeks out of risk of

splenic ruptu re.

Note: Maculopapular rash can be

seen in cases of infectious mono­

n ucleosis that a re treated with

a moxici l l i n (ie, when confused

with streptococcus-associated pharyngitis).

Diagnosis can be made by PCR of

sam ples from affected sites.

IV ganciclovir or oral valganciclovi r

for treatment.

to EBV mononucleosis, but with negative Monospot).

In the i m m u n ocom promised, CMV can cause

encephalitis, reti nitis, pneumonitis, col itis,

hepatitis, and esophagitis ("shal low" u lcers

on endoscopy), and cytopenias.

(continues)

1 00

TA B L E 2 .2-4.

H I G H -YI E L D FACTS I N

D E R M ATO LOGY

Human Herpesviruses (continued)

VIRUS

HISTORY AND MANIFESTATIONS

DIAGNOSIS

MANAGEMENT

HHV-6 and

Transmitted via saliva.

Typically a clinical diag nosis.

Self-limited i l l ness.

H HV-7 (roseolovi ruses)

Can cause roseola infantum in childrenhigh fevers for several days followed by

onset of macular rash with centrifugal spread.

H HV-8

Transmitted via sexual contact.

Associated with Kaposi sarcoma in H IV/AIDS and transplant patients.

No gold standard for d iagnosis of

H HV-8.

Kaposi sarcoma diagnosed by biopsy.

Diagnosis ■ Clinical diagnosis: Grouped vesicles on an erythematous base. ■ Most accurate test: Viral culture or PCR test of lesion. Direct fluorescent antigen is the most rapid test. ■ Classic multinucleated giant cells on Tzanck smear (see Fig. 2.2-16B) support the diagnosis.

0-,,. KEY FACT

Herpetic whitlow presents as painful blisters/sores on the hand resembling "dew drops on a rose'.' It is common in healthcare workers, respiratory therapists, dentists, and dishwashers.

Treatment ■ First episode: lmmunocompetent patients with small lesions only need supportive therapy, but acyclovir, famciclovir, or valacyclovir may be given to speed healing and reduce viral shedding. ■ lmmunocompromised patients or those with a severe painful outbreak should receive an antiviral drug within 72 hours of the start of the outbreak. ■ Recurrent episodes: Minor lesions can be managed supportively. Acyclo­ vir, famciclovir, or valacyclovir can be given during the episode to reduce healing time by ~2 days. ■ Severe frequent recurrences (>6 outbreaks per year) : Daily prophylaxis with acyclovir, famciclovir, or valacyclovir. ■ In patients with AIDS, HSY can persist, with ulcers remaining resistant to antiviral therapy. Symptomatic HSY infection lasting > 1 month can be considered an AIDS-defining illness.

s. Dermatitis herpetifor­ mis. This disorder typically displays pru­ FIGURE 2.2-1

ritic, grouped papulovesicles on elbows, knees, buttocks, and posterior scalp. Ves­ icles are often excoriated due to associ­ ated pruritus. (Reproduced with perm ission from

Longo DL et al. Harrison's Principles of Internal Medicine, 1 8th ed. New York, NY: McGraw-Hill; 201 1 .)

F I G U R E 2.2- 1 6.

tongue.

Herpes simplex. (A) Herpes labialis. (B) HSV- l lesions of the oral mucosa and

(Image A reprod uced with permission from the US Department of Health and Human Services and Dr. Herrmann. Image B reproduced with permission from the US Department of Health and Human Services and Robert E. Sumpter.)

D E RMATO LOGY

H I G H -YI E L D FACTS I N

1 01

Va ricella-Zoster Virus VZV causes two different diseases - varicella and herpes zoster - with trans­ mission occurring via respiratory droplet or by direct contact. VZV has an incubation period of 10 to 20 days, with contagion beginning 24 hours before the eruption appears and lasting until lesions have crusted. History/PE Varicella: ■ A prodrome of malaise, fever, headache, and myalgia occurs 24 hours before the rash. ■ Pruritic lesions appear in crops over 2 to 3 days, evolving from red mac­ ules to vesicles that then crust over. At any given time, patients may have all stages of lesions present. The trunk, face, scalp, and mucous membranes are involved. ■ In adults, chickenpox is often more severe, with systemic complications such as pneumonia and encephalitis. Zoster: ■ Herpes zoster (shingles) represents the recurrence of VZV in a specific nerve, with lesions appearing along the nerve's dermatomal distribution. Outbreaks are usually preceded by intense local pain (acute herpetic neu­ ralgia) followed by grouped blisters on an erythematous base (Fig. 2.2-17). Zoster can become disseminated in immunocompromised persons. ■ Acute herpetic neuralgia: Pain persisting less than 30 days from rash onset. ■ Subacute herpetic neuralgia: Pain persisting longer than 30 days but less than 4 months from rash onset. ■ Postherpetic neuralgia: Pain persisting greater than 4 months from rash onset. ■ Herpes zoster oticus (Ramsay Hunt syndrome): Reactivation of VZV in the geniculate ganglion affecting cranial nerves (CNs) VII and VIII. Pres­ ents with shingles in the ear canal and pinna; CN VII involvement causes facial paralysis, and CN VIII involvement causes vertigo and sensorineural hearing loss. ■ Herpes zoster ophthalmicus: Reactivation of VZV along trigeminal nerve dis­ tribution. Presents with shingles in the trigeminal nerve distribution of Vl, herpes zoster keratitis. This is a medical emergency, as it can cause blindness. ■ Older patients with zoster can develop postherpetic neuralgia (severe nerve pain that persists for >4 months at the infection site after rash onset). Diagnosis PCR or viral culture test of lesion. Characteristic exam findings and history. Treatment ■ Varicella is self-limited in healthy children. A live attenuated vaccine is available that should be given to children in two doses at ages 1 and 4. Also recommended for adults over 60 years of age. May be given to HIV patients with CD4+ cell count >200. ■ Adults should be treated with systemic acyclovir to treat symptoms and prevent complications. Pain control with NSAIDs for acute and subacute herpetic neuralgia and neuropathic agents (gabapentin, pregabalin, tricy­ clic antidepressants) for postherpetic neuralgia. ■ Postexposure prophylaxis is rarely needed, as most patients in the United States have been vaccinated or had childhood varicella. If needed, immuno­ compromised individuals, pregnant women, and newborns should receive varicella-zoster immune globulin within 10 days of exposure. lmmunocom­ petent adults should receive a varicella vaccine within 5 days of exposure.

F I G U R E 2.2- 1 1 . Varicella zoster. The unilateral dermatomal distribution of the grouped vesicles on an erythematous base is characteristic. (Reproduced with per­

mission from Wolff K et al. Fitzpatrick's Color Atlas &

Synopsis of Clinical Dermatology, 5th ed. New York, NY:

McGraw-Hill; 2005.)

H I G H -YI E L D FACTS I N

1 02

D E R M ATO LOGY Complications Herpes zoster ophthalmicus, herpes zoster oticus (Ramsay Hunt syndrome), and congenital varicella syndrome. Molluscum Contagiosum

A poxvirus infection that is most common in young children and in AIDS patients. It is spread by direct skin-to-skin contact (sports, sex) or sharing infected clothing or towels. FIGURE 2.2-1 s.

Molluscum contagiosum.

Flesh-colored, dome-shaped papules pres­ ent on the face of an adolescent. (Repro­ duced with permission from Dr. Richard Usatine.)

0-n

KEY FACT

If you see g i a nt m o ll usc u m contag iosum, th i n k H IV or ! cel l u l a r i m m u n ity.

History/PE ■ Presents as tiny, flesh-colored, dome-shaped, waxy papules, frequently with central umbilication. In children, lesions are found on the trunk, extremities, or face (Fig. 2.2-18). In adults, they are considered sexually transmitted infections (STls) and are commonly found on the genitalia and in the perinea! region. Typically spares palms and soles. ■ Lesions are asymptomatic unless they become inflamed or irritated. Diagnosis ■ Characteristic exam findings and history are sufficient. ■ Most accurate test: If the diagnosis is uncertain, Wright and Giemsa stains show presence of large inclusion or molluscum bodies on histology. Treatment ■ Local destruction: Curetting, cryotherapy, laser ablation, or applying can­ tharidin (a blistering agent) to the lesions. ■ In children, lesions resolve spontaneously over months to years and are occasionally left untreated. Verrucae (Warts) Warts are caused by human papillomavirus (HPV) and can occur on skin, mucous membranes, and other epithelia. Although usually benign, some sub­ types of HPV (especially 16 and 18) lead to squamous malignancies. Spread is by direct contact. History/PE Common warts are most often seen on the hands, though they can occur anywhere. ■ Classic genital warts (condyloma acuminatum, caused by HPV subtypes 6 and 11) are cauliflower-like papules or plaques appearing on the penis, vulva, or perianal region (Fig. 2.2-19).

F I G U R E 2.2- 1 9. Verrucae (warts) caused by HPV. (A) Soft, tan-65 years of age and men > 70 years of age and those with other risk factors for osteoporosis. ■ Osteoporosis: Bone mineral density (T-score) is 2. 5 standard deviations (SDs) less than normal. ■ Osteopenia: T-score is between 1 and 2. 5 SDs below normal. ■ Lab tests: Secondary causes reveal themselves through measurements of calcium, phosphate, parathyroid hormone (PTH), TSH, free T4 , liver enzymes, creatinine, and electrolytes. If estrogen deficiency or hypogonad­ ism is suspected, laboratory tests should include follicle-stimulating hor­ mone (FSH), luteinizing hormone (LH), estradiol, and testosterone.

F I G U R E 2.3-9. Radiographic findings in osteoporosis. Lateral thoracic spine

radiograph shows osteoporosis and an anterior wedge deformity (red arrow) of a lower thoracic vertebral body with asso­ ciated kyphosis. This is a typical insuf­ ficiency fracture in osteoporotic patients. (Reproduced with permission from Fauci AS, et al.

Harrison's Principles ofInternal Medicine. 1 7th ed. New

York, NY: McGraw-Hill; 2008.)

Treatment ■ Lifestyle modifications: Adequate calcium and vitamin D intake (supple­ mentation can be used for prevention), smoking cessation, avoiding heavy alcohol use, and weight-bearing exercises. ■ Best initial treatment: Bisphosphonates (eg, alendronate, risedronate, ibandronate, zoledronic acid) used in the treatment of osteoporosis; treat­ ment also offered to individuals with osteopenia who have a high calcu­ lated osteoporotic fracture risk based on the FRAX calculator. ■ Other medications: Teriparatide (PTH analogue), denosumab (a monoclo­ nal antibody to RANK-L), and selective estrogen receptor modulators (eg, raloxifene).

ENDOCRINO LOGY Compl ications

Hip fracture is the most devastating consequence of low bone mineral density/ osteoporosis, carrying a 50% i in mortality in the year following hip fracture.

PAGET DISEASE OF BONE Characterized by an i rate of bone turnover with both excessive resorption and formation of bone. Suspected to be caused by the effects of latent viral infection in genetically susceptible individuals. Associated with primary hyperparathyroidism and i risk for osteosarcoma. The disease can affect one (monostotic) or more (polyostotic) bones, with the skull, vertebral bodies, pel­ vis, and long bones most commonly affected. History/PE ■

■

Usually asymptomatic. May present with aching bone or joint pain, bony deformities, fracture at a pagetoid site, nerve entrapment, headaches, and hearing loss (latter two occur if involving the skull).

H I G H -YI E L D FACTS I N

1 39

0-,,. KEY FACT

Upper gastrointestinal side effects such as reflux, esophagitis, and esophageal ulcers are common reasons for oral bisphosphonate (alendronate and risedronate) intolerance.

0-,,. KEY FACT

Increased serum alkaline phosphatase with normal gamma-glutamyl transpeptidase (GGT) points to bone etiology, not liver etiology, as the cause of elevation.

Diagnosis

Best initial test: Plain film x-rays (lytic and sclerotic lesions; see Fig. 2. 310) usually diagnostic. ■ Radionuclide bone scan necessary to characterize extent and sites of dis­ ease (see Fig. 2.3-11). ■ Lab values: i serum alkaline phosphatase with normal calcium and phos­ phate levels. Must be distinguished from metastatic bone disease. ■

Treatment

F I G U R E 2.3 - 1 0.

Most patients are asymptomatic and require no treatment. There is no curative treatment, but the goal is to reduce pain and disease progresswn. ■ Pharmacologic: Bisphosphonates (first line), calcitonin (if intolerant to bisphosphonates), calcium and vitamin D supplementation, analgesics (NSAIDs and acetaminophen). ■ Adjunctive therap : Physiotherapy, occupational therapy. y ■ Surgery : If necessary, such as in the case of fractures, severe deformities, and osteoarthritis.

demonstrates a thickened cortex (a,ww), thickened trabeculae (arrowhead), and expansion of the right femoral head, classic signs of Paget disease. (Reproduced with permis­

■ ■

Complications

Osteoarthritis, pathologic fractures, high-output cardiac failure (from atrio­ ventricular [AV] connections), and osteosarcoma (up to 1%).

HYPERPARATHYROIDISM See Figure 2. 3-8 for the effects of PTH on serum calcium and phosphate reg­ ulation. For a more thorough review of hypocalcemia and hypercalcemia, see the Renal/Genitourinary chapter. ■

Primary hyperparathyroidism: Most cases (80%) caused by a single hyper­ functioning adenoma, with the rest (1 5 %) resulting from parathyroid hyperplasia and, rarely ( 5 % ), parathyroid carcinoma.

Radiographic findings in Paget disease. Pelvic radiograph

sion from Fauci AS, et al. Harrison's Principles ofInternal

Medicine. 1 7th ed. New York, NY: McGraw-Hill; 2008.)

t:,.◊ MNEMONIC

Symptoms and signs of Paget disease of bonePAN ICS

Pain Arthralgia Nerve compression/Neural deafness Increased bone density Cardiac fai l u re Sku l l involvement/Sclerotic vertebra

0-,,. KEY FACT

Bone pain and hearing loss ➔ think Paget disease.

1 40

H I G H -YI ELD FACTS I N

ENDOCRINOLOGY ■

■

■

Secondary hyperparathyroidism: A physiologic i of PTH in response to renal insufficiency (caused by ,J, production of 1-25 dihydroxy vitamin D), calcium deficiency, or vitamin D deficiency. Tertiary hyperparathyroidism: Seen in patients on dialysis with long­ standing secondary hyperparathyroidism, which leads to hyperplasia of the parathyroid glands. When one or more of the glands become autonomous, tertiary hyperparathyroidism results. Pseudohypoparathyroidism: PTH resistance. i PTH levels but ineffective at target organs, resulting in hypocalcemia and hyperphosphatemia. Asso­ ciated with Albright hereditary osteodystrophy (may have shortened fourth and fifth metatarsal or metacarpal bones).

History/PE Most cases of primary hyperparathyroidism are asymptomatic but may show signs and symptoms of hypercalcemia (see Renal/Genitourinary chapter).

4,

Fi

F I G U R E 2.3-1 1 . Radionuclide bone scan in Paget disease. Dark areas represent

increased bone-seeking isotope uptake and depicts severe disease in the left femur. (Reproduced with permission from Takigami I, et al.

Functional bracing for delayed union of a femur fracture associated with Paget's disease of the bone in an Asian patient: a case report. J Orthop Surg Res. 201 0;5:33.)

0-n KEY FACT Hyperca lcemia is associated with "stones, bones, moa ns, g roa ns, and psych iatric overtones'.' Treatment: IV fl uids (fi rst-line treatment) a n d calcito n i n . If secondary t o m a l i g n a n cy, add bisphosphonates.

0-n KEY FACT Etiologies of hypoparathyroidism include iatrogenic (postsu rgica l), a utoi m m u ne, congen ita l (DiGeorge), a n d infi ltrative (hemochro matosis, Wilson) diseases.

Diagnosis ■ Lab results in primary hyperparathyroidism reveal hypercalcemia, hypo­ phosphatemia, and hypercalciuria. Intact PTH is inappropriately i rela­ tive to total and ionized calcium (see Table 2.3-9). A 99mTc sestamibi scan, in conjunction with thyroid ultrasonography, can help localize a solitary adenoma. DEXA may reveal low bone mineral density or frank osteoporosis in the distal radius or other sites. Renal imaging can look for nephrocalcinosis and nephrolithiasis. Treatment ■ Best initial treatment: For acute hypercalcemia, IV fluids and calcitonin. IV bisphosphonates suitable for long-term treatment. Parathyroidectomy if the patient is symptomatic or if certain criteria are met (T i calcium, i creatinine, ,J, bone mineral density, < 50 years of age). In the case of a solitary adenoma, 1 gland can be removed. In the set­ ting of hyperplasia, 3. 5 glands must be removed.

TAB L E 2.3-9.

Lab Values in Hyperparathyroidism

PTH

CALC I U M

P04

Primary

i

i

,I,

Secondary

ii

N l/t

i (when etiology is renal failure)

Tertiary

i

ii

i

Ectopic PTHrP•

,I,

ii

Normal/,!,

•PTH-related peptide (PTH rP) is a member of the PTH family and acts on the same PTH receptors. Some tumors (eg, breast, lung) produce PTH rP, causing hypercalcemia of malignancy.

ENDOCRINO LOGY

H I G H -YI E LD FACTS I N

1 41

■

In patients with chronic kidney disease, oral phosphate binders (calcium salts, sevelamer hydrochloride, and lanthanum carbonate) and restriction of dietary phosphate intake to prevent secondary hyperparathyroidism. ■ Cinacalcet - a calcimimetic that acts to lower serum PTH levels ­ approved for use in hyperparathyroidism caused by renal failure or in patients who cannot undergo surgery.

Compl ications Hypercalcemia is the most severe complication of primary hyperparathy­ roidism. Following parathyroidectomy, the physician should watch for hun­ gry bone syndrome (severe and prolonged hypocalcemia caused by acute reversal of PTH and i in bone uptake of calcium, phosphate, and magnesium).

0-Tr KEY FACT Hyperparathyroidism ca n be caused by ectopic PTH-related peptide (PTH rP) p rod uction, parti c u l a rly from carcinomas of the breast, l u ng, a n d h e a d a n d neck.

PITUITARY AND HYPOTHALAMIC DISORDERS Figure 2.3-12 illustrates the hypothalamic-pituitary axis. The following sec­ tions outline the manner in which the components of this axis interact with target organs in various pathologic states.

DEFICIENCY OF PITUITARY HORMONES Hypopituitarism is the deficiency of anterior pituitary hormones. If the poste­ rior pituitary hormones are also affected, it is known as panhypopituitarism. Common causes include: Damage to pituitary gland/hypothalamus by surgery, radiation, mass lesions (tumors such as a nonfunctioning pituitary adenoma, craniopharyngioma) ■ Sheehan syndrome (pituitary infarction seen in severe hemorrhage, classi­ cally in postpartum patients) ■ Pituitary apoplexy (hemorrhage) Infiltrative disorders (hemochromatosis) ■ Infections ■

+ ++

Hypothalamus

CRH

Anterior pituitary

ACTH

LH

FSH

Basophils (basophilic)

+ p -e--------:r GHRH

GnRH

TSH � Somatostatin

GH

I DA

+

Prolactin

F I G u R E 2 . 3 - 1 2 . The hypothalamic-pituitary axis. ACTH, Adrenocorticotropic hormone; CRH, corticotropin-releasing hormone; DA, dopamine; FSH, follicle-stimulating hormone; CH, growth hormone; GHRH, growth hormone-releasing hormone; GnRH, gonadotropin­ releasing hormone; LH, luteinizing hormone; TRH, thyrotropin-releasing hormone; TSH, thyroid-stimulating hormone. (Modified with permission from USMLE-Rx.com.)

•

An a sym ptomatic 36-yea r-old m a n presents fo r h i s a n n u a l physica l . Routi n e l a bs reveal a seru m calc i u m l evel o f 1 1 .3 mg/d l. He ret u r n s i n 2 weeks, and h i s seru m ca l c i u m l evel rema i n s e l evated . Additional stu d ies s h ow a normal seru m PTH l evel a n d a l ow 24- h o u r u r i n a ry calci u m level. What i s the most l i kely d iagnosis?

1 42

H I G H -YI E L D FACTS I N

ENDOCRINOLOGY

Gonadotropins and growth hormone are often affected first; consequently, children first present with growth retardation, and adults present with hypogo­ nadism. Pituitary hormone deficiencies include adrenocorticotropic hormone (ACTH) deficiency, secondary hypothyroidism, growth hormone (CH) defi­ ciency, gonadotropin deficiency, central diabetes insipidus (DI), and prolac­ tin deficiency. H i story/PE

See Table 2.3-10 for a presentation of pituitary hormone deficiencies. Clini­ cal manifestations may present suddenly (apoplexy, Sheehan syndrome) or gradually (radiation, infiltrative diseases). Diagnosis

Routine hypopituitarism testing includes 8 am cortisol (on at least two sepa­ rate occasions), free T4 (TSH is not diagnostic), testosterone/estradiol levels, urine, and plasma osmolality. After the diagnosis of a pituitary hormone defi­ ciency, a brain MRI can check for underlying causes. (See Table 2.3-10.) Treatment

Hormone replacement therapy and treatment of the underlying disorder. Corresponding sections outline treatments of specific hormone deficiencies.

DIABETES INSIPIDUS Inability to produce concentrated urine as a result of antidiuretic hormone (ADH) dysfunction, resulting in free water loss from the kidneys. The two subtypes of diabetes insipidus (DI) are as follows: ■

0-,,. KEY FACT I n patients with suspected DI, check seru m or u ri n a ry g l ucose to rule out dia betes mel l itus.

Central DI (ADH deficiency): The posterior pituitary gland fails to secrete ADH. Causes include tumor, ischemia (Sheehan syndrome), pitu­ itary hemorrhage, traumatic brain injury, infection, metastatic disease, and autoimmune disorders (see Fig. 2.3-13). ■ Nephrogenic DI (ADH resistance) : The kidneys fail to respond to circu­ lating ADH. Causes include renal disease and drugs (eg, lithium, demeclocycline).

t urine osmolality J, urine volume Th e patient most l i kely has fa m i l ia l hypoca lci u ric hyperca lcemia (FH H), an i n herited d isorder ca u sed by m utations i n a calciu m-sensing receptor p resent i n the parathyroid and k i d n ey, which p resents with el evated seru m calci u m levels. U n l i ke patients with primary hyperpa rathyro i d i sm, these patients a re asym ptomatic and h ave l ow u rinary ca l c i u m levels. No t reatment is req u i red.

SIADH

dj [:edullary collecting 4 Aquaponn channels ADH �

$

ADH antagonists Lithium Nephrogenic DI

F I G U R E 2.3- 1 3 . The hypothalamic-pituitary axis: Diabetes insipidus. In central DI, ADH is not secreted from the posterior hypothalamus. In nephrogenic DI, ADH is secreted from the posterior hypothalamus, but its end-organ effects at the kidney are blocked. Both central and nephrogenic DI result in an increase in plasma osmolarity due to excessive free-water diuresis. DI, Diabetes insipidus; SIADH, syndrome of inappropriate secretion of ADH. (Reproduced with permission from USMLE-Rx.com.)

ENDOCRINO LOGY TA B L E 2.3- 1 0.

H I G H -YI E LD FACTS I N

1 43

Pitu itary Hormone Deficiencies

DEFICIENT HORMONE

CLINICAL MAN I F ESTATION

DIAGNOSIS

NOTES

ACTH

Weakness, hypotension, hypona­

Measure AM cortisol:

Secondary adrenal insufficiency presents with predominant cortisol deficiency (aldosterone

tremia, hypoglycemia, weight loss

1 8 mcg/dl: Al unlikely TSH

Cold intolerance, lethargy, con­ stipation, dry skin, delayed deep tendon reflex relaxation, weight gain

Free T4 (TSH is not diagnostic)

insufficiency Secondary hypothyroidism; rule out ACTH deficiency before starting on hormone replacement, as levothyroxine increases cor­ tisol clearance and can precipitate adrenal crisis

GH

Short statu re in children; adu lts present with decreased bone density, muscle atrophy, increased fat mass and

Deficiencies of various other pitu­ itary hormones is suggestive Insulin-like growth factor (IGF)-1 GH stimulation test

dyslipidemia FSH/LH

Females: primary amenorrhea, secondary amenorrhea, infertility Males: decreased energy and

LH/FSH Testosterone Estrogen

Hypogonadotropic hypogonadism Measurements of serum prolactin levels in males can rule out hypogonadism secondary to a prolactinoma

libido, infertility, loss of male pattern hair, gynecomastia, testicular atrophy ADH

Polyuria, polydipsia

U rine osmolality and plasma osmolality

Central diabetes insipidus

If abnormal, perform water depriva­ tion test Prolactin

Failure to lactate after delivery

Serum prolactin

Usually occurs i n conjunction with other pitu­ itary hormone deficiencies

History/PE DI presents with polydipsia, polyuria, and persistent thirst with dilute unne. If access to water is limited (eg, in people who are institutionalized or older adults), patients may present with dehydration and severe hyperna­ tremia, which lead to altered mental status, lethargy, seizures, and coma. Diagnosis Lab tests: Serum osmolality > urine osmolality, possible hypernatremia.

.!. urinary sodium, and

I A 23-yea r-old man with a h i story of schizo p h renia presents with com p l a i nts of fatig ue, wea kn ess, cram ps, and headache for the past severa l days. He deni es any oth er sym pto ms, a lthou g h h e had t o u rinate severa l times while i n the office. Routi n e l a bs reveal hyponatremia. With water deprivation, h i s u rine os m olal ity i. What is the most l i kely d iagnosis ?

1 44

H I G H -YI ELD FACTS I N

ENDOCRINOLOGY Dehydration phase

Desmopressin phase ----- Normal

1000

Central DI

800

-�

600

Primary polydipsia Partial nephrogenic DI

400 200

Complete nephrogenic DI

2 F I G U R E 2.3- 1 4. USM LE-Rx.com.) TA B L E 2.3-1 1 .

4

6 8 Time (hours)

10

12

Water deprivation test: DI; Diabetes insipidus. (Reproduced with permission from

Water Deprivation Test

CENTRAL DI

NEPHROGENIC DI

PRIMA RY POLYDI PSIA

Serum sodium

High

Normal

Low

Response to water depriva­

Mild i or no change

Mild i or no

i >600 mOsmol/

i

No response

No response

tion (urine osmolality) Response to desmopressin

change

kg

(urine osmolality)

■

■

■

The most l i kely d iagnosis i s primary (psychogen ic) polyd i psia, a condition i n whic h patie nts consume l a rg e vo l u mes o f hypoto ni c f1 u id, res u lti n g i n polyuria. I t most often occu rs i n patients with psyc h iatric d isorders. Patients prese nt with sym pto ms s i m i l a r to DI, but fo l l owing a water dep rivation test, u r i n e o s m ol a l ity i (vs DI, i n which u ri n e rem a i n s d i l ute) .

Water deprivation test: In psychogenic polydipsia and normal renal physi­ ology, water restriction will lead to more concentrated urine. In central and nephrogenic DI, patients excrete a high volume of inappropriately dilute urine (see Fig. 2.3-14, Table 2.3-11). Desmopressin acetate replacement test: ■ Also known as vasopressin, a synthetic analogue of ADH. ■ Central DI: J, urine output and i urine osmolarity (by 50%-100%). ■ Nephrogenic DI: No effect on urine output or urine osmolarity. MRI may show a pituitary or hypothalamic mass in central DI.

Treatment ■ Treatment of underlying cause. ■ Central DI: Administration of desmopressin intravenously, intranasally, or orally. ■ Nephrogenic DI: Salt restriction, hydrochlorothiazide, amiloride, low­ protein diet in adults.

EXCESS OF PITUITARY HORMONES Acromegaly Elevated CH levels in adults, most commonly caused by a benign pituitary CH-secreting adenoma (see Fig. 2. 3-15). Children with excess CH produc­ tion present with gigantism.

ENDOCRINO LOGY Sleep, hypoglycemia, stress, puberty, exercise

-

Aging, obesity, hyperglycemia

---- Growth hormone

t t t t t t

Amino acid uptake Protein synthesis DNA and RNA synthesis Chondroitin sulfate Collagen Cell size and number

IGF-1 /4,dd uptake Protein synthesis

Glucose uptake Lipolysis

F I G U R E 2.3-1 s. The hypothalamic-anterior pituitary axis (hypophyseal portal system): acro­ megaly. GHRH, Growth hormone-releasing hormone; IGF- 1 , insulin-like growth factor 1 . (Reprod uced with perm ission from USMLE-Rx.com.)

History/PE ■ Pituitary adenoma may cause headache, cranial nerve defects, and bitem­ poral hemianopsia due to compression of the optic chiasm. ■ Systemic manifestations include: ■ Increased skeletal and soft tissue growth manifesting as enlargement of the skull (frontal bossing, wide-spaced teeth) and hands and feet, malocclusion of the jaw, coarsening of facial features, and carpal tunnel syndrome ■ Degeneration of cartilage resembling osteoarthritis ■ Skin thickening and skin tags Hyperhidrosis ■ Organomegaly (eg, tongue enlargement) ■

Associated with an increased risk for: ■ Obstructive sleep apnea ■ Cardiovascular abnormalities such as hypertension, left ventricular hypertrophy, and cardiomyopathy with diastolic dysfunction (most common cause of death) ■ Type 2 OM ■ Diverticulosis ■ Colon cancer

H I G H -YI E L D FACTS I N

1 45

1 46

H I G H -YI ELD FACTS I N

ENDOCRINOLOGY

F I G U R E 2.3 - 1 6. Diagnostic algorithm for acromegaly. CH, Growth hormone; GHRH,

Clinical suspicion of acromegaly (enlargement of nose, frontal bones and jaw)

growth hormone-releasing hormone; IGF- 1 , insulin-like growth factor 1 . (Reproduced with per­ mission from USMLE-Rx.com.)

IG F-1 level Normal

Ru les out a cromegaly

Elevated

i

Oral g lucose suppression test Adequate GH suppression

Inadequate GH suppression

MRI of bra i n

Rules out acromegaly

Normal pituitary

Eva luate for extra pituita ry ca use of acromega ly (eg, ectopic GH and GHRH-secreting tu mors)

Pituitary mass

Surgical vs medical management

Diagnosis ■

■

Lab tests: Levels of ICF-1 increase with acromegaly; diagnosis can be con­ firmed with an oral glucose suppression test (CH levels will remain ele­ vated despite glucose administration). Baseline CH is not a reliable test, as CH levels fluctuate widely throughout the day (see Fig. 2.3-16). Imaging: MRI shows a sellar lesion.

Treatment

0-,,. KEY FACT

Measurement of IGF-1 levels-not GH levels-can confirm acromegaly!

■ ■

■

Surgery: Trans-sphenoidal surgical resection. Medical therapy: Octreotide or lanreotide (somatostatin analogues) to suppress CH secretion; pegvisomant (a CH receptor antagonist) to block the peripheral actions of CH. Radiation: Effective when surgical and medical therapies fail.

HYPERPROLACTINEMIA Hyperprolactinemia refers to elevated prolactin levels, most commonly caused by a pituitary adenoma (see Fig. 2.3-17). Prolactinoma is the most common functioning pituitary tumor. Other causes include physiologic ones (pregnancy, lactation); pituitary stalk compression from other masses (eg, cra­ niopharyngioma, meningioma, nonsecreting pituitary tumor); hypothalamic dysfunction; drugs (eg, dopamine antagonists, selective serotonin reuptake inhibitors [SSRis ]); and systemic conditions such as renal failure, cirrhosis, and hypothyroidism.

0-,,. KEY FACT

Rule out pregnancy in all cases of hyperprolactinemia!

H i story/PE

Elevated prolactin inhibits CnRH secretion and consequently lowers LH and FSH secretion, manifesting as infertility, galactorrhea, gynecomastia, impo­ tence, and amenorrhea. Bitemporal hemianopsia may also be present. Diagnosis ■ ■

Serum prolactin level is typically > 200 ng/mL. Pregnancy test to exclude pregnancy. MRI shows a sellar lesion.

ENDOCRINO LOGY

Stimuli

Hypothalamus

Medications Chest wall i nj u ry (via ANS) Pri mary hypothyroidism N i pple stimulation Sig ht/cry of baby

1

Dopamine

TRH

1 47

F I G U R E 2.3- 1 7. The hypothalamic-anterior pitu­ itary axis (hypophyseal portal system): Prolactin regulation. ANS, Autonomic nervous system; FSH,

follicle-stimulating hormone; GnRH, gonadotropin­ releasing hormone; LH, luteinizing hormone; TRH, thyrotropin-releasing hormone. (Reproduced with permis­ sion from USMLE-Rx.com.)

P rolactin

Anterior pituitary

P eripheral action

Preg nancy: Estrogen

H I G H -YI E L D FACTS I N

Gonadal axis (GnRH)

Ovulation Spermatogenesis

Ci>

Milk production

Treatment ■ Best initial treabnent: Dopamine agonists (eg, cabergoline, bromocriptine). ■ Trans-sphenoidal surgery: Indicated in adenomas refractory to medical management or with compressive effects (eg, visual loss). ■ Radiation: Rarely indicated.

SYNDROME OF INAPPROPRIATE ANTIDIURETIC HORMONE SECRETION Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a common cause of euvolemic hyponatremia that results from persistent ADH release independent of serum osmolality.

H istory/P E May be associated with central nervous system (CNS) disease (eg, head injury, tumor), pulmonary disease (eg, sarcoid, chronic obstructive pulmo­ nary disease [COPD], pneumonia), ectopic tumor production/paraneoplastic syndrome (eg, small cell lung carcinoma), and drugs (eg, antipsychotics, anti­ depressants, NSAIDs). Euvolemic on physical exam.

Diagnosis Serum osmolality 100 mOsm/kg_ in the setting of serum hypo-osmolarity without a physiologic reason for T ADH (eg, congestive heart failure, cir­ rhosis, hypovolemia) ■ Urinary sodium level often 2:40 mEq/L

■ ■

Treatment ■ Exploration of and addressing the underlying cause ■ Best initial treatment: Restriction of fluid ■ Persistent or symptomatic hyponatremia ( 9 \CWY-CRM > 2 months)

I

I I

1st dose

I I

I

1 dose st

I

2-dose series

21Hl dose

2 dose

I

I

I I

al B (MenB)

�I polysaccharide

■

I I I

Jmavirus (HPV)

:ond dose

I

nd

theria, and acellular p: > ? years)

id ages for all children:

Third dose

■

Fourth dose

■

Fifth dose

17-

3• dose

V41

epA)

16 years

2nd dose

Annual vaccination 1 or 2 doses

1ps. rubella (MMR)

13-lSyears

3" dose

RVl (2-dose series); eries)

lliovirus (IPV:

15 months

D certain Range of recommended ages for D catch-up Range of recommended ages for high-risk groups immunization

I

D and Recommended based on shared clinical decision-making can be used in this age group

I I I

!' dose

I I I

I

I

D Not routinely recommended

Recommended vaccinations for children 0-1 8 years of age. (Data collection courtesy of the Centers for Disease Control and Prevention, Atlanta, GA, httpsJ/www.cdc.gov/vaccines/schedu les/downloads/chik ned-schedule.pdf. Data from 202 1 .)

1 72

H I G H -YI ELD FACTS I N

Influenza inactivated (IIV) or Influenza recombinant (RIV4) Influenza live, attenuated (LAIV4) Tetanus, diphtheria, pertussis (Tdap o r Td) Measles, mumps, rubella (MMR) Varicella (VAR) Human papillomavi rus (HPV)

a,

'iJ

Zoster recombinant (RZV) Pneumococcal (PCV15, PCV20, PPSV23) Hepatitis A (HepA) Hepatitis B (HepB) Meningococcal A, C, W, Y (MenACWY) Meningococcal B (MenB) Haemophilus influenzae type B (Hibl

EPIDEMIOLOGY

19-23 years

Age

I

46-49 years

27-45 years

24-26 years

50-65 years

1 dose annually

I

1 dose annually

> 65 years

or

1 dose Tdap each pregnancy; 1 dose Td/Tdap for wound management 1 dose Tdap + Td or Tdap booster every 10 years I

I

I

I

1 or 2 doses if indicated (born before 1957) I

2 doses (born after 1980) I

2 or 3 doses depending on age of initial vaccination or conditions

2 doses

I

2 doses for immunocompromising conditions

2 doses

.I

1 dose PCV15 followed by PPSV23 OR l dose Pero 2 or 3 doses depending on vaccine 2, 3, or 4 doses depending on vaccine or condition 1 or 2 doses depending on indication, then booster every five years if risk remains 2 or 3 doses depend ing on vaccine 1 or 3 doses depending on indication

Recommended for adults who meet the age requirement, 0 Recommended for adults with other indications 0 lack documentation of vaccination or lack evidence of past infections

D Recommended based on shared clinical decision-making O No recommendation

F I G U R E 2 .4- 1 1 . Recommended vaccinations for adults. (Data collection courtesy of the Centers for Disease Control and Prevention, Atlanta, GA, https//www.cdc.gov/ vaccines/schedu les/down loads/adu lt/adu lt-combined-sched ule.pdf. Data from 202 1 .)

EPID EMIOLOGY

H I G H -Y I E L D FACTS I N

1 73

Medical condition or other indication Pregn a ncy

lmmun oco mpro mised (excluding HIV infecti o n)

HIV infectio n CD4 percenta ge a nd co unt 200 mm 5

Asplenia, co mplement def ciencies i

Influenza inactiva ted (IIV) o r Influenza reco mbin a nt (RIV4)

�

1 dose Tdap each pregnancy

Measles, mumps, rubella (MMR)

Contraindicated

Contraindicated

Va ricella (VAR)

Contraindicated

Contraindicated

Hum a n pa pillo m avirus (HPV)

Not recommended

Precaution

Men who have sex with men

Hea lth ca re perso nnel

1 dose annually

Zoster reco mbina nt (RZV)

1 or 2 doses depending on indication

2 doses

3 doses through age 26 years

2 or 3 doses through age 26 years depending on age at initial vaccination or condition

2 doses at age > 19 years

2 doses at age > SO years

1 dose PCV15 followed by PPSV23 or 1 dose PCV20

2 or 3 doses depending on vaccine

Hepatitis A (HepA)

Hepatitis B (HepB)

3 doses

Meningococca l A. C. W, Y (MenACWY)

1 or 2 doses depending on indication

1 or 2 doses depending on indication

o i benef t of i :���:�t��;�������� r�sr�����::;:����o �

2 or 3 doses depending on vaccine

1 or 2 doses depending on indication

2 or 3 doses de ending on vaccine and indication

Precaution

a e i �e;���:����ci ���i���:��i����:C�fn�:� :,�:zke evidence of pa st infectio ns

2 or 3 doses depending on vaccine

2,3 o r 4 d oses depending on vaccine or condition

3 doses HSCT recipients only

H a em o philus influenzae type B (Hibl

□

Dia betes

1 dose Tdap, then Td o r Tdap booster every 10 years

Pneumococca l (PCV!S, PCV20, PPSV2l)

□

Chro nic liver disease

or Contraindicated

Teta nus, diphtheria , pertussis (Tda p o r Td)

Mening ococca l B (MenB)

Hea rt o r lung disease; a lco h o lism

1 dose annually

Influenza live, attenua ted (LAIV4)

·o

End-stage ren a l disease, o r on hem odia lysis

! dose

0 Contraindicated or not recommended- vaccine should not be administered

7N

o

reco mmendatio n/ N ot a pplica ble

F I G U R E 2.4-1 2. Recommended vaccines for special populations. (Data collection courtesy of the Centers for Disease Control and Prevention, Atlanta, GA, httpsJ/www.cdc. gov/vaccines/schedules/downloads/adult/adult-combined-schedule.pdf. Data from 202 1 .)

Live vaccines should not be administered to patients with immunosup­ pression (Fig. 2.4-12). They are also contraindicated in pregnant patients, owing to a theoretical risk for maternal-fetal transmission. A possible excep­ tion to this rule can be some asymptomatic HIV/AIDS patients who may be candidates for the measles, mumps, and rubella (MMR) vaccine.

COVID - 1 9 VACCIN ES At the time of writing this book, in the light of the ongoing pandemic, COVID-19 vaccines are recommended for everyone aged 6 months and older. Number of doses, duration between doses, and need for booster doses vary by manufacturer. Three commonly available vaccines are Pfizer-BioN­ tech, Moderna, and Janssen/Johnson & Johnson.

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SCREENING RECOMMENDATIONS Tables 2.4-4 and 2.4-5 outline recommended healthcare screening measures by gender and age. TA B L E 2 . 4 - 4 .

AGE I N YEARS 1 9-39

Health Screening Recommendations for Women by Age

RECOMMEN DATION CARDI OVASCULAR

BREAST/REPROD UCTIVE

OTHER

BP screening at least once every 2 years

Pap test every 3 years starting at 2 1 years of age; co-testing (Pap + HPV) may be done every 5 years

Diabetes: Blood glucose or H bA,c screening starting if BP> 1 35/80 mm Hg or taking medi­

Cholesterol screening starting at 20 years

starting at 30 years of age Chlamydia test yearly until 24 years of age if sexually active. Women 2:25 years of age should be tested

cation for hypertension

of age for patients at i risk of heart disease

40-49

BP screening at least once every 2 years Cholesterol screening for women >45 years of age

only if there is an i risk HIV test at least once to ascertain status Test for gonorrhea and syphilis if at i risk Pap test every 3 years or co-testing every 5 years Pelvic exam yearly; chlamydia test if patient has new or

multiple partners HIV test at least once to ascertain status Test for gonorrhea and syphilis if at i risk

Diabetes: Blood glucose or H bA 1 c screening if BP > 1 35/80 mm Hg or taking medication for hypertension Colorectal: For patients >45 years with no family history; FOBT yearly; flexible sigmoid­ oscopy every 5 years or colonoscopy every 1 O years

50-64

Mammogram once every 1 -2 years (can start at 40 years of age, if patient chooses) Pap test every 3 years Chlamyd ia test if patient has new or m u ltiple

Diabetes: Blood glucose or H bA 1 c screening if BP > 1 35/80 m m Hg or taking medication for hypertension Bone: DEXA scan can be done i n patients with

partners HIV test at least once to ascertain status Test for gonorrhea and syphilis if at i risk

other osteoporosis risk factors Colorectal: FOBT yearly; flexible sigmoidoscopy every 5 years or colonoscopy every 1 0 years

BP screening at least once every 2 years

Mammogram once every 1 -2 years until 75 years of age Discuss Pap test with physician or nurse

Diabetes: Blood gl ucose or H bA 1 c screening if blood pressure higher than 1 35/80 or

Cholesterol screening

Chlamydia test if patient has new or multiple partners Discuss HIV test with physician or nurse Test for gonorrhea and syphilis if at i risk

taking medication for hypertension Bone: DEXA sca n at least once Colorectal: Screening with FOBT, sigmoidos­ copy, or colonoscopy every 1 0 years u ntil 75

BP screening at least once every 2 years Cholesterol screening for women >45 years of age

for women >45 years of age

yea rs of age Modified with permission from the U.S. Department of Health and Human Services, Washington, DC.

BP, Blood pressure; DEXA, dual-energy x-ray absorptiometry; FOBT, fecal occult blood test; HbA 1 c, hemoglobin A 1 c; HPV, human papillomavirus.

EPID EMIOLOGY TA B L E 2 . 4 - s .

AGE I N YEARS 1 9-39

H I G H -YI E LD FACTS I N

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Health Screening Recommendations for Men by Age

RECOMMEN DATION CARDIOVASCULAR

REP RODUCTIVE

OTH ER

BP screening at least once every 2 years

Both partners should be tested for STls, including H IV, before initiating sexual

N/A

Cholesterol screening starting at 20 years of age for patients at i risk for heart disease. Screen

intercourse Test for syphilis if at i risk

all men > 35 years of age 40-49

BP screening at least once every

Discuss DRE and PSA with physician or

Diabetes: Blood glucose or HbA 1 c screening if

2 years Cholesterol screening for all

n u rse HIV test at least once to ascertain status Test for syphilis if at i risk

BP > 1 35/80 mm Hg or taking medication for hypertension Colorectal: For patients age >45 with no family

men > 35 years of age

history; FOBT yearly; flexible sigmoidoscopy every 5 years or colonoscopy every 1 O years 50-64

BP screening at least once every 2 years Cholesterol screening for all men > 35 years of age

Discuss DRE and PSA with physician or n u rse HIV test at least once to ascertain status

Diabetes: Blood glucose or H bA,, screening starting if BP > 1 35/80 mm Hg or taking medication for hypertension

Test for syphilis if at i risk

Colorectal: Screening with FOBT yearly; flexible sig­ moidoscopy every 5 years or colonoscopy every 1 0 years Lung: Cancer screening with low-dose CT for indi­ vidual with > 20-year smoking history who are presently smoking or q uit within the last 1 5 years

BP screening at least once every 2 years Cholesterol screening for all men > 35 years of age

Discuss DRE and PSA with physician or n u rse

Diabetes: Blood glucose or HbA,, screening starting if BP > 1 35/80 mm Hg or taking medication for

Discuss HIV test with physician Test for syphilis if at i risk

hypertension Colorectal: Screening with FOBT; flexible sigmoidos­ copy every 5 years or colonoscopy every 1 0 years until age 75 Abdominal aortic aneurysm: One-time screening for men who have ever smoked or have a family history Lung: Cancer screening with low-dose CT for indi­ vidual age less than 81 with > 20-year smoking history who are presently smoking or quit within the last 15 years

Modified with permission from the US Department of Health and Human Services, Washington, DC. BP, Blood pressure; DRE, digital rectal exam; FOBT, fecal occult blood test; HbA 1 c, hemoglobin A 1 c; PSA, prostate-specific a ntigen; ST/s, sexually transmitted infections.

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EPIDEMIOLOGY

REPORTABLE DISEASES By law, disease reporting is mandated at the state level, and the list of diseases that must be reported to public health authorities varies slightly by state. The Centers for Disease Control and Prevention (CDC) has a list of nationally notifiable diseases that states voluntarily report. These diseases include but are not limited to those listed in Table 2.4-6. TA B L E 2 . 4 - 6 .

DISEASE CATEGORY

Common Reportable Diseases

EXAMPLES

STls

H IV/AI DS, syphilis, gonorrhea, chlamydia, chancroid, HCV

Tick-borne disease

Lyme d isease, ehrlich iosis, Rocky Mountain spotted fever

Potential biowea pons

Anthrax, small pox, plague

Vaccine-preventable

Diphtheria, tetan us, pertussis, measles, m u m ps, rubella, polio, va ri­

disease

cel la, HAV, H BV, H influenzae (invasive), meningococcal disease

Water-/food-borne

Cholera, giard iasis, Legionella, listeriosis, botulism, shigel losis,

disease

Shiga toxin-prod ucing Escherichia coli, salmonel losis, trichinel losis,

typhoid

Zoonoses

Tu laremia, psittacosis, brucel losis, rabies

M iscella neous

TB, leprosy, toxic shock synd rome, SARS, COVI D-1 9, West Nile virus, VRSA, coccidioidomycosis, cryptosporidiosis; MRSA is reportable in several states

HAV, Hepatitis A virus; HBV, hepatitis B virus; MRSA, methici l l i n-resistant Staphylococcus aureus; SARS, severe acute respiratory synd rome; ST/s, sexua lly transmitted infections; TB, tubercu losis; VRSA, va ncomycin-resista nt 5 aureus.

HEALTH SYSTEMS SCIENCE Health System Delivery HEALTH INSURANCE PLANS MEDICARE AND MEDICAID PALLIATIVE (ARE

Communication PATIENT-(ENTERED, EVIDENCE-BASED INTERVIEWING EFFORTS TO ESTABLISH RA PPORT (HALLENGING (ONVERSATIONS GENDER- AND SEXUALITY-INCLUSIVE HISTORY TAKING (ULTURALLY INCLUSIVE HISTORY TAKING MOTIVATIONAL INTERVIEWING (OMMUNICATING WITH PATIENTS WITH DISABILITIES INTERPRETERS BEHAVIORAL (OUNSELING

Patient Safety and Quality SAFETY CULTURE PDSA CYCLE Swiss CHEESE MODEL MEASURING QUALITY OUTCOMES ERRORS IN HEALTHCARE HEALTHCARE WORKER BURNOUT AND FATIGUE ANALYZING MEDICAL ERRORS

Ethics and Legal Issues GENERAL./(ORE PRINCIPLES DOCTOR-PATIENT PROFESSIONAL RELATIONSHIP DOCTOR-PATIENT SEXUAL RELATIONSHIP

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1 79 1 80 1 81 1 81 1 81 1 82 1 82 1 82 1 82

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1 82 1 83 1 83 1 83 1 84 1 84 1 85

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1 85 1 86 1 86

Competence and Decision-Making Capacity (OMPETENCE DECISION-MAKING (APACITY INFORMED CONSENT

End-of-Life Issues ADVANCE DIRECTIVES SURROGATE DECISION MAKING WITHDRAWAL OF LIFE-SUSTAINING TREATMENT EUTHANASIA AND CLINICIAN-ASSISTED SUICIDE HOSPICE (ARE FUTILE TREATMENT

1 86 1 86 1 87 1 87

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Complementary and Alternative Medicine Therapy

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Disclosure

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FULL DISCLOSURE SITTING FOR DELIVERING NEWS

1 91 1 92

Confidentiality

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Clinical Research

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(ORE PRINCIPLES OF CLINICAL RESEARCH ETHICAL (ONCERNS

Conflict of Interest GIFTS FROM PATIENTS GIFTS FROM DRUG (OMPANIES

Malpractice DEFINING MALPRACTICE IMPAIRED PRACTICING CLINICIANS

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1 95

1 95 1 95

1 96 1 96 1 96

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HEALTH SYSTEMS SCIENCE

HEAL TH SYSTEM DELIVERY HEALTH INSURANCE PLANS Patients may receive insurance from various providers that reimburse the insured patients based on the services they utilize. These services may provide payment to the clinician or organization in one of the following ways: ■

■

■ ■

■

Bundled payment: Organization receives set amount per service, regard­ less of ultimate cost, with the received amount divided among all provid­ ers and facilities involved. Capitation: Clinicians receive a set amount per patient per period regard­ less of the healthcare utilization. This is usually provided by health main­ tenance organizations. Fee-for-service insurance: The patient or insurer pays for each individual service. Discounted fee-for-service insurance: The patient or insurer pays for each utilized service at a discounted rate predetermined by providers and pay­ ers. This service is mostly used by preferred provider organizations. Global payment: The patient or insurer pays for all expenses for a single incident of care, with a single payment once the service is used. For exam­ ple, payment for elective surgeries may cover the cost of the surgery and the necessary preoperative and postoperative visits.

MEDICARE AND MEDICAID Many patients may have Medicare or Medicaid enrollment, and this may affect the services they choose to utilize (Table 2. 5-1).

TA B L E 2.5- 1 .

Medicare vs Medicaid

MEDICARE

MEDICAID

Administrating body

Federal

Federal and state

Type of program

Insura nce

Assistance

Eligibility

Patients 2:65 years of age, younger patients with disability, patients undergoing dialysis

Low-income patient of any age

Bill payment

Trust funds

Patients do not pay any part of expenses

Copays and

Small monthly premiums for nonhospital coverage

Small copayment (in some cases)

Part A: Hospital admissions including hospice, skil led n u rsing Part B: Basic medical bills (eg, clinician fees, diagnostic testing)

Basic healthcare and prescription drug costs, long-term care, medical equipment, prescrip­ tion glasses, dental care, and more

deductibles Coverage

Part C: Delivered by approved private companies (delivers all services of parts A and B) Part D: Prescription drugs

HEALTH SYSTEMS SCIENCE PALLIATIVE CARE This is an approach to improve the quality of life for patients experiencing life-threatening illness and for their families. The goals of palliative care are to minimize suffering and to provide care that is consistent with the patient's val­ ues. This includes addressing symptoms such as pain, dyspnea, or any other physical symptoms; psychological distress; and social issues. A palliative care team also provides support to families during the patient's illness and their family's subsequent bereavement. Care is provided via a team approach (eg, clinicians, nurses, social workers) to support patients and their caregivers. The palliative care approach has five stages: 1. Stable: A treatment plan is created, and medical interventions are placed to control symptoms and enhance quality of life. 2. Unstable: Existing symptoms worsen, or patients may have unexpected symptoms, and the care of plan is changed. This stage requires provision of mental, emotional, and spiritual support. 3. Deteriorating: Overall health and body functions of the patient gradually decline, leading to mental and physical distress in the patient and for their family. Emotional support is key during this stage. 4. Terminal: The patient may become bedridden, have loss of appetite, have difficulty in swallowing, and may require daily medical interventions. A hospital setting may be needed for further care provision. 5. Bereavement: This is the stage where the patient has died. The care plan focuses on caring for the patient's loved ones and addressing their emo­ tional needs, including connection with specific support groups; spiritual support, eg, via a pastor, priest, or rabbi; and psychosocial support to tackle grief, loss, and adjustment. When the prognosis for the patient is less than 6 months, or when life­ prolonging treatment is no longer beneficial, the patient may be referred to end-of-life care or hospice care.

COMMUNICATION PATIENT - CENTERED, EVIDENCE - BASED INTERVIEWING There are certain key ways to make a patient feel comfortable being inter­ viewed about their medical history. Following these steps can help obtain a reliable history. These steps are outlined here: ■ Setting the stage: Welcome the patient and use the patient's preferred name or pronouns to address them directly. Introduce yourself and identify your specific role. Ensure privacy and comfort for the patient. When patients are accompanied by a caregiver, let them introduce themselves and clarify with the patient if they would want their caregiver to be included in the discussion. ■ Agenda of interview: Indicate the time available and obtain a list of issues the patient wants to discuss. Let the patient understand how the interview will proceed, and summarize the agenda for that interview. ■ Nonfocused expression/reflection: Always start with open-ended ques­ tions or requests and use nonverbal cues (eg, patient gestures) to obtain more information.

H I G H -YI E LD FACTS I N

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HEALTH SYSTEMS SCIENCE

Focused expression/validation: Elicit further history with a focus on description of symptoms, with perspective of impact on the patient's per­ sonal, psychosocial, and emotional contexts. Identify the patient's beliefs and attributions, while addressing feelings and emotions (Naming, Under­ standing, Respecting, Supporting, Exploring, or the NURSE technique). ■ Recapitulate: Check accuracy by providing a brief summary of what has been dealt with and what needs further input. ■ Facilitation: Encourage the patient to speak freely and ask questions throughout the interview. ■ Open-ended skills: Can be nonfocusing techniques like silence (to allow patient to speak), nonverbal encouragement, and prompting the patient to continue with their story, or focusing techniques like echo­ ing (repeating what the patient just said and asking if this is correct), requesting, and summarizing. ■ Emotion-seeking skills: Ask directly how this condition or situation affects the patient's emotions. Indirect assessment can include impact of condition on life, beliefs the patient has about their problems, and any triggers that relate to the problem. ■ Empathy: Name, Understand, Respect, Support, Explore.

EFFORTS TO ESTABLISH RAPPORT Establishing rapport may be the most difficult part of the medical interview, but there are certain steps to follow for this process as well. Establishing rap­ port can be done by either the PEARLS model (see Table 2. 5-2) or the Ask­ Tell-Ask model. TA B L E 2.s-2.

PEARLS Model of Establishing Rapport

KEY STEP

DEFI N ITION

EXAM PLE

Partnership

Working with the patient to identify primary problems and

You may have difficult times a head, but we will work

preferred solutions

together to provide you the best possible care consistent with your goals, values, and beliefs.

Empathy

Identifying emotions displayed and understanding why the patient feels that way

You appear sad. I understand that the news about you r illness may have upset you, especially with your family so far away right now.

Apology

Taking personal responsibility when appropriate

I am very sorry that I had to attend to personal business and you had to wait for me. However, I still would like to understand your concerns so that I can help you, if you choose to allow me.

Respect

Positively encouraging patients, especially when they discuss a difficult problem, helping them navigate through challenging circumstances, or other constructive behavior

I understand that you have had a tough time trying to stop drinking alcohol. I also know that you have given your best effort every time, and I appreciate the effort you put in.

Legitimization

Validating the patient's emotions and letting them know that feeling a certain way during challenging situations is normal or com mon

It is understandable to feel anxious about the uncertainty as we move forward to identify the problem and complete the workup.

Support

Reassuring the patient about the presence of your continued support throughout the patient's time of need and offering

I am here to support you through the process and answer any q uestions you may have along the way.

them appropriate resources to tackle such situations

HEALTH SYSTEMS SCIENCE

H I G H -YI E LD FACTS I N

1 81

CHALLENGING CONVERSATIONS When delivering challenging or difficult-to-break news to patients, one should think about the following (SPIKES): ■ ■

■

■

■

■

Setting: As with effective history taking, one should ensure a private envi­ ronment free from distractions when presenting news to the patient. Perception: Before proceeding with delivering the news, always ask first what the patient thinks about their illness and what their understanding of their current state of health is. Invitation: Ask the patient if they have any queries they would like answered and the amount of information that they would like to obtain from the clinician before delivering the news. Knowledge: Educate the patient about the news in small pieces, allowing time for them to process. Assess the patient's understanding of the news step by step. Emotions: Acknowledge that the patient responds to the news in a certain way and acknowledge their emotions. When dealing with patients' emo­ tions, always be empathetic to their responses and listen to them without interrupting. Strategy: Once the patient feels ready to further discuss treatment options or prognosis and goals of care, offer them an agenda about how to pro­ ceed, and schedule their appointments around these agendas.

GENDER- AND SEXUALITY -INCLUSIVE HISTORY TAKING When interviewing the patient, always take into consideration the following points: ■ Avoid making assumptions about a patient's sexual orientation, gender identity, gender expression, or sexual behavior without first asking the patient about these. If gender or sexuality is not relevant for the presenting illness, do not bring it up. ■ Note that the patient may identify as a different gender than the sex assigned to them at birth. ■ Try to use gender-neutral terms (eg, partner or spouse) rather than assum­ ing their gender. ■ Always reassure the patient that whatever they share is confidential and even if they are not open about their sexual orientation or gender identity, this information will not be revealed to anyone else without the patient's prior permission or request.

CULTURALLY INCLUSIVE HISTORY TAKING As stressed earlier, patients have their own cultures and specific goals, values, or beliefs and may not accept the treatment plan proposed for them. Always note the following points when interviewing patients: Try to think about the illness from the patient's perspective. Try to understand what factors in the patient's cultures or background (or even some past experiences) influences certain decisions they make. ■ Also note how their culture would affect future decisions for treatment.

■ ■

For example, note that patients who belong to the Jehovah's Witnesses may refuse lifesaving blood transfusion for themselves (if adult and compe­ tent), but they cannot do the same if their child (who is below the legal age and not an emancipated minor) because this amounts to child abuse.

0-,,. KEY FACT

When treatment can save a child's life, or if the outcome of treatment is a normal life with a reasonably good quality of life, or if the medical community is in agreement about the course of action and if the parent is refusing to grant consent to proceed, the healthcare decisions for that child can be taken over by the State.

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HEALTH SYSTEMS SCIENCE MOTIVATIONAL INTERVIEWING Make sure to counsel the patient on every visit about improving their behavior to help treat their illness if these behaviors affect them directly or indirectly. For example, if a patient smokes, mention smoking cessation at every visit. When the patient notes a desire to change their behavior, document this step and encourage them. If the patient is not expressing such a desire, assess any barriers that may lead to difficulty in changing these behaviors. Expressing a desire to change their behavior is the first step to changing the behavior itself. Once a patient has expressed a desire to change, set goals for the patients to meet. These goals should be Specific, Measurable, Achievable, Relevant, and Time bound (SMART).

COMMUNICATING WITH PATIENTS WITH DISABILITIES When interviewing a patient who has a disability or disabilities, speak directly to the patient and ask them how they identify themselves: person first (ie, a person with a disability) or identity first (ie, a disabled person). Obtain addi­ tional information from caregivers when appropriate. Do not assume that these patients cannot complete a certain task; rather, ask them if they require assistance. ■ For patients with speech difficulties, provide extra time for an interview. ■ For patients with cognitive impairment, use specific language and ask sim­ ple, direct questions. For patients who are hard of hearing ask them about their preferred mode of communication. Consider using an interpreter when necessary. Also, do not bring up a disability if it is not relevant to the presenting ill­ ness. Do not skip portions of the physical examination even if the disabil­ ity makes the exam challenging.

INTERPRETERS When patients who do not speak English or have difficulty in conversing in the language, always use a professionally trained medical interpreter for com­ munication unless the clinician is conversationally fluent in the patient's pre­ ferred language. If professional interpreters are unavailable or if you are working with the patient outside service hours, a telephone or video call may provide the same service. If the patient prefers to use a family member for interpretation, this should be recorded in the chart. In emergencies, facilitate communication using any tools available (eg, friends, family, interpreter apps) even though this may not be standard procedure otherwise.

BEHAVIORAL COUNSELING When offering counsel, clinicians should tailor their education and sugges­ tions to the individual patient, as well as to their stage of change (see Table 2. 5-3 and Fig. 2. 5-1).

PATIENT SAFETY AND QUALITY SAFETY CULTURE This is a part of an organization's culture, which is a collection of the beliefs, perceptions, and values that the employees share in relation to risks within the

HEALTH SYSTEMS SCIENCE TA B L E 2.5-3.

H I G H -YI E L D FACTS I N

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Stages of Change in Behavioral Counseling

STAGE OF CHANGE

CHARACTERIZATION

EXAM PLE

Precontemplation

Denial or ignorance of the

A patient with a substance use disorder

problem Contemplation

Ambivalence or conflicted emotions; assessing bene­ fits and barriers to change

Preparation

Experimenting with small changes; collecting i nfor­ mation about change

Action

Taking direct action toward achieving a goal

Maintenance

Maintaining a new behavior; avoiding temptation

has not even thought about cessation A patient with a substance use dis­ order considers treatment for his addiction A patient with a substance use disorder visits his doctor to ask ques­

Precontemplation No intention of changing behaviOr

enters a rehabilitation facility for treatment of addiction

no specific actions

l

i

Preparation Made the decision to change, intentllfl taking action

Maintenance Sustaining change

Action Active modification ofbehavior

tions about quitting A patient with a substance use disorder

Contemplation Problem acknowledgment,

Relapse Fall back into old patterns of behavior

F I G U R E 2.5- 1 .

/

Stages of change model.

(Reproduced with perm ission from USMLE-Rx.com.)

A patient with a substance use disorder contin ues to visit recovery meetings to gain su pport and reinforcement against relapse

1. Plan

2. lmplement Implement the plan

Establish the objectives to beachieved

Execute the processes

Establish the processes necessary to deliverresutts

Collect the data

Define the expected output

healthcare sector. To note, the Institute of Medicine noted that the "focus must shift from blaming individuals for past errors to a focus on preventing future errors by designing safety into the system." So, patient safety includes the process of amelioration, avoidance, and prevention of adverse injuries or outcomes that arise because of the healthcare process.

PDSA CYCLE The PDSA cycle involves four key processes (Plan, Do, Study, Act) that help identify possible deficiencies in provisions of care, which when changed, can affect healthcare delivery and improve patient safety. This is a continuous cycle, and it requires reassessment of strategies after implementation of a plan for a set time period (see Fig. 2. 5-2).

4. lmprove

3. Evaluate

Assess the results from the evaluate stage Determine the changes needed in order to ensure the plan·s objectives can be met

F I G U R E 2.5-2.

MEASURING QUALITY OUTCOMES Delivery of quality healthcare can be assessed by various processes that may evaluate the structure, process, outcome, and balance of any healthcare system:

Gather the feedback Compare the rest.Jlts to see if the plan's objectives and requirements have been met

PDSA cycle. (Reproduced with

permission from USM LE-Rx.com.)

SWISS CHEESE MODEL This is a model of risk assessment that focuses on systems and conditions that may align to lead to an adverse event. The Swiss cheese model tries to mitigate such threats at various levels. However, despite provisions of multiple safe­ guards, an error may occur and lead to harm to the patient when the "holes in the cheese line up" (see Fig. 2. 5-3). During a root cause analysis, the goal is to understand how the adverse event occurred, including by understanding how each of these safeguards may have failed. In doing so, efforts to add additional safeguards or revise prior safeguards can help minimize future adverse events.

Study the resultsof the implement stage

Potential failures in defense strategy

strategies

F I G U R E 2.5-3. Swiss cheese model of safeguarding del ivery of care. (Repro­ d uced with permission from USM LE-Rx.com.)

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HEALTH SYSTEMS SCIENCE

■ Structural: Assessment of physical equipment, resources, and facilities. Example: Machine used to evaluate the potassium levels of patients. ■ Process: Assessment of function of the healthcare system as planned. Example: Number of patients undergoing dialysis utilizing the said function. ■ Outcome: Measurement of the impact of the healthcare measure on patients. Example: Average levels of potassium of patients undergoing dialysis. ■ Balancing: Assessment of impact on other systems or outcomes. Example: Average drop in levels of potassium of patients who underwent dialysis compared with the initial levels.

ERRORS IN HEALTHCARE Although a clinician may have no intention to bring about harm to the patient, certain processes may align to bring about errors in healthcare deliv­ ery (see Table 2. 5-4). These are called adverse events (errors in medical or surgical treatment that can potentially lead to patient harm, rather than as an impact of the patient's previous underlying medical condition). The outcome of such events may be unintended injury to the patient, prolonged hospital­ ization, or even disability.

HEALTHCARE WORKER BURNOUT AND FATIGUE The leading causes of errors in provision of care include healthcare worker burnout and fatigue. Burnout may occur with prolonged, excessive stress and lead to reduced professional efficacy. Burnout can be due to intrinsic TA B L E 2.5-4.

Errors in Healthcare Delivery

TYPE OF ERROR

DESCR I PTION

I M PACT

Active error

At the level of the front-line operator

I m m ediate impact (eg, wrong intravenous [IV) dose or il legible hand­ writi ng on orders).

Latent error

Indirect processes that affect patient care

Accident waiting to happen (eg, different types of IV pumps used with in the same hospita l).

Never event

Errors that should never happen

Major error that should never occur (eg, wrong foot am putated).

Near miss

Event that cou ld have led to harm but did not

I ntervention by someone or someth ing that led to prevention but should have not happened (eg, medication i nteractions that may be recognized by the pharmacist and corrected or cancel led).

Medical error

Negl igence

Fai lure to complete the i ntended plan of action

Deviation from process of care. May lead to unintended consequence

or implementation of wrong plan to achieve

(eg, administration of a wrong dose of medication when a full stop was

treatment goal

interpreted as a comma, ie, 1 00 U insulin when the order stated 1 0.0 U).

Fai lure to meet expected standard of care

May lead to direct harm to the patient (eg, m issing the pathology report of a cancer diagnosis).

Senti nel event

Any unexpected occu rrence involving death or serious harm to the patient or the risk thereof

When repeated, there is a significant chance for a serious adverse outcome (eg, using the same bronchoscope for several patients and not cleaning between the proced u res due to miscom m u nication between staff).

HEALTH SYSTEMS SCIENCE

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demands of the job, individual susceptibility, and poor work organization. Deprivation of sleep or rest can lead to cognitive impairment and lead to medical errors. This can be minimized by: ■

Setting clear and consistent goals for the staff Providing training to increase coping strategies, increase role effectiveness, and nurture better conflict resolution strategies ■ Encouraging support groups and resource exchange networks ■ Organizing workload and working time, with regular breaks and flexible schedules ■ Providing accommodation for health workers during emergency opera­ tions with access to food services, sanitary facilities, and adequate recre­ ational opportunities ■

ANALYZING MEDICAL ERRORS When analyzing medical errors, two different models may be commonly used: (1) root cause analysis and (2) failure mode and effects analysis. The root cause approach is applied after a system failure to find out what went wrong, whereas the failure mode and effects analysis is applied before an error occurs to prevent failure. ■

■

Root cause analysis: Typically evaluates sentinel events to decrease the odds of repeating the same event. This process uses records and partici­ pant interviews (eg, five whys approach, fishbone diagrams, cause-effect diagrams, process maps) to identify the underlying cause of error. Failure mode and effects analysis: Concludes that errors will occur even if healthcare professionals are careful. This process helps build redundan­ cies that act as safety nets to trap errors. The process uses inductive reason­ ing to identify all the ways a system might fail and prioritizes them by their probability of occurrence and impact on patients.

ETHICS AND LEGAL ISSUES GENERAL/CORE PRINCIPLES ■

Respect for autonomy: This is absolute. Clinicians are obligated to respect patients as individuals and to honor their preferences. Example: A pregnant patient has the right to refuse a cesarean section despite potential risk to the fetus. This is called the principle of mater­ nal autonomy - as long as the mother has capacity, she has ultimate rights over her unborn child (in the United States), as the fetus is con­ sidered a part of the mother.

■

Beneficence: Clinicians have a responsibility to act in the patient's best interest. Respect for patient autonomy may conflict with beneficence. In general, if a patient is mentally competent, respect for patient autonomy supersedes beneficence even if the clinician believes the patient is not act­ ing in their best interest. ■ Example: The clinician has a responsibility to recommend a lifesaving transfusion to a Jehovah's Witness (beneficence) and respect the patient's autonomy if they should refuse. However, when a parent who is a Jeho­ vah's Witness refuses emergency medical treatment for their child (claiming the same status for the child), the clinician is not obliged to agree with the parent, but must instead provide the necessary treatment to save the life of the child (beneficence outweighs autonomy).

0-n KEY FACT

In some cases (eg, psychiatric illness, grave disability), the Baker Act allows for the involuntary hospitalization of patients against their will if they are deemed a threat to themselves or others and are neglectful.

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Nonmaleficence: "Do no harm." All medical interventions involve benefits and risks, and clinicians should generally only recommend treatments for which the likely benefits outweigh the known risks. Also, it should be made clear to the patient the consequences of refusing treatment if they were to choose so. This category includes most medical and surgical interventions. Example: A surgeon declines to perform a procedure because they think the patient will die intraoperatively. Justice: Healthcare is a scarce resource. Fairness and equality in distribu­ tion and delivery of healthcare are ongoing challenges for health policy and in the clinical arena. ■ Example: A state designs a program for homeless people.

DOCTOR-PATIENT PROFESSIONAL RELATIONSHIP ■

0-,,. KEY FACT Cli nicians a re not obl igated to accept every person com i n g to them as a patient. Furthermore, c l i n icians have the right to end a doctor-patient relation s h i p but m u st g ive the patient the resou rces and time to find a nother c l i n ician.

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The doctor-patient relationship is a voluntary relationship. It occurs when the patient agrees to seek medical attention with a specific doctor and when the doctor agrees to care for the patient. In a hospital setting, emergency management should be provided to all patients. ■ In the absence of an emergent condition, the clinician is under no legal obligation to accept caring for patients. ■ Example: If a pregnant patient is seeking to get an abortion, the clini­ cian is under no legal obligation to perform the procedure if that goes against their moral standards. To end a doctor-patient relationship, the doctor should allow patients to have adequate time to find an alternative clinician.

DOCTOR-PATIENT SEXUAL RELATIONSHIP ■ ■ ■

Sexual contact between clinicians and their patients is always inappropri­ ate, independent of who initiates contact. A patient and clinician should terminate their professional relationship before engaging in a sexual relationship. No clear recommendation exists as to when it is appropriate to initiate a sexual relationship after termination of the professional relationship.

COMPETENCE AND DECISION-MAKING CAPACITY COMPETENCE ■

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A person's global and legal capacity to make decisions and to be held accountable in a court of law. All individuals are competent unless legally proven otherwise. Competence is assessed by the courts and is distinct from the term decision-making capacity. Incompetent patients, as assessed by the courts, or temporarily incapaci­ tated patients may still be able to provide assent for treatment or refuse treatment. However, the need to treat supersedes the refusal of an incapac­ itated patient in emergency situations. ■ Example: A severely hypoxic patient with altered mental status who is unable to accept treatment may receive respiratory supportive therapy, as this constitutes a medical emergency.

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Incompetent patients can nonetheless have adequate decision-making capac­ ity to refuse treatment. In these cases, autonomy supersedes beneficence. ■ Example: Patients living with a psychiatric illness, autism, or intellec­ tual disabilities may be deemed incompetent in managing their finances, while maintaining adequate capacity to refuse treatment.

DECISION- MAKING CAPACITY The ability of a patient to understand relevant information, appreciate the severity of the medical situation and its consequences, communicate clear and consistent choices, and deliberate rationally about their values in relation to the decision being made. This can be assessed by any clinician, based on a neurologic exami­ nation evaluating memory, comprehension, reasoning, and judgment. ■ ■

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Testing for decision-making capacity does not always require a psychiatric consultation. It can be assessed by any clinician. Decision-making capacity is best understood as varying with the complex­ ity of the decision involved. ■ Example: The level of capacity needed for a decision about liver trans­ plantation is different from that needed to choose between two types of pain medication for fracture-related pain. In general, patients who have decision-making capacity have the right to refuse or discontinue treatment. Example: A patient living with cancer with decision-making capacity can opt out of oncologic treatment. A patient's decision to refuse treatment can be overruled if the choice endangers the health and welfare of others. Example: A patient with active tuberculosis (TB) must undergo antibi­ otic treatment because not treating the patient would pose a public health threat. All suicidal patients are considered to lack capacity. Occasionally, psychiatric evaluation can be recommended when decision­ making capacity is equivocal. In cases where capacity is clearly impaired or intact, there is no need for psychiatric evaluation.

INFORMED CONSENT ■ Willing and voluntary acceptance of a specific medical intervention by a patient after adequate discussion with a clinician about the nature of the intervention along with its indications, risks, benefits, and potential alter­ natives (including no treatment). ■ Informed consent should be obtained in the language the patient can clearly communicate and understand. If needed, an official translator should be used. ■ Informed consent is given for a specific procedure, so that autonomy supersedes beneficence. ■ Informed consent should be obtained by the doctor performing the procedure. ■ Patients may change their minds at any time even after agreeing to the procedure. The healthcare team will honor the wishes of the patients and support the decision. ■ Informed consent is required for significant procedures except for the following: ■ When emergency treatment is required. Example: An unconscious patient presents with cerebral edema after a motor vehicle collision, or a patient without previously indicated DNR/DNI (do not resuscitate/do not intubate) status undergoes cardiac arrest.

A 47-year-old ma n is d i a g n osed with pa ncreatic cancer. H i s d iagnosis and treatment opti ons a re d i scu ssed, but the patient d oes n ot want a ny inte rventi o n . He states that he wou l d l i ke t o g o home t o h i s spouse and c h i l d ren to d i e pea cefu l l y. What is the most a ppropriate next step i n manageme nt?

A 5 1 -yea r-old man is b ro u g h t to the emergency department afte r he was struck by a motor ve h i c l e w h i l e crossi n g t h e street. H e is u n respon sive a n d in need of em ergent su rgery. H i s spouse a n d c h i l d re n ca n not be reached. What is the m ost approp riate n ext ste p i n treatment?

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Patients with psych iatric i l l ness ca n g ive consent if their decision-making capacity is i ntact.

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In the case a patient lost consciousness and no family member is around to provide consent for a procedure, a witnessed telephone con­ sent is valid if that is the only way to obtain consent. When a patient lacks decision-making capacity. In this case, consent is still required but must be obtained from a surrogate decision maker. Example: Minors generally require surrogate decision makers until they demonstrate adequate decision-making capacity or are of legal age.

I nformed Consent for I ncapacitated Individua ls When individuals lose consciousness, doctors and caregivers should respect the patient's last known wishes, whether expressed orally or in writing. Oral wishes, however, are more difficult to prove. ■

0.◊ MNEMONIC

BRAIN of informed consent­

Benefits Risks Alternatives Ind ications Natu re

Example: If a patient repeatedly did not want a procedure performed, per­ forming the surgery cannot be performed once the patient loses consc10usness.

I nformed Consent i n Reproductive Health The mother's autonomy supersedes the rights of the fetus. Except when acting as a surrogate decision maker for the pregnant patient, the father has no legal right to provide informed consent on a pregnancy­ related procedure. ■ Adults have access to sterilization. Consent is needed from the patient only and not the partner. ■ ■

I nformed Consent for a Never-Competent Person In the case informed consent should be obtained for a never-competent person but no guardian is present, a court-designated third party should make a deci­ sion for the medical intervention based on the best interests of the patient. I nformed Consent for Minors In general, minors (people < 18 years of age) cannot consent for their own medical treatment and require parents or guardians to consent on their behalf (one parent is sufficient as long as that parent has custody), except in the following situations: ■ Life-threatening emergencies: When parents cannot be contacted, parental consent is implied. A court order is not appropriate, as it delays the urgent intervention. ■ Legal emancipation: Emancipated minors do not require parental consent for medical care. Although emancipation laws vary from state to state, in general minors are emancipated if they are married, are in the armed services, are the parent of a child that they themselves sup­ port, or are financially independent of their parents and have obtained legal emancipation. ■ Sexually transmitted infections and substance abuse treatment: Rules concerning contraception, pregnancy, HIV, and treatment for drug and alcohol dependency vary across the United States. ■ In cases discussed earlier, treating the patient without notifying the parents is acceptable. ■ In cases where minors are seeking an abortion, encouraging patients to dis­ cuss with and notify parents/guardians is likely the best answer. There is no national standard as to whether clinicians should notify parents themselves. ■

Th e most a ppropriate n ext step i n management i s t o respectfu l l y ask the patient a bout h i s reasons fo r not wa nti ng to p u rs u e treatm ent. Patients often n eed cla rification a n d reassu ra nce. If h e conti n ues t o decl i n e treatm ent, a b i d e b y hi s decision (respect for a uton omy) .

T h e most appropriate n ext step is to proceed with the s u rgery. A c l i n i ci a n may give emergent treatment i n t h e a bsence of i nfo rmed consent w h e n i m mediate inte rventio n is n ecessa ry to preve nt serious harm or death.

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In some states, the clinician is left with the decision of informing parents about adolescent use of confidential services in the interest of best serving the patient; other states limit disclosure.

Refusal of Treatment A parent has the right to refuse treatment for their child as long as those decisions do not pose a serious threat to the child's well-being. ■ In nonemergent situations, the clinician in charge is responsible of engag­ ing with the parents and care partners by providing education and clarifi­ cation regarding the treatment. ■ When faced with continued resistance despite discussions with the par­ ents, if the parental decision is not in the best interest of the child, the clinician should seek a court order (eg, refusing immunizations is not considered a serious threat; therefore the parent has the right to refuse). ■ In an emergent situation, if a parental decision is not in the best interest of the child, a clinician may provide treatment against parental wishes. As such, if withholding treatment jeopardizes the child's safety, treatment can be initiated on the basis of legal precedent. In the case a child presents to the hospital with severe intra-abdominal hemorrhage and a blood transfu­ sion appears to be necessary and lifesaving, the clinician can override the request of parents not to transfuse in order to save the life of the patient. ■ Example involving Jehovah's Witnesses: A clinician provides a blood transfusion to save the life of a 6-year-old child seriously injured in a motor vehicle collision despite parental requests to withhold such a measure. ■

0-Tr KEY FACT Bra i n death is the i rreversible loss of a l l bra i n activity and is eq u iva lent to cardiopul monary death. If a patient is bra i n dead, no consent is needed to stop thera py. Two c l i n icians a re req u i red to perform a bra i n death exa m i nation to leg a l ly declare a patient bra i n dead.

END-OF-LI FE ISSUES ADVANCE DIRECTIVES An advance directive is a way for patients to let their doctors know what the patient's wishes are in case they lose capacity. A formal advance directive, such as a living will or healthcare proxy, will override the wishes of the family. Advance directives are legal documents that should be completed by any competent adult. ■ Living will: A written advance directive outlining the patient's wishes. It addresses a patient's wishes to maintain, withhold, or withdraw life­ sustaining treatment in the event of terminal disease or a persistent vege­ tative state when the patient has lost the capacity to make decisions. A living will can be a detailed document describing what tests and inter­ ventions the patient would refuse or consent to. If the living will is not detailed, decision making can be more difficult (eg, a living will stating no extraordinary care). This advance directive does not provide enough clarification and medical guidance for clinicians to follow. The defini­ tion of extraordinary care is subjective. It can mean no chemotherapy to some patients, but for others it could mean no invasive blood draws. ■ Physician orders for life-sustaining treatment: POLST is a clinician's order that outlines a plan for end-of-life care that reflects both the patient's preferences concerning care at life's end and the clinician's judgment, based on a medical evaluation. The POLST is not a replacement of an advance directive.

I A 5-yea r-o ld g i rl with hydroce pha l u s needs a nother revision o f h e r ventri c u l operitoneal s h u nt. Th ere a re no satisfa ctory a lte rnatives ava i la b l e t o re l i eve her sym ptoms. Her father conse nts, but her mother does not wa nt to proceed with the proce d u re, expla i n i n g that she has been t h ro u g h e n o u g h proced u res i n her you n g l ife. What is the most appropriate n ext ste p in management?

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In the absence of a living will or durable power of attorney for healthcareThe spouse CHIPS in For the patient

Ad ult CHIidren Parent Sibling Friend (in this order)

0-,,. KEY FACT Do not resuscitate (DN R) a n d do not i ntu bate (DNI) orders do not mean "do not treat'.'

HEALTH SYSTEMS SCIENCE SURROGATE DECISION MAKING When faced with an unconscious patient or a patient who cannot make a decision for themselves, surrogate decision making comes into play. The fol­ lowing scenarios are examples of such: ■

Durable power of attorney for healthcare or "healthcare proxy": Legally designates a surrogate healthcare decision maker if a patient lacks decision-making capacity. More flexible than a living will. Surrogates should make decisions consistent with the person's stated wishes regarding medical care only. The healthcare proxy makes decisions based on the patient's verbal and written communicated wishes. The proxy's decision outweighs the family wishes. No living will: If no living will or durable power of attorney for healthcare exists, decisions should be made by close family members (spouse, adult children, parents, and adult siblings) or friends, in that order. ■ When all are in agreement, decision making on an intervention is straightforward. ■ When family members disagree, the clinician should first encourage discussion. If no consensus is agreed upon, refer the case to the hospi­ tal's ethics committee. Seek a court referral as a last resort. Ethics committees or court orders can be helpful when the patient lacks capacity, when the patient has no proxy or advance directive and there is disagreement among family members, or when there is disagreement between the family and healthcare team (eg, in cases of medical futility or parental refusal of necessary treatment for minors).

Do not resuscitate (DNR)/do not intubate (DNI) orders: DNR and DNI orders are based on patient preferences regarding cardio­ pulmonary resuscitation (CPR) and intubation only. Patients can refuse all nonpalliative treatments or specific therapies (eg, CPR, intubation, antibiotics, feeding tubes). ■ A DNR/DNI order does not prevent people from getting other interven­ tions (eg, dialysis, chemotherapy, blood transfusions). ■

WITHDRAWAL OF LIFE -SUSTAINING TREATMENT Patients and their decision makers have the right to forego or withdraw life-sustaining treatment. Also, the clinician should never use the term withdrawal of care because healthcare staff do not stop caring for a patient. Clinicians should seek to understand patients and their reasons for refus­ ing beneficial treatments. When a patient has full capacity and decides to withdraw life-sustaining treatment, there is no need for a psychiatric evaluation. Psychiatric evalua­ tions are only needed if the patient's capacity to understand is uncertain. ■ No ethical distinction is made between withholding a treatment and with­ drawing a treatment, because a patient may choose to refuse an interven­ tion either before or after it is initiated. This can include ventilation, fluids, nutrition, and medications such as antibiotics. ■

Th e most a ppropriate next step i n ma nagement is t o proceed w i t h t h e s h u nt revision. The consent o f o n e pa rent is sufficient t o proceed with the treatment of a m i nor, pa rtic u l a rly when it is u n eq u ivoca l l y clear that the decision i s i n the c h i l d 's best i nterest.

EUTHANASIA AND CLINICIAN-ASSISTED SUICIDE ■

Euthanasia is the administration of a lethal agent by the healthcare pro­ vider with the intent to end life. ■ It is opposed by the American Medical Association (AMA) Code of Medical Ethics and is illegal in all states.

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■ Patients who request euthanasia should be evaluated for inadequate pain control and comorbid depression. ■ Clinician-assisted suicide consists of prescribing a lethal agent to a patient who will self-administer it to end his or her own life. This is currently ille­ gal except in the states of Oregon, Washington, Vermont, Colorado, and California. This is also legal via court order in Montana.

HOSPICE CARE Hospice care is a subtype of palliative care focused on palliation of symp­ toms for patients with a poor prognosis. The focus is on pain management, quality of life, and bereavement. ■ If the intent is to relieve suffering and if the medications administered are titrated for that purpose, it is considered ethical to provide palliative treat­ ment to relieve pain and suffering even if it may hasten a patient's death (principle of double effect).

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FUTILE TREATMENT Clinicians are not ethically obligated to provide treatment and may refuse a patient's or family member's request for further intervention on the grounds of futility under any of the following circumstances: ■ There is no evidence or pathophysiologic rationale for the treatment. ■ The intervention has already failed. ■ Maximal intervention is currently failing. ■ Treatment will not achieve the goals of care.

COMPLEMENTARY AND ALTERNATIVE MEDICINE TH ERAPY Complementary and alternative medicine (CAM) represents medical services and practices that are not part of standard medical care. As the name indi­ cates, this can include special diets, vitamins, dietary/herbal supplements, meditation, yoga, and hypnosis, among others. These alternative treatments help patients cope with their disease and side effects of their disease such as nausea, fatigue, pain, and loss of appetite. If a patient is interested in alterna­ tive/nontraditional treatment, the clinician should obtain more information as to why the patient is interested. The clinician should provide as much information as possible, and they should not dismiss the patient.

DISCLOSURE FULL DISCLOSURE ■

Disclosure is the act of making something known in its entirety. Medi­ cally, patients have a right to know about their medical status, prognosis, and treatment options (full disclosure). They have the legal right to obtain copies of their medical records within a specified timeframe. ■ Per the right to autonomy, a patient's family cannot require that a clini­ cian withhold information from the patient without the knowledge and consent of the patient. When such a case arises, the clinician should explore why the family member does not want the diagnosis revealed. Ulti­ mately, however, the patient should be told.

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A clinician may withhold information only if the patient requests not to be told or in the rare and controversial case in which a clinician determines that disclosure would cause severe and immediate harm to the patient (therapeutic privilege). This may include any information that may lead to depression, anxiety, or even resignation from a current job. Therapeutic privilege does not involve withholding medical information in emergency situations or reporting errors in patient care. Disclosure is to be done at the earliest time possible, and it is withheld only if there are contraindications to communicating this information to the patient (ie, if it might do more harm to the patient than good). If issues arise when information is being disclosed, this is handled by dis­ cussing with the patient's family as to why they don't want disclosure with­ out actually letting them know the details of the information being disclosed. Alternatively, this can be discussed with the ethics committee if there appears to be a conflict of interest (eg, debate between two siblings over what their parent would have wanted).

SETTING FOR DELIVERING NEWS News, good or bad, needs to be disclosed to the patient in a way that it is fully understood, without breach of privacy, and with empathy. A setup needs to be created whenever delivering news so that all these concerns, and more, can be addressed directly. The most commonly followed model is the SPIKES model. This includes the following: ■

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Setup: This requires the area of news delivery to be in a private area, ide­ ally with everyone sitting down for the discussion. It is also important to include people who the patient thinks should hear the news (with consent by the patient first). Perception of the patient: The next step entails asking the patient what they know so far about their illness. It is important to know what they think that the news might be: good or bad? Invitation from the patient: Also, it is important to ask them what informa­ tion they would like to hear and what they feel like they want to exclude. If the patient thinks they do not want to include certain pieces of informa­ tion in the discussion, they should be asked why. However, it is important to remember that as much as the patient has the right to know, the patient also has the right not to know.

After this, the clinician should initiate discussion of the news in stages. ■

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Knowledge: This stage is when all the information about the patient's cur­ rent condition is delivered. It is important to know the intellectual level of the patient to make sure that the patient understands what information is being communicated to them. Special circumstances may require detailed diagrams that are simplified for the patient. ■ Always give information bit by bit, and make sure that you consider the patient's current emotional state prior to giving information. ■ Always be gentle and caring. Examples include statements like: "I am sorry to let you know ... ", or "I think this piece of information may be particularly disturbing for you ... " Emotions: Prepare to identify any emotion that the patient shows, acknowledge it, and respond in an empathetic manner. Examples include statements such as "I know this might be difficult to hear..." or "I know this might not be what you had anticipated... "

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Strategy and summary: Let the patient know that the information you needed to deliver, and that the patient wanted to hear, has now been com­ municated. Ask the patient if they have any queries or if they have anything else that they might want to hear. If not, ask them about when they may be ready for the next meeting and briefly outline what will be discussed next. Examples include statements like: "Although this news might have come unexpectedly for you, we still need to discuss your care plan. While you pro­ cess this information, we also need to discuss how to proceed further with your care. If you want to discuss this further, then we can continue. Other­ wise, please let me know when you would like to discuss this next."

CONFIDENTIALITY Patient security, privacy, and healthcare data protection fall within the auspices of the Health Insurance Portability and Accountability Act (HIPM). This law addresses three main issues: privacy (for use and disclo­ sure of individuals' health information), security (to set national standards for protecting confidentiality, integrity, and availability of electronically protected healthcare-related information), and breach rules (to notify affected individuals, federal government, and media about unsecured pro­ tected health information). ■ Protected health information is any information that is transmitted or maintained in electronic media directly related to an individual's health­ care. This may also involve any verbal communications (eg, talk over cof­ fee or lunch) that discloses patients' identifiable health information (eg, name, patient number). This can also include bills, admission profile, pre­ scription records, referral, discharge, and follow-up appointments. ■ Information disclosed by a patient to their clinician and information about a patient's medical condition are strictly confidential and should be dis­ cussed and accessed only by those directly involved in the patient's care, with few exceptions (described later). This may require disclosure to a healthcare monitoring body like the Centers for Disease Control or Pre­ vention (CDC), or even to the police. ■ Who has access to medical records? This is typically reserved only for the patient or their authorized representatives. A patient may waive the obliga­ tion of the clinician to protect confidentiality (eg, with insurance compa­ nies, authorized family members), preferably by way of written or verbal consent. The clinician should disclose only the minimally necessary infor­ mation to these personnel. ■

It is ethically and legally necessary to override confidentiality in the following situations: ■

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Patient intent to commit a crime against an identifiable victim (Tarasoff decision): Clinicians have a duty to protect the intended victim through reasonable means (eg, warn the victim, notify police). Suicidal patients. Child abuse/neglect and elder abuse/neglect. Reportable infectious diseases (eg, HIV, sexually transmitted infections, TB, polio, diphtheria, rabies, enteric fever, tetanus, COVID-19): There is a duty to warn public officials and identifiable people at risk. It is normally best to encourage patients themselves to inform sexual contacts who are at risk for contracting the illness. Reportable noninfectious diseases: These include cancer, carbon monox­ ide poisoning, silicosis, and lead poisoning.

,0.0 MNEMONIC Overriding confidentiality­ WAITT a SEC before letting a patient in danger go!

WOU N DS Automobile-driving impairment I nfectious disease Tarasoff (violent crimes) and human Trafficking Suicide Elder a buse and neglect Child abuse

0-,,. KEY FACT

Signs of suspected child abuse: History given not consistent with injury Unusual child or parental behavior Delay in seeking medical care Subdural hematomas Retinal hemorrhages Spiral, bucket-handle, or rib fractures Injuries in different stages of healing

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Potential signs of elder abuse and neglect: Cuts, bruises, pressure ulcers, burns Uncommon fractures Malnutrition or dehydration Anogenital injury or infection Evidence of poor caretaking or financial exploitation

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Guiding principles for overriding confidentiality: There is an identifiable third party at risk for harm. The harm is significant and probable. Disclosure will help prevent or mitigate the harm. Other measures, such as convincing the patient to self-disclose, have failed.

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Notifiable outbreaks: These include food- and water-borne disease outbreaks. Currently patients affected by the COVID-19 pandemic are also reportable. Gunshot and knife wound (a duty to notify the police): Such instances also include emergency scenarios when the treating clinician is trying to obtain consent over the phone because the patient cannot provide one. The patient is a danger to others (eg, impaired automobile drivers): Cur­ rently, only six states have mandatory clinician reporting laws. ■ Example: A patient begins to drive 1 week after hospitalization for sei­ zures, although the department of motor vehicles in his state requires that licensed drivers be without seizures for at least 3 months.

CLINICAL RESEARCH The formal definition of clinical research is any medical research that tests new investigations, treatments, and therapies on people or against a standard of care. ■

The role of ethics in research is to ensure patient safety and integrity of research results. The Declaration of Geneva of the World Medical Associ­ ation (WMA) binds the clinician with the statement: "The health of my patient will be my first consideration," which implies that the patient should be protected from harmful treatment at all times. As such, patients may choose voluntarily to be research subjects, or they may be enrolled in a therapeutic trial for a disease without any known cure to see if they may benefit from the treatment. The Declaration of Helsinki addresses ethical concerns that arise from such studies. It also mentions that clinicians are obligated to inform patients considering involvement in a clinical research protocol about the purpose of the research study and the entire study design as it will affect the patient's treatment. This includes the possible risks, benefits, and alter­ natives to the research protocol. Clinicians are also obligated to inform the participants that no patient identification label (eg, name, patient num­ ber) will be used in the research.

CORE PRINCIPLES OF CLINICAL RESEARCH The principles followed include the following: ■ ■ ■

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Mandatory reporting of intimate partner violence is controversial and varies by state. Nonetheless, clinicians should document the encounter, offer support, and have resources available for assistance.

Ensuring compliance with clinical protocols, with repeated reviews con­ firming the same during the time of research Verification of scientific validity of results Ensuring a fair system of selecting treatment options, with removal of bias where possible Obtaining informed consent discussing benefit and harm prior to partici­ pation in research

ETHICAL CONCERNS Pregnant patients: Ensure no harm comes to both mother and child dur­ ing research. Children: It is important to obtain assent of the child, even though the consent from the guardians is mandatory. Incurable illness: The clinician should make sure the patient does not enroll only for anticipated personal benefit.

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Monetary compensation: Such compensation may provide undue influence for accepting the risk for contracting a disease or being harmed in the pro­ cess. Most trials provide only out-of-pocket expenses as compensation. ■ Recruitment: The patient may be enrolled in multiple trials due to lack of volunteers or due to rarity of disease. This may lead to bias in the results and to patient harm. ■ Stem cell research: Such research has an intrinsic difficulty with maintaining anonymity of human tissue donors, ownership of tissue, long-term storage of samples, and manipulation of genetic material to create new organisms. ■ Inability for patient to consent: In this case, the clinician should obtain consent from the legally authorized representative. ■ Incarcerated patients: Incarceration does not change the process of con­ sent or healthcare rights. ■

CONFLICT OF INTEREST Occurs when clinicians find themselves having a personal interest in a given situation, which influences their professional obligations. ■ Example: A clinician may own stock in a pharmaceutical company (financial interest) that produces a drug he is prescribing to his patient (patient care interest). ■ Clinicians should disclose existing conflicts of interest to affected parties (eg, patients, institutions, audiences of journal articles or scientific meetings). ■ Accepting gifts from pharmaceutical companies can influence a clini­ cian's practice and should generally be avoided. Nonmonetary gifts should be accepted only if they will directly benefit patient care and are of small monetary value. A clinician should never accept cash. ■

GIFTS FROM PATIENTS ■

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A patient or patient party may offer to provide the treating healthcare team or a particular member in the team with a gift as an expression of gratitude or as a reflection of the patient's culture. Accepting gifts or cash to influence care or preferential treatment is unac­ ceptable. This can further harm the patient-doctor relationship. When accepting a gift, keep the following in mind: ■ Do not allow the gift or offer to influence care. ■ Decline gifts if acceptance may present financial or emotional hard­ ship to the gifting family. ■ Be sensitive about the patient's emotions and be fair (accepting a gift may lead to impaired relationship among team members if the gift is provided disproportionately).

GIFTS FROM DRUG COMPANIES ■

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Governed by the Sunshine Act, which requires clinicians to disclose any financial information or conflict of interest required by employers, advi­ sory board, and institutions that provide research funding. Includes gifts that cost more than $10 or any small gifts that are worth cumulatively a sum of more than $100. Exemptions include certified and accredited continuing medical educa­ tion, buffet lunch and snacks at large-scale events, product samples not intended for sale, education material for patients, charitable items, or loaned devices for a trial period of less than 90 days.

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A 35-yea r-old wom a n visits a p ri m a ry care c l i nician after h u rting her wrist. P hysica l exa m reveals ci rcu mfe renti a l bruises o f her wrist, neck, a n d a rms. The pati ent a d m its that the i nj u ries we re i nfl icted by her partner. What is the most a ppropriate next step i n management?

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However, such gifts, or any advertisements, if received, should not alter the appropriate evidence-based practice of medicine on the clinician's part.

MALPRACTICE DEFINING MALPRACTICE

:(t MNEMONIC The 4 Ds of malpractice­

Duty Dereliction Damage Direct cause

Medical malpractice occurs when a hospital, doctor, or other healthcare pro­ fessional causes injury or permanent harm to a patient as a result of a negli­ gent act of carelessness. This may occur in two different ways: acts of omission (breach of duty to provide appropriate care to patients when capacity to con­ sent was not determined) or acts of commission (doing something purpose­ fully that leads to harm, despite being aware of possible outcomes without notifying the patient of such outcomes). The essential elements of a civil suit under negligence include: ■ ■ ■

The clinician has a Duty to the patient. Dereliction of duty occurs. There is Damage to the patient. Dereliction is the Direct cause of damage.

An exception involves the "Good Samaritan" laws, which protect those who have completed basic first aid training and are certified by a healthcare organi­ zation. These laws apply mostly to those people who may respond to victims in good faith and in a rational manner, with the aim of preventing harm. Unlike a criminal suit, in which the burden of proof is "beyond a reason­ able doubt," the burden of proof in a malpractice suit is "a preponderance of the evidence." Clinicians may refuse inappropriate requests, such as demanding to be seen after hours. The clinician should set clear limits and professional boundaries while remaining calm. If the patient has a nonurgent condition, the clinician should not recommend that the patient visit the emergency department.

IMPAIRED PRACTICING CLINICIAN

Th e most appropriate next step would be to offer s u p po rt and acknowledge the co u rage it ta kes to d iscuss a b u se. Assess the safety of the wo m a n a n d of a ny c h i l d ren i nvolved, i ntrod u ce the con cept of an emergency plan, and enco u rage the use of com m u n ity resou rces. I f the patient conse nts, report the abuse to releva nt a uthorities.

Impairment does not only refer to aging. Per the AMA, an impaired clinician is one whose physical or mental health interferes with their ability to safely engage in professional activities. This can also involve anxiety (a major stress factor that affects job performance and working memory), burnout (emotional exhaustion, depersonalization, reduced sense of personal accomplishment), and those clinicians with substance use disorder. A clinician has an obligation to protect patient interests and ensure appro­ priate care and assistance to other clinicians/colleagues who may be impaired physically or mentally. They should also report impaired colleagues to the peer review body of the hospital (eg, the program director or medical direc­ tor) or the local or state medical board when the physician does not have hos­ pital privileges. When compromise of a patient's health and safety is an immediate threat, report directly to the state licensing board. If the situation does not change despite prior reporting, report to a higher authority. ■

Example: A clinician notes that his colleague, a surgeon, gets repeatedly drunk at parties throughout the week and comes late to work the next day. A patient has recently filed a malpractice lawsuit, as the patient believes that the doctor operated on him in a drunken state. Your responsibility in this case is to refer the clinician to a clinician health program for further evaluation and possible therapy.

GASTROINTESTINAL Ora l a n d Sal iva ry Gland Disease ORAL LESIONS OROPHARYANGEAL CANCERS SALIVARY GLAND DISEASE

Esophagea l Disease DYSPHAGIA/ODYNOPHAGIA INFECTIOUS ESOPHAGITIS PILL (MEDICATION-INDUCED) ESOPHAGITIS EOSINOPHILIC ESOPHAGITIS ESOPHAGEAL RINGS PLUMMER-VINSON SYNDROME DISTAL ESOPHAGEAL SPASM ACHALASIA ESOPHAGEAL DIVERTICULA GASTROESOPHAGEAL REFLUX DISEASE HIATAL HERNIA ESOPHAGEAL CANCER

Ga stroi ntesti nal Bleed i n g UPPER GI BLEEDING

Disorders of the Stomach a n d Duoden u m DYSPEPSIA GASTRITIS GASTRIC CANCER PEPTIC ULCER DISEASE ZOLLINGER-ELLISON SYNDROME GASTROPARESIS M�NETRIER DISEASE GASTRIC BEZOAR BARIATRIC SURGERY

Disorders of the Sma l l Bowel DIARRHEA MALABSORPTION/MALDIGESTION CARBOHYDRATE MALDIGESTION

1 99

1 99 20 1 20 1

203

203 204 205 206 207 207 207 208 209 209 21 1 21 1

21 1

CARCINOID SYNDROME SMALL BOWEL OBSTRUCTION ILEUS ACUTE ABDOMEN DUODENAL HEMATOMA MESENTERIC ISCHEMIA ACUTE APPENDICITIS

Disorders of the Large Bowel CLOSTRIDIUM DIFFICILE COLITIS DIVERTICULAR DISEASE LARGE BOWEL OBSTRUCTION IRRITABLE BOWEL SYNDROME COLORECTAL CANCER COLORECTAL CANCER-ASSOCIATED CONDITIONS ISCHEMIC COLITIS MICROSCOPIC COLITIS

Anorecta l Disease HEMORRHOID GRADING

224 224

225 226

228

229 230

23 1

23 1 232 234 234 236 237 238 240

241

241

21 2

I nfl a m matory Bowel Disease

214

241

Hernias

243

B i l i a ry Disease

244

214 214 216 216 21 7 21 7 218 218 21 9

220

220 223 224

CHOLELITHIASIS AND BILIA RY COLIC CHOLECYSTITIS CHOLEDOCHOLITHIASIS CHOLANGITIS GALLSTONE ILEUS PosTCHOLECYSTECTOMY SYNDROME BILIARY CYST CHOLANGIOCARCINOMA

Liver Disease ABNORMAL LIVER ASSOCIATED ENZYMES HEPATITIS

244 244 246 246 246 247 247 247

248 248 249

1 97

GASTROINTESTINAL CIRRHOSIS SPONTANEOUS BACTERIAL PERITONITIS ISCHEMIC HEPATITIS ACUTE LIVER FAILURE HEPATORENAL SYNDROME HEPATOPULMONARY SYNDROME HEPATIC HYDROTHORAX TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT PROCEDURE PRIMARY SCLEROSING CHOLANGITIS PRIMARY BILIARY CHOLANGITIS NONALCOHOLIC FATTY LIVER DISEASE

1 98

252 254 256 256 257 257 258 258 259 259 259

HEPATOCELLULAR CARCINOMA HEMOCHROMATOSIS WILSON DISEASE (HEPATOLENTICULAR DEGENERATION) LIVER TRANSPLANTATION BENIGN LIVER LESIONS

Pancreatic Disease PANCREATITIS PANCREATIC CYSTS

PANCREATIC NONENDOCRINE TUMORS (PN ETs) PANCREATIC CANCER

259 260 261 261 262

264

264 264 266 267

GASTROINTESTINAL

H I G H -YI E L D FACTS I N

1 99

ORAL AND SALIVARY G LAND DISEASE ORAL LESIONS Table 2.6-1 describes common benign oral lesions. Figure 2.6-1 depicts pre­ malignant oral lesions. Although these lesions are noncancerous, they can transform into squamous cell carcinoma (SCC).

TA B L E 2 . 6 - 1 .

Selected Benign Oral Lesions

CO N DITION A N D ETIOLOGY

PRESENTATI O N

DIAGNOSIS AND TREATMENT

Recurrent aphthous stomatitis

Recurrent aphthous stomatitis i s the most

Ad min isteri ng topical corticosteroids and systemic

Ulcers are painful, shallow, and round/oval with a

Treati ng u nderlyi ng systemic conditions

common cause of ulcerations in the mouth central yellow exudate

Condition begins in childhood or adolescence, often resolving later in life

i m m u nomodu lators

Avoiding m ucosal trau ma, correcting vita m i n deficien­ cies, or excluding obvious dietary causes ca n help

Differential includes underlying causes of recur­

rent ulceration due to systemic conditions (eg,

inflammatory bowel disease [I BD), celiac disease,

systemic lupus erythematosus [SLE)) Torus palati nus

Torus palatinus is a benign and asym ptomatic

midline bony overgrowth of the hard palate

I maging and biopsy are requ i red for fast­

growi ng mass, atypical appearance, or if the

General ly, no treatment is req ui red; surgical excision

can be considered if the lesion is causing significant discomfort

overgrowth is not i n the midline

Prevalence is u p to 27% of the population

(Image 1 adapted with permission from Peterson DE, O'Shaugh nessy JA, Rugo HS, et al. Oral mucosa l i nj u ry caused by mammalian ta rget of rapa mycin i n h i bitors: Emerging perspectives on pathobiology and impact on clinical practice. Cancer Med. 201 6;5 [8) : 1 897-1 907. I mage 2 adapted with permission from Chao PJ, Yang HY, Huang WH, et al. Ora l tori in chronic hemodialysis patients. Bio-med Res Int. 201 5;20 1 5:897674.)

F I G U R E 2.6- 1 . Premalignant lesions of the mouth. (A) Leukoplakia. (B) E rythroplakia. (C) Oral submucosal fibrosis. (I mages A and B reproduced with permission from van der Waa l I . Oral leukoplakia, the ongoing d iscussion on definition and term ino logy. Med Oral Poto/ Oral Cir Bucal. 20 1 5;20[6]:e685-e692. Image C reproduced with permission from Sa rava nan K, Narayanan V. The use of buccal fat pad i n the treatment of oral sub mucous fibrosis: A newer method. /ntJ Dent. 201 2;20 1 2:935 1 35.)

200

H I G H -YI ELD FACTS I N

0-,,. KEV FACT

Oral hairy leukoplakia is a

manifestation of EBV seen in immunocompromised patients. It affects the lateral portion of the tongue and presents with painless plaques that have a feathery or hairy appearance and cannot be scraped off. There is no malignancy potential.

GASTROINTESTINAL Oral Leukoplakia Generally asymptomatic, premalignant lesions that occur in 1. 5 % to 4.3 % of the normal population. H istory/PE Patients present with white patches in the oral mucosa that cannot be scraped off (Fig. 2.6- lA). The reported rates of transformation to SCC vary widely, ranging from < 1 % to 36%. Risk Factors ■ Tobacco use, alcohol use, and human papilloma virus (HPV) infection. ■ Risk factors for transformation to SCC: Nonhomogeneous lesions, large lesions (>4 cm diameter), multiple anatomic sites, high-risk location (lateral tongue and floor of the mouth), and dysplasia on histologic examination. Diagnosis Definitive diagnosis is based on biopsy and histopathology. ■ Histopathology may show hyperkeratosis, atrophy, inflammation, hyperpla­ sia, or dysplasia. Occasionally there may be carcinoma in situ or invasive carcinoma.

■

0-,,. KEV FACT

Oral candidiasis is an opportunistic, intraoral infection that most commonly presents with confluent white patches and plaques in the oral mucosa. They are differentiated from leukoplakia because they can be scraped or wiped off, exposing an erythematous base (see the Candidiasis section in the Dermatology chapter).

Treatment ■ Options include surgical excision of smaller high-risk lesions. For larger lesions, risk factor modification (eg, cessation of smoking and alcohol use) and close clinical and histologic surveillance are indicated. ■ Other options include destruction (with laser ablation or cryosurgery) and medical treatment (eg, retinoids, vitamin A). ■ Lifelong follow-up is required due to high recurrence and development of squamous cell carcinoma. Erythroplakia Red, well-demarcated lesions commonly located on the floor of the mouth, the ventral tongue, or soft palate that cannot be explained by another disease (Fig. 2.6- l B). An estimated 2. 7% of cases annually transform to SCC. ■ ■ ■ ■

Hx/PE: Generally asymptomatic in patients Risk factors: Most often found in older adults who use tobacco and con­

sume alcohol Dx: Definitive diagnosis based on biopsy and histopathology, which may show dysplasia, carcinoma in situ, or even invasive SCC Tx: Similar to leukoplakia

Oral Submucosal Fi brosis Submucosal fibrosis of the whole oral mucosa (Fig. 2.6- l C). The estimated annual rate of transformation to SCC is 2% to 8%. ■

■

■ ■

Hx/PE: Burning, ulceration, and pain of the oral mucosa Risk factors: Chewing araca nuts (including betel quid, paan) Dx: Based on history and biopsy findings Tx: Discontinuing betel products - mainstay of treatment

Oral Lichen Planus Inflammatory lesions of the skin and oral mucosa with a prevalence of 1 % to 3 % (see the Miscellaneous Skin Disorders section in the Dermatology chapter for more details). Oral lesions appear as reticular white plaques, mucosa!

GASTROINTESTINAL

H I G H -YI E LD FACTS I N

201

erythema, or erosions most commonly affecting the buccal mucosa. The overall transformation rate to sec is 1 % to 2%.

■ Hx/PE: Reticular white plaques that are rarely symptomatic. Other lesions can present with pain. ■ Dx: Based on clinical evaluation and biopsy findings ■ Tx: No treatment for asymptomatic disease. Treatment of symptomatic dis­ ease is with topical corticosteroids (first line). If ineffective, topical tacroli­ mus or pimecrolimus can be used. Refractory disease may require oral glucocorticoids or systemic immunomodulators.

OROPHARVNGEAL CANCERS Oropharyngeal SCC is the most common oral cancer (up to 90% of all oral neoplasms). It most commonly arises from premalignant lesions such as leu­ koplakia, erythroplakia, or lichen planus. History/PE

Patients may be initially asymptomatic. Most commonly, oral cancer presents as an ulcerated lesion with a central necrotic area with rolled-up borders. The most common locations are the lateral and ventrolateral parts of the tongue, although other areas may also be affected. Risk Factors

Smoking or use of smokeless tobacco ■ Alcohol consumption ■ Areca (betel) nut ingestion (more prevalent in developing countries) ■ Sometimes associated with HPV-16 and chronic irritants (eg, certain mouthwashes) ■

Diagnosis

Diagnosis is based on biopsy and histopathology. Laryngoscopy, bronchoscopy, and esophagoscopy are required to rule out simultaneous second primary cancers. After diagnosis, head and neck CT and a x-ray of the chest (CXR) or positron emission tomography (PET)/CT are done to determine extent of spread and metastasis. Treatment

Treatment is initially surgical resection. Lymph node dissections are done with significant nodal disease or deep invasion. Surgical reconstruction is important to reduce postsurgical disability. Postoperative chemotherapy or radiotherapy is used with high-risk or advanced disease. In many patients for whom surgical resection is challenging or overly morbid (or in certain subtypes [eg, HPV-asso­ ciated cancers]), chemotherapy and radiation will be used as the definitive treatment with curative intent, with no surgery except in cases of relapse.

SALIVARY GLAND DISEASE The sublingual, submandibular, and parotid glands are the most prominent salivary glands. Clinically, salivary gland swelling may be approached by iden­ tification of chronicity (acute vs chronic) and distribution (unifocal [ single gland] vs multifocal [ multiple glands]) of swelling. Figure 2.6-2 shows an out­ line of salivary gland pathologies based on this approach. Tables 2.6-2 and 2.6-3 describe selected non-neoplastic and neoplastic conditions in detail.

t,.◊ MNEMONIC

The RULE acronym helps physicians decide which oral lesions to biopsy. Red/red-white lesions Ulcers Lumps Especially in combination or if indurated

202 TA B L E 2 . 6 - 2 .

CON DITION

H I G H -YI ELD FACTS I N

GASTROINTESTINAL

Selected Non-neoplastic Causes of Salivary Gland Swelling

ETIOLOGY

PRESENTATION

DIAGNOSIS

TREATMENT

Sialadenosis

Noninflammatory, noninfectious, and painless swelling of parotid glands Common causes: malnutrition, bulimia, diabetes, alcoholism, obesity, and liver diseases

History compatible with predisposing causes (eg, diabetes or alcoholism)

Compatible history Physical examination: no signs of infection (eg, pain, fever, suppurative discharge) Ultrasound ± contrast CT to rule out other causes of salivary gland swelling

Acute sup-

Bacterial infection of salivary glands that most commonly affects parotid Most commonly due to Staphylo-

Acute, unifocal salivary gland swelling and pain Purulent secretions from duct Associated with fever, tender lymphadenopathy When secondary to stone, pain and swelling occur before fever develops

Initial testing: ultrasound ± contrast CT to identify suppuration (diffuse vs abscess), stones, or strictures Pus exuded intraorally from gland is sent for culture and sensitivity

Hydration, sialagogues (eg, sour candy), warm compresses, nonsteroidal a nti-inflammatory drugs (NSAIDs) Initial empiric antibiotics (eg, amoxicillin clavulanate), which may change later, according to culture and sensitivity If an abscess is present, it needs surgical drainage

Most common cause: viral parotitis due to mumps virus; other viral causes include coxsackie and cytomegalovirus (CMV) Most common in children < 1 5 years of age Highly contagious by airborne droplets

Viral prodrome of fever, malaise, headache Acute, multifocal parotid gland swel ling and pain (ie, initial ly unilateral, progressing to bilateral) May be associated with aseptic meningitis, encephalitis, or pancreatitis

Real-time reverse transcriptasepolymerase chain reaction (RT-PCR) of samples (serum, buccal, or oral swabs) Viral culture Serology (positive lgM)

Observation and supportive care (hydration, pain control) Prevention: vaccination (measles, mumps, and rubella [MMR])

Sialolithiasis

Caused by stone in salivary duct Most often affects submandibular gland (up to 90%), followed by parotid Most common cause of acute, unifocal salivary gland swelling Risk factors: smoking, hypovolemia, anticholinergics

Acute, unifocal salivary gland swel ling and pain that are intermittent and occur after meals (postprandial pain)

Ultrasound Noncontrast CT

I nitial: sialagogues (eg, sour candy), warm compress, hydration, massage of gland, and NSAIDS Antibiotics for infection If conservative treatment is ineffective, minimally invasive (eg, sialoendoscopy) or surgical (eg, sialoadenectomy) treatment may be indicated Prevention: risk factor modification (ie, stop smoking and using anticholinergics; avoid dehydration)

Ranula

Pseudocysts ofthe major salivary glands (sublingual or submandibular ducts) May be congenital or acquired (due to oral trauma)

Transl ucent blue swel ling in floor of mouth lateral to midline

Mucocele

Pseudocysts of minor salivary glands

Smooth swellings in the buccal mucosa on ocelusive plane; not blue in appearance

Diagnosis based on clinical appearance I maging (ultrasound sonography [USG), CT, MRI) can help to assess cause and extent and determine surgical approach

Generally, resolve spontaneously Persistent, recurrent, or symptomatic lesions may be treated by surgical excision, marsupialization, or other techniques (eg, laser ablation, cryotherapy, electrocautery) Aspiration is not effective due to high recurrence rates

pu rative

sialadenitis

coccus aureus

May be primary or secondary to obstruction (stone or stricture) Risk factors: dehydrated states (hospitalized/postoperative patients), chronic conditions (hypothyroidism, renal failure), drugs (anticholinergics) Acute non-

suppurative sialaden itis

Management of underlying condition

H I G H -YI E LD FACTS I N

GASTROINTESTINAL TA B L E 2 . 6 - 3 .

203

Selected Benign and Malignant Tumors of Salivary Glands

CO N DITION

ETIOLOGY

PRESENTATI O N

DIAGNOSIS

TREATMENT

Pleomorphic ade­ nomas (benign)

Most common salivary gland tumor in adults Majority in parotid gland, fol­ lowed by submandibular May undergo malignant trans­ formation and recurrence Associated with ionizing radiation exposure, viral infection, smoking, and exposure to chemicals (eg, rubber manufacturers, cosmetics)

Chronic, unifocal, painless, slow-growing mass or swelling in malignant and benign disease Signs of malignancy: pain, facial paresis, fixed mass, and cervical lymphadenopathy

Ultrasonography, CT, MRl, and sometimes PET-CT Fine-needle aspi­ ration biopsy or ultrasound­ guided core needle biopsy can confirm diagnosis

Surgical excision-mainstay of treatment no matter if benign or malignant Postoperative radiation therapy in select cases Cervical lymph node dissection may be required in malignant disease or with presence of other high-risk features (eg, high-grade tumors, facial nerve weakness) Important risks of surgery: facial nerve dysfunction and Frey syn­ drome (sweating when chewing)

Mucoepidermoid carcinoma (malignant)

Most common malignant salivary gland neoplasm Commonly in parotid Low-grade with good prog­ nosis if treated early

Painless, slow-growing mass that is firm or hard

Based on histologic examination

Surgical excision recommended for localized resectable disease Postoperative or palliative radio­ therapy may be used

Adenoid cystic carcinoma (ACC; malignant)

Second most common malignant salivary gland neoplasm Most common in sub­ mandibular gland Invades facial nerve early, thus facial weakness or paralysis Local recurrence after excision

Most commonly arises in the salivary glands or other areas within the head and neck region; symptoms of ACC of the salivary glands may include numbness of the lower lip and/or other facial areas, nerve impairment causing weakness of certain facial muscles, ongoing pain, and/or other associated abnormalities

Based on histologic examination

Standard therapy includes surgical removal of the malignancy and affected tissue followed by radia­ tion; if initial surgery is not an option due to the specific loca­ tion and/or progression of the malignancy, therapy may include radiation alone

[Salivary gland swelling]

Unifocal

Bacterial sialaden itis Sialolith iasis

F I G U R E 2.6-2.

Acute

Multifocal

Mumps Drug side effects I nflam matory Juve n i le recu rrent parotitis

Chro n ic/progressive

Unifocal

Benign tumors Malignant tumors

Multifocal

Autoi m m u n e Gra n u lomatous H I V-associated Metabolic sialosis

Causes of salivary gland swelling. (Reproduced with perm ission from USM LE-Rx.com.)

ESOPHAGEAL DISEASE DVSPHAGIA/ODYNOPHAGIA Difficulty swallowing (dysphagia) or pain with swallowing (odynophagia) caused by abnormalities of the oropharynx or esophagus. Figure 2.6-3 illus­ trates approaches to the diagnosis of esophageal dysphagia.

0-,,. KEY FACT I n a n i m m u nocom promised person with odynophagia, ca ndidiasis should be one of the d ifferential diagnoses.

204

H I G H -YI ELD FACTS I N

GASTROINTESTINAL Dysphagia d escription

Food ·stuck· while swallowing

Esophageal dysphagia

Difficulty initiating swallowing

Oropharyngeal dysphagia

!

Solid food

Mechanical obstruction

Trigger of dysphagia

Progressive

Esophageal webs/rings/stricture Eosi nophilic eso p hagitis

Neuromuscular etiology

!

Dysphagia timing

Intermittent

Solid food or liquids

Peptic stricture Esophageal mass

Dysphagia timing

Intermittent

Diffuse esophageal spasm

Progressive

Achalasia Scleroderma

F I G U R E 2.6-3. Approach to differential diagnosis of esophageal dysphagia. (Reproduced with perm ission from USMLE-Rx.com.)

0-n

KEY FACT

Patients who are immunocompromised (eg, H IV) with odynophagia or dysphagia may be empirical ly treated for candida esophagitis. EGO can be considered if there is no response to diagnose other causes (eg, CMV or HSV esophagitis).

Oropharyngea l Dysphagia

Problem with initiation of swallowing that may lead to aspiration of food into the lungs or regurgitation of food. Etiology: Neurologic or muscular, including stroke, Parkinson disease, myasthenia gravis, prolonged intubation, and Zenker diverticula. ■ Usually more of a problem with liquids than with solids. ■ History/PE: Rule out alternative syndromes such as xerostomia, globus, and esophageal dysphagia through history and physical examination. Investigations: Consider specific laboratory tests to rule out myopathies, myasthenia, or other conditions. Consider brain imaging if central ner­ vous system (CNS) tumor, stroke, or head trauma is suspected. ■ Best initial test: Modified barium swallow (video fluoroscopic swallowing exam) ± esophageal manometry. Esophagogastroduodenoscopy (EGD) may also be appropriate to rule out structural disease (see Fig. 2.6-3). Treatment: Diet modification, swallowing therapy, and sometimes tempo­ rary nonoral feeds. Severe dysphagia may require nonoral feeding (eg, per­ cutaneous endoscopic gastrostomy) or tracheostomy. Structural causes must be treated specifically (eg, cricopharyngectomy and diverticulectomy for Zenker diverticula). ■

Esophagea l Dysphagia

0-n

KEY FACT

Esophageal webs are associated with iron-deficiency anemia and glossitis (Plummer-Vinson syndrome).

Generally, patients complain that food gets "stuck" in the throat; however, there is no issue initiating swallowing. Can be caused by an obstruction (eg, strictures, Schatzki rings, webs, carci­ noma) or motility disorder (eg, achalasia, scleroderma, esophageal spasm). ■ Obstructions usually more of a problem with solids than with liquids; motility disorders cause both solid and liquid food dysphagia. ■ Best initial test: EGD; consider pre-EGD barium swallow (aka esophagram) if history of esophageal radiation, caustic ingestion, esophageal or laryngeal cancer surgery, or strictures, as these patients may be at higher risk for esoph­ ageal perforation; this may be followed by manometry in some cases.

INFECTIOUS ESOPHAGITIS Inflammation of the esophageal lining. Seen in immunocompromised patients. Table 2.6-4 outlines the etiology, diagnosis, and treatment of infec­ tious esophagitis.

GASTROINTESTINAL TA a L E 2 . 6 - 4 .

H I G H -YI E L D FACTS I N

205

Causes of Infectious Esophagitis

ETIOLOGIC AG ENT

EXAM F I N D I NGS

U PPER EN DOSCOPY

TREATMENT

Candida albicans

± ora l thrush

Yel low-wh ite plaq ues adherent to the mucosa;

Fluconazole PO (treat with

biopsy shows yeasts and hyphae invading mucosa l cells

Herpes sim plex virus

Oral u lcers

more than a topica l agent

alone)

Small, deep ulcerations with "volcano-li ke" a ppear­

a n ce; m u ltinucleated giant cells with i ntra n uclear

Acyclovir IV or PO

incl usions on biopsy + Tzanck smear

Cytomegalovirus

Retinitis, colitis

La rge, linear, su perficial u lcerations; i ntra n uclear and i ntracytoplasmic incl usions on biopsy

Ganciclovir IV

I mage reprod uced with permission from Kantarjian HM, et al. MD Anderson Manual of Medical Oncology. New York: McGraw-Hill; 2006.

History Commonly presents with odynophagia and/or dysphagia.

PILL (MEDICATION- INDUCED) ESOPHAGITIS Caused by ingestion of medications that have a direct toxic effect on the mucosa of the esophagus through creation of a localized acidotic or alkaline environment by the inciting medication.

Risk Factors ■ Medication known to be associated with pill esophagitis (see related key fact) ■ Taking medications without water or immediately before lying down ■ Anything that might increase dwell time of medications in the esophagus (eg, geriatric-related low saliva production, altered esophageal anatomy, drinking insufficient water with medication, and motility disorders of the esophagus)

0-,,. KEV FACT

Candida esophagitis is an AI DS-defining il lness, typically when CD4+ cel l count < l 00 cel ls/µL.

0-,,. KEV FACT

High-risk factors for Candida esophagitis include broad-spectrum antibiotics, corticosteroids, treatment in an intensive care unit (ICU), cancer, diabetes, organ transplant, mechanical ventilation, and indwel ling catheter.

History/PE ■ Dysphagia/odynophagia ■ Retrosternal burning ■ Taking an offending medication Diagnosis ■ Clinical diagnosis for patients with symptoms who took a medication known to cause pill esophagitis ■ Upper endoscopic evaluation with biopsy for patients with severe symp­ toms (hematemesis, abdominal pain, weight loss) or symptoms that don't resolve after discontinuing the medication for at least 1 week ■ Endoscopic evaluation usually reveals a localized ulceration of the esoph­ agus with surrounding normal mucosa (see Fig. 2.6-4)

F I G U R E 2.6-4. An ulcer in the esophagus caused by nonsteroidal anti-inflamma­ tory drugs (NSAIDs). (Adapted with perm is­ sion from H u S·W, Chen A·C, Wu S-F. Drug-induced esophageal u lcer in adolescent population: Experi­ ence at a single medical center in centra l Ta iwan. Medicina. 202 1 ;57[1 2] : 1 286. https://doi.org/1 0.3390/ medicina571 2 1 286.)

206

H I G H -YI ELD FACTS I N

GASTROI NTESTI NAL

Treatment ■ Discontinuing inciting medication (symptoms usually resolve in 7 to 10 days after discontinuation) ■ If medication cannot be discontinued, switching to liquid formulation

EOSINOPHILIC ESOPHAGITIS Immune-mediated disorder of the esophagus in which eosinophils are recruited to the esophagus causing dysphagia or food impaction.

History/PE Past medical history of seasonal allergies and asthma ■ Presents with dysphagia, food impaction, centrally located chest pain not relieved by proton pump prohibitors (PPis) ■

Diagnosis Diagnosis is based on clinical presentation and evaluation with EGO and esophageal biopsy ■ EGO most commonly shows ringlike structures (44%) or thickened linear furrows (48%) (see Fig. 2.6-5) ■ Biopsy is the most accurate test and will reveal esophageal inflammation with an eosinophilic-predominant infiltrate ( 2:: 15 eosinophils per high­ powered field on light microscopy) ■

0-,,. KEY FACT

Common causes of pil l esophagitis include antibiotics (especial ly tetracyclines), nonsteroidal anti-inflammatory drugs (NSAI Ds), bisphosphonates, ascorbic acid, potassium chloride, ferrous sul­ fate, acetaminophen, warfarin, and chemotherapy regimens.

Treatment ■ Best initial treatment: PPis and elimination of possible causes (ie, six­ food elimination diet involving exclusion of wheat, milk, egg, nuts, soy, and fish/shellfish). ■ If initial treatment fails, swallowed inhalational steroids or swallowed topi­ cal steroids are recommended for further management. ■ Esophageal dilation may be required in refractory disease.

rn

FIGURE 2.6-5. Concentric rings. Mucosal irregularity (A: black arrow) and furrows (B: black arrowhead) seen in esophagus with eosinophilic esophagitis on fluoroscopic evaluation (A) and endoscopic evaluation

(B).

(Images modified with permission from Al-Hussa i n i A, AboZeid A, Hai A. How does esophagus look on barium esopha­

gram i n pediatric eosi nophilic esophagitis? Abdom Radiol (NY). 201 6;4 1 [8] : 1 466- 1 473.)

GASTROINTESTINAL

H I G H -YI E L D FACTS I N

207

ESOPHAGEAL RINGS Concentric "ring" of tissue protruding into the esophageal lumen. Most com­ monly seen in the distal esophagus. Often associated with another condition (eg, eosinophilic esophagitis and hiatal hernia). Schatzki rings are the most common type of esophageal ring. In Figure 2.6-5, rings are shown on fluoro­ scopic evaluation (A) and endoscopic evaluation (B). History/PE

Patients with esophageal rings are usually asymptomatic, but they may present with dysphagia to solids. With more chewing, symptoms tend to be less severe. Increased severity of symptoms is associated with internal diameter of esophageal lumen associated with ring. Diagnosis

■ ■

Barium swallow: Thin, symmetric, circumferential narrowing EGO and biopsy: Thin, smooth, circumferential membrane; biopsy used to evaluate for esophagitis

Treatment

Goal of therapy is to relieve symptoms if present and prevent recurrent symptoms. ■ Initial therapy: Esophageal dilation followed by 6 weeks of PPI therapy ■ Recurrent and refractory symptoms: Repetition of EGO to confirm absence of eosinophilic esophagitis (can be patchy) and repetition of esophageal dilation + addition of long-term PPL

PLUMMER- VINSON SYNDROME History/PE

Plummer-Vinson syndrome presents with a classic triad of dysphagia, iron-defi­ ciency anemia (fatigue and weakness), and esophageal webs. Additional find­ ings may include glossitis, angular cheilitis, koilonychia, splenomegaly, and thyromegaly. Figure 2.6-6 shows a radiographic view of an esophageal web. Diagnosis

Laboratory testing reveals iron-deficiency anemia, and an esophageal web appears on esophagram, videofluoroscopic evaluation, or upper endoscopy. Treatment

■ Iron repletion: Rapid resolution of dysphagia in some patients ■ Esophageal dilation: May be necessary in those with significant esopha­ geal lumen obstruction ■ Annual upper endoscopy + biopsy: Recommended by some to watch for development of SCC, although no change in outcomes has been observed in the literature with this screening

DISTAL ESOPHAGEAL SPASM Motility disorder in which normal peristalsis is periodically interrupted by high-amplitude, nonperistaltic contractions (see Fig. 2.6-7A).

F I G U R E 2.6-6. Radiographic evaluation revealing an esophageal web. (Reproduced with permission from Ohtaka M, Kobayashi S, Yoshida T, et al. Use of Sato's cu rved laryngoscope and an insu lated-tip knife for endoscopic incisional therapy of esophageal web. Dig Endosc. 201 S May;27(4):522-S26. doi: 1 0. 1 1 1 1 /den.1 2334.)

0-,r KEY FACT

Steakhouse syndrome refers to food impaction of the esophagus after eating a piece of food without sufficient chewing. This most often involves a meat bolus. Steakhouse syndrome must be differentiated from other causes of dysphagia.

0-,r KEY FACT

Esophageal webs are like esophageal rings, but they are noncircumferential and are associated with different conditions (eg, Plummer-Vinson syndrome, which occurs in patients with long-term iron-deficiency anemia). Esophageal webs are often ruptured during EGO and do not cause recurrent or refractory symptoms.

() MNEMONIC Plumbers DIE from Plummer-Vinson syndrome:

Dysphagia I ron-deficiency anemia Esophageal web

208

H I G H -YI E L D FACTS I N

GASTROINTESTINAL

F I G U R E 2 . 6 - 7 . Esophageal disease on bar­ ium esophagram. (A) Esophageal spasm.

(B) Achalasia. Note the dilated esophagus tapering to a "bird's beak" narrowing (arrows) at the LES. (Image A reproduced with permission from

USMLE-Rx.com. Image B reproduced with permission from Doherty GM. Current Diagnosis & Treatment Surgery. 1 3th ed. New York, NY: McGraw-Hill; 201 0.

0-rr

KEY FACT

The musculature of the upper one-third of the esophagus is skeletal, whereas that of the lower two-thirds is smooth muscle.

H i story/PE

Presents with heartburn, chest pain, dysphagia, and odynophagia. Often pre­ cipitated by ingestion of hot or cold liquids; relieved by nitroglycerin. Diagnosis ■

■

EGD with biopsy: Best initial test to rule out other esophageal disorders. Barium swallow: Shows a corkscrew-shaped esophagus. Done as initial test prior to EGD in selected patients at risk of perforation (proximal esophageal lesion [ eg, Zenker diverticulum, radiation therapy], known complex strictures [ due to radiation exposure or caustic injury] ). Most accurate test: Esophageal manometry, which allows for definitive diagnosis. High-amplitude, simultaneous contractions shown in greater than 20% of swallows.

Treatment

";:.:'.::I

1 00 : : :

Wet swallow

■

.

.. :

:

■

Symptomatic relief: Calcium channel blockers, tricyclic antidepressants (TCAs), or nitrates Severe, incapacitating symptoms: Growing evidence supporting peroral endoscopic myotomy (POEM)

ACHALASIA Motility disorder of the esophagus characterized by impaired relaxation of the lower esophageal sphincter (LES) and loss of peristalsis in the distal two-thirds of the esophagus. Etiology

Degeneration of the inhibitory neurons in the myenteric (Auerbach) plexus. H i story/PE

Progressive dysphagia (solids and liquids), chest pain, regurgitation of undi­ gested food, weight loss, and nocturnal cough. Diagnosis ■ F I G U R E 2.6-8.

1 5 sec

Achalasia. Manometry

■

with incomplete LES relaxation.

(Repro­ duced with permission from Farrokhi F, Vaezi MF. Id iopathic (primary) achalasia. Orphanet J Rare Dis. 2007;2:38.)

■

Best initial test: EGD to rule out structural disorders, including mechani­ cal obstruction, pseudoachalasia, cancer. Most accurate test: High-resolution manometry, which shows increase in resting LES pressure, incomplete LES relaxation upon swallowing, and decrease in peristalsis in the body of the esophagus (see Fig. 2.6-8). Barium swallow is useful when manometric findings are equivocal. Will show esophageal dilation with "bird's beak" tapering of the distal esopha­ gus (see Fig. 2.6-7B).

GASTROINTESTINAL

H I G H -YI E L D FACTS I N

Treatment ■ Definitive: First-line treatment options are laparoscopic Heller myotomy, POEM, and less often, pneumatic dilation. ■ If first-line options are unsuitable, the physician may administer injection of botulinum toxin. ■ Calcium channel blockers and phosphodiesterase inhibitors or nitrates may provide short-term relief. They are only used in patients who are not candi­ dates for definitive therapy and have failed botulinum toxin injection.

209

Thyropharyngeus muscle ·-=-- Killian triangle

..,✓7---\--Cricopharyngeus muscle Zenker diverticulum

ESOPHAGEAL DIVERTICULA Diverticula can be present in any location in the esophagus or pharynx. Usu­ ally due to distal stricture/stenosis leading to increased pressure in the proxi­ mal esophagus. Zenker diverticulum is a cervical outpouching through the cricopharyngeus muscle. It is a posterior, false diverticulum (outpouching only through submucosa and mucosa). See Figure 2.6-9. ■ Hx/PE: Chest pain, dysphagia, halitosis, and regurgitation of undigested food. ■ Dx: Barium swallow to demonstrate outpouchings. ■ Tx: If symptomatic, surgical excision of the diverticulum. For Zenker diverticulum, myotomy of the cricopharyngeus required to relieve the high-pressure zone.

GASTROESOPHAGEAL REFLUX DISEASE Gastroesophageal reflux disease (GERO) is symptomatic reflux of gastric con­ tents into the esophagus, most commonly from transient LES relaxation. Incompetent LES, gastroparesis, or hiatal hernia can all contribute to GERO, but they are not the sole causes. Figure 2.6-10 depicts the general manage­ ment approach to GERO-like symptoms. GERO-like symptoms

F I G U R E 2.6-9. Illustration of a Zenker diverticulum (A) and fluoroscopic eval­ uation revealing a Zenker diverticulum (B). (Image A reproduced with permission from USMLE-Rx.com. Image B reproduced with permis· sion from Dion igi G, Sessa F, Rovera F, et al. Ten yea r surviva l after excision of sq uamous cell cancer in Zenker's d iverticu lum: report of a case. World J Surg Oneal. 2006;4: 1 7. doi:1 0.1 1 86/1 477-78 1 9-4- 1 7.)

Alarm symptoms for malignancy: weight loss, dysphagia, melena, hematemesis and/or anemia Yes

No

Perform endoscopy

Trial proton p u m p i n h i b itor f o r 8 weeks Assess for clin ical response Yes

Continue proton pump i n h i b itor

No

Increase proton pump inhibitor to twice daily or change to a d ifferent proton p u m p inhibitor Clinical response Yes

Continue current proton pump i n h ibitor

No

P erform endoscopy or pH manometry

F I G U R E 2.6- 1 0 . Management of GERO-like symptoms. (Reproduced with permission from USMLE­ Rx.com.)

H I G H -YI E L D FACTS I N

21 0

GASTROINTESTINAL

H istory/PE ■ Heartburn and/or regurgitation that commonly occurs 30 to 90 minutes after a meal; worsens with reclining; and often improves with antacids, sit­ ting, or standing. Uncommon symptoms: sour taste, a globus sensation, unexplained cough, morning hoarseness, and chest pain mimicking coro­ nary artery disease. ■ Normal unless a systemic disease (eg, scleroderma) is present.

F I G U R E 2.6-1 1 .

B a r rett e s o p h a g u s o n u p p e r e n d oscopy. Shown is proximal

extension of Z-line (squamocolumnar junction between esophagus and stom­ ach) caused by columnar metaplasia. The squamocolumnar junction must extend at least l cm above the gastro­ esophageal junction to diagnose Barrett's esophagus. (Reproduced with perm ission from Fauci AS et al. Harrison's Principles of Internal Medicine, 1 7th ed. New York, NY: McGraw-Hill; 2008.)

0--n

KEY FACT

GERD can mimic angina or myocardial infarction.

0--n

KEY FACT

GERD is not a result of the presence of Helicobacter pylori. GERD arises from a transient relaxation of the LES.

Diagnosis ■ Primarily a clinical diagnosis, with empirical treatment first in patients without alarm symptoms (see later for lifestyle modification and medical treatment). ■ Most accurate test: 24-hour pH monitoring with impedance; indicated if the diagnosis is uncertain. ■ EGO with biopsy: Performed in patients whose symptoms are the following: ■ Refractory to initial empiric therapy ■ Long-standing (to rule out Barrett esophagus and adenocarcinoma; see Fig. 2.6-11) Associated with alarm symptoms like hematemesis, weight loss, dysphagia/ odynophagia, or chest pain. ECG (or cardiac referral) should be done first. ■ Other studies: Indicated for refractory symptoms or if concern for other causes. May include esophageal manometry. Although barium swallow may demonstrate reflux, its role is limited due to a high prevalence of physiologic reflux. ■

Treatment ■ Lifestyle modifications: Indicated for all patients, these modifications include weight loss; head-of-bed elevation; small meals; avoidance of nocturnal meals; avoidance of substances like alcohol, chocolate, or coffee that ,J, LES tone. ■ An initial, empirical trial of pharmacotherapy (eg, 8 weeks of PPI) is indi­ cated in patients with clinical features of GERO in the absence of alarm symptoms. ■ In the presence of alarm symptoms (weight loss, dysphagia, and gastrointesti­ nal [GI] bleeding) and in patients with multiple risk factors for Barrett esophagus, endoscopy should be performed initially (ie, before trial of PPI). ■ Pharmacologic treatment: ■ Mild/intermittent: Antacids ■ Chronic/frequent: Hrreceptor antagonists (cimetidine, famotidine) or PPis (omeprazole, lansoprazole, pantoprazole, rabeprazole, esomepra­ zole, dexlansoprazole). Fundoplication surgery or other antireflux pro­ cedures may benefit carefully selected patients. ■ Severe/erosive: PPis first; fundoplication surgery or other antireflux procedures may benefit carefully selected patients. Complications: Erosive esophagitis, esophageal peptic stricture, aspiration pneumonia, upper GI bleeding, Barrett esophagus, adenocarcinoma. ■ Management of Barrett esophagus: Optimization of medical therapy (should be on chronic PPI therapy). Based on biopsy findings, manage­ ment calls for the following steps: ■ No dysplasia: Repeat endoscopy every 3 to 5 years ■ Indefinite dysplasia: Repeat endoscopy in 2 to 6 months ■ Oysplasia or intramucosal carcinoma: Treat with endoscopic eradication ■ Esophageal adenocarcinoma: See Esophageal Cancer section of this chapter for treatment. ■

GASTROINTESTINAL

H I G H -Y I E L D FACTS I N

21 1

HIATAL HERNIA Herniation of stomach upward into the chest through the diaphragm. Com­ mon types: ■ ■

■ ■ ■

Sliding hiatal hernia (95 %) : Gastroesophageal junction and a portion of the proximal stomach are displaced above the diaphragm (see Fig. 2.6-12). Paraesophageal hiatal hernia ( 5 % ): Gastroesophageal junction remains below the diaphragm while the fundus herniates into the thorax (see Fig. 2.6-12). Hx/PE: Most are asymptomatic. Patients with sliding hernias may present with GERO; paraesophageal hernias can cause strangulation. Dx: Incidental finding is apparent on CXR; it is also frequently diagnosed by barium swallow or EGO. Tx: ■ Sliding hernias: Medical therapy and lifestyle modifications to J, GERO symptoms ■ Paraesophageal hernias: Surgical gastropexy to prevent gastric volvu­ lus in some cases

ESOPHAGEAL CANCER SCC is the most common type of esophageal cancer worldwide. Adenocarci­ noma is the most common type of esophageal cancer in the United States, Europe, and Australia.

Risk Factors ■ SCC : Alcohol use, tobacco use, and nitrosamines ■ Adenocarcinoma: Barrett esophagus (intestinal metaplasia of the distal esophagus secondary to chronic GERO) History/PE Progressive dysphagia - initially to solids and later to liquids - is common. Weight loss, odynophagia, GERO, GI bleeding, and vomiting are also seen. Diagnosis Best initial and most accurate test: EGO + biopsy; required to establish diagnosis ■ CT and endoscopic ultrasound (EUS) for tumor staging Treatment ■ Best initial treatment: Chemoradiation and surgical resection. If there is metastatic disease that is considered incurable, systemic therapy alone 1s used. ■ Resection is also indicated in cases of high-grade Barrett dysplasia ■ Has a poor prognosis

GASTROINTESTINAL BLEEDING Overt GI bleeding presents as hematemesis, hematochezia, and/or melena. Bleeding may be from the upper GI tract (ie, bleeding from lesions proximal to the ligament of Treitz, the anatomic boundary between the duodenum and jejunum) or from the lower GI tract. Table 2.6-5 presents the features of upper and lower GI bleeding, and this chapter discusses selected conditions.

Herniated gastric fundus

F I G U R E 2.6- 1 2. (A) Sliding hiatal and (B) paraesophageal hiatal hernia. (Reproduced

with permission from USMLE-Rx.com.)

0-,,. KEY FACT

sec of the esophagus tends to occur in the upper and middle thirds of the esophagus, whereas adenocarcinoma occurs in the lower third.

0-,,. KEY FACT No radiologic test can diagnose esophageal cancer. Diagnosis must be made with a tissue biopsy.

0-,,. KEY FACT Esophageal cancer metastasizes early, because the esophagus lacks a serosa.

0-,,. KEY FACT Resection is required for esophageal cancer treatment to be curative.

0-,,. KEY FACT One unit of packed RBCs should i hemog lobin by 1 g/dl and hematocrit by 3 to 4 units.

21 2 TABLE 2.6-5.

H I G H -YI E L D FACTS I N

GASTROINTESTINAL

Featu res of Upper and Lower G I Bleeding

VARIABLE

UPPER GI BLEED I N G

LOW ER GI BLEEDING

History/exam

Hematemesis ("coffee-ground" emesis), melena >

Hematochezia > melena, b u t c a n b e either

hematochezia, hypovolemia (eg, elevated blood

u rea n itrogen [BUN), tachycardia, lig htheaded ness, hypotension) Diagnosis

Nasogastric (NG) tu be and lavage (may be 8 i n 1 5% of u pper GI bleeds); endoscopy is defi nitive

Rule out u pper GI hemorrhage with NG lavage if brisk

Anoscopy/sigmoidoscopy for patients PO vancomycin Recurrent (first episode): PO fidaxomicin is preferred over PO vancomycin Recurrent (second or sub­ sequent recurrence): PO fidaxomicin > PO vancomycin Fulminant without ileus: PO vancomycin + parenteral metronidazole Fulminant with ileus: PO or rectal vancomycin + par­ enteral metronidazole Surgical resection and albendazole

by compression of other structures (continues)

222

H I G H -YI ELD FACTS I N

TA B L E 2 . 6 - 8 .

I N FECTIOUS AGENT

GASTROINTESTINAL

Causes of Infectious Diarrhea (continued)

H I STO RY

EXAM A N D TEST RESU LTS ( N OTE: ALL HAVE F ECAL RBCs A N D WBCs)

COMM ENTS

TREATMENT

Entamoeba histolytica

Caused by ingestion of contaminated food or water May relate to possible patient history of traveling in developing countries Incubation period lasting up to 3 months

Presents with severe abdominal pain and fever Endoscopy shows "flask-shaped" ulcers

Chronic amebic colitis mimics lBD

Steroids can lead to fatal perforation Treat with metronidazole

Escherichia coli

Caused by ingestion of contaminated food

Presents with severe abdominal pain, low-grade fever, and

It is important to rule out GI bleeding and ischemic

Antibiotic or antidiarrheal therapy to be avoided because they i HUS risk

(raw meat) Affects children and older adults Generally lasts 5-1 0 days

vomiting

colitis Hemolytic uremic syndrome (HUS) is a potential complication (especially for serotype O1 57:H7), primarily in children

Salmonella spp.

Classically caused by ingestion of contaminated poultry or eggs,

Presents with a prodromal headache, fever, myalgia, and a bdominal pain

but many other foods may be contaminated Affects young children and the elderly; gener-

Sepsis is a concern, as 5%- 1 0% of patients become bacteremic Sickle cell patients are susceptible to i nvasive disease leading to osteomyelitis

Extremely contagious; transmitted between people by the fecal-oral route

Presents with high fever, a bdominal pain, and cramping

Affects young children and institutionalized patients Taenia solium

Trichinella spiralis

Presents with signs of elevated i ntracranial pressu re (head-

undercooked pork

aches, vomiting, seizures, visual changes)

developing countries (especially Mexico and Thailand)

Shigella spp. May lead to severe dehydration It can also cause febrile seizures in the very young Diarrhea may initially be watery and progress to become bloody/mucoid

Pork tapeworm Acquired by ingestion of

Acquired by ingestion of undercooked meat (primarily pork) in

remia treated with oral quinolone or trimethoprimsulfamethoxazole (TMP-SMX)

ally lasts 2-5 days Shigella spp.

First fluids; at-risk patients (eg, sickle cell patients) or those with bacte-

Classic triad of myositis, periorbital edema, and eosinophilia Possibility for migrating larvae to cause vasculitis, leading to splinter hemorrhages

T solium is diagnosed via CT or MRI showing several cysts with edema

Mu ltiorgan involvement is possible

A fluoroquinolone + azith romycin + third generation cephalosporin or TMP-SMX + ampicillin to prevent person-toperson spread Treatment with albendazole and with symptomatic management of CNS symptoms Albendazole (or mebendazole); corticosteroids in severe cases

GASTROINTESTINAL Treatment

Acute diarrhea: Oral rehydration key. Antibiotics are not indicated (except in C difficile infection or in the epidemic setting) because they do not shorten the course of illness. ■ Chronic diarrhea: Treatment specific to etiology. ■

H I G H -YI E L D FACTS I N

0-rr

223

KEY FACT

Dia rrhea after i ngestion of raw eggs or dairy: thi n k Salmonella.

MALABSORPTION/MALDIGESTION Inability to absorb macronutrients and/or micronutrients. Presents with chronic diarrhea with weight loss, growth failure, and macronutrient and/ or micronutrient deficiencies. ■ Celiac disease: Characterized by mucosa! inflammation, villous atrophy, and crypt hyperplasia in response to dietary gluten exposure. Celiac dis­ ease is associated with extraintestinal manifestations, including dermatitis herpetiformis (see Fig. 2.6-18) and autoimmune diseases of thyroid gland, liver, and type 1 diabetes. ■

Diagnosis

Serologic testing is crucial for screening and diagnosis and includes anti­ tissue transglutaminase-IgA (TTG-IgA) and endomysial IgA antibody testing. ■ Histologic analysis of duodenal biopsies is gold standard for diagnosis and grading based on Marsch classification. ■ Celiac disease has a strong genetic component, but testing for HLA-DQ2/ DQ8 has limited role in diagnosis. ■

F I G U R E 2.6- 1 8.

Dermatitis herpetifor­ mis. Grouped, papulovesicular, pruritic

skin lesions are shown. Lesions tend to be symmetrically located on the extensor surfaces of the elbows, knees, buttocks, and posterior scalp and are associated with celiac disease. (Reprod uced with permis­ sion from Caproni M et al. Celiac d i sease and der­ matologic man ifestations: many skin clues to u nfold g l uten-sensitive enteropathy. Gastroenterol Res Pract.

201 2;201 2:952753.)

Management

Lifelong adherence to gluten-free diet is the cornerstone of treatment and results in symptom resolution and mucosa! healing. ■ Concomitant testing for micronutrient deficiencies (iron, folic acid, vita­ mins D, B 12 ) should be undertaken and repleted as needed. ■ Close monitoring for other autoimmune diseases (thyroid, liver, type 1 diabetes) and osteoporosis should be done. ■

Other etiologies of malabsorption: ■ Mucosal abnormalities: Whipple disease (also presents with arthritis, lymphadenopathy, cardiac issues, periodic acid-Schiff [PAS]-positive granules in lamina propria on biopsy), tropical sprue (chronic diarrhea and nutritional malabsorption [ vitamin B 12 and vitamin B9 ] and living > 1 month in an endemic area) ■ Bile salt deficiency: Beal disease in Crohn disease or small bowel resec­ tions (> 100 cm of terminal ileum), bacterial overgrowth ■ Short bowel syndrome: Caused by resection of small bowel; results m malabsorption of both micronutrients and macronutrients; amount of bowel resection weakly correlated to likelihood/degree of symptoms ■ Small intestinal bacterial overgrowth (SIBO): Bacterial overgrowth due to various causes, resulting in bloating or chronic watery diarrhea. Under­ lying etiologies include abnormal motility, abnormal anatomy (eg, after abdominal surgery, GI cancer), metabolic and systemic disorders (eg, dia­ betes), and immune disorders (eg, IgA deficiency) ■ Hx/PE: Presents with pale, foul-smelling, bulky stools (steatorrhea or fat maldigestion) associated with abdominal pain, flatus, bloating, weight loss, nutritional deficiencies, and fatigue.

0-rr

KEY FACT

The i n itia l tests specific for celiac d isease a re lgA a nti-transgl uta m i nase a nti body or a ntiendomysia l a nti body. The gold sta ndard is i ntesti n a l biopsy, which wi l l s how i ncreased i ntraepi­ thelial lym phocytes (>25 per 1 00 enterocytes), crypt hyperplasia, a n d vi l lous atrophy.

224

H I G H -YI ELD FACTS I N

GASTROINTESTINAL ■ Dx: Multiple laboratory tests based on clinical suspicion. Biopsy is definitive. ■ Tx: Etiology dependent. In severe cases, patients may require total paren­ teral nutrition (TPN), immunosuppressants, and anti-inflammatory medi­ cations. Dapsone can be used for dermatitis herpetiformis.

CARBOHYDRATE MALDIGESTION Lactase Deficiency Can be primary (autosomal recessive) or secondary (acquired conditions that affect structural or functional integrity of small bowel). Common among populations of African, Asian, and Native American descent, also transiently after an acute episode of gastroenteritis. Hx/PE: Presents with abdominal bloating, flatulence, cramping, and watery diarrhea following dairy ingestion. Dx: Often treated empirically with lactose-free diet. Hydrogen breath test reveals i hydrogen following the ingestion of lactose. Tx: Avoidance of dairy products; oral lactase enzyme replacement.

0-Tr

Other Forms of Carbohydrate Maldigestion Sucrase-isomaltase deficiency: Rare homozygous recessive disorder with sucrose maldigestion. Maltase-glucoamylase deficiency. ■ Fructose intolerance: Can be hereditary (presents in infancy) or dietary (later in life). No standardized diagnostic tests are available, but fructose breath test can be suggestive. Treatment involves restricting fructose in diet.

KEV FACT

Patients with ca rci noid syndrome also develop niaci n/vita m i n B 3 deficiency (pel lagra) beca use tryptophan is meta bol ized i nto seroto n i n .

:0-

MNEMONIC

The classic presentation of pellagra (deficiency in niacin/vitamin 8 3 ) is the 4 Ds: Diarrhea, Dementia, Dermatitis, and Death.

0-Tr

CARCINOID SYNDROME Carcinoid syndrome is caused by metastasis of carcinoid tumors, which most commonly arise from the ileum and appendix and produce serotonin. Prior to metastasis, most secreted hormones undergo first-pass metabolism by the liver and do not cause systemic symptoms.

Hx/PE: Cutaneous flushing, watery diarrhea, abdominal cramps, wheezing, and right-sided cardiac valvular lesions are the most common manifestations. ■ Dx: High urine levels (elevated 24-hour urinary excretion) of the serotonin metabolite 5-hydroxyindoleacetic acid ( 5-HIM) (best initial test) are diag­ nostic. CT and In-111 octreotide scans are used to localize the tumor. Tx: Treatment includes octreotide for symptomatic patients and surgery in resectable disease. ■

SMALL BOWEL OBSTRUCTION KEV FACT

I n the U n ited States, the leading cause of SBO i n c h i l d ren is hernia. The leading cause of SBO i n adu lts is ad hesions.

Partial or complete blockage of passage of bowel contents through the small bowel. Etiologies: Adhesions (60% of cases), hernias (10%-20% ), neoplasms (10%-20% ), intussusception, gallstone ileus, stricture, and volvulus Partial SBO: Continued passage of flatus, but no stool ■ Complete SBO: No passage of flatus or stool (obstipation)

GASTROINTESTINAL

H I G H -YI E L D FACTS I N

225

History/PE ■ History: Crampy abdominal pain at 4- to 5-minute intervals. Vomiting typically follows the pain. ■ Abdominal exam: Distention, tenderness, prior surgical scars, or her­ nias; hyperactive bowel sounds (high-pitched tinkles and peristaltic rushes). ■ Complications: Ischemic necrosis and bowel rupture with prolonged or complete obstruction. Patients present with peritonitis manifested by fever, hypotension, rebound tenderness, and tachycardia. Diagnosis Best initial test: Abdominal x-ray demonstrates a stepladder pattern of dilated small bowel loops, air-fluid levels (see Fig. 2.6-19), and a paucity of gas in the colon. ■ Most accurate test: CT scan of the abdomen further characterizes obstruction and evaluates for etiology. ■ Complete blood cell count (CBC) may demonstrate leukocytosis if there is ischemia or necrosis of bowel. ■ Lab tests often reveal dehydration. Lactic acidosis is a prognostic sign, as it suggests necrotic bowel. Treatment ■ Best initial treatment: Fluid resuscitation. ■ Partial obstruction : Supportive care sufficient. It should include NPO status, NG suction, IV hydration, correction of electrolyte abnormali­ ties, Foley catheterization to monitor fluid status, and pain manage­ ment. Patient should avoid opioids and anticholinergics (slow GI motility). ■ Complete obstruction: Exploratory laparotomy indicated if there is pres­ ence of bowel necrosis, perforation, ischemia, or a surgically correctable cause of complete SBO. Otherwise, nonoperative management can be uti­ lized for a period of time if the patient is clinically stable.

I:

cs

F I G U R E 2.6- 1 9. Small bowel obstruc­ tion. (A) Supine x-ray of the abdomen

shows dilated air-filled small bowel loops with relatively little gas in the colon. (B) Left lateral decubitus x-ray on the same patient demonstrates multiple air-fluid levels (arrows) at different levels. These are typical plain film findings of com­ plete SBO. (Reproduced with permission from Doherty GM. Current Diagnosis & Treatment: Surgery, 1 3th ed. New York, NY: McGraw-Hill; 201 0.)

ILEUS

0-n

Loss of peristalsis without structural obstruction.

Ga l l stone ileus is a form of SBO that occurs when a g a l l stone lodges at the i l eoceca l va lve after passi ng thro ugh a cholecystoenteric fistu la.

Risk Factors Recent surgery/GI procedures, severe medical illness, immobility, hypokale­ mia or other electrolyte imbalances, hypothyroidism, OM, and medications that slow GI motility (eg, anticholinergics, opioids). History/PE ■ Diffuse, constant abdominal discomfort; nausea and vomiting; an absence of flatus or bowel movements. ■ Diffuse tenderness, abdominal distention, and .J.. or absent bowel sounds. A rectal exam is required to rule out fecal impaction in older adult patients.

KEY FACT

A 53-yea r-o l d wo m a n with a h i story of carci noid tumor of the a p pe n d ix (status post-resection) presents to a local c l i n i c with sym metric, d ry, hyperpig me nted skin lesions and pers istent d ia rrhea. Her h u sband expresses concern that the pati ent does not seem to be herself anym ore; he re po rts that she has been i rritable, confused, and fo rgetful . What is the most l i kely diag nosis?

226

H I G H -YI ELD FACTS I N

0-n KEY FACT I n ileus, there is a i r present throug hout the small a n d la rg e bowel o n x-ray of the abdomen.

GASTROINTESTINAL

Diagnosis ■ Must consider clinical history in diagnosis. ■ Best initial test: Abdominal film showing distended loops of small and large bowel, with air seen throughout the colon and rectum (SBO has no air distal to the obstruction). ■ Most accurate test: CT scan of the abdomen. Treatment ■ -l, or discontinue the use of narcotics and any other drugs that reduce bowel motility. ■ Bowel rest: Temporarily -l, or discontinue oral feeds. ■ Bowel decompression: Initiate low, intermittent NG suctions and paren­ tal feeds. Supportive care: Replete electrolytes as needed; hydrate with IV fluids.

ACUTE ABDOMEN Any condition that presents with new-onset, severe abdominal pain and ten­ derness. These patients usually require surgery, but many nonsurgical mimics exist. A physician should always consider a gynecologic etiology in females and nonsurgical causes of acute abdomen (see Table 2.6-9) in all patients.

History/PE In general, four pathologies contribute to acute abdomen and present with characteristic symptoms. Considering these mechanisms along with location and associated history/physical examination findings may help delineate the diagnosis. The four pathologies are perforation/rupture, obstruction, inflammation, and ischemia. Perforation or rupture: Sudden onset of diffuse, excruciating pain. Patients will lie still to minimize pain. Peritoneal signs are prominent. ■ Esophageal perforation (Boerhaave syndrome) : Has been associated with recurrent vomiting/hematemesis, but up to 4 5 % of patients have no history of emesis. Esophageal perforation commonly presents with retrosternal and epigastric pain. ■ Other GI perforation: Associated with PUD, cancer, diverticulitis, and IBO. ■ Ruptured abdominal aortic aneurysm (AAA): Most common in male smokers >65 years of age. Patient may describe periumbilical pain that radiates to the back. Abdominal exam may reveal pulsatile mass. TA B L E 2 . 6 - 9 .

Pel lagra, a deficiency of vita m i n B 3 (niacin), secondary to a recu rrent carcinoid tumor. Carcinoid tumors prod uce se roto n i n, which is a derivative of trypto p h a n . However, trypto p h a n is a l so the prec u rsor of niaci n . I n patie nts with carc i n o i d tumors, the tumor can b e so a ctive that most tryptophan is used for seroto n i n prod uction, resu lti ng i n n i a c i n deficiency.

ETI O LOGY Extra-a bdominal

Nonsurgical Causes of Acute Abdomen

CAUSE Myoca rd ial infarction (Ml), pulmonary embolism, pneumonia

All of these cause right or left u pper quadra nt pai n

Hematologic

Sickle c e l l crisis, leukemia

Meta bolic

Diabetic ketoacidosis, u remia

Genetic/familial

Fa milial Mediterranean fever, acute i ntermittent porphyria

Toxic

Lead and heavy metal poison ing, black widow spider bite

GASTROINTESTINAL ■ Ruptured ectopic pregnancy/ovarian cyst: Presents with first-trimester bleeding, abdominal pain, and hypovolemic shock. Abdominal pain is usually localized to the pelvis, but specific location and quality of pain are variable. Obstruction: Sudden onset of severe, colicky, intermittent pain. Patients cannot sit still. Peritoneal signs are usually absent. ■ SBO: Most commonly from adhesions years after abdominal surgery, incarcerated hernias, or in patients with IBD or cancer. Patient may complain of obstipation (failure to pass stool or gas). Bowel sounds are high-pitched early in the disease process. ■ Volvulus: Abdominal pain with insidious onset that is continuous, with intermittent exacerbations associated with peristalsis. Patients also com­ plain of nausea, distention, and obstipation. Immediate surgery is pur­ sued in the presence of peritonitis or perforation. Otherwise, endoscopic detorsion may be used for management of sigmoid volvu­ lus. Cecal volvulus is mostly treated surgically. ■ Ureteric/biliary obstruction: Nephrolithiasis causing colicky pain that usually radiates to the groin and may cause hematuria/pyuria. This condition may also cause costovertebral angle tenderness (CVAT). Bil­ iary colic is most common in multiparous, overweight women (female, forty, fertile, and fat). Fatty meals exacerbate pain. There are no signs of peritoneal irritation. ■

Inflammation: Gradual onset (over 10-12 hours) of constant, poorly local­ ized pain that later localizes to problem area. Patients lie still to minimize pain. Peritoneal signs are prominent. Appendicitis: Detailed later. ■ Cholecystitis: Same demographics as biliary colic. Pain is constant. Peritoneal irritation is evident. Murphy sign causes pain on inspiration. ■ Diverticulitis: Prominent left lower quadrant (LLQ) pain, change in bowel habits, and ± nausea/vomiting. Palpation or rectal exam may reveal mass (abscess). ■ Pelvic inflammatory disease (PIO): Associated with history of sexually transmitted diseases or occurring in young females with unsafe sexual practices. Pelvic exam elicits cervical motion tenderness and adnexal tenderness.

■

Ischemia: Variable presentation, dependent on specific etiology. ■ Acute mesenteric ischemia: Sudden onset of pain and hematochezia in a patient with history of atrial fibrillation. ■ Ischemic colitis: Postprandial abdominal pain ± hematochezia/ melena in a patient with significant atherosclerotic disease (prior myo­ cardial infarction [Ml] /stroke, peripheral artery disease). ■ Strangulated hernia: Irreducible bulge in abdominal wall. ■ Ovarian torsion: Sudden onset of adnexal pain ± nausea/vomiting. Character of pain is variable. Palpation usually reveals a mass. Condi­ tion has a high index of suspicion in females since clinical presentation is variable.

Diagnosis ■

To rule out OB/GYN causes in women: Urine J3-human chorionic gonadotropin (J3-Hcg) for ectopic pregnancy; pelvic ultrasound for ovarian torsion, ruptured cyst, or fibroids; pelvic exam ± swab for PID To rule out extra-abdominal mimics in patients with upper abdominal pain: Troponins and ECG for Ml, o-dimer/CT angiogram for pulmonary embolism, CXR for pneumonia

H I G H -YI E LD FACTS I N

227

0-,r KEY FACT �-H u m a n chorionic gonadotropin (�-Hcg) is a vita l sign i n women with acute a bdomen. E0 �-Hcg in the setti ng of shock is a ru ptu red ectopic preg na ncy u ntil proven otherwise.

0-,r KEY FACT Ad hesions a re the most common cause of s m a l l bowel obstruction i n patients with a h i story of abdom i n a l s u rgeries.

0-,r KEY FACT Acute a bdom i n a l pai n with blood per rectu m is acute mesenteric ischemia u ntil proven otherwise. Classical ly, an older ad u lt patient with a history of atrial fibril lation or recent AAA repair presents with these fi n d i ngs.

228

H I G H -YI E L D FACTS I N

GAST ROINTESTINAL

■

■

To rule out nonsurgical abdominal causes, if appropriate: Amylase/lipase in patient consistent with pancreatitis (nausea/vomiting, epigastric pain, hunched over), CT abdomen without contrast in patient consistent with kidney stones/pyelonephritis (hematuria, flank pain, radiation to groin), paracentesis for patient consistent with systolic blood pressure (ascites, fever) Additional diagnostic tests to rule in surgical diagnoses: ■ X-ray of the abdomen: Perforation (see Fig. 2.6-20), SBO, volvulus ■ CT with contrast: Appendicitis, diverticulitis, IBO, abscess, cancer, AAA Right upper quadrant (RUQ) ultrasound: Cholecystitis, biliary colic, choledocholithiasis

M anagement F I G U R E 2.6-20.

Pneumoperitoneum.

Free gas under the right and left hemi­ diaphragms visible in erect CXR. This may occur due to small bowel perfora­ tion. (Reprod uced with perm ission from Buckle C. Holdridge C. Xu T, et al. Acute abdominal pain and

A detailed approach to management is beyond the scope of this section, but general concepts are as follows: ■ ■ ■

rad iological pneu moperito neum: always a n I n d ication for Laparotomy? J C/in Med Res 201 3;5:2. doi: 1 0.402 1 / jocm r929w.)

■

In the presence of peritoneal signs or shock: Exploratory laparotomy. All unstable patients and those with suspected potential hemorrhage: Blood typing, cross-matching and transfusion as needed. Patients with perforation or signs of sepsis: Broad-spectrum antibiotics. Treatment with these antibiotics is also indicated for patients with sus­ pected infectious processes (cholecystitis, diverticulitis, pyelonephritis). Stable patients: Expectant management, possibly including NPO status, NG tube placement (for decompression of bowel in the setting of obstruc­ tion or acute pancreatitis), IV fluids, placement of a Foley catheter (to monitor urine output and fluid status), and vital sign monitoring with serial abdominal exams and serial labs.

DUODENAL HEMATOMA Duodenal injuries are rare and involve the second part of the duodenum most commonly. Duodenal injuries are often accompanied by injuries to sur­ rounding structures in the retroperitoneal region. Initial presentation can be nonspecific, and suspicion should arise in cases with direct blow or impact to the mid-abdomen. Etiology ■ ■

In children, may result from a blunt trauma through bicycle handlebar or abuse. In adults, more often due to penetrating injuries (eg, gun shot, stabbing). Nonpenetrating injuries with duodenal hematoma in adults are typically due to steering wheel injuries.

History/PE ■

■

■

Typically, signs and symptoms are nonspecific. Patients may have signs and symptoms of GI obstruction such as bilious emesis, with an inability to tolerate oral intake. Presentation is often insidious, leading to delayed diagnosis. Some patients develop symptoms 48 hours after injury (due to gradual fluid shift into hyperosmotic hematoma). PE: Ecchymosis may be seen in the pattern of injury (crush injury with bicycle handlebar or seat belt sign), abdominal tenderness, peritonitis, or palpable upper abdominal mass.

GASTROINTESTINAL

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229

Diagnosis

■ Abdominal CT is the best initial test for duodenal injury in hemodynami­ cally stable patients. Alternatively, upper GI series may be used (shows coiled spring sign or obstruction). ■ Patients who are hemodynamically unstable may be diagnosed during explorative laparotomy. ■ Pancreatic injury and other associated injuries should be excluded. Treatment

For blunt duodenal injuries, nonsurgical management in hemodynami­ cally stable patients is recommended. Patients receive NG suction and TPN. Patients are followed with upper GI series or ultrasound at 5-day to 7-day intervals if signs of obstruction do not abate. Surgery may be consid­ ered if conservative management fails. ■ For penetrating duodenal injury, conservative management is not recom­ mended, and patients should undergo surgery (duodenal repair, decom­ pression, and other procedures). ■

MESENTERIC ISCHEMIA Insufficient blood supply to the small intestine, resulting in ischemia and, potentially, necrosis. See Table 2.6-10 for clues to differentiating between types of ischemia. The most common causes are as follows: ■ Embolism: Most commonly originates in the heart. Risk factors include atrial fibrillation and stasis from -I, ejection fraction. ■ Acute arterial occlusion from thrombosis: Most commonly occurs in the proximal superior mesenteric artery (SMA). The primary risk factor is atherosclerosis. ■ Other causes: Nonocclusive arterial disease (atherosclerosis of mesenteric vessels, arteriolar vasospasm), venous thrombosis (caused by hypercoagula­ ble states), or shock state. History/PE

Presents with severe abdominal pain out of proportion to the examination, nausea, vomiting, diarrhea, bloody stools, prior episodes of abdominal pain after eating ("intestinal angina"). Diagnosis

Best initial test: X-ray or CT scan of the abdomen may reveal bowel wall edema ("thumbprinting") and air within the bowel wall (pneumatosis intestinalis). ■ Most accurate test: Mesenteric/CT angiography is the gold standard for diagnosis of arterial occlusive disease, but conventional angiography allows for intervention of thrombosis/embolism. ■

Treatment

■ Best initial treatment: Volume resuscitation, broad-spectrum antibiotics ■ For acute arterial thrombosis or embolism: Anticoagulation and either laparotomy or angioplasty

I A 65-year-old male smoker is brought to the emerge n cy d epartment for sudde n-on set a bdominal and back pa i n . The a nxious pati ent com p l a i n s of "ri p p i n g pa i n :' Physical exa m i n ation reveals a l a rg e, pu lsati l e mass be h i nd the u m b i l icus. BP i s 80/50 mm Hg; hea rt rate 1 25 beats per m i n ute (bpm). Th e physician beg i n s crysta l l oid and blood i nfu sions. What is the most ap propriate next ste p i n manageme nt?

230

H I G H -YI ELD FACTS I N

TA B L E 2 .6- 1 o .

Site

GASTROINTESTINAL

Differentiating Types of Mesenteric lschemia

ACUTE MESENTERIC ISCHEMIA

ISCHEM I C COLITIS

CHRON I C MESENTERIC ISCHEMIA

Small intestine

Large i ntestine, watershed zones (splenic flexure and rectosigmoid

Small and large intestine

junction) Etiology

Thromboembolic (atherosclerosis, atrial fi bril­ lation, oral contraceptives) Cholesterol emboli after percutaneous vascular procedu res

Symptoms

Sudden-onset severe pain, diarrhea (bloody or non bloody)

Transient decrease in perfusion

Fixed progressive obstruction

pressure due to decreased cardiac output (eg, dehydration, shock)

in blood flow (eg, atheroscle­ rosis of mesenteric vessels)

Bloody diarrhea with abdominal pain

Postprandial pain leading to fear of eating and weight loss

X-ray: Thumbprinting of watershed

Duplex ultrasonography

Pain "out of proportion" to examination findings Investigations

X-ray: ileus, may show portal venous gas or gas within the walls (pneumatosis intestinalis) CT angiogram-investigational method of choice

Treatment

Supportive measures with fluids; antibiotic thera py followed by su rgery/embolectomy

0-n KEY FACT Cholesterol embolism may occur after cardiac catheterization, and ischemia of multiple organs may be seen (bowel, kidney, pancreas, lower extremity skin causing livedo reticularis).

■ ■

areas of colon

Optimization of cardiac output with fluids, treatment of shock

May require revasculariza­ tion, either surgical or percutaneous

For venous thrombosis: Anticoagulation Surgery: Resection of infarcted bowel

Com plications Sepsis/septic shock, multisystem organ failure, death

ACUTE APPENDICITIS Obstruction of the appendiceal lumen with subsequent inflammation and infection. Rising intraluminal pressure leads to vascular compromising of the appendix, ischemia, necrosis, and possible perforation. Etiologies include hypertrophied lymphoid tissue (5 5 %-65 %), fecalith (3 5 %), foreign body, tumor (eg, carcinoid tumor), and parasites. Incidence peaks in the early teens (most patients 10-30 years of age), and the male-to-female ratio is 2 : 1.

Based on the clin ical findings of shock, a bdom inal pain, a nd a pulsatile mass, the patient has a ru ptu red abdom ina l aortic aneurysm (AAA), which is a surgica l emergency. If the patient is sta ble, u ltrasonography or urgent CT a re the next best steps and inform i ntervention; however, im med iate laparotomy or endovascular repai r should not be delayed in the unstable patient.

H istory/PE ■ Classically presents with dull periumbilical pain lasting 1 to 12 hours with subsequent migration to sharp right lower quadrant (RLQ) pain at McBur­ ney point. ■ Can present with nausea, vomiting, anorexia, and low-grade fever. ■ Psoas, obturator, and Rovsing signs not sensitive tests, but their presence i the likelihood of appendicitis. Psoas abscess can present similarly to

GASTROINTESTINAL

H I G H -YI E LD FACTS I N

231

appendicitis, but with a more insidious onset over days. CT can distin­ guish between the two. ■ In perforated appendicitis, possibility of partial pain relief. Peritoneal signs (eg, rebound, guarding, hypotension, i WBC count, fever) will ultimately develop. ■ Atypical presentations possible in children, older adults, pregnant patients, and those with retrocecal appendices. These may result in misdiagnosis and i mortality. Atypical or nonspecific features include indigestion, flatu­ lence, diarrhea, bowel irregularity, and generalized malaise. Diagnosis

■ Appendicitis is a clinical diagnosis in patients with classic history (described earlier), fever, and leukocytosis. ■ Investigation can occur with CT IV contrast (see Fig. 2.6-21) or RLQ ultrasound (preferred in children and pregnant patients). Treatment

■ IV antibiotics with anaerobic and gram 8 coverage (eg, cefoxitin or cefazolin plus metronidazole). The patient should be NPO and receive IV hydration, analgesia, and antiemetics. ■ Uncomplicated appendicitis: Surgery or observation with antibiotics depending on the clinical scenario. If appendicitis not found, complete exploration of the abdomen is performed. There is no need to administer antibiotics postoperatively. ■ Perforation: Perform immediate open or laparoscopic appendectomy. Administer antibiotics postoperatively until the patient is afebrile with a normalized WBC count. If open approach is used, the incision should be closed by delayed primary closure. ■ Abscess: Broad-spectrum antibiotics and CT-guided drainage. Interval appendectomy should be performed 6 to 8 weeks after resolution of abscess.

F I G U R E 2.6-2 1 .

d uced with permission from USMLE-Rx.com.)

0-n

CLOSTRIDIUM DIF FICILE COLITIS Traced to spore-forming, toxin-producing, gram-positive anaerobic bacteria that colonize the colon after normal gut flora are disrupted by antibiotics (penicil­ lins, quinolones, cephalosporins, clindamycin). Infection with C difficile classi­ cally affects adult patients in hospitals and nursing homes, as well as those with significant risk factors (eg, IBO). History/PE

■ It is typically associated with recent antibiotic usage. ■ Additional risk factors include age >65 years, recent hospitalization, and use of PPis. ■ Symptoms range from asymptomatic carriage to profuse diarrhea (> 3 watery loose stools in 24 hours), fever, abdominal pain, and possible sys­ temic toxicity. ■ Infection with C difficile most commonly causes colitis, but it can involve the small bowel.

KEY FACT

The McBurney point is located one­ third of the distance from the a nterior superior iliac spine to the umbilicus. It is an important part of a physical examination because this location corresponds to the base of the appendix.

0-n DISORDERS OF THE LARGE BOWEL

Acute appendicitis.

Contrast-enhanced CT demonstrating an enlarged, hyperenhancing appendix with an appendicolith visualized in the lumen and periappendiceal fat stranding located anterior to the right posts muscle. (Repro­

KEY FACT

Surgical incisions can be closed by the following: ■ Primary closure (primary intent): Surgical a pproximation using sutures or staples ■ Secondary closure (secondary intent): No approximation, typical ly packed with gauze, fi l led in with gra n u lation tissue ■ Delayed primary closure (tertiary intent): Similar to primary closure; occurs after several days of observation to permit drainage

232

0-n

H I G H -YI ELD FACTS I N

GAST ROI NTESTI NAL

Diagnosis

KEY FACT

Lack of history of a ntibiotic usage does not exclude the possibility of C. difficile colitis. Leukocytosis in the a bsence of diarrhea in a hospitalized patient can be related to C. difficile colitis.

■

C difficile toxin can be identified in the stool sample. Sigmoidoscopy may be normal or show patchy erythema in mild cases and pseudomembranes in severe cases.

Treatment

Cessation of the inciting antibiotic Nonsevere (WBC 1. 5): PO fidaxomicin > PO vancomycin ■ Recurrent (first, second, and subsequent episodes): PO fidaxomicin > PO vancomycin and consider fecal microbiota transplantation ■ Fulminant without ileus: PO vancomycin + parenteral metronidazole ■ Fulminant with ileus: PO and rectal vancomycin + parenteral metronidazole ■ ■

Com plications

Toxic megacolon presents with large bowel dilatation (>7 cm diameter in colon or > 12 cm in the cecum).

DIVERTICULAR DISEASE Diverticula: Outpouching of mucosa and submucosa (false diverticula) that herniate through the colonic muscle layers in areas of high intralumi­ nal pressure; most commonly found in the sigmoid colon ■ Diverticulosis: Presence of many diverticula - most common cause of acute lower GI bleeding in patients >40 years of age. Predominantly left­ sided in Western countries. Bleeding usually results from weakened intes­ tinal vasa recta vessels. Diverticulitis Inflammation following microperforations secondary to fecalith impaction and high luminal pressure. Risk factors: Diets that worsen constipation (eg, low fiber, red meat, and high-fat content), advanced age (65 % occur in those >80 years of age), and connective tissue disorders. ■

0-n

KEY FACT

Diverticulosis is the most common cause of acute lower GI bleeding in patients >40 years of age.

History/PE

Diverticulosis (see Fig. 2.6-22): Often asymptomatic until patients pres­ ent with sudden, intermittent, painless bleeding, which can cause symp­ toms of anemia when bleeding is severe. Diverticulosis is associated with chronic constipation, which increases intraluminal pressure of the colon and worsens outpouchings. ■ Diverticulitis: LLQ abdominal pain, fever, nausea, and vomiting. Perforation is a serious complication that presents with peritonitis and shock. Diagnosis ■ ■

Clinical history is important to diagnosis. CBC may show leukocytosis or anemia.

GASTROINTESTINAL

F I G U R E 2.6-22.

ticulosis.

H I G H -YI E L D FACTS I N

233

(A) An endoscopic view of diverticulosis. (B) An axial CT slice visualizing diver­

(Image A adapted with perm ission from USMLE-Rx.com. I mage B adapted with permission from Sartelli M, Moore FA, Ansaloni L, et al. A proposal for a CT d riven classification of left colon acute d iverticulitis. World J Emerg Surg. 20 1 5; 1 0:3. https://doi. org/1 0.1 1 86/1 749-7922-1 0-3.)

■ Most accurate test: Colonoscopy provides definitive diagnosis in divertic­ ular disease; however, sigmoidoscopy/colonoscopy should be avoided in patients with acute diverticulitis because of the risk for perforation. ■ In acute diverticulitis, CT scan is the best test for diagnosis; it may reveal inflammation or abscess (see Fig. 2.6-23).

Treatment ■ Uncomplicated diverticulosis: Routine follow-up is indicated. Patient should receive encouragement to follow a high-fiber diet or take fiber supplements. ■ Diverticular bleeding: Bleeding usually stops spontaneously; physician should transfuse and hydrate patient as needed. If bleeding does not stop, hemostasis by colonoscopy, angiography with embolization, or surgery is indicated. ■ Diverticulitis: Treat with bowel rest (NPO), NG tube placement (if severe), and broad-spectrum antibiotics (if complicated, metronidazole and a fluoroquinolone or a second- or third-generation cephalosporin). Uncomplicated, left-sided diverticulitis may initially be treated in the out­ patient setting without antibiotics. Colonoscopy can occur after the initial stage. ■ Hospitalization: If there is evidence of peritonitis or systemic signs of infection. ■ For perforation: Immediate surgical resection of diseased bowel via a Hartmann procedure with a temporary colostomy. Compl ications Diverticulitis may cause fistulas in other organs, leading to pneumaturia, ster­ ile pyuria, fecaluria, or fecal discharge from the vagina. The physician should diagnose with CT and treat with surgical resection.

F I G U R E 2,6-23.

Acute diverticulitis.

Coronal reconstruction from a contrast­ enhanced CT demonstrates sigmoid diverticula with presigmoid inflammatory "fat stranding." The area of abnormality is circled in red. GB, gallbladder; L, liver; S, stomach; UB, urinary bladder. (Reproduced with perm ission from USMLE-Rx.com.)

0-n

KEY FACT

Sigmoidoscopy should be avoided when there is clinical and imaging evidence of diverticulitis because of the risk for perforation.

234

TABLE 2.6- 1 1 .

H I G H -YI ELD FACTS I N

GASTROINTESTINAL

Characteristics of Small and Large Bowel Obstruction

VA RIABLE

SMALL BOWEL OBSTRUCTION

LARGE BOWEL OBSTRUCTION

History

Moderate to severe acute abdominal pain; copious emesis Cramping pain with distal SBO

Constipation/obstipation, deep and cramping a bdominal pain (less intense than SBO), nausea/vomiting (less than that of

Fever, signs of dehydration, and hypotension possible Examination

Abdominal distention (distal S BO), abdominal tenderness, visible peristaltic waves, fever, hypovolemia Examination for surgical scars/hernias; rectal examination High-pitched "tin kly" bowel sounds; later, a bsence of bowel sounds

SBO, but more commonly feculent) Significant distention, tympany, and tenderness Examination for peritoneal irritation or mass Fever or signs of shock suggesting perforation/peritonitis or ischemia/necrosis High-pitched "tinkly" bowel sounds; later, absence of bowel sounds

Etiologies

Adhesions (postsurgery), hernias, neoplasm, volvu lus, Crohn disease, intussusception, hematoma, foreign body, cystic fi brosis (CF), gallstone ileus

Colon cancer, volvulus, diverticulitis, intussusception, fecal i mpaction, benign tumors Colon cancer assumed until proven otherwise

Differential

LBO, paralytic ileus, gastroenteritis

SBO, paralytic ileus, appendicitis, IBD, Ogilvie syndrome (pseudo-obstruction)

Diagnosis

CBC, electrolytes, lactic acid, x-ray of the abdomen, con­ trast studies (determine if it is partial or complete), CT

CBC, electrolytes, lactic acid, x-ray of the abdomen, CT scan, water contrast enema (if perforation is suspected), endoscopic evaluation if stable and prior imaging equivocal for bowel perforation

scan

Treatment

Hospitalize. Partial SBO can be treated conservatively with NG decompression, IV fluids, and N PO status. Patients with com plete SBO should be managed aggressively with N PO status, NG decompression, IV fluids, electrolyte replacement, and surgical correction. Underlying causes of obstruction (eg hernia or cancer) should be treated as well, if present.

:() MNEMONIC 3-6-9 Rule of Bowel Dilation I n general, when the bowel is dilated greater than the following dimensions, physicians consider it dilated. Obstruction should be considered. Small bowel: pedunculated

High-fat, low-fiber diet, alcohol, and sedentary lifestyle

GASTROINTESTINAL TA B L E 2 .6- 1 3 .

H I G H -YI E LD FACTS I N

237

Screening Recommendations for Colorectal Cancer

RISK CATEGORY

RECOMMENDATIONS

No past medical or family

Starting at 45 years of age (U.S. Preventive Services Task Force grade B): ■ Annual fecal occult blood test (FOBT), fecal immunochemical

history

test (FIT) or DNA-based stool tests (eg, Cologuard; in certain patients)

■ Colonoscopy every 1 0 years or ■ Sigmoidoscopy every 5 years First-degree relative with colon cancer

Colonoscopy every 5 years, starting at 40 years of age, or co­ lonoscopy every 5 years, starting 1 0 years before the age of affected family member at time of diagnosis (whichever comes first). Average-risk screening resumes at age 60.

I nflammatory bowel disease

Colonoscopy every 1 -2 years starting 8-1 0 years after diagnosis

Hereditary nonpolyposis colon cancer syndrome

Colonoscopy every 1 -2 years starting at 25 years of age

Familial adenomatous polyposis

Sigmoidoscopy every year starting at 1 2 years of age

High-risk colonoscopy

Colonoscopy every 3-5 years

fi ndings (eg, high-grade dysplasia, > 1 cm, and villous com ponent)

Treatment

■ Best initial treatment: Surgical resection of the tumor ( colectomy of vary­ ing length depending on tumor size) ± radiation for rectal cancer ■ Neoadjuvant chemotherapy and/or radiotherapy usually administered to reduce tumor burden ■ Follow-up with serial carcinoemb ryonic antigen (CEA) levels to detect recurrence; colonoscopy 1 year after resection and every 3 to 5 years there­ after; LFTs, CXR, and abdominal CT to screen for metastases

COLORECTAL CANCER-ASSOCIATED CONDITIONS Several genetically linked diseases may increase the risk of lower intestinal malignancy, including Lynch syndrome, Peutz-Jeghers syndrome, Gardner syndrome, Turcot syndrome, and juvenile polyposis syndrome. Lynch Syndrome

Also called heredita ry nonpolyposis colorectal cancer (HNPCC). Autosomal dominant disease that increases risk for Colorectal, Endometrial, Ovarian ("CEO") cancers due to a mismatch-repair gene deficiency (eg, MSH2, MLHl , MSH6, PMS2, and EPCAM).

■ Hx/PE: Patient aged < 50 years with positive family history of colorectal (eg, three or more members) or associated cancers ■ Dx: Genetic testing for most commonly mutated genes (listed earlier) ■ Tx: Colonoscopy eve ry 1 to 2 years starting at age 20 to 25 years or 5 years prior to earliest family Lynch diagnosis

I A 60-year-old pati ent with no past medica l h i story p resents with fever, dyspnea, a n d o rt h o p n ea of 2 wee ks' d u ration. P hysi ca l exa m i nation reveals spli nter hemorrhages a nd a new IV/ VI d i a sto l i c decrescendo m u rm u r. Echocard iog ra m confi rms aortic valve endoca rd itis, and IV a nti biotics a re sta rted. Blood c u lt u res a re EB fo r Streptococcus bovis. What is the n ext d iag nostic ste p7

238

H I G H -YI E L D FACTS I N

GAST ROI NTESTI NAL

Peutz-Jeghers Syndrome

Autosomal dominant disease that leads to benign, hamartomatous polyps. Although polyps are benign, patients remain at higher risk for GI, breast, and gynecologic cancers. ■ ■

F I G U R E 2.6-26. A classic finding of Peutz-Jeghers syndrome: pigmented macules on oral mucosa. (Reproduced with perm ission from Gondak RO, da Silva-Jorge R, Jorge J, Lopes MA, Vargas PA. Ora l pig mented lesions: Clinico­ pathologic features and review of the l iterature. Med Oral Pata/ Oral Cir Bucal. 201 2;1 7[6]:e91 9-e924.)

■

Hx/PE: Numerous mucocutaneous pigmented macules and possibly a

family history of the associated cancers Dx: Diagnosis with two of three clinical history criteria: family history of Peutz-Jeghers, presence of hyperpigmented macules (see Fig. 2.6-26), and/ or hamartomatous polyps found in GI tract Tx: Requires GI cancer screening at diagnosis (EGD, video capsule endoscopy, and colonoscopy) with subsequent screening based on pres­ ence of polyps

Gardner Syndrome

Familial adenomatous polyposis (FAP) + osteomas + fibromatosis ■

■ ■

Hx/PE: Painless bone growths typically on the skull or facial bones.

Unerupted teeth. Dermal manifestations such as lipomas or fibromas. Fundoscopy showing hypertrophy of retinal pigment epithelium. Dx: Occurs through genetic testing or presence of > 100 colorectal polyps and classic skin and bone findings. Tx: Screening for GI cancer with sigmoidoscopy every year, starting at 12 years of age (similar to that of FAP).

Tu rcot Syndrome

FAP + brain tumors (eg, medulloblastoma) ■ ■ ■

Hx/PE: Signs of neurologic deficit in the setting of positive family history

or > 100 polyps found on colonoscopy Dx: Genetic testing for APC mutation or mismatch-repair gene mutations (eg, MLH 1 and PMS2) Tx: Similar screening to FAP, as well as neurologic screenings (brain imaging for screening not typically done)

J uven i l e Polyposis Synd rome {J PS)

Autosomal dominant condition causing numerous hamartomatous polyps in the GI tract in young patients. ■

■

■

Colonoscopy is the next d iag nostic step. Although the mechanism of association has yet to be determi ned, there is a wel l-established association between 5. bovis and colon ca ncer. Clostridium septicum is also associated with colon ca ncer.

Hx/PE: Most commonly presents in a young adult ( 5 polyps found in colorectum, or multiple polyps anywhere else in GI tract. Tx: Colonoscopy every 1 to 3 years or yearly if polyps are found; upper endoscopy every 2 to 3 years or yearly if polyps are found.

ISCHEMIC COLITIS Insufficient blood supply to the colon that results in ischemia and, poten­ tially, necrosis. Most commonly affects the left colon, particularly the "watershed area" at the splenic flexure (see Fig. 2.6-27 for anatomic illustra­ tion of watershed areas of the colon). Usually occurs in the setting of atherosclerosis.

GASTROINTESTINAL

Abdominal aorta

Superior mesenteric artery

Superior and inferior mesenteric artery watershed

,/\

I

{'

I'

(-­ \ 't--

Hypogastric artery -�-1 (internal iliac artery)

Inferior mesenteric ro=,=,e....------l and hypogastric artery watershed Rectosigmoid junction

F I G U R E 2.6-27.

Watershed areas of the GI tract. (Reproduced with permission from USMLE-Rx.com.)

History/PE ■ Presents with crampy lower abdominal pain followed by bloody diarrhea after meals or exertion or in the heat. Fever and peritoneal signs suggest bowel necrosis. ■ Risk factors: Surgery that may reduce blood flow to colon (eg, AM repair, coronary artery bypass), atherosclerosis risk factors (eg, diabetes and hyper­ tension), hypercoagulability, and constipation. Differential ■ Acute mesenteric ischemia: History more suggestive of a thrombus or embolus in the setting of arrythmia or long-standing atherosclerosis. Infarction is not localized to watershed areas. ■ Chronic mesenteric ischemia: Less acute presentation than ischemic coli­ tis. Patient has history of food aversion due to postprandial pain. Diagnosis ■ Best initial test: CT scan with contrast possibly showing thickened bowel wall, atherosclerosis ■ Most accurate test: Angiography ■ Colonoscopy possibly showing a pale mucosa with petechial bleeding Treatment ■ Supportive therapy with bowel rest, IV fluids, and broad-spectrum antibiotics ■ Surgical bowel resection indicated for infarction, fulminant colitis, or obstruction

H I G H -YI E L D FACTS I N

239

240

H I G H -YI ELD FACTS I N

TA B L E 2 .6- 1 4 .

GASTROINTESTINAL

Differentiating Anorectal Diseases

DISEASE

CLASSIC PRESENTATION

RISK FACTORS

TREATMENT

External hemorrhoids (below dentate

Pruritic and painfu l

Obesity, constipation, older age, and pregnancy

High-fiber diet, sitz baths, stool soft­ eners, topical analgesia

May appear blue if thrombosed

line)

Hemorrhoidectomy if refractory

Internal hemorrhoids (above dentate line)

Pai nless, bright-red bleeding after defecation or wiping

Obesity, sitting on toilet for

Rectal prolapse

Protruding, erythematous mass with concentric rings seen when patient bears down

Multiparity, prior pelvic surgery, older age,

May have preceding discomfort, constipation, incontinence Anal fissure

Intense pain lasting for hours typically following defecation ± blood on toilet paper Classically seen on examination at posterior

extended times, older age, and pregnancy

chronic constipation or diarrhea, or post-stroke

High-fi ber diet, sitz baths, stool soft­ eners, topical analgesia Rubber band ligation if refractory Diet and l ifestyle modifications (increased fiber and water intake) If refractory and symptomatic, pos­ sibly surgery (rectopexy)

Constipation or diarrhea, Crohn disease, or malignancy

Diet and lifestyle modifications; topical anesthetics and vasodilators If refractory, lateral sphincterotomy

Constipation, DM, and

Prompt incision and drainage fol­

midline of anal canal Abscess

Tender, fluctuant mass at anal verge May have fever or other systemic symptoms

immunosuppression

lowed by empiric antibiotics

Anorectal fistula

I ntermittent, malodorous perianal drainage and pain with defecation

Perianal a bscesses, Crohn disease, and malignancy

Surgical closure (fistulotomy)

Proctalgia fugax

Recu rrent rectal pain unrelated to defecation, lasting seconds to minutes No organic cause identifiable

Higher incidence among females and age -�-60 years of age). ■ Can convert to acute myeloid leukemia in a small proportion of patients. ■

■

Relative erythrocytosis is associated with hypovolemia and dehydration: diuresis, gastroenteritis, alcohol, burns.

Diagnosis

■

PCV: Best initial test is a CBC showing elevated RBCs/WBCs/platelets (I reticulocyte, i Hb, i Hct, i packed cell volume) with an arterial blood gas (ABC) and EPO level. -1- EPO, normal 0 2 , and Hct >60% (key find­ ing) suggest packed cell volume. Most accurate test is the JAK2 mutation (present in 9 5 % of patients). ■ Relative erythrocytosis also has an i Hct and splenomegaly, but EPO is normal or increased, and 0 2 is often low compared to packed cell volume. Treatment ■

PCV: Target is hematocrit 20% blasts. Myelodysplastic syndromes stem from de novo mutations or from exposures (chemotherapy, radiation).

0-,,. KEY FACT

PCV has low EPO and normal 02 levels. Relative erythrocytosis has normal or increased EPO with low 0 2 levels.

•

A 49-year-old man comes into the clinic com plaining of"tiredness" over the last several months. His past medical history is significant for hypertension, diabetes mel litus, and alcohol overuse. A CBC reveals a low Hb a nd an MCV of 1 1 5 Fl. What is the most likely cause of his anemia?

296

H I G H -YI ELD FACTS I N

TABLE 2.7-1 3.

HEMATOLOGY

Transfusion Reactions

VARIABLE

ALLERGIC REACTION

ANAP HYLACTIC REACTION

FEBRILE N O N H EMOLYTIC REACTION

H EMOLYTIC TRANSFUSION REACTION

Mechanism

Antibody formation

Severe a l lergic reac­

Cytokine formation d u ring

Acute (within the fi rst hour post

agai nst donor plasma

proteins, usually after

receiving plasma-con­

taining prod uct

Type I hypersensitivity

tion in lgA-deficient

individ uals who m ust receive blood prod­

ucts without lgA

storage of blood

Host a ntibodies against

the donor H LA a ntigens and WBCs

Type II hypersensitivity reaction

reaction

transfusion) or delayed (with in

3-1 0 days post transfusion) due to

recipient a ntibodies agai nst donor

eryth rocytes

l ntravascu lar hemolysis (ABO blood g roup i ncompati bility) or extra­

vascu lar hemolysis (host a nti body reaction agai nst donor foreign

a ntigen on donor RBCs)

Type II hypersensitivity reaction Presentation

Promi nent u rtica ria, pruritus, wheezi ng, fever

Dyspnea, broncho­

spasm, respiratory

arrest, hypotension, and shock

Fever, headache, chil ls, flush ing, rigors, and

malaise 1 -6 hours after

transfusion

Fever, hypotension, chil ls, nausea,

flush ing, burning at the IV site, tachy­ ca rdia, tachypnea, fla n k pai n/renal

fai l u re, hemoglobi n u ria (intravascular hemolysis), jaundice (extravascular),

d uri ng or shortly after the transfusion Treatment

Stop transfusion i mmediately, i ndependent of

severity of the reaction,

Give antih istamines.

Give epinephrine is

severe reaction. If mild reaction (urticaria

Should stop the tra nsfusion and give epinephrine

Should treat ana-

phylactic shock as req u i red

Should stop the trans­ fusion and give

aceta mi nophen

Leu kored uction of donor blood

Should stop the transfusion im mediately!

Vigorous IV fl uids and maintain good u rine output

wa nes and there is no evidence of dyspnea, hypotension, or ana­

phylaxis), can resume

transfusion.

0-n KEY FACT Premed ication with aceta m i nophen a n d d i phen hyd ra m i n e is someti mes used to prevent minor tra nsfusion reactions.

The pati ent has megaloblasti c a ne m i a caused by e i t he r a vita m i n B 1 2 or fol ate defi ciency. His h i story of a l cohol dependence strongly s uggests fol ate deficiency, as that is the most common ca use of mega l o b l astic a n e m i a i n people with a h i story of acute/c h ronic a l cohol overuse.

BLOOD TRANSFUSION REACTIONS Transfusions are generally safe but may result in adverse reactions (see Table 2. 7-13). Febrile nonhemolytic and allergic reactions are the most common, occurring in 3 % to 4% of all transfusions.

WHITE BLOOD CELL DISORDERS NEUTROPENIA An absolute neutrophil count (ANC) < 1500 cells/mm 3 , where ANC = (WBC count) x (% bands + % segmented neutrophils). Neutropenia may be caused by a combination of -!. production, migration away from the vascular space, and i destruction or utilization. It may be acquired or intrinsic. The most common causes of neutropenia in adults are infections and drugs. Other

H EMATO LOGY

H I G H -YI E LD FACTS I N

297

common causes include diseases that infiltrate the bone marrow such as leu­ kemias or lymphomas, aplastic anemias, or vitamin B 1 2 /folate deficiencies. Felty syndrome: Neutropenia along with splenomegaly and rheumatoid arthritis

History/PE Patients are at i risk for infection. Severe infections are typical when < 500 cells/mm 3 . ■ Acute neutropenia: Associated with Staphylococcus aureus, Pseudomonas, E coli, Proteus, and Klebsiella sepsis. ■ Chronic and autoimmune neutropenia: Presents with recurrent sinusitis, stomatitis, gingivitis, and perirectal infections rather than sepsis. Some chronic neutropenias are accompanied by splenomegaly (Felty syndrome, Gaucher disease, sarcoidosis). ■ The physician should look for drug or toxin exposure, infection, autoim­ munity, or neoplastic disease. ■

Diagnosis ■ Best initial test: CBC with a peripheral blood smear. Neutropenia is fol­ lowed up with ANC and thrombocytopenia or anemia with bone marrow aspiration and biopsy. ■ Serum immunologic evaluation, antinuclear antibody (ANA) levels, and a workup for collagen vascular disease may be merited. Treatment ■ Infection management: Neutropenic patients cannot mount an effective inflammatory response. ■ It is important to discontinue drugs implicated in neutropenia. Neutropenic fever: In the context of neutropenia, fever is a medical emergency that calls for immediate treatment with broad-spectrum antibiotics such as cefepime that provide Pseudomonas coverage. Sus­ pected fungal infections also call for appropriate treatment. Hematopoietic stem cell factors such as granulocyte colony stimulating factor (G-CSF) (filgrastim) can be used to shorten the duration of neu­ tropenia. Rarely, IVIG and allogeneic BMT may be used. Neutropenic Fever Defined as a single oral temperature of 2:38.3 ° C (2:101 ° F) in a neutropenic patient or a temperature of 2: 38 ° C (2: 100.4 ° F) for 2: 1 hour in a neutropenic patient (ie, an ANC < 500 cells/mm 3 ) . H istory/PE Common in cancer patients undergoing chemotherapy (neutropenic nadir 7-10 days postchemotherapy). Signs of infection may be minimal or absent. Thorough physical examination but no rectal examination in light of the bleeding and infection risk. Diagnosis ■ CBC with differential, serum creatinine, blood urea nitrogen (BUN), and transaminases. Testing of blood, urine, lesion, sputum, and stool cultures. The physician should also consider testing for viruses, fungi, and mycobacteria. ■ X-ray of the chest (CXR) for patients with respiratory symptoms; a CT scan to evaluate for abscesses or other occult infection.

0-,,. KEY FACT

The ANC (cells/mm3) can be obtained by multiplying the total WBCs found on CBC by percentage of polymorphonuclear cells.

298

H I G H -YI ELD FACTS I N

TA B L E 2 . 7 - 1 4 .

HEMATOLOGY

Lymphopenia vs Eosinopenia

LYM PHOPENIA

EOS INOPENIA

Definition

Absol ute lymphocyte count (ALC) < 1 500/m m' ( 100,000/mm3, and drug reaction with eosinophilia and systemic symptoms (DRESS) must be spotted early and should be treated with steroids and discontinuation of offending agents. Nonemergency treatment: Several steroid-sparing agents can be considered. ■

Mepolizumab: A monoclonal antibody directed against IL-5, a growth factor needed for the maturation of eosinophils and their activation ■ Benralizumab: Monoclonal anti-IL-5 receptor alpha (IL-5R) ■ Hydroxyurea: Works by suppressing eosinophilopoiesis ■ Other treatments: lmatinib (features of myeloid disease), interferon alpha, alemtuzumab (anti-CD52 antibody), and JAK inhibitors (tofacitinib and ruxolitinib) Treatment-specific indications: ABPA: Steroids are the mainstay of treatment. Antifungal therapy with itraconazole or voriconazole can be considered in addition to steroids dur­ ing an acute exacerbation. ■ Omalizumab: Humanized monoclonal antibody can be considered in the setting of poorly controlled asthma. Mepolizumab and benralizumab can be considered in severe asthma and hypereosinophilia. ■ EGPA: Steroids are the mainstay of treatment. During remission, steroid­ sparing agents with azathioprine or methotrexate are considered. Mepoli­ zumab and benralizumab can be considered. ■ CEP: Steroids are the mainstay of treatment. ■

0-,,. KEY FACT A characteristic sign for AML s u btype M3 (APL) is the Auer rod seen in the WBC cytoplasm (see Fig. 2.7- 1 4), a lthough Auer rod s ca n be seen i n other AML s u btypes.

LEU KEMIAS Malignant proliferations of hematopoietic cells, categorized by the type of cell involved and their level of differentiation Acute Leukemias

F I G U R E 2.7- 1 4. Auer rod in AML. The red rod-shaped structure (arrow) in the cytoplasm of the myeloblast is pathogno­ monic. (Reproduced with permission from Dr. Peter McPhedran, Ya le Department of Hematology.)

Th is patient presents with sym ptoms of hypoca lcemia fo l l owing m u ltiple b l ood tra n sfu s ions. Blood prod u cts often conta i n citrate, which b i n d s to seru m calci u m, leading to hypocalcem ia, which can cause prolon ged QT inte rvals.

Acute myelogenous and lymphocytic leukemias are clonal disorders of early hematopoietic stem cells (blasts), resulting in unregulated growth and differ­ entiation of WBCs in bone marrow. As the bone marrow becomes replaced by leukemia cells, patients present with features of pancytopenia: anemia ( -l, RBCs), infection (,£ mature WBCs), and hemorrhage ( j, platelets). Acute lymphocytic leukemia (ALL) and acute myelogenous leukemia (AML) are two subtypes that most commonly affect children and adults, respectively. History/PE ■ Rapid onset and progression. Patients present with signs and symptoms of anemia (pallor, fatigue), thrombocytopenia (petechiae, purpura, bleed­ ing), infections (ineffective and immature WBCs), and disseminated intra­ vascular coagulation (DIC, most commonly seen in acute promyelocytic leukemia [APL]). Medullary expansion into the periosteum may lead to bone pain (common in ALL). ■ Physical examination may show hepatosplenomegaly and swollen/bleed­ ing gums from leukemic infiltration and -l, platelets. Leukemic cells also infiltrate the skin and the central nervous system (CNS).

H EMATO LOGY

H I G H -YI E L D FACTS I N

301

Diagnosis Best initial test: CBC with smear showing blast cells. Most accurate test: Bone marrow biopsy with flow cytometry to classify leukemia type. ■ Marrow that is infiltrated with blast cells is consistent with leukemia. In AML, the leukemic cells are myeloblasts; in ALL, they are lymphoblasts. These cells can be distinguished by morphology (see Fig. 2.7-15), cytoge­ netics, and immunophenotyping (see Table 2. 7-16). WBC count can be elevated, but the cells are dysfunctional, and patients may be neutropenic with a history of frequent infection. If the WBC count is very high (eg, > 100,000/mm 3 ) , there is a risk for leukosta­ sis (blasts occluding the microcirculation, leading to pulmonary edema, CNS symptoms, ischemic injury, and DIC). ■ ■

Treatment ■ In general, ALL and AML cases are treated with chemotherapy. Bone marrow transplantation is considered in some patients, especially those with higher risk cytogenetics and those who do not respond appropriately to upfront chemotherapy. ■ All-trans-retinoic acid (ATRA) combined with arsenic trioxide is highly effective in APL. ■ To prevent tumor lysis syndrome (hyperuricemia, hyperkalemia, hypocal­ cemia, renal insufficiency, as blasts are destroyed by chemotherapy), patients should be well hydrated. If WBC counts are i, they may also be started on allopurinol or rasburicase (often used in the pediatric popula­ tion) to decrease serum uric acid as renal protection. Rasburicase is con­ traindicated in G6PD deficiency. ■ Leukostasis syndrome may be treated with hydroxyurea ± leukapheresis to -l, WBC count. ■ Indicators of poor prognosis: ■ ALL: Age < 1 year or > 10 years; an i in WBC count to > 50,000/mm 3 ; presence of the Philadelphia chromosome t(9;22) (associated with B-cell cancer); CNS involvement at diagnosis ■ AML: Age >60 years; elevated LDH; poor-risk or complex karyotype

F I G U R E 2.7-1 s . AML and ALL o n periph­ eral smear. (A) AML. Large, uniform

myeloblasts with round or kidney-shaped nuclei and prominent nucleoli are char­ acteristic. (B) ALL. Peripheral blood smear reveals numerous large, uniform lymphoblasts, which are large cells with a high nuclear-to-cytoplasmic ratio. Some lymphoblasts have visible clefts in their nuclei. (Reproduced with perm ission from Dr. Peter McPhedran, Ya le Department of Hematology.)

Chronic Lym phocytic Leukemia

A malignant, clonal proliferation of mature but functionally incompetent lymphocytes that accumulate in the bone marrow, peripheral blood, lymph nodes, spleen, and liver. All CLL cases involve well-differentiated B lympho­ cytes. Primarily affects older adults (median age 65 years); the male-to-female ratio is 2: 1. History/PE Often asymptomatic; patients present with fatigue, malaise, and infection. Common physical findings are lymphadenopathy, hepatomegaly, and splenomegaly. Diagnosis ■ Best initial test: CBC with differential and smear showing mature lym­ phocytosis (B cells > 500/mm 3 ) and characteristic smudge cells (fragile leukemia cells crushed by the slide). See Figure 2.7-16. ■ Most accurate test: Flow cytometry shows monoclonal B cells with CD5 and CD23 markers.

I A 70-yea r-old woman with a history of hypertension and lymphoma presents with na usea, vomiting, and fever of 2 days' d u ration. She just completed her second cycle of hig h-dose chemothera py. She has a tem perature of 38.5 °C (1 0 1 .3 ° F). Her CXR is unchanged, and her WBC count is 900/ m m 3 with 25% neutrophi ls. After u rine and blood cultures have been sent, what is the next step in management?

302

H I G H -YI ELD FACTS I N

HEMATOLOGY TAB L E 2.7- 1 6.

F I G U R E 2.7- 1 6. CLL with characteristic smudge cells. The numerous small,

mature lymphocytes and smudge cells (arrows; fragile malignant lymphocytes are disrupted during blood smear prepa­ ration) are characteristic. (Reproduced with

The presence of smudge cel l s may ind icate CLL. S m udge cel l s result from the covers l i p cru s h i n g the fragile l e u kemia cells.

VA RIABLE

MYELOBLAST

LYM PHOB LAST

Size

Larger (2-4 times RBC)

Smaller ( 1 .5-3.0 times RBC)

Amount of cytoplasm

More

Less

N u cleoli

Conspicuous

I nconspicuous

G ra n u les

Common, fine

U ncommon, coarse

Auer rods

Present in 50% of cases

Absent

Myeloperoxidase

(f)

8

Terminal

8

(f)

deoxyn ucleotidyltransferase (TdT)

perm ission from Dr. Peter McPhedran, Ya le Depart­ ment of Hematology.)

0-,,. KEY FACT

Myeloblasts vs Lymphoblasts

Granulocytopenia, anemia, and thrombocytopenia are common as leuke­ mic cells infiltrate bone marrow. Abnormal function by the leukemic cells leads to hypogammaglobulinemia. ■ Bone marrow biopsy is rarely required for diagnosis but may provide prog­ nostic information. ■

Treatment ■ CLL often does not require treatment due to its indolent natural history. Disease progression should be monitored every 3 to 6 months. ■ Initial preferred agents are ibrutinib (BTK inhibitor), rituximab (anti­ CD20 mAb therapy), venetoclax (anti-BCL2 therapy) ■ Treatment, however, is palliative and is often withheld until patients are symptomatic: recurrent infection, severe lymphadenopathy or splenomeg­ aly, anemia, or thrombocytopenia (poorest prognosis). ■ Although CLL has a low likelihood of long-term cure, extended disease­ free intervals may be achieved with adequate treatment of symptoms. The clinical stage correlates with expected survival.

CHRONIC MYELOGENOUS LEUKEMIA

The physic i a n s h o u l d a d m it the patient a n d beg i n IV a ntibiotics with a n a nti pseud omonal �-lactam (eg, cefep i me, p i peraci l l i n-tazobacta m, meropenem, i m i pe n em). Fe b ri l e, neutropenic patients who a re o n hig h-dose che mothera py, have a hematolog ic m a l ig n a n cy, or have been neutropenic fo r > 1 4 d ays s h o u l d be adm itted fo r empi ric I V a ntibiotics.

Clonal expansion of myeloid progenitor cells, leading to leukocytosis with excess granulocytes and basophils and sometimes i erythrocytes and platelets as well. To truly be CML, the BCR-ABL translocation must be present. In >95% of patients, this is reflected on conventional cytogenetic analysis by the Philadelphia chromosome t(9;22). CML primarily affects middle-aged patients (median age 50 years).

H istory/PE ■ Many patients are asymptomatic at diagnosis. Typical signs and symptoms are those of anemia. Patients can have splenomegaly with left upper quadrant (LUQ) pain and early satiety. Constitutional symptoms of weight loss, anorexia, fever, and chills may also be seen.

H E MATO LOGY TA B L E 2.7- 1 7 .

H I G H -Y I E L D FACTS I N

303

Leukemoid Reaction vs CML 1 9 LEUKEMO I D REACTION

CML

0-rr KEY FACT

Leucocyte count

50,000/mm'

Often > 1 00,000/mm')

Cause

Severe infection

BCR-ABL fusion

LAP score

High

Low

The presence of smudge cells may indicate CLL. Sm udge cel ls result from the coverslip crushing the fragile leukemia cells.

Neutrophil precursors

More mature (metamyelocytes > myelocytes)

Less mature (metamyelocytes < myelocytes)

Absolute basophilia

Not present

Present

Toxic granulation in neutrophils

Present

Not Present

■ Patients with CML in the absense of treatment go through three disease phases: Chronic: Without treatment, this phase typically lasts 3. 5 to 5 years. Infection and bleeding complications are rare. Accelerated: This phase embodies a transition toward blast crisis, with an i in peripheral and bone marrow blast counts. It should be sus­ pected when the differential shows an abrupt i in basophils and thrombocytopenia (platelet count 100,000/mm 3 at diag­ nosis, sometimes reaching > 500,000/mm 3 . The differential shows granu­ locytes (predominantly neutrophils) in all stages of maturation. Rarely, the WBC count will be so elevated as to cause a hyperviscosity syndrome. ■ CML can be confused clinically with a leukemoid reaction (acute inflam­ matory response to infection with i neutrophils and a left shift). Leuko­ cyte alkaline phosphatase score is low in CML and other hematologic malignancies, and LAP is high in leukemoid reactions. See Table 2.7-17. ■

0-rr KEY FACT The likely diagnosis of leukemia is based on age at presentation: ■ ALL: < 1 3 years (but can present in a ny age group) ■ AML: 1 3 to 40 years (but can present in any age group) ■ CML: 40 to 60 years ■ CLL: >60 yea rs

0-rr KEY FACT Lymphocytosis is a com mon lab finding of CLL (i B cel ls) vs CML, which shows gra n u locytosis (i granulocytes: neutrophils, eosinophils, or basophils).

A 40-year-old woman sees a physician for a 6-month history of weight loss, fevers, and abdominal discomfort. Her WBC count is 56,000/mm 3 . The physician orders a leukocyte alkaline phosphatase (LAP) to distinguish between a leukemoid reaction and a hematologic malignancy. What is the expected result in a leukemoid reaction?

Treatment

■ Chronic: Tyrosine kinase inhibitors (eg, imatinib). Young patients may be candidates for allogeneic stem cell transplantation if a matched sibling donor is available. ■ Blast crisis: Same as that for acute leukemia, or second-generation tyro­ sine kinase inhibitors (eg, dasatinib, nilotinib) plus hematopoietic stem cell transplantation or a clinical trial.

A 41 -year-old man is diagnosed with AML. Fl uorescence in situ hybridization (FISH) analysis reveals that he has APL, M3 subtype (FAB classification). What is the preferred therapy for this subtype of AML7

304

H I G H -YI ELD FACTS I N

H EMATOLOGY

TAB L E 2.7- 1 8.

Non-Hodgkin vs Hodgkin Lymphoma

NON-HODGKI N LYM PHOMA

HODGKIN LYM PHOMA

Many peripheral nodes involved; extranodal, noncontiguous

Single group of localized nodes, spreads contiguously and rarely involves extranodal sites

spread

F I G U R E 2.7- 1 7.

Hairy cell leukemia.

Note the hairlike cytoplasmic projections from neoplastic lymphocytes. Villous lymphoma can also have this appear­ ance. (Reproduced with permission from Dr. Peter

Mainly B cells, sometimes T cells

Reed-Sternberg cells: distinct CDl 5+ and CD30+ B cells

Peak i ncidence 65-75 years of age

Bimodal: young and old

HIV and a utoimmune association

EBV association

McPhedran, Ya le Department of Hematology.)

Adapted with permission from Le T, et al. First Aid for the USMLE Step 1 20 18. New York, NY: McGraw­ Hill; 201 8.

0-,,. KEY FACT

Hairy Cel l Leukemia

l matinib is a selective inhibitor of the BCR-ABL tyrosine kinase, the product of the t(9;22) translocation, or Philadelphia chromosome.

A malignant disorder of well-differentiated B lymphocytes. Hairy cell leuke­ mia (HCL) is a rare disease that accounts for 2% of adult leukemia cases and most commonly affects older males (median age 50-5 5 years). History/PE ■ Typically presents with pancytopenia, bone marrow infiltration, and splenomegaly. ■ Patients complain of weakness, fatigue, petechiae, bruising, infection (especially with atypical mycobacteria such as Mycobacterium avium­ intracellulare), abdominal pain, early satiety, and weight loss. Presentation is similar to that of CLL except that patients rarely have lymphadenopathy. Diagnosis ■ Best initial test: CBC with smear showing pathognomonic "hairy cells" (mononuclear cells with many cytoplasmic projections; see Fig. 2.7-17) that stain with tartrate-resistant acid phosphatase (TRAP). Leukopenia can sometimes be seen as well. Most accurate test: Flow cytometry identifying the "hairy cells"

The LAP score would be i . Hematologic maligna ncies, in contrast, have J, LAP score.

Treatment ■ Best initial treatment: Cladribine. ■ Alternative treatment options: Pentostatin, rituximab, and interferon (IFN)-a. ■ Median survival without treatment is 5 years. If left untreated, most patients will develop progressive pancytopenia and splenomegaly, eventu­ ally requiring therapy.

LYM PHOMAS

APL has a favorable prognosis, beca use it is responsive to ATRA therapy This AML su btype is a lso associated with an i i ncidence of DIC and a chromosomal translocation i nvolvi ng chromosomes 1 5 and 1 7.

Malignant transformations of lymphoid cells residing primarily in lymphoid tissues, especially the lymph nodes. Classically organized into Hodgkin lym­ phoma (HL) and non-Hodgkin lymphoma (NHL). See Table 2. 7-18. Non-Hodgkin Lymphoma

NHL represents a diverse group of mature B- and T-cell neoplasms. Most NHLs (almost 8 5 %) are of B-cell origin. NHL is the most common hemato­ poietic neoplasm and is five times more common than HL.

H EMATO LOGY

TABLE 2.7- 1 9.

H I G H -YI E LD FACTS I N

305

Non-Hodgkin Lymphoma Types

TYPE

OCCURS I N

GEN ETI CS/ETI OLOGY

COMMENTS

Adults (mean age SS

t(l 4;1 8)-translocation of heavy-chain lg ( 1 4) and

I ndolent course or low grade Pai nless waxing and waning adenopathy

B-CELL NEOPLASMS Follicular lymphoma

years) Diffuse large B-cell lymphoma

Usually middle-aged and elderly

BCL-2 (1 8) Mutations in BCL-2, BCL-6, and MYC

Localized disease ( 1 5%) may be cured with radiation therapy I ntermediate grade Most com mon NHL i n adults Often presents with single rapidly growing mass High cure rate with R-CHOP therapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone)

Burkitt lymphoma

Children and adolescents

Mantle cell lymphoma

Older adult Males

t(8; 1 4)-translocation of c-myc (8) and heavy­ chain lg ( 1 4)

t(l 1 ;1 4)-translocation of cyclin Dl ( 1 1 ) and

High grade, "starry sky" appearance on lesion biopsy Jaw lesion in Africa, abdominal lesion in Americas Associated with EBV and t(8;1 4) translocation Aggressive treatment with chemotherapy CDs + Rarest form of NHL

heavy-chain lg ( 1 4), CDS+ Primary CNS lymphoma

Adults

EBV related; associated with HIV/AIDS

An AIDS-defining illness Variable presentation: confusion, memory loss, seizures CNS mass (often single, ring-enhancing lesion on MRI) in immunocom­ promised patients Distinguished from toxoplasmosis via CSF analysis or other lab tests

HELL NEOPLASMS Adult T-cell

Adults

lymphoma

Mycosis fun­ goides/Sezary syndrome

Adults

Caused by HTLV (associ­ ated with IV drug use)

High grade, can progress to ALL Presents with cutaneous lesions Caused by HTLV, associated with IVDA Mycosis fungoides is a T-cell lymphoma of the skin Cutaneous eczema-like lesions and pruritus are common presentations On skin biopsy see lymphoid cells with "cerebriform" n uclei Can progress to Sezary syndrome (T-cell leukemia) with characteristic Sezary cells seen on blood smear

Adapted with permission from Le T et al. First Aid for the USMLE Step 1 2022. New York, NY: McGraw-Hill; 2022.

H istory/PE The median patient age is > 50 years, but NHL may also be found in chil­ dren, who tend to have more aggressive, higher-grade disease. Patient presen­ tation varies with disease (see Table 2.7-19), but often includes painless peripheral lymphadenopathy, "B" symptoms (fevers, night sweats, weight loss), and masses on examination. Diagnosis ■ Best initial test: Excisional lymph node biopsy ■ A C S F exam should be done in patients with HIV, neurologic symptoms, or primary CNS lymphoma.

306

H I G H -YI ELD FACTS I N

H E MATO LOGY

F I G U R E 2.7- 1 8. Hodgkin lymphoma. (A) CXR of a 27-year-old man presenting with several weeks of fevers and night sweats shows bulky bilateral hilar (arrows) and right paratracheal lymph­ adenopathy (arrowhead). (B) Lymph node sampling shows a mixed inflammatory infiltrate and a classic binucleate Reed-Sternberg cell (circle) consistent with Hodgkin lymphoma. (I mage A adapted

0-rr

with permission from Harrison N K. Cough, sarcoidosis and id iopathic pulmonary fibrosis: raw nerves and bad vibrations. Cough.

KEY FACT

The treatment of high-grade N H L may b e complicated by tumor lysis syndrome, in which rapid cel l death releases intracellular contents a nd leads to hyperkalemia, hyperphosphatemia, hyperuricemia, a nd hypocalcemia.

201 3;9(1 ):9. Publ ished 201 3 Mar 6. doi: 1 0. 1 1 86/1 745-9974-9-9. I mage B courtesy of Dr. Andrea Subhawong.)

Treatment ■ Radiation and/or chemotherapy can be used as a therapy. ■ Low-grade indolent NHL treatment is generally palliative. ■ High-grade aggressive NHL treatment is aggressive chemotherapy with a curative approach. A common regimen is rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). Hodg kin Lymphoma

0-rr

KEY FACT

On physical examination, lymph nodes suspicious for malignancy are genera l ly described as firm, fixed, nontender, circumscribed, rubbery, and > 1 cm in diameter. Benign nodes (usually from infection) are genera l ly described as bilateral, 10% monoclonal CD138+ plasma cells. ■ CBC with smear may show rouleaux formation, whereas urine protein electrophoresis may show Bence Jones protein (paraprotein). Gamma gap (total serum protein - serum albumin) is often elevated. ■ M protein alone is insufficient for the diagnosis of MM, as MGUS, CLL, lymphoma, Waldenstrom macroglobulinemia, and amyloidosis can also i M protein.

Albumin

F I G U R E 2.7- 1 9.

a1 a2

�

y

Multiple myeloma.

Serum protein electrophoretic tracing showing M protein spike IgG/A (diag­ nostic of MM). Note that M protein spike IgM indicates Waldenstrom macro­ globulinemia. (Reproduced with perm ission from USMLE-Rx.com.)

0-,r KEY FACT

Chemotherapy often induces nausea in cancer patients and should be managed with ondansetron, a serotonin 5-hydroxytryptamine (5-HT3) receptor antagonist.

Q◊

MNEMONIC

Clinical features of multiple myeloma-

CRAB

Cooselycemia Renal involvement/Recurrent infections Anemia/Amyloidosis Bone lytic lesions/Back pain

0-,r KEY FACT

Similar to MM, MGUS is a monoclonal expansion of plasma cells that is asymptomatic and may eventually lead to multiple myeloma (1 %-2% per year). No "CRAB"findings.

F I G U R E 2.1-20. Multiple myeloma skeletal survey. Characteristic lytic bony lesions of mul­ tiple myeloma involving the tibia and fibula (A) and the skull (B) are seen. (Image A reproduced with permission from Lichtman MA et al. Williams Hematology. 8th ed. New York, NY: McGraw-Hill; 201 0. I mage B reproduced with permission from Kantarjian HM, Wolff RA, Koller CA. MD Anderson Manual ofMedical Oncology. 2nd ed. New York, NY: McGraw-Hill; 201 1 .)

308

H I G H -YI ELD FACTS I N

0-,,. KEY FACT

MM damaging renal tubules can produce adult Fanconi syndrome.

HEMATOLOGY

■

Patients should also be evaluated for anemia, hypercalcemia, and renal failure. Bone lesions are also seen with imaging such as a skeletal survey (see Fig. 2.7-20).

Treatment

0-,,. KEY FACT

As MM is an osteoclastic process, a bone scan, which detects osteoblastic activity, may be 0.

0-,,. KEY FACT

Cryoglobulinemia and cold agglutinins are different disorders caused by lgM antibodies. Cryoglobulinemia is most often seen in hepatitis C virus (HCV) and has systemic signs such as joint pain and renal involvement. Cold agglutinins may cause finger or toe numbness and hemolytic anemia upon cold exposure and are seen with EBV, mycoplasmal infection, and Waldenstrom macroglobulinemia.

Patients who are candidates can be treated with chemotherapy and autolo­ gous bone marrow transplant. Common chemotherapeutic agents are cyclophosphamide, bortezomib, daratumumab (anti-CD138 mAb), dexa­ methasone, and lenalidomide. ■ Patients who are not candidates for bone marrow transplantation can be treated with daratumumab, lenalidmoide, dexamethasone, cyclophospha­ mide and/or bortexomib. ■

WALDENSTROM MACROGLOBULINEMIA A clonal disorder of B cells that leads to malignant monoclonal gammopathy. i levels of IgM result in hyperviscosity syndrome, coagulation abnormalities, cryo­ globulinemia, cold agglutinin disease (leading to AIHA), and amyloidosis. Tis­ sue is infiltrated by IgM and neoplastic plasma cells. A chronic, indolent disease of older adults. History/PE

Presents with nons pecific symptoms: Lethargy, weight loss, and Raynaud phenomenon from cryoglobulinemia. Organomegaly and organ dysfunc­ tion can be present. ■ Neurologic problems ranging from mental status changes to sensorimotor peripheral neuropathy and blurry vision (engorged blood vessels can be noted on eye exam) are also seen. As with MM, MGUS is a precursor to disease. ■

Diagnosis

Most accurate test: Bone marrow biopsy and aspirate. Marrow shows abnormal plasma cells, classically with Dutcher bodies (periodic acid­ Schiff [PAS] EB IgM deposits around the nucleus). Serum and urine pro­ tein electrophoresis and immunofixation are also used. Nonspecific findings include i erythrocyte sedimentation rate (ESR), uric acid, LDH, and alkaline phosphatase. Treatment

Rituximab and chemotherapy for patients with symptomatic disease. Plasma­ pheresis can remove excess immunoglobulin for patients who present with signs or symptoms of hyperviscosity.

AMYLOIDOSIS Extracellular deposition of amyloid protein fibrils resulting from a variety of causes (see Table 2.7-20). Classically a disease of older adults. History/PE ■

Clinical presentation depends on the type, amount, and tissue distribution of amyloid. In the most common forms of systemic amyloidosis, primary (AL) and secondary (M), the major sites of clinically important amyloid deposition are in the kidneys, heart, and liver.

H EMATO LOGY

TABLE 2.7-20.

H I G H -YI E LD FACTS I N

309

Types o f Amyloidosis

AMYLO I D

CAUSE

AL

A plasma cell dyscrasia with deposition of monoclonal light-chain

AA

Deposition of the acute-phase reactant serum amyloid A Associated with chronic inflammatory diseases (eg, rheumatoid arthritis), i nfections, and neoplasms

Dialysis related

Deposition of 132-microglobulin, which accumulates in patients' joints

fragments Associated with multiple myeloma and Waldenstrom macroglobulinemia

(scapulohumeral joint and carpal tunnel) on long-term dialysis. Heritable

Deposition of abnormal gene products (eg, transthyretin, also known as prealbumin). A heterogeneous group of disorders

Senile-systemic

Deposition of otherwise normal transthyretin

AA, Secondary a myloidosis; AL, primary a myloidosis.

■ In some disorders, amyloid deposition is limited to one organ (eg, cerebral amyloid angiopathy in Alzheimer disease). Diagnosis

Most accurate test: Tissue biopsy with Congo red staining showing apple­ green birefringence under polarized light. Treatment

Primary amyloidosis is treated with chemotherapy and/or autologous stem cell transplant. Chemotherapy agents are similar to those used in MM. ■ Secondary amyloidosis is treated by addressing the underlying condition. ■

TRANSPLANT MEDICINE Three types of tissue transplantation are increasingly used to treat diseases: ■ Autologous: Transplantation from the patient to himself or herself ■ Allogeneic: Transplantation from a donor to a genetically different patient ■ Syngeneic: Transplantation between identical twins (from a donor to a genetically identical patient) ■ With allogeneic donation, efforts are made to ABO- and HLA-match the donor and recipient. Despite matching and immunosuppression, however, transplants may be rejected. There are three types of solid organ rejection: hyperacute, acute, and chronic (see Table 2.7-21). ■ Graft-vs-host disease (GVHD) is a complication specific to allogeneic BMT in which donated T cells attack host tissues, especially the skin, liver, and GI tract. It may be acute ( < 100 days posttransplant) or chronic (> 100 days afterward). ■ Minor histocompatibility antigens are thought to be responsible for GVHD, which presents with skin changes, cholestatic liver dysfunc­ tion, obstructive lung disease, or GI problems. ■

A 45-year-old woman presents to the emergency department with fever, chil ls, nausea, vomiting, and severe fl ank pai n . She has a history of m u ltiple u rinary tract i nfections (UTls) and was recently hospita l ized for pyeloneph ritis. Urina lysis (UA) revea ls pyu ria and bacteriu ria. U ltrasound performed in the emergency department shows what appears to be a peri nephric a bscess. What is the next most appropriate step in ma nagement?

An 80-yea r-old man is seen i n clinic after a n i ncidenta l fi nding of elevated lgG on a recent hospital adm ission for pneumonia. He has no signs of kid ney damage, anemia, or bone lesions. The lgG level is 2 1 00 mg/d l, and a su bseq uent bone marrow biopsy shows 3% plasma cel ls. What is the next best step?

31 0

H I G H -YI ELD FACTS I N

HEMATOLOGY TAB L E 2.1-2 1 .

Types of Solid Organ Transplant Rejection

VA RIABLE

HYPE RACUTE

ACUTE

CHRO N I C

Timing after transplant

Within minutes (intraopera­ tively)

Between 5 days and 3 months

Months t o years

Pathogenesis

Preformed

Mixed T-cell and B-cell mediated

Chronic immune

antibodies

Tissue findings

Vascular thrombi; tissue ischemia

response against mismatched HLA class I and class II antigens Laboratory evidence of tissue destruction such as i gamma­

reaction causing fibrosis

Gradual loss of organ function

glutamyl transferase (GGT), alkaline phosphatase, LOH, BUN, or creatinine Prevention

Check ABO compatibility

N/A

N/A

Treatment

Cytotoxic agents

Confi rmation with sampling

No treatment; biopsy to rule

of transplanted tissue; initial treatment with corticosteroids. Additional immunosuppressive

out treatable acute reaction

therapy can include antilym­ phocyte antibodies (OKT3), tacrolimus, or mycophenolate mofetil (MMF)

Patients with m u lti p l e myeloma freq uently h ave re n a l dysfu nction seco n d a ry t o u r i n a ry i m m unog l o b u l i n s (a lso known as Bence Jones protein) that have the a b i l ity to form casts, lead i n g to cast nephropathy.

Patients are treated with high-dose corticosteroids. ■ Typical posttransplant immunosuppression regimens include prednisone; mycophenolate mofetil (MMF); FK506 (tacrolimus) to suppress immune­ mediated rejection; and TMP-SMX, ganciclovir, and fluconazole to pre­ vent subsequent infection in the immunosuppressed host. ■ A variant of GVHD is the graft-vs-leukemia effect, in which leukemia patients who are treated with an allogeneic bone marrow transplant have significantly lower relapse rates of leukemia than those treated with an autologous transplant. This difference is thought to be caused by a recog­ nition of leukemia cells by the donor T cells.

MU LTISYSTEM H EMATOLOGY Th is patient has MG US, as seen by the el evated lgG i n the a bsence of other clin ical abnormal ities or sym ptoms. No treatment is req u i red, but beca use MGUS can progress to MM, this patient should be seen reg ula rly for signs of renal fa i l u re, anem ia, or bone pa i n .

HEMOPHAGOCVTIC LYMPHOHISTIOCVTOSIS Life-threatening state of severe immune system activation due to overactive macrophages and lymphocytes with phagocytosis of blood cells. This com­ monly occur in infants O to 18 months but can be seen in adults of all ages.

HEMATOLOGY

History/PE Presents with fever, rash, hepatosplenomegaly, neurologic disturbances (sei­ zures, mental status changes, ataxia), multiorgan dysfunction, cytopenias, coagulopathies, and hemodynamic instability. Diagnosis ■ Clinical findings as mentioned: i inflammatory markers (ferritin, Scd25, CXCL9)1 ■ Bone marrow aspirate demonstrating hemophagocytosis Treatment Acutely ill: dexamethasone and etoposide. Supportive care (blood transfusion for anemia and thrombocytopenia), antibiotics for infection due to pancytope­ nia, immunosuppressive/cytotoxic therapy (eg, dexamethasone and etoposide).

MASTOCVTOSIS Rare disorder involving excessive release and accumulation of mastocytes in the skin (cutaneous mastocytosis) or other tissues (systemic mastocytosis).

History/PE Physical examination demonstrating skin findings: diffuse red-brown cutane­ ous maculopapular lesions and splenomegaly. Diagnosis ■ Skin or bone marrow biopsy with KIT stain (mast cell specific) with i mast cell concentration, KIT mutation, serum tryptase > 20. ■ Bone marrow biopsy can also show cytopenia due to crowding of the bone marrow by mastocytosis. Treatment Aimed at preventing mast cell degranulation with antihistamines, cromolyn, and antileukotrienes.

LANGERHANS CELL HISTIOCVTOSIS Proliferative disorders of Langerhans cells characterized by histiocyte infiltra­ tion of tissue, commonly due to BRAF V600E mutation.

History/PE ■ Langerhans cell histiocytosis can present with multisystem involvement (eg, skin, lymph nodes, liver, lung, CNS). ■ Skin or oral mucosa: Can present with eczematous rash (resembles Can­ dida infection) and/or brown, purplish papules. ■ Lung: Can present with nonproductive cough, dyspnea, chest pain, constitutional symptoms (eg, fever, weight loss). ■ Bone: Can present with localized bone pain due to osteolytic lesions. ■ CNS: Can manifest as cognitive dysfunction and ataxia. ■ Diabetes insipidus: Present with polyuria, nocturia, and polydipsia due to hypothalamic-pituitary axis (HPA) involvement. May also have associated endocrinopathies (hypogonadism, growth failure, impaired glucose toler­ ance/diabetes mellitus, and thyroid enlargement).

H I G H -YI E LD FACTS I N

31 1

31 2

H I G H -YI ELD FACTS I N

H EMATOLOGY

Diagnosis Bone or skin biopsy, pathology demonstrating collections of histiocytes and Langerhans cells (large ovoid mononuclear cells). Treatment Treatment depends on the organ involved in the disease. Bone disease can be treated with curettage or radiotherapy. Skin involvement can be treated with topical steroids or methotrexate. Lymph node involvement can be treated with vinblastine chemotherapy. Steroids can also be considered when the lungs are involved, along with smoking cessation.

MUSCULOSKELETAL Whole Body COMMON ADULT ORTHOPEDIC INJURIES OTTAWA ANKLE RULES SALTER-HARRIS PEDIATRIC FRACTURE CLASSIFICATION COMMON PERIPHERAL NERVE INJURIES COMPLEX REGIONAL PAIN SYNDROME OSTEOSARCOMA SEPTIC ARTHRITIS OSTEOMYELITIS OSTEOARTHRITIS OSTEOPOROSIS RHEUMATOID ARTHRITIS SERONEGATIVE SPONDYLOA RTHROPATHY POLYMYOSITIS AND DERMATOMYOSITIS TEMPOROMANDIBULAR JOINT DISORDERS MYOFASCIAL PAIN SYNDROME SYSTEMIC SCLEROSIS SYSTEMIC LUPUS ERYTHEMATOSUS SERUM SICKNESS-LIKE REACTION GIANT CELL ARTERITIS TAKA YASU ARTERITIS BEH65 years of age. Risk factors for older patients are radiation, Paget disease, Li-Fraumeni syndrome, and familial retinoblastoma.

History/PE ■ An osteosarcoma manifests with progressive and eventually intractable pain that worsens at night. ■ Constitutional symptoms such as fever, weight loss, and night sweats may be present. ■ Erythema and enlargement over the site of the tumor may be seen.

I A 55-yea r-old m a n with a h i story of prostate cancer presents with low back pa i n (LBP) and bilate ra l leg wea kness. O n exa m i nation, h e is fo u n d to h ave point tenderness on the l u mbar spine and .J, sensati on i n h i s legs. What is t h e best n ext ste p7

324

HIGH-YIELD FACTS IN

TA B L E 2.s-3.

Common Bone Tu mors

MUSCULOSKELETAL

BEN IGN TUMORS DI SEASE

CLASSIC F I N DINGS

KEY HISTO RY F I N D I NGS

T R EATM E N T

Enchondroma

I maging via x-ray of lytic lesion on

Young adult with family history of

Monitoring with serial x-rays

OIiier disease or Maffucci syndrome

hand or foot Giant cell tumor

"Soap bubble" fi nding on x-ray

Young adult, Paget disease of bone

Denosumab

Osteoblastoma

Typically spinal lesions and > 2 cm

Pain that does not improve with

Resection with or without chemo-

NSAI Ds

"blasted in the back"

therapy/radiation, depending on margins

Osteochondroma

Projects out of the growth plate

Young males ( ALT, creatine kinase (CK) should be checked to evaluate for myopathy.

334

HIGH-YIELD FACTS IN

MUSCULOSKELETAL On physical examination, muscles of mastication may be tender, tooth wear from bruxism can be present, and jaw movement may result in click­ ing or even locking that limits jaw opening. ■ The diagnosis of TMJ disorders is primarily based on history and physical examination findings. ■

Treatment

Patient education and self-care measures: Avoidance of triggers and con­ sumption of a soft diet ■ Pharmacology: NSAIDs and corticosteroid injections ■ Dental splints if bruxism is suspected ■

MYOFASCIAL PAIN SYNDROME Myofascial pain syndrome shares some clinical features with fibromyalgia. It is characterized as a regional pain disorder having trigger points within fascia and muscles.

History/PE Patients complain of a deep aching pain, often accompanied by a burning sensation and restricted active motion in the affected area. Physical examination reveals indurated regions known as trigger points. Palpating a trigger point reproduces pain in another location known as the "target zone." Myofascial pain syndrome differs from fibromyalgia, as the latter does not have indurated trigger points and the tender points are located within tissues other than muscles. Treatment

Multidisciplinary patient education, sleep hygiene, low-impact exercise, phys­ ical therapy, non-narcotic pain medications, antidepressants, gabapentin, and muscle relaxants.

(l- MNEMONIC CREST syndrome­ Calcinosis Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangiectasias

SYSTEMIC SCLEROSIS Also called scleroderma; characterized by chronic inflammation leading to progressive tissue fibrosis through excessive deposition of types I and III colla­ gen. Commonly manifests as CREST syndrome (limited form, 80% of cases), but can also occur in a diffuse form (20% of cases) involving the skin and multiple organ systems. Risk factors include female sex and age between 3 5 and 50 years.

History/PE ■ Examination that may reveal symmetric thickening of the skin of the face and/or distal extremities ■ Limited cutaneous: Head, neck, distal upper extremities (earliest areas of involvement) ■ Diffuse cutaneous: Torso, abdomen, proximal upper extremity/ shoulder ■ ■

CREST syndrome: Associated with limited cutaneous type Diffuse form: Leading to gastrointestinal (GI) dysmotility, pulmonary

fibrosis, cor pulmonale, acute renal failure (scleroderma renal crisis), Raynaud phenomenon, and malignant hypertension

MUSCULOSKELETAL ■

HIGH -YIELD FACTS IN

335

Scleroderma renal crisis is characterized by an abrupt onset of hyper­ tension and acute renal failure in patients with scleroderma. Early rec­ ognition is crucial, as scleroderma renal crisis has a high mortality rate if not treated.

Diagnosis ■ ■ ■

Diagnosis and categorization depend on constellation of symptoms. RF and ANA may be EB. Ninety-five percent of patients will have EB ANA. Antiscleroderma (anti-Sci )-70/antitopoisomerase-I is highly specific (>99%) but only has ~ 20% to 4 5 % sensitivity. These antibodies are asso­ ciated with diffuse disease and poor prognosis. ■ RF may also be EB. ■ Anticentromere antibodies are specific for CREST syndrome (see Table 2.8-9). Severe disease may cause microangiopathic hemolytic anemia with schistocytes. Treatment ■

Organ-based treatment that includes frequent monitoring for progressive damage and symptomatic support where necessary ■ Corticosteroids for acute flares (but they increase the patient's risk for renal crisis); methotrexate for limited scleroderma ■ Calcium channel blockers (dihydropyridine) such as amlodipine for Raynaud phenomenon ■ Angiotensin-converting enzyme inhibitors for treatment of renal crisis Compl ications

Mortality is most commonly caused by complications of ILD/pulmonary fibrosis, pulmonary hypertension, and resulting renal or cardiac disease.

0-,r KEY FACT Presence of anti-RNA polymerase I l l antibody in scleroderma is a risk factor for renal crisis.

0,�

MNEMONIC

Criteria for SLE­ DOPAMI NE RASH Discoid rash Oral ulcers Photosensitivity Arthritis (nondeforming) Malar rash (see Fig. 2.8-1 3) Immunologic criteria: anti-dsDNA, anti-Sm proteins, antiphospholipids Neurologic symptoms (lupus cerebritis, seizures) Elevated ESR Renal disease ANA EB Serositis (pleural or pericardia! effusion) Hematologic abnormalities

SYSTEMIC LUPUS ERYTHEMATOSUS A chronic multisystem autoimmune disorder related to antibody-mediated cellular attack and deposition of antigen-antibody complexes in any organ sys­ tem, resulting in variable clinical manifestations and presentations. Black women are at highest risk. SLE usually affects women of childbearing age. H i story/PE

Presents with nonspecific symptoms such as fever, anorexia, weight loss, and symmetric joint pain. Clinical heterogeneity and nonspecific symptoms pres­ ent a diagnostic difficulty in many patients, making SLE a diagnosis of exclusion. Diagnosis

The mnemonic DOPAMINE RASH summarizes the criteria for diagnos­ ing SLE, an adaptation of the American College of Rheumatology (ACR) criteria. Patients must have four of the criteria to consider a diagnosis of SLE (96% sensitive and specific). ■ A EB ANA is highly sensitive (present in 9 5 %-99% of cases). If EB ANA, the physician should test for other antibodies, mainly anti-dsDNA and anti­ Sm. Both are highly specific but not as sensitive (see Table 2.8-9). ■ Drug-induced SLE: EB antihistone antibodies are seen in 100% of cases but are nonspecific. Common medications include hydralazine, procainamide, and isoniazid. ■

F I G U R E 2.8 - 1 3 . Systemic lupus erythematosus (SLE). The malar rash

of SLE is red to purple with a continuous plaque extending across the bridge of the nose and to both cheeks. It typically spares the nasolabial folds. (Reproduced with permission from Bondi EE. Dermatology: Diagnosis and

Therapy. Stamford, CT: Appleton & Lange; 1 99 1 .)

336

HIGH-YIELD FACTS IN

MUSCULOSKELETAL

■

0-rr KEY FACT

■

The lupus anticoagulant (antiphospholipid antibodies) occurs in 5% to 1 0% of SLE cases. lgM or lgG binds proteins in a clinical assay test and prolongs partial thromboplastin time (PTT).

0-rr KEY FACT Libman-Sacks Endocarditis: Noninfectious vegetations often seen on the mitral valve in association with SLE and antiphospholipid syndrome. Note L-S Endocarditis corresponds with S-L-E.

0-rr KEY FACT SLE can cause a false EEl Venereal Disease Research Laboratory (VDRL) test or rapid plasma reagin (RPR) test!

0-rr KEY FACT SLE and RA both affect the MCP and PIP joints; the difference is that in SLE, the joint disease is nondeforming and nonerosive, a pattern cal led Jaccoud arthropathy.

Neonatal SLE: It is associated with EB anti-Ro antibodies transmitted from mother to neonate. The presence of anti-Ro antibodies may cause heart block.

The following may also be seen: ■ Low complement levels during acute flares ■ Antiphospholipid antibodies (antibodies to anticardiolipin, anti13z-glycoprotein, or lupus "anticoagulant"). All cause a hypercoagulable state and may cause thromboembolism and recurrent spontaneous abortion. ■ Raynaud phenomenon ■ Anemia, leukopenia, and/or thrombocytopenia ■ Proteinuria and/or casts

Treatment ■ ■ ■

NSAIDs for mild joint symptoms. Corticosteroids for acute exacerbations. Be wary of Cushing syndrome and possible AYN from chronic use. Corticosteroids, hydroxychloroquine, cyclophosphamide, mycophenolate, and azathioprine for progressive or refractory cases. A few have specific uses: ■ Hydroxychloroquine: Can be used for isolated skin and joint involvement. ■ Cyclophosphamide or mycophenolate: Used for severe cases of lupus nephritis. The physician should be sure to get a renal biopsy for patients with nephritic symptoms.

Com plications

Common causes of morbidity and mortality are important to know for SLE, including renal failure, cardiovascular disease, and infections.

SERUM SICKNESS-LIKE REACTION Self-limited fever, urticaria! rash, arthralgia, lymphadenopathy, and protein­ uria within 1 to 2 weeks of exposure to a 13-lactam antibiotic (eg, penicillin, amoxicillin) or sulfa drug (eg, trimethoprim-sulfamethoxazole). Symptoms resolve upon discontinuation of the drug.

GIANT CELL ARTERITIS Most common of the systemic vasculitides. Formerly called temporal arteritis. Caused by subacute granulomatous inflammation of the large vessels, includ­ ing the aorta, external carotid (especially the temporal branch), and vertebral arteries (see Fig. 2.8-14). Risk factors include polymyalgia rheumatica (affects almost one half of patients), > 50 years of age, and female sex. History/PE ■

Presents with new headache (unilateral or bilateral), scalp pain, temporal tenderness, and jaw claudication Fever, permanent monocular blindness, transient monocular vision loss, aortic aneurysm, weight loss, and myalgias and/or arthralgias (especially of the shoulders and hips) also seen

Diagnosis ■ ■

Best initial test: ESR > 50 mm/hr (influenced by age). Most accurate test: Temporal artery biopsy. The physician should look for thrombosis; necrosis of the media; and the presence of lymphocytes, plasma cells, and giant cells.

MUSCULOSKELETAL

HIGH -YIELD FACTS IN

337

Giant cell a rteritis

Ta kaya su arteritis

t

I

Granulomatous arteritis Large vessel vasculitis

Capi lla ries

Med i u m vessel vasculitis

l

Necrotizing a rte ritis

Po lya rteritis nodosa Kawasaki disease

j L

Small ""'' "�" ""'

Paucity of vascular lg (often with AN CA)

!

_j

I m m u n e complex med iated

Microscopic polyangi itis

SLE

Eosinop h i lic g ra n u lomatosis with polya ngiitis (Chu rg-Stra uss syndrome)

Anti - G B M vasculitis (Good pasture disease)

Gra n u lomatosis with polya ngi itis (Weg ener g ra n u lomatosis)

M ixed cryoglobulinemia

lgA vascu litis (Henoch-Schbnlein purpura)

N ote: Beh�et is an inflammatory vasculitis that can affect blood vessels of any size.

F I G U R E 2.8- 1 4. Classification of vasculitis acoording to vessel size. (Reproduced with perm ission from USM LE-Rx.com.)

Treatment

■ Best initial treatment: High-dose prednisone immediately to prevent ocu­ lar involvement (or involvement of the remaining eye after onset of mon­ ocular blindness). If suspected as contribution to vision loss, give pulse-dose steroids. ■ A temporal artery biopsy to confirm diagnosis. However, do not delay treatment. Conduct a follow-up eye examination. Compl ications

The most feared manifestation is blindness from anterior ischemic optic neu­ ropathy (AION) secondary to occlusion of the posterior ciliary artery, a branch of the ophthalmic artery, which itself is a branch of the internal carotid artery. Central retinal artery (a branch of the internal carotid artery) occlusion is less common; however, if affected it may initially present as tran­ sient vision loss.

TAKAVASU ARTERITIS Large-vessel autoimmune vasculitis affecting the aorta and primary branches. Common in Asian females 50 years of age or secondary to spinal cord tumors (eg, astrocytomas, ependymomas).

Tabes dorsalis

Caused by tertiary syphilis. Results from degeneration (demyelination) of dorsal columns and roots (especially at lumbosacral levels) ➔ impaired sensation and proprioception, sharp fleeting pain in the legs, progressive sensory ataxia (inability to sense or feel the legs) ➔ poor coordination. Associated with Charcot joints (repeated unknowing trauma to joint caused by lack of pain), shooting pain, Argyll Robertson pupils. Examination will demonstrate absence of deep tendon reflex and EB Romberg sign.

�

Vitamin B 1 2 deficiency

Subacute combined degeneration-demyelination of dorsal colu mns, lateral corticospinal tracts, and spinocerebellar tracts; ataxic gait, paresthesia, impaired position and vibration sense.

Cauda equina syndrome

Neurosu rgical emergency. Compression of spinal roots L2 and below, most likely caused by disc herniation, epidural abscess, trauma, or metastatic cancer. Clinical: Saddle anesthesia, loss of bladder and anal sphincter control, and absent knee and ankle jerk reflexes. Diagnosis: MRI and surgical eval uation (in that order).

Comp,cauda equina

Con us medullaris

Similar to cauda eq uina with robust parasympathetic dysfu nction, but with sym metric weakness. UMN signs will be present (eg, Ba binski reflex). Treatment: Same as cauda equina.

Table modified with permission from Le T et al. First Aid for the USMLE Step 1 2022. New York, NY: McGraw-Hill Education; 2022. Illustrations reproduced with permission from USMLE-Rx.com. MRI adapted with permission from Dooley MC, Foroozan R. Optic neuritis. J Ophthalmic Vis Res. 201 0;5(3):1 82-1 87.

NEUROLOGY

HIGH-YIELD FACTS IN

361

ASCENDING Central canal Posterior horn

Dorsal column (pressure, vibration, fine touch, [conscious] proprioception ) lus cUneatus (Upper body, arms )

DESCENDING Lateral corticospinal tract (voluntary motor)

F I G U R E 2.9-8. Cross-section of spinal cord (thoracic) showing arrangement of various tracts. (Reproduced with permission from USM LE-Rx.com.)

H EADAC H ES Headaches can either be primary/idiopathic (eg, migraine, cluster, tension type) or secondary (resulting from underlying disease, such as tumor or intra­ cranial hemorrhage). The differences in these types of headaches are dis­ cussed in Table 2.9-7.

MIGRAINE HEADACHE Recurrent headache disorder with attacks that last 4 to 72 hours. Headache typically unilateral, pulsating, and moderate or severe in intensity and aggra­ vated by routine physical activity. Usually preceded by auras (auditory or visual most common). Affects women more than men; often familial; onset usually by teens to early 20s, but peak age is 30 to 39 years. Linked to changes in vascular tone and neurotransmitters (especially calcitonin gene-related pep­ tide [CGRP]). Triggers: Certain foods (eg, red wine, cheese), fasting, stress, menses, oral contraceptive pills (OCPs), bright light, and disruptions in nor­ mal sleep patterns, among others. Increased stroke risk in women with classic migraine headaches who take OCPs and smoke. TA B L E 2.9-7.

Common Types of Headache

M I G RA I N E H EADACHE

CLUSTER H EADACH E

Pattern

Typica lly unilatera l, usually

Severe pai n behind the eye, usually

Onset

Preceded by aura in classical type

Without preceding aura and 1 5- to

throbbing

u n ilateral, and usually in smokers

1 80-minute headaches that cluster Treatment

Abortive therapy (best initial treat­ ment): Triptans, nonsteroidal anti-inflammatory drugs (NSAI Ds) Next step: Prevention with propran­ olol, topiramate, or nortriptyline

Best initial treatment: 1 00% oxygen for 1 5 minutes Next step: Prevention with verapamil

TENSION-TYPE H EADACHE Bandlike tightening or pressing, typically bilateral Without aura, usually at the end of the day; patient can function during headache Best initial treatment: NSAIDs, acetaminophen Relaxation therapy, ice packs potentially helpful

362

HIGH-YIELD FACTS IN

NEUROLOGY

Diagnosis ■ Based on history and recognition of pattern of headache (usually preceded by an aura and a trigger). Physician should query about past events that may have started the headache at first. Recognition of associated features such as nausea, dizziness, light sensitiv­ ity, smell changes, and disturbed bowel movements. Headaches typically last 4 to 72 hours (untreated or successfully treated), with two of four char­ acteristics (unilateral, pulsating, moderate/severe pain intensity, aggravated by routine physical activity) and association with nausea/vomiting or pho­ tophobia/phonophobia. ■ At least five attacks needed for migraine diagnosis. Treatment ■ Avoidance of known triggers ■ Abortive therapy, including triptans (after over-the-counter [OTC] nonste­ roidal anti-inflammatory drugs [NSAIDs] have failed), alone or in addition to other analgesics such as naproxen. The physician should consider symp­ tomatic treatment for nausea. IV metoclopramide can be used for abortive therapy in the ED. Other treatments include ergots or CGRP antagonists (rimegepant, ubrogepant). ■ Prophylaxis for frequent or severe migraines, including anticonvulsants (eg, valproate, topiramate), tricyclic antidepressants ([TCAs], eg, amitripty­ line), and 13-blockers (propranolol; first-line prevention in pregnant patients). Erenumab blocks CGRP action and has a prophylactic role. Chronic cases may benefit from injection of botulinum toxin. Routine aerobic exercise and good sleep hygiene.

CLUSTER HEADACHE Men are affected more often than women; average age of onset is 25 years. Patients who smoke have a higher risk of this type of headache.

0-n

KEY FACT

If a 25-year-old man wakes up repeatedly during the night with unilateral periorbital pain associated with ipsilateral lacrimation, think cluster headache.

History/PE ■ Presents as a brief, excruciating, unilateral, periorbital headache that lasts from 30 minutes to 3 hours, during which time the patient tends to be extremely restless. Patients do not have auras (vs migraine headache). Tends to occur in clusters of time, affecting the same part of the head at the same time of day (commonly during sleep) during a certain season of the year. ■ Associated autonomic symptoms include ipsilateral lacrimation, conjunctiva! injection, Horner syndrome, and nasal stuffiness. Diagnosis Classic presentations with a history of repeated attacks over an extended period do not need imaging. First episodes require a workup (eg, MRI, carotid artery ultrasound) to exclude structural brain lesion or disorders associ­ ated with Horner syndrome (eg, carotid artery dissection, cavernous sinus infection). Treatment ■ Acute therapy: High-flow 0 2 or sumatriptan injection Prophylactic therapy: Verapamil - first-line treatment, typically pre­ scribed with prednisone (10-day course); alternatives include lithium, val­ proic acid, and topiramate

NEUROLOGY TENSION- TYPE HEADACHE ■

■

■

Hx: Presents with tight, bandlike pain around the head that is triggered by fatigue or stress. Nonspecific symptoms (eg, anxiety, poor concentration, difficulty sleeping) may also be seen. Dx: Must have at least two of the following characteristics: bilateral loca­ tion, pressing/tightening quality, mild to moderate intensity, and not aggra­ vated by routine physical activity. The physician should rule out giant cell arteritis in patients >65 years of age with new headaches (usually accom­ panied by jaw claudication) by obtaining an erythrocyte sedimentation rate (ESR), even if headaches are mild with no associated constitutional or vascular symptoms. Tx: Relaxation, massage, hot baths, and avoidance of exacerbating factors. NSAIDs and acetaminophen are first-line abortive therapy.

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363

0-,,. KEY FACT

If a 30-year-old woman complains of headaches at the end of the day that worsen with stress and improve with relaxation or massage, think tension­ type headache.

SECONDARY HEADACHES The physician should consider secondary headaches when "red flags" (eg, sudden onset, great severity, nocturnal presentation, age >65 years of age, with focal neurologic symptoms, post-head trauma) are present. History/PE ■

■

■ ■

Significant findings include fever or rash (consider meningitis or other infectious causes), jaw claudication (specific for temporal arteritis), or con­ stitutional symptoms such as weight loss (associated with neoplastic, inflammatory, or infectious conditions). Photophobia, nausea, vomiting, and neck stiffness can be associated with aneurysmal subarachnoid hemorrhage (SAH) and meningitis caused by meningeal irritation. Full general and neurologic examinations, including a funduscopic exam­ ination, should occur. Neurologic sequelae: The physician should look for diplopia, altered mental status or associated symptoms (numbness, weakness, dizziness, ataxia, visual disturbances), papilledema, or pupillary abnormalities (par­ tial CN III palsy or Horner syndrome).

0-,,. KEY FACT

Headache red flags-First, worst, sudden onset of most severe headache ever; neurologic sequelae; nocturnal headache; morning vomiting; onset of headache >65 or < 1 0 years of age; focal neurologic signs or symptoms; papilledema; headache subsequent to head trauma.

Diagnosis

Based on the etiology. ■

■

If SAH is suspected: Procure a head CT without contrast. If CT is nega­ tive, perform a lumbar puncture to look for xanthochromia, which, if posi­ tive, supports the diagnosis of SAH. In the emergency room, when SAH is suspected: ■ Check ABCs and Glasgow Coma Scale (GCS). Consider early intuba­ tion if required. ■ Treat seizures with benzodiazepines and start fluid infusion to main­ tain intravascular volume. ■ Obtain a complete blood cell count (CBC) to check for systemic infections. ■ If temporal arteritis is suspected, obtain an ESR. CT of the head is indicated for "red flag" symptoms.

I A 28-year-old woman with no prior med ical history presents with throbbi ng, u n i l atera l headache that is exacerbated by menstruation and m i n i mally rel ieved by acetam i n ophen and lying in a da rk room. She wou ld l ike somethi ng that wou ld provide more symptomatic relief. What abortive therapy s h o u l d the physician prescri be?

364

HIGH-YIELD FACTS IN

NEUROLOGY Treatment ■

■

Directed toward underlying cause of headaches. Some conditions such as SAH may require emergency surgery, whereas cases of temporal arteritis may require steroid use. Analgesics administered for pain relief as search for underlying disorders begins.

TRIGEMINAL NEURALGIA Recurrent, severe, and shocklike shooting or stabbing pain along distributions of the trigeminal nerve (CN V), often triggered by cold, minor trauma, or even chewing food or brushing teeth. The pain is often unilateral. It lasts a maximum of 2 minutes. ■ ■

■ ■

Pathophysiology: Compression of trigeminal nerve root most common cause. Demyelination occurs in multiple sclerosis. Dx: Clinical, with precipitation by innocuous stimulus and not accounted for by another diagnosis. If bilateral, the physician should suspect multiple sclerosis. Ddx: Herpes zoster-induced trigeminal neuropathy, postherpetic neuropathy. Tx: First line with carbamazepine or oxcarbazepine. Surgery (microvascu­ lar decompression and rhizotomy, among various options) is required for refractory cases only.

VASCULAR DISORDERS TRANSIENT ISCHEMIC ATTACK A transient ischemic attack (TIA) is a transient episode of neurologic dysfunc­ tion caused by focal brain, spinal cord, or retinal ischemia, without acute infarction. Most TIAs last 5 to 10 minutes, but can rarely last up to 24 hours. The highest risk for stroke is on the day following the TIA, a risk estimated with the ABCD 2 score. Pathophysiology

Embolism, lacunar, or small-vessel obstruction; low flow in a large vessel. Diagnosis

Based on clinical features of transient attack (transient monocular blindness, aphasia/dysarthria, hemianopia, hemiparesis, or sensory loss). Treatment ■

This patient's sym ptoms a re consistent with m igraine headaches. The p hysi cian should prescr i be a tri pta n fo r a bo rtive therapy.

If symptoms are ongoing and potentially disabling: Emergency evaluation for intravenous (IV) thrombolysis + thrombectomy If symptoms resolve or are ongoing but nondisabling: Evaluation with MRI or neurovascular imaging to rule out stroke Once cause is identified, a plan is implemented to reduce the risk of future stroke by use of medications (antiplatelet therapy), modification of stroke risk factors (eg, hypertension, diabetes), or carotid endarterectomy

N E U ROLOGY HIGH -YIELD FACTS IN STROKE

TA B L E 2.9-8. Modifiable and Nonmodifiable Risk Factors for Stroke

Disruption of cerebral blood flow leads to death of brain cells, resulting in acute onset of focal neurologic deficits. A stroke can be ischemic (80%) or hemorrhagic (20% ). Table 2.9-8 contrasts modifiable and nonmodifiable risk factors associated with stroke. Common etiologies are listed later. ■ Atherosclerosis of the extracranial (carotid and vertebral) and intracranial vessels (internal carotid, cerebral, basilar, and vertebral arteries) ■ Chronic hypertension, hypercholesterolemia, and diabetes - conditions that can damage perforating vessels supplying deep regions of the brain, leading to lacunar infarcts ■ Cardiac or aortic emboli ■ Other causes: Hypercoagulable states, craniocervical dissection, venous sinus thrombosis, sickle cell anemia, vasculitis (eg, giant cell arteritis)

History/PE Symptoms are dependent on the vascular territory affected (see Table 2.9-9). Diagnosis ■ Best initial step: Head CT without contrast (see Fig. 2.9-9A) to differenti­ ate ischemic from hemorrhagic stroke and identify potential candidates for thrombolytic therapy. Ischemic strokes 60

Hypercholesterol-

Eth nicity (Black,

Ca rotid stenosis emia

Smoking

Male sex

Hispanic, Asia n)

Hypertension

(hig hest risk

factor)

Dia betes

Drug use

(cocaine, IV

d rugs)

Common Stroke Symptoms by Vessel Territory

VESSEL TERRITORY

DISTI NGUISHING SYM PTOMS

Middle cerebra l a rtery

Contra latera l paresis and sensory loss in the face and a rm; gaze (eyes deviated towards the lesion); contra­ latera l homonymous hemia nopsia

Nondominant hemisphere-neglect

Dominant hemisphere (90% left side)-a phasia Anterior cerebral artery

Contra latera l paresis and sensory loss i n the leg; cogn itive or personal ity changes; urinary incontinence

Posterior cerebra l a rtery

Contra latera l homonymous hemia nopia with macular spa ring. Alexia without agraphia is seen in left PCA strokes

Weber synd rome: Occlusion of bra nch of posterior cerebra l a rtery lead ing to ipsilatera l CN I l l palsy, contra­ latera l hemipa resis, and parkinsonian rigidity (may not be present if su bsta ntia nigra is spared)

Lacunar

Symptoms a re pure motor, pure sensory, ataxic hemiparesis, dysa rth ria, or clumsy hand

U nderlying pathology incl udes formation of microatheromas and l i pohya l i nosis, com monly secondary to hypertension, dia betes, hyperlipidemia, or smoking

Strokes affecting the thalamus may cause thalamic pain synd rome severa l weeks after the event, with hypersensitive pa in response over the contra latera l affected a rea of the body

Posterior i nferior cerebra l a rtery (PICA) stroke/vertebral (Wal­ len berg synd rome)

Ca rotid a rtery dissection

Loss of pa in and temperature sensation on ipsilateral face and contra latera l body l psi latera l bulbar wea kness/dysa rth ria

l psi latera l Horner syndrome (ptosis, m iosis, and seldom with facial anhidrosis)

Vertigo, nystagm us, hiccups

Sudden headache, neck pain, Horner synd rome

Ca used by oropharyngeal i njury (most com mon)

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NEUROLOGY

F I G U R E 2.9-9.

Acute ischemic stroke. Acute left hemiparesis in a 62-year-old woman. (A) Noncontrast head CT with loss of gray and white matter differentiation, cortical efface­ ment, and asymmetrically decreased size of the right lateral ventricle in a right middle cere­ bral artery (MCA) distribution (indicating mass effect). (B) Diffusion-weighted MRI with reduced diffusion in the same distribution, consistent with an acute infarct. Diffusion-weighted sequences are the most sensitive modality for diagnosing an acute ischemic infarct. (C) Mag­ netic resonance angiography (MRA) shows the cause: an abrupt occlusion of the proximal right MCA (red arrow) Compare with the normal left MCA (yellow arrowhead). Note pronounced midline shift evident on both MRI and MRA. (Reproduced with perm ission from USMLE-Rx.com.)

Check blood glucose first. Labs to draw immediately, in case thrombolytic therapy or intervention may be required, include CBC, prothrombin time (PT)/partial thromboplastin time (PTT), cardiac enzymes and troponin, and blood urea nitrogen (BUN)/creatinine. ■ Diffusion-weighted MRI (follow-up to CT) (see Fig. 2.9-9B) to identify early ischemic changes not detected on CT. ■ Determine underlying cause of stroke: ■ Cardioembolic: ECG; echocardiogram; Holter monitor if initial ECG normal. ■ Thrombotic: Carotid ultrasonography; MRA; CT angiography (CTA); transcranial Doppler; conventional angiography (see Fig. 2.9-9C). ■ Other potential causes that should be worked up if there is a high index of suspicion: Hypercoagulable states; sickle cell disease; vasculitis. ■

-0� MNEMONIC

Contraindications to tPA therapy (major ones italicized)-

SAMPLE STaGES Stroke or head trauma within the last 3 months *Anticoagulation with I N R > 1 .7 or prolonged PTT Ml in past 3 months Prior intracranial hemorrhage Low platelet count ( < 1 00,000/mm') *Elevated BP: Systolic > 1 85 mm Hg or diastolic > 1 1 0 mm Hg Major Surgery in the past 14 days TIA (mild symptoms or rapid improvement of symptoms) within 6 months GI or urinary bleeding in the past 21 days or glucose 400 mg/dl) or t ( CT and shows ventriculomegaly without cortical atrophy = without sulcal enlargement (see Fig. 2.9-20). ■ Best confirmatory test: Improvement of symptoms following LP (opening pressure is normal/slightly T). Treatment

Definitive treatment: Ventriculoperitoneal (VP) shunting. A lumbar drainage trial, over the course of 2 to 7 days, can be used to determine potential response to VP shunting. Predominant gait symptoms indicate a favorable sur­ gical response, while the presence of moderate to severe dementia does not.

CREUTZFELDT - JAKOB DISEASE Although it is the most common prion disease, Creutzfeldt-Jakob disease (CJD) remains an extremely rare form of dementia. CJD occurs when an abnormal protease-resistant prion protein accumulates in the brain, causing spongy degeneration, neuronal loss, and astrocytic proliferation. History/PE

CJD causes a subacute dementia with ataxia and/or startle-induced myo­ clonic jerks, with rapid clinical progression that is noted weeks to months after symptom onset. ■ New-variant CJD (mad cow disease) is a more slowly progressive prion dis­ ease seen in younger people with a history of eating contaminated beef or contaminated human brains (kuru). ■ Iatrogenic CJD can be seen after ophthalmologic or neurosurgical proce­ dures such as corneal transplant due to poor equipment sterilization. ■

Alzheimer d isease is the l i kely d i a g nosis. The key d iffe rences between Alzheimer d i sease and normal a g i n g a re that i n normal a g i n g, patients ca n perform their activities of d a i ly l iving, co m pl a i n of memory loss yet provide deta iled i nformation a bout t h e i r fo rgetfu l n ess, and have a score > 27 on the MMSE.

Diagnosis ■

Definitive diagnosis can be made only by brain biopsy or autopsy, but clin­ ical features, imaging, and lab results are sufficient to make a probable diagnosis.

NEUROLOGY

i 14-3-3 protein in CSF and periodic sharp wave complexes on EEG. Real-time quaking-induced conversion (RT-QuIC) is a new method to detect real-time protein misfolding within CSF samples. ■ Brain MRI may show hyperintensity in the caudate and putamen ("hockey stick sign") or a cortical ribboning pattern. ■ Differential diagnosis includes paraneoplastic syndromes, drug induced, and autoimmune encephalitis. Probable diagnosis of CJD is made when there is a rapid progression of symptoms with no other cause identified by MRI, EEG, or CSF samples, plus corroborating findings as listed in this section.

■ ■

Treatment

Symptomatic management. Most patients die within 1 year of symptom onset as there is no cure and progression cannot be halted.

LEWY BODY DEMENTIA Lewy body dementia is an umbrella term for PD (Lewy body) dementia and dementia with Lewy bodies (DLB) - distinguished by the I-year rule (see Diagnosis). DLB is the second most common cause of dementia (10%-20% of cases) and classically presents in patients age >65 and classically presents in patients age >65 with associated depression and suicidality.

History/PE Classic triad of dementia + parkinsonism + visual "hallewynations." Dementia is characterized by executive and visuospatial dysfunction. Parkinsonism = extrapyramidal motor symptoms = TRAP (Tremor, Rigidity, Akinesia/bradykinesia, Postural instability). ■ Visual hallucinations are well-formed + detailed (animals, people, abstract shapes/colors). ■ Fluctuations are present in cognition, arousal, and attention (range from baseline to brief decline to strokelike). ■ Rapid eye movement (REM) sleep behavior disorder ➔ recurrent dreamrelated vocalizations/motor behaviors.

■ ■

Diag nosis Clinical diagnosis. One-year rule distinguishes PD dementia from DLB: ■ DLB is diagnosed when cognitive impairment occurs with parkinsonism or within < 1 year before parkinsonism. ■ Parkinson disease dementia (POD) is diagnosed when cognitive impair­ ment occurs > 1 year after parkinsonism. ■ Imaging: MRI can show cortical atrophy with limited hippocampal atro­ phy (vs AD). ■ Pathology: PD, POD, and Lewy body dementia all have round, eosino­ philic inclusions of u-synuclein (Lewy bodies) on pathologic examination and represent a disease spectrum. Inclusions are found in cortical cells (Lewy body dementia) and basal ganglia (PD, POD). Treatment

Acetylcholinesterase inhibitors for dementia: Donepezil, galantamine, rivastigmine. Only used in AD + Lewy body dementias ■ Parkinsonian symptoms: Similar to PD treatment with an emphasis on lower doses/slower titration to avoid exacerbating psychotic symptoms and hallucinations via i dopamine

■

HIGH -YIELD FACTS IN

395

396

HIGH-YIELD FACTS IN

NEUROLOGY

MOVEMENT DISORDERS HUNTINGTON DISEASE An autosomal dominant neurodegenerative disorder caused by excessive tri­ nucleotide (CAG) repeats on chromosome 4 during spermatogenesis ➔ abnormally long huntingtin protein ➔ glutamate excitotoxicity via the NMDA-R➔ degeneration of cerebrum + striatum (caudate + putamen) ➔ loss of gamma-aminobutyric acid (GABA)/ACh, unbalanced dopamine (DA) activity ➔ triad of motor (chorea), memory (executive dysfunction), and mood (irritability) symptoms. Huntington disease (HD) is slowly progressive, with only symptomatic treatments, and results in death in 10 to 20 years.

History/PE Typically presents at 30 to 50 years of age with a family history that is indica­ tive of anticipation (worsening/earlier symptoms in successive generations) and/or with relatives being misdiagnosed with psychiatric conditions/suicide. HD is characterized by a triad of motor, memory, and mood symptoms:

Motor: Hyperkinetic early in disease (chorea, hyperreflexia, i urine flow [incontinence], hyperhidrosis) early in disease ➔ hypokinetic late in dis­ ease (rigidity, dystonia, bradykinesia, dysphagia, dysarthria). Chorea refers to random, brief, and irregular involuntary movements of the face, trunk, and limbs that flow between muscle groups. Early in the disease, chorea may manifest as restlessness/fidgetiness with delayed saccades. ■ Memory: Cognitive impairment (executive dysfunction) + dementia. ■ Mood: Symptoms of irritability, depression, and disruption of relationships precede chorea. Other psychiatric symptoms include obsessive-compulsive tendencies, aggression, anxiety, paranoia, delusions, hallucinations, suicid­ ality, and psychosis, which may be mistaken for substance abuse. ■

Diagnosis ■ Clinical features (motor, memory, mood) + family history (look for antici­ pation and misdiagnosed parents/grandparents with psychiatric conditions/ suicide). Test creators may obscure these details in adopted children + genetic confirmation (CAG trinucleotide repeat expansions 2: 36 repeats). ■ Imaging: ■ FOG-PET early in disease shows altered glucose metabolism in stria­ tum (caudate + putamen). ■ CT/MRI is important in diagnosing + assessing severity of disease (mainly). Late disease findings are marked atrophy of the cerebrum and the striatum (caudate + putamen). Atrophy, especially head of the caudate nuclei, ➔ dilation of the lateral ventricles (hydrocephalus ex vacuo; see Fig. 2.9-21). FIGURE 2.9-2 1 . Atrophy of the head of the caudate nuclei in Huntington Disease.

(A) Noncontrast CT in a 54-year-old patient with Huntington disease shows atrophy of the cerebrum and caudate nuclei (arrows) ➔ dilation of the lateral ventricles (hydrocephalus ex vacuo). (B) A normal 54-year-old patient with normal cerebral mass, caudate nuclei (arrows), and lateral ventricles. (Modified with permis­ sion from Ropper AH, Samuels MA. Adams & Victor's Principles ofNeurology, 9th ed. New York, NY: McGraw­ Hill; 2009.)

Treatment ■ No cure exists, and disease progression cannot be halted. Genetic counsel­ ing is recommended. Medical treatment is symptomatic and aimed at motor and associated psychiatric features. Mood disorders such as psychosis/agitation (atypical neuroleptics) and depression/anxiety (first-line treatment: selective serotonin reuptake inhibitors [SSRis] ) should be treated. When approaching treatment, it is important to consider the pres­ ence of chorea that interferes with functioning, as it guides drug selection. ■ Chorea + psychiatric features result from unbalanced DA (loss of GABN ACh) in brain:

NEUROLOGY

HIGH -YIELD FACTS IN

397

Chorea + comorbid depression, agitation :±: psychosis ➔ atypical neu­ roleptics ( ,J, risk of parkinsonism versus typical psychotics; monitor for adverse effects on motor function). ■ Chorea without comorbid depression, agitation, and/or psychosis ➔ inhibit vesicular monoamine transporter 2 (VMAT2) (tetrabenazine or deutetrabenazine) = monoamine depleting agents ➔ inhibit VMAT2 ➔ ,J, DA packing into vesicles ➔ ,J, DA release. ■

PARKINSON DISEASE AND PARKINSONISM A progressive neurodegenerative disorder characterized by the loss of neuromelanin-containing dopaminergic neurons of the substantia nigra pars compacta (part of the basal ganglia). Pathologic examination reveals round, eosinophilic inclusions of alpha-synuclein in degenerating neurons. Typical age at presentation is > 50 years, and risk factors include older age and family history of PD - with smoking being protective. PD is the most common hypo­ kinetic movement disorder and is characterized by a unilateral hand tremor ("pill rolling"), oscillating rigidity ( cogwheel), akinesia/bradykinesia (shuffling gait), and postural instability (frequent falls). PD is an idiopathic condition, but the motor symptoms (parkinsonism) can occur secondary to other neuro­ degenerative disorders, trauma, and medications. Treatment aims to i dopa­ minergic activity (mainly) + ,J, cholinergic activity.

H istory/P E Preclinical stage: Characterized by nonmotor signs of constipation, anosmia, sleep disturbances (REM disorders, restless leg syndrome, excessive sleepi­ ness) and by mood disorders (depression, apathy, anxiety) that predate motor symptoms by up to 20 years.

0-rr

KEY FACT

The gaits of NPH and PD are similar. A significant difference between them, for diagnostic purposes, is the arm swing: ■ PD gait = Red uced arm swing ■ NPH gait = Preservation of arm swing

Clinical: Characterized by motor signs (mainly), autonomic dysfunction, and neuropsychiatric features:

■ Motor signs: Unilateral at onset with possible progression to the contralat­ eral side. Asymmetric (worse on one side). See the TRAPSS mnemonic to recall the following motor signs = parkinsonism: ■ Tremor: Unilateral resting (4-6 Hz) tremor that improves with move­ ment and worsens with distraction. "Pill-rolling" quality arises from 4- to 6-Hz frequency of thumb and finger movements. Rigidity: Increased resistance to passive movement that can be uniform (lead pipe) but is classically cogwheeled, resulting in oscillating ("ratchet-like") joint movements. ■ Akinesia/bradykinesia: Lack of or slowness of movements that can man­ ifest as a narrow-based Shuffling gait with shortened strides + lack of arm swing; Small handwriting (micrographia); hypomimia (masked faces/limited facial expressions); hypophonia (soft speech). ■ Postural instability: Characterized by a flexed axial posture, loss of bal­ ance with stopping/turning, and an increased risk for frequent falls. ■ Autonomic dysfunction: Neurogenic orthostatic hypotension (supine hypertensive and/or orthostatic hypotension), increased sweating, oily skin, constipation, sexual dysfunction, urinary urgency, dysphagia. ■ Neuropsychiatric: Dementia (advanced disease, see "Diagnosis"), execu­ tive/visuospatial dysfunction, visual hallucinations, delusions, sleep disor­ ders (insomnia, restless leg syndrome, REM disorders, daytime sleepiness with sleep attacks, vivid dreams, sleep fragmentation [ night awakenings] ), depression, anxiety, apathy, anosmia.

0,0 MNEMONIC Parkinson disease TRAPSS the body-

Tremor ("pill rolling") Rigidity (cogwheeling) Aki nesia/bradykinesia Postura l instability Shuffling gait Small handwriting (microg raphia)

398

HIGH-YIELD FACTS IN

NEUROLOGY Diagnosis

Symptoms of parkinsonism (TRAPSS) may be present in other conditions (secondary). Clinical suspicion for alternative causes i when there is lack of response to levodopa and and the following findings: a lack of adequate response to levodopa and the following findings: Parkinsonism + ... ■ ■ ■ ■ ■

■ ■

0-n KEY FACT Treatment of Pa rkinson disease is based on i dopami nergic activity + j, choli nergic activity as these circu its become u n ba la nced with the loss of dopa m i nergic neurons in the su bsta ntia nigra pa rs com pacta. ■ Dopa m i ne activates the d i rect pathway and i n h i bits the i n d i rect pathway to promote movement. ■ Acetylchol i n e i n h i bits the d i rect pathway and activates the i n d i rect pathway to i n h i bit movement.

0-n KEY FACT I n a patient with severe Pa rkinson disease who develops psychosis, the patient should not stop PD med ications (this wou ld lead to severe a ki nesia); instead, the patient should use q uetia pine (which is the a nti psychotic with the least amount of movement side effects-it's "q u iet").

Dysautonomia (mainly orthostasis) = multisystem atrophy-Parkinsonian Type (MSA-P) (Shy-Drager syndrome) Cerebellar ataxia = multisystem atrophy-cerebellar type (MSA-C) Oculomotor deficits (vertical gaze palsy) with no tremor = progressive supranuclear palsy (PSP) Impaired cognition, dystonia, sensory deficits, myoclonus = corticobasal degeneration Certain patient drugs (drug-induced .!. dopamine activity): ■ Common causes: Typical > atypical antipsychotics, antiemetics (meto­ clopramide), vesicular dopamine depleters (tetrabenazine, reserpine), CCBs (flunarizine, cinnarizine). ■ Infrequent causes: Atypical antipsychotics (clozapine and quetiapine [it's "quiet" so use in PD psychosis]), mood stabilizer (lithium), antiepi­ leptics (valproic acid, phenytoin), antiarrhythmic (amiodarone), MPTP (used in illegal drugs ➔ destruction of substantia nigra ➔ PD). Liver findings: Consider Wilson disease or hemochromatosis. Dementia = POD or Lewy body dementia (see "Dementia" section).

Treatment

General guidelines: With the loss of dopaminergic neurons in the sub­ stantia nigra pars compacta, cholinergic circuits operate relatively unop­ posed. Treatment aims to restore balance by i dopaminergic activity + l cholinergic activity (see Fig. 2.9-22). General side effects of agents that i DA activity include nausea/vomiting, orthostatic hypotension, sleepi­ ness, dyskinesias, and confusion/hallucinations. Initial treatment, if possi­ ble, involves agents other than levodopa-carbidopa (especially in patients age 50% of patients). ■ MRI of abdomen: Evaluate for renal disease (cysts, angiomyolipoma, and/or carcinoma). ■ EEG: Evaluate for seizure activity. ■ ■

Treatment

Treatment should be based on symptoms (eg, cosmetic surgery for facial sebaceous adenomas). ■ Seizures should be treated. ■ When standard epileptic medications fail, mTOR inhibitors (eg, evero­ limus) can help reduce seizures. ■ If infantile spasms are present, first-line treatment is with vigabatrin, which enhances GABA transmission. Adrenocorticotropic hormone (ACTH) can be used as an alternative/adjunctive. ■ Surgical intervention may be indicated in the setting of i ICP from obstructive hydrocephalus or for seizures associated with an epileptogenic focus or severe developmental delay. ■

STURGE - WEBER SYNDROME Sturge-Weber syndrome is due to a somatic mosaic mutation in the GNAQ gene, and therefore is not an inheritable disease. H i story/PE

Facial capillary malformation (port wine stain) is usually in a VI distribution. This finding in isolation does not qualify for the diagnosis of Sturge-Weber syndrome since GNAQ mutations constitute a spectrum disorder with isolated port wine stain as the mildest form. Twenty percent have a leptomeningeal capillary-venous malformation of the brain and eye that is adjacent to the port wine stain and are thus diagnosed as having Sturge-Weber syndrome. The brain parenchyma may be atrophic and contain calcific deposits. Seizures, strokelike episodes, hemiparesis, and mental retardation commonly occur. Diagnosis

Preferred diagnostic test is brain MRI with contrast. The image may reveal intracranial calcifications that resemble a "tramline."

NEUROLOGY Treatment ■ Photothermolysis can be used for treatment of port wine stains. ■ Low-dose aspirin beginning in infancy may decrease the frequency and duration of seizures. ■ Anticonvulsant medications are first line for management of seizures.

VON HIPPLE - LINDAU SYNDROME Von Hipple-Lindau (VHL) syndrome is an inherited autosomal dominant condition characterized by the presence of benign and malignant tumors in many organ systems, including the retina, CNS, pancreas, kidneys, and adre­ nals. It is caused by a mutation in the VHL tumor suppressor gene. Patients with type 1 VHL have a very low risk of developing pheochromocytoma, but patients with type II VHL (caused by missense mutations) have a higher risk for pheochromocytoma.

History/PE ■ Hemangioblastomas are the hallmark tumors of VHL. When they occur in the CNS, especially the cerebellum and spinal cord, they can cause ataxia and headaches. Retinal hemangioblastomas may cause vision loss, and they are often identified on fundoscopy. ■ One half of all cases of pheochromocytomas under the age of 18 years are due to VHL, and they result in episodes of palpitations, hypertension, and sweating. ■ Other VHL-associated tumors include: ■ Renal cell carcinoma (clear cell type) and renal cysts ■ Serous cystadenoma of the pancreas ■ Endolymphatic sac tumor ■ Papillary cystadenoma of epididymis and broad ligament Epididymal cysts Diagnosis ■ Genetic testing for individuals with manifestations of VHL disease or family history ■ MRI of the brain and spinal cord to evaluate for lesions ■ Levels of catecholamines to evaluate for presence of pheochromocytoma ■ Complete audiologic exam to evaluate for hearing loss (can be caused by endolymphatic sac tumors) Treatment ■ Routine surveillance of the abdomen can check for renal cell carcinoma, pheochromocytomas, and pancreatic tumors. Annual retinal exams should be done to evaluate for retinal hemangioblastomas. ■ For patients with symptomatic hemangioblastomas, surgical intervention should be considered. If unresectable or if there are contraindications to surgery, treatment with belzutifan (hypoxia-inducible factor [HIF]-2 alpha inhibitor) can be considered. In VHL disease, levels of HIF- lA and HIF-2A transcription factors are elevated, causing an increase in physiologic angio­ genesis via upregulation of vascular endothelial growth factor (VEGF) and platelet-derived growth factor (PDGF). ■ Patients with renal cell carcinoma > 3 cm should undergo surgical inter­ vention rather than routine surveillance.

HIGH -YIELD FACTS IN

409

41 0

HIGH-YIELD FACTS IN

NEUROLOGY ATAXIA - TELANGIECTASIA Autosomal recessive disease caused by a mutation in the ATM gene, which encodes DNA repair enzymes. Leads to aberrant repair of double-stranded DNA breaks, causing cell death (eg, Purkinje cells in the cerebellum are par­ ticularly susceptible).

History/PE Cerebellar ataxia is often the first presenting symptom in infancy or early childhood. Patients may also present with ocular apraxia (inability to con­ trol purposeful eye movements), cognitive impairment, extrapyramidal symptoms, and peripheral neuropathy. ■ Telangiectasias are common, most often in the face, eyes, and ears. ■ Immune deficiency (especially lgA) predisposes patients to recurrent sino­ pulmonary infections, which may lead to interstitial lung disease. ■ Up to one fourth of patients will develop a malignancy, most often lym­ phomas or leukemias. ■

Diagnosis Usually clinical, but genetic testing supportive of diagnosis Low lgA ■ Elevated levels of a-fetoprotein (AFP) often seen ■ Imaging studies involving ionizing radiation to be minimized ■

Treatment No specific treatment exists for progressive ataxia and cerebellar degenera­ tion. Infections should be treated with antibiotics. Prophylactic antibiotics can be considered.

OTHER NEUROLOGIC DISEASES NUTRITIONAL DEFICIENCIES Table 2.9-22 describes neurologic syndromes commonly associated with nutri­ tional deficiencies.

IDIOPATHIC INTRACRANIAL HYPERTENSION A disorder characterized by raised ICP with neurologic manifestations such as headaches, papilledema (most common), and vision loss, without any indica­ tion toward another cause of raised ICP on neuroimaging. The disease has no proven etiology (idiopathic), but is believed to be either due to decreased resorption and/or increased production of CSF. Other proposed mechanisms include vascular (transverse sinus stenosis), hormonal (aldosterone excess), and cellular (increased outflow resistance to CSF). Risk factors include female sex, obesity, use of tetracyclines, and hypervitaminosis A.

History/PE Patients usually have bilateral, frontal (retrobulbar) headache, more pro­ nounced in the mornings and on Valsalva; transient visual loss; diplopia; pulsatile tinnitus. ■ Sixth nerve (abducens) palsy may be a nonlocalizing sign of raised ICP.

NEUROLOGY TA B L E 2 . 9 - 2 2 .

HIGH -YIELD FACTS IN

41 1

Neurologic Syndromes Associated With N utritional Deficiencies

VITAM I N

SYNDROME

SIGNS/SYMPTOMS

CLASSIC PATIENTS

TREATMENT

Thiamine (vitamin B , )

Wernicke

Classic triad consisting of

Patients with acute/

Reversible almost

encephalopathy

encephalopathy, ophthal­ moplegia, and ataxia

chronic alcohol overuse (toxin effect on cerebellar Purkinje fibers), hyperemesis, starvation, renal dialysis, AIDS. Usually brought on or exacerbated by

immediately with thi­ amine administration Always give thiamine before glucose

high-dose glucose administration Korsakoff dementia

Above plus a nterograde and retrograde amnesia, horizontal nystagmus, and confabulations

Cobalamin (vitamin B 1 2)' Subacute combined degeneration

Peripheral neuropathy; subacute combined degeneration (SCD)

Tabes dorsalis

Folate"

Folate deficiency

Same as above; usually occurs in Wernicke syn­ drome that was treated

I rreversible

too late or inadequately

Gradual, progressive gait disorder due to profound loss of proprioception Symmetric paresthesia,

Patients with pernicious anemia; strict vegetarians; status postgastric or ileal resection; ileal

stocking-glove sensory neu ropathy, leg stiffness, spasticity, paraplegia, bowel

disease (eg, Crohn); patients with acute/ chronic alcohol

and bladder dysfunction, sore tongue, and dementia Associated with elevated meth­ ylmalonic acid levels

overuse or others with malnutrition

Irritability; personality changes without the neurologic

Patients with acute/ chronic alcohol overuse

B 1 2 injections or large oral doses

Reversible if corrected early

symptoms of SCD 'Associated with i homocysteine and an i risk for vascular events. (I mages reproduced with permission from USMLE-Rx.com.)

Diagnosis ■ Neuroimaging (MRI with venography) is preferred to rule out other causes of raised ICP. CT done when MRI contraindicated. ■ CSF opening pressure >25 cm H2 O in adults is suggestive of diagnosis. ■ Ophthalmologic evaluation is mandatory to rule out papilledema (see Ophthalmology section). Treatment ■ Goals of therapy include alleviation of symptoms and preservation of VISIOn .

■ Best first treatment when patients have minimal symptoms is to address risk factors/comorbidities and advise weight loss. ■ Carbonic anhydrase inhibitor, acetazolamide, is best first medication to use in these patients.

A 6 1 -yea r-old ma n presents to the emergency d epartment with a 6-month h istory of prog ressively worse n i n g n a u sea and mo r n i n g headache. T h e patient i s i n no apparent acute d i stress. What is the p referred diag nostic stu dy?

41 2

HIGH-YIELD FACTS IN

NEUROLOGY ■ When medical therapy fails, the physician should consider topiramate and serial LPs (not preferred, as CSF reforms within 6 hours) prior to surgery. ■ Surgery is advised when patients have worsening visual defects despite medical therapy, and presence of visual acuity loss is secondary to papill­ edema. Performed procedures include optic nerve sheath fenestration (ONSF) and CSF-shunting procedures.

OPHTHALMOLOGY VISUAL FIELD DEFECTS Figure 2.9-26 illustrates common visual field defects and the anatomic areas with which they are associated.

HORDEOLUM Painful abscess of the eyelid, also called a stye, caused by inflammation or infection of a sebaceous gland located around the eyelash (Zeis and Moll) or inner lid margin (meibomian). S aureus is the most common pathogen. Hor­ deolum presents with localized swelling and erythema. Initial management consists of warm, moist compresses, topical antibiotic/steroids ointment, and lid massage several times a day to facilitate drainage. Refractory hordeola may be referred to ophthalmologists for incision and drainage.

CHALAZION Granulomatous lesion of the eyelid that presents with localized swelling and a nontender, hard nodule. Differentiated from a hordeolum by a comparative lack of erythema and pain. Management includes warm compress, topical antibiotic/steroid combination, and possible incision and drainage (refractory lesions).

0 Macula 0

Central scotoma

(macular degeneration)

Optic nerve

Left anopia

@ Optic chiasm

Bitemporal hemianopia

0 Optic tract

Right homonymous hemianopia

0 Meyer loop

Right u pper quadrantanopia

(left parietal lesion)

0 Dorsal optic radiation This patient presents with symptoms that a re concern i n g for i n c reased ICP As he i s not in acute d i stress, MRI i s the preferred study beca use it i s bette r fo r vis u a l izing soft tissue and vascu l a rity.

0

Right lower quadrantanopia

(left temporal lesion) /if PCA infarct) Visual cortex

Right hemianopia with macular sparing

L eye

R eye

0 0

eo

() ()-----­ () ()

�­ c- c----n �

() ()�

F I G U R E 2.9-26. CNS lesions cause characteristic visual field defects. (Reprod uced with permission from USMLE-Rx.com.)

NEUROLOGY

F I G U R E 2.9-27.

Blepharitis. (Left image

HIGH-YIELD FACTS IN

41 3

reprod uced with perm ission from USM LE-Rx.com. Right image reprod uced

with permission from Va nzzi n i Zago V, Alcantara Castro M, Nara njo Tackman R. Su pport of the laboratory i n the diagnosis of

fu ngal ocular infections. int J Inf/am. 201 2;20 1 2:643 1 04. doi: 1 0. 1 1 5 5/201 2/643 1 04.)

BLEPHARITIS A chronic inflammatory condition of the eyelash follicles (anterior) or Meibo­ mian glands (posterior) of the eyelid, producing lid irritation that may lead to erythema and pruritis of the eyelid or conjunctiva (Fig. 2.9-27), excessive tear production, eyelash crusting, and light sensitivity. Initial treatment is with warm compresses, lid hygiene, and lubricating drops to prevent corneal dry­ ness. Patients who do not respond to initial therapy are treated with topical erythromycin. Oral antibiotics (doxycycline, azithromycin) may be used for refractory cases. Lid hygiene, including washing, and avoidance of irritants such as smoke, eye makeup, or contact lenses is emphasized to prevent future symptoms.

CORNEAL ABRASION

F I G U R E 2.9-28. (A) Corneal abrasion; (B) Wood's lamp examination with corneal abrasion. (Image A reprod uced with perm ission from Gilani CJ, Ya ng A, Yonkers M, Boysen-Osborn M. Differentiati ng u rgent and emergent causes of acute red eye for the emergency physician. West

J Emerg Med. 2 0 1 7;1 8[3]:509-5 1 7. doi:1 0.5 8 1 1 /

westjem.20 1 6. 1 2.3 1 798. Image B reprod uced with perm ission from USM LE-Rx.com.)

Damage to the corneal epithelium (Fig. 2.9-28A) caused by trauma (includ­ ing from foreign bodies or contact lenses). Patients report severe eye pain, for­ eign body sensation, and photophobia. Diagnosis is made by fluorescein examination, where there is increased uptake of fluorescein stain in the area of abrasion under a Wood's lamp (Fig. 2.9-28B). Treatment consists of remov­ ing any retained foreign body, pain control, and topical antibiotic therapy. Fluoroquinolone drops are used for contact lens wearers due to risk of Pseudomonas infection, whereas erythromycin ointment or polymyxin/ trimethoprim drops are suitable for patients who do not wear contact lenses.

PRESEPTAL (PERIORBITAL) CELLULITIS Infection of the eyelid and surrounding soft tissue anterior to the orbital sep­ tum. Presents with painful erythema and edema of the eyelid. S aureus is the most common pathogen. Patients with no skin trauma are treated with amoxicillin-clavulanic acid, whereas patients with skin trauma are covered for methicillin-resistant S aureus (MRSA) with the addition of trimethoprim­ sulfamethoxazole or clindamycin. This condition is differentiated from the more serious orbital (postseptal) cellulitis by lack of ophthalmoplegia, pain with eye movement, or proptosis. CT of the orbit can be used to differentiate the site of infection in unclear cases.

ORBITAL (POSTSEPTAL) CELLULITIS Infection of the ocular muscles or orbital fat, sparing the globe, that may lead to vision loss or death if left untreated. Commonly caused by direct spread of

•

A 24-year-old fe male with a body mass i n dex (BMI) of 33 presents with a 3-week h i sto ry of consta nt retro­ orbital headache with occasional n a u sea, vomiting, and t i n n itus. S h e a l so d eveloped new-onset d i p l opia 2 h o u rs before presentation. On p hysica l exa m i n ation, s h e is noted to h ave papi l l edema. What is the m ost l i kely d i a g nosis, a n d what a re the risk factors for t h i s co n d ition 7

41 4

HIGH-YIELD FACTS IN

NEUROLOGY

infection from the paranasal sinuses. Other causes include trauma, ophthal­ mic surgeries, dental infections, and dacryocystitis. It is usually caused by staphylococci (including MRSA), streptococci, and H inf/.uenzae (in chil­ dren). In patients with diabetes and immunocompromise, Mucor and Rhizo­ pus must be included in the differential diagnosis.

F I G U R E 2.9-30.

Conjunctivitis. (Reproduced

with permission from USMLE-Rx.com.)

0-,,. KEY FACT Orbital cel l u l itis ca n be disti nguished from preseptal cel l u l itis by the presence of the following clinical features: restricted or painfu l eye movements, J, visual acuity, diplopia, proptosis, and presence of a relative afferent pupillary defect.

0-,,. KEY FACT Neisseria conj u n ctivitis is a n ocu lar emergency often req uiring i n patient pa rentera l a nti biotic thera py.

History/PE Presents with acute-onset fever, proptosis, -1- extraocular movement, ocular pain, and -1- visual acuity. The physician should look for a history of ocular trauma/surgery or sinusitis. Palatal or nasal mucosa! ulceration with coexisting maxillary and/or ethmoid sinusitis suggests mucormycosis or Rhizopus. Diagnosis Mostly clinical. Best initial test: Blood and tissue fluid culture. CT scan or MRI of the orbit and sinuses to rule out orbital abscess and intracranial involvement. Treatment ■ The patient should be admitted and administered broad-spectrum IV anti­ biotics such as vancomycin and ceftriaxone. Metronidazole is added for intracranial extension; case requires an ophthalmologic/ear, nose, throat (ENT) consult. ■ Abscess formation or a worsening condition may necessitate surgery. ■ Patients with diabetes or immunocompromise should be treated with amphotericin B and surgical debridement (often associated with cavern­ ous sinus thrombosis) if Mucor or Rhizopus is diagnosed.

CONJUNCTIVITIS Inflammation of the conjunctiva producing a red-appearing eye (Fig. 2.9-30). It is most often allergic, viral, or bacterial but can also be fungal, parasitic, or chemical. It is essential to differentiate potentially vision-threatening infec­ tious etiologies from allergic or other causes of conjunctivitis and to identify other vision-threatening conditions that may mimic conjunctivitis. Table 2.9-23 lists the common etiologies of infectious conjunctivitis. ■

F I G U R E 2.9-3 1 .

■

Dacryocystitis. (Reproduced

with permission from USMLE-Rx.com.)

G iven this patient's symptoms and risk factors, she proba bly has pseudotumor cerebri, a lso known as id iopathic i ntracra n i a l hyperte n sion ( I I H ) . In I I H, sym ptom s a re sugg estive of a b ra i n t u m o r, and CSF pressu re w i l l b e i ; however, n e u roimaging wi l l be norma l. Obes ity, tetracyc l i ne, growth hormone, a n d excess vita m i n A a re risk factors for the d i sease. Treatment is with acetazo l a m i de, topi ra mate, optic nerve fe n estration, and ventricul operitoneal s h u nt if needed.

■

Allergic conjunctivitis: Bilateral predominantly itchy eyes. Treat with antihistamine (olopatadine) eye drops. Bacterial conjunctivitis: Purulent discharge. Treat with erythromycin ointment or fluoroquinolone drops in contact lens wearers. Viral conjunctivitis: Watery discharge, often bilateral. Self-limiting. Treat with antihistamine (olopatadine) or lubricating eye drops for symptomatic relief.

ACUTE DACRYOCYSTITIS Infection of the lacrimal sac, usually by Staphylococcus or Streptococcus spp. May progress to orbital cellulitis or meningitis if left untreated. Congenital nasolacrimal duct stenosis and dacryocystocele (nasolacrimal duct cyst) are predisposing conditions. ■ Hx/PE: Presentation with purulent eye discharge, as well as inflammation overlying the lacrimal system and medial eyelids (medial canthal region) (Fig. 2.9-31). Less commonly fever and i WBC. ■ Dx: Clinical diagnosis. ■ Tx: If mild, oral anti-staphylococcus/streptococcus agents. If serious, immediate empiric broad-spectrum antibiotics to prevent orbital cellulitis.

NEUROLOGY TA B L E 2.9-23. Bacterial

HIGH -YIELD FACTS IN

Common Causes of Infectious Conjunctivitis PATHOGEN

CHARACTERISTICS

DIAGNOSIS

TREATME NT

Sta phylococci,

Foreign body sensation, purulent discharge

G ra m sta i n

Anti biotic d rops/ointment

streptococci,

Neisseria gonorrhoeae

and cultu re

if severe

Haemophi/us, Pseudomonas, Moraxel/a An emergency-corneal involvement ca n lead to perforation and blind ness

G ra m sta i n

showi ng g ra m e

i ntracellular

d i plococci

Chlamydia trachomatis A-C

Recu rrent epithelial keratitis in child hood, trichiasis,

Trachoma (global)

Leading cause of preventa ble blind ness world wide

Chlamydia trachomatis D-K

tracing/treat sexua l pa rtners.

Ophthalmia neonatorum: Mucopurulent conjuncti-

Ophthalmia

Ophthalmia neonatoru m:

Ad ult incl usion conjunctivitis: Chronic conjunctivitis

Giemsa

vitis i n neonates

neonatorum:

stain, PCR

Ad u lt inclu-

conjuncti-

vitis: PCR common)

in patient treatment if

com plicated. Contact

Azith romycin (si ngle ora l

often), PCR

sion

Adenovi rus (most

IM or IV ceftriaxone;

Clinical (most

corneal sca rri ng, and entropion

with mild mucopurulent discharge i n adu lts

Viral

41 5

Copious watery discharge, severe ocu lar i rritation,

prea u ricu lar lym phadenopathy, pharyngitis (with

dose) i n mass treatment

Erythromycin (fi rst l ine),

azith romycin

Ad ult inclusion conju nctivitis: Azith romycin (single oral dose),

screening for gonorrhea and contact tracing

Contagious; self-limited

adenovirus, "pharyngoconjunctiva l fever")

Occu rs in epidemics

HERPES SIMPLEX KERATITIS Viral infection of the cornea. Common cause of visual impairment in the United States.

■ Hx/PE: Presents with pain, blurred vision, tearing, and redness. The virus remains dormant along the trigeminal nerve and may reactivate during periods of immunocompromise such as illness. ■ Dx: Typically clinical. However, dendritic ulcers (Fig. 2.9-32) are charac­ teristic. Epithelial scrapings show multinucleated giant cells. PCR of con­ junctival/corneal swab for confirmation. ■ Tx: Oral or topical antiviral therapy.

CONTACT LENS KERATITIS Medical emergency, often caused by Pseudomonas infection. ■ Hx/PE: Typically presents with painful, red eye and opacification and ulceration of the cornea. Most patients report a history of improper con­ tact lens hygiene, such as reusing lens solution or showering/swimming with lenses in. Severe disease can cause corneal perforation and perma­ nent vision loss.

F I G U R E 2.9-32. Herpes simplex kerati­ tis with dendritic ulcer. (Reproduced with permission from Gilani CJ, Yang A, Yonkers M, Boysen­ Osborn M. Differentiating u rgent and emergent causes of acute red eye for the emergency physician. WestJ Emerg Med. 201 7; 1 8[3]:509-5 1 7. doi:1 0.581 1 / westjem.20 1 6. 1 2.3 1 798.)

41 6

HIGH-YIELD FACTS IN

NEUROLOGY

■ ■

Dx: Clinical. Corneal scrape and bacterial culture are usually performed for antibiotic sensitivity. Tx: Immediate removal of the contact lens and administration of topical broad-spectrum antibiotics.

UVEITIS Infectious or noninfectious inflammation of the uvea - specific name is based on location within the affected eye. Anterior uveitis: iritis; posterior uveitis: choroiditis and/or retinitis. Associated with systemic inflammatory disorders (eg, sarcoidosis, inflammatory bowel disease, rheumatoid arthritis, juvenile idiopathic arthritis, human leukocyte antigen (HLA)-B27-associated conditions) F I G U R E 2.9-33. Uveitis. (Reproduced with perm ission from Barut K, RzayevT, Canpolat N, et al. Acute granulomatous iridocyclitis in a child with tubuloi nterstitial nephritis and uveitis syndrome. J Ophthalmic lnflamm Infect. 201 5;5:3. Published 201 5 Feb 1 3. doi: 1 0. 1 1 86/s l 2348-0 1 5-0035-2.

History/PE ■ Patients with anterior uveitis may have pain and redness of the eye - often worse at the limbus ( corneal-scleral junction) (Fig. 2. 9-3 3 ). They may have hypopyon (accumulation of pus in anterior chamber). ■ Posterior uveitis presents with visual changes such as decreased visual acuity or floaters in the visual fields. Diagnosis Diagnosis is made by slitlamp examination showing leukocytes in the anterior chamber, vitreous humor, or other signs of inflammation of the retina. Treatment Treatment of infectious uveitis is targeted toward the specific pathogen. ■ Noninfectious uveitis is treated with topical glucocorticoids. Oral or intra­ ocular glucocorticoids are used for refractory cases.

REFRACTIVE ERRORS Common cause of impaired vision, correctable with glasses, contact lenses, or refractive surgery. Hyperopia, also called "farsightedness." Eye too short for refractive power of cornea and lens ➔ light focused behind retina. Correct with convex (converging) lenses. ■ Myopia, also called "nearsightedness." Eye too long for refractive power of cornea and lens ➔ light focused in front of retina. Correct with concave (diverging) lens. ■ Asti gmatism, irregular curvature of cornea ➔ different refractive power at different axes. Correct with cylindrical lens. ■

PRESBYOPIA Aging-related impaired accommodation (focusing on near objects), primarily due to .J, lens elasticity, changes in lens curvature, and .J, strength of the ciliary muscle. Patients often need "reading glasses" (magnifiers).

NEUROLOGY

HIGH -YIELD FACTS IN

GLAUCOMA

41 7

0-,,. KEY FACT

Closed-angle glaucoma headaches are triggered by darkness (caused by pupillary dilation). Migraine headaches are triggered by bright lights.

In the eye, aqueous humor produced by the ciliary body behind the iris trav­ els through the pupil into the anterior chamber and is then drained back into the bloodstream via the trabecular meshwork in the angle of the anterior chamber. ■ Any process that disrupts this natural flow (Fig. 2.9-34) can i intraocular pressure (IOP), damaging the optic nerve head and causing visual field deficits. Glaucoma is the result of such damage to the nerve. ■ Open-angle glaucoma is much more common in the United States than closed-angle glaucoma (see Fig. 2.9-3 5 and Table 2.9-24).

0-,,. KEY FACT

Open-angle glaucoma generally occurs bilaterally, but closed-angle glaucoma usually presents unilaterally.

CATARACT ■ Lens opacification resulting in obstructed passage of light (Fig. 2.9-36). Associated with diabetes, HTN, advanced age, and exposure to radiation or corticosteroids. Congenital risk factors: classic galactosemia, galactokinase deficiency, trisomies (13, 18, 21), TORCH infections (eg, rubella), Marfan syndrome, Alport syndrome, myotonic dystrophy, and neurofibromatosis 2. ■ Hx/PE: Presents with reduced visual acuity, especially at night, and loss of the red reflex. ■ Tx: Surgical lens removal and replacement.

Open-angle glaucoma

Angle-closure glaucoma

- Normal aqueous flow

- Abnormal aqueous flow t trabecular outflow resistance

/

�

.- )C_::

F I G U R E 2.9-34.

K

Obstruction of drainage pathways by the iris

/ '"'n"" '""

t

Aqueous humor flow in glaucoma. (Reproduced with permission from USM LE-Rx.com.)

I

F I G U R E 2.9-35. Findings in open- and closed-angle glaucoma. (A) Normal optic disc. (B) Cupping (increased cup-to-disc ratio) seen in open-angle glaucoma. (Reproduced with perm ission from EyeRou nds.)

A 39-year-old man presents to the emergency department with severe eye pain, photophobia, and a persistent sensation that something is in his eye. The physician is suspicious of a cornea l a brasion. What a re the risk factors for this con d ition, and what d iagnostic test ca n you do to confirm your suspicion ?

41 8 TA B L E 2.9-24.

Etiology

HIGH-YIELD FACTS IN

NEUROLOGY

Closed-Angle vs Open-Angle Glaucoma

CLOSED-ANGLE GLAUCOMA

OPEN-ANGLE GLAUCOMA

Disrupted flow of aq ueous h u mor into the a nterior chamber

Diseased trabecular meshwork resu lts i n J, d rainage,

results i n i pressu re i n the posterior chamber, leading to

angle closure that J, d rai nage. This ophthalmic emergency can

leading to g rad ual i in IOP and progressive vision loss.

cause blind ness. Although this disease usually presents unilat­ era l ly, it often affects both eyes sequential ly.

Risk factors

Fa m i ly history, older age (55-70 years), Asian descent, hyper­

opia, prolonged pupillary dilation (prolonged time in a dark

a rea, stress, medications), a nterior uveitis, and lens dislocation. History/PE

Extreme, sudden-onset eye pain, blu rred vision, headache, nausea, and vomiti ng.

A hard, red eye is seen; the pupil is di lated and non reactive to lig ht.

Diagnosis

Black eth nicity, dia betes, and myopia.

Usually asymptomatic u nti l late i n the clin ical cou rse,

when it can cause g radual loss of peri pheral vision if left u ntreated.

Cupping of the optic nerve head is seen on fund uscopic exam.

Best initial test: Ocular tonometry (to measure IOP) ca n quickly provide additional information.

Best diagnostic test: Assessing the corneal angle with gonios­ copy is the gold standard.

Treatment

Age >40 yea rs, family history of open-angle glaucoma,

Treatment to J, IOP uses a com bination of topical and systemic therapy as follows:

■ Eye d rops (timolol, apraclonidi ne, pilocarpine, dorzolamide, latanoprost)

■ Systemic medications (ora l or IV acetazolamide, or IV mannitol)

■ Laser peri pheral i ridotomy, which creates a hole in

the peripheral i ris, is cu rative, and can be performed prophylactica lly

The patient should not have a ny medications that ca use

Best initial test: Tonometry.

Best diagnostic test: Ophtha l moscopic visualization of the optic nerve head (enlarged cu p-to-disc ratio) and

visual field testing.

Topical medications such as prostaglandins (latanoprost) and cholinergic agonists (pilocarpine) i aq ueous

outflow, whereas 13-blockers (ti molol, betaxolol) and ca rbonic a n hydrase i n h ibitors (acetazolamide) J,

aq ueous prod uction. Al pha-agonists (apraclonidine, brimonidi ne) work via both mechanisms.

Prostaglandins or 13-blockers a re chosen as fi rst-line

thera py. If medication fai ls, laser trabeculoplasty or a trabeculectomy can improve aqueous d rai nage.

pupi llary di lation (atropine or other medications with a nticho­ li nergic activity such as a ntih istam ines and a ntidepressa nts).

AGE - RELATED MACULAR DEGENERATION

R i s k facto rs fo r corneal abrasion i nc l u d e tra u ma, foreign body, a n d contact l e n s use. U s e a pen l i g h t to document pupi l l a ry fu nction and the presence/a bsence of a fo re i g n body. A fl uoresce i n exa m i nation can be diag nostic a n d wi l l s h ow a corneal sta i n i n g d efect. Pa i n persists eve n after departu re of fore i g n body d u e to damage a n d sens itization of cornea l ne rves.

Age-related macular degeneration (AMD) is more common among White individuals, females, people who smoke, and those with a family history.

History/PE ■ Presents with painless loss of central vision. Early signs include distortion of straight lines (metamorphopsia) and loss of other aspects of fine visual acuity.

NEUROLOGY

HIGH -YIELD FACTS IN

41 9

■ Atrophic ("dry") macular degeneration: Responsible for 80% of cases. It causes gradual vision loss. ■ Exudative or neovascular ("wet") macular degeneration: Much less common but associated with more rapid and severe vision loss.

Diagnosis ■ Atrophic ("d ry ") macular degeneration: Fundoscopy reveals drusen (accumulation of white/yellow extracellular material) and/or pigmentary changes (Fig. 2.9-3 7). ■ Exudative or neovascular ("wet") macular degeneration: Hemorrhage and subretinal fluid are present.

F I G U R E 2.9-36.

Cataract.

(Reproduced with

perm ission from Roshan M, Kabekkod u SP, Vijaya PH, et al. Analysis of mitochondrial DNA variations i n I n d i a n patients with congenital cataract. Mot Vis. 201 2;1 8: 1 8 1 - 1 93.)

Treatment Atrophic AMO: No treatment is currently available, although a combina­ tion of vitamins (vitamin C, vitamin E, beta-carotene, and zinc) has been found to slow disease progression. The physician should be cautious about giving high doses of vitamin E and beta-carotene to patients who smoke, as there is an association of i mortality rate from lung cancer in people taking high doses of these supplements. ■ Exudative AMO: lntravitreal injections of VEGF inhibitors have been shown to improve vision (aflibercept, ranibizumab, bevacizumab) or slow visual loss in patients with exudative AMO.

RETINAL VASCULAR OCCLUSION Occurs in older adult patients and is strongly related to cardiovascular disease (see Table 2.9-25).

OPTIC N EURITIS

F I G U R E 2.9-37. Macular degeneration with evidence of drusen and fibrosis on fundoscopic exam. (Reprodu ced with permis­ sion from USM LE-Rx.com.)

Inflammation of the optic nerve that is commonly associated with MS. Other optic neuropathies may be caused by ischemia, infection/postinfection (men­ ingitis, encephalitis), vasculitis, connective tissue disease (systemic lupus ery­ thematosus [SLE], Sjogren), genetic conditions, and drugs (eg, methanol, ethambutol, linezolid, infliximab).

History/PE/Diagnosis ■ Optic neuritis produces painful vision loss that is often unilateral. It causes relative afferent pupillary defect (Marcus-Gunn pupil). ■ Fundoscopy may show disc swelling that looks similar to papilledema; however, fundoscopy is normal in two thirds of patients because the inflammation is posterior to the optic nerve head. ■ The diagnosis is clinical, based on history and physical exam. Diagnosis may be confirmed with MRI of the orbits.

0-n

KEY FACT

For optic neu ritis, g ive IV, not ora l, corticosteroids.

420

HIGH-YIELD FACTS IN

NEUROLOGY

Treatment Methylprednisolone IV for 3 days, followed by oral steroids. Oral steroids alone may increase risk of recurrence. Most patients gradually recover vision even without treatment, although IV methylprednisolone increases the rate of recovery.

TA B L E 2.9-25.

History/PE

Central Reti nal Artery vs Central Retinal Vein Occlusion

CENTRAL RETINAL ARTERY OCCLUSION

CENTRAL RETI NAL VEIN OCCLUSION

Presents with sudden, painless, unilateral vision loss (eg, scotoma), as well as relative afferent pupillary defect Patients present with a cherry-red spot on the fovea (blue arrow in image), retinal swelling (whitish a ppearance to the nerve

Presents with rapid, painless vision loss of variable severity; associated with hypertension A swollen optic disc with hemorrhages, venous stasis retinal hemorrhages, cotton-wool spots, and macu lar

fi ber layer), and retinal arteries that may appear bloodless Transient occlusion is com parable to transient ischemic attack and is known as amaurosis fugax

Etiology

Atherosclerosis is the biggest risk factor; other risk factors include cardioembolism (atrial fibrillation, endocarditis), giant cell a rteritis, Behcet syndrome, and sickle cell disease

edema may be seen on funduscopic exam

Hypertension is the biggest risk factor; other risk factors include conditions that cause hypercoagulability (factor V Leiden, proteins C and S deficiency, antith rombin [AT] Il l deficiency, a nti-phospholipid antibody syndrome, monoclonal gammopathies)

Workup

Diagnosis is based on history and fu ndoscopy The next best tests to order after diagnosis are a carotid duplex + echocardiogram to evaluate for atherosclerotic disease and

Hypercoagulability testing should be performed if there is a suggestive history or other causes have been excluded

cardioembolic sou rces Giant cell arteritis is ruled out using erythrocyte sedimentation rate and (-reactive protein i n patients over 50 years who have no visualized retinal emboli Treatment

Ocular massage with high-flow oxygen administration; intra­

Laser photocoagulation for ischemic central retinal vein

arterial thrombolysis within 8 hours Other treatments target the specific etiology and may include anticoagulation, carotid endarterectomy, and secondary pre­

occlusion (CRVO) to reduce risk of neovascularization VEGF inhibitors treat macular edema (cause of vision loss i n CRVO); optimization of risk factor treatment ([HTN, diabetes mellitus [DM]) should occur

vention of vascular events such as stroke or Ml

I mage A reproduced with permission from USMLE-Rx.com. I mage B reproduced with permission from Alasil T, Rauser ME. l ntravitreal bevacizumab in the treatment of neovascu lar glaucoma secondary to central retinal vein occlusion: a case report. Cases J. 2009;2:1 76.

NEUROLOGY

HIGH-YIELD FACTS IN

42 1

RETINAL DETACHMENT Separation of the retina from the underlying retinal pigment epithelium (RPE) and choroid. May be caused by retinal tears, proliferative diabetic reti­ nopathy, or trauma. High myopia (>6 diopters of correction required) is a risk factor.

History/PE ■ Sudden-onset painless monocular vision loss is often associated with float­ ers and flashes of light. ■ Visual field defect may resemble a curtain ascending or descending over the eye. ■ Retinal detachment and other trauma may cause vitreous hemorrhage, where the vitreous humor fills with blood. This complication presents with black spots and cobweb shapes in the visual field. Diagnosis ■ All patients should have visual field and acuity testing. ■ The detached retina can be seen floating in the posterior chamber on ultrasound. ■ Definitive diagnosis requires a dilated funduscopic exam, where the detached retina can be directly visualized (Fig. 2.9-38). Treatment Tamponading of the retina via surgery (vitrectomy or scleral buckle).

DIABETIC RETINOPATHY Retinal damage due to chronic hyperglycemia. Two types: Nonproliferative - Damaged capillaries leak blood ➔ lipids and fluid seep into retina causing hemorrhages and macular edema. Treatment: Blood sugar and blood pressure control. ■ Proliferative - Chronic hypoxia results in new blood vessel formation with resultant traction on retina ➔ retinal detachment. Treatment: Anti-VEGF injections, peripheral retinal photocoagulation, or surgery.

■

HYPERTENSIVE RETINOPATHY Chronic uncontrolled HTN ➔ endothelial disruption ➔ fibrinoid necro­ sis ➔ retinal damage. ■ Flame-shaped retinal hemorrhages, arteriovenous nicking, microaneu­ rysms, macular star, cotton-wool spots (blue arrow in Fig. 2.9-43). Presence of papilledema in a hypertensive patient is indicative of malignant hyper­ tension and requires immediate lowering of BP. ■ Associated with risk of stroke, CAD, and kidney disease. ■

RETINITIS PIGMENTOSA Inherited progressive dystrophy of RPE and photoreceptors. It may be associ­ ated with abetalipoproteinemia. Early findings include nyctalopia (night blindness) and peripheral vision loss. Fundoscopy may show triad of optic disc

F I G U R E 2.9-38. Retinal detachment on fundoscopic examination. (Reproduced with permission from USMLE-Rx.com.)

0-,,. KEY FACT

Retinal detachment presents with sudden-onset flashing lights and blurred vision. Patients typically describe a curtain coming down over their eye. Ophthalmoscopy shows a gray, elevated retina.

422

HIGH-YIELD FACTS IN

NEUROLOGY pallor, retinal vessel attenuation, and retinal pigmentation with bone spicule­ shaped deposits (Fig. 2.9-39).

PAPILLEDEMA Optic disc swelling with blurred disc margins and dilated/tortuous retinal veins (usually bilateral) due to i ICP (eg, secondary to mass effect). Enlarged blind spot and elevated optic disc (Fig. 2.9-40) with blurred margins.

LEUKOCORIA F I G U R E 2.9-39. Retinitis pigmentosa on fundoscopic exam. (Modified with permission from USMLE-Rx.com.)

Loss (whitening) of the red reflex (Fig. 2.9-41). Important causes in children include retinoblastoma and congenital cataract.

RELATIVE AF FERENT PUPILLARY DEFECT Also called Marcus-Gunn pupil. When the light shines into a normal eye, constriction of the ipsilateral (direct reflex) and contralateral pupil (consen­ sual reflex) is observed. When the light is then swung to the affected eye (side of optic nerve defect), both pupils dilate instead of constricting due to impaired conduction of a light signal along the injured optic nerve. It is asso­ ciated with optic neuritis (eg, MS) and optic neuropathies (eg, giant cell arteritis).

HORNER SYNDROME F I G U R E 2.9-40.

Papilledema on fundo­

scopic exam. (Mod ified with perm ission from USMLE-Rx.com.)

Sympathetic denervation of face causing: ■ ■

Ptosis (slight drooping of eyelid: superior tarsal muscle) Anhidrosis (absence of sweating) and flushing of affected side of face Miosis (pupil constriction)

Associated with lesions along the sympathetic chain (Fig. 2.9-42): First neuron: Pontine hemorrhage, lateral medullary syndrome, spinal cord lesion above T l (eg, Brown-Sequard syndrome, late-stage syringomyelia) ■ Second neuron: Stellate ganglion compression by Pancoast tumor ■ Third neuron: Carotid dissection (painful); anhidrosis usually absent ■

F I G U R E 2.9-4 1 . Leukocoria with loss of red reflex. (Reproduced with perm ission from Aerts I, Lumbroso-Le Rouic L, Gauthier-Vi lla rs M, et al. Retinoblastoma. Orphanet J Rare Dis. 2006; 1 :3 1 . Pub­ l ished 2006 Aug 25. doi: 1 0. 1 1 86/1 750-1 1 72- 1 -3 1 .)

Long ciliary nerve To sweat glands of forehead

,>--/--=K �To smoolh muscle of eyelid To pupillary dilator

-==,�LL,.----< To sweat glands of face Third neuron

flrSl neuron

Superior cervical ganglion

Synapse in lateral horn Spmal cord

1 1

1

Second neuron

F I G U R E 2.9-42. Sympathetic chain anat­ omy in Horner syndrome. (Reproduced with perm ission from USMLE-Rx.com.)

ORBITAL BLOWOUT FRACTURE Orbital floor fracture; usually caused by direct trauma to eyeball or intraor­ bital rim. i risk of inferior rectus muscle and/or orbital fat entrapment. It may lead to infraorbital nerve injury.

CAVERNOUS SINUS SYNDROME Cavernous sinus syndrome presents with variable ophthalmoplegia (CNs III and VI involved most frequently), .J, corneal sensation, Horner syndrome, and decreased maxillary sensation. Secondary to pituitary tumor mass effect, carotid-cavernous fistula, or cavernous sinus thrombosis related to infection

NEUROLOGY

HIGH -YIELD FACTS IN

423

INTRANUCLEAR OPHTHALMOPLEGIA MLF: Pair of tracts that interconnect CN VI and CN III nuclei (Fig. 2.9-43). Coordinates both eyes to move in same horizontal direc­ tion. When patient looks left, the left nucleus of CN VI fires, which con­ tracts the left lateral rectus and stimulates the contralateral (right) nucleus of CN III via the right MLF to contract the right medial rectus. Lesions may be unilateral or bilateral (latter classically seen in MS, stroke). ■ Lesion in MLF: Internuclear ophthalmoplegia (INO), a conjugate hori­ zontal gaze palsy. Lack of communication occurs so that when CN VI nucleus activates ipsilateral lateral rectus, the contralateral CN III nucleus does not stimulate the medial rectus to contract. Abducting eye displays nystagmus (CN VI overfires to stimulate CN III). Convergence is normal. ■ Directional term (eg, right INO, left INO) refers to the eye that is unable to adduct. ■

OTOLOGY OTITIS MEDIA Acute Otitis Media H istory/PE Pain in the ear, redness, decreased hearing, and fever. On otoscopy, bulging, hypomobile, and decreased light reflex of tympanic membrane are seen.

Lateral rectus

l

-J

7'1

R

Medial ·'J. rectus ----'1

CN VI

Midbrain

Right MLF - Paramedian pontlne reticular formation (PPRF)

Pons

-- Abducens (CN VI) nucleus

'

-------------➔--

Medulla

-----------

I

F I G U R E 2.9-43. Neural pathways associ­ ated with intranuclear ophthalmople­ gia. (Reproduced with permission from USM LE-Rx. com.)

O◊

MNEMONIC

/NO-

lpsilatera l adduction failure, Nystagmus Opposite.

Diagnosis Best initial test: Usually empirically treated with antibiotics. Consider tympa­ nocentesis with culture in case of recurrent infections. Treatment Initial best treatment: Amoxicillin for 10 days. If no response, the patient has been treated with amoxicillin recently, or symptoms return within 1 month, amoxicillin-clavulanate should be used.

Chronic Suppurative Otitis Med ia Similar to acute otitis media except: ■ ■ ■

Continuous suppuration for >6 weeks with tympanic membrane perforation Prolonged duration of symptoms - drainage, pain, and hearing loss Absence of systemic symptoms such as fever

Otitis Media With Effusion/Serous Otitis Media/G l ue Ear Fluid in the middle ear for > 3 months without an infection.

I

History/PE ■ May be asymptomatic. Painless pressure in the ear post-acute otitis media in children. ■ Otoscopy shows air-fluid level behind the bulging tympanic membrane and hypomobility.

A 55-year-old m a n presents to the emergency department with sudden­ on set headache and a d i l ated p u p i l i n h i s rig ht eye that is non reactive to l i g ht. His rig ht eye i s h a rd to the tou c h . What is the most l i kely d iag nos is, an d what medicati ons should be avo i ded i n this patient?

424

HIGH-YIELD FACTS IN

NEUROLOGY Treatment Monitoring for 3 months. If hearing impairment is resulting in speech impair­ ment, tymapnostomy tubes may be considered.

OTITIS EXTERNA Inflammation of the external audito ry canal, also known as "swimmer's ear." Pseudomonas and Staphylococcus are the most common etiologic agents. Both grow in the presence of excess moisture. Necrotizing (malignant) otitis externa (Pseudomonas in >95%) can lead to osteomyelitis of the skull base.

History/PE Presents with pain, pruritus, hearing loss, and possible purulent discharge. Examination reveals pain with movement of the tragus/pinna (unlike otitis media), an edematous and erythematous ear canal, and granulation tissue if necrotizing type. Cranial nerve palsies may be visible in the necrotizing type. See the Pediatrics chapter for a discussion of otitis media. Diagnosis A clinical diagnosis. A culture for severe or refractory cases. CT scan if the patient appears toxic. Treatment ■ Best initial treatment: Aural toilet (clean and dry ear [ eg, using wick and/ or astringents]). Mild otitis externa can be treated with topical acetic acid (7-10) days. ■ Moderate otitis externa is treated with topical antibiotics (ofloxacin or cip­ rofloxacin) and steroid ear drops. ■ Older adults with diabetes and individuals who are immunocompromised are at risk for necrotizing otitis externa and may require IV antibiotics (usually a fluoroquinolone or fourth-generation cephalosporin). Consider wick placement if occlusion of canal. ■ To prevent, patients should avoid getting moisture in the ear and should thoroughly dry their ears after swimming,

MALIGNANT OTITIS EXTERNA H istory/PE ■ Severe infection of the external auditory meatus occurs with ear pain and suppurative drainage. Granulation tissue is seen on otoscopy. Malignant otitis externa is commonly seen in patients with poorly controlled diabetes and individuals who are immunosuppressed. Complications include cranial osteomyelitis and facial nerve palsy. ■ The cause is Pseudomonas infection.

This patie nt's prese ntati on is consistent with cl osed-a n g l e g l aucoma. T h e physician shou l d avoid p u p i l -d i lati ng medicati ons su ch as atropi ne, w h i c h w i l l i IOP a n d preve nt d ra i nage o f a q u e o u s h u m o r.

Treatment ■ Drug of choice - IV ciprofloxacin. Other IV antibiotics against Pseudomo­ nas can also be used (eg, ceftazidime, cefepime, quinolones, aztreonam, piperacillin/tazobactam). ■ Surgical debridement and biopsy are required with failure to respond to antibiotics.

NEUROLOGY SENSORINEURAL HEARING LOSS Etiology

■ Meniere disease - hearing loss, tinnitus, vertigo, and aural fullness due to excessive endolymph in the inner ear. ■ Presbycusis - bilateral, symmetric age-related hearing loss to high­ frequency sounds due to degenerative changes in inner ear and CN VIII. Hearing is worse in noisy environments. ■ Drug-induced hearing loss ■ Noise-induced hearing loss ■ Acoustic neuroma Diagnosis

See Figure 2.9-44. ■ Rinne test: Reduced bilaterally ■ Weber test: Localized to normal ear ■ Pure tone audiometry: Increased auditory threshold in au and bone conduction ■ Other tests include impendence audiometry and otoscopy for visual assessment

Tympanic membrane

CN VII Semicircular canals .......--- CN VIII

Auricle ( pinna)

Vestibule ..==��-+----- Oval window External auditory canal

----- Cochlea

-------- Pharyngotympanic ( eustachian) tube

Conductive hearin g loss Rinne: abnormal Weber: affected ear

F I G U R E 2.9-44.

J

SensoriNeu ral a in g loss , �.__h_e_r_ ____---: , ,

1 1

, ,

Rinne: Normal Weber: N ormal ear

External (outer) ear

Middle ear

Internal (inner) ear

Osteoma Cerumen i m paction Otitis

C holesteatoma Tym panic membrane perforation Otosclerosis

Presbycusis* Meniere's d isease Acoustic neuroma Ototoxic drugs Cochlear nerve damage*

,

Diagnosing hearing loss. (Reproduced with permission from USMLE-Rx.com.)

HIG H -YIELD FACTS IN

425

426

HIGH-YIELD FACTS IN

NEUROLOGY CONDUCTIVE HEARING LOSS Etiology

Otitis media: Acute, chronic suppurative, serous Wax impaction Cholesteatoma: Growth of keratinizing squamous epithelium, which erodes ear ossicles; on otoscopy, discrete white plaque is seen on the tym­ panic membrane ■ Otosclerosis: Sclerotic changes in the ear ossicles ■ ■ ■

Diagnosis

See Figure 2.9-44. Rinne test: Bone > air ■ Weber test: Localized to affected ear ■ Pure tone audiometry: Increased auditory threshold in air conduction only

OBSTETRICS The Basics of Preg nancy

428

Diagnosis of Preg nancy

428

BETA-HUMAN (HORIONIC GONADOTROPIN (!3-H(G) ULTRASONOGRAPHY

428 428

Physiologic Changes in Preg n a n cy

429

Prenata l Ca re and Diag nostic Testi ng

430

GROUP B STREPTOCOCCUS TESTING AND TREATMENT PRENATAL DIAGNOSTIC TESTING

43 1 43 1

Teratology

434

Congen ita l I nfections

435

Abortion

436

SPONTANEOUS ABORTION ELECTIVE TERMINATION OF PREGNANCY (OMPLICATIONS OF ABORTION

Maternal Com p l ications of Preg n a n cy HYPEREMESIS GRAVIDARUM DIABETES HYPERTENSIVE DISEASE OF PREGNANCY URINARY TRACT INFECTIONS AND ASYMPTOMATIC BACTERIURIA

436 438 438

438

438 439 44 1

444

INTRAHEPATIC (HOLESTASIS

444

ACUTE FATTY LIVER IN PREGNANCY

445

Obstetric Co m p l ications of Preg n a n cy ECTOPIC PREGNANCY GESTATIONAL TROPHOBLASTIC DISEASE ANTEPARTUM HEMORRHAGE AND ABNORMAL PLACENTATION MULTIPLE GESTATION FETAL GROWTH RESTRICTION FETAL MACROSOMIA POLYHYDRAMNIOS OLIGOHYDRAMNIOS RH ISOIMMUNIZATION ANTEPARTUM FETAL SURVEILLANCE

445

445 446 446 448 449 449 450 450 45 1 45 1

Normal La bor and Del ivery DEFINITION AND STAGES OF LABOR OBSTETRIC EXAMINATION FETAL HEART RATE MONITORING OBSTETRIC ANALGESIA AND ANESTHESIA

Abnormal La bor and Del ivery INDICATIONS FOR (-SECTION PRETERM LABOR RUPTURE OF MEMBRANES FAILURE TO PROGRESS INTRA-AMNIOTIC INFECTION FETAL MALPRESENTATION UMBILICAL (ORD PROLAPSE SHOULDER DYSTOCIA EPISIOTOMY UTERINE INVERSION UTERINE RUPTURE

Puerperi u m POSTPARTUM HEMORRHAGE POSTPARTUM INFECTION PERIPARTUM (ARDIOMYOPATHY SHEEHAN SYNDROME ( POSTPARTUM PITUITARY NECROSIS)

Lactation a n d Breastfeed i n g PHYSIOLOGY (ONTRAINDICATIONS TO BREASTFEEDING MASTITIS/BREAST ABSCESS BREAST ENGORGEMENT NIPPLE INJURY LOCALIZED PLUGGED Duer GALACTOCELE

454

454 454 455 455

457

457 457 458 459 460 460 461 461 461 462 462

462

462 462 464 464

464 464 465 465 466 466 466 466

427

428

HIGH-YIELD FACTS IN

OBSTETRICS

THE BASICS OF PREGNANCY The terms and concepts that follow are central to an understanding of the physiologic processes of pregnancy: Gravidity: Number of times a woman has been pregnant Parity: Number of pregnancies that led to a birth beyond 20 weeks' gesta­ tional age or an infant weighing > 500 g ( l lb, 2 oz). ■

In prenatal assessment, TPAL expresses the number of term deliveries (T), the number of preterm deliveries (P), the number of abortuses (A), and the number of living children (L).

Emb ryonic age: Number of weeks + days since fertilization; usually unknown Gestational age (GA): The number of weeks and days measured from the first day of the last menstrual period (LMP). GA can also be determined by the following:

■

0-,,. KEY FACT

A G3Pl patient has had three pregnancies but only one birth beyond 20 weeks and/or an infant who weighs at least 500 g.

■

Fundal height: Reaches umbilicus (approximately 22 cm) at 20 weeks; + l cm/week of gestational age thereafter Fetal heart tones (Doppler) : Typically, 10 to 12 weeks Quickening or appreciation of fetal movement: Occurs at 16 weeks for multiparas and 20 weeks for primiparas at the earliest Ultrasonography: ■ Most accurate dating method in early pregnancy: Fetal crown-rump length (CRL) at 6 to 12 weeks ■ Preferred measurement from 1 3 weeks+: Biometry using biparietal diameter (BPD), femur length (FL), and abdominal circumference (AC)

DIAGNOSIS OF PREGNANCY 0-,,. KEY FACT

Get a quantitative serum [3-hCG: To help diagnose ectopic pregnancy and follow the trend for resolution after treatment ■ To help diagnose miscarriage ■ To monitor after treatment of trophoblastic disease (rising levels concerning for choriocarcinoma) ■ To screen for feta l a neuploidy

(3-HUMAN CHORIONIC GONADOTROPIN ( (3-hCG )

■

■ ■ ■

The standard for diagnosing pregnancy. Can be detected m serum or unne. Serum 13-hCG more sensitive and preferred if menstrual period 1s < 1 week late. Produced by the placenta; peaks at 100,000 mlU/mL by 10 weeks. ,J, throughout the second trimester; plateaus in the third trimester. 13-hCG levels double approximately every 48 hours during early preg­ nancy; failure of hCG levels to double every 48 hours concerning for mis­ carriage or ectopic pregnancy.

ULTRASONOGRAPHY ■ ■

■

Used to confirm an intrauterine pregnancy Gestational sac visible on transvaginal ultrasonography by: ■ 5 weeks ■ A 13-hCG in the range of 1500 to 3 500 mlU/mL Transabdominal ultrasound (US) typically reserved for second-/third­ trimester measurements

OBSTETRICS

HIGH -YIELD FACTS IN

429

PHYSIOLOGIC CHANGES IN PREGNANCY Table 2.10-1 describes the physiologic changes seen in pregnancy, as well as their mechanisms. These changes occur to increase perfusion to the fetus, opti­ mize materno-fetal gas exchange, and alter the maternal pelvis to aid in delivery. TA B L E 2 . 1 0-1

Physiologic Changes in Preg nancy

SYSTEM

CHANGES

MECHA N I SMS

Cardiovascu lar

Stroke vol u me increases to maximum at 19 weeks and then plateaus Heart rate gradually increases 20% Ca rdiac output rises rapidly by 20% and then gradually increases an additional 1 0% by 26 weeks

Stroke volume + i heart rate ➔ i cardiac output i progesterone ➔ -!- peripheral vascular resistance ➔ -!- blood pressure

Peripheral vascular resistance progressively decreases to term Blood pressure gradually decreases 1 0% by 34 weeks and then increases to prepregnancy values Peripheral venous distention progressively increases to term The increase in stroke volume may cause physiologic systolic flow murmur Circulatory

Blood volume i by 50% in the second trimester Fibrinogen i Hematocrit -!- slightly -!- platelet count

i fibrinogen, factor VII and VII I + -!- protein S ➔ hypercoagu lable state (-!- intrapartum

blood loss risk) Plasma vol ume ii > i RBC ➔ -!- hemato­ crit (dilutional anemia)

Pulmonary

Tidal volu me ii Respiratory rate, vital capacity: Unchanged Expiratory reserve: Gradual decline

Dyspnea (due to pressure from uterus) Respiratory al kalosis with meta bolic com­ pensation (progesterone mediates an increase in tidal volume and alveolar ventilation)

Renal

Renal flow i 25%-50% Glomerular fi ltration rate (GFR) i early and then plateaus -!- serum creatinine (Cr) and blood u rea nitrogen (BUN) Hyponatremia 4-5 m Eq/L below prepregnancy levels (due to dilution and increased antidiuretic hormone secretion) i urinary frequency Glucosuria

Gastrointestinal

-!- esophageal sphincter tone + i gastric emptying time ➔ reflux -!- gastrointestinal (GI) motility ➔ constipation -!- gallbladder motility ➔ gallstones -!- venous return ➔ hemorrhoids

Musculoskeletal

Low back pain common in third trimester, caused by i pressure from the uterus and laxity of muscles and joints

Skin

Chloasma (melasma): patchy brown discoloration of the face Linea nigra Nipple hyperpigmentation (continues)

430 TA B L E 2.1 0-1

HIGH-YIELD FACTS IN

OBSTETRICS

Physiologic Changes in Pregnancy (continued)

SYSTEM

CHANGES

MECHANISMS

Endocrine

Progressive i in estrogen, progesterone, and prolactin i in �-hCG, peak at 1 2 weeks, and -!- until plateau reached at about 24

Thyroid-binding globulin (TBG) levels i in

weeks i in total T3 and T4 and -!- in thyroid-stimu lating hormone (TSH) due to i negative feedback (see Fig. 2.1 0-1 )

response to estrogen i n pregnancy. Most T, and T4 circulate bou nd to TBG, so the T, and total T4 will also i, but the levels of free T, and T4 will not change.

PRENATAL CARE AND DIAGNOSTIC TESTING

t hCG

The goal of prenatal care is to prevent, diagnose, and treat conditions that can lead to adverse fetal or maternal outcomes in pregnancy. Expected weight gain, nutrition, and exercise recommendations are outlined in Table 2.10-2. Maternal failure to gain appropriate weight is associated with fetal growth restriction (FGR), whereas excess weight gain is associated with diabetes.

TAB L E 2 . 1 0-2.

CATEGORY

RECOMMENDATIONS

Weight gain

Guidelines for weight gain according to prepregnancy body mass index (BMI): Underweight (BMI < 1 8.5): 1 2- 1 8 kg (28-40 lb) Acceptable (BMI 1 8.5-24.9): 1 1 - 1 6 kg (25-35 lb) ■

t estrogen ----©----+ t TBG F I G U R E 2. 1 0- 1 . Effect of pregnancy on the pituitary-thyroid axis. hCG, Human

Recommendations for Standard Prenata l Care

Overweight (BMI 25-29.9): 7-1 1 kg ( 1 5-25 lb) Obese (all classes, BMI >30): 5-9 kg (1 1 -20 lb)

N utrition

chorionic gonadotropin; TBG, thyroid­ binding globulin; TSH, thyroid-stimulat­ ing hormone. (Reproduced with permission from USMLE-Rx.com.)

Guidelines for nutritional supplementation: ■

An additional 1 00-300 kcal/day; additional 500 kcal/day during breastfeeding

■ Folic acid supplements 400 mcg daily or 4000 mcg if previous neural tube defect or maternal use of valproate or carbamazepine (folate supplementation -!- neural tube defects for al l reproductive-age women) Iron ■ Calcium ■ Additional guidelines for vegans and others limiting meat/dairy intake: ■ Vitamin D ■ Vitamin B,, Exercise

Thirty minutes of moderate exercise daily, while avoiding contact sports

Ha rmfu l su bstances

■ ■

Avoid fish with high mercury levels Moderate caffeine i nta ke Avoid uncooked meat, fish, eggs Avoid unpasteu rized dairy

OBSTETRICS

HIGH -YIELD FACTS IN

43 1

G ROU P B STREPTOCOCCUS TESTING AN D TREATMENT Screening for group B streptococcus (GBS) : Rectovaginal swab at 36 to 38

weeks.

Indications for intrapartum prophylaxis:

■ CBS-positive rectovaginal swab at 36 to 38 weeks ■ CBS bacteriuria/urinary tract infection (UTI) any time during pregnancy Prior infant with early-onset CBS sepsis Unknown CBS status with any of the following: labor at 18 hours lntrapartum prophylaxis: Intravenous (IV) penicillin (regardless of mode of birth); first-generation cephalosporins for patients with low-risk penicillin allergy; clindamycin or vancomycin for high risk of anaphylaxis

PRENATAL DIAGNOSTIC TESTING Table 2.10-3 outlines a typical prenatal diagnostic testing schedule by week. The sections that follow describe each recommended screening modality.

TA B L E 2 . 1 0-3.

Prenatal Visit and Diagnostic Testing Schedule

PRENATAL DIAGN OSTIC TESTING Prenatal visits

Weeks 0-28: Every 4 weeks Weeks 29-35: Every 2 weeks Weeks 36-birth: Every week

Initial visit

Heme: Complete blood cell count (CBC); type and screen (important for determining Rh status) Infectious disease: U rinalysis (UA) and culture; rubella antibody titer; hepatitis B surface antigen (HBsAg); Hepatitis C antigen; rapid plasma reagin (RPR)Nenereal Disease Research Laboratory (VDRL); cervical gonorrhea and chlamydia; pu rified protein derivative (PPD); HIV; tuberculosis (TB) testing (or Mycobacterium tuberculosis [M.tb]); Pap smear (to check for dys­ plasia); consideration of hepatitis C vaccine (HCV) and varicella vaccine, based on history If indicated: Hemoglobin (H b)A 1 c, sickle cell screening Discussion of genetic screening: Tay-Sachs disease, cystic fibrosis, spinal muscular atrophy

1 0-22 weeks

Aneuploidy screening: Multiple options are possible: ■ Cell-free DNA screening most sensitive and specific screening tool available for trisomies 1 3, 1 8, and 21 (see Table 2.1 0-4). It is accepted as a primary screening option ■ Fu ll integrated test: Serum pregnancy-associated plasma protein A (PAPP-A) collected 1 1 - 1 4 weeks; crown-rump length measured 1 0- 1 4 weeks, + quadruple screen in second trimester (hCG, inhibin A, alpha-fetoprotein, unconjugated estriol) ■ Combined test: 13-hCG, PAPP-A, measure of n uchal translucency 1 0- 1 4 weeks

1 8-20 weeks

U ltrasonography for ful l anatomic screen

24-28 weeks

1 -hour SO-gram glucose challenge test for gestational diabetes screen; if positive: 3-hour 1 00-gram glucose challenge test

28-30 weeks

Rh (D a ntigen•) immune globulin for Rh0 women (after antibody screen)

35-37 weeks

GBS culture; repeat CBC

34-40 weeks

I n high-risk patients, cervical chlamydia and gonorrhea cultures, HIV, RPR

•Rh immune globu lin to treat any u nsensitized Rh0 woman during any occasion of fetal-maternal blood mixing (eg, spontaneous abortion [SABI, placental abruption, abdominal trau ma) even if 1 000

Treatment of infants with prophylactic

AZT; avoidance of breastfeeding

(continues)

436 TA B L E 2.1 0-7.

HIGH-YIELD FACTS IN

OBSTETRICS

Diagnosis and Treatment of Common Congenital Infections (continued)

DI SEASE

TRA NSMISSION

SYMPTOMS

DIAGNOSIS

TREATMENT

PREVENTION

Syphilis

l ntra partu m; transpla-

Maculopapular skin rash

Dark-field micros-

Penicillin (if

Penici l l i n i n preg nant

cental transmission possible

Lym phadenopathy

copy, VDRU

Hepatomegaly

RPR, fl uorescent

"Snuffles": Mucopurulent

treponemal a nti-

Osteitis

(FTA-ABS)

body a bsorption

rhinitis

Late congen ita l syphilis:

allergic,

should desen-

patients who test EB

sitize and give penici l l i n)

Saber shins

Saddle nose

CNS i nvolvement

Hutchi nson triad: Peg-

shaped central i ncisors, deafness, interstitial keratitis

Zika virus

Tra nsplacenta l

transm ission

Microcephaly

Cra niofacial disproportion

Zika RNA detection

Neurologic and ocular abnormalities

None

Avoida nce of tropical mosquito-infested regions

ABORTION SPONTANEOUS ABORTION The loss of gestation before 20 weeks' gestation. More than 80% of cases occur in the first trimester. Associations are as follows: ■

■

Maternal factors: Inherited thrombophilia: Factor V Leiden, prothrombin, antithrom­ bin, protein C and S deficiencies, methylene tetrahydrofolate reduc­ tase deficiency (hyperhomocysteinemia) ■ Immunologic issues: Antiphospholipid antibodies; alloimmune factors ■ Anatomic issues: Uterine and cervical abnormalities, cervical insuffi­ ciency, cervical conization or loop electrosurgical excision procedure (LEEP), cervical injury, diethylstilbestrol (DES) exposure ■ Endocrinologic issues: Diabetes mellitus (DM), hypothyroidism, pro­ gesterone deficiency ■ Genetics: Osteogenesis imperfecta type II; this is severe and lethal; infants present with multiple fractures and die in utero or shortly after birth. ■ Other: Maternal trauma, i maternal age, infection, dietary deficiencies Environmental factors: Tobacco, alcohol, excessive caffeine (> 500 mg/ day), toxins, drugs, radiation Fetal factors: ■ Anatomic malformation ■ Chromosomal abnormalities: A factor in approximately 50% of spon­ taneous abortions (SABs) in the first trimester, 20% to 30% in second­ trimester losses, and 5 % to 10% in third-trimester losses Recurrent SAB: Two or more consecutive SABs or three SABs in 1 year; causes dependent on timing; determining possible causes requires karyo­ typing of both parents, workup of mother for hypercoagulability, and eval­ uation of uterine anatomy ■

OBSTETRICS

HIGH -YIELD FACTS IN

437

■ Early (< 12 weeks): Chromosomal abnormalities likely cause ■ Late ( 1 2-20 weeks) : Hypercoagulable states (eg, antiphospholipid syn­ drome, systemic lupus erythematosus [SLE], factor V Leiden, protein S deficiency), cervical insufficiency If antiphospholipid antibodies are detected, false EB Venereal Disease Research Laboratory (VDRL) and falsely prolonged partial thromboplastin time (P'IT) may be seen. Low-molecular-weight heparin and low-dose aspirin provide prophylaxis against recurrent SAB.

H istory/P E SAB can be categorized by visual examination of the cervical os (open vs closed) and the presence or status of the fetus and products of conception. See Table 2.10-8 for types of SAB. TA B L E 2 . 1 o-s.

Types of Spontaneous Abortion

TYPE

SYM PTOMS/SIGNS

DIAGNOSIS

TREATMENT

Complete

Cessation of bleeding and cramping

Closed os Ultrasonography showing no POC

None

Closed os + intact membranes +

Follow-up u ltrasonography to assess

Expulsion of products of conception (POC) Threatened

Uterine bleeding ± abdominal pain (often painless) No POC expulsion

I ncomplete

Partial POC expulsion; bleeding/mild cramping

fetal cardiac motion on ultrasonography Visible tissue on examination Open os POC present on ultraso­ nography Treatment

viability of fetus

Manual uterine aspiration (MUA) if < 1 2 weeks or dilation and curettage (D&C); may also use misoprostol or expectant management in inevitable and missed SAB

Visible tissue on examination

Open os

POC present on ultrasonography

Inevitable

Uterine bleeding and cram ps No POC expulsion

Open os ± rupture of membranes (ROM) POC present on ultrasonography

Missed

Asymptomatic ± cramping

Closed os

No bleeding

No fetal cardiac activity; POC present on ultrasonography

Foul-smelling discharge, abdominal pain, fever, and cervical motion tenderness; ± POC expulsion

Hypotension, hypothermia, i WBC count Blood cultures

Manual uterine aspiration or D&C and IV a ntibiotics

Uterus small for GA; no fetal

If 20 week

heart tones or movement on ultrasonography

If > 24 weeks, induction of labor within 1 -2 weeks, based on patient preference Offer autopsy to attem pt to determine cause of death

438

HIGH-YIELD FACTS IN

TA B L E 2 . 1 0-9.

OBSTETRICS

Elective Abortion

TRIM ESTER

PROCEDURE

GESTATIONAL AGE

Fi rst (90% of a bortions)

Medical management: ora l mifepristone (low dose) + ora l/

< 1 0 weeks

Surgical management:

> 1 0 weeks

vaginal misoprostol

■ Manual uterine aspiration

■ Dilation and cu rettage (D&C) with vacu u m aspiration Second ( 1 0% of abortions)

Obstetric management: induction of labor (typically with

1 3-24 weeks (depending on state laws)

Surgical management: Dilation and evacuation (D&E)

Same as above

prostaglandins, a m niotomy, and oxytocin)

Diagnosis ■

0--n KEY FACT

If fever, vomiting, purulent discharge, and/or hemodynamic instability are seen after a spontaneous or elective abortion, septic abortion should be suspected. This is a medical emergency that requires broad-spectrum antibiotics and immediate surgery to remove infected tissue.

0-

MNEMONIC

The l's are open.

Findings in cervica l exa m d u ring SAB: Inevita ble and Incomplete show open os.

Diagnosis by clinical presentation and physical examination Nonviable pregnancy: Gestational sac >25 mm without a fetal pole or absence of fetal cardiac activity when CRL > 7 mm on transvaginal ultrasonography Best initial test: Ultrasonography can identify the following: ■ Gestational sac 5 to 6 weeks from the LMP ■ Fetal pole at 6 weeks ■ Fetal cardiac activity at 6 to 7 weeks Next best test: Serum J3-hCG

Treatment ■ ■

See Table 2.10-8 for treatment specific to the type of SAB. Administer Rh immune globulin if the mother is Rh 8.

ELECTIVE TERMINATION OF PREGNANCY It has been estimated that 50% of all pregnancies in the United States are unintended. About 2 5 % of all pregnancies end in elective abortion. Options for elective abortion depend on GA and patient preference (see Table 2.10-9).

COMPLICATIONS OF ABORTION Septic abortion Retained products of conception ➔ disseminated intravascular coagula­ tion (DIC) ■ Endometritis ■ ■

MATERNAL COMPLICATIONS OF PREGNANCY HYPEREMESIS GRAVIDARUM Persistent vomiting not related to other causes, leading to starvation ketosis and weight loss (usually at least a 5 % .J, from prepregnancy weight). More common in first pregnancies, multiple gestations, and molar pregnancies ■ T J3-hCG and i estradiol have been implicated in pathophysiology ■

OBSTETRICS History/PE Distinguished from nausea and vomiting of pregnancy (NVP) by severity (presence of weight loss and ketosis) and timing. Acid reflux, gastroenteritis, hyperthyroidism, and neurologic conditions can also cause NVP and should be on the differential diagnosis.

HIGH -YIELD FACTS IN

439

0-,r KEV FACT

Rule out trophoblastic disease with US in a pregnant patient who presents with severe nausea and vomiting.

Diagnosis Clinical diagnosis. Best initial test: Ultrasonography; evaluation for trophoblastic disease or multiple gestation. ■ Evaluation for electrolyte abnormalities (eg, hypokalemia), abnormal liver enzymes, amylase, and lipase. ■ Wernicke encephalopathy from vitamin B 1 deficiency possible in severe cases. The physician can look for gait ataxia and oculomotor dysfunction. ■

Treatment ■ Best initial treatment: ■ Dietary changes and doxylamine-pyridoxine ■ If no response, discontinue doxylamine-pyridoxine and use metoclo­ pramide, promethazine, or prochlorperazine ■ Consider ondansetron if vomiting is not resolved with treatments men­ tioned earlier ■ If dehydrated, administration of IV fluids, IV nutritional supplementation, and ondansetron IV

DIABETES Diabetes in pregnancy is divided into the following two categories: pregesta­ tional OM and gestational OM. ■ Pregestational DM: Onset before pregnancy (Type 1 or Type 2 OM) ■ Gestational DM: Onset during pregnancy Consider early screening (fasting glucose, first-trimester HbA l c, or early glucose tolerence test) for the following risk factors: ■ Overweight or obese (BMI >25) ■ Physical inactivity First-degree relative with OM Have previously given birth to an infant weighing 4000 g (approximately 9 lb) or more ■ Previous gestational OM ■ Women with polycystic ovarian syndrome ■ Hemoglobin A l e (HbA l c) 2: 5.7%, impaired glucose tolerance, or impaired fasting glucose on previous testing ■ History of cardiovascular disease ■ Other clinical conditions associated with insulin resistance (eg, metabolic syndrome, acanthosis nigricans)

Pregestational Diabetes Observed in 1 % of all pregnancies. Insulin requirements may i as much as threefold. Poorly controlled OM is associated with an i risk for congenital malformations, fetal loss, and maternal/fetal morbidity during labor and delivery.

0-,r KEV FACT

Greater than 8, investigate! If H bA 1 c is >8%, look for congenital abnormalities.

440

HIGH-YIELD FACTS IN

OBSTETRICS Testing and Treatment Mother: Renal, ophthalmologic, and cardiac evaluation to assess for end-organ damage. ■ Best initial treatment: Lifestyle modification with diet and exercise. Addi­ tion of insulin therapy if poor response. ■ Strict glucose control is important to minimize fetal defects: ■ Fasting morning: :::;95 mg/dL ■ 2-hour postprandial: < 120 mg/dL ■ Delivery and postpartum: ■ Maintain normoglycemia (80-100 mg/dL) during labor with an IV insulin drip and hourly glucose measurements. ■ Consider delivery in the setting of poor maternal glucose control, pre­ eclampsia, macrosomia, or evidence of fetal lung maturity (preferably after 32 weeks). ■ Consider C-section in the setting of an estimated fetal weight (EFW) >4500 g. ■

Fetus: 1 6-24 weeks: Ultrasonography to determine fetal anatomy (18-20 weeks) ■ 3 2-34 weeks: ■ Antepartum fetal surveillance (eg, nonstress test [NST], contraction stress test [CST], biophysical profile [BPP]) if poor glucose control or small vessel disease ■ Hospitalization if maternal DM has been poorly controlled or fetal parameters are a concern ■ Serial ultrasonograms for fetal growth ■

0-rr

KEY FACT

Hyperglycemia in the first trimester suggests preexisting DM and should be managed as pregestational DM.

■

Com plications Pregestational DM is a risk factor for a variety of antepartum, intrapartum, and postpartum maternal and fetal complications (Table 2.10-10). Tight gly­ cemic control is the best way to prevent these complications Gestational Diabetes Carbohydrate intolerance of variable severity first diagnosed during preg­ nancy. Occurs in 3 % to 5 % of all pregnancies and is usually diagnosed in the second trimester (24-28 weeks). TAB L E 2 . 1 0-1 o.

Complications of Pregestational Diabetes Mel l itus

MATERNAL COMPLI CATIONS

FETA L COM P LICATIONS

Diabetic ketoacidosis ([OKA], type 1) or hyperg ly­

Small left colon syndrome

Preeclam psia/eclampsia

Cardiac and renal defects

cemic hyperosmolar non ketotic coma (type 2)

Cephalopelvic disproportion (from macrosomia) and need for (-section

Preterm labor

Macrosomia or FGR

Neural tube defects (eg, sacra l agenesis)

Hypoca lcemia Polycythemia

Infection

Hyperbi lirubi nemia

Postpa rtu m hemorrhage

Respi ratory distress synd rome (RDS)

Polyhyd ram n ios

Maternal mortality

Hypog lycemia from hyperinsulinemia Birth injury (eg, shoulder dystocia) Perinata l mortality

OBSTETRICS

HIGH -YIELD FACTS IN

441

H i story/PE ■ Typically asymptomatic ■ May present with edema, polyhydramnios, or a large-for-gestational-age infant (>90th percentile) Diagnosis ■ Best initial test: Routine screening with a I-hour 50-g glucose challenge test ■ Venous plasma glucose measured 1 hour later ■ Performed at 24 to 28 weeks ■ Values 2: 140 mg/dL considered abnormal ■ Confirmation with an oral 3-hour (100-g) glucose tolerance test (GTT; next test if EB screening test) showing any two of the following: ■ Fasting: >95 mg/dL ■ 1 hour: > 180 mg/dL ■ 2 hours: > 15 5 mg/dL ■ 3 hours: > 140 mg/dL Treatment Mother: Best initial treatment: American Diabetes Association (ADA) diet, regular exercise, and strict glucose monitoring (four times per day). ■ Insulin is the gold standard if dietary control is insufficient. Tight maternal glucose control (fasting ::595 mg/dL; 1 hour postprandial ::5 l 40 mg/dL; 2 hours postprandial :::S 120 mg/dL) improves outcomes. ■ lntrapartum insulin and dextrose to maintain tight control during delivery. ■

Fetus: ■ ■

Periodic ultrasonography and NSTs to assess fetal growth and well-being Recommended induction of labor at 39 to 40 weeks in patients with gestational diabetes controlled on insulin

Compl ications More than 50% of patients with gestational diabetes develop glucose intoler­ ance and/or type 2 OM later in life. At 6 to 12 weeks postpartum, the physi­ cian should screen for OM (7 5-g 2-hour GTT) and repeat testing every 3 years if normal results.

HYPERTE NSIVE DISEASE IN PREGNANCY Chron ic and Gestational Hypertension Defined as follows: Chronic hypertension: Presents before conception or at 12 weeks postpartum Possibility for up to one third of patients with chronic hypertension to develop superimposed preeclampsia ■ Gestational hypertension: ■ Idiopathic hypertension (systolic BP 2: 140 mm Hg or diastolic BP 2:90 mm Hg measured twice >4 hours apart) without significant pro­ teinuria ( < 300 mg/L) Develops at 2:20 weeks ■

■ ■

0-n

KEV FACT

Keys to the management of gestational diabetes: ( 1 ) ADA diet, (2) insulin if needed, (3) ultrasonography for fetal growth, and (4) antepartum surveillance if requiring insulin or an oral hypoglycemic agent.

442

HIGH-YIELD FACTS IN

OBSTETRICS ■ ■

Possibility for up to 2 5 % of patients with gestational hypertension to develop preeclampsia Must normalize within 12 weeks after pregnancy

Treatment ■ Close monitoring of BP ■ Best initial treatment: Treatment with appropriate antihypertensives (eg, methyldopa, labetalol, nifedipine) ■ If systolic BP 2: 160 mm Hg or diastolic BP 2: 110 mm Hg, this is a hyper­ tensive crisis that calls for labetalol, hydralazine, or nifedipine because of short onset of action The patient should not take angiotensin-converting enzyme (ACE) inhibi­ tors or diuretics ■ ACE inhibitors are known to lead to uterine ischemia and fetal renal damage/defects ■ Diuretics can aggravate low plasma volume to the point of uterine ischemia Com plications Similar to those of preeclampsia (see next).

(J.◊ MNEMONIC

HELLP syndrome­ Hemolysis Elevated liver function tests (LFTs) Low Platelets

Preeclampsia ■ New-onset hypertension (systolic BP 2: 140 mm Hg or diastolic BP 2:90 mm Hg) and proteinuria (> 300 mg of protein in a 24-hour period or elevated urine or protein/creatinine ratio of 0. 3 or more) occurring at > 20 weeks gestation up to 6 weeks postdelivery ■ Preeclampsia with severe features defined as new-onset hypertension with new onset of any of the following features (with or without proteinuria): ■ Platelet count 1. 1 mg/dL or doubling of creatinine concentration in the absence of other renal disease ■ Liver transaminases at least twice the upper limit of normal ■ Pulmonary edema Neurologic or visual symptoms ■ New-onset headache unresponsive to medication and not accounted for by alternative diagnoses ■ HELLP syndrome: A variant of severe preeclampsia with severe features ■ Consists of hemolytic anemia, elevated liver enzymes, and low platelets. ■ Etiology unknown. Clinical manifestations are explained by vasospasm leading to distention of hepatic capsule, hemorrhage, and organ necrosis. Risk factors: Nulliparity, extremes of age ( 3 5 years), multiple ges­ tation, molar pregnancy, renal disease (caused by SLE or type 1 OM), a family history of preeclampsia, and chronic hypertension. ■ Indication for delivery H istory/PE See Table 2.10-11 for the signs and symptoms of preeclampsia. Treatment ■ Recommend low dose aspirin daily during pregnancy ■ Delivery of the fetus is the only cure for preeclampsia. ■ Close-to-term or worsening preeclampsia: Induce delivery with IV oxyto­ cin, prostaglandin, or amniotomy. ■ Delivery should occur no later than 3 7 weeks.

OBSTETRICS TA B L E 2 . 1 0-1 1 .

HIGH -YIELD FACTS IN

443

Presentation of Preeclampsia and Eclampsia

D I SEASE SEVERITY

S I G N S AND SYM PTOMS

DELIVERY

Preeclampsia

Usually asym ptomatic

Del ivery at 37 weeks

BP 2:: 1 40/90 m m Hg on two occasions >4 h r apart

and

Proteinuria (> 300 mg/24 hours or two E9 u rine dipsticks) Preeclampsia with severe features

Any one of the following:

Hospitalization

BP 2:: 1 60/1 1 O m m Hg on two occasions >4 hr

BP control

apart

Del ivery by 34 weeks,

Cerebral cha nges: Severe headache, somnolence

m ust balance

Visual changes: Blu rred vision, scotomata

maternal risk with

Other: Progressive renal insufficiency, pul monary

risks of prematu­

edema; right u pper quadrant (RUQ) pain, hemo­

rity in the infa nt

lysis, elevated liver enzymes, throm bocytopenia (HELLP synd rome) Eclampsia

Most common signs preceding an eclamptic

I mmediate del ivery

attack: Headache, visual changes, and RUQ/ epigastric pai n Seizu res severe if not control led with anticonvu l­ sant therapy

Far from term ( < 34 weeks) : Provide expectant management with close surveillance; hospital admission for uncontrolled severe-range blood pressures. ■ Prevent intrapartum seizures with a continuous magnesium sulfate drip. ■ Continue seizure prophylaxis for 24 hours postpartum. ■ Treat magnesium toxicity with IV calcium gluconate. ■ Preeclampsia with severe features: ■ Control BP with labetalol and/or hydralazine (goal 100,000 mlU/mL). ■ X-ray of the chest (CXR) may show lung metastases. D&C reveals "cluster-of-grapes" tissue. Treatment ■ Best initial treatment: Evacuate the uterus with D&C. ■ Follow with weekly 13-hCG to undetectable (or negative) weekly, and then monthly for 6 months. Contraception for at least 6 months. ■ Treat malignant disease with chemotherapy (methotrexate or dactinomycin). ■ Treat residual uterine disease with hysterectomy. Chemotherapy and irradiation are highly effective for metastases.

ANTEPARTUM HEMORRHAGE AND ABNORMAL PLACENTATION ■ ■ ■

Any bleeding that occurs after 20 weeks Complicates 3 % to 5 % of pregnancies Most common causes: Placental abruption and placenta prev1a (see Table 2.10-13 and Figs. 2.10-5 and 2.10-6)

OBSTETRICS TA B L E

2 . 1 0- 1 3 .

HIGH -YIELD FACTS IN

447

Placental Abruption vs Placenta Previa vs Vasa Previa

VARIABLE

PLACENTA L ABRUPTION

PLACENTA PREVIA

VASA PREVIA

Pathophysiology

Prematu re (before delivery) separa-

Abnormal placental location:

Velamentous umbilical cord

tion of normally i m planted placenta

Tota l: Placenta covers the cervica l os

insertion and/or bi lobed

Marginal: Placenta extends to the margin of

placenta causing vessels to

the os

pass over the internal os

Low lyi ng: Placenta is in close proxi mity to the os I ncidence

1 in 1 00

1 in 200

1 in 2500

Risk factors

Hypertension, abdominal/pelvic

Prior (-sections, uterine surgeries, grand

M u ltiple gestation, in vitro

Symptoms

trau ma, tobacco or cocaine use,

m u ltipa rity, advanced maternal age, mul­

fertil ization (IVF), accessory

previous abruption, rapid decom­

tiple gestation, prior placenta previa

placenta l lobes, single u mbil­

pression of an overdistended

ical artery, placenta previa,

uterus, excessive sti m u lation

low-lyi ng placenta

Painfu l vaginal bleeding that does not sponta neously cease Abdom inal pa in; uterine hypertonicity

Diagnosis

Pain less bleeding at rupture

ceases in 1 -2 hours with or without

of mem branes with fetal

uterine contractions

bradycardia

Feta l distress

Usually no fetal distress

Primarily clinical

Tra nsabdomina l/transvaginal ultrasonog­

Tra nsvaginal u ltrasonography

ra phy sensitivity >95%; the physician

with color Doppler showing

nography sensitivity only 50%; the

should look for a n abnormally positioned

vessels passing over the

physician should look for a retropla­

placenta. Partial previa can resolve as

i nternal os

centa l clot; most usefu l for ruling

the lower uterine segment expa nds with

out previa

pregnancy progression.

Transabdomina l/transvaginal ultraso­

Management

Painless, bright red bleed ing that often

Stabil ize patients with mild abruption and a premature fetus; manage expecta ntly (hospita lize; start IV

Do not perform a transvaginal exam or US Sta bil ize patients with a prematu re fetus; provide active survei llance

Acute bleeding = emergency (-section delivery Diagnosis before bleeding: Ste­

and fetal monitoring; type and

Give tocolytics

roids at 28-32 weeks to help

cross-match blood)

Use serial u ltrasonograms to assess feta l

with fetal lung matu rity, hos­

Moderate to severe abruption: I m me­ diate delivery is indicated (vaginal delivery with a m niotomy if mother

growth, resol ution of partia l previa Ad minister beta methasone at 28-32 weeks to help with fetal lung matu rity

and fetus are stable and delivery

Del iver by (-section

is expected soon; (-section for

I n dications for del ivery include labor,

maternal or fetal distress)

pita l ization at 30-32 weeks for close monitoring and schedu led (-section del ivery as clinically indicated

life-th reatening bleed ing, fetal distress, documented fetal lung maturity, and 36 weeks

Com plications

Hemorrhagic shock

Risk for placenta accreta

DIC in 1 0% of patients

Vasa previa

Recurrence risk: 5%- 1 5%

Preterm delivery, PROM, FGR, congenita l

Feta l hypoxia

anomalies Recurrence risk: 4%-8%

Feta l exsa nguination

448

Serosa

HIGH-YIELD FACTS IN

OBSTETRICS Normal placenta

Myometrium

Placenta previa

Vasa previa

Placental abruption

Normal placenta

Placenta accreta

F I G U R E 2 . 1 0-6

Placenta increta

■

Placenta

F I G U R E 2 . 1 0-s. Placenta accreta spec­ trum. (Reproduced with permission from USMLE-Rx. com.)

0-,,. KEY FACT With third-tri mester bleed i ng, t h i n k a natomica l ly: ■ Vagina: vag i n itis, vag i n a l lesion/ tra u m a ■ Cervix: bloody show (la bor), cervica l lesion/tra u m a ■ Placenta: Placenta l abru ption, placenta previa ■ Fetus: Feta l bleed i ng

■ ■

Placental Implantation. (Reproduced with perm ission from USMLE-Rx.com.)

Other causes: Other forms of abnormal placentation (see later), ruptured uterus, genital tract lesions, and trauma ■ Abnormal placental implantation (Figs. 2.10-5 and 2.10-6) results from an abnormality of the decidua basalis and is an important risk factor for postpartum maternal hemorrhage. ■ Placenta accreta: Abnormal implantation of the placenta such that the placental viii are attached to the myometrium. Placenta increta: Abnormal implantation of the placenta such that the placental viii penetrate into the myometrium ■ Placenta percreta: Abnormal implantation of the placenta such that the placental villi penetrate through the myometrium and into the serosa. Risk factors: Prior uterine incisions (C-section, fibroid removal), low-lying placentation, placenta previa, fetal Down syndrome Complications: Maternal hemorrhage, fetal asphyxiation, death.

MULTIPLE GESTATION Affects 3 % of all live births. Since 1980, the incidence of monozygotic (identi­ cal) twins has remained steady, whereas the incidence of dizygotic (fraternal) and higher-order births has i. H i story/PE

Characterized by rapid uterine growth, excessive maternal weight gain, and palpation of three or more large fetal parts on Leopold maneuvers. Diagnosis ■ ■

Ultrasonography 13-hCG, human placental lactogen, and MSAFP elevated for GA

Treatment

■

Multifetal reduction and selective fetal termination options for higher­ order multiple pregnancies Antepartum fetal surveillance for FGR

Complications ■

■

Maternal: Patients six times more likely to be hospitalized with complica­ tions of pregnancy. i incidence of placenta previa and need for C-section delivery. Fetal: Twin-to-twin transfusion syndrome (most common in monochori­ onic twins), cord entanglement (commonly in monoamniotic twins), FGR, preterm labor, and T incidence of congenital malformations.

OBSTETRICS FETAL GROWTH RESTRICTION An EFW less than 10th percentile for GA.

History/PE Risk factors include: ■ ■ ■ ■

■

■

Maternal systemic disease leading to uteroplacental insufficiency (intrauterine infection, hypertension, anemia). Maternal substance use. Placenta previa. Multiple gestation. Symmetric FGR results from aneuploidy, congenital anomalies, and intra­ uterine infection. This usually occurs in the first trimester. Asymmetric FGR ("head-sparing growth lag") results from uteroplacental insufficiency, maternal hypertension, or other maternal chronic disease. This usually occurs in the second/third trimester.

Diagnosis ■ Best initial test: US to confirm GA and fetal weight. ■ Antepartum serial fundal height measurements with ultrasonography and weekly biophysical profiles; umbilical artery Doppler velocimetry. Treatment ■ Explore the underlying etiology and correct if possible. ■ If the patient is near due date, administer steroids (eg, betamethasone) to accelerate fetal lung maturity; this treatment is required 48 hours before delivery. ■ Perform antepartum fetal monitoring. ■ A nonreassuring status near term may prompt delivery. Compl ications i perinatal morbidity and mortality.

FETAL MACROSOMIA A birth weight >95th percentile. A common sequela of gestational diabetes due to fetal hyperglycemia.

Diagnosis ■ Best initial test: US to estimate fetal size ■ Most accurate test: Weighing the newborn at birth (prenatal diagnosis is imprecise) Treatment Consideration of planned C-section delivery for an EFW > 5000 g in a preg­ nant patient without OM and for an EFW >4500 g in a pregnant patient with OM. Compl ications i risk for shoulder dystocia (leading to brachia! plexus m1ury and Erb­ Duchenne palsy) as birth weight i.

HIGH -YIELD FACTS IN

449

450

HIGH-YIELD FACTS IN

OBSTETRICS POLYHYDRAMNIOS An amniotic fluid index (AFI) 2::24 or single deepest pocket 2::8 cm on ultra­ sonography. May be present in normal pregnancies, but fetal chromosomal developmental abnormalities must be considered.

Etiolog ies Maternal OM Multiple gestation Isoimmunization Pulmonary abnormalities (eg, cystic lung malformations) Fetal gastrointestinal (GI) tract anomalies (eg, duodenal atresia, tracheoesophageal fistula, anencephaly) ■ Twin-twin transfusion syndrome ■ ■ ■ ■ ■

H istory/PE Usually asymptomatic. Diagnosis Sonographic documentation of excessive amniotic fluid volume defined as an AFI greater than or equal to (symbol) 24 cm or a single deepest pocket greater than or equal to (symbol) 8 cm. Additional evaluation should include ultraso­ nography for fetal anomalies, glucose testing for OM, and Rh screen. May note fundal height greater than expected for GA. Treatment Therapeutic serial amniocentesis to remove fluid for severe symptomatic poly­ hydramnios with shortness of breath; treatment of underlying cause if possible Compl ications Preterm labor, fetal malpresentation, cord prolapse

OLIGOHYDRAMNIOS An AFI < 5 on US or largest visible pocket 41 weeks) ■ Rupture of membranes Diagnosis The sum of the deepest amniotic fluid pocket in all four abdominal quadrants on ultrasonography. Treatment Rule out rupture of membranes.Treat the underlying cause if possible. Compl ications ■ Associated with a 40-fold i in perinatal mortality ■ Other complications: Musculoskeletal abnormalities (eg, clubfoot, facial distortion), pulmonary hypoplasia, umbilical cord compression, and FGR

OBSTETRICS

,\' ,\' _., ,\'

- .,.

-.....,. -

Maternal Rh8 red blood cell

.,.

.,.

-�

Fetal RhE& red blood cell in the maternal circulation

).

.. :;,

B

Agglutination of fetal RhE& red blood cells leads to HON

,

C

45 1

' •

"".. 'I-

-'-< -1. ..... -� Anti-Rh -(...

A

HIGH -YIELD FACTS IN

antibodies

D

F I G U R E 2 . 1 0-7.

Maternal antibodies, from Rh isoimmunization at the time of the previous del ivery, cross the placenta and cause hemoly­ sis of RBCs in the fetus. (A) Rh 8 mother before pregnancy. (B) Rh EB fetus in Rh 8 mother. (C) After delivery, the mother develops antibod­ ies to Rh antigen.

(D)

Rh EB fetus in the next pregnancy. HDN, Hemolytic disease of the newborn. (Reproduced with permission from USMLE-Rx.com.)

RH ISOIMMUNIZATION Fetal RBCs leak into the maternal circulation, and maternal anti-Rh lgG anti­ bodies form that can cross the placenta, leading to hemolysis of fetal RhEB RBCs (erythroblastosis fetalis; see Fig. 2.10-7). Rh isoimmunization occurs only in Rh8 women; i risk with previous SAB or therapeutic abortion (TAB) or previous delivery with no Rho (D antigen) immune globulin given. Diagnosis

Sensitized Rh8 pregnant patients with titers > 1 : 16. Anti-D antibody titers should be closely monitored for evidence of fetal hemolysis. Treatment

In severe cases, the physician should initiate preterm delivery. Before delivery, intrauterine blood transfusions can be given to correct a low fetal hematocrit. Prevention

If the patient is Rh8 and the other parent is RhEB (or the status is unknown), give Rh immune globulin at 28 weeks. ■ If the baby is RhEB, give the mother Rh immune globulin postpartum. The dose is based on the Kleihauer-Betke test. Inadequate dosing can lead to alloimmunization. ■ Give Rh immune globulin to Rh8 mothers who undergo abortion or who have had an ectopic pregnancy, amniocentesis, vaginal bleeding, or pla­ centa previa/placental abruption. Type and screening are critical. ■

Com pl ications

■

■

Hydrops fetalis when fetal hemoglobin is < 7 g/dL Fetal hypoxia and acidosis, kernicterus, prematurity, death

ANTEPARTUM FETAL SURVEILLANCE In general, antepartum fetal surveillance should occur in pregnancies in which the risk for antepartum fetal demise is i. Testing is initiated in most at­ risk pregnant patients at 32 to 34 weeks (or 26-28 weeks if there are multiple worrisome risk factors). The following assessments take place:

452

H I G H -YI E L D FACTS I N

O B STETRICS

TA B L E 2 . 1 0 - 1 4.

Non stress Test I nterpretation

Reactive NST (normal response)

Two accelerations i n FHR over 20-minute period (see Fig. 2.1 0-8): ■ > 1 0 bpm for 1 0 seconds if 1 5 bpm for 1 5 seconds above baseline for > 32 weeks

Nonreactive NST

I nsufficient accelerations over a 40-mi nute period Possibility for FHR accelerations to not occur because of any of the following reasons: Fetal sleeping (most common); can use vibroacoustic stimulation to wake up fetus 5

li mbs or spine

SCORE 2 - normal

0 - abnormal 2 - normal

0 - abnormal 2 - normal

0 - abnormal 2 - normal

0 - abnormal 2 - normal

0 - abnormal

■ CST: Performed in the lateral recumbent position ■ FHR monitored during spontaneous or oxytocin-induced contractions ■ Contraindicated in pregnant patients with preterm membrane rupture or known placenta previa, those with a history of uterine surgery, and in those who are at high risk for preterm labor ■ See Table 2.10-15 for CST interpretation ■ Biophysical profile (BPP): Uses real-time ultrasonography to assign a score of 2 (normal) or 0 (abnormal) to five parameters: fetal tone, breath­ ing, movement, amniotic fluid volume, and NST (Table 2.10-16) ■ 8 to 1 0 : Reassuring for fetal well-being ■ 6: Considered equivocal; test repeated in 24 hours if fetus 35 years of age Migraine with visual aura

Suspected gynecologic malignancy Copper IUD alone: ■ Copper intolerance (allergy, Wilson

Diabetic retinopathy or neuropathy ■

disease) Severe dysmenorrhea and/or menorrhagia

Progestin IUD alone: ■ Levonorgestrel allergy ■ Breast cancer ■ Acute liver disease or liver tumor •Includes OCPs, vaginal ring, and transdermal patch.

GYNECOLOGY TA B L E 2 . 1 1 -6.

HIG H -YIELD FACTS IN

481

Emergency Contraceptive Methods

M ETHOD A N D MECHA N ISM

ADVANTAGES

DISADVANTAGES'

Ulipristal: Selective progesterone receptor

Does not disrupt embryo postimplantation Safe for all patients

Expensive Requires a prescription

antagonist; delays ovulation; can be used up to 1 20 hours after i ntercou rse

Very effective Can be used in active pelvic i nfection Can be used longer after intercourse More effective due to 2 mechanisms of action Preferred in patients with higher BMI

Levonorgestrel: A progestin-only pill that delays

ovulation; m ust be used within 72 hours of intercourse Oral contraceptive taper: Delays ovulation;

Fewer nausea/vomiting adverse effects than an oral contraceptive taper Available without a prescription

Less effective than other methods Shorter window after intercourse for efficacy

Useful for patients who have OCPs at home

Nausea, vomiting, fatigue, headache,

most effective withi n 72 hours of inter­ course but can be used up to 1 20 hours after intercourse Copper IUD: Copper particles disrupt sperm

and ovum function, preventing fusion; may prevent implantation; can be used up to 7 days after intercourse

dizziness, breast tenderness Requires a prescription

The most effective emergency contraceptive method (99% effective) Can be used as emergency contracep­

High initial cost of insertion Must be inserted by the provider Should test for pregnancy and STls before

tive and contin ued for up to 1 O years of contraception

insertion Cannot be placed during active infection

'None of these methods provides protection from or treatment for H IV or other sexually transmitted infections.

REPRODUCTIVE ENDOCRINOLOGY CONGENITAL ADRENAL HYPERPLASIA CAH is a deficiency of at least one enzyme required for the biochemical syn­ thesis of cortisol from cholesterol (see Fig. 2.11-6 and Table 2.11-7). Includes the following: ■ 2 1-Hydroxylase deficiency: Accounts for ~90% of CAH cases. "Classic" form is most severe and presents as a newborn female with ambiguous genita­ lia and adrenal insufficiency (with or without life-threatening salt wasting). "Nonclassic" is a late-onset form that presents with androgen excess, or it could be asymptomatic. Cannot convert 17-hydroxyprogesterone to 11-deoxycortisol ➔ J, cortisol synthesis ➔ i adrenal stimulation ➔ i ACTH and androgens. ■ 1 1 �-Hydroxylase deficiency: Second most common cause of adrenal hyperplasia. Cannot convert 11-deoxycortisol to cortisol or 11-deoxycorti­ costerone to corticosterone, also leading to i ACTH and androgens.

History/PE Androgen excess: Genital ambiguity, premature pubarche, menstrual irregu­ larity, infertility, hirsutism, acne, and, rarely, a palpable abdominal mass.

0-n

KEY FACT

In CAH, .if the first number in the name of the deficient enzyme is a 1 , it is associated with hypertension. If the second number is a 1 , it is associated with hyperandrogenism.

482

HIGH-YIELD FACTS IN

Cholesterol des molase 3�-hyd roxysteroid dehydrogenase

GYNECOLOGY

Cholesterol (via SIAR•) l

1 l

Pregnenolone -----+-++

l

l l

Progesterone -----+-i,. 17- hyd roxyprogesterone --+--1+ And rostenedione

_d l

Estrone

1

m_ ata e A ro_ Testosterone ___ _s_+-++ Estra diol

11-deoxycorti costerone

11-0-

1

Aldosterone

Angiotensin I I

Anastrozole. exemestane

Aromatase

Cortisone

ZONA GLOM ERULOSA Minera locorticoids

Glycyrrhetinic acid

ZONA FASCICU LATA Glucocorticoids

Capsule 1------l

Cortex

Sa-reductase .__-------+--++ Dihydrotestosterone ( D HT) Finasteride

ZONA RETICULARIS Androgens

PERIPHERAL TISSU E Estrogens, DHT

Adrenal gland

*SIAR: Steroidogenic acute regu latory protein. Rate limiting step in steroid synthesis.

F I G U R E 2 . 1 1 -6.

Glucocorticoid biosynthesis pathway. (Modified with permission from USMLE-Rx.com.)

Diagnosis ■ ■

0-,,. KEY FACT

2 1 -Hydroxylase deficiency can present with hypotension, whereas 1 1 �-hydroxylase and 1 7-hydroxylase deficiencies can present with hypertension caused by accumulation of deoxycorticosterone.

Physical examination 2 1-Hydroxylase deficiency: i i 7-0H progesterone levels (a substrate for 21-hydroxylase). This is part of the newborn screen. Cosyntropin (ACTH) stimulation test - gold standard but not necessary if ii 17-0H. ■ 1 1 1}-H ydroxylase i serum 11-deoxycortisol and deficiency : 11-deoxycorticosterone. ■ Both: Next, assessment of the following levels: ■ Cortisol ➔ decreased. ■ Androstenedione ➔ elevated in 21-hydroxylase and l l l3-hydroxylase deficiency [ right arrow] elevated. Also consider adrenal/ovarian neoplasm. ■ Dehydroepiandrosterone ([DHEA]) ➔ elevated in 21-hydroxylase and l l l3-hydroxylase deficiency [ right arrow] elevated. Also consider adre­ nal neoplasm, Cushing syndrome. ■ If salt wasting: Will also have -!- aldosterone, ,J, sodium, i potassium, and i renin associated with hypovolemia.

GYNECOLOGY TA B L E 2.1 1 -7.

HIGH -YIELD FACTS IN

483

Overview of Congen ital Adrenal Hyperplasia

ENZYME DEFICI ENCY 1 7a-hyd roxylase•

MINERALOCORTICOIDS

i

CORTISOL

t

SEX HORMON ES

t

BP

i

[ K+] t

LABS

t androstenedione

PRESENTATION

XV: Ambiguous geni-

ta lia, undescended testes

XX: Lacks sec-

ondary sexua l development

2 1 -hyd roxylase•

i

t

i

i ren i n activity

i 1 7-hyd roxyprogesterone

Most com mon

Presents in infa ncy (salt wasting) or

child hood (precocious puberty)

XX: Vi ri l ization 1 1 13-hyd roxylase•

t aldosterone

i 1 1 -deoxycortico-

t

i

i

t

t ren i n activity

XX: Vi ri l ization

sterone (resu lts in i BP)

•All congenital adrenal enzyme deficiencies are characterized by a n enlargement of both adrenal glands and hyperpigmentation ca used by i ACTH stim ulation (caused by t cortisol). Modified with permission from Le T et al. First Aid for the USMLE Step 1 2022. New York, NY: McGraw-Hill; 2022.

Treatment ■ Glucocorticoids (eg, dexamethasone). Medical therapy for adrenal and ovarian disorders prevents new terminal hair growth but does not resolve hirsutism. ■ Addition of mineralocorticoid therapy (eg, fludrocortisone) if salt wasting or hypotension is present. ■ Laser ablation, electrolysis, or conventional hair removal techniques for removal of unwanted hair.

POLYCYSTIC OVARIAN SYNDROME A syndrome of excess testosterone and excess estrogen, PCOS has a preva­ lence of 6% to 10% among U. S. females of reproductive age and is the most common cause of infertility in females. Diagnosis requires fulfillment of two of the following three (Rotterdam criteria): ■ Polycystic ovaries (via ultrasonography) ■ Oligo-ovulation and/or anovulation ■ Clinical and/or biochemical evidence of hyperandrogenism

History/PE ■ Common presentation: Obesity (body mass index [BMI] > 30 kg/m2 ) , menstrual cycle disturbances, infertility, acne, androgenic alopecia, and hirsutism from hyperandrogenism ■ Females with PCOS also at i risk for the following: ■ DM type 2: Acanthosis nigricans possibly seen on examination ■ Metabolic syndrome: Insulin resistance, atherogenic dyslipidemia, and hypertension

(J.◊ MNEMONIC

The most severe form of PCOS is

HAIR-AN syndrome:

HyperAndrogenism, Insulin Resistance, and Acanthosis Nigricans.

484

HIGH-YIELD FACTS IN

F I G U R E 2 . 1 1 -1. Polycystic ovary with prominent multiple cysts. ( Reprod uced with permission from DeCherney AH, Nathan R. Current Diagnosis & Treatment: Obstetrics & Gynecology, 1 0th ed. New York, NY: McGraw-Hill; 2007.)

GYNECOLOGY Diagnosis ■ Biochemical testing of hyperandrogenemia: i testosterone ■ i free testosterone more sensitive than total testosterone (total can be normal) because of low sex hormone-binding globulin ■ Excluding other causes of hyperandrogenism: DHEAS to rule out adrenal tumor ■ Pelvic ultrasound to rule out androgen-secreting ovarian tumor ■ 17-Hydroxyprogesterone to rule out nonclassical CAH ■ Screening in the setting of clinical signs of Cushing syndrome (eg, moon facies, buffalo hump, abdominal striae) or acromegaly (eg, i head size) ■ Evaluation for metabolic abnormalities: ■ Two-hour oral glucose tolerance test ■ Fasting lipid and lipoprotein levels (total cholesterol, HDL, LDL, triglycerides) ■ Optional tests: Not necessary if both oligomenorrhea and signs of hyper­ androgenism are present ■ Transvaginal ultrasonography: Look for more than 11 small (2-9 mm), subcapsular follicles forming a "pearl necklace" sign (see Fig. 2.11-7). Seen in roughly two thirds of females with PCOS. ■ Gonadotropins: i LH/FSH ratio (>2:1) ■ 24-hour urine for free cortisol: Adult-onset CAH or Cushing syndrome Treatment ■ Females who are not attempting to conceive: The physician can treat these patients with combined hormonal contraception or progestin :±: an antiandrogen like spironolactone if there are symptoms of hyperandrogen­ ism. Patients with metabolic syndrome or insulin resistance may benefit from the addition of metformin. ■ Females who are attempting to conceive: Letrozole (aromatase inhibitor) :±: metformin is first-line treatment for ovulatory stimulation. Clomiphene (selective estrogen receptor modulator) is second-line. Symptom-specific treatment: ■ Hirsutism: Combination OCPs first line; antiandrogens (spironolac­ tone, finasteride) and metformin ■ Obesity, cardiovascular risk factors, lipid levels: Diet, weight loss (can also help regulate ovulation), and exercise plus potentially lipid­ controlling medication (eg, statins)

0-n

KEY FACT

Combined hormonal contraception or progestin ! the risk for endometrial hyperplasia/carcinoma a mong women with PC0S.

0-n

KEY FACT

Female causes of infertil ity are more com mon than male causes. An investigation should begin with history (including menstrual) and physical examination of the female and then progress to semen analysis before further workup in the female, as semen analysis is simple and noninvasive.

Complications ■ Infertility ■ Miscarriage ■ Type 2 DM ■ Metabolic syndrome ■ i long-term risk for breast and endometrial cancers because of unopposed estrogen secretion

INF ERTILITY Definition: The inability to conceive after 12 months of regular, unprotected sex­ ual intercourse in women < 3 5 years of age ■ The inability to conceive after 6 months of regular, unprotected sexual intercourse in women 2: 3 5 years of age ■ Primary infertility characterized by no prior pregnancies; secondary infer­ tility distinguished by at least one prior pregnancy. Etiologies are shown in Figure 2.11-8 and Table 2.11-8. ■

■

GYNECOLOGY

HIGH -YIELD FACTS IN

485

Primary hypogonadism Ii FSH) 30%-40% Secondary hypogonadism (-l, FSH, j, LHI 2%

Male causes 25%

Disordered sperm transport 10%-20% Unknown 40%-50% Inferti lity

Unexplained 17% Amenorrhea/ ovu latory dysfunction 46%

Female causes 58%

Tu bal defect 38% Endometriosis 9%

Hypothalamic/ pitu itary causes 51% Polycystic ovary syndrome 30% Premature ovarian failure 12% Uterine or outflow tract disorders 7%

Other 7%

F I G U R E 2 . 1 1 -8. Causes of infertility. ( Reproduced with permission from USM LE-Rx.com.) TA B L E 2 . 1 1 -8.

I nfertility Workup

ETIOLOGY

H I STO RY/PE

DIAGNOSIS

TREATMENT

Male factors

Testicular i njury or i nfection

Semen ana lysis

Treatment of hormonal deficiency

Prolactin

Donor insemination

Medications (corticosteroids, cimeti-

TSH

Pituita ry, thyroid, or liver disease

Karyotyping (to rule out Klinefelter

dine, spironolactone)

Signs of hypogonadism

Va ricocele Ovu latory factors

i incidence with age

Sym ptoms of hyperthyroidism/ hypothyroidism

Galactorrhea

Menstrua l cycle a bnorma lities Pituita ry tumors

syndrome)

I ntrauterine insemination (IUI) In vitro fertilization (IVF)

l ntracytoplasmic sperm i njection

Menstrua l history

Treatment depends on the etiology

Ovulation predictor

I n d uction of ovulation with clomi­

Early follicular FSH ± estradiol level (ova ria n

IUI

Basal body tem perature

Midl uteal progesterone reserve)

TSH, prolactin, androgens

(eg, levothyroxine, dopa m i ne) phene, gonadotropins

IVF

Ova rian sonography (antral foll icle cou nt) Endometrial biopsy (l utea l-phase defect)

Tu ba l/pelvic factors

History of PID, a ppendicitis, endo-

metriosis, pelvic ad hesions, tubal

surgery

Hysterosa lpingogram

Potentia l laparoscopy

La paroscopic resection or a blation of endometriomas or fibroids

IVF

Cervica l

Cryothera py, conization, or diethyl-

Physica l exa m

IUI

Uteri ne

Polyps

U ltrasound

Surgical treatment

factors factors

stil bestrol (DES) exposu re in utero

Fibroids

Congenital anomalies

Hysterosa lpingogram

IVF

486

HIGH-YIELD FACTS IN

GYNECOLOGY

MENOPAUSE Cessation of menses for a minimum of 12 months as a result of follicular depletion. H i story/PE ■

The average age of onset is 51 years. Symptoms include hot flashes, pruritus, vaginal dryness caused by vaginal atrophy, insomnia, anxiety/irritability, poor concentration, mood changes, dyspareunia, and loss of libido. ■ "Premature menopause" (also known as premature ovarian insufficiency) is cessation of menses before 40 years of age. Diagnosis

A clinical diagnosis. The following studies are not routine but may be helpful: ■ Labs: i FSH ■ Serum TSH should be measured because of overlap of symptoms and common age of presentation of hypothyroidism and menopause Treatment

Best initial treatment: HRT (combination estrogen and progestin) in symptomatic patients without contraindications 60 and/or greater than 10 years from the menopausal transition ■ Contraindications: Vaginal bleeding, breast cancer (known or sus­ pected), untreated endometrial cancer, history of thromboembolism, chronic liver disease, hypertriglyceridemia, known coronary artery disease ■ Non-HRT (for those with contraindications to HRT listed earlier): Selective serotonin reuptake inhibitors (SSRis)/serotonin-norepinephrine reuptake inhibitors (SNRis), clonidine, and/or gabapentin to .J, the fre­ quency of hot flashes ■ Topical estrogen preparation: Useful for vaginal atrophy; topical estro­ gen preparation does NOT have the same contraindications as systemic HRT ■ Calcium supplements ± bisphosphonates: Useful for osteoporosis; dual­ energy x-ray absorptiometry (DEXA) scan is used to measure bone mineral density (BMD); supplemental treatment includes daily calcium/vitamin D and weight-bearing exercise ■

0-,,. KEY FACT Postmenopausal women should be routinely screened for osteoporosis, starting at 65 years of age or earlier if there are additional risk factors.

GYNECOLOGY

HIGH -YIELD FACTS IN

487

GYNECOLOGIC DISORDERS CYST AND ABSCESS OF THE BARTHOLIN DUCT Obstruction of the Bartholin duct may lead to cyst formation, as mucus con­ tinues to accumulate behind the obstruction, causing cystic dilation. An obstructed Bartholin duct that becomes infected can develop a polymicrobial abscess.

History/PE ■ Cysts: 1 to 3 cm in size, unilateral, and often asymptomatic. Larger cysts lead to periodic, painful swelling and dyspareunia. ■ Clinical diagnosis: Mass at medial labia majora or lower vestibular area on physical examination. ■ Abscess: Extremely painful, warm, fluctuant mass at medial labia majora or lower vestibule with possible cellulitis and fever. Treatment ■ Asymptomatic cysts: No therapy :±: warm soaks. The physician can con­ sider drainage and biopsy if patient >40 years of age to exclude carcinoma. ■ Abscess: Aspiration or incision and drainage to prevent reaccumulation. The physician should order tests for gonorrhea and chlamydia and other pathogens. ■ Antibiotics are unnecessary unless cellulitis or sexually transmitted infec­ tion (STI) is present.

VAGINITIS A spectrum of conditions that cause vulvovaginal symptoms such as itching, burning, irritation, and abnormal discharge. The most common causes are bacterial vaginosis, vulvovaginal candidiasis, and trichomoniasis (see Table 2.11-9).

History/PE ■ Presents with a change in discharge, malodor, pruritus, irritation, burning, swelling, dyspareunia, and dysuria ■ Normal secretions as follows: ■ Midcycle estrogen surge: Clear, elastic, mucoid secretions ■ Luteal phase/pregnancy: Thick and white secretions that adhere to the vaginal wall ■ A thorough examination of the vulva, vaginal walls, and cervix ■ Many WBCs and no organism on saline smear - suspect Chlamydia tra­ chomatis, an intracellular organism

I A 5 6-yea r-o ld wo man presents with presents with i n somnia, vag i n a l d ryness, and lack o f menses fo r 1 3 months. What is the most l i kely d i a g nosis7

488

HIGH-YIELD FACTS IN

TA B L E 2 . 1 1 -9.

GYNECOLOGY

Causes of Vagin itis

VA RIABLE

BACTERIAL VAG I N OSIS

TRICHOMONIASIS

CAN D I DIASIS

Incidence

1 5%-50% (most com mon)

5%-50%

1 5%-30%

Etiology

Not an infection: shift in vaginal flora (i anaerobes such as Gardnerella

Protozoa! flagellates (an STD)

Usually Candida albicans

Pregnancy, multiple sexual partners,

Unprotected sex with multiple

DM, antibiotic use, pregnancy, corti­

vagina/is, -!- lactobacilli)

Risk factors

female sexual partner, frequent douching History Examination

partners

"Fishy" odor, thin homogenous white or gray discharge

i yellow-green discharge, odor,

Mild vulvar i rritation, thin homog­

"Strawberry petechiae" i n the

enous white or gray discharge; pH >4.5

pru ritus, dysuria upper vagina/cervix; pH >4.5

costeroids, HIV, OCP use, frequent intercourse, tight-fitting clothing Pruritus, dysu ria, burning, cottage cheese discharge Erythematous, excoriated vulva/vagina with cottage cheese discharge; pH 4.5 Positive amine (whiff)test

KOH prep

l

Wet mount

Clue cells compose >20% of epithelial cells

Bacterial vag i n o s i s

F I G U R E 2 . 1 1 -9.

Negative amine (whiff) test

Wet mount

Motile trichomonads !

Trichoma niasis

HIGH -YIELD FACTS IN

489

Normal pH (4.0-4.5)

KOH p re p

Negative amine (whiff)test l

Pseudohyphae J

Wet mount

Vu lvova g i n a l ca ndidiasis

Normal l

Normal fluid

Using vaginal pH and wet mount to diagnose common vaginal infections.

(Reproduced with permission from USM LE-Rx.com.)

cultures for Neisseria gonorrhoeae or C trachomatis to rule out cervicitis. NMT is the gold-standard test. ■ Treatment: Etiology specific (see Table 2.11-9).

0-,,. KEY FACT Criteria for the clinical diagnosis of bacterial vaginosis (th ree of four are req uired): ■ Abnormal whitish-gray discharge ■ Vaginal pH >4.5 ■ E0 amine ("whiff") test ■ Clue cells composing >20% of epithelial cells on wet mount

CERVICITIS Inflammation of the uterine cervix. Etiologies are as follows: Infectious (most common) : ■ C trachomatis and N gonorrhoeae are most common. ■ C trachomatis is more common than N gonorrhoeae in mucopurulent cervicitis. ■ Trichomonas and herpes simplex virus (HSY) are less common causes of cervicitis. ■ Noninfectious: Trauma, radiation exposure, malignancy ■ All sexually active women cervical Number of deaths: Ovarian > endometrial > cervical

504

HIGH-YIELD FACTS IN

GYNECOLOGY TA B L E 2 . 1 1 - 1 4.

F I N DING

Benign vs Malignant Pelvic Masses

BEN I G N

MALIGNANT

EXAM INATIO N: PELVIC MASS Mobil ity

Mobile

Fixed

Consistency

Cystic

Solid or fi rm

Cul-de-sac

Smooth

Nodular

TRANSVAG INAL U LTRASONOGRAPHV: ADNEXAL MASS

■

Size

8 cm

Consistency

Cystic

Solid or cystic and solid

Septations

U n ilocular

M u ltilocu lar

Location

U n i lateral

Bilateral

Other

Calcifications

Ascites

Table 2.11-14 differentiates the benign and malignant characteristics of pelvic masses.

Diagnosis

0-,,. KEY FACT

Granulosa cell tumors predispose to endometrial hyperplasia and carcinoma because of unopposed estrogen secretion.

Tumor markers (see Table 2.11-1 5): i CA-125 is associated with epithelial cell cancer (90% of ovarian cancers), but is used only as a marker for pro­ gression and recurrence. ■ Premenopausal women: i CA-125 may point to benign disease such as endometriosis or a tubo-ovarian abscess (TOA). ■ Postmenopausal women: i CA-125 (>3 5 units) indicates an i likeli­ hood that the ovarian tumor is malignant. ■ Transvaginal ultrasonography: Used to screen high-risk patients and as the first step in the workup of symptomatic females (eg, pelvic fullness, pelvic pain). A solid mass with thick septations :±: ascites on ultrasound is highly suggestive of neoplasm.

0-,,. KEY FACT

Treatment

Any ovarian or adnexal mass in a premenarchal or postmenopausal patient is suggestive of an ovarian neoplasm.

Ovarian masses in premenarchal females: Masses > 2 cm in diameter require close clinical follow-up and often surgical removal. Ovarian masses in premenopausal females: Observation is appropriate for asymptomatic, mobile, unilateral, simple cystic masses 8 to 10 cm in diameter and those that are complex and/or unchanged on repeat pelvic examinations and ultrasonography. ■

Ovarian masses in postmenopausal females: Closely follow with ultrasonography asymptomatic, unilateral simple cysts < 5 cm in diameter with a normal CA-125. Surgically evaluate palpable masses.

GYNECOLOGY TA B L E 2 . 1 1 - 1 s .

Ovarian Tu mor Characteristics

TUMOR

MARKER

CHARACTERISTICS

Epithelial

CA- 1 25

Serous adenocarcinoma-the most common. A tumor may present with abdominal distention, bowel obstruction, and adnexal mass.

Endodermal sinus (yolk sac)

a-Fetoprotein (AFP)

Embryonal carcinoma

AFP, 13-hCG

Choriocarcinoma

13-hCG

Very aggressive. An endodermal sinus may be seen in ovaries and/or sacrococcygeal area in young children; gross examination shows a yellow, friable, solid mass. Very rare. Embryonal carcinoma may be seen in ado­ lescents; it may present with precocious puberty and abnormal uterine bleeding. Can develop during or after pregnancy and after molar pregnancies. This is a malignancy of tropho­ blastic tissue. Choriocarcinoma may be associated with bilateral theca-lutein cysts. It spreads hematogenously.

Dysgerminoma

Lactate dehydro­ genase (LDH)

Most commonly seen i n adolescents with sheets of uniform "fried egg" cells.

Gra nulosa cell

l nhibin

Most common malignant stromal tumor. Granulosa cells a re often seen i n females in their SOs; produc­ tion of estrogen and/or progesterone may lead to postmenopausal bleedi ng, Cal l-Exner bodies on histology.

Ovarian cancer treatment: Surgery: Surgical staging: Hysterectomy/BSO with omentectomy and pelvic and paraaortic lymphadenectomy ■ Benign neoplasms warrant tumor removal or unilateral oophorectomy ■ Perioperative chemotherapy: Routine except for females with early-stage or low-grade ovarian cancer ■ Radiation therapy: Reserved for advanced germ cell tumors, in particular dysgerminomas (not effective in epithelial ovarian cancer) ■

■

Prevention ■ Females with the BRCAl gene mutation should be screened every 6 months with ultrasonography and CA-125 testing. Prophylactic oophorec­ tomy is recommended by 40 years of age or whenever childbearing is completed. ■ OCP use ,J, the risk for ovarian cancer. ■ There is no routine screening for ovarian cancer.

HIGH -YIELD FACTS IN

SOS

506

HIGH-YIELD FACTS IN

GYNECOLOGY

U ROLOGIC GYN ECOLOGY PELVIC ORGAN PROLAPSE Risk factors for pelvic organ prolapse include multiple vaginal deliveries, genetic predisposition, advancing age, prior pelvic surgery (hysterectomy), connective tissue disorders, and i intra-abdominal pressure associated with obesity or straining with chronic constipation.

H istory/PE ■ Presents with the sensation of a bulge or protrusion in the vagina (see Fig. 2.11-16). ■ Urinary or fecal incontinence, a sense of incomplete bladder emptying, and/or dyspareunia are also seen. Diagnosis The degree of prolapse can be evaluated by having the patient perform the Valsalva maneuver while in the lithotomy position. Treatment ■ Supportive measures include a high-fiber diet, weight reduction in obese patients, and limitations on straining and lifting. ■ Pessaries may reduce prolapse and are helpful in patients who do not wish to undergo surgery or in those for whom surgery is contraindicated. ■ Surgical procedure depends on where the prolapse is located: ■ Uterine prolapse is treated by a hysterectomy with vaginal vault suspens10n. ■ Anterior/posterior vaginal wall: A prolapse here calls for repair of the anterior or posterior wall (colporrhaphy).

Normal

Uterine prolapse

Marked prolapse /I� (procidentia) · ,. �

F I G U R E 2 . 1 1 - 1 6.

Uterine prolapse. Diagrams depicting different degrees of uterine prolapse.

(Reproduced with permission from USMLE-Rx.com.)

GYNECOLOGY

SEXUAL DISORDERS GENITOPELVIC PAIN DISORDER (VAGINISMUS) Involuntary spasm of outer one third of the vagina

History/PE ■ Some caused by situational/psychosocial causes ■ Can be associated with gynecologic disorders, chronic medical conditions, or certain medications Treatment ■ Most effective - cognitive and behavioral psychotherapy with systematic desensitization ■ Deep muscle relaxation techniques and dilators

VULVODYNIA Localized pain of the vulva, usually pain with insertion/at introitus.

History/PE Typically chronic and idiopathic Treatment ■ At start of treatment: Detailed history and physical examination ■ Multidisciplinary treatment with pelvic floor physical therapy and counseling

FEMALE SEXUAL INTEREST/AROUSAL DISORDER Inability to complete sexual activity

History/PE ■ Common in patients with depression or chronic disease ■ Common in younger patients due to situational circumstances or depression Treatment ■ Usually resolves when underlying condition or medication is adjusted ■ Individual or couples psychotherapy

HIGH -YIELD FACTS IN

507

508

HIGH-YIELD FACTS IN

GYN ECO LOGY NOTES

PEDIATRICS Child Development DEVELOPMENTAL MILESTONES PRIMITIVE REFLEXES GROWTH SEXUAL DEVELOPMENT

Genetic Disease (YSTIC FIBROSIS

Neonatol ogy APGAR SCORING (ONGENITAL MALFORMATIONS NEONATAL JAUNDICE RESPIRATORY DISTRESS SYNDROME GERMINAL MATRIX HEMORRHAGE APNEA OF PREMATURITY NEONATAL EXTRACRANIAL INJURIES (ONGENITAL HYPOTHYROIDISM BENIGN NEONATAL RASHES NEONATAL ABSTINENCE SYNDROME

Congen ita l Heart Disease ACYANOTIC (ONGENITAL HEART LEFT-TO-RIGHT SHUNTS SEPTAL DEFECTS PATENT DuCTUS ARTERIOSUS (OARCTATION OF THE AORTA CYANOTIC CONGENITAL HEART RIGHT-TO-LEFT SHUNTS TRANSPOSITION OF THE GREAT ARTERIES TETRALOGY OF FALLOT

Ped iatric Gastroi ntestinal Disease PYLORIC STENOSIS INTUSSUSCEPTION MALROTATION WITH VOLVULUS MECKEL DIVERTICULUM H1RSCHSPRUNG DISEASE NECROTIZING ENTEROCOLITIS FOOD PROTEIN-INDUCED ALLERGIC PROCTOCOLITIS PEDIATRIC (ONSTIPATION

51 1

51 1 51 1 51 1 514

514

514

520

520 520 520 524 526 526 526 526 527 527

528

529 529 53 1 53 1 532 532 533

534

534 535 536 536 537 538 538 539

Ped iatric U rology VESICOURETERAL REFLUX (RYPTORCHIDISM INGUINAL HERNIA HYPOSPADIAS AND EPISPADIAS

Ped iatric I m m u nology IMMUNODEFICIENCY DISORDERS KAWASAKI DISEASE JUVENILE IDIOPATHIC ARTHRITIS

Ped iatric I nfectious Disease ACUTE OTITIS MEDIA BRONCHIOLITIS (ROUP (LARYNGOTRACHEOBRONCHITIS) EPIGLOTTITIS MENINGITIS

OCULAR INFECTIONS OF THE NEONATE PERTUSSIS (WHOOPING (OUGH) VIRAL EXANTHEMS TO RCH INFECTIONS PINWORM INFECTION NEONATAL FEVER

( 20 seconds in premature infants (gestational age girls). Etiology is unclear in most children. Risk fac­ tors include conditions with potential lead points, including Meckel diverticu­ lum, intestinal lymphoma (>6 years of age), submucosal hematoma (as in Henoch-Schonlein purpura), polyps, and CF (lead point is inspissated stool). An antecedent viral GI illness or upper respiratory infection (URI) is seen in many children, which may cause formation of a lead point through enlarge­ ment of Peyer patches (lymphatic tissue in the bowel). There is a small risk of intussusception after the oral rotavirus vaccine.

History/PE ■ Intussusception presents with abrupt-onset, episodic abdominal pain in appar­ ently healthy children, often accompanied by flexed knees and vomiting. The child may appear well between episodes if intussusception is released. ■ The classic triad involves severe abdominal pain, vomiting (initially non­ bilious and then bilious as obstruction develops), and bloody mucus in stool ("currant jelly stool," a late finding). However, this classic triad is only present in one third of patients. ■ During examination, the physician should look for abdominal tenderness, a EB stool guaiac test, a palpable "sausage-shaped" right upper quadrant (RUQ) abdominal mass, and "empty" right lower quadrant (RLQ) on pal­ pation (Dance sign).

I A 4-week-o ld boy, born at term, is bro u g ht to the e m e rg e ncy department after experiencing vo m iti n g of i n creasing freq u e n cy a n d i ntens ity fo r the past wee k. H i s pa rents state that he now vom its fo rcefu l l y after every meal and enthusi astica l ly attem pts to eat i m m ed i ately after vo m iti ng. The infa n t appea rs l etha rgic, with s u n ke n fo nta n e l l es a n d d ecreased s k i n tu rgor. Th e abdomen is soft, nontender, a n d nond istended; n o masses a re felt. What is the most l i kely cause of t h i s infa nt's sym pto m s ?

536

HIGH-YIELD FACTS IN

PEDIATRICS Diagnosis/Treatment ■ Ultrasonography is the initial test of choice and may show a "target sign" (see Fig. 2.12- l 0B). An ultrasound must be conducted during a painful episode to diagnose intussusception. ■ X-rays of the abdomen are often normal early in the disease, but later they may show small bowel obstruction, perforation, or a soft tissue mass. ■ The physician should correct any volume or electrolyte abnormalities, check CBC for leukocytosis, and consider placement of a nasogastric (NG) tube for decompression. ■ High clinical suspicion calls for an air insufflation enema without delay, as it is diagnostic and curative in the vast majority of patients. ■ Surgical resection is indicated if the child has peritoneal signs, air insufflation enema reduction is unsuccessful, or a pathologic lead point is identified. ■ Air insufflation enema is preferred over water or barium-contrast enema for diagnosis and management of intussusception, as it is faster and carries a lower risk for complications.

MALROTATION WITH VOLVULUS Congenital malrotation of the midgut results in abnormal positioning of the small intestine (cecum in the right hypochondrium) and formation of fibrous bands known as Ladd bands (Fig. 2.12-11), which predispose to obstruction and volvulus with constriction of blood flow.

F I G U R E 2.1 2-1 1 .

Ladd bands. (Reproduced

with permission from USMLE-Rx.com.)

History/PE ■ Often presents in the first month of life with bilious emesis, crampy abdominal pain, distention, and passage of blood or mucus in the stool. ■ Postsurgical adhesions can lead to obstruction and volvulus at any point in life. Diagnosis ■ Barium contrast enema may reveal the characteristic narrowed "bird-beak" appearance and air-fluid levels, but may also appear normal. ■ Upper GI series is the study of choice if the patient is stable and shows an abnormal location of the ligament of Treitz. Ultrasound may be used, but sensitivity depends on the user's experience.

Meckel d iverticulum

Small x i ntestine

F I G U R E 2.1 2-1 2.

Meckel diverticulum.

(Mod ified with permission from USMLE-Rx.com.)

This i nfa nt is most l i kely suffe r i n g from pyloric stenosis, a n o bstru ction of the gastric outlet secondary to hypertrophy and hyperplasia of the m u s c u l a r laye rs of the pyl orus. N ote that some i nfa nts, but n ot a l l , m a y prese nt w i t h a n ol ive-s h a ped a bdo m i n a l mass.

Treatment ■ NG tube insertion to decompress the intestine; IV fluid hydration ■ Emergent surgical correction is needed when there is ischemic bowel/GI tract; definitive management is surgical (Ladd's procedure)

MECKEL DIVERTICULUM Caused by failure of the omphalomesenteric (or vitelline) duct to obliterate, resulting in the formation of a true diverticulum containing all three layers of the small intestine (Fig. 2.12-12). Some Meckel diverticula have heterotopic gastric tissue, which places patients at risk for intestinal ulceration and pain­ less hematochezia. This is the most common congenital abnormality of the small intestine, affecting up to 2% of children (boys > girls).

History/PE Typically asymptomatic and often discovered incidentally. Patients most commonly symptomatic 14 days postbirth) Chediak-Higashi syndrome

An a utosomal recessive disorder that leads to a defect in neutrophil che­ motaxis/microtubule polymerization The syndrome includes partial ocu­ locutaneous albinism, peripheral neuropathy, and neutropenia

Job syndrome (hyperimmuno­ globu lin E syndrome)

A defect in neutrophil chemotaxis Remember the mnemonic FATED: Coarse Facies

ii incidence of overwhelming

pyogenic infections with S pyo­ genes, S aureus, and Pneumococcus

tosis (particularly neutrophilia) BMT is cu rative Giant granules in neutrophils BMT is the treatment of choice

species

Recurrent S aureus i nfections and abscesses

Treatment with penicillinase-resis­ tant a ntibiotics and IVIG

Can lead to life-threatening airway edema

Total hemolytic complement (CHSO) to assess the quantity and func­ tion of complement Purified Cl inhibitor (Cl INH) con­

Abscesses (5 aureus) Retained primary Teeth Hyper-lgE (eosinophilia) Dermatologic (severe eczema)

COMPLEMENT DISORDERS Cl esterase inhibitor deficiency (hereditary a ngioedema)

An a utosomal dominant disorder with recurrent episodes of angioedema lasting 2-72 hours and provoked by stress or trauma

centrate and fresh frozen plasma (FFP) can be used before surgery Terminal comple­ ment deficiency (CS-C9)

I nability to form membrane attack complex

Recurrent Neisseria infections, meningococcal, or gonococcal Rarely, patients have lupus or glomerulonephritis

■ B-cell deficiencies: Most common (50%). Typically present after 6 months of age with recurrent sinopulmonary, GI infections, and/or with encapsu­ lated organisms (Haemophilus infiuenzae, Streptococcus pneumoniae, Neis­ seria meningitidis) . Treatment calls for intravenous immunoglobulin (IVIG), except for cases of IgA deficiency. ■ Bruton agammaglobulinemia can be confused with transient hypogam­ maglobulinemia of infancy (THI), as both are characterized by f sus­ ceptibility to infections at ~6 months of age, when transplacental maternal IgG is no longer active. B cells are ,J, in Bruton, whereas those in THI are normal. Bruton agammaglobulinemia and common variable immunodefi­ ciency (CVID) also have similar symptoms. However, the former is found in boys ~6 months of age, whereas CVID is seen in older males and females (15-3 5 years of age), and its symptoms are less severe.

Meningococcal vaccine and appro­ priate antibiotics

546

0-n

HIGH-YIELD FACTS IN KEY FACT

Flashback to immunology: ■ B cells make immunoglobulins and are responsible for immunity against extracellular bacteria. ■ T cells are responsible for immunity against intracellular bacteria, viruses, and fungi.

PEDIATRICS T-cell deficiencies: Tend to present earlier (1-3 months of age) with opportunistic and low-grade fungal, viral, and intracellular bacterial infec­ tions (eg, mycobacteria). Secondary b-cell dysfunction can also be seen. ■ Phagocyte deficiencies: Characterized by mucous membrane infections, abscesses, and poor wound healing. Infections with catalase EB organisms (eg, S. aureus), fungi, and gram 8 enteric organisms are common. ■ Complement deficiencies: These deficiencies are characterized by recur­ rent bacterial infections with encapsulated organisms. ■

KAWASAKI DISEASE

0,0 MNEMONIC

Comp lications: Untreated children may develop coronary artery aneurysms (25 % ); all patients should be assessed by echocardiography at diagnosis.

Kawasaki disease symptoms­

An acute multisystem medium-vessel vasculitis that primarily affects young children. Usually affects children age < 5 years (I incidence in people of Jap­ anese and Korean descent). Kawasaki disease is divided into acute, subacute, and convalescent phases.

CRASH and BURN Conjunctivitis (without discharge)

Rash

Adenopathy (unilateral and > 1 .5 cm) Strawberry tongue Hands and feet (red, swollen, flaky skin) BURN (fever >40°( [> 1 04° F) for 2:s days)

0-n

KEY FACT

0-n

KEY FACT

Untreated Kawasaki disease can lead to coronary aneurysms in up to 25% of patients.

Kawasaki disease and scarlet fever may both present with "strawberry tongue;' rash, desquamation of the hands and feet, and erythema of the mucous membranes. However, children with scarlet fever have normal lips and no conjunctivitis.

0-n

KEY FACT

ASA is used for Kawasaki disease in the pediatric population, despite fear of Reye syndrome, a rare but serious condition. Although the exact mechanism is unclear, Reye syndrome results from mitochondrial injury and fatty degenerative liver failure, which leads to hyperammonemia and ultimately encephalopathy.

H istory/PE

Five days of fever and at least four of the following five criteria: 1. Conjunctivitis: Bilateral, nonexudative, painless with limbal sparmg (acute phase) 2. Oral mucosal changes: Erythematous mouth/pharynx, "strawberry tongue," and/or cracked lips (acute phase) 3. Rash: Primarily truncal, polymorphous, erythematous (acute phase) 4. Peripheral extremity changes: Edema of hands and feet, palmar erythema (acute phase), and desquamating palms and soles (convalescent phase) 5. Cervical lymphadenopathy ( > 1. 5 cm): Generally painful and unilateral (acute phase) Other manifestations (not required for diagnosis) include sterile pyuria, gall­ bladder hydrops, hepatitis, and arthritis, hyponatremia, and hypoalbuminemia Diagnosis ■

Laboratory workup: Normochromic anemia, leukocytosis with left shift, thrombocytosis, increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). The physician should obtain a baseline echocardiogram at diagnosis for lon­ gitudinal follow-up of coronary artery morphology. Follow-up of uncompli­ cated cases should occur at 2 weeks and 6 to 8 weeks after diagnosis.

Treatment ■

Best initial treatment: High-dose aspirin (acetylsalicylic acid [ASA]), for anti-inflammatory, antipyretic, and antithrombotic effects and IVIG to reduce the risk of coronary artery aneurysms from 2 5 % to < 5 %. ■ Low-dose ASA is then continued until normalization of laboratory inflam­ matory markers. Aspirin is continued if children develop coronary aneu­ rysms. Although young children taking ASA are at risk for Reye syndrome, the risk/benefit favors treatment with ASA to prevent coronary artery aneurysms. ■ Corticosteroids may be used in !VIG-refractory cases, but routine use is not recommended.

PEDIATRICS TA B L E

2.1 2-1 9.

HIGH-YIELD FACTS IN

547

Juvenile Idiopathic Arthritis Subtypes

SUBTYPE

PRESENTATION

RF A N D A N A STATUS

N OTES

Oligoarth ritis JIA

I nvolves four or fewer joi nts (usual ly weight-

Antin uclear a ntibody (ANA) EEl

This is the most com mon

bea ring); large joi nts com monly affected; no

systemic sym ptoms

Rheumatoid factor (RF) 0

presentation of JIA

Oligoarth ritis is usually

Uveitis common; req uires slitla m p examination

diagnosed in young

girls

for diagnosis

Polyarthritis JIA

Present i n five or more joints; genera l ly

RF positivity ra re (indicates severe

Systemic symptoms ra re

Young children with milder disease may

symmetric

disease)

be ANA EEl though this is associated

Rheu matoid nodules may

be seen i n children with RF-positive d isease

with i risk of developi ng uveitis Systemic JIA

I nvolves one or more joints; recurrent, quotid ian high fever (> 39°C [> 1 02.2 ° F])

Hepatosplenomegaly

ANA 0 RF 0

Joint i nflam mation may not occur for months

to yea rs after systemic sym ptoms appear

Lymphadenopathy

Salmon-colored macular rash Enthesitis-related

Macrophage activation syndrome: A com mon

ANA 0

Onset occu rs in boys

Psoriatic arth ritis

Presents with a rth ritis and/or enthesitis

ANA EEl

Females a re com monly

a rth ritis

life-th reatening complication

Sacroiliac joint tenderness or l u m bosacra l infl a m matory pain

RF 0 RF 0

>6 yea rs of age

affected

Acute a nterior uveitis

History of human leu kocyte a ntigen (H LA)­ B27-associated d isease

U n differentiated a rth ritis

Children with psoriasis and a rth ritis OR

Children with arth ritis and two of the fol­

lowing: Psoriasis i n a fi rst-degree relative,

dactylitis, and nail pitti ng or onycholysis

Does not meet/overlap criteria of a ny of the su btypes

JUVENILE IDIOPATHIC ARTHRITIS An autoimmune disorder manifesting as arthritis with "morning stiffness" and gradual loss of motion that is present for at least 6 weeks in a patient < 1 6 years of age. Formerly known as j uvenile rheumatoid arthritis (JRA). Approximately 9 5 % of cases are resolved by puberty. This disorder is more common in girls than in boys . The most common clinical and laboratory findings of different subtypes of juvenile idiopathic arthritis (JIA) are described in Table 2. 1 2- 19. Diagnosis

See Table 2.12- 19.

A 2-yea r-ol d boy is broug h t to the pediatrician fo r a skin i n fection that sta rted on h i s c h i n a n d ra p i d l y s p read to i nvolve much of his face and neck. Th is is his t h i rd such i n fection this yea r, a n d h e i s consta ntly plagued by sinus i nfections and bouts of p n e u m o n i a . The re is no fa m i l y h i story o f recu rrent infections. Th e patient a ppea rs u n comforta b l e, a n d dermatologic exa m i s nota ble fo r eros ions coated i n ye l low crust that a re widespread across the pati ent's face a n d neck. He a l so has patchy white pigmentati on of the s ki n , l i g h t blonde h a i r, a n d bl ue eyes. W h a t i s the most l i kely d i a g n osis ?

548

HIGH-YIELD FACTS IN

PEDIATRICS Treatment ■ ■

Best initial treatments: NSAIDs and strengthening exercises Corticosteroids (for myocarditis) and immunosuppressive medications (methotrexate, anti-tumor necrosis factor agents such as etanercept) are second-line agents

PEDIATRIC INFECTIOUS DISEASE ACUTE OTITIS MEDIA A suppurative infection of the middle ear cavity that is common in children. Sixty percent of children will develop one or more episodes of AOM before 4 years of age. Common pathogens include S pneumoniae; nontypeable H infiuenzae; Moraxella catarrhalis; and viruses such as influenza A, respiratory syncytial virus (RSV), and parainfluenza virus. H istory/PE

Symptoms include ear pain, fever, crying, irritability, difficulty feeding or sleeping, vomiting, and diarrhea. Young children may tug on their ears. Risk Factors

Family history, day care, and tobacco smoke and air pollution exposure. Rate peaks between 6 and 12 months of age. ■ Protective factors: Breastfeeding, oral xylitol. ■

Diagnosis

Diagnosis is made clinically. Otoscopic examination reveals an erythematous tympanic membrane (TM) effusion, bulging, or retraction of the TM and -l, TM mobility (test with an insufflator bulb). Viral causes may result in serous otitis media with blue-gray bulging membranes. Serous otitis media is the presence of effusion without active infection. Examination shows a dull TM. Treatment

Best initial treatment: For mild cases of unilateral otitis media in children >6 months of age, treatment options include supportive care (pain and fever control) and close follow-up rather than antibiotics. ■ If antibiotics are used, the physician can prescribe high-dose amoxicillin (80-90 mg/kg/day) for 10 days for empiric therapy. Patients with recent amoxicillin use and those with resistant or recurrent cases may require amoxicillin/clavulanic acid. If patient is allergic to penicillin, the physi­ cian can consider cephalosporin (mild delayed reaction) or azithromycin (immediate serious or delayed reaction). ■ Complications include TM perforation, mastoiditis, meningitis, cholestea­ tomas, and chronic otitis media. Recurrent otitis media can cause hearing loss with resultant speech and language delay. Chronic otitis media may require tympanostomy tubes. ■

Th is child most l i kely has Ched iak­ H igashi synd rome, cau sed by a utosomal recessive defects in the synthesis/mai ntenance of storage g ra n u les i n a n u m ber of cel l types (including leu kocytes, platelets, neutroph i ls, and melanocytes). I n add ition to partia l ocu l ocuta neous a l bi n ism, these patients experience hepatosplenomegaly and recu rrent, serious i nfections of the skin and respi ratory tract by 5 aureus, Streptococcus pyogenes, and Pneumococcus species. Chediak-Higashi synd rome is often fata l i n child hood beca use of overwhe l m i n g i nfection.

PEDIATRICS BRONCHIOLITIS

HIGH -YIELD FACTS IN

549

0-,r KEV FACT

An acute inflammatory illness of the small airways of the lower respiratory tract that primarily affects infants and children 4 months of age. Treatment Treatment should begin early even though the condition may resolve on its own before 2 weeks of age. ■ 60 degrees ■ 6 to 1 8 months: Spica cast ■ > 1 8 months: Open reduction followed by spica cast

Barlow test

(hip initially reduced)

Unilateral ex.tension of ingui

Ortolani test

(hip initially dislocated)

Developmental dys­ plasia of the hip. (Reprod uced with perm ission F I G U R E 2 . 1 2-26.

from USMLE-Rx.com.)

576

HIGH-YIELD FACTS IN

PEDIATRICS Complications ■

Joint contractures and AYN of the femoral head Without treatment, significant disability

LEGG - CALVE -PERTHES DISEASE Idiopathic AYN and osteonecrosis of the femoral head (see Fig. 2.12-27). Most common in boys 4 to 10 years of age. Can be a self-limited disease in younger patients, with symptoms lasting < 18 months. H i story/PE

Generally asymptomatic at first. Patients can develop a painless limp, anta­ lgic gait, and thigh muscle atrophy. ■ Pain sometimes present. If it is present, it can be in the groin or anterior thigh, or it may be referred to the knee. ■ Limited abduction and internal rotation; atrophy of the affected leg. Usually unilateral (8 5 %-90%). ■

Diagnosis

Initial x-rays can be normal but later can show a flattened and fragmented femoral head. Treatment

Observation is sufficient if limited femoral head involvement or if full range of motion (ROM) is present. ■ If extensive necrosis or ,J, ROM, the physician can consider bracing, hip abduction with a Petrie cast, or an osteotomy. ■ The prognosis is favorable if the patient is 10 degrees (Cobb angle). It is sometimes associated with kyphosis or lordosis. Most commonly idiopathic. Develops in early adolescence. Other etiologies are congenital or associated with neuro­ muscular, vertebral, or spinal cord disease. The male-to-female ratio is 1:7 for curves that progress and require treatment.

F I G U R E 2.1 2-28. Slipped capital femoral epiphysis. Frog-leg anteroposterior x-ray

demonstrates medial and inferior dis­ placement of the right femoral epiphysis (red arrow) relative to the femoral neck. In comparison, the left side (blue arrow) is normal. (Reprod uced with permission from USMLE-Rx.com.)

History/PE Idiopathic disease is usually identified during school physical screening. Vertebral and rib rotation deformities are accentuated by the Adams forward bending test.

■ ■

Diagnosis X-rays of the spine (posterior, anterior, and full-length views). Treatment ■ Monitoring every 6 months for 10 to 30 degrees of curvature. Spinal bracing for 2: 30 degrees of curvature in patients with remaining growth. Curvature may progress even with bracing. ■ Surgical correction for 2:40 to 50 degrees of curvature. Compl ications Severe scoliosis can create restrictive lung disease.

CHILD ABUSE Also known as nonaccidental trauma (NAT); includes neglect and physical, sex­ ual, and psychological maltreatment of children. The physician should suspect abuse if the history is discordant with physical findings or if there is a delay in obtaining appropriate medical care. Certain injuries in children, such as retinal hemorrhages and specific fracture types, are highly suspicious for abuse.

History/PE Abuse should be suspected if the history is not consistent with the injury pattern or with the child's developmental age - for example, if parents claim their 2-month-old child "rolled off the couch" (developmentally, 2-month-olds cannot roll yet). Abuse also should be suspected if the his­ tory continually changes or is vague. ■ Look for skin bruising patterns (indicative of the object used), bruises of different ages and color, and burns that are well circumscribed. ■

0-n

KEY FACT

Suspect sexual abuse if there is genital trauma, bleeding, discharge, or if children have an excessive preoccupation with or knowledge of adult sexual behavior. Vaginal foreign body may be an alternative diagnosis.

578

HIGH-YIELD FACTS IN

TA B L E 2 . 1 2-3 1 .

PEDIATRICS

Common Presentations and Mim ics of Child Abuse

TYPE OF ABUSE

PRESENTATION/IMAG I N G FINDI NGS

M I M ICS

Bruises

Most com mon physical finding

Congenital dermal melanocytosis, formerly called "Mongolian spots"

Often located on head and torso

Burns

May be in pattern reflecting implement (hand, belt)

Coining/cupping (a therapy used in certain cultures where suction cups are attached to the skin) Bleeding diathesis

Contact burns: Cigarette/curling iron

Scalded skin syndrome, severe contact dermatitis,

I mmersion burns: Hot water; on buttocks, with sparing of flexor surfaces, or stocking-glove distribution with sharp lines of demarcation and uniform burn depth Fractures

Spiral fractures: H u merus/femur (in children not yet wal king); epiphyseal-metaphyseal "bucket" fractures

accidental burn injury

Osteogenesis imperfecta (blue sclerae, hearing loss, opalescent teeth)

Posterior rib fractures: Indication of squeezing Abusive head trauma

Lethargy, feeding difficulty, apnea, seizures, retinal hemorrhage, subdural/epidu ral hematoma

Accidental head trauma

I mage reproduced with permission from Wolff K et al. Fitzpatrick's Dermatology in General Medicine, 7th ed. New York, NY: McGraw-Hill; 2008.

■

0-,,. KEY FACT

0steogenesis imperfecta (01) is a great mimicker of child abuse and is often tested. It is a genetic disease that affects type I collagen. Those with 01 can present with a broad spectrum of clinical features-most classically blue sclera, easy bruising, opalescent teeth, conductive hearing loss, skeletal anomalies, and easily fractured bones.

0-,,. KEY FACT

In infants, Neisseria gonorrhoeae isolated on a vaginal culture is definitive evidence of sexual abuse. Chlamydia is not, because it can be acquired from the mother during delivery and can persist for up to 3 years.

■

■ ■ ■

Injuries with high specificity for child abuse include posterior rib frac­ tures, metaphyseal corner "bucket handle" fractures, and spiral fractures of long bones such as the femur (but only before the child can walk, as a spi­ ral fracture may be developmentally appropriate in a walking child). Risk factors: These include parents with a history of abuse as a child, partner violence, and/or a history of alcohol or drug use; premature chil­ dren; children with complex medical problems; infants with colic (excessive crying for > 3 hours per day for > 3 days per week); and repeated hospitalizations Infants: Abuse or neglect in infants may present as apnea, seizures, feed­ ing intolerance, excessive irritability, somnolence, or FIT. Older children: Neglect in older children may present as poor hygiene or behavioral abnormalities. See Table 2.12-31 for exam findings.

Diagnosis ■ An x-ray skeletal survey can show fractures in various stages of healing. X-rays may not show fractures until 1 to 2 weeks after injury (although they may show evidence of prior trauma in children 40 lb]). No solid foods should be given before 6 months of age; solid foods should then be introduced gradually and one at a time. Do not give cow's milk before 12 months of age (increases risk of iron-deficiency anemia). Syrup of ipecac (an emetic) is no longer routinely recommended for acci­ dental poisoning. Poison control should be contacted immediately for assistance. Other guidance important to mention in a well-child visit includes the following: ■ Children should wear helmets when riding bicycles. ■ Parents should ensure that firearms are not loaded and are locked away. ■ Smoke detectors should be present and in working order. Intake of sugar-sweetened drinks and juices is strongly discouraged; juice should be avoided before 12 months of age. ■ Safe sex education should be provided for adolescents. ■ Screen time should be limited for children, as hands-on learning is essential for children younger than 24 months.

HEARING AND VISION SCREENING Objective hearing screening (otoacoustic emissions and/or auditory brain­ stem response) for newborns before discharge is a standard of care. Chil­ dren who fail these screening tests require further diagnostic workup. ■ Objective hearing screening is indicated for children with a history of meningitis, TORCH infections, measles and mumps, and recurrent otitis media. The most common cause of childhood conductive hearing loss is repeated ear infections. ■

0-,,. KEY FACT S I DS: Sudden i nfa nt death syn d rome. Most com m o n cause of u n expla i n ed death i n c h i l d ren < 1 yea r of age. U n known pathogenesis. BRU E: Brief resolved u nexp l a i ned eve nt. < l m i n ute i n d u ration with no known ca use. May present with a n abru pt change i n respiration, ! res ponsiveness, cya nosis, a n d c h a n g e i n m uscle tone. No longer thoug ht to be a precu rsor of SI DS.

•

A mother presents with h e r p reviously hea lthy 3-mo nth-o ld i nfa nt boy, stating that he has been i n c rea s i n g l y difficu lt t o rouse for the p a s t 4 h o u rs and has l ost i nte rest i n feed i ng; she l eft the ba by alone with her boyfriend while s h e ra n erra nds. Wh i l e en route to the hospita l, the ba by sto pped breath i n g . PE is nota b l e for occ i p ital bru i s i n g . What is the most l i kely cause of t h i s c h i l d 's a p nea?

580

HIGH-YIELD FACTS IN

PEDIATRICS ■

■

0-n KEY FACT Leu kocoria i n d icates reti noblastoma, congen ita l cata racts, or retinopathy of prematu rity. Al l cases of leu kocoria req u i re i m med iate ophthal mologic workup.

■

The red reflex should be checked at birth. Leukocoria is the lack of a red reflex and can indicate the presence of retinoblastoma. It can also be an incidental finding in a baby's first photos. Vision screening to detect strabismus (ocular misalignment), amblyopia (suppression of retinal images in a misaligned eye, leading to permanent vision loss), and other conditions should be performed at all health visits in children < 5 years of age. Strabismus is normal until 3 months of age; beyond 3 months of age, chil­ dren should be evaluated by a pediatric ophthalmologist and may require corrective lenses, occlusion, and/or surgery to prevent amblyopia. Treat­ ment of strabismus includes occluding the normal eye with an eye patch to strengthen the muscles and use of the abnormal eye.

CHILDHOOD VACCINATIONS The Epidemiology chapter summarizes Centers for Disease Control and Pre­ vention (CDC)-recommended vaccinations for the pediatric population. Contraindications and precautions in this population are as follows. Contraindications: ■

■ ■ ■

Severe allergy to a vaccine component or a prior dose of vaccine. Patients who have life-threatening allergies to eggs may receive measles, mumps, and rubella (MMR) and influenza vaccinations under observation. The physician should exercise caution in administering yellow fever vaccina­ tions to those with egg allergies. Encephalopathy within 7 days of prior pertussis vaccination. Personal history of intussusception and SCIO contraindications for the rotavirus vaccine. Live vaccines (rotavirus, oral polio vaccine, varicella, MMR, intranasal influenza, yellow fever) to be avoided in immunocompromised and preg­ nant patients (exception: HIV patients with CD4+ cell count >200 copies/ mm 3 may receive MMR and varicella).

Precautions: ■ ■

■

Current moderate to severe illness (with or without fever) Prior reactions to pertussis vaccine (fever >40. 5 ° C [ > 104.9 ° F]), a shock­ like state, persistent crying for > 3 hours within 48 hours of vaccination, or seizure within 3 days of vaccination History of receiving IVIG in the past year

The following are not contraindications to vaccination: ■ ■ ■

The most l i kely ca use of this infant's a pnea is a b u sive head tra u ma, which is most co m mon in 3- to 4-month-old i nfa nts a n d presents early with nonspecific sym ptoms (letha rgy, i rrita b i l i ty, poor feed i n g, vo miting) a n d later with seizu res o r apnea. There i s genera l ly n o repo rted h i story of head tra u m a . S u bd u ra l hematoma and e d e m a account for most n e u rolog ic fi n d i ngs. I n babies with a b u s ive head tra u ma, there is a 50%-70% cha nce of prior a b u se.

Mild illness and/or low-grade fever Current antibiotic therapy Prematurity - all vaccines should be given, based on the child's chrono­ logic age, even if the child is premature

LEAD POISONING Most exposure in children is caused by lead-contaminated household dust from lead paint. Screening should be routinely performed at 12 and 24 months of age for patients living in high-risk areas (pre- l 970s homes or zip codes with high percentages of elevated blood lead levels). Universal screen­ ing is not recommended.

PEDIATRICS

HIGH -YIELD FACTS IN

581

F I G U R E 2 . 1 2-29.

Basophilic stippling (arrows) in lead poisoning. (Reproduced cou rtesy of van Dij k HA, Fred H L. Images of memorable cases: case 8 1 . Connexions Web site. December 3, 2008. Available at hnps//cnx.org/contents/ MZa_Ph4e@4/lmages-of-Memorable-Cases-Case.)

History/PE ■ Children are usually asymptomatic. Children 70 µg/dL) is characterized by i ICP, vomiting, confusion, seizures, and coma.

� KEY FACT

New evidence has shown impaired intelligence and neurodevelopmental outcomes among children exposed to lead levels as low as 1 0 µg/dl.

Diagnosis The physician should do a fingerstick test as an initial screen at 1 or 2 years of age; if elevated, then a serum venous blood lead level should be obtained. ■ CBC and peripheral blood smear show microcytic, hypochromic anemia and basophilic stippling (see Fig. 2.12-29). Sideroblastic anemia may also be present. Note that concurrent iron-deficiency anemia may also be present, as both lead poisoning and iron deficiency share similar risk factors. ■ X-ray of the abdomen may be useful in assessing for ingestion of objects containing lead. Treatment Blood lead levels (see Fig. 2-12-30): ■ < 5 µg/dL: Family education and annual test of blood lead levels ■ 5 to 14 µg/dL: Retest at 1 to 3 months; remove sources of lead 15 to 44 µg/dL: Retest within 1 to 4 weeks; remove sources of lead exposure Venous blood lead concentration (µg/dl) 70 µg/dL: Retest within 24 hours; urgent evaluations, hospitalization, and chelation therapy (succimer + edetate calcium disodium [CaNa2 EDTA] )

PERIANAL DERMATITIS The commonly tested perianal dermatoses are listed in Table 2.12-32. TA B L E 2 . 1 2-32.

Perianal Dermatoses

DIAGNOSIS

DESCRIPTION

TREATMENT

I rritant contact dermatitis

Most common in infants. Presents as erythematous rash that spares the skinfolds. Classically due to skin breakdown by exposu re to urine or stool.

Topical barrier ointment (eg, zinc oxide, petrolatum)

Candida diaper dermatitis

Second most com mon i n infants. Presents as beefy red, confl uent plaques with satellite lesions. Does not spare the skinfolds.

Topical a ntifungal (eg, nystatin)

Perianal streptococcus

Occurs in school-aged children in addition to infants. Presents

Oral 13-lactam antibiotics

as a red, sharply demarcated perianal rash with associated pruritis and pain. May have blood-streaked stools or constipa­ tion from withholding stool due to pain. Diagnosis can be confirmed with perianal bacterial culture.

PEDIATRICS HIGH -YIELD FACTS IN

583

PIGMENTED LESIONS IN CHILDHOOD The commonly tested lesions are listed in Table 2.12-33. TA B L E 2 . 1 2-33.

Pigmented Lesions in Childhood

LESION

DESCRIPTION

Congenital melanocytic nevus

Benign proliferation of melanocytes with an increased density of hair follicles. They present as solitary hyperpigmented lesions with coarse hair (Image A). The melanocytes may enlarge during infancy; large lesions are often surgically removed due to increased risk of transformation to melanoma.

Congenital dermal melanocytosis (CDM) (Mongolian spot)

Multiple poorly circumscribed, nontender, flat, blue-gray patches that do not blanch (Image 8). They are often located on the lower back and sacral region and fade spontaneously during childhood. More com mon in people of Asian and Black descent. Congenital dermal melanocytosis may be mistaken for bruises due to abuse; however, bruises are tender, show color variation, and fade quickly. Presence of CDM should be documented on initial evaluation.

Cafe au lait spots

Flat, hyperpigmented patches having "coffee with milk" appearance (Image C). Isolated spots are most often idiopathic/benign, but multiple spots may be associated with McCune-Albright syndrome and neurofibromatosis.

I mage A reproduced with permission from Kinsler VA, O'Hare P, Jacques T, Hargrave D, Slater 0. MEK inhibition appears to improve symptom control in primary N RAS-driven CNS melanoma in children. Br J Cancer. 201 7;1 1 6(8):990-993. doi:1 0.1 038/bjc.201 7.49. I mage B reproduced with permission from Thomas AC et al. Mosaic activating mutations in GNA 1 1 and GNAQ are associated with phakomatosis pigmentovascularis and extensive dermal melanocytosis. J Invest Dermatol. 201 6;1 36(4):770-778. doi: 1 0. 1 0 1 6/j.jid.201 5.1 1 .027. I mage C reproduced with permission from Khalil J, Afif M, Elkacemi H, et al. Breast cancer associated with neurofibromatosis type 1 : a case series and review of the literature. J Med Case Rep. 201 5;9:61 doi:1 0.1 1 86/s 1 3256-01 5-0533-8.

584

HIGH-YIELD FACTS IN

PEDIATRICS NOTES

PSYCH IATRY C h i l d hood and Adolescent Disorders ATTENTION-DEFICIT/HYPERACTIVITY AUTISM SPECTRUM DISORDER DISRUPTIVE BEHAVIORAL DISORDERS INTELLECTUAL DEVELOPMENTAL DISORDER/INTELLECTUAL DISABILITY TouRETTE SYNDROME SEPARATION ANXIETY DISORDER

Psychotic Disorders SCHIZOPHRENIA SCHIZOPHRENIFORM

586

586 587 587 588 589 589

590

590 591

Dissociative Disorders

593

Anxiety Disorders

594

GENERALIZED ANXIETY DISORDER p ANIC DISORDER PHOBIAS (SOCIAL AND SPECIFIC)

Obsessive-Com p u l s ive Disorder and Related Disorders OBSESSIVE-COMPULSIVE DISORDER OBSESSIVE-COMPULSIVE-RELATED DISORDERS

Tra u m a a n d Stressor-Related Disorders POSTTRAUMATIC STRESS DISORDER

Neu rocog n itive Disorders DEMENTIA (MAJOR NEUROCOGNITIVE DISORDER) DELIRIUM

Mood Disorders MAJOR DEPRESSIVE DISORDER PERSISTENT DEPRESSIVE DISORDER ( DYSTHYMIA) ADJUSTMENT DISORDER BIPOLAR AND RELATED DISORDERS

594 595 596

597

597 597

598

Persona l ity Disorders

607

Su bsta nce Use Disorders

61 0

ALCOHOL USE DISORDER

MANAGEMENT OF DRUG WITHDRAWAL

Eating Disorders

ANOREXIA NERVOSA BULIMIA NERVOSA

Sexual Disorders SEXUAL CHANGES WITH AGING PARA PHILIC DISORDERS GENDER DYSPHORIA SEXUAL DYSFUNCTION

Sleep Disorders PRIMARY INSOMNIA PRIMARY HYPERSOMNIA NARCOLEPSY SLEEP APNEA CIRCADIAN RHYTHM SLEEP DISORDER

Somatic Sym ptom and Related Disorders

598

SOMATIC SYMPTOM DISORDER

599

ILLNESS ANXIETY DISORDER CONVERSION DISORDER

61 3 614

614

614 61 6

61 6

61 6 61 6 61 6 61 7

61 8

61 8 61 8 61 8 61 9 61 9

620

620 620 620

599 60 1

Factitious Disorders and Mali ngering

62 1

602

Sexual and Physical Abuse

62 1

602 604 605 605

SEXUAL ASSAULT

Su icidal ity

622

622

585

586

HIGH-YIELD FACTS IN

PSYCHIATRY

CHILDHOOD AND ADOLESCENT DISORDERS ATTENTION- D E FICIT/HYPERACTIVITY DISORDER A persistent pattern of excessive inattention and/or hyperactivity/impulsivity. Typically presents between 3 and 13 years of age; more common in males; often shows a familial relationship H i story/PE

Attention-deficit/hyperactivity disorder (ADHD) presents with core symptoms that can be divided into the following two categories: Inattention: Exhibits a poor/short attention span in schoolwork/play; dis­ plays poor attention to detail or careless mistakes; has difficulty following instructions or finishing tasks; is forgetful and easily distracted ■ H yperactivity/impulsivity: Fidgets; leaves seat in classroom; runs around inappropriately; cannot play quietly; talks excessively; does not wait for their turn; interrupts others ■

Diagnosis

Symptoms must be present and cause an impairment in functioning for 2:6 months in at least two independent settings (eg, home and school). Age < 17 years: Diagnosis requires six or more symptoms from either cate­ gory of core symptoms (inattention and/or hyperactivity/impulsivity) Age 2: 17 years: Diagnosis requires five or more symptoms from either cate­ gory of core symptoms Symptoms must be present before 12 years of age ■ Alternative causes of inattention and/or hyperactivity must be considered (such as substance abuse, organic reasons [eg, lead toxicity], hearing/visual impairment, thyroid disorders, sleep disorders, absence seizures) Treatment

Four to five years of age: Best initial treatment: Behavior therapy. The physician can add medica­ tion if behaviors do not improve. Six years of age or older:

0-,,. KEY FACT

Use of stimulants is contraindicated for individuals with illicit substance abuse at risk for addiction and those whose parent/legal guardian is against their use.

0-,,. KEY FACT

Children must exhibit ADH D symptoms in two or more settings (eg, home and school).

Best initial treatment: Pharmacologic therapy + behavior therapy First line: Central nervous system (CNS) stimulants (eg, methylphenidate, dextroamphetamine, amphetamine salts [ dextroamphetamine and amphetamine combo]) ■ Adverse effects: Weight loss (-l, appetite), insomnia, anxiety, irritability, headache, tic exacerbation, and .!. growth velocity (normalizes when medi­ cation is stopped) ■ Because of stimulants' potential for causing weight loss, it is recommended to give them after meals ■ Alternatives: Nonstimulants ■ Atomoxetine (norepinephrine reuptake inhibitor; second-line treat­ ment): Can be tried first because of the negative side-effect profile of CNS stimulants ■ Third-line treatments: clonidine/guanfacine ( Uz-agonist), bupropion, and tricyclic antidepressants (TCAs) ■ ■

All ages: Continuation of regular diet. Sugar and food additives are not con­ sidered etiologic factors.

PSYCHIATRY

HIGH -YIELD FACTS IN

587

AUTISM SPECTRUM DISORDER Developmental disorder characterized by impairments in two major domains: (1) social interaction and communication and (2) repetitive/restricted behav­ ior, interests, or activities. Autism spectrum disorder (ASD) is more common in males. Severity is based on the level of support required for each domain. The Diagnostic and Statistical Manual of Mental Disorders (DSM)-5 does not distinguish among the pervasive developmental disorders.

History/PE ■ Deficits in social interaction and communication: Reduced interest in socialization, reduced empathy, inability to form relationships, impaired language development, inability to understand social cues, poor eye contact ■ Prognosis is best determined by language development because its development is based on social interaction ■ Restricted/repetitive patterns of behavior, interests, or activities: Highly fix­ ated or restricted interests, inflexibility to change, hand flapping or other stereotypies, increased/decreased response to sensory input (eg, indiffer­ ence to temperature, excessive touching/smelling, adverse responses to sounds) ■ Symptoms must impair function (eg, academic, social) ■ Symptoms must be present in the early developmental period (typically < 3 years of age) ■ Conditions that produce symptoms suggestive of ASD must be excluded: Intellectual disability or global developmental delay ■ Hearing impairment: Ruled out with audiometry before making diagnosis ■ Selective mutism: Refusal to speak only in social situations ■ Rett syndrome: Similar to ASD; X-linked disorder characterized by marked physical and psychomotor regression at approximately 6 months of age after normal development. Predominantly seen in females; patients have classic hand-wringing movements ■ Fragile X syndrome: X-linked dominant disorder caused by hypermeth­ ylation of FMRl gene; most common cause of inherited intellectual disability and autism; presents with the following characteristics: macro-orchidism, long face, prominent jaw and forehead, high arched palate, large and everted ears, autism, connective tissue laxity, and /or mitral valve prolapse Treatment ■ Intensive special education, behavioral management (specifically applied behavior analysis therapy), and symptom-targeted medications (eg, stimu­ lants or az-agonists for ADHD; neuroleptics for aggression and mood insta­ bility; selective serotonin reuptake inhibitors [SSRis] for stereotypical behavior, anxiety, and mood) ■ Family support and counseling - crucial components of treatment

DISRUPTIVE BEHAVIORAL DISORDERS Includes conduct disorder, oppositional defiant disorder (ODD), and disrup­ tive mood dysregulation disorder (DMDD). More common in males and in patients with a history of abuse. Psychiatric comorbidities are common (eg, post-traumatic stress disorder [PTSD], depression, substance abuse, somato­ form conditions, personality disorders).

0-,r KEY FACT I n patients with ASD, th i n k a bout associated congen ita l cond itions, such as Rett synd rome, tu berous sclerosis, and fra g i l e X synd rome.

588

HIGH-YIELD FACTS IN

0-,,. KEY FACT Conduct d i sorder is diag nosed i n C h i l d ren. Antisocial personal ity disorder is diagnosed i n Ad u lts.

PSYCHIATRY History/PE ODD: A pattern of negative, defiant, disobedient, and hostile behavior toward authority figures (eg, losing temper, arguing) for 2:::6 months. May progress to conduct disorder. ■ Conduct disorder: A repetitive, persistent pattern of violating the basic rights of others or age-appropriate societal norms/rules for 2::: 1 year in chil­ dren < 18 years of age. Behaviors can be aggressive (eg, rape, robbery, ani­ mal cruelty) or nonaggressive (eg, destruction of property, stealing, lying, running away, and truancy). After 18 years of age, conduct disorder is con­ sidered antisocial personality disorder. ■ DMDD: A pattern of severe, recurrent verbal (eg, screaming) or behav­ ioral (eg, physical aggression) outbursts that are out of proportion to the sit­ uation and a persistently irritable or angry mood between outbursts. ■ Symptoms must occur for 2::: 1 year; they may progress to depression in adulthood. ■ DMDD should not be diagnosed before 6 years of age or after 18 years of age. Treatment Psychotherapy is the first-line treatment for all disruptive behavioral disorders.

INTELLECTUAL DEVELOPMENTAL DISORDER/INTELLECTUAL DISABILITY Disorder of cognitive, social, and practical functioning. Associated with male sex, chromosomal abnormalities, congenital infections, teratogens (including alcohol/illicit substances), and inborn errors of metabolism. Most often no identifiable cause is found in most patients with intellectual disability.

0-,,. KEY FACT ■

■

■

Down syn d rome (trisomy 2 1 ): Th e most com mon c h romosomal cause of i ntel l ectua l d isabil ity Feta l a lcohol synd rome (FAS): The most com mon preventable cause of i ntel l ectua l d isabil ity Frag i le X syn d rome: The most common i n h erited form of i ntel l ectua l d isabi l ity

History/PE ■ Patients have deficiencies in multiple domains as follows: ■ Intellectual deficits: Poor reasoning, problem solving, planning, and performance on standardized testing ■ Adaptive functioning deficits: Poor hygiene, social functioning, activi­ ties of daily living (ADLs) ■ Onset: During developmental period ( < 18 years of age) ■ Severity of intellectual disability: Determined by level of support required to address impaired adaptive functioning (IQ cutoffs for severity are no longer used with the DSM-5) ■ Mild (independent in ADLs); moderate (some teaching and support for ADLs); severe (significant support for ADLs); profound (dependent on support for all AD Ls) ■ Differential diagnosis: Specific learning disorder (reading, math, or writ­ ing skills that are significantly lower than expected for age and intelligence) ■ Reading disorder is the most common Treatment ■ Primary prevention: Educating the public about possible causes of intel­ lectual disability and providing optimal prenatal screening and care to pregnant patients ■ Treatment measures: Family counseling and support, speech and lan­ guage therapy, occupational/physical therapy, behavioral intervention, educational assistance, and social skills training Learning disorder: Remedial therapy directed toward patient's deficiency

PSYCHIATRY

HIGH -YIELD FACTS IN

589

TOURETTE SYNDROME Disorder characterized by both motor and vocal tics. More common in males; shows a genetic predisposition. Associated with ADHD, learning disorders, and obsessive-compulsive disorder (OCD). Symptoms can be temporarily suppressed with effort or exacerbated by stress and fatigue.

History/PE Symptoms begin 1 year ■ Tics recurrent (occur many times per day and/or nearly every day) ■ ■

Treatment Best initial treatment: Behavior therapy; habit reversal therapy is most effec­ tive. If behavior therapy fails or tics are severe/disabling, the next step is phar­ macologic management. Antidopaminergic agents: ■ Dopamine-depleting agents (eg, tetrabenazine [ vesicular monoamine transporter (VMAT)-2 inhibitor; results in ,J, uptake of monoamines ]): Preferred over dopamine-blocking agents; does not cause tardive dyski­ nesia (TD) ■ Dopamine-blocking agents: Antipsychotics (eg, fluphenazine, risperi­ done, haloperidol, pimozide) ■ For severe, refractory tics: Typical antipsychotics (eg, haldol, pimozide) ■ a2-agonists (eg, clonidine, guanfacine ): Less effective at tic reduction; more favorable side-effect profile ■

Differential Diagnosis Persistent (chronic) tic disorder consisting of either motor or vocal tics (but not both) which last > 1 year.

SEPARATION ANXIETY DISORDER Disorder characterized by fear of separation from an attachment figure (eg, parent) or home. Separation anxiety normally begins at about 1 year of age and peaks at 18 months. Considered pathologic if it becomes extreme or per­ sists. May be precipitated by a stressful event.

History/PE Fear of separation from an attachment figure or home lasting for 2::4 weeks in children or 2::6 months in adults. May present as complaints of somatic symp­ toms to avoid school or work.

0-n

KEY FACT

Coprolalia = Repetition of obscene words Echolalia = Repetition of words spoken by others

590

HIGH-YIELD FACTS IN

PSYCHIATRY Diagnosis

Excessive fear of separation from attachment figures and three of the following: ■ ■ ■ ■ ■ ■

Separation that leads to extreme distress Separation that leads to social, academic, or occupational dysfunction Excessive worry about losing attachment figure Reluctance to leave home, sleep alone, or be alone Nightmares of separation Physical symptoms (eg, a stomachache) when separated

Treatment

Cognitive-behavioral therapy (CBT), family therapy, SSRis as an adjunct to therapy.

PSYCHOTIC DISORDERS SCHIZOPHRENIA Disorder of thought process characterized by psychotic symptoms that are fur­ ther divided into positive symptoms (hallucinations, delusions, disorganized thought/behavior) and negative symptoms (fl.at affect, social withdrawal, apathy).

CM,, KEY FACT

Epidemiology: Prevalence is ~1 % (males > females). Peak onset is ear­ lier in males (18-25 years of age) than in females (2 5-3 5 years of age). Schizophrenia in first-degree relatives i risk. Up to 5 0% of patients attempt suicide; 10% of patients with schizophrenia complete suicide. ■ Etiology: Unknown. Theories focus on neurotransmitter abnormalities such as dopamine dysregulation (frontal hypoactivity and limbic hyperac­ tivity) and brain abnormalities on CT and MRI (enlarged ventricles and ,J, cortical volume). ■

Psychosis (ha l l uci nations a nd/ or d e l u sions without insig ht) schizophrenia. Differential diag nosis m ust also incl ude org a n i c diseases, other psych iatric i l l nesses, a n d su bsta nce-i nduced psychosis.

*

History/PE

Schizophrenia presents with chronic or recurrent psychosis, disorganiza­ tion, and/or negative symptoms. ■ Cognitive impairment in multiple areas (eg, processing speed, working memory, attention, social cognition) may be present. ■ Mood and anxiety symptoms are common. ■

Diagnosis

Requires two or more of the following symptoms for 2::6 months with social or occupational dysfunction; at least one of the symptoms must be hallucina­ tions, delusions, or disorganized speech:

:0

MNEMONIC

Five As of schizophrenia diagnosis-Affect (flat), Avolition,

Asociality, Anhedonia, Apathy

■ ■ ■

Hallucinations (most often auditory) Delusions Disorganized speech Disorganized or catatonic behavior Negative symptoms: Flattened affect, social withdrawal, anhedonia, apa­ thy, ,J, emotion; may mimic depression

PSYCHIATRY

HIGH -YIELD FACTS IN

591

SCHIZOPHRENIFORM Disorder of thought process. Presentation similar to schizophrenia, but psy­ chotic symptoms are present for 1 to 6 months only. See Table 2.13-1 for the differential diagnosis of psychosis.

Prognosis Better prognosis: ■ Acute onset with positive manifestations (hallucinations and delusions) ■ No family history

■

Rapid symptom resolution with treatment initiation

Poor prognosis:

■ ■ ■

Symptoms arise before 13 years of age Insidious course with depressive states Developmental delay; timid, introverted, and uncommunicative before manifesting symptoms

Treatment ■ Treatment is usually lifelong and often involves a combination of medica­ tions, psychotherapy, social skills training, and coordinated specialty care services. ■ Pharmacologic intervention with antipsychotic medications can be used to treat the acute symptoms of psychosis, as well as for long-term manage­ ment and prevention of symptoms (see Table 2.13-2). TA B L E 2 . 1 3 - 1 .

DISORDER Psychotic

disorders

Differential Diagnosis of Psychosis

DU RATI O N/CHARACTERISTICS Brief psychotic d isorder: 1 day to 1 month

Schizophreniform d isorder: 1 -6 months

Note: Both present simi larly to schizophrenia but a re differentiated by d u ra­

tion of i l l ness; ca n be preceded by stressor(s), a re less likely to have negative sym ptoms, and have a better lifeti me prog nosis than sch izoph renia

Schizophrenia: >6 months

Schizoaffective disorder: Psychosis + mood disorder (mania or depression); Requires history/presence of:

■ Psychosis + mood episode AND

■ Psychosis for 2:2 weeks without mood episode Persona l ity

Schizotypa l: "Magical thinking"

Delusional

Persistent delusions (often nonbizarre) without disorganized thought process,

disorders disorder

Schizoid: "Loners"

hallucinations, or negative symptoms of schizophrenia; su btypes a re

jea lous, para noid, somatic, erotomanic, or grandiose

Symptoms m ust be present 2: 1 month

Day-to-day fu nctioning is mostly u naffected

Folie a deux: A shared delusion (commonly between parent and chi ld); the

best course of action is to sepa rate the patient pair and treat individ ually

0-n

KEY FACT

0-n

KEY FACT

0-n

KEY FACT

0-n

KEY FACT

0-n

KEY FACT

In addition to psychotic symptoms, a patient must have mood symptoms present the majority of the time for a physician to differentiate schizoaffective disorder from schizophrenia. Terms used to describe components of psychosis are as follows: ■ Delusion: A fixed false idiosyncratic belief ■ Hallucination: Perception without an existing external stimulus ■ Illusion: Misperception of an actual external stimulus In those with poor medication adherence or noncompliance, depot (injectable) is preferred because they are longer-acting medications.

Atypical antipsychotics (olanzapine, risperidone, quetiapine) are preferred to typical antipsychotics (haloperidol, thioridazine, chlorpromazine), given fewer EPSs and anticholinergic effects. Resistance is considered when a minimum of two drugs have been attempted previously without improvement of symptoms.

•

A 24-year-old woman presents to the clinic. She has been "hea ring voices" and has isolated herself from her friends and family. She fi rst noticed the voices about 2 months ago when she was feel i ng sad and reported sleepi ng poorly. She reports that her mood has since i m proved and denies a ny cu rrent sleep disturbances but is sti l l heari ng the voices. What is her most l i kely diagnosis?

592

PSYCHIATRY

HIGH-YIELD FACTS IN

TA B L E 2 . 1 3-2.

Antipsychotic Medications

DRUG CLASS

M ECHAN ISM

EXAMPLES

I N D I CATIONS

ADVERSE EFFECTS

Typical a nti­

D , a ntagonist

Haloperidol',

Psychotic disorders, acute agitation,

EPSs (see Table 2.1 3-3) > a nticholin­

psychotics

(high potency)

fl uphenazi ne'

acute man ia, Tou rette synd rome

Thought to be more effective for positive symptoms of schizophrenia

If compliance is a major issue, the physician ca n consider a ntipsychotics

avai lable i n long-acting depot form' D, a ntagonist

(low potency)

Thioridazi ne,

chlorpromazine

Same as high potency

ergic symptoms (d ry mouth, urinary retention, consti pation)

QTc prolongation and torsades

de pointes, especially with IV haloperidol

Neuroleptic malignant syndrome Anticholi nergic > EPSs More sedating

Greater risk for orthostatic hypotension

Thioridazine ca uses QTc prolongation

and irreversible reti nal pigmentation

Atypical a ntipsychotics

D, a ntago-

nist, 5-HT,A

a ntagonist

Risperidone',

quetiapine,

olanzapine',

pali peridone',

ziprasidone, clozapine

D, partial agonist, 5-HT,A pa rtial

Aripiprazole"

agonist, 5-HT,A

Fi rst-line treatment for schizophrenia,

given fewer EPSs and a nticholinergic effects

Clozapine is reserved for severe treat­ ment resista nce and severe tardive

dyski nesia

Same as other atypicals, except it does not ca use hyperprolactinemia due

to its ability to act as a D, receptor agonist u nder hypodopam i nergic

a ntagonist

conditions and as a D, receptor

t EPSs (due to 5- HT,A a ntagonism)

Weight gain, dyslipidemia, type 2 DM, somnolence, sedation, and QTc

prolongation (zi prasidone); hyperp­ rolactinemia (risperidone)

Clozapine can cause agra n u locytosis; its use req uires weekly CBC moni­

toring during fi rst 6 months

a ntagonist d u ring hyperdopami­ nergic conditions

'Also available as a long-acti ng depot i njection. CBC, Complete blood cell cou nt; DM, diabetes mellitus; EPS, Extrapyramidal sym ptom; HT, hydroxytrypta mi ne; JV, i ntravenous.

■

■ ■

This pati ent most l i kely has sch izoaffective disorder, which is c h a racterized by psyc h osis and i nte rm ittent mood sym pto ms. The diag nosis req u i res ( 1 ) psychotic sym pto ms A N D mood sym pto ms a n d (2) a t least 2 weeks w h e n psyc h otic sym pto ms a re p resent WITHOUT mood sym ptoms. Pati ents often have c h ronic psychotic sym pto ms, even after mood sym ptoms have resolved.

Supportive psychotherapy, training in social skills, vocational rehabilita­ tion, and illness education may help. In particular, family psychoeduca­ tion/therapy decreases the risk for relapse. Negative symptoms may be more difficult to treat than positive symptoms; atypical antipsychotics are the drug of choice. Catatonia (awkward posturing, mutism, and immobility) may be seen in severe disease; this can be treated with benzodiazepine challenge and electroconvulsive therapy (ECT). Find a description of ECT in the Major Depressive Disorder section later in this chapter.

PSYCHIATRY TA B L E 2 . 1 3-3.

(J.0 MNEMONIC

Extrapyramidal Symptoms and Treatment

SU BTYPE

DESCRIPTION

TIME O F O N S ET

TREATMENT

Acute

Prolonged, pai nful tonic

Hours

Anticholi nergics (benztropine or

dystonia

muscle contraction or

spasm (eg, torticollis,

diphenhyd ra m ine) for acute

therapy

oculogyric crisis)

Patients who are prone to dystonic reactions may need regular pro-

phylactic dosing (eg, benztropi ne) Akath isia

Subjective/objective

restlessness that is

Weeks

Dyskinesia

Pseudo-pa rkinsonism

(eg, tremor, sh uffling

dyski nesia

Stereotypic, invol u ntary, painless ora l-facia l

Weeks

Months

movements

dose may i n itia l ly worsen tardive dyskinesia

If disconti nuing or t neuroleptic

dose is i neffective, try va l benazi ne

Often irreversible (50%)

synd rome

elevated creatine

kinase and white blood cel ls, delirium

possibly change neuroleptic (eg,

Anticholinergics or t neuroleptic

mine blockade

a utonomic i nstability,

Disconti nue or t dose of neuroleptic; to cloza pine or quetiapine)

from chronic dopa-

Fever, muscle rigid ity,

(if tolerated)

dopamine agonist (ama ntadine)

receptor sensitization

malignant

t dose of neuroleptic or d isconti nue Anticholinergics (benztropine) or

Probably from dopamine

Neuroleptic

(propranolol)

choli nergics (benztropine)

gait, cogwheel rigidity) Tardive

t dose of neuroleptic; 13-blockers Benzodiazepi nes (lorazepa m) or a nti-

perceived as being

d istressing

or deutetrabenazine Any

time

HIGH -YIELD FACTS IN

Disconti nue medication; provide

su pportive ca re in the i ntensive care u n it (ICU); administer dan-

trolene or bromocri ptine

DISSOCIATIVE DISORDERS Includes dissociative identity disorder, depersonalization/derealization disor­ der, and dissociative amnesia. Symptoms are not better explained by sub­ stance use, another medical condition, or another mental disorder. See Table 2.13-4 for an overview of the dissociative disorders. Treatment ■ Best initial treatment: Psychotherapy ■ Appropriate pharmacologic treatment (eg, SSRis) can be added to address comorbidities (eg, depression, anxiety, substance abuse, PTSD)

Evolution of extrapyramidal symptoms4 and A

4 hours: Acute dystonia 4 days: Akinesia 4 weeks: Akathisia 4 months: Tardive dyskinesia (often permanent)

593

594

HIGH-YIELD FACTS IN

PSYCHIATRY TAB L E 2 . 1 3-4.

Overview of Dissociative Disorders

DISORDER

CHARACTERISTICS

Dissociative identity

Presence of two or more d istinct personalities or identities with

d isorder (formerly

m u ltiple personal ity

d isorder)

sepa rate memories and behavior patterns that domi nate at

different times. Patients a re usually unawa re of the other per­

sonalities and com plain of freq uent gaps in recal l and memory

lapses. Dissociative identify d isorder is more com mon in fema les.

Associated with history of tra u ma, child abuse, PTSD, borderline personality disorder, somatic sym ptom disorder, and major

depressive disorder. Depersonal ization/ derea l ization d isorder

Recu rrent or persistent• experiences of one or more of the following:

• Depersonalization: Feeling of detach ment from one's body, actions, thoughts, and perceptions Patient may feel like a n outside observer or have "out-of-body" experience

■ Derea lization: Experiencing one's surroundings as u n real

■ Sti l l able to identify reality, has insight i nto the distinction

between i nternal and external rea l ity, u n l i ke in patients with psychosis

Dissociative a m nesia

I nability to reca l l memories or important personal information,

usually after a tra u matic or stressful event; proced ural memory is preserved.

Dissociative fugue: Su btype of dissociative a mnesia characterized by sudden, unexpected travel in a d issociated state and su bse­

quent am n esia of the travel.

Increased risk for suicide as a m nesia resolves and memories of trauma return.

"Tra nsient depersonalization/derea lization may occur d u ring times of severe stress; this does not meet diagnostic criteria for DOD.

ANXIETY DISORDERS GENERALIZED ANXIETY DISORDER Uncontrollable, excessive anxiety or worry about multiple topics that leads to significant impairment or distress. H istory/PE

Clinical onset is usually in the early 20s. Generalized anxiety disorder (GAD) is more common in females. Diagnosis

■

GAD is diagnosed when excessive anxiety or worry about multiple activi­ ties is experienced on most days for 2:::6 months. ■ Symptoms of anxiety/worry are associated with three or more somatic symptoms (only one required in children): restlessness, easy fatigue, diffi­ culty concentrating, irritability, muscle tension, sleep disturbances.

PSYCHIATRY ■ ■ ■

Symptoms cause a clinically significant impairment (eg, social, occupational). Disturbances are not caused by substances (eg, drug of abuse [eg, cocaine], medication [ eg, amphetamines]). Disturbance is not better explained by another psychiatric disorder (eg, excessive worry about panic attacks).

Treatment ■ Best initial treatment (first-line) : Psychotherapy (CBT, applied relax­ ation, biofeedback) + SSRis (eg, fluoxetine, sertraline, escitalopram) or serotonin norepinephrine reuptake inhibitors (SNRis, eg, venlafaxine, duloxetine). See Table 2.13-5. ■ Alternative treatment (second-line): Buspirone, TCAs, benzodiazepines (short-term treatment).

PANIC DISORDER Characterized by recurrent, unexpected periods of intense fear that last for several minutes and cause excessive worry about having another panic attack.

HIGH -YIELD FACTS IN

595

(J.0 MNEMONIC The diagnosis of GAD in an adult requires the presence of three or more of the Worry WARTS symptoms: Wound up (i rritability) Worn out (fatig ue) Absent-mindedness (difficu lty concentrating) Restlessness Tension i n m uscles Sleep distu rbance

0-,r KEY FACT Li ke SSRls, buspirone should not be used in conj u nction with monoa m i n e oxidase i n h i bitors. (See Ta bles 2 . 1 3-5 and 2 . 1 3-1 1 .)

History/PE ■ Recurrent episodes of intense fear and discomfort. Symptoms usually last :s 30 minutes. ■ Associated with agoraphobia, increased risk of suicide. ■ More common in females; may occur at any age. There is a strong genetic disposition.

TA B L E 2 . 1 3-5.

Anxiolytic Medications

DRUG CLASS

I N D ICATIONS

ADVERS E EFFECTS

SSRls (eg, fluoxeti ne, sertra li ne, pa r­

First-line treatment for GAD, OCD, panic

Nausea, G I upset, somnolence, sexua l dys­

SNRls (eg, venlafaxi ne, duloxeti ne)

Fi rst-line treatment for GAD

Hypertension, sti mu lant effects

5-HT partial agon ist (eg, buspirone)

Second-line treatment for GAD, social

Headaches, dizziness, nausea

oxetine, cita lopram, escita lopra m)

disorder

phobia

Used if sexua l dysfu nction experienced with

fu nction, agitation

No tolera nce, dependence, or withd rawa l

SSRls

13-Blocker (propra nolol)

Performa nce-only social a nxiety disorder

Bradyca rd ia, hypotension

Benzod iazepines (eg, clonazepa m,

Anxiety (short-term), insomn ia, a lcohol

i sleep d u ration; risk for abuse, tolerance,

a l prazolam)

withdrawal, m uscle spasm, night terrors, sleepwa l king

and dependence; disinhibition in young or older patients; confusion

Abru ptly stopping a short-acti ng benzo­

diazepine (eg, a l prazolam) can result in seizu res

GAD, Generalized a nxiety disorder; GI, gastroi ntesti nal; OCD, obsessive-compulsive d isorder.

596

HIGH-YIELD FACTS IN

0-,,. KEY FACT Differentia l diag nosis for panic disorders: ■ Med ica l cond itions: Angi na, myoca rd ial infarction (Ml), a rrhyt h m ias, hyperthyroidism, pheochromocytoma, hypog lycem ia ■ Psych iatric cond itions: Su bsta nce­ i n d uced a nxiety, GAD, PTSD

PSYCHIATRY Diagnosis Panic attacks: Discrete periods of intense fear or discomfort in which four or more of the following symptoms develop abruptly and peak within 10 minutes: ■ Tachycardia or palpitations, diaphoresis, chest pain, shortness of breath, nausea, trembling, dizziness, fear of dying or "going crazy," depersonalization, hot fl.ashes, chills or heat sensation, paresthesia ■ Increased sensitivity to lactate infusion, which may precipitate an attack in the susceptible Panic disorder: ■ Recurrent, unexpected panic attacks ■ Attacks followed by 2:: 1 month of at least one of the following ■ Persistent worry/concern of having additional attacks ■ Maladaptive change in behavior (eg, avoidance of unfamiliar situations) Worry about consequences of attack (eg, losing control) ■ Not explained by substances, medications, or another medical condition Treatment ■ See Table 2.13-5 for medications. ■ Acute, initial treatment: Benzodiazepines (eg, clonazepam). Long-term use should be avoided due to concerns of potential dependence and abuse. Benzodiazepines should be tapered as soon as long-term treatment is effective. ■ Long-term treatment: SSRis (eg, sertraline). ■ Psychotherapy: CBT; higher rates of response and sustained effects com­ pared with placebo and pharmacotherapy alone

PHOBIAS (SOCIAL AND SPECIFIC) Disorders characterized by excessive fear that is unreasonable and stimulated by the presence or anticipation of a specific object or situation. Patients recog­ nize the fear is excessive. Symptoms are persistent, usually lasting 2::6 months.

0-,,. KEY FACT Agora phobia is defi ned as fea r of being alone in p u b l ic places. Litera lly tra n s lated, it mea ns "fear of the ma rketplace:'

History/PE ■ Social anxiety disorder: Presents with excessive fear of criticism, humilia­ tion, and embarrassment in multiple situations requiring social interac­ tion. Patients may have anxiety in anticipation of the event, palpitations and sweating during the event, and they may avoid triggers (eg social events, parties, school). ■ Performance-only subtype: Symptoms provoked only by performance situations (eg, public speaking, test taking, sexual intercourse). ■ Specific phobia: Excessive anxiety and fear provoked by exposure to a feared object or situation (eg, animals, heights, airplanes). Most cases begin in childhood. ■ Agoraphobia: Fear/anxiety of developing paniclike symptoms in two or more situations from which it may be difficult to escape or get help, result­ ing in avoidance of those situations. Patients may become completely con­ fined to the home. Agoraphobia is associated with panic disorder. Treatment ■ Social anxiety disorder: Both CBT and SSRis are first line, and the choice of treatment is dependent on patient preference. CBT involves desensitization through incremental exposure to the feared object or

PSYCHIATRY

■ ■

situation along with relaxation techniques. Second-line treatment includes benzodiazepines (if no history of substance use disorder) or phenelzine (if patient has a history of or risk factors for substance use disorder). ■ Performance-only subtype: First-line treatment with 13-blockers (eg, propranolol) before the event or as needed. Second-line treatment includes CBT, benzodiazepines, and/or SSRis. Specific phobia: First-line treatment is CBT. Second-line treatment includes SSRis, benzodiazepines. Agoraphobia: CBT, SSRis.

OBSESSIVE-COMPULSIVE DISORDER AND RELATED DISORDERS OBSESSIVE- COMPULSIVE DISORDER Characterized by obsessions and/or compulsions that lead to significant dis­ tress and dysfunction in social or personal areas. Compulsions are typically time-consuming, often requiring > 1 hour daily. OCD typically presents in late adolescence or early adulthood; prevalence is equal in male and female patients. It is often chronic and difficult to treat.

History/PE ■ Obsessions: Persistent, unwanted, and intrusive ideas, thoughts, impulses, or images that lead to marked anxiety or distress (eg, fear of contamina­ tion, fear of harming oneself or loved ones) ■ Compulsions (or rituals): Repeated mental acts or behaviors that neutral­ ize anxiety from obsessions (eg, handwashing, elaborate rituals for ordinary tasks, counting, excessive checking) ■ Patients recognize their behaviors as excessive and irrational (vs obsessivecompulsive personality disorder [OCPD] ; see Table 2.13-6) ■ Patients wish they could get rid of obsessions and/or compulsions

HIGH -YIELD FACTS IN

0-n

597

KEY FACT

I n patients with a history of su bsta nce abuse, benzod iazepi nes should be avoided due to their h i g h potentia l for add iction.

0-n

KEY FACT

Abnormal ities on bra i n i maging a re common i n patients with OCD, specifica l ly in the orbitofronta l cortex a n d basa l g a n g l ia. The cortico-striato­ thalamo-cortica l (CSTC) circu its have been i m pl icated in the pathophysiology of the disorder.

0-n

KEY FACT

Many patients with OCD i n itia lly present to a non-psych iatrist (eg, they may consult a dermatologist with a skin com plaint secondary to overwashing their hands).

Treatment ■ Best initial treatment: SSRis (high dose) ■ Alternative: Clomipramine (TCA) ■ CBT using exposure and desensitization relaxation techniques ■ Patient education is imperative

OBSESSIVE- COMPULSIVE-RELATED DISORDERS Obsessive-compulsive-related disorders are characterized by unwanted, intrusive, recurrent, and persistent thoughts, urges, or images, as well as repetitive behaviors or mental acts in response to those preoccupations. TA B L E 2 . 1 3-6. Disorder

Obsessive-Compulsive Disorder vs Obsessive-Compulsive Personal ity

O BSESSIVE-COMPULSIVE DISORDER

OBSESSIVE-COMPU LSIVE PERSO NALITY DISORDER

Characterized by obsessions and/or

Patients are excessively conscientious and

Patients recognize the obsessions/compulsions

Patients do not recognize their behavior

com pu lsions.

and wa nt to be rid of them (ego dyston ic).

inflexi ble.

as problematic (ego syntonic).

I A 22-yea r-old m a n presents to a physicia n's office. He freq uently washes his ha nds, refu ses to sit on c h airs i n p u b l i c places, a n d will n ot use public trans portation fo r fea r of co ntracting d i seases. He does not t h i n k his behaviors a re a b n ormal, n or d o e s he thi n k h is behaviors i nte rfe re with h is d aily activities. What is the d i a g n osis?

598

HIGH-YIELD FACTS IN

PSYCHIATRY TAB L E 2 . 1 3-7.

Obsessive-Compulsive-Related Disorders

DISORDER

CHARACTERISTICS

Body dysmorphic

Preoccu pation with imagi ned or slight defects i n physical appearance

d isorder

that are usually i m perceptible to others, leading to significant distress/ impairment

The physician should suspect body dysmorphic disorder in patients with a n extensive history of cosmetic proced u res

Therapeutic approach: Acknowledge distress; avoid referring to com­

plai nts as imagined; evaluate level of insight; encourage patients to

avoid u n n ecessary cosmetic, surgica l, or medica l treatments Hoarding d isorder

Difficulty d iscarding possessions, regard less of value; attempts at d is­

carding objects causes signifi ca nt distress; hoa rding disorder resu lts in

accumu lation of objects and ca n lead to a n u nsafe livi ng environment Excoriation (skin

picking) d isorder

Recu rrent skin picki ng resulting i n skin lesions

Excoriation disorder ca uses clin ica l ly signifi cant distress or i m pairment

i n social fu nctioning; patient may report repeated attempts to stop or

decrease skin picking

Differential diagnosis: Pruritus ca used by a medical condition (eg, primary biliary cholangitis [PBC))

Trichoti llomania (hair-pulling

d isorder)

Recu rrent hair pulling leading to hair loss

Trichotillomania causes clinically significant distress or i m pairment in

social fu nctioni ng; patients may report repeated attem pts to stop or decrease the behavior; clin ical ly, hair follicles i n d ifferent stages of growth and hair of different lengths will be found

Differential diagnosis: Ti nea capitis, alopecia Kleptomania

Persistent and recu rrent impulse to steal items without motivators such as financial gain or personal need

Reg ularly associated with other psych iatric disorders such as OCD, a nxiety, eati ng disorders, and a lcohol and su bstance abuse

Some obsessive-compulsive-related disorders are primarily body-focused (eg, hair pulling, skin picking) with repeated attempts to decrease or stop the behaviors. Others involve mental acts that an individual feels driven to per­ form in response to an obsession or according to a rigid set of self-defined "rules" (see Table 2.13-7). All OCD-related disorders can be treated with CBT and SSRis. This person suffe rs fro m obsessive­ com p u l sive personal ity d isorder (OC PD). These patie nts a re perfecti o n i sts, a re preoccu pied with rules and order, and a re ofte n i nfl exible. U n l i ke patients with obsessive-com p u l sive d isorder, those with OCP D typica l l y a re not d i sturbed by the i r d isease.

TRAUMA AND STRESSOR-RELATED DISORDERS POST- TRAUMATIC STRESS DISORDER Disorder characterized by clinically significant distress or impairment in daily functioning caused by exposure to an extreme, life-threatening traumatic event (eg, war, assault, injury, rape, accident, violent crime). Event can be either directly experienced or witnessed.

PSYCHIATRY History/PE ■ Patients experience severe psychological distress when exposed to stimuli that remind them of the event, resulting in avoidance of situations where exposure to triggers is possible ■ High incidence of substance abuse, anxiety, and/or depression Diagnosis ■ Exposure to a traumatic event and the presence of one or more of the following: 1. Intrusive symptoms: Re-experiencing the event through nightmares, flashbacks, or intrusive memories 2. Avoidance of stimuli associated with the trauma 3. Negative alterations in mood and cognition: Numbed responsiveness (eg, detachment, anhedonia), guilt, blaming of oneself 4. Changes in arousal and reactivity: i arousal (eg, hypervigilance, exag­ gerated startle response), sleep disturbances, aggression/irritability, and poor concentration ■ Symptoms lead to significant distress or impairment in functioning ■ Symptoms must persist for > 1 month ■ Acute stress disorder: Diagnosed if symptoms are present for ::5 l month ■ Clinical presentation is the same as PTSD Symptoms last 2:: 3 days but < 1 month ■ Symptoms present within 1 month of experiencing the traumatic event Treatment ■ Best initial treatment: PTSD: CBT + SSRis or SNRis ■ Acute stress disorder: Trauma-focused CBT or eye movement desensiti­ zation and reprocessing therapy ■ Pharmacotherapy: SSRis or SNRis as a second-line option ■ Prazosin (u 1 -blocker) is used to treat PTSD-related nightmares ■ CBT alone, SSRis alone, or a combination of both have been found to be similarly effective

NEUROCOGNITIVE DISORDERS Disorders that affect memory, orientation, judgment, and attention.

DEMENTIA (MAJOR NEUROCOGNITIVE DISORDER) A decline in cognitive functioning with global deficits. Level of consciousness is stable (vs delirium). Prevalence is highest among those > 8 5 years of age. The course is persistent and progressive. The most common causes are Alzheimer disease (65 %) and vascular dementia (20% ). Other causes are out­ lined in the mnemonic DEMENTIASS.

History/PE ■ Patients with dementia are usually not concerned about their cognitive decline and are often accompanied to the doctor visit by a family member or friend (vs major depressive disorder [MDD] /pseudodementia).

HIGH -YIELD FACTS IN

599

0-,,. KEY FACT

Top causes of PTSD in male patients are (1 ) sexual assault and (2) war. Top causes of PTSD in female patients are (1 ) childhood abuse and (2) sexual assault.

0 MNEMONIC Causes of dementia­ DEMENTIASS

Degenerative diseases (Parkinson, H u ntington, dementia with Lewy bodies [DLB)) Endocrine (thyroid, parathyroid, pituita ry, adrenal) Metabolic (alcohol, electrolytes, vitamin B 1 2 deficiency, glucose, hepatic, renal, Wilson disease) Exogenous (heavy metals, carbon monoxide, d rugs) Neoplasia Trauma (su bdu ral hematoma) I nfection (meningitis, encephalitis, endoca rditis, syphilis, H IV, prion d iseases, Lyme d isease) Affective disorders (pseudodementia) Stroke/Structu re (vascu lar dementia, ischemia, vasculitis, normal-pressure hyd rocephal us)

600

HIGH-YIELD FACTS IN

PSYCHIATRY ■

■

Dementia is characterized by progressive memory impairment that can be classified into the following four stages: ■ Preclinical: Slight forgetfulness, fully oriented, and capable of caring for oneself. ■ Mild: Moderate memory loss, impaired executive function, impaired function at home but capable of maintaining most chores. Personal hygiene may need prompting. ■ Moderate: Severe memory loss, inability to recognize friends (agnosia), impaired social judgement; requires assistance with dressing and per­ sonal hygiene. ■ Severe: Severe memory loss, oriented only to person, completely dependent on others for ADLs; may develop aphasia and become incommunicable. Personality, mood, and behavior changes are common (eg, wandering and aggression).

Diagnosis

■ ■

TA B L E 2 . 1 3-8.

Diagnosis is clinical. History, PE, and Mini-Mental State Examination (MMSE) 1 8 years of age Evidence of conduct disorder before 15 years of age

CLUSTER C: "WORRIED AND WIMPY"

Obsessivecompulsive

Preoccupied with perfection, order, and control at the expense of efficiency

I nflexible morals and values Note: Remember, in contrast to obsessive­ compulsive disorder, patients with

psychiatry appointment. When a teenager, he was in juvenile detention for theft. He says he does not need to be seen by a "shrink," and that because he was offended by the victim, they deserved to be assaulted.

A 35-year-old woman presents to the office at the request of her boss, who feels she is too focused on minute details on team projects and does not allow others to participate for fear of unwa nted errors. She does not see anything wrong with this style of work, as she believes her coworkers cannot be trusted to pay adequate attention to detail.

obsessive-compulsive personality disorder do not feel their behavior is problematic (ego-syn­ tonic); they also do not have true obsessions and compulsions (continues)

PSYCHIATRY TA B L E 2 . 1 3- 1 5.

HIGH -YIELD FACTS IN

609

Signs and Symptoms of Personal ity Disorders (contin ued)

DISORDER

CHARACTERISTICS

CLI N ICAL PRESENTATION

Avoidant

Socially inhibited, sensitive to rejection Fear of being disliked or ridiculed, yet desires to have friends and social interactions

A 33-year-old man stays at home to avoid an office party, as he fears having to make small talk. He wants to go, but he is more afraid that he

Submissive, clingy; feels a need to be taken care of

A 30-year-old woman presents to the physician's office in crisis, saying that her parents just kicked her out of their house and that she is struggling

Dependent

Has difficulty making decisions Feels helpless

will be inadequate or rejected by others.

to survive on her own. She says she cannot make her own choices at the grocery store, as her mother would always care for her, and now these decisions are overwhelming. She has been sitting outside of their house daily, hoping they will let her live there again.

Defense mechanisms are methods of dealing with anxiety or conflicts of the ego (eg, anger, guilt, inadequacy, grief) . These can be immature (more primitive) or mature (more sophisticated). Immature defense mechanisms are common in personality disorders. Important defense mechanisms are out­ lined in Table 2.13-16. TA B L E 2 . 1 3- 1 6.

Defense Mechanisms

I MMATU RE Acting out

Expressing u nacceptable feelings and thoughts through actions

Denial

Acting as if an aspect of reality does not exist; refusing to accept the situation

Displacement

Transferring feelings or impulses to a more neutral object

l ntellectualization

Using facts and logic to avoid stressful thoughts or emotions

Passive aggression

Demonstrating hostile feelings in a nonconfrontational manner

Projection

Attributing an unacceptable internal impulse to others (vs displacement) Associated with paranoid personality disorder

Rationalization

Explaining unacceptable behaviors in a rational or logical manner

Reaction formation

Behaving in a manner opposite to one's true feelings and thoughts

Regression

I nvoluntarily reverting to an earlier developmental stage Associated with dependent personality disorder

Splitting

Believing that people are either all bad or all good Associated with borderline personality disorder

MATURE Sublimation

Channeling an u nacceptable thought/wish i nto a socially acceptable outlet or behavior

Altruism

Coping with difficult stressors by meeting the needs of others

Suppression

I ntentionally avoiding unwanted thoughts or feelings to deal with reality

H umor

Joking about an uncomfortable or anxiety-provoking situation

61 0

HIGH-YIELD FACTS IN

0.0 MNEMONIC Characteristics ofpersonality disordersMEDIC

Maladaptive Enduring Deviate from cultural norms Inflexible Cause impairment in social or occupational functioning

PSYCHIATRY Diagnosis

Diagnosis is clinical, and detailed history taking is imperative. Collateral information may be helpful. Patients typically deny or do not realize they have a problem (ego syntonic). Treatment ■

Best initial treatment: Psychotherapy Pharmacotherapy is reserved for cases with comorbid mood, anxiety, or psychotic signs/symptoms

SUBSTANCE USE DISORDERS Substance use disorder is a maladaptive pattern of substance use that leads to clinically significant impairment. It can be applied to most substances of abuse. The patient must meet 2:2 of the 11 criteria within a I-year period for diagnosis. The criteria can be grouped into four categories of symptoms and are as follows: Impaired control: 1. Consumption of greater amounts of the substance than intended 2. Failed attempts to cut down use or abstain from the substance 3. Increased amount of time spent acquiring the substance, using it, or recovering from effects 4. Craving ■ Social impairment: 5. Failure to fulfill responsibilities at work, school, or home 6. Continued substance use despite recurrent social or interpersonal problems secondary to the effects of such use (eg, frequent arguments with spouse over the substance use) 7. Isolation from life activities ■ Risky use: 8. Use of substances in physically hazardous situations (eg, driving while intoxicated) 9. Continued substance abuse despite recurrent physical or psychological problems secondary to the effects of the substance use ■ Pharmacologic: 10. Tolerance and use of progressively larger amounts to obtain the same desired effects 11. Withdrawal symptoms when not taking the substance ■

0-,,. KEY FACT Pi n point pupils a re not always a rel iable sign of opioid i ngestion, beca use co-i ngestions can lead to normal or enlarged p u p i l s. Also look for a J, respi ratory rate, track marks, a n d J, breath sou nds.

Tolerance and withdrawal are not needed to make the diagnosis. Withdrawal: ■ Physiologic syndrome that occurs when concentrations of a substance decline in an individual who has had prolonged heavy use of that substance ■ Symptoms vary greatly across substances, but when they occur, the individual is likely to consume the substance again in order to relieve the symptoms ■ For most substances, a history of withdrawal is usually associated with a more severe clinical course

0-,,. KEY FACT Acute pain management is the same for a l l patients with severe pa i n refractory to NSAIDs rega rd less of a h istory of su bsta nce abuse. In the appropriate c l i n ical setting, prior substance abuse is not a contra i n d ication to the use of opioids i n pa i n ma nagement and req u i res a nonjudg menta l environ ment and shared decision making.

Diagnosis/Treatment ■ ■

Diagnosis is typically clinical, and detailed history-taking is imperative Lab tests: Urine and blood toxicology screens, liver function tests (LFTs), and serum ethanol (EtOH)

PSYCHIATRY ■

HIGH -YIELD FACTS IN

61 1

Severity is determined by number of symptoms present ■ Mild: Two to three; moderate: four to five; severe: six or more ■ Symptoms of intoxication and withdrawal from selected drugs are described in Table 2.13-17.

TA B L E 2 . 1 3 - 1 7.

Signs and Symptoms of Substance Abuse

DRUG

I N TOXICATION

WITH DRAWAL

Disinhibition, emotional !ability, slurred speech, ataxia, aggression, blackouts, hallucinations, memory impairment,

6-24 hours: Anxiety, tremor, tachycardia, HTN 1 2-24 hours: Hallucinations 1 2-48 hours: Seizures 48-96 hours: DTs, fever, agitation, HTN,

DEPRESSANTS Alcohol

impaired judgment, stupor, coma

hallucinations Opioids

Euphoria leading to apathy, CNS depression, constipation, pupillary constriction, respiratory depression (life­ threatening in overdose), bradycardia Acute reversal of opioid i ntoxication: Naloxone (short acting so repeat dosing needed) To prevent relapse: Naltrexone (longer acting)

Synthetic opioids

Contains MPTP (synthetic heroin) leading to Parkinson-like dis-

Dysphoria, insomnia, anorexia, myalgias, fever, lacrimation, diaphoresis, dilated pupils, rhi­ norrhea, piloerection, tachycardia, nausea, vomiting, stomach cramps, diarrhea, yawning Opioid withdrawal is not life-th reatening, "hurts all over;' and does not cause seizures; it can be treated with buprenorphine or methadone None

order and loss of pigmented neurons in the substantia nigra Barbiturates

Low safety margin; respiratory depression

Anxiety, seizures, delirium, life-threatening car­ diovascular collapse

Benzodiazepines

I nteractions with alcohol, a mnesia, ataxia, somnolence, mild respiratory depression Should not be used for insomnia in older adults; can cause paradoxical agitation even in relatively low doses

Rebound anxiety, seizures, tremor, insomnia, HTN, tachycardia, death

Inhalants (solvents, glue,

Tachycardia; nystagmus; tremor; ataxia; slurred speech; uncon-

Dysphoria, headache, irritability

fuels)

sciousness followed by drowsiness and headache; perioral rash, common among adolescents Short duration of action Long-term use can lead to irreversible CNS damage and poly­ neuropathy (due to vitamin B 1 2 deficiency)

STI M U LANTS Amphetamines

Psychomotor agitation, impaired judgment, HTN, pupillary dilation, tachycardia, fever, diaphoresis, anxiety, angina, euphoria, grandiosity, prolonged wakefulness/attention, arrhyth mias, del usions, seizures, hallucinations, skin excoria­ tions, poor dentition ("meth mouth") Haloperidol can be given for severe agitation and symptom­

Postuse "crash" with a nxiety, lethargy, head­ ache, stomach cramps, i appetite, fatigue, depression/dysphoria, sleep disturbance, nightmares

targeted medications (eg, antiemetics, NSAIDs) (continues)

61 2

HIGH-YIELD FACTS IN

TA B L E 2 . 1 3 - 1 7.

PSYCHIATRY

Signs and Symptoms of Substance Abuse (continued)

DRUG

INTOXICATION

WITHD RAWAL

Cocaine

Psychomotor agitation, euphoria, impaired judgment,

Postuse "crash" with hypersom nolence, depres­ sion, malaise, i appetite, angina, suicidality,

tachycardia, pupillary dilation, HTN, paranoia, hallucinations, "cocaine bugs" (the feeling of bugs crawling under one's skin), sudden death Chronic use causes -!- appetite, weight loss, erythema of the

nightmares

nasal turbinates and septum perforation ECG changes from ischemia are often seen ("cocaine chest pain") Tx: Benzodiazepines, nonselective a-/�-blockers (eg, labetalol) Caffeine

Restlessness, insomnia, diuresis, muscle twitching, arrhyth­ mias, tachycardia, flushed face, psychomotor agitation

Headache, lethargy, depression, weight gain, irritability, craving

Nicotine

Restlessness, insomnia, anxiety, arrhythmias

I rritability, headache, anxiety, weight gain, craving, bradycardia, difficulty concentrating, insomnia

HALLUCI NOGENS Phencyclidine hydrochloride (PCP)

Assaultive/combative, belligerence, psychosis, violence, impulsiveness, psychomotor agitation, fever, tachycardia, vertical/horizontal nystagmus, HTN, impaired judgment,

Recurrence of intoxication symptoms caused by reabsorption in the GI tract; sudden onset of severe, random violence

ataxia, seizures, delirium Benzodiazepines or haloperidol can treat severe symptoms; otherwise, physician should offer reassurance Gastric lavage can help eliminate the drug Lysergic acid diethyl­ amide (LSD)

Marked a nxiety or depression, delusions, visual hallucina­

None

tions, flashbacks, pupillary dilation, impaired judgment, diaphoresis, tachycardia, HTN, heightened senses (eg, colors become more intense) Tx: supportive counseling, traditional antipsychotics for psy­ chotic symptoms, benzodiazepines for anxiety

Marijuana (tetrahydro­ can nabinol [THC], cannabis)

Euphoria, laughter, slowed sense of time, impaired judgment, social withdrawal, appetite, dry mouth, conjunctiva! injec­ tion, hallucinations, a nxiety, paranoia, -!- motivation

I rritability, anxiety, -!- appetite, insomnia

Bath salts (synthetic cathinones)

Stimulant drug that causes agitation, combativeness, delirium,

Anxiety, depression, insomnia

and psychosis that may last for weeks; not detected on routine urine toxicology screens

Gamma-hydroxybutyric acid (GH B)

Hypotension, bradycardia, respiratory depression, disinhibi­ tion, i libido, seizures

I rritability, anxiety, tremor, autonomic instability

MOMA (ecstasy)

Amphetamine derivative with hallucinogenic properties; popular at dance parties or "raves" I ntoxication: HTN, euphoria, perceptual changes, bruxism,

Depression, anxiety, difficulty concentrating

hyperthermia, heat exhaustion, hyponatremia; may a lso precipitate serotonin syndrome DTs, Delirium tremens; HTN, hypertension; MPTP, 1 -methyl-4-phenyl-1 ,2,3,6-tetrahydropyridine; NSA/Ds, nonsteroidal anti-inflammatory drugs.

PSYCHIATRY

HIGH -YIELD FACTS IN

61 3

ALCOHOL USE DISORDER Occurs more often in males (4: 1) and in those 21 to 34 years of age, although the incidence in females is rising. Alcohol use disorder is associated with a positive family history.

History/PE See Table 2.13-17 for the symptoms of intoxication and withdrawal. The phy­ sician should look for palmar erythema or telangiectasias and for other signs and symptoms of end-organ complications. Patients often present with sleep disturbances or anxiety symptoms caused by mild withdrawal. Diagnosis ■ Screening can be done with the CAGE questionnaire. The physician should monitor vital signs for evidence of withdrawal. ■ Labs may reveal i LFTs (classically aspartate aminotransferase [AST]: alanine aminotransferase [ ALT] ratio > 2:1), i lactate dehydrogenase (LDH), i carbohydrate-deficient transferrin, and i mean corpuscular volume. Treatment ■ Abstinence: Best initial treatment: Naltrexone (µ-opioid receptor blocker) ,J, crav­ ings; can start while patient is still drinking Long-term rehabilitation (eg, Alcoholics Anonymous) ■ Aversion: Disulfiram (acetaldehyde dehydrogenase inhibitor): Produces an unpleasant response (eg, flushing, nausea, vertigo, palpitations) when EtOH is consumed ■ Withdrawal: ■ Stabilization of vital signs; correction of electrolyte abnormalities ■ Thiamine (administer before glucose to prevent Wernicke encephalop­ athy), glucose, and folic acid Medium-length benzodiazepine taper (eg, lorazepam, diazepam, chlordiazepoxide) Addition of haloperidol for hallucinations and psychotic symptoms Com pl ications ■ Gastritis (gastrointestinal [GI] bleeds, ulcers), vances, or Mallory-Weiss tears ■ Pancreatitis, liver disease, delirium tremens (DTs), alcoholic hallucinosis (see Table 2.13-18, Fig. 2.13-2), peripheral neuropathy, Wernicke enceph­ alopathy, Korsakoff psychosis, fetal alcohol syndrome, cardiomyopathy, anemia, aspiration pneumonia, i risk for sustaining trauma (eg, subdural hematoma) TA B L E 2 . 1 3 - 1 8.

Alcoholic Hall ucinosis vs Del irium Tremens

ALCOHOLIC HALLUCI NOSIS

DELIRIUM TR EM ENS

1 2-24 hours since last drink Visual, auditory, and tactile hallucinations

48-96 hours since last drink Autonomic instability (hyperadrenergic state; i blood pressure [BP], i heart rate [HR]) Disorientation, agitation Hallucinations

l,).t>

MNEMONIC

CAGE questionnaire:

1 . Have you ever felt the need to Cut down on you r drinking? 2. Have you ever felt Annoyed by criticism of you r drinking? 3. Have you ever felt Gui lty a bout drinking? 4. Have you ever had to take a morning Eye opener?

More than one "yes" answer makes alcohol use disorder likely.

0-n

KEY FACT

Naltrexone is a first-line pharmacotherapy to reduce the craving for alcohol. It works by blocking the µ-opioid receptor and can be given to patients who are still drinking.

Alcoholic hallucinosis (usually visual)

Withdrawal seizu res

Tremors, insomnia, diaphoresis, agitati on, G I u pset

I I I

0 3 6

12

24

36

Deli rium tremens

48

I►

96

Time from last drink (hours) F I G U RE 2 . 1 3-2. Alcohol withdrawal timeline. Alcohol withdrawal can have

overlapping symptoms. Time from last drink is important to delineate the type of withdrawal and subsequent manage­ ment. (Reprod uced with permission from USMLE­ Rx.com.)

61 4 TA B L E 2 . 1 3 - 1 9.

HIGH-YIELD FACTS IN

PSYCHIATRY

Symptoms and Treatment of Drug Withdrawal

DRUG

WITHDRAWAL SYM PTOMS

TREATMENT

Alcohol

Life-threatening (mortality up to 5%) Mild withdrawal: Tremor (fi rst symptom); tachy­

Benzodiazepines (can require massive doses); thia­ mine, folate, m ultivita min replacement (banana

cardia, HTN, agitation (within 48 hours) Alcoholic hallucinations: Visual hallucinations without delirium ( 1 2-48 hours) Delirium tremens: Visual hallucinations with severe

bag-does not affect withdrawal, people with alcoholics use disorder)

a utonomic instability, delirium, seizures, and possibly death (within 2-7 days) Benzodiazepines and barbiturates

Life-threatening (mortality is rare) Tremor, rebound anxiety, insomnia, delirium/hal­ lucinations, seizures

Benzodiazepine taper

May mimic alcohol withdrawal, but HTN/ tachycardia usually absent Cocaine/amphetamines

Not life-th reatening Depression, hyperphagia, hypersomnolence, con­ stricted pupils

IV benzodiazepines and supportive treatment Avoidance of pure �-blockers (lead to unopposed et activity, causing hypertensive crisis)

Opioids

Not life-th reatening

Mild: Ondansetron, loperamide, benzodiazepines,

Anxiety, insomnia, flulike symptoms, piloerection, fever, rhinorrhea, lacrimation, yawning, nausea, stomach cramps, diarrhea, dilated pupils

NSAIDs Severe: Clonidine for a utonomic symptoms; buprenorphine or methadone for craving

0-,,. KEY FACT

Neonatal abstinence syndrome occurs when a neonate suffers symptoms of substance withdrawal because of in utero exposure to that substance on a consistent basis.

MANAGEMENT OF DRUG WITHDRAWAL Table 2.13-19 summarizes common drug withdrawal symptoms and treatment.

EATING DISORDERS ANOREXIA NERVOSA Risk factors include female sex, low self-esteem, and high socioeconomic sta­ tus. Associated with OCD; MDD; anxiety; and careers/hobbies such as mod­ eling, gymnastics, ballet, and running

H istory/PE ■ Patients are often perfectionists and high achieving. They have a distorted body image and fear of gaining weight. Anorexia nervosa is divided into two subtypes: ■ Restrictive: Severe restriction of food intake is primary method of weight loss. ■ Binge eating/purging: Food intake is compensated by purging (eg, excessive exercise, vomiting, laxative/diuretic abuse).

PSYCHIATRY

HIGH -YIELD FACTS IN

61 5

■ Si gns and symptoms: Cachexia, body mass index (BMI) < 18. 5 kg/m2 , lanugo, d ry skin, bradycardia, lethargy, hypotension, cold intolerance, and hypothermia (as low as 3 5 ° C [9 5 ° F]). ■ See Table 2.13-20 to differentiate anorexia nervosa from bulimia nervosa.

Diagnosis ■ Measure height and weight; check BMI; check CBC, electrolytes, endocrine levels, and ECG. ■ Perform a psychiatric evaluation to screen patients for comorbid conditions. Treatment See Table 2.13-20. Com pl ications See Table 2.13-20 and Table 2.13-21. Mortality from suicide or medical com­ plications is > 10%. TA B L E 2 . 1 3-20.

Anorexia Nervosa vs Bulimia Nervosa

CHARACTERISTIC

ANOREXIA N ERVOSA

BULIMIA N ERVOSA

Presentation

Persistent restriction of caloric intake resulting in low body

Episodes of binge eating followed by compensa­

weight; intense fear of gaining weight; distorted body image (patients perceive themselves as overweight or obese)

tory behaviors (eg, purging, fasting, excessive exercise) Episodes occur at least once a week for 2:3 months

Weight

Patients are underweight (BMI < 1 8.S kg/m')

Patients are of normal weight or are overweight (BMI > 1 8.S kg/m')

Attitude toward illness

Patients are typically not distressed by their ill ness and may

Patients are typically distressed about their symp­

thus be resistant to treatment Treatment

toms and are thus easier to treat

Monitor calorie intake and weight gain; hospitalize if

Psychotherapy :t a ntidepressants (SSRls)

necessary Watch for refeeding syndrome (electrolyte abnormalities [-I, phosphate], arrhythmias, respiratory failure, and seizures

Treat comorbidities; avoid bupropion because of risk for seizure

after sudden increase in caloric intake) Psychotherapy: Address maladaptive family dynamics Antidepressants (SSRls): Note that these are not effective until weight is restored Treat comorbidities; avoid bupropion because of risk for seizure

TA B L E 2 . 1 3-2 1 .

Medical Compl ications of Eating Disorders

CO N STITUTI ONAL

CARDIAC

GASTROI NTESTINAL

GEN ITOU RINARY

OTHER

Cachexia

Arrhythmias

Dental erosions and

Amenorrhea

Dermatologic: Lan ugo

Hypothermia Fatigue Electrolyte abnormalities

Sudden death Hypotension Bradycardia Prolonged QT i nterval

decay Abdominal pain Delayed gastric emptying

Nephrolithiasis

Hematologic: Leukopenia Neurologic: Seizures Musculoskeletal: Osteoporosis, stress fractures

(hypokalemia, pH abnormalities)

61 6

HIGH-YIELD FACTS IN

PSYCHIATRY BULIMIA NERVOSA Eating disorder characterized by recurrent episodes of binge eating and com­ pensatory purging behavior (eg, vomiting, laxative/diuretic abuse, excessive exercise). More common in females; associated with low self-esteem, mood disorders, and OCD.

History/PE Patients often have a long history of other comorbid psychiatric conditions (eg, anxiety, depression) and are concerned about their behaviors. Signs: Dental enamel erosion, enlarged parotid glands, scars on the dorsal hand surfaces (if there is a history of repeated induced vomiting), and BMI > 18. 5 kg/m2 are signs of bulimia nervosa. ■ See Table 2.13-20 to differentiate anorexia nervosa from bulimia nervosa. Treatment See Table 2.13-20. Complications See Table 2.13-21 for a summary of complications related to eating disorders.

SEXUAL DISORDERS SEXUAL CHANGES WITH AGING ■

Interest in sexual activity usually does not -!. with aging. Males usually require i stimulation of the genitalia for longer periods of time to reach orgasm; intensity of orgasm -!., and the length of the refrac­ tory period before the next orgasm i. In females, estrogen levels -!. after menopause, leading to vaginal dryness and thinning, which may result in discomfort during coitus. The patient may be treated with hormone replacement therapy, estrogen vaginal sup­ positories, or other vaginal creams.

PARAPHILIC DISORDERS Preoccupation with or engagement in unusual sexual fantasies, urges, or behaviors for >6 months with clinically significant impairment of one's life. There are eight classified disorders, characterized by disordered court­ ship (voyeurism, exhibitionism, and frotteurism), disordered preferences (pedophilia, transvestic fetishism, fetishism), and pleasure in inflicting/ receiving pain (sadism, masochism). See Table 2.13-22. ■ Tx: Includes insight-oriented psychotherapy and behavioral therapy. Anti­ androgens (eg, a medroxyprogesterone injection) have been used for hypersexual paraphilic activity. ■

GENDER DYSPHORIA Significant incongruence between one's gender identity and one's gender assigned at birth, lasting >6 months and leading to persistent distress. Individ­ uals experience marked discomfort with assigned gender, which interferes with social, academic, and other areas of function. More common in males than females.

PSYCHIATRY

History/PE More common in males than in females. Gender dysphoria is associated with depression, anxiety, substance abuse, and personality disorders. ■ Some individuals who are transgender will experience gender dysphoria. Nonconformity to one's assigned gender itself is not a mental disorder. Gender dysphoria is associated with depression, anxiety, substance abuse, and personality disorders. Treatment ■ Address comorbid psychiatric conditions. If the patient is interested, dis­ cuss options for gender-affirming surgery or hormonal treatment. ■ In teens, hormone suppression therapy can be offered to delay puberty, but this decision should be made with support from family, if possible. One should also assess safety and multidisciplinary specialist services.

SEXUAL DYSFUNCTION History/PE ■ Problems in sexual arousal, desire, or orgasm or pain with sexual intercourse ■ Prevalence: 30%; one-third of cases are attributable to biologic factors, and another third to psychological factors Treatment Depends on the condition. Pharmacologic strategies include phosphodiester­ ase type 5 (PDE5) inhibitors (eg, sildenafil, tadalafil). If dysfunction is caused by antidepressants (SSRis), the physician can institute a switch to bupropion. Psychotherapeutic strategies include sensate focusing. TA B L E 2 . 1 3 - 2 2 .

Features of Common Paraphilic Disorders

DISORDER

CLI N I CAL MAN I F ESTATI O N S

Exhibitionistic

Sexual arousal from exposing one's genitals to a stranger

Pedophilic

U rges or behaviors i nvolving sexual activities with children

Voyeuristic

Observing unsuspecting people unclothed or involved in sex

Fetishistic

Use of nonliving objects (often clothing) for sexual arousal

Transvestic

Cross-dressing for sexual arousal

Frotteu ristic

Touching or rubbing one's genitalia against a nonconsenting person (common in crowded places)

Sexual sadism

Sexual arousal from inflicting suffering on sexual partner

Sexual masochism

Sexual arousal from being hurt, humiliated, bound, or th reatened

HIG H -YIELD FACTS IN

61 7

61 8

HIGH-YIELD FACTS IN

PSYCHIATRY

SLEEP DISORDERS Up to one-third of all American adults suffer from some type of sleep disorder during their lives. Dyssomnia describes any condition that leads to a distur­ bance in the normal rhythm or pattern of sleep. Insomnia is the most com­ mon example. Risk factors include female sex, the presence of mental and medical disorders, substance abuse, and advanced age. Normal age-related sleep changes include more frequent waking, decreased total time asleep, and increased napping.

0-rr

KEY FACT

Sleep hygiene measures-stim u l us control therapy to reestablish a 24-hour sleep/wake cycle: ■ Establish a sleep sched ule ■ Limit caffeine intake ■ Avoid naps ■ Take warm baths in the evening ■ Use the bed room for sleep and sexual activity only ■ Exercise ea rly i n the day ■ Employ relaxation tech niques ■ Avoid large meals nea r bedtime

PRIMARY INSOMNIA Affects up to 30% of the general population; causes sleep disturbance that is not attributable to physical or mental conditions. Insomnia is often exacerbated by anxiety, and patients may become preoccupied with getting enough sleep. H i story/PE

Patients present with a history of nonrestorative sleep or difficulty initiating or maintaining sleep that is present at least three times per week for 1 month. Treatment ■ ■

Best initial treatment: Initiate good sleep hygiene measures. Next best treatment: Psychotherapy, specifically CBT for insomnia (CBTi). Pharmacotherapy can be initiated with care for short periods of time ( 1 month. The excessive somnolence cannot be attributable to medical or mental illness, medications, poor sleep hygiene, insufficient sleep, or narcolepsy. Treatment ■ ■

Best initial treatment: CNS stimulants (eg, amphetamines) Antidepressants such as SSRls may be useful in some patients

NARCOLEPSY Onset typically occurs by young adulthood, generally before 30 years of age. Some forms of narcolepsy may have a genetic component. Diagnosis ■

■

Manifestations include excessive daytime somnolence and .J, rapid eye movement (REM) sleep latency at least three times a week for at least 3 months. Hypocretin deficiency (confirmed by cerebrospinal fluid [CSF] sampling) is also diagnostic of this condition. Sleep attacks are the classic symptom; patients cannot avoid falling asleep. Characteristic excessive sleepiness may be associated with the following: ■ Cataplexy: Sudden loss of muscle tone that leads to collapse ■ H ypnagogic hallucinations: Occur as the patient is falling asleep

PSYCHIATRY

■

H ypnopompic hallucinations: Occur as the patient awakens Sleep paralysis: Brief paralysis upon awakening

Treatment

A regimen of scheduled daily naps plus stimulant drugs such as amphet­ amines or modafinil; SSRls for cataplexy.

HIGH -YIELD FACTS IN

61 9

0.0 MNEMONIC HypnaGOgic = halluci nations while GOing to bed HypnoPOMPic = halluci nations while POMPing out of bed

SLEEP APNEA ■

Sleep apnea occurs secondary to disturbances in breathing during sleep that lead to excessive daytime somnolence and sleep disruption. Etiologies can be either central or peripheral. ■ Central sleep apnea (CSA) : A condition in which both airflow and respiratory effort cease. CSA is linked to morning headaches, mood changes, and repeated awakenings during the night. ■ Obstructive sleep apnea (OSA): A condition in which airflow ceases as a result of obstruction along the respiratory passages. OSA is strongly associated with snoring. Risk factors: Male sex, obesity, prior upper air­ way surgeries, a deviated nasal septum, a large uvula or tongue, and ret­ rognathia (recession of the mandible). ■ In both forms, arousal results in cessation of the apneic event. ■ Sleep apnea is associated with sudden death in infants and older adults, headaches, depression, i systolic blood pressure (BP), and pulmonary hypertension. Diagnosis

Sleep study (polysomnography) to document the number of arousals, obstruc­ tions, and episodes of .J, 0 2 saturation; distinguish OSA from CSA; and iden­ tify possible movement disorders, seizures, or other sleep disorders. Treatment

■ OSA: Nasal continuous positive airway pressure (CPAP). Weight loss if obese. In children, most cases are caused by tonsillar/adenoidal hypertro­ phy, which is corrected surgically. ■ CSA: Mechanical ventilation (eg, bilevel positive airway pressure [BiPAP]) with a backup rate for severe cases.

CIRCADIAN RHYTHM SLEEP DISORDER A spectrum of disorders characterized by a misalignment between desired and actual sleep periods. Subtypes include jet lag, shift work, delayed sleep phase ("night owls"), advanced sleep phase ("early birds"), and unspecified causes. Treatment ■

■ ■

Jet-lag type usually resolves within 2 to 7 days without specific treatment. Shift-work and delayed sleep-phase types may respond to light therapy. Modafinil is approved for shift-work sleep disorder. Oral melatonin may be useful if given 30 minutes before the desired bedtime.

I A 57-year-old morbid ly obese man prese nts to his physician with co ncerns a bout i d ayti me s l eepi ness and J, wo rk prod u ctivity. H i s wife adds that he has excessive snoring that so u n d s l i ke "the snort of a stea m e n g i n e:' What lo n g-term co m p l ications a re of concern for this patient?

620

HIGH-YIELD FACTS IN

PSYCHIATRY

SOMATIC SYMPTOM AND RELATED DISORDERS SOMATIC SYMPTOM DISORDER Patients often present with excessive thoughts, anxiety, and behaviors driven by the presence of somatic symptoms that are distressing and negatively affect daily life. Somatic symptom disorder may occur with or without any medical illness present. High health care utilization is often present. Disorder may present with multiple recurrent somatic symptoms that may be specific (eg, localized pain) or nonspecific (eg, fatigue). Even normal bodily symptoms can be perceived as unduly threatening, even when there is evidence to the contrary. Treatment

Scheduling regular appointments with one clinician as primary caregiver Avoiding unnecessary diagnostics but legitimizing symptoms ■ Psychotherapy focused on reducing psychosocial stressors ■

ILLNESS ANXIETY DISORDER

0-,,. KEY FACT Psychogen ic/nonepileptic spel ls ca n co-occu r with a seizu re d i sorder.

Formerly known as hypochondria. For at least 6 months, patients have anxiety about and preoccupation with acquiring a serious medical illness despite hav­ ing no somatic symptoms (or mild somatic symptoms), a normal physical examination, negative tests, and reassurance from a health care provider. The patient's preoccupation with illness is not better explained by another disor­ der. In addition to the aforementioned, patients must have one of the follow­ ing: excessive health behaviors (eg, repeated checking for signs of an illness) or maladaptive avoidance of situations (eg, health care settings, visiting sick family members). Treatment

CBT (first line) Another type of psychotherapy (second line) ■ Antidepressant medication (third line) ■

CONVERSION DISORDER Also known as functional neurologic symptom disorder. Characterized by symptoms or deficits of voluntary motor or sensory function (eg, blindness, seizurelike movements, paralysis) incompatible with medical processes. Close temporal relationship to stress or intense emotion. Diagnosis

Symptoms unexplained by other medical or neurologic causes Signs during physical examination suggesting nonorganic cause of symptoms: ■ Presence of Hoover sign (extension of affected leg when asked to raise the unaffected contralateral leg) when attempting to rule out leg paralysis ■ Eyes closed and resistant to opening during seizure; negative simulta­ neous EEG ■ Disappearance of tremors with distraction ■ La belle indifference: Patients are strangely indifferent to their symptoms; commonly associated but not required for the diagnosis ■

This patient has obstructive sleep a p n ea . Serious conseq uences i nc l u d e leg swel l i ng, hyperte n s i o n , car p u l mona le, stroke, a n d c l i n ica l depression.

PSYCHIATRY

HIGH -YIELD FACTS IN

62 1

Treatment ■ Psychotherapy, physical therapy (PT)/occupation therapy (OT), treating comorbid psychiatric issues (anxiety, depression, trauma) ■ Goal: Improve function

FACTITIOUS DISORDERS AND MALINGERING Diagnosis

Factitious disorder (formerly Munchausen syndrome) : Characterized by the fabrication of symptoms or self-injury to assume the sick role (primary gain) ■ Factitious disorder imposed on another (formerly Munchausen by proxy) : Caregiver exaggerates or falsifies medical/psychiatric symptoms or intentionally induces illness in someone else to receive benefit by taking on the role of concerned caregiver ■ Malingering: Patients intentionally cause or feign symptoms for secondary gain (eg, financial, housing, legal) ■

0-,,. KEY FACT

Factitious disorders and malingering are distinct from somatoform disorders in that they involve conscious and intentional processes.

Treatment ■ Psychotherapy ■ Minimal diagnostics and treatment to avoid reinforcement of behaviors ■ Contacting appropriate legal authorities (factitious disorder imposed on another)

SEXUAL AND PHYSICAL ABUSE Most frequently affects females < 3 5 years of age who : Are experiencing marital discord and have a personal history of, or a partner with, substance abuse Are pregnant, have low socioeconomic status, or have obtained a restraining order ■ Victims of childhood abuse are more likely to become adult victims of abuse

■

History/PE

Patients typically have multiple somatic complaints, frequent emergency department visits, and unexplained injuries with delayed medical treat­ ment. They may also avoid eye contact or act afraid or hostile. ■ Children may exhibit precocious sexual behavior, genital or anal trauma, sexually transmitted diseases (STDs), UTis, and/or psychiatric/behavioral problems (see Pediatrics chapter). ■ Other clues include a partner who answers questions for the patient or refuses to leave the examination room. ■

Treatment ■ Perform a screening assessment of the patient's safety domestically and in their close personal relationships. ■ Provide medical care, emotional support, and counseling. ■ Educate the patient about support services and refer the patient appropriately. ■ Documentation is crucial. Know local laws for reporting suspected child/ elder abuse.

0-,,. KEY FACT

Sexual abusers are usually male and are often known to the victim (and are often family members).

622

HIGH-YIELD FACTS IN

PSYCHIATRY SEXUAL ASSAULT Any sexual act performed on another individual without their consent. Diagnostic Eval uation and Testing ■ ■ ■

Assessment for physical inj ury with focus on genital trauma Psychological evaluation Pregnancy test Testing for sexually transmitted infections ■ Smear/culture for chlamydia and gonorrhea ■ Wet mount and culture for trichomonas ■ Consideration of testing for HIV, herpes simplex virus (HSY), hepatitis B virus (HBV), syphilis, and cytomegalovirus (CMV) Forensic evaluation with detailed history and samples from buccal mucosa, vagina, rectum, fingernail scraping and clippings, blood samples, and saliva samples ■ Important historical details include contraceptive use, last time of coitus, condom use before the assault, drug or alcohol use, history of STDs, description of the assailant, location and time of the assault, cir­ cumstances of the assault (eg, penile penetration, use of condoms, extragenital acts, use or display of weapons), and the patient's actions since the assault (eg, douching, bathing, brushing teeth, urination/def­ ecation, changing clothes).

Postexposure Prophylaxis a n d Eva l uation

Follow-up medical visit within 1 to 2 weeks Ceftriaxone plus azithromycin ± metronidazole for prophylaxis against gonorrhea and chlamydia ■ Hepatitis B booster vaccination if unknown vaccination and immune sta­ tus or if unvaccinated ■ HIV: Antiretroviral drug offered within 72 hours of assault; options are tenofovir-emtricitabine + raltegravir ■ Human papillomavirus (HPV) vaccination recommended at time of initial evaluation in female survivors ages 9 to 26 years and male survivors ages 9 to 21 years ■ ■

Contraception

Emergency contraception: Progestin/antagonist/agonist ulipristal or levonorg­ estrel or combination ethinyl estradiol and levonorgestrel (Yuzpe regimen) ■ Offer for mental health services ■

0,◊ MNEMONIC

SUICIDALITY

Risk factors for suicide attempts­ SAD PERSONS Sex (male) Age (older) Depression Previous attempt (greatest risk) Ethanol/substance abuse Rational thinking loss Sickness (chronic illness) Organized plan/access to weapons No spouse Social support lacking

Accounts for 45,000 deaths per year in the United States; the 10th overall cause of death in the United States. Approximately one suicide occurs every 11 minutes. Risk factors: Previous suicide attempt (primary risk factor), male sex, >45 years of age, psychiatric disorders (eg, MOD, presence of psychotic symptoms), history of psychiatric hospitalization, history of violent behav­ ior, ethanol or substance abuse, recent severe stressors, poor social support, and a family history of suicide (see SAD PERSONS mnemonic). ■ Females are more likely to attempt suicide. Men use more lethal methods (eg, firearms) and are more likely to complete suicide. ■

PSYCHIATRY Adults aged 45 to 64 have higher rates of death from suicides than other age groups. However, young adults aged 18 to 25 are at higher risk for sui­ cidal thoughts and attempts than other age groups. ■ Protective factors: Social support, family connectedness, religiosity, pregnancy, and parenthood. ■

Diagnosis ■ Perform a comprehensive psychiatric evaluation. ■ Ask about family history, previous attempts, ambivalence toward death, and hopelessness. ■ Ask directly about suicidal ideation, intent, and plan, and look for avail­ able means. Treatment ■ A patient who endorses suicidality requires emergent inpatient hospitaliza­ tion even against their will. ■ Suicide risk may increase after antidepressant therapy is initiated and is considered an adverse effect of the medication. There is a black box warn­ ing on all antidepressant medications when used in those 0.70

Norma l/i

FEV, (% of predicted)

80%-1 20%

t

FVC (% of predicted)

80%-1 20%

t

t

FRC (% of pred icted)

80%-1 20%

i

TLC (% of predicted)

80%-1 20%

t

i

t

Asth ma triggers i n c l ude a l lerge ns, u p per respi ratory i nfections (URls), cold a i r, exercise, drugs (eg, aspirin, NSAI Ds, [3-blockers), a n d stress in both adu lts and c h i l d re n .

Lung capacities

Lung volumes

ERV

KEY FACT

t

FEVl : Forced expiratory vol u me in 1 second; FRC: fu nctional resid ual capacity; FVC: forced vita l capacity; TLC: tota l l u ng capacity.

I RV

0--n

Norma l/t

RV

Volume (L)

1 .2

---------� o

llI

IC

l

VC TLC

FRC

F I G U R E 2. 1 4- 1 . Lung volumes in the interpretation of pulmonary function tests (PFTs). Left panel shows lung volumes, and right panel shows lung capacities. ERV, Expiratory reserve volume; FRC, functional residual capacity; IC, inspiratory capacity; IRV, inspiratory reserve volume; RV, residual volume; TLC, total lung capacity; TV, total volume; VC, vital capacity. (Reproduced with permission from USMLE-Rx.com.)

PULMONARY ■

Aspirin-exacerbated respiratory disease: Samter triad has three clinical fea­ tures: asthma, chronic rhinosinusitis with nasal polyps, and intolerance to aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs; most commonly upper and lower respiratory tract symptoms but occasionally also rash, abdominal pain, or vomiting). Pseudoallergic reaction (not lgE mediated)

Diagnosis ■ Best initial test: Spirometry/pulmonary function tests (PFTs); obstructive pattern that is reversible with short-acting J3 2-agonists (SABAs) Forced expiratory volume in 1 second/forced vital capacity (FEV i /FVC) 200 mL in FEV 1 with SABA (albuterol). PFTs are often normal between exacerbations. ■ Methacholine challenge: Tests for bronchial hyperresponsiveness; useful when PFTs are normal but asthma is still suspected. The methacholine challenge is considered positive with 2:::20% decrease in FEV 1 • The test is sensitive but not specific. ■ Arterial blood gas (ABG): Early exacerbation: Respiratory alkalosis is caused by hyperventilation (j, Paco 2 , I pH). Late/severe exacerbation (impending respiratory failure): Respiratory muscle fatigue results in respiratory acidosis caused by inability to ven­ tilate (normalizing Paco 2 , normalizing pH, .J, partial pressure of oxygen in arterial blood [Pao 2 ] ) . ■ X-ray of the chest (CXR) : Normal appearance to hyperinflation with flattening of the diaphragm. Treatment In general, avoidance of allergens or any potential triggers. See Tables 2.14-2 and 2.14-3 for asthma medications and management guidelines. ■

Acute exacerbation: 0 2 , SABA (albuterol is first-line), systemic glucocorticoids. SABN ipratropium and magnesium can be used in severe exacerbations. lpratropium should not be used alone in asthma treatment. OBSTRUCTIVE

Loop shifts to the left ,, ' '

'

I I I I I

I

I I I I

\'

'

'

' '

'

\ '

'

-',

NORMAL 8

8

I

' ... _ _ __ ,, ,

,,

,'

,

The physician should suspect impending respiratory failure in a patient with severe asthma exacerbation and normal or normalizing Paco2 and pH.

0-,r KEY FACT

Summary of asthma medications: PRN (as needed) medications­ short-acting bronchodilators (eg, albuterol) ■ Long-term medications-inhaled corticosteroids, long-acting !3 2 agonists (eg, salmeterol), long­ acting muscarinic antagonists (LAMAs), leukotriene antagonists (eg, montelukast), and PO (by mouth) corticosteroids. ■

t:).0 MNEMONIC Medications for asthma exacerbationsASTHMA

Al buterol (bronchodilator) Steroids (anti-inflammatory) Theophyl line (ra rely used bronchodilator due to narrow therapeutic index) Humidified 02 (in hypoxemic patients) Magnesium (bronchodilator used in severe exacerbations) Anticholinergics (bronchodi lator)

4

4

Loop shifts to the right

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I I I I

I

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\

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0

8 4 8

6

2

!­ --- - -�_.......,____ Volume (L)

0 1

!>RV :>---VC----ai >-- TLC -t--"

8

627

0-,r KEY FACT

RESTRICTIVE

\

2:

HIGH -YIELD FACTS IN

0

4 8

F I G U RE 2 . 1 4-2. Obstructive vs restrictive lung disease. Shown are typical alterations in lung volumes and capacities in restrictive and obstructive diseases. Normal flow-volume loops shown in center panel. Obstructive lung disease (left) causes increased reserve volume and total lung capacity due to air trapping. Restrictive lung disease shows a reduction in all lung volumes due to reduced lung expansion (right). RV, Residual volume; TLC, total lung capacity; VC, vital capacity. (Reprod uced with perm ission from USM LE-Rx.com.)

628

HIGH-YIELD FACTS IN

TA B L E 2.1 4-2.

PULMONARY

Common Asthma Medications and Their Mechanisms

DRUG

MECHANISM OF ACTION

13,-agonists

Albuterol: Short-acting (SABA); relaxes bronchial smooth muscle (!32-adrenoceptors) Salmeterol + inhaled corticosteroids (ICS): Long-acting (LABA) agent for maintenance therapy Formoterol + ICS: Maintenance and reliever therapy (MART) with both short-acting and long-acting effects

Corticosteroids

Inhaled corticosteroids: First-line treatment for long-term control of asth ma Beclomethasone, prednisone: I n hibit the synthesis of cytokines

Muscarinic antagonists

lpratropium: Short-acting muscarinic antagonist (SAMA); competitively blocks muscarinic receptors, preventing bronchoconstriction Tiotropium: Long-acting muscarinic antagonist (LAMA)

Methylxanthines

Theophylline: Causes bronchodilation by inhibiting phosphodiesterase, thereby t cAMP hydrolysis and cAMP levels; limited usage because of narrow therapeutic-toxic index (cardiotoxicity, neurotoxicity)

Cromolyn

Prevents the release of vasoactive mediators from mast cells Useful for exercise-induced bronchospasm Effective only for the maintenance of asthma; not effective during an acute attack; toxicity is rare

Antileukotrienes

Zileuton: A 5-lipoxygenase pathway inhibitor; blocks conversion of arachidonic acid to leukotrienes Montelu kast, zafirlukast: Block leukotriene receptors

Anti-lgE

Omalizumab: Monoclonal antibody against lgE; inhibits lgE binding to lgE receptor (FceRI) on mast cells; used in patients with allergic asth ma and high baseline lgE level

Anti-lL-5/anti-lL-SR

Mepolizumab, reslizumab: Monoclonal antibody against i nterleukin (IL)-5 (potent chemoattractant for eosinophils) Benralizumab: Monoclonal antibody against IL-5 receptor, which blocks binding of IL-5, resulting in inhibition of eosinophil differentiation and maturation in bone marrow; refer to Table 2.1 4-3

Anti-lL-4R

Dupilumab: Binds IL-4 receptor and inhibits IL-4 and IL-1 3 cytokine-induced responses, thus inhibiting release of inflammatory cytokines, chemokines, and lgE; refer to Table 2.1 4-3

0-n

KEY FACT

Corticosteroids i n h a led in a rush ca n lead to thrush!

0-n

KEY FACT

Ad u lts a n d adolescents diagnosed with asthma, even m i l d forms, should use ICSs to control a i rway i nfl a m m ation. Benefits of low-dose ICSs incl ude i m p roved l u ng fu nction a n d red uctions i n sym ptoms, severe exacerbations, morta l ity, a n d exercise-i n d u ced bronchoconstriction.

The physician should consider intubation in severe cases (cyanosis, inability to maintain respiratory effort, altered mental status) or in patients with a Paco 2 > 50 mm Hg or Pao 2 < 50 mm Hg. ■ Initiation and adjustment of maintenance therapy: ■ Initiation of treatment is determined by asthma symptom severity at baseline. ■ Therapy may include a combination of controller medications (which prevent exacerbations, eg, salmeterol + ICS) and reliever medications (which treat exacerbations acutely, eg, albuterol). Certain medications (formoterol + ICS) have both controller and reliever effects. ■ Controller therapy may be stepped up or down in intensity according to patient's needs. ■

BRONCHIECTASIS A disease caused by recurrent cycles of infection and inflammation in the bronchi/bronchioles that leads to fibrosis, remodeling, and permanent dila­ tion of bronchi (see Fig. 2.14-3).

HIGH -YIELD FACTS IN

P U LMO NA RY

TA B L E 2 . 1 4-3.

629

Initiation of Asthma Treatment in Adults and Adolescents Aged 1 2 + Based on Global Initiative for Asthma (GINA) Guidelines

PREFERRED TRACK' STEP 1

STEP 2

STEP 3

STEP 4

STEP 5

As-needed low-dose

As-needed low-dose

Low-dose maintenance

Medium-/high-dose

Addition of LAMA

ICS/formoterol

ICS/formoterol

ICS/LABA

maintenance ICS/LABA

Phenotypic assessment ± anti-lgE, anti-l L-5/5R, anti-lL-4R Consideration of high-dose ICS/LABA

Reliever medication: Low-dose ICS/formoterol as needed for all steps Criteria for initiation of treatment at various steps:

STEP 1 : Symptoms less than two times/month STEP 2: Symptoms fewer than 4-5 days/week STEP 3: Symptoms on most days OR on waking more than once a week STEP 4: Symptoms daily OR on waking more than once a week with low lung function When to step up ongoing treatment:

Treatment can be stepped up or down a long one track or can be switched between tracks' according to an individual patient's need. Before stepping up or down, the physician should check inhaler technique, patient adherence, and environmental exposures and confirm that symptoms are due to asthma. 'Alternate track: As a n alternative, SABA can be taken as needed as a reliever mediation. This can be combined with low-dose ICS as needed

(step 1 ), or low-dose ICS maintenance therapy can be started (step 2). All other add-on medications (steps 3-5) are the same as the preferred track.

History/PE ■ Presents with chronic productive cough accompanied by frequent bouts of yellow or green sputum production, dyspnea, and possible hemoptysis and halitosis ■ Associated with a history of cystic fibrosis (CF), pulmonary infections (eg, Pseudomonas, atypical mycobacteria), allergic bronchopulmonary asper­ gillosis, hypersensitivity, immunodeficiency, localized airway obstruction, aspiration, autoimmune disease, or inflammatory bowel disease (IBD) ■ PE: Reveals rales, wheezes, rhonchi, purulent mucus, and occasional hemoptysis Diagnosis ■ CXR: Shows i bronchovascular markings and tram lines (parallel lines outlining dilated bronchi as a result of peribronchial inflammation and fibrosis). ■ Most accurate test: High-resolution CT. Dilated airways (ie, larger than pulmonary arteries) and ballooned cysts are seen at the end of the bron­ chus (mostly lower lobes). ■ Spirometry/PFfs: Obstructive pattern with .J, FEVi/FVC ratio. ■ Additional tests to identify underlying etiology: Depends on clinical sus­ picion. Sputum microscopy and culture may reveal chronic infection (eg, Pseudomonas, Escherichia coli, tuberculosis) or suggest allergic broncho­ pulmonary aspergillosis (ie, presence of eosinophils or hyphae). u 1 -Antitrypsin levels can rule out deficiency. Sweat chloride and/or genetic testing for CFTR mutations may suggest CF. Rheumatoid factor (RF), antinuclear antibody (ANA), or other screening tests for autoim­ mune disease may also be considered.

F I G U R E 2 . 1 4-3. Bronchiectasis. CT of the chest demonstrates markedly dilated and thick-walled airways (arrows) con­ sistent with bronchiectasis in this cystic fibrosis ( CF) patient. (Reproduced with permis­ sion from USM LE-Rx.com.)

I A 1 0-yea r-old c h i l d with a h i story of asthma on d a ily fl uticasone has been u s i n g a n a l buterol i n h aler once a day a s needed fo r seve ra l weeks. What c h a nges sho u l d be made to the cu rre nt reg i m e n ?

630

HIGH-YIELD FACTS IN

PULMONARY Treatment ■ Medications: Antibiotics for exacerbations ( .J, bacterial load and airway/ systematic inflammatory mediators) ■ Empiric therapy: Respiratory fluoroquinolone (levofloxacin, moxifloxacin) ■ Tailoring of treatment to sputum culture results, if available ■ Allergic bronchopulmonary aspergillosis (ABPA): Systemic glucocorticoids and antifungals (voriconazole, itraconazole) Lifestyle: Bronchopulmonary hygiene (cough promotion, postural drain­ age, chest physiotherapy). Surgery: Consideration of lobectomy for localized disease or lung trans­ plantation for severe disease.

CHRONIC OBSTRUCTIVE PULMONARY DISEASE 0-rr KEY FACT I n patients with COPD a n d c h ronic hyperca pn ia, excess s u p p l ementa l oxyg en ca n decrease ve ntilatory d rive, res u lting in worsen i n g hyperca pnia a n d respi ratory acidosis.

0-rr KEY FACT Consider a 1 -a ntitrypsi n deficiency i n a patient who is < 60 yea rs of age, has a fa m i ly history of COPD, has m i n i m a l or no smoking h istory, has l iver disease, and has basi l a r-predom i na nt COPD.

0-rr KEY FACT S u pplementa l 02 a n d smoking cessation a re the only i nterventions proven to i m p rove su rviva l i n patients with COPD.

A disease with .J, lung function associated with airflow obstruction. Can be divided into two major subtypes: Chronic bronchitis: Productive cough for > 3 months per year for 2 con­ secutive years (clinical diagnosis) Emphysema: Destruction and dilation of structures distal to the terminal bronchioles (pathologic diagnosis) that may be secondary to smoking (cen­ trilobular) or to u 1 -antitrypsin deficiency (panlobular)

History/PE ■ Symptoms are minimal or nonspecific until the disease is advanced. ■ The clinical spectrum is shown in Table 2.14-4 (most patients are a combi­ nation of the two phenotypes). ■ Symptoms: Classic barrel chest, use of accessory chest muscles, jugular vein distention (JVD), end-expiratory wheezing, dyspnea on exertion, and muffled breath sounds. ABGs: Hypoxemia with acute or chronic respiratory acidosis (I Paco 2 ) . ■ Gram stain and sputum culture: Considered if bacterial infection is sus­ pected (eg, fever, productive cough, new infiltrate on CXR). Diagnosis ■ Best initial test: Spirometry (PFTs); obstructive pattern that is nonrevers­ ible with SABA. ■ FEV 1 /FVC 55%), LTOT is indicated when Spo2 ::589% or Pao, ::559 m m Hg.

■ Supplementa l 02 ca n worsen hyperca pnia. The goal oxygen satu ration is 90%-93%.

■

CXR: Hyperinflated lungs, .J, lung markings with flat diaphragms, and a thin-appearing heart and mediastinum are sometimes seen. Parenchymal bullae or subpleural blebs are also seen (see Fig. 2.14-4).

Treatment See Table 2.14-5.

F I G U R E 2.1 4-4.

COPD. (A) Posteroan­ terior (PA) and (B) lateral radiographs of a patient with emphysema show hyperinflation with large lung volumes, flattening of the diaphragm, and minimal peripheral vascular markings. (Reproduced with permission from USMLE-Rx.com.)

0-n

Treatments for acute asthma and COPD exacerbations both involve !3 2-agonists and corticosteroids. During an acute COPD exacerbation, antibiotics may also be given. During an acute asth ma exacerbation, magnesiu m can be given.

:(i MNEMONIC Treatment for COPD­ COPD

RESTRICTIVE LUNG DISEASE Characterized by a loss of lung compliance, restrictive lung diseases result in i lung stiffness and .J, lung expansion. Table 2.14-1 and Figure 2.14-2 contrast obstructive with restrictive lung disease. The etiologies of restrictive lung dis­ ease are shown in the AIN'T mnemonic.

KEY FACT

Corticosteroids Oxygen (if resting Spo2 1 with ho-spital admission

< 1 with no hospital admissions

I

I

Mild (CAT < 10)

Severe (CAT � 10)

M i ld (CAT < 10)

Severe (CAT � 10)

Group A Short-acting bronchodilator (SABA, SAMA, or combination)

Group B SABA PRN + long acting bronchodilator (LABA or LAMA)

Group e SABA PRN + LAMA

Group D SABA PRN + LAMA If severe symptoms (CAT > 20), LAMA and LABA If overlapping asthma, LABA and ICS

l

l

Initial COPD treatment based on severity assessed using COPD assessment test (CAT).

(Reproduced with permission from USM LE-Rx.com.)

(l- MNEMONIC Etiology of restrictive lung diseaseIf the lungs AIN'T compliant

Alveolar (edema, hemorrhage, pus) I nterstitia l lung disease (idiopath ic pulmonary fibrosis, usual insterstitia l pneumon ia, nonspecific interstitial pneumon ia, chronic hypersensitivity pneumonitis, sarcoidosis with interstitial pneumonia) Neurom uscu lar (myasthenia, phrenic nerve palsy, myopathy) Thoracic wall (kyphoscol iosis, obesity, ascites, pregnancy, a n kylosing spondylitis)

0-,r KEY FACT Med ications a n d i nterventions that ca n cause or contri b ute to ILD i n c l ude a m ioda rone, busulfan, n itrofu ra ntoin, bleomycin, methotrexate, rad iation, a n d long-term h i g h 02 concentration (eg, ventilators).

INTERSTITIAL LUNG DISEASE A heterogeneous group of disorders characterized by inflammation and/or fibrosis of the interstitium. In advanced disease, cystic spaces can develop in the lung periphery, a development that leads to the characteristic "honey­ comb" pattern seen on CT (see Fig. 2. l 4-6A). Interstitial lung disease (ILD) is also called diffuse parenchymal lung disease (DPLD). Subgroups of ILD: Exposure related: Asbestosis, silicosis, berylliosis, coal worker's pneumo­ coniosis, medications (eg, amiodarone, bleomycin), hypersensitivity pneu­ monitis, radiation-induced injury ■ ILD associated with systemic disease or connective tissue diseases: Polymyositis/dermatomyositis, sarcoidosis, amyloidosis, vasculitis, sclero­ derma (CREST syndrome) ■ Idiopathic: Idiopathic pulmonary fibrosis (IPF), cryptogenic orgamzmg pneumonia, acute interstitial pneumonia ■

History/PE

Presents with shallow, rapid breathing; progressive dyspnea with exertion; and a chronic nonproductive cough May have cyanosis, inspiratory squeaks, fine or "Velcro-like" crackles, clubbing, or right heart failure Diagnosis ■ ■

Best initial test: CXR; reticular, nodular, or ground-glass pattern Next best step: If CXR is suspicious for ILD, then high-resolution CT; CT shows "honeycomb" pattern in severe disease

PULMONARY

HIGH -YIELD FACTS IN

633

■ PFTs: Restrictive pattern. Normal/I FEV i /FVC, ,j, FVC, ,j, FEV 1 , ,j, TLC, t FVC, t DLCO ■ If systemic disease is suspected as the cause, the physician can consider serologic testing (eg, ANA, anti-cyclic citrullinated peptide [ anti-CCP], creatine kinase [CK], aldolase, anti-Jo l , antineutrophil cytoplasmic anti­ body [ANCA], antitopoisomerase, anti-double-stranded [ds] DNA). ■ Most accurate test: Surgical biopsy. This is not recommended if CT find­ ings are characteristic (see Fig. 2. l 4-6A). In IPF and rheumatologic dis­ ease, a surgical biopsy is only performed when the diagnosis is uncertain.

Treatment ■ Supportive: Avoidance of exposure to causative agents ■ Medications: Anti-inflammatory/immunosuppressive agents for some dis­ ease (eg, corticosteroids), antifibrotic agents (pirfenidone, nintedanib) for IPF ■ Sur gery: Referral for lung transplantation indicated at late stages of IPF

CRYPTOGENIC ORGANIZING PNEUMONIA Cryptogenic organizing pneumonia is a form of diffuse ILD. "Cryptogenic" refers to the idiopathic nature of the condition. "Organizing pneumonia" refers to the typical pathologic appearance of the condition wherein buds of granulation tissue form in distal air spaces (alveoli and bronchiolar lumen [bronchiolitis obliterans ]). These findings are not specific to a disease, but just a type of inflammatory process.

History/PE Typically presents with subacute fever, dry cough, shortness of breath, weight loss, anorexia, and malaise that have failed to respond to antibiotic therapy. Diagnosis ■ Other causes such as infection or autoimmune disease must be excluded. ■ Radiographic findings on CXR or CT include bilateral, peripheral patchy opacities that may migrate. ■ PFTs show a restrictive defect with diffusion impairment. ■ Pathologic examination of biopsy specimens is diagnostic (generally surgical biopsy is required, although transbronchial biopsy can be attempted).

F I G U R E 2 . 1 4-6. Idiopathic pulmonary fibrosis. (A) Chest CT showing the char­

acteristic "honeycomb" lung that is seen in advanced disease. (B) Lung biopsy specimen demonstrating increased inter­ stitial fibrosis and nonspecific inflam­ mation with alveolar thickening. (Image A

reprod uced with perm ission from Wa lsh SLF, Wells AU, Sverzel lati N, et al. Relationship between fibroblastic foci profu sion and hig h-resolution CT morphology in fibrotic lung disease. BMCMed. 20 1 5;1 3:241 . I mage B reprod uced with perm ission from USM LE-Rx.com.)

Treatment Treatment with corticosteroids results in dramatic clinical and radiologic response. Relapse 1s common on tapering steroids. Overall, prognosis is excellent.

SYSTEMIC SARCOIDOSIS A multisystem disease of unknown etiology characterized by infiltration of noncaseating granulomas. In the United States, more commonly found in females (although it occurs in males too) of African or Northern European descent. Most often arises in the third or fourth decade of life.

History/PE Systemic sarcoidosis can present with fever, cough, dyspnea, malaise, weight loss, or arthritis. ■ Lofgren syndrome: Erythema nodosum, bilateral hilar adenopathy, migra­ tory polyarthralgia, and fever; associated with good prognosis ■

I A 25-yea r-old Black wo man presents to the physicia n's offi ce with pai nfu l b u m ps on her s h i ns, weight loss, and co u g h . Exa m i nation revea l s a p ro m i n ent 1 -c m right axi l l a ry lymph node. What is the next best ste p for d iag nosis?

634

HIGH-YIELD FACTS IN

PULMONARY ■

Extrapulmonary manifestations can involve the following organs: liver, eyes (uveitis), skin (erythema nodosum, violaceous skin plaques), central nervous system (CNS), heart (third-degree heart block, arrhythmias), and kidneys.

Diagnosis ■

■

F I G U R E 2 . 1 4-7. Bilateral hilar lymph­ adenopathy (arrows) in a patient with pulmonary sarcoidosis. (Reproduced with permission from USMLE-Rx.com.)

■ ■

0,0 MNEMONIC Learning the features of sarcoid can be G RU ELING-

Best initial test: CXR shows bilateral hilar adenopathy and reticular opaci­ ties (upper lobe predominant). Figure 2.14-7 illustrates CXR findings of sarcoidosis. High-resolution CT is usually done following suspicious CXR. Next best step: If CXR/CT is suspicious, then bronchoscopic biopsy. Non­ caseating granulomas are diagnostic in the presence of a compatible clini­ cal picture with exclusion of other diseases. PFfs: Restrictive pattern and ,J, DLCO. Other findin gs: T serum angiotensin-converting enzyme (ACE) levels (neither sensitive nor specific), hypercalcemia, hypercalciuria, i alkaline phosphatase (with liver involvement), lymphopenia, cranial nerve defects, arrhythmias.

Treatment

Asymptomatic: Observation Symptomatic: Systemic corticosteroids indicated for deteriorating respira­ tory function, constitutional symptoms, hypercalcemia, and extrathoracic organ involvement Refractory disease: lmmunosuppressants (eg, methotrexate, azathioprine, tumor necrosis factor [TNF]-a inhibitors) Lofgren syndrome: NSAIDs and supportive therapy

Granu lomas aRthritis Uveitis Erythema nodosum Lymphadenopathy (particularly hilar, seen on CXR) I nterstitial fi brosis Negative tubercu losis (TB) test Ga mmaglobulinemia

■

0-,,. KEY FACT

Alveolar thickening and noncaseating granulomas secondary to environmen­ tal exposure (eg, farmer's lung seen in farmers and cattle workers due to chronic inhalation of mold that grows on hay and grain or pigeon breeder's disease due to chronic inhalation of particles from feathers or bird droppings)

Lofg ren syn d rome is a type of sarcoidosis with the fol lowing triad: a rth ritis, erythema nodos u m , a n d bi latera l h i l a r adenopathy. Associated with a good prog nosis.

■ ■

HYPERSENSITIVITY PNEUMONITIS

History/PE

■

Acute: Dyspnea, fever, malaise, shivering, and cough starting 4 to 6 hours after exposure; the physician should gather a job/travel history to deter­ mme exposure Chronic: Presents with progressive dyspnea; physical examination reveals fine bilateral rales

Diagnosis

Appearance on CXR/CT is variable, but upper lobe fibrosis is a common fea­ ture of chronic disease. Treatment

The patient should avoid ongoing exposure to inciting agents; the physician should prescribe corticosteroids to ,J, chronic inflammation. Th is is presumed sa rcoidosis. B i opsy of the rig ht axi l l a ry lym p h n ode i s the next best ste p for d i a g n osis and is less i nva sive than tra n s b ro n c h i a l l u n g biopsy.

PNEUMOCONIOSIS Pneumoconiosis refers to lung conditions caused by the inhalation of organic or nonorganic airborne dust and fibers. Risk factors include prolonged occu­ pational exposure and inhalation of small inorganic dust particles.

PULMONARY TA B L E 2 . 1 4-6.

HIGH -YIELD FACTS IN

635

Diagnoses of Pneumoconioses

DISORDER

H I STORY

IMAGING F I N DINGS'

COMPLICATIONS

Asbestosis

Work involving the manufacture of tile

Linear opacities at lung bases and interstitial fibrosis; calcified pleural plaques

i risk for mesothelioma (rare) and

or brake linings, insu lation, construc­ tion, demolition, or shipbuilding Presents 1 5-20 years after initial exposu re

Coal workers' disease

Work in underground coal mines

Silicosis

Work in mines or quarries or with glass,

Small nodular opacities ( < 1 cm) in upper

lung cancer; the risk for lung cancer is higher in smokers; the most common malignancy associ­ ated with asbestos exposure is bronchogenic carcinoma Progressive massive fibrosis

lung zones

pottery, or silica Beryll iosis

(I mage A) indicative of benign pleural disease; I mage B shows ferruginous bodies in alveolar septum

Work in high-technology fields such as aerospace, n uclear, and electronics plants; ceramics industries; foundries; plating facilities; dental material sites;

Small ( < 1 cm) nodular opacities in upper lung zones; eggshell calcifications Diffuse infiltrates; hilar adenopathy

i risk for TB; need annual TB test Progressive massive fibrosis Requires chronic corticosteroid treatment

or dye manufacturing

•Spirometry, consistent with restrictive disease. I mage A reproduced with permission from Miles SE, Sandrini A, Johnson AR, et al. Clinical consequences of asbestos-related diffuse pleural thickening: a review. J Occup Med Toxicol 2008;3:20. I mage B reproduced with permission from Mizell KN, Morris CG, Carter JE. Antemortem diagnosis of asbestosis by screening chest radiograph correlated with postmortem histologic featu res of asbestosis: a study of 273 cases. J Occup Med Toxicol. 2009 Jun 1 2;4:1 4. doi: 1 0.1 1 86/1 745-6673-4-1 4.

History/PE/Diagnosis Table 2.14-6 outlines the findings and diagnostic criteria associated with com­ mon pneumocomoses. Treatment Avoidance of triggers; supportive therapy and supplemental 0 2 .

EOSINOPHILIC PULMONARY SYNDROMES A diverse group of disorders characterized by eosinophilic pulmonary infil­ trates and abnormal peripheral blood eosinophilia. Includes ABPA, Loffler syndrome, acute and chronic eosinophilic pneumonia, eosinophilic granulo­ matosis polyangitis, and drug-induced disorders (eg, NSAIDs, nitrofurantoin, sulfonamides).

636

HIGH-YIELD FACTS IN

0-,r KEY FACT

Loffier syndrome is a form of eosinophilic pulmonary disease characterized by absent or mild respiratory symptoms (most often dry cough), fleeting migratory pulmonary opacities, and peripheral blood eosinophilia.

PULMONARY H i story/PE

Presents with dyspnea, cough, potentially blood-tinged sputum, and/or fever. Diagnosis ■ ■ ■

Complete blood cell count (CBC): May reveal peripheral eosinophilia (2:: 500 eosinophils/uL) CXR: Shows pulmonary infiltrates Bronchoalveolar lavage: i eosinophils (> 2 5 %)

Treatment

Removal of the extrinsic cause or treatment of underlying infection (eg, hel­ minths) if identified. Corticosteroid treatment may be used.

ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS ABPA is a hypersensitivity reaction of airways to colonization by Aspergillus. As a result of recurrent inflammation, obstruction, and mucous secretions, bronchiectasis and fibrosis develop. Early treatment may prevent progression to bronchiectasis or pulmonary fibrosis. H i story/PE

Patients often have underlying asthma or CF, and they present with recurrent fever, bronchial obstruction, brownish expectoration, and hemoptysis. Diagnosis ■

■ ■

Initial tests look for evidence of Aspergillus sensitization (eg, Aspergillus­ specific lgE antibodies or positive Aspergillus skin prick test). Negative results rule out aspergillosis. Patients sensitized to Aspergillus should undergo further laboratory testing for total serum lgE, Aspergillus precipitins, and eosinophil counts (gener­ ally > 500 cells/uL). Sputum may have eosinophil-rich plugs and Charcot-Leyden crystals. Culture of sputum may grow Aspergillus. Imaging with CT of chest or a CXR may show evidence of bronchiectasis.

Treatment ■ ■ ■

Systemic glucocorticoids (prednisone) and antifungal therapy (itracon­ azole or voriconazole) may be used. Optimization of asthma treatment with possible addition of biologic agents (eg, omalizumab). Patients with CF may also benefit from omalizumab. Patients should reduce Aspergillus exposure at home or work.

Complications

Complications include acute/chronic invasive pulmonary aspergillosis and aspergilloma.

HIGH -YIELD FACTS IN

PULMONARY

637

ACUTE RESPIRATORY FAILURE HYPOXEMIA Hypoxemia is a below normal arterial oxygen level, normally defined as PaO2 20 mm Hg (normal: 8-20 mm Hg) Treatment ■ Treat underlying disease. ■ Supportive therapy: Supplemental 0 2 , diuretics; some patients may also benefit from exercise therapy ■ Consider digoxin and anticoagulation in patients with underlying left ven­ tricle systolic dysfunction and atrial fibrillation ■ Group 1 (primary PAH): Prostanoids (eg, beraprost, epoprostenol, ilo­ prost, treprostinil), endothelin receptor antagonists (eg, ambrisentan, bosentan, and macitentan), and phosphodiesterase (PDE) inhibitors (eg, sildenafil, tadalafil, vardenafil) can be added to improve hemodynamics and increase exercise tolerance. Some patients have vasoreactivity and respond well to calcium channel blockers ■ Group 4 (thromboembolic disease): Surgical thromboendarterectomy; anticoagulation is recommended for everyone; balloon pulmonary angio­ plasty and riociguat are the alternatives for chronic thromboembolic pul­ monary hypertension (CTEPH)

0-n

KEY FACT

Other etiologies of embolic disease include postpartum status (amniotic fluid emboli), fracture (fat emboli), cardiac surgery (air emboli), and endovascular procedure (cholesterol emboli).

644

HIGH-YIELD FACTS IN

PULMONARY TAB L E 2 . 1 4- 1 1 .

0.◊ MNEMONIC

Virchow Triad for Venous Thrombosis

VENOUS STASIS

ENDOTHELIAL INJURY

HYPERCOAGU LABI LITY

I m mobility CHF

Trauma Surgery

Pregnancy, postpartum Cigarette smoking

Obesity i central venous pressure

Recent fracture Previous DVT

Oral contraceptive pill (OCP) use Coagulation disorders (eg, protein C/ protein S deficiency, factor V Leiden) Malignancy

(eg, renal failure)

Wells criteria-Consider when the history and physical exam are suggestive of DVT to assess the risk of pulmonary embolism SHIT PMH Symptoms of DVT: 3 points History of DVT or PE: 1 .5 points Immobilization (2:3 days): 1 .5 points Tachycardia (HR > 1 00/min): 1 .5 points Postop (surgery within previous 4 weeks): 1 .5 points Malignancy: 1 point Hemoptysis: 1 point

Total point value = 1 1

(t MNEMONIC V/Rchow triad-risk factors for venous thrombosis: Vascular trauma Increased coagulability Reduced blood flow (stasis)

Severe burns

PULMONARY THROMBOEMBOLISM An occlusion of the pulmonary vasculature by a blood clot. Ninety-five per­ cent of emboli originate from deep venous thrombosis (DVT) in the deep leg veins (eg, femoral vein). May lead to pulmonary infarction, right heart failure, and hypoxemia. History/PE

Factors predisposing to thromboembolism are summarized by the Virchow triad (see Table 2.14-11). Presents with sudden-onset or subacute dyspnea, pleuritic chest pain, low­ grade fever, cough, tachypnea, tachycardia, and rarely, hemoptysis (indi­ cates pulmonary infarction). ■ May have history of immobility (eg, long plane ride, bedbound) ■ Examination that may reveal a loud P2 and prominent jugular A waves with right heart failure. Acute massive pulmonary embolism: Presents with hypotension, JVD, and new-onset right bundle branch block. Diagnosis ■ ■

■

■ FIGURE 2.14-12.

Pulmonary embolus.

Axial slice from a CT pulmonary angiogram shows a filling defect of the angiogram dye that corresponds to a pul­ monary embolus (red arrow) extending from the main pulmonary artery into the right and left pulmonary arteries, con­ sistent with a saddle embolus. (Reproduced with permission from Chen MY et al. Basic Radiology, 2nd ed. New York, NY: McGraw-Hill; 201 1 .)

■ ■

Best initial step: Calculation of modified Wells score (Table 2.14-12) Pulmonary embolism unlikely (modified Wells score :::54): ■ Best initial test: o-dimer used to rule out pulmonary embolism; high negative predictive value and sensitivity; not specific. If i o-dimer ( 2:: 5 00 ng/mL) ➔ CT of chest with contrast (or V/Q scan if unable to obtain CT with contrast). If normal o-dimer ➔ pulmonary embolism excluded. Pulmonary embolism likely (modified Wells score >4) : ■ Best initial test: CT of chest with contrast high sensitivity and specific­ ity (see Fig. 2.14-12) Ventilation/perfusion (V/Q) scan: Used when CT scan is contraindicated (I creatinine [ (Cr) relative contraindication to contrast], pregnancy [ con­ traindication to radiation]). May reveal areas of V/Q mismatch to predict low, indeterminate, or high probability of pulmonary embolism. A V/Q scan is sensitive for pulmonary embolism but not specific, especially if there is underlying lung disease. ABGs: Respiratory alkalosis caused by hyperventilation ( -l, Pao 2 [ 1 00/min)

1 .5

I m mobilization (2::3 days) or surgery in last month

1 .5

Previous pulmonary embolism/DVT

1 .5

Hemoptysis Malignancy

TRADITIONAL CLI N I CAL PROBABILITY ASSESSM ENT High

>6

Moderate

2-6

Low

4

Pulmonary embolism u n l i kely

:54

ECG: Most commonly reveals sinus tachycardia. The classic triad of S l Q3T3 is rare (acute right heart strain with an S wave in lead I, a Q wave in lead III, and an inverted T wave in lead III). ■ Lower extremity venous ultrasound: Specific and sensitive for DVT, which may be the cause of the pulmonary embolism. ■

Treatment ■ Anticoagulation: See Figure 2.14-13. ■ Acute: Unfractionated heparin, subcutaneous low-molecular-weight hepa­ rin (LMWH), subcutaneous fondaparinux, or direct oral anticoagulants (rivaroxaban, apixaban). In patients with high probability of pulmonary embolism, anticoagulation should be given before confirmatory testing. Patients with renal failure require unfractionated heparin or apixaban. ■ Chronic: LMWH, direct oral anticoagulants (preferred), or warfarin (goal for international normalized ratio [INR] = 2-3). Use LMWH in preg­ nancy (warfarin is contraindicated). ■ Inferior vena cava (IVC) filter: Indicated in patients with a documented lower extremity DVT/pulmonary embolism if anticoagulation is contrain­ dicated or if patients experience recurrent emboli while on therapeutic doses of anticoagulation. ■ Thrombolysis: Indicated in cases of massive pulmonary embolism causing right heart failure and hemodynamic instability (saddle pulmonary embolism). ■ DVT prophylaxis: Treatment for all immobile patients. The physician should prescribe subcutaneous heparin or low-dose LMWH, early ambu­ lation (most effective), and intermittent compression of the lower extremi­ ties (less effective).

0-n

KEY FACT

Dyspnea, tachyca rd ia, and a normal CXR i n a hospita l ized and/or bed ridden patient should ra ise sus picion of PE.

646

HIGH-YIELD FACTS IN

PULMONARY Anticoa g u lation i n suspected PE

Contra i n d ication to anticoagulation?• Yes

Diagn ostic evaluation

1

PE confirmed

!

IVC fi lte r

0-,,. KEY FACT

Lung nodule clues based on history: ■ Recent immigrant-think TB ■ From the Southwestern United States-think coccidioidomycosis ■ From the Ohio River Valley-think histoplasmosis or blastomycosis

l

PE excluded

!

No f u rth e r eva luation

No

l

Clinical suspicion of PE b

1

l

Moderate/low

High

!

Sta rt emperic a nticoa g u lation

!

!

D i a g n ostic c evaluation

l

PE confirmed

PE excluded

Sta rt/continue anticoagu lation

N o anticoag u lation (d isconti nue if started empirica lly)

F I G U R E 2 . 1 4- 1 3 . Guide to anticoagulation in hemodynamically stable patients with a sus­ pected pulmonary embolism (PE). "Contraindications to anticoagulation include recent

surgery, hemorrhagic stroke, active bleeding (eg, GI bleed), and aortic dissection. hCJinical suspicion of pulmonary embolism is determined using the modified Wells criteria: High >6, moderate 2 to 6, low 2 years), no further evaluation is required. ■ If the nodule is new, increasing in size, or no prior CT scans are available, then determine risk for malignancy.

PULMONARY TAB L E 2 . 1 4- 1 3.

VARIABLE

HIGH -YIELD FACTS IN

647

Risk for Malignancy in Patients With Solitary Pulmonary Nodules

LOW

INTERMEDIATE

H IGH

Diameter o f n o d u l e (mm)

15

5-1 5

90%.

pyogenes is the most common bacterial cause of pharyngitis. (Reproduced cou rtesy of Dr. Heinz F. Eichenwa ld from the Centers for Disease Control and Prevention, Atlanta, GA.)

Treatment

If GAS is suspected, begin empiric antibiotic therapy with penicillin for l 0 days. Cephalosporins, amoxicillin, and azithromycin are alternative options. Symptom relief can be attained with fluids, rest, antipyretics, and saltwater gargles. Com pl ications

■ Nonsuppurative: Acute glomerulonephritis

TA B L E 2 . 1 4- 1 9.

F I G U R E 2 . 1 4-28. Pharyngeal erythema with palatal petechiae. Streptococcus

rheumatic

Modified Centor Criteria (Centor Criteria

CRITERIA

fever,

poststreptococcal

+ Age) POI NTS

Fever Tonsillar exudate Tender anterior cervical lymphadenopathy Lack of cough 3- 1 4 years of age

1

1 5-45 years of age >45 years of age

0 -1

If 4-5 points, treat empirically with a ntibiotics.

If 2-3 points, perform rapid antigen test. If E9 antigen test, treat with antibiotics; if 0 antigen

test, perform throat culture.

If 0-1 point, no testing or antibiotics are required (symptomatic treatment only).

662

HIGH-YIELD FACTS IN

0-,,. KEY FACT Ac ute necrotizi ng mediasti n itis is a l ife­ t h reate n i n g com pl ication of u ntreated retropharyngeal a bscess that presents with fever, chest pa i n , and dys pnea. It req u i res u rgent s u rgical d ra i nage to prevent spread to the posterior mediasti n u m, which may ca use letha l p l e u ra l and pericard i a ! effusions.

PULMONARY Suppurative: Cervical lymphadenitis, mastoiditis, sinusitis, otitis media, retropharyngeal or peritonsillar abscess, and, rarely, thrombophlebitis of the jugular vein (Lemierre syndrome) caused by Fusobacterium, an oral anaerobe ■ Peritonsillar abscess may present with odynophagia, trismus ("lockjaw"), a muffled "hot potato" voice, unilateral tonsillar enlargement, and ery­ thema, with the uvula and soft palate deviated away from the affected side; culture abscess fluid and localize the abscess via intraoral ultrasound or CT; treat with antibiotics and surgical drainage ■

0-,,. KEY FACT A l l patients with a h i story of rhe u m atic fever should be g iven routi ne penici l l i n prophylaxis t o prevent recu rre nt g ro u p A Streptococcus infection.

ORAL INFECTIONS LUDWIG ANGINA Rapidly progressive cellulitis of the submandibular space that may cause air­ way compromise from rapidly expanding edema. Usually caused by polymi­ crobial infection in the setting of poor oral hygiene. IV broad-spectrum antibiotics and diligent airway management are necessary; surgical drainage is performed if there is abscess formation (uncommon).

ACUTE LYMPHADENITIS Unilateral and rapid onset ( < 1 week), commonly caused by S aureus and S pyogenes, typically involving the submandibular lymph nodes. Antibiotics are required if symptoms (fluctuance, fever, cellulitis) are present to prevent abscess formation.

0-,,. KEY FACT Ludwig a n g i n a is a bilatera l cel l u l itis of the s u bmental, su bmaxi l l a ry, a n d s u b l i ng u a l s paces t h a t u s u a l ly resu lts from an infected tooth. It presents with dysphag ia; d roo l i ng; fever; a n d a red, wa rm mouth, and it can lead to death from asphyxiation.

0-,,. KEY FACT Potential com pl ications of sin usitis incl ude m e n i n g itis, fronta l bone osteomyel itis, cavernous s i n u s t h rom bosis, a n d a bscess formation.

0-,,. KEY FACT Bewa re of i nvasive a n d life-th reaten i n g fu ngal s i n usitis (ca used b y Mucor a n d Rhizopus) in patients with poorly control led d i a betes m e l l itus, i m m u n e com p rom ise, or neutropenia.

SINUSITIS Refers to inflammation of the paranasal sinuses. The maxillary sinuses are most commonly affected. Subtypes include the following: Acute sinusitis (symptoms lasting < l month) : Most commonly associ­ ated with viruses, S pneumoniae, H infl.uenzae, and M catarrhalis. Bacte­ rial causes are rare and characterized by purulent nasal discharge, facial or tooth tenderness, hyposmia/anosmia, and symptoms lasting > 10 days. ■ Chronic sinusitis (symptoms persisting > 3 months) : A chronic inflam­ matory process often caused by obstruction of sinus drainage and ongoing low-grade anaerobic infections. ■

H i story/PE ■

Presents with fever, facial pain/pressure, headache, nasal congestion, and discharge. Examination may reveal tenderness, erythema, and swelling over the affected area. High fever, leukocytosis, and a purulent nasal discharge are suggestive of acute bacterial sinusitis.

Diagnosis ■

A clinical diagnosis. Culture and imaging are generally not required for acute sinusitis but may guide the management of chronic cases.

PULMONARY

HIGH -YIELD FACTS IN

663

■ Transillumination shows opacification of the sinuses (low sensitivity). ■ CT is the test of choice for sinus imaging (see Fig. 2.14-29) but is usually necessary only if symptoms persist after treatment.

Treatment Most cases of acute sinusitis are viral and/or self-limited and are treated with symptomatic therapy (decongestants, antihistamines, nasal saline lavage, pain relief). ■

■

Acute bacterial sinusitis: The physician should consider either amoxicil­ lin/clavulanate for 10 days or clarithromycin, azithromycin, trimethoprim­ sulfamethoxazole (TMP-SMX), a fluoroquinolone, or a second-generation cephalosporin for 10 days. Chronic sinusitis: ■ Antibiotics like those used for acute disease may be prescribed for chronic sinusitis, although a longer course (3-6 weeks) may be necessary. ■ Adjuvant therapy with intranasal corticosteroids, decongestants, and/or antihistamines may be useful in combating the allergic/inflammatory component of the disease. ■ Surgical intervention may be required.

H EMOPTYSIS Hemoptysis is the expectoration of blood from the lower respiratory tract, below the vocal cords, which can be caused by various etiologies. These etiologies can be divided into life-threatening and non-life-threatening hemoptysis. Bleeding from the pulmonary arteries (low-pressure system) is commonly non-life­ threatening, whereas bleeding from the bronchial arteries (high-pressure sys­ tem) may be life-threatening. Hemoptysis is often classified as mild ( 100 ml/hr or > 150 m l/24 hr) Respiratory distress Hemodynamic instability

No

La bs, CXR, CT c hest ± contrast Bronchoscopy if normal imaging or if no other etiologies id entified

l Other etiologies identified

Eva luate and treat findings

F I G U R E 2 . 1 4-30

Yes

Source of bleeding identified

Secure a i rway, b reath i n g a n d circulation

Treat with bronchoscopy, I R embolization, or resection

*Most common causes of massive hemoptysis: bronchiectasis, active pulmonary TB, malignancy

Evaluation of hemoptysis. (Reproduced with permission from USMLE-Rx.com.)

Treatment ■

■

In the case of impending airway compromise due to massive hemoptysis, secure the airway by placing an endotracheal tube while evaluating the cause. Management of hemoptysis includes hemodynamic stabilization and treatment of the underlying cause. In the case of persistent bleeding, bron­ choscopic interventions and embolization can be performed to control the bleeding (Fig. 2.14-30).

PLEURAL DISEASE PLEURAL EF FUSION An abnormal accumulation of fluid in the pleural space. Classified as follows:

i pulmonary capillary wedge pressure (PCWP) or -l, oncotic pressure ■ Exudate: Secondary to i pleural vascular permeability See Figure 2.14-31 for an algorithm showing etiology of pleural effusion. Table 2.14-20 lists the possible causes of both transudates and exudates. ■

Transudate: Secondary to

H i story/PE

Presents with dyspnea, pleuritic chest pain, and/or cough. Exam reveals dull­ ness to percussion and 1- breath sounds over the effusion (see Table 2.14-21). A pleural friction rub may be present. Diagnosis ■

Best initial test: CXR, blunting of the costophrenic angle. Lateral decubi­ tus view is most sensitive; it also is used to assess for loculation.

PULMONARY

HIGH -YIELD FACTS IN

Diagnostic thoracocentesis

Meets no criteria

Transudative

t hydrostatic pressure CHF

ESRD Pulmonary embolism (early)

Light criteria

Meets :;, 1 criteria

Pleural protein + serum protein >0.5 Pleural LDH + serum LDH >0.6 Pleural LDH > 2/3 ULN serum LDH

,J, oncotic pressure Cirrhosis Nephrotic syndrome

Exudative

lntrathoracic

Infection Neoplasm Autoimmune (eg, RA. SLE) Drugs Pulmonary embolism Chylothorax Hemothorax

I

Extrathoracic Right-sided

Meigs syndrome Endometriosis

Left-sided

Pancreatitis Esophageal rupture

Treat underlying cause Further workup (eg, pleural fluid analysis, culture, AFB, TB antigen, RF, CCP, ANA) Etiology of pleural effusion based on pleural fluid analysis. (Reproduced with perm ission from USM LE-Rx.

F I G U R E 2 . 1 4-3 1 .

com.)

TA B L E 2 . 1 4-20.

Causes of Pleural Effusions

TRANSU DATES

EXU DATES

Congestive heart fa i l u re

Ci rrhosis (hepatic hyd rothorax) Nephrotic syndrome

Pneumonia (parapneumonic effusion)

TB

Malignancy

Pulmonary embolism

Collagen vascular disease (SLE) Pa ncreatitis

Trauma

Chylothorax (triglycerides)

TA B L E 2 . 1 4-2 1 .

Pulmonary Physical Exam Findings LUNG CONSOLI DATION (eg, LO BAR PN E U M ON IA)

PLEURAL EFFUSION

P N E U M OTHORAX

Percussion

Dull

Dull

Hyperresonant

Tactile fremitus

t

j,

j,

Bronchial

j,

t/Absent

Bronchophony

j,

j,

Present (often)

Absent

Absent

Breath sounds Voice transmission

Crackles

Egophony

665

666

HIGH-YIELD FACTS IN

TAB L E 2 . 1 4-22. Effusions•

Light Criteria for Pleural

MEASURE

VA LUE

Pleural protein/

>0.5

PULMONARY

■

serum protein Pleural LDH/

>0.6

serum LOH Pleural fluid LOH

More than 2/3 of the U LN serum LOH

Complications:

■ Parapneumonic effusion and empyema: Pleural effusions that arise as result of pneumonia, lung abscess, or bronchiectasis; see Table 2.14-23 ■ Recurrent effusion

•An effusion is an exudate if any of the previous criteria are met.

0-n

KEY FACT

Complicated parapneumonic effusions necessitate chest tube drainage.

Next step: Thoracentesis. It is indicated for new effusions > 1 cm in the decubitus view, except with bilateral effusions and other clinical evidence of CHF. Using Light criteria can determine if the effusion is transudative or exuda­ tive (see Table 2.14-22). ■ Transudative effusions: Typically have a pH of 7.4 to 7. 5 5 ■ Exudative effusions: Typically have a pH of pH 7.2

pH < 7.2

pH < 7.2

Glucose: Normal/.!­ LOH ratio >0.6

Glucose: .!, LOH ratio >0.6

Glucose: .!, LOH ratio >0.6

Negative

Negative

Positive

Antibiotics

Antibiotics Chest tube

Antibiotics Chest tube

and culture Treatment

Treatment ■ Tension pneumothorax: Requires immediate needle decompression (sec­ ond intercostal space at the midclavicular line) followed by chest tube placement. ■ Small pneumothorax ( :::s 2 cm) : Observation ± supplemental 0 2 . It may resorb spontaneously. ■ Large (> 3 cm), symptomatic pneumothorax: Needle aspiration or small­ bore chest tube placement. ■ Patients who are unstable or who have recurrent pneumothorax: Chest tube placement.

PULMONARY SLEEP DISORDERS OBSTRUCTIVE SLEEP APNEA Obstructive sleep apnea (OSA) is a sleep disorder characterized by transient obstruction of the upper airway that causes hypoxemia. Etiology may be cen­ tral (eg, stroke), secondary (eg, obesity), or mixed. Risk factors include male sex, older age, obesity, craniofacial abnormalities, upper airway abnormalities (adenotonsillar hypertrophy [children]), sedative use (eg, alcohol, benzodiaze­ pines), smoking, and many others.

History/PE Cardinal features: ■

Irregular respiratory pattern during sleep: Obstructive apneas, hypop­ neas, or respiratory effort-related arousals (RERAs)

F I G U R E 2 . 1 4-32.

Pneumothorax.

(A)

Pneumothorax. CT shows collapsed left lung. (B) Tension pneumothorax. (Image A reproduced with perm ission from M i u ra K, Ko ndo

R, Kurai M, et al. Birt-Hogg-Dube syndrome detected i ncidenta l l y by asymptomatic bi latera l pneu motho­ rax in health screen i n g : a case of a yo ung Japa nese

woman. Surg Case Rep. 201 5 ; 1 : 1 7. doi:1 0.1 1 86/s40792-

0 1 5-00 1 4-8.lmage B reproduced with permission from Rosal A, Diaz C. Reexpansion pulmonary edema after

drainage of tension pneu mothorax. Pan Afr Med J.

201 5;22: 1 43. doi: 1 0. 1 1 604/pamj.20 1 5 .22.1 43.8097.

668

0--n

HIGH-YIELD FACTS IN KEY FACT

The STOP-BANG survey is a clinical questionnaire that can be used to assess the risk of OSA and direct further sleep testing: Snoring, Tiredness, Observed stop in breathing, increased blood Pressure, Body mass index (BMI) > 35 kg/m 2, Age >50 years, Neck circu mference >40 cm, male Gender. The presence of ;;;,:3 positive items should prompt sleep testing.

PULMONARY Daytime symptoms related to poor sleep: Somnolence, fatigue, poor concentration, morning headaches ■ Signs of disturbed sleep: Snoring, gasping, choking, restlessness ■

Complications:

i cardiovascular morbidity (systemic hypertension, PAH, coronary artery disease, arrhythmias, heart failure, polycythemia, and stroke) ■ i risk for insulin resistance and type 2 diabetes mellitus ■ i risk for motor vehicle collisions caused by impaired alertness ■

Diagnosis

Best initial test: Polysomnography (sleep study) based on apnea-hypopnea index ([AHi] = apneas + hypopneas/total hours of sleep) and presence or absence of related symptoms ■ Diagnosis is confirmed with the following: ■ AHi 2: 5 PLUS symptoms ■ AHi 2: 15 regardless of symptoms ■

Treatment ■ ■

Best initial therapy: Weight loss (if applicable) and CPAP Alternatives: Oral appliances, hypoglossal nerve stimulation, and maxillo­ mandibular advancement (bones of upper and lower jaw surgically reposi­ tioned to relieve obstruction) Last resort: Tracheostomy

OBESITY HYPOVENTILATION SYNDROME Obesity hypoventilation syndrome (OHS) is a sleep disorder defined as awake alveolar hypoventilation in an obese individual that cannot be attributed to other conditions associated with alveolar hypoventilation. H istory/PE

Presents with hypersomnolence and obesity. OHS is further characterized by coexisting sleep disturbances: ■

OHS with OSA (90% of patients): Presents with symptoms of OSA (see earlier section) ■ OHS with sleep-related hypoventilation ( 1 0%): Presents the same as OHS + OSA but witnessed apneas during sleep are uncommon Diagnosis

Diagnosis of exclusion. Patient must meet all of the following criteria: ■ ■ ■

Obesity (body mass index [BMI] > 30 kg/m2 ) Awake alveolar hypoventilation (Paco 2 >45 mm Hg) Exclusion of alternative causes of hypercapnia and hypoventilation

Treatment ■

Best initial treatment: Weight loss and noninvasive positive airway pres­ sure (PAP) ■ OHS + OSA: CPAP ■ Initiate bilevel positive airway pressure (BiPAP) if initial management with CPAP fails ■ OHS + hypoventilation: BiPAP

PULMONARY ■ Next best treatment: Bariatric surgery ■ Tracheostomy (last resort)

NOSE A N D TH ROAT RHINITIS Rhinitis is characterized by symptoms of rhinorrhea (posterior or anterior), nasal congestion, sneezing, and itching. Although most forms of rhinitis involve inflammation, some forms, such as vasomotor rhinitis, do not. It may be further classified into allergic and nonallergic causes.

Allergic Rhinitis Pathogenesis ■ Results from IgE-mediated type 1 hypersensitivity reaction of the nasal mucosa ■ Commonly associated with atopic diseases such as asthma and eczema History/PE Presents with rhinitis in response to allergens. Based on temporal pattern, can be further classified as follows: Intermittent/seasonal: Allergic reactions to grass/trees or pollen (hay fever); occurs in late spring/summer ■ Persistent/perennial: Allergic reactions to house dust, dust mites, molds, dogs, cats ■ Food allergens: May also be contributory; however, evidence is lacking ■

Diagnosis ■ Clinical diagnosis based on typical history and nasal examination ■ Skin prick test involves introducing common allergens into the skin to observe for hypersensitivity reactions; this can help identify allergens ■ Serum total IgE is generally increased ■ Serum radio-allergosorbent test (RAST) is a blood test that identifies IgE antibodies to specific allergens Treatment ■ Allergen avoidance ■ Medications: ■ Second-generation oral nonsedating antihistamines such as loratadine, cetirizine, and fexofenadine ■ Intranasal steroids such as fluticasone, beclomethasone, or mometa­ sone nasal sprays titrated to minimum effective dose; sometimes these internal steroids may be combined with intranasal antihistamines ■ Leukotriene antagonists such as montelukast; this is also beneficial with concomitant asthma ■ lmmunotherapy: Allergen exposure and desensitization

Nonallergic Rhinitis Nonallergic rhinitis is a subtype of rhinitis without an allergic or infectious cause. It accounts for up to 50% of cases of rhinitis in adults.

HIGH -YIELD FACTS IN

669

670

HIGH-YIELD FACTS IN

PULMONARY Etiology/Pathogenesis ■ Irritants: Cigarette smoke (tobacco), pollutants, occupational (chemicals such as cleaning products) Vasomotor: Caused by increased blood flow to the nasal mucosa; it is instigated by temperature changes or d ry air and irritant odors Gustatory: Clear rhinorrhea after ingestion of food (most often spicy) ■ Drug induced: Due to antihypertensives, NSAIDs, PDE-5 inhibitors, or cocame ■ Hormonal rhinitis: Onset during pregnancy; it resolves with end of pregnancy ■ Senile rhinitis (also called atrophic rhinitis): occurs in older adults when the nasal glands that produce moisture fail to function adequately Diagnosis Workup to exclude allergic rhinitis (see earlier information). Treatment ■ Treatment is symptom driven. ■ The patient should avoid any precipitating factors. ■ Intranasal corticosteroids (eg, fluticasone) and intranasal antihistamines (eg, azelastine) alone or in combination treat nasal congestion, postnasal drip, rhinorrhea, and sneezing. Intranasal anticholinergics (eg, ipratropium) treat rhinorrhea. Deconges­ tants (eg, phenylephrine, oxymetazoline) help with nasal congestion. Nasal irrigation and intranasal capsaicin may help.

NASAL POLYPS Nasal polyps are benign outgrowths of nasal mucosa, and they represent the most common tumors of the nasal cavity. They commonly occur in associa­ tion with allergic rhinitis, acute and chronic infections, and CF. History/PE

Patients present with nasal obstruction, postnasal discharge, congestion, sneezing, rhinorrhea, hyposmia, and anosmia. ■ Important associations: ■ Aspirin allergy ■ Sinus infections ■ Asthma ■

Diagnosis ■ ■ ■

Coronal sinus CT scanning is first-line imaging modality. Endoscopy can sometimes be helpful for evaluation in the clinic. Nasal masses that do not appear typical or respond to treatment should be biopsied.

Treatment

Medical treatment: ■ Oral corticosteroids are the most effective. ■ Intranasal corticosteroids (mometasone, beclomethasone) are less effective. Other medical options include leukotriene antagonists (mon­ telukast) or IL inhibitors (dupilumab). ■ Surgical removal is indicated in select cases due to severe symptoms of obstruction or infection refractory to medical treatment. ■ The physician should concomitantly treat predisposing factors (eg, under­ lying allergy). ■

PULMONARY

HIGH -YIELD FACTS IN If conservative treatment ineffective, perform rhinoscopy to localize source to anterior vs posterior

EPISTAXIS Epistaxis (bleeding from the nose) may either be anterior or posterior, based on the location of bleeding. ■ Anterior epistaxis: This is the most common (90%) and tends to be self­ limited. Bleeding is most often from the Kiesselbach plexus (eg, the area of anastomosis of the septa! branch of the anterior ethmoidal artery, the lateral nasal branch of the sphenopalatine artery, and the septa! branch of the superior labial branch of the facial artery). ■ Posterior epistaxis: This is less common (10%) and may result in signifi­ cant hemorrhage. Bleeding occurs from the posterolateral branches of the sphenopalatine artery and, rarely, the carotid artery.

Etiology Local causes of epistaxis include mucosa! irritation (eg, nose picking, dry air, rhinitis, foreign body), facial trauma, intranasal drugs (cocaine, intranasal cor­ ticosteroids), or tumors (nasopharyngeal carcinomas). Systemic conditions or drugs may also cause epistaxis (eg, anticoagulation, antiplatelet medications, alcohol, bleeding disorders [ eg, von Willebrand dis­ ease], vascular malformations [ nasal hemangioma], or hypertension).

67 1

Anterior bleeding

Chemical or electrical nasal cautery Anterior packing (tampon, balloon, guaze) Perform bilateral nasal packing

!f i neffective !

Posterior bleeding

Posterior packing (balloon, Foley catheter) Consider admission and ENT consultation

Consider and treat as posterior source If ineffective !

F I G U R E 2 . 1 4-33. Approach to treatment of active nosebleed. (Reproduced with permis­ sion from USM LE-Rx.com.)

Treatment Figure 2.14-3 3 outlines the approach to the management of epistaxis. ■

Initial assessment and resuscitation: Assess and treat for airway, breathing, and cardiovascular (fluid resusci­ tation, redundant large-bore IV lines as indicated) compromise. ■ Target history to rule out conditions that predispose to bleeding ( detailed earlier). ■ Laboratory tests: Coagulation studies (for anticoagulated patients, CBC, type and cross). ■ Initial conservative treatment: Position patient: Elevate body and bend forward. ■ Administer topical vasoconstrictor (eg, oxymetazoline) and local anes­ thetic (lidocaine) and pinch nostrils for 10 to 1 5 minutes. ■ Apply cold compress. ■ If conservative measures fail, examine nose to look for sources of bleed­ ing (rhinoscopy, speculum). ■ Subsequent treatment of anterior bleeding: ■ Cauterization: This is considered first line; either chemical (silver nitrate) or electrical cautery is possible. ■ Nasal packing: Nasal tampons, ribbon gauze, or nasal balloon cathe­ ters can be used; if unilateral packing ineffective, bilateral nasal pack­ ing can be performed. ■ Subsequent treatment of posterior bleeding: ■ Balloon catheter is preferred; alternatively, a Foley catheter can be used. ■ These patients may require hospitalization and urgent ear, nose, and throat (ENT) consultation. ■

Compl ications Prolonged retention of nasal packing ( > 72 hours) increases the risk of com­ plications, including necrosis, toxic shock syndrome, sinus or nasolacrimal infections, and dislodgment.

0-rr

KEY FACT

0-rr

KEY FACT

Brisk bleeding even after adequate nasal packing may indicate a posterior source of bleeding.

Consider toxic shock syndrome in a patient with fever, hypotension, desquamation, and mucosal hyperemia after receiving nasal packing.

672

HIGH-YIELD FACTS IN

PULMONARY ADENOTONSILLAR HYPERTROPHY Adenoids develop at the posterior surface of the nasopharynx and grow to a final size at 6 to 7 years of age. Palatine tonsils are located toward the end of the soft palate. Both structures are dense in B and T lymphocytes and play a role in local immunity and in host immune defense. Adenotonsillar hypertro­ phy is characterized by recurrent infections and hypertrophy of the lymphoid­ rich structures. H i story/PE

Adenotonsillar hypertrophy usually manifests in children as recurrent infec­ tions and sleep-disordered breathing (SOB).

■

Adenoid hypertrophy: History of mouth breathing, hyponasal voice, ade­ noid facies, rhinorrhea, and postnasal drip is commonly described. Tonsillar hypertrophy: History of recurrent infections (most commonly viral and then bacterial) and airway and feeding difficulties, such as dys­ phagia and SOB with OSA. Voice changes and dental malocclusion may be seen. SOB: History of excessive daytime somnolence, failure to thrive, enuresis, poor school performance, and/or behavioral disturbance due to chronic sleep deprivation.

Diagnosis ■

■ ■

Adenoid hypertrophy: Rule out other causes of snoring and SOB such as anterior nasal obstruction. ■ Best diagnostic test: Nasopharyngoscopy to visualize the hypertro­ phied adenoid tissue ■ Other: Lateral x-ray of the neck to visualize the adenoids Tonsillar hypertrophy: Tonsil grading by physical exam is informative. SOB: Best diagnostic test: Polysomnography.

Treatment ■

■

■

Infections should be first treated with supportive treatment as needed (pain control, adequate fluid intake, antibacterial if streptococcal infection is suspected). Use modified Centor criteria to decide on the use of antibiotics against S pyogenes (most common bacterial pathogen): Absence of cough, swol­ len/tender anterior cervical lymph nodes, temperature > 38 ° C (100.4 ° F), tonsillar exudate, age of individual. Patients with recurrent infections ( > 7 episodes in the preceding year or > 5 in the preceding 2 years or >4 in the preceding 3 years): Consider tonsillectomy. Patients with obstructive SOB diagnosed by polysomnography: Perform adenoidectomy and tonsillectomy.

ACUTE AND CHRONIC LARYNGITIS Laryngitis results from laryngeal inflammation due to several factors: excessive coughing, infections, vocal abuse/strain, gastroesophageal reflux, irritants such as smoking. Chronic laryngitis usually results from multiple factors lead­ ing over time to persistent inflammation.

PULMONARY History/PE ■ Acute: Less than 3 weeks of hoarseness. ■ Chronic: More than 3 weeks of hoarseness. ■ History of the chief complaint reveals aforementioned inciting factors. Diagnosis ■ Perform physical examination of the head and neck (lymph nodes can be suggestive of malignancy). ■ In select cases, perform nasopharyngoscopy to visualize the vocal cords (lesions on the vocal cord such as polyps or nodules, bilateral motion of the vocal cords, edema). Treatment ■ Supportive management is often needed with vocal hygiene (absolute silence is not required), hydration, cough suppression, and avoidance of precipitating/irritating factors such as smoking. ■ Proton pump inhibitors are needed if reflux is suspected or diagnosed.

LARYNGOPHARYNGEAL REFLUX Reflux of caustic gastric contents causing irritation of laryngeal tissue. Related to resting upper and lower esophageal sphincter tone and also to duration/ magnitude of increased intra-abdominal pressure (eg, obesity would cause chronic increased intra-abdominal pressure).

History/PE ■ Dysphonia ■ Cough ■ Globus ■ Throat clearing ■ Dysphagia Diagnosis Largely based on clinical signs/symptoms Treatment ■ Dietary changes (avoidance of caffeine, chocolate, peppermint, alcohol, and acidic foods) ■ Behavioral changes (avoidance of smoking, waiting 2 hours after eating for vigorous exercise, avoidance of eating/drinking 3 hours before sleep) ■ Acid suppression (proton pump inhibitors [PPis], H 2 antagonist)

BENIGN AND MALIGNANT LARYNGEAL LESIONS Vocal Cord Polyp Most common benign laryngeal lesion. Typically caused by vocal cord over­ use (eg, singers, teachers), smoking, and/or gastroesophageal reflux disease (GERD). ■ ■ ■

Hx/PE: Presents with hoarseness and, in severe cases, dyspnea Dx: Laryngoscopy or stroboscopy Tx: Voice therapy, microsurgery

HIGH-YIELD FACTS IN

673

674

HIGH-YIELD FACTS IN

PULMONARY

Voca l Cord Nodule

Typically caused by vocal cord overuse (eg, singers, teachers), smoking, and/ or GERO. Presents with hoarseness. ■ Dx: Laryngoscopy or stroboscopy ■ Tx: Voice therapy, smoking cessation, PPI if GERO symptoms present, ste­ roid injections, microsurgery Recu rrent Respiratory Pa pillomatosis

Recurrent respiratory papillomatosis is a rare disease caused by human pap­ illomavirus (HPV) infection of the upper airway, resulting in warty growths. In about 3 % to 5 % of patients, malignant transformation to squamous cell carcinoma may occur. The juvenile form is due to peripartum transmission from an infected mother. Children may need up to 20 repeat procedures during their life­ time, causing significant morbidity; however, remission may occur after several years. ■ The adult form is probably transmitted through oral sex. Risk Factors ■ Juvenile form: Triad of being firstborn, vaginal delivery, and mother's age 145 mEq/L. Usually caused by free water loss rather than sodium gain. H istory/PE

Often presents with thirst caused by hypertonicity. Patients can present with neurologic symptoms including altered mental status, weakness, focal neuro­ logic deficits, and seizures. Diagnosis

The etiology of hypernatremia can be determined by measunng urme osmolality. If urine osmolality is >600 mOsm/kg, hypernatremia most likely stems from extrarenal water loss (insensible losses, nasogastric tube suction, diar­ rhea) or excess sodium intake. Measuring urine sodium through a frac­ tional excretion of sodium can be helpful in distinguishing extrarenal losses (< l % ) from sodium gain (>2% ). ■ If urine osmolality is 48 hours) should be accomplished gradually over 48 to 72 hours ( :::s0. 5 mEq/L/hr) to prevent neurologic damage secondary to cerebral edema. In acute hypernatremia ( 14 mg/dL, a patient requires urgent treatment with iso­ tonic IV fluids (± furosemide) and calcitonin; bisphosphonates (eg, zole­ dronic acid, pamidronate) should be considered as well. High sodium intake (in isotonic fluids) facilitates renal calcium excretion and prevents renal complications (stones). ■ Asymptomatic patients with serum calcium between 12 and 14 mg/dL do not require emergent treatment - they may follow the same precautions noted earlier for mild hypercalcemia, as well as some therapies (eg, iso­ tonic fluids) to facilitate excretion of calcium before symptoms develop.

HYPOCALCEMIA Serum calcium 44 mm Hg

Respiratory acidosis Hypoventilation

Airway obstruction Acute lung disease Chronic lung disease Opioids, sedatives Weakening of respiratory muscles

Pco, 12 mEq/L

GOLDMARK:

1

Alkalemia

Metabolic alkalosis Check urine Cl-

Hyperventilation

> 20 m Eq / L

Anxiety/panic attack Hypoxemia (eg, high altitude) Salicylates (early) Tumor Pulmonary embolism Pregnancy

8-12 mEq/L

Normal anion gap HARDASS

Hyperchloremia/hyperalimentation Addison disease Renal tubular acidosis Diarrhea Acetazolamide Spironolactone Saline infusion

(

Saline-resistant

45

40

E

.s

Respiratory acidosis

Vomiting Recent loop/ thiazide diuretics Antacids Pco = 40 rtim Hg Mixed alkalosis

30

�� 25 ;:,::_ 20 � 19 15 10

Metabolic acidosis

5

Respi ratory alkalosis

6.9 7.0 7.1 7.2 7.3 7.4 7.5 7.6 7.7 7.8 7.9 pH

Diagnostic algorithm for acid-base disorders. (Ada pted with permission from USMLE-Rx.com.)

I

URINE ANION GAP ■ Calculated for normal anion gap acidosis to distinguish renal vs GI bicar­ bonate loss. ■ Urine anion gap (UAG) = [Urine Na + ] + [Urine K + ] - [Urine CI- J . ■ Urine NH4+ (which represents renal acid excretion but is difficult to mea­ sure) is excreted along with CI- in the urine. A negative UAG indicated increased urine CI-, which suggests that acid (NH/) is being excreted by the kidneys. This can be seen in cases of GI HC0 3- loss or proximal/type 2 RTA. TA B L E 2 . 1 5 - 1 .

'\

Saline-responsive

Hyperaldosteronism Bartter syndrome Gitelman syndrome

2 35 0

< 20 mEq/L

Compensation for Acid-Base Disorders

PRI MARY DISORDER

EXPECTED COM PENSATION

Meta bolic acidosis

PaCO 2 = (1 .5 x HCO, -i + 8 ± 2 (Wi nters formula)

Meta bolic a l ka losis

10 m Eq/L i i n [HCO, -1 ➔ 7 m m Hg i PaCO 2

Respi ratory acidosis (chronic)

10 m m Hg i PaCO2 ➔ 4 m Eq/L i in [HCO, -1

Respi ratory a l ka losis (chronic)

10 m m Hg t PaCO2 ➔ 4 m Eq/L t in [HCO, -1

A 26-yea r-o l d wo m a n with a h i story of d epress ion p resents to the emergency depa rtment with a l te red menta l status, t i n n itus, nau sea, a n d vo miting. An a rterial blood gas (ABG) assessment shows a pH of 7.4, Paco2 of 22, a n d a HCO3 - of 1 3. What is the m ost l i kely diag nosis, a n d what is her aci d-base d i sorder ?

I A 1 7-year-old boy with a h i story of ast h m a p rese nts to the em erge ncy depa rtment with severe sh ortness of breath. H is a rteri a l pH has g o n e fro m 7.49 t o 7 . 3 8 a n d h i s Paco2 from 30 to 50 m m Hg s i n ce the ti me of adm issi o n . What is the n ext best step i n management?

684

HIGH-YIELD FACTS IN

RENAL/GENITOURINARY

A EB UAG suggests impaired NH/ excretion, which is seen in cases of distal/type 1 RTA. ■ GI bicarbonate loss (eg, diarrhea) ➔ 8 UAG ■

RENAL TUBULAR ACIDOSIS

0-n

KEY FACT

A history of AKI and nephrotoxin exposure should make the physician suspect a diagnosis of acute tubular necrosis (ATN).

0,0 MNEMONIC Indications for urgent dialysis if refractory to medical managementAEIOU Acidosis Electrolyte abnormalities (hyperkalemia) Ingestions (salicylates, theophylline, methanol, barbiturates, lithium, ethylene glycol) Overload (fluid) Uremic symptoms (pericarditis, encephalopathy, bleeding, nausea, pruritus, myoclonus)

The most l i kely diagnosis is an aspirin overdose. Though her pH is normal, she has a mixed metabolic acidosis and respiratory alka losis. Her bica rbonate is low, indicati ng a metabolic acidosis. Wi nters formula pred icts that the patient's Paco2 under normal com pensation should be 29 [Paco2 = 1 S (HCO 3 -) + 8] . Her Paco2 is lower than this at 22, which ind icates a concu rrent respi ratory alkalosis.

The patient's symptoms and lab resu lts indicate respi ratory muscle fatigue. The next best step i n management may be u rgent i ntu bation.

A net .!. in either tubular H + secretion or HCO 3 - reabsorption that leads to a non-anion gap metabolic acidosis. There are three main types of RTA; type IV (aldosterone deficient/resistant) is the most common form (see Table 2.1 5-2).

ACUTE KIDNEY INJURY Formerly known as acute renal failure, acute kidney injury (AKI) is defined as renal function, compared with a previous baseline within a period of 40% of left ventricular function)

Obstructive

Identify the cause and treat if possible; give ino­

i

Ca rdiac ta mponade, tension pneu­ mothorax, massive pulmonary

-!.

embolism

i i

Treat the u nderlying ca use: pericardiocentesis, decom pression of pneumothorax, and/or

throm bolysis

Equalization of pressu res in all ca rdiac chambers disti nguishes ta m ponade from other obstruc­

tive shock i

Distributive Septic

Any i nfection, but particularly

common with bacteremia, espe­ cia lly gram 8 organisms

Ad minister broad-spectrum a ntibiotics

Give crysta l loid fl uids u p to 30 m Ukg ideal body weight

Vasopressors (norepi nephrine, vasopressin) may

be needed if hypotension persists despite fl uid resuscitation

Obta i n cultures before administration of a ntibi­ otics, when possible

Anaphylactic

Bee sti ng, medications, food al lergy

Manage with 1 : 1 000 epinephrine with potentia l

Systemic

Pancreatitis, burns, trauma

Manage underlyi ng ca use

Brain or spinal cord injury

Mainta i n pressu res with fluid and pressor su pport

inflam matory

adjuncts of H,/H, a ntagonists and steroids

response

synd rome Neurogenic

CHF, Congestive heart fa i l u re; H, histami ne; Ml, myocardial i nfarction.

MULTI SYSTEM HIGH -YIELD FACTS IN 0-,,. KEY FACT

FEVER POSTOPERATIVE FEVER Occurs in 40% of all postoperative patients. Timing after surgery determines the most likely cause (Fig. 2.16-1). Fever before day 3 is rarely of infectious origin. Table 2.16-2 summarizes the most common etiologies, based on post­ operative day (POD) of onset.

FEVER OF UNKNOWN ORIGIN A temperature >38.3 ° C (100.9 ° F) of at least 3 weeks' duration that remains undiagnosed following three outpatient visits or 1 week of hospitalization.

History/PE Presents with fever. May complain of headache, myalgia, and malaise. The differential diagnosis includes the following: ■ Infectious: Tuberculosis (TB), endocarditis (eg, HACEK organisms; see the Infective Endocarditis section in the Cardiovascular chapter), occult abscess (abdominal, prostatic ), osteomyelitis, catheter infections, sinusitis.

Anytime Wind

Drug reactions

Atelectasis Pneumonia

0-,,. KEY FACT

Immediate fever after administration of halothane or succinylcholine should raise concern for malignant hyperthermia. Assess for rigidity, metabolic acidosis, and electrolyte derangements. Treat with dantrolene and active cooling.

0-,,. KEY FACT

Overall, infections and cancer account for the majority (60%) of cases of fever of unknown origin. Autoimmune diseases account for - 1 5%. In older adults, rheumatic diseases account for one third of cases.

Subacute/delayed

Acute

Wonder drugs

Endometritis is an additional cause of postoperative fever after (-section. Onset occurs anytime from POD 2 to 1 0.

Wound

Water Walking

DVT PE

UTI

Surgical site infection

Surgery ------•----• -•--• --• -•-.....• -------

POD l

2

3

4

5

6

7+

F I G U R E 2.1 6- 1 . Common causes of postoperative fever by timing. The 5Ws mnemonic can be used to remember the order of causes of postoperative fever. POD, Postoperative day. (Repro­ d uced with permission from USMLE-Rx.com.)

TA B L E 2 . 1 6-2.

71 9

Postoperative Fever Timing

TIMING

ETIOLOGY

PREVENTION

MNEMONIC

Any time

Drug reactions

N/A

Wonder drugs

I n centive spirometry, early mobil ization

Wind

Exa mples: malignant hyperthermia

(related to i ntraoperative anesthetics),

a ntibiotics, blood prod uct transfusion reactions

Postoperative days 1 -3

Atelectasis

Pneumonia (day 3)

Antibiotics

PODs 3-4

U rinary tract infection

Short-term Foley catheter use

Water

PODs 4-5

Deep venous throm bosis/pulmonary

Early mobilization, heparin, seq uential

Wa lking

PODs 7 +

Surgica l site i nfection

Dressing changes, preoperative

Wound

embolism

compression socks

a ntibiotics

720

HIGH-YIELD FACTS IN

MULTISYSTEM

■ ■

■

■

In HIV patients, consider Mycobacterium avium complex (MAC), histo­ plasmosis, and cytomegalovirus (CMV). Neoplastic: Lymphomas, leukemias, hepatic and renal cell carcinomas. Autoimmune: Still disease, systemic lupus erythematosus (SLE), cryoglob­ ulinemia, polyarteritis nodosa, connective tissue disease, granulomatous disease (including sarcoidosis). Miscellaneous: Pulmonary emboli/deep venous thrombosis (DVT), inflammatory bowel disease (IBO), alcoholic hepatitis, drug fever, familial Mediterranean fever, factitious fever. Idiopathic (10%-1 5 % ).

Diagnosis

Confirm the presence of fever and take a detailed history, including family, social, sexual, occupational, dietary, exposures (pets/animals), and travel. ■ Labs: Obtain a complete blood cell count (CBC) with differential; eryth­ rocyte sedimentation rate (ESR) or C-reactive protein (CRP); serum pro­ tein electrophoresis; multiple blood cultures (similar to osteomyelitis workup); sputum Gram stain and culture; urinalysis (UA) and culture; and purified protein derivative (PPD). Complete appropriate cancer screening. Specific tests (antinuclear antibody [ANA], rheumatoid factor [RF], creatine kinase [CK], viral cultures, viral serologies/antigen tests) can be obtained if an infectious or autoimmune etiology is suspected. ■ Imaging: Obtain a x-ray of the chest (CXR). CT of the chest, abdomen, and pelvis should be done early in the workup of a true fever of unknown origin (FUO). Invasive testing (marrow/liver biopsy) is generally low yield. Nuclear medicine and laparoscopy are higher yield as second-line tests (after CT). Case by case: Consider serologic testing for less common etiologies like Brucella spp., Coxiella spp., and Bartonella spp. Treatment

Stop unnecessary medications. Remove indwelling lines or other potential sources of infection. Patients with FUO who have a completely 8 workup have a favorable prognosis, with fevers resolving over months to years.

SEPSIS Sepsis is defined by organ dysfunction caused by a dysregulated host response to infection. Septic shock is essentially sepsis with associated profound meta­ bolic, cellular, and circulatory disturbances that confer an increased risk of mortality. History/PE

Sepsis can be identified when there are signs of organ dysfunction in the set­ ting of known or suspected infection. Clinical tools such as the sequential organ failure assessment (SOFA) or "quick" SOFA scores help identify and characterize dysfunction of several organ systems by noting the presence of tachycardia, altered mental status, and hypotension, among other findings. ■ Septic shock is typically a "warm" shock, with warm skin and extremities. This contrasts with cardiogenic shock, which typically presents with cool skin and extremities. Petechiae, ecchymoses, and/or abnormal coagulation tests suggest dissemi­ nated intravascular coagulation (DIC; 2%-3 % of cases). ■

MULTISYSTEM HIGH -YIELD FACTS IN

72 1

Diagnosis ■ Laboratory results frequently show leukocytosis or leukopenia with i bands, thrombocytopenia (50% of cases), evidence of J, tissue perfusion (I creati­ nine, i liver function tests [LFTs], i lactate), and abnormal coagulation studies (I international normalized ratio [INR]). Hypotension requiring vasopressors to maintain mean arterial pressure (MAP) >65 mm Hg or per­ sistently elevated lactate (> 2 mEq/L) despite adequate fluid resuscitation diagnoses septic shock. ■ It is critical to obtain cultures of all appropriate sites (eg, blood, sputum, cerebrospinal fluid [CSF], wound, urine). Obtaining cultures should not delay antibiotic administration. ■ Imaging (CXR, CT) may aid in establishing the etiology or site of infection. Treatment ■ Intensive care unit (ICU) admission may be required. Treat aggressively with empiric antibiotics (based on the likely source of infection). Volume resuscitation with intravenous (IV) crystalloid and vasopressors should be used as needed to maintain adequate MAP (>65 mm Hg) and optimize end-organ perfusion. ■ Treat underlying factors (eg, remove urinary catheter or infected lines, drain abscesses).

H EMATOLOGIC INFECTIONS MALARIA A protozoa! disease caused by five species of the genus Plasmodium (P falci­ parum, P vivax, P ovale, P malariae, P knowlesi) and transmitted by the bite of an infected female Anopheles mosquito. P falciparum has the highest morbid­ ity and mortality, occasionally within 24 hours of symptom onset. Travelers to endemic areas should take chemoprophylaxis and use mosquito repellent and bed nets to minimize exposure.

History/PE ■ Patients have a history of exposure in a malaria-endemic area, with peri­ odic attacks of sequential chills, fever (up to 41 ° C [105.8 ° F]), myalgias, headache, and diaphoresis occurring over 4 to 6 hours. ■ Splenomegaly often appears 4 or more days after symptom onset. Patients are often asymptomatic between attacks, which recur every 2 to 3 days, depending on the Plasmodium species involved. ■ Severely ill patients may present with hyperpyrexia, prostration, impaired consciousness, pulmonary edema, acidosis, hyperventilation, and bleed­ ing. The presence of a rash, skin ulcer, eosinophilia, lymphadenopathy, neck stiffness, or photophobia suggests a different or additional diagnosis. Diagnosis ■ Timely diagnosis of the correct species is essential because P falciparum can be fatal and is often resistant to standard chloroquine treatment. ■ The physician should send Giemsa- or Wright-stained thick and thin blood films for evaluation to detect Plasmodium and determine the species type, respectively, and the degree of parasitemia (see Fig. 2.16-2). ■ CBC usually demonstrates normochromic, normocytic anemia, with retic­ ulocytosis and thrombocytopenia early in the disease.

F I G U R E 2 . 1 6-2. Plasmodium falciparum

hyperparasitemia in the thin smear of a patient with cerebral malaria. (Reproduced with permission from the US Department of Health and Human Services and Steven Glenn.)

722

HIGH-YIELD FACTS IN

0-,r KEY FACT Cerebral malaria presents with headache, altered mental status, neurologic signs, retinal hemorrhages, convulsions, and delirium. If left untreated, it can rapid ly progress to coma and death.

0-,r KEY FACT Antimalarial contraindications: Primaq uine: Requires testing for G6PD first ■ Mefloquine: Seizure, psychiatric conditions, a nd cardiac cond uction disorders ■ Atovaquone/proguanil: Pregnancy/ breastfeeding, renal disease ■ Chloroq uine: Psoriasis ■

MULTISYSTEM ■

■

If resources allow, more sensitive serologic tests are available, including rapid antigen detection methods, fluorescent antibody methods, and poly­ merase chain reaction (PCR). In patients with altered mental status, the physician should obtain a fin­ gerstick glucose to rule out hypoglycemia.

Treatment ■ Uncomplicated malarial infection can be treated with oral agents. Chloro­ quine has historically been the standard antimalarial medication, but high resistance rates often necessitate the use of other medications such as mefloquine, atovaquone-proguanil, or artemisinins (for severe cases). In cases of P vivax, P ovale, or an unknown species, primaquine is added to eradicate the hypnozoites in the liver. For patients traveling to endemic regions, the physician should prescribe prophylaxis consisting of atovaquone-proguanil or mefloquine given at least 2 weeks before travel and continued for 4 weeks after returning. Compl ications Cerebral malaria, severe hemolytic anemia, renal impairment, noncardio­ genic pulmonary edema, hypoglycemia, lactic acidosis, acute hepatopathy, and gram 8 bacteremia.

OTHER MOSQUITO- BORNE VIRUSES The following viruses are carried by the Aedes mosquito and present with rash, fever, and myalgias: ■ ■

■

Chikungunya: Notably causes joint pain. Supportive care is the treatment. Dengue ("breakbone fever"): Presents with bone pain and can be com­ plicated by severe thrombocytopenia, bleeding, and shock. Findings of low WBCs and i LFTs. Treatment calls for fluids and blood products as needed. Zika: Flavivirus that causes conjunctivitis and headache. Associated with Guillain-Barre syndrome. It can cause microcephaly of the fetus if the patient is infected during pregnancy. Treatment is supportive care.

TICK-BORNE INFECTIONS LYME DISEASE 0-,r KEY FACT Ehrlichiosis is a disease transmitted by the lone star tick endemic to the south-centra l and southeastern U nited States. It causes headache, fever, chil ls, a ltered mental status, and myalgias, but rash is uncommon. Leukopenia, throm bocytopenia, and i liver enzymes are com mon laboratory findings. Doxycycline is the treatment of choice.

A tick-borne disease caused by the spirochete Borrelia burgdorferi. Usually seen during the summer months and carried by Ixodes ticks on white-tailed deer and white-footed mice. Endemic to the Northeast, northern Midwest, and Pacific coast.

H istory/PE ■ Presents at the onset of rash with fever, malaise, fatigue, headache, myalgias, and/or arthralgias. Infection usually occurs after a tick feeds for > 36 hours. ■ Primary (early localized disease): Erythema migrans begins as a small ery­ thematous macule or papule that is found at the tick-feeding site and expands slowly over days to weeks. The border may be macular or raised, often with central clearing ("bull's-eye"; see Fig. 2.16-3).

MULTISYSTEM HIGH -YIELD FACTS IN

723

■ Secondary (early disseminated disease) : Presents with migratory polyar­ thropathies, neurologic phenomena (eg, facial nerve palsy; bilateral is clas­ sic for Lyme disease), lymphocytic meningitis and/or myocarditis, and conduction abnormalities (third-degree heart block). ■ Tertiary (late disease) : Arthritis and subacute encephalitis (memory loss and mood change). Diagnosis

■ Early Lyme disease is diagnosed on clinical presentation alone (erythema migrans + endemic area). Serologic tests are not required or recom­ mended, as IgM becomes EB 1 to 2 weeks, and IgG 2 to 6 weeks, after onset of erythema migrans. ■ Early disseminated or late Lyme disease presenting with consistent symp­ toms and exposure risk factors should be diagnosed with serology. If enzyme-linked immunosorbent assay (ELISA) IgM and IgG are EB or equivocal, then Western blot can be used for confirmation. Western blot should not be used for "screening" or nonspecific symptoms. Western blots sent without ELISA have high false EB rates. Treatment

■ If the tick is still attached, remove it with forceps and thoroughly disinfect the area. Treat early disease with doxycycline (or amoxicillin in pregnant patients). Short courses of doxycycline may be used in young patients. More advanced disease (eg, central nervous system [CNS], cardiac, or arthritic disease) should be treated with ceftriaxone. ■ Consider empiric therapy for patients with the characteristic rash, arthral­ gias, or a tick bite acquired in an endemic area. Prevent with tick bite avoidance. ■ Prophylaxis: Give one dose of doxycycline if all of the following apply: tick is Ixodes scapularis and has been attached for 2:: 36 hours, prophylaxis is started -:::; 72 hours of removal, patient has no contraindications to doxy­ cycline, and local rate of infection of ticks with B burgdorferi is > 20%. If criteria are not met, observe and treat only if erythema migrans develops.

BABESIOSIS Tick-borne protozoa! illness also transmitted by I scapularis (high rate of coin­ fection with Lyme disease). Causes flulike symptoms, intravascular hemolysis, anemia, and jaundice. Ring-shaped or "Maltese cross" organisms may be seen on blood smear. Can become severe if risk factors are present (eg, asplenia, immunocompromise, or malignancy). Treat with oral azithromycin (IV if severe) and atovaquone.

ROCKY MOUNTAIN SPOTTED FEVER A disease caused by Rickettsia rickettsii and carried by the American dog tick (Dermacentor variabilis) . Cases are most common during June and July and in Midwestern and Eastern states (Arkansas, Missouri, Virginia, and North Carolina). The organism invades the endothelial lining of capillaries and causes small vessel vasculitis. ■ Hx/PE: Presents with headache, fever, malaise, and rash. The characteris­ tic rash is initially macular (beginning on the wrists and ankles) but

F I G U R E 2 . 1 6-3. Erythema chronicum migrans seen in Lyme disease. Note the

classic "bull's-eye" lesion, which consists of an outer ring where the spirochetes are found, an inner ring of clearing, and central erythema caused by an allergic response at the site of the tick bite. (Repro­ duced with perm ission from the US Department of Health and H u m a n Services and James Gatha ny.)

0-,r KEY FACT

"Tick testing" is a common incorrect answer choice; it has no effect on management and is not performed in a Lyme disease workup.

724

HIGH-YIELD FACTS IN

MULTISYSTEM

■ ■

becomes petechial/purpuric as it spreads centrally (see Fig. 2.16-4). Altered mental status or DIC may develop in severe cases. Dx: Clinical diagnosis should be confirmed with biopsy and indirect immunofluorescence of the skin lesion. Tx: Empiric therapy with doxycycline. The condition can be rapidly fatal if left untreated. If clinical suspicion is high, treatment should begin while awaiting testing. Chloramphenicol can be used during the first two trimes­ ters of pregnancy in uncomplicated cases, but if it is not available, doxycy­ cline therapy should be initiated.

INFECTIOUS MONONUCLEOSIS F I G U R E 2 . 1 6-4.

Rocky Mountain spot­

ted fever. These erythematous macular

lesions will evolve into a petechial rash that will spread centrally. (Reproduced with

permission from Wolff K, Johnson RA, Saavedra AP. Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatol­ ogy, 7th ed. New York, NY: McGraw-Hill; 201 3.)

0--n

KEY FACT

Rocky Mounta i n spotted fever sta rts on the wrists a n d a n kles a n d then spreads centra l ly.

0-,r KEY FACT Lym p hocytosis in EBV i nfection is pred o m i n a ntly ca used by B-ce l l prol iferation, b u t t h e atypica l cel l s a re T lym phocytes.

Most commonly occurs in young adult patients; usually caused by acute Epstein-Barr virus (EBY) infection. Transmission most often occurs through exchange of body fluids, most commonly saliva.

History/PE ■ Presents with fever and pharyngitis. Fatigue invariably accompanies initial the illness and may persist for 3 to 6 months. Examination may reveal low­ grade fever, generalized lymphadenopathy (especially posterior cervical), tonsillar exudate and enlargement, palatal petechiae, a generalized macu­ lopapular rash, splenomegaly, and bilateral upper eyelid edema. Symp­ toms appear 2 to 5 weeks after infection. ■ In older children and adults, it may cause mesenteric lymphadenitis, mim­ icking appendicitis. ■ Patients who present with pharyngitis as their primary symptom may be misdiagnosed with streptococcal pharyngitis (30% of patients with infec­ tious mononucleosis are asymptomatic carriers of group A streptococcus [GAS] in their oropharynx). ■ The differential diagnosis includes CMV, toxoplasmosis, HIV, human her­ pesvirus (HHV) 6, other causes of viral hepatitis, and lymphoma. Diagnosis ■ Best initial test: Heterophile antibody (Monospot) test. It may be 8 in the first few weeks after symptoms begin. ■ An EBY-specific antibody test can be ordered in patients with suspected mononucleosis and a 8 Monospot test. Infectious mononucleosis syn­ dromes with a 8 Monospot test and 8 EBY antibody are most often caused by CMV infection. Acute HIV and other viral etiologies should be considered. ■ CBC with differential often reveals mild thrombocytopenia with relative lymphocytosis and > 10% atypical T lymphocytes. ■ A comprehensive metabolic panel usually reveals mildly elevated transaminases, alkaline phosphatase, and total bilirubin. Treatment Treatment is supportive, as there is no effective antiviral therapy. Corticoste­ roids are indicated for airway compromise caused by tonsillar enlargement, severe thrombocytopenia, or severe autoimmune hemolytic anemia. Compl ications ■ CNS infection: Can present as aseptic meningitis, encephalitis, menin­ goencephalitis, cranial nerve palsies (particularly cranial nerve [CN]

MULTI SYSTEM HIGH -YIELD FACTS IN

■

■ ■

■ ■ ■

VII), optic and peripheral neuritis, transverse myelitis, or Guillain-Barre syndrome. Splenic rupture: Occurs in

600

3 ,-

500 400 300

Primary infection

1

Months

2

4

6

Yea rs

7

8

10

F I G U R E 2 . 1 6-6. Time course of HIV infection. Note that the level of CD4+ cells remains normal for many years but then declines, resulting in the immunodeficiency stage, which is characterized by opportunistic infections and malignancies. (Reproduced with permission from USMLE-Rx.com.)

HIV SEROLOGY MONITORS DISEASE PROGRESSION ■ CD4 + cell count: Best indicator of immunosuppression, risk for disease progression, and opportunistic infections (Ols). Guides 01 prophylaxis and assesses response to antiretroviral therapy (ART). ■ Viral load: Assesses response to ART and provides prognostic information.

History/PE Depends on stage of infection (see Fig. 2.16-6). ■ Acute HIV: Initial infection is asymptomatic in 10% to 60% of cases. If symptomatic, the acute HIV syndrome occurs 2 to 4 weeks after exposure and presents as a mononucleosis-type syndrome (suspect HIV in person with sexually transmitted infection [STI] risk factors) and/or a flulike syn­ drome. Symptoms may include fever, sore throat, cervical lymphadenopa­ thy, maculopapular rash, headache, painful mucocutaneous ulcers, and gastrointestinal (GI) symptoms. Notably, the viral RNA load is elevated, but seroconversion has not yet occurred (antibodies nonreactive). ■ Chronic (clinically latent) HIV: Can be asymptomatic for a period of 8 to 10 years. Persistent generalized lymphadenopathy may be present along with fatigue. Notably, Candida infections of thrush or vaginitis in a patient with risk factors for STis should raise clinical suspicion for HIV (see Fig. 2.16-7). Seborrheic dermatitis is also a common finding in this stage. Overall, the CD4+ T-cell count progressively declines over multiple years in this phase. The latency of this phase is why screening is recommended (see later diagnosis section).

F I G U R E 2 . 1 6-7. Oral thrush in H IV­ positive patient. (Reproduced with permission

from Ors. John Molinari and Sol Silverman, Jr., Centers for Disease Control and Prevention, Atlanta, GA.)

728

HIGH-YIELD FACTS IN

MULTISYSTEM ■

■

■ ■

■

AIDS: The average time, in the absence of treatment, from exposure ➔ AIDS is 8 years. Defined as a CD4+ T-cell count 20,000 feet). Diagnosis

Clinical diagnosis.

0-,,. KEY FACT Common microbiology of bites: Pasteurella species, Capnocytophaga canimorsus, Bartone/la, and Staphylococcus and Streptococcus species.

0-,,. KEY FACT Bites i nvolving sharp teeth a n d res u lting i n d e e p pu ncture s h o u l d n o t b e sutured closed. Treat with a moxici l l i n/clavu l a n ic acid and mon itor for developing deep tissue i nfections, including osteomyelitis.

Acute mountain sickness: dizziness, headache, fatigue, nausea, vomiting High-altitude pulmonary edema: cough, shortness of breath, hypoxia, crackles on auscultation ■ High-altitude cerebral edema: severe fatigue, confusion, ataxia ■ ■

Treatment ■ Supplemental 0 2 ■ Nonsteroidal anti-inflammatory drugs (NSAIDs) for treating headaches Acetazolamide ■ Dexamethasone ■ Descent from high altitude

BITES AND STINGS Figures 2.16-14 and 2.16-15 summarize the recommended prophylaxis for rabies and tetanus. Table 2.16-7 outlines the management of common bites and stings.

MULTISYSTEM HIGH -YIELD FACTS IN

I

Dom estic animal (pet cat/dog)

Animal bite

L

Wild animal (bats, racoons, skun ks)

{ ----+

Vaccination status known/healthy animal Vaccination status unknown

Euthanize and test bra i n for ra bies

Proper rabies prophylaxis Previously immun ized - vaccine o n ly Never i m m u n ized - vacci n e + rabies l g

F I G U RE 2 . 1 6- 1 4.

--

{

No prophylaxis needed

Quaranti n e 10d for signs of ra bies. If animal not { available, req uire post exposure pro phylaxis Animal negative Animal positive for rabies or u nava i lable

741

S i g n s of rabies --+ No prophylaxis needed No signs

--+

Pt needs prophy laxis

-- No prophylaxis needed --

Pt n eeds prophylaxis

Rabies postexposure prophylaxis algorithm. (Reproduced with perm ission from USM LE-Rx.com.)

Cl�o. miom woaods [

10 years ago Otherwise

Assess wound

< 3 lifetime toxoids All other wounds

Td (or TdaP)

---+

---+ Td (or TdaP) + tetanus lg

Last toxoid >5 years ago ---+ Otherwise

No prophylaxis

---+

Td (or TdaP) No prophylaxis

F I G U RE 2 . 1 6- 1 5. Tetanus prophylaxis algorithm. (Reprod uced with perm ission from USMLE-Rx.com.)

TA B L E 2 . 1 6-7.

Management of Bites and Stings

SO U RCE

POTENTIAL COMPLICATION

MANAGEMENT

Bees and wasps

Anaphylaxis

Anti histamines and steroids; i ntramuscular (IM) epineph­

Spiders

Black widow: Muscular spasms (can mimic rigid acute

Black widow: Antivenin; classic treatment with ca >+ gluco­

Brown recl use: Necrosis, flu l i ke symptoms, d issemi­

Brown recl use: Cold compresses slow necrosis; dapsone

a bdomen but no rebound)

nated intravascular coagu lation

rine if anaphylaxis develops

nate is largely proven i neffective

may help (contraindicated in G6PD deficiency); debride­ ment should be limited to obviously necrotic tissue

(continues)

742 TA B L E 2.1 6-7.

HIGH-YIELD FACTS IN

MULTISYSTEM

Management of Bites and Stings (contin ued)

SOURCE

POTENTIAL COMPLICATION

MANAGEMENT

Scorpions

I n severe cases, neuromuscu lar toxicity manifests as cranial nerve dysfunction, excessive motor activity (can be mistaken for seizure), autonomic dysfunction (hypersalivation), and/or respiratory

Antivenin if neuromuscular symptoms develop; if no anti­ venin is administered, benzodiazepines and analgesics help pain and spasms

compromise Snakes (Crotaline species: rattlesnake, copperhead)

Local necrosis, distributive shock, disseminated intravascular coagulation

Antivenin (Crotalidae polyvalent immune Fab) is the mainstay of treatment; keep the affected limb below the heart Compression bands, tourniquets, prophylactic fasciotomy, and resection are ineffective or outdated treatments and probably the wrong answer

Dogs and cats

Amoxicillin/clavulanate for puncture wou nds, bites to hands/feet, and high-risk or immunocompromised patients

Infection, rabies/tetanus

The physician should consider imaging cat bites for pos­ sible tooth fragments implanted in wound Humans

Infection

Amoxicillin/clavulanate

Rodents

Low risk for infection; not known to carry rabies Contact with wild rodents is a risk factor for leptospirosis

Local wound care only

Shellfish (Vibrio

Severe necrotizing fasciitis and hemorrhagic bullous lesions

IV doxycycline and ceftriaxone, emergent surgical debridement

vulnificus)

Patients with preexisting liver disease (especially hemochromatosis) have an increased risk

TOXICOLOGY RESUSCITATION OF THE POISONED PATIENT Evaluation

Consider and evaluate for toxic ingestion in any patient presenting with altered mental status or unexplained seizure-like activity. ■ Airway, breathing, and circulation always take precedence in the resuscita­ tion of the potentially poisoned patient. ■ ECG changes and cardiac dysrhythmias are common in overdose. An ini­ tial ECG should be obtained in the evaluation of the poisoned patient. ■ A thorough neurologic examination, including pupil reactivity, muscle tone, and reflexes, is important to note and can help differentiate toxi­ dromes that otherwise present very similarly. ■

Decontam ination

As part of the resuscitation of a poisoned patient, decontamination should be considered. The main goal of decontamination is preventing further drug

MULTISYSTEM HIGH -YIELD FACTS IN

743

absorption. Recent ingestions ( 1 0 mm Hg with inspiration; seen in cardiac tamponade

Classic ECG fi ndings in pericarditis

Low-voltage, diffuse ST-segment elevation

Water bottle-shaped heart

Perica rdia! effusion; look for pulsus pa radoxus

Endocarditis prophylaxis regimens

Oral surgery-amoxicillin for certain situations GI or GU procedures-not recommended

Prolonged PR interva l i n infective endocarditis suggests

Possible aortic root abscess

Classic physical findings for endoca rditis

Fever, heart murmur, Osler nodes, splinter hemorrhages, Janeway lesions, Roth spots

Duke criteria for endocarditis

Major: Positive blood cultures, new murmur, positive echocardiogram Minor: Risk factors, > 38°C, vascu lar or immunologic phenomena, echocardiogram or cultu re evidence that does not meet major criteria

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Patient develops endocarditis 3 weeks after receiving a prosthetic heart valve. What organism is suspected?

S aureus or 5 epidermidis

Patient develops endocarditis in a native valve after having a dental cleaning

S viridans

IV drug use with JVD and a holosystolic murmur at the left sternal border. Treatment?

Treat existing heart failure, and replace the tricuspid valve

Eval uation of a pulsatile abdominal mass and bruit

Abdominal ultrasound and CT (concern for abdominal aortic aneurysm)

Indications for surgical correction of abdominal aortic aneurysm

>5.5 cm, rapidly enlarging, symptomatic, or ruptured

Syncope with arm exercise

Subclavian steal syndrome

Protamine

Reverses the effects of heparin

Prothrombin time

The coagulation pa rameter affected by warfarin

Virchow triad

Stasis, hypercoagulability, endothelial damage

Syncope after wea ring a tie

Carotid sinus syndrome

Figure 3 sign on CXR

Aortic coarctation

Diagnostic test for pulmonary embolism

CT pulmonary angiogra m

DERMATOLOGY "Stuck-on" waxy appearance

Seborrheic keratosis

Erythematous plaques with silvery scales

Psoriasis

Most common malignant skin cancer. The lesion is a pearly nodule with superficial telangiectasias.

Basal cell carcinoma

Honey-crusted lesions

I mpetigo

Febrile patient with history of diabetes presents with a red, swollen, painful lower extremity

Cellulitis

EB N i kolsky sign, flaccid bullae. Treatment?

Pemphigus vulgaris; high-dose systemic steroids + immunomodu­ lator therapy

0 N i kolsky sign, tense bullae. Treatment?

Bullous pemphigoid; topical corticosteroids

Obese patient presents with hyperpigmented, velvety patches on back of neck

Acanthosis nigrica ns; check fasting blood glucose to ru le out diabetes

Dermatomal distribution of crusted vesicles

Herpes zoster (shingles) due to reactivation of the varicella zoster virus

Violaceous, flat-topped, pru ritic, polygonal pa pules or plaques

Lichen planus

l rislike target lesions

Erythema mu ltiforme

Lesion occurring in a geometric pattern i n areas where skin comes

Contact dermatitis

into contact with clothing or jewelry

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Presents with one large patch and many smaller ones in a treelike distribution

Pityriasis rosea

Flat, often hypopigmented lesions on the chest and back. KOH prep has a "spaghetti-and-meatballs" appearance

Tinea (pityriasis) versicolor

Five characteristics of a nevus suggestive of melanoma

Asymmetry, border irregularity, color variation, large diameter, clinical evolution (ABCDE)

Premalignant lesion caused by sun exposu re that can lead to SCC

Actinic keratosis

Widespread pruritic lesions at various rash stages

Va ricella (chicken pox)

"Cradle cap:'Treatment?

Seborrheic dermatitis Treat conservatively with bathing and moisturizing agents

Associated with Propionibacterium acnes and changes in androgen levels

Acne vulgaris

Most effective treatment for severe acne. Adverse effects?

Oral isotretinoin; teratogen and elevated LFTs; requires monthly blood tests and two forms of contraception for women

Painful, recurrent vesicular eruption of mucocutaneous surfaces

Herpes simplex

I nflammation and epithelial thinning of the anogenital area, predominantly in postmenopausal women

Lichen sclerosus

The second most common skin cancer. Erythematous ulcerated nodule with erosion or ulceration on sun-damaged skin.

Squamous cell carcinoma

Name the orga nism: Lesions along draining lymphatics i n a gardener

Sporothrix schenckii

ENDOCRINOLOGY Most common cause of hypothyroidism

Hashimoto thyroiditis

Lab fi ndings in Hashimoto thyroiditis

High TSH, low T4, a ntibodies to thyroid peroxidase or to thyroglobulin

Exophthalmos, pretibial myxedema, and J,, TSH

Graves disease

Most common cause of Cushing syndrome

Iatrogenic corticosteroid administration; the second most common cause is Cushing disease

Post-thyroidectomy patient presents with signs of hypoca lcemia and hyperphosphatemia

Hypoparathyroidism (iatrogenic)

"Stones, bones, groans, psychiatric overtones"

Signs and symptoms of hyperca lcemia (kidney stones, bone disease, gastrointestinal symptoms, alterations in mentation)

Hypertension, hypoka lemia, and metabolic a lkalosis

Primary hyperaldosteronism (caused by Conn's syndrome or bilateral adrenal hyperplasia)

Patient presents with tachycardia, wild swings in BP, headache, dia­ phoresis, a ltered mental status, and a sense of panic

Pheochromocytoma

Which should be used first in treating pheoch romocytoma, a- or

a-antagonists (phenoxybenzamine)

13-antagonists?

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Patient with history of lithium use presents with copious amou nts of dilute urine

Nephrogenic DI

Treatment of central DI

DDAVP

Postoperative patient with significant pai n presents with hypona­ tremia and normal vol u me status

SIADH due to stress

Antihyperglycemic agent associated with lactic acidosis

Metformin

Patient presents with weakness, nausea, vomiting, weight loss, and new skin hyperpigmentation. Lab resu lts show hyponatremia and hyperkalemia. Treatment?

Primary adrenal insufficiency (Addison disease); treat with glucocorti­ coids, mineralocorticoids, and IV fluids

Treatment of DKA

Fluids, insulin, and electrolyte repletion (chiefly K•)

Bone pain, hearing loss, i alkaline phosphatase

Paget disease

i IGF-1

Acromegaly

Galactorrhea, amenorrhea, bitemporal hemianopia

Prolactinoma

i serum 1 7-hydroxyprogesterone

Congenital adrenal hyperplasia (21 -hydroxylase deficiency)

Pancreas, pituitary, parathyroid tumors

MEN type 1

EPIDEMIOLOGY Chronic diseases such as systemic lupus erythematosus-higher prevalence or incidence?

Higher preva lence

Epidemics such as influenza-higher prevalence or i ncidence?

Higher i ncidence

What is the difference between incidence and prevalence?

Preva lence: percentage of cases of disease in a population at one point i n time I ncidence: percentage of new cases of disease that develop over a given period among the total population at risk (prevalence = inci­ dence x duration)

Describe a test that consistently gives identical results, but the results are wrong

High reliability (precision), low validity (accuracy)

The proportion of people who have the disease and test E9 is the

Sensitivity

Sensitive tests have few fa lse negatives and are used to ru le __ a disease

Out

PPD reactivity is used as a screening test because most people with TB (except those who a re anergic) will have a E9 PPD h ighly sensi­ tive or specific?

Highly sensitive for TB

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Odds ratio?

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In cohort studies, the odds of developing the disease in the exposed group divided by the odds of developing the disease in the nonex­ posed group I n case-control studies, the odds that the cases were exposed divided by the odds that the controls were exposed In cross-sectional studies, the odds that the exposed group has the disease divided by the odds that the nonexposed group has the disease

Attributable risk?

The difference in risk in the exposed and unexposed groups (ie, the risk that is attributable to the exposure)

Relative risk?

I ncidence in the exposed group divided by the incidence in the non­ exposed group

Hypothetical study found an association between ASA intake and risk for heart disease. How do you interpret an RR of 1 S?

I n patients who took ASA, the risk for heart disease was 1 S times that of patients who did not take ASA

Cross-sectional survey-incidence or prevalence?

Prevalence

Cohort study-incidence or prevalence?

I ncidence and prevalence

Case-control study-incidence or prevalence?

Neither

Difference between a cohort and a case-control study

Cohort divides groups by an exposure and looks for development of disease Case-control divides groups by a disease and assigns controls, and then goes back and looks for exposures

How do you interpret the following 95% Cl for an RR of 0.582: 95% Cl 0.502, 0.673?

Data are consistent with RRs ranging from 0.502 to 0.673 with 95% confidence (ie, we are confident that, 95 out of 1 00 times, the true RR will be between 0.502 and 0.673)

Bias introduced into a study when a clinician is aware of the patient's treatment type

Observational bias

Bias introduced when screening detects a disease earlier and thus lengthens the time from diagnosis to death, but does not improve

Lead-time bias

survival

If you wa nt to know if geographic location affects i nfant mortality

Confounding variable

The percentage of cases within 1 SD of the mean? 2 SDs? 3 SDs?

68%, 95.4%, 99.7%

Most common cancer i n men and most com mon cause of death from cancer in men

Prostate cancer is the most common cancer in men, but lung cancer causes more deaths

rate but most variation in infant mortality is predicted by socioeco­ nomic status, then socioeconomic status is a __

Birth rate?

Number of live births per 1 000 population in 1 year

Mortality rate? Neonatal morta lity rate? I nfant mortality rate?

Number of deaths per 1 000 population in 1 year Number of deaths from birth to 28 days per 1 000 live births in 1 year Number of deaths from birth to 1 yea r of age per 1 000 live births (neonata l + postnatal mortality) in 1 year

Maternal mortality rate?

Number of deaths during pregnancy to 90 days postpa rtum per 1 00,000 live births in 1 year

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HEALTH SYSTEMS SCIENCE When is hospice care indicated?

When prognosis is 40; BMI 35-39.9 with at least one comorbidity (eg, OSA, DM, HTN); BMI 30-34.9 with uncontrolled type 2 DM or metabolic syndrome

Diagnosis of celiac disease

lgA anti-transglutaminase antibody, anti-endomysial antibody

Presentation of acute mesenteric ischemia?

Acute abdominal pain with blood per rectum

Management of acute GI bleeding

ABCs, stabilize hemodynamics

Diagnosis of spontaneous bacterial peritonitis?

Ascitic fluid PMNs > 250

Causes of acute hepatitis causing LFTs > 1 000 U/L?

Drug-induced hepatitis, viral hepatitis, ischemic hepatitis

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HEMATOLOGY Five causes of microcytic a nemia

IRON LAST-IRON deficiency, Lead poisoning, Anemia of chronic

disease, Sideroblastic anemia, Thalassemia Elderly man with hypochromic microcytic anemia. Diagnostic test?

Suspect colorectal cancer, sigmoidoscopy/colonoscopy

Precipitants of hemolytic crisis in G6PD deficiency

Sell fava beans in INDIA-Sulfa drugs, fava beans, Infections, Nitro­

furantoin, Dapsone, lsoniazid, Antimalarials Most common inherited cause of hypercoagulability

Factor V Leiden mutation

Most common inherited bleeding disorder

von Willebrand disease

Most common inherited hemolytic anemia

Hereditary spherocytosis

Diagnostic test for hereditary spherocytosis

Osmotic fragility test

How do you differentiate between AIHA and heredita ry spherocytosis?

Spherocytes, EEl ve osmotic fragility tests, but only AIHA has EEl direct Coombs test

Pure RBC aplasia

Diamond-Blackfan anemia

Anemia associated with absent radii and thumbs, diffuse hyperpig­ mentation, cafe au lait spots, microcephaly, and pancytopenia

Fanconi anemia

Medications and viruses causing Aplastic a nemia

Ch loramphenicol, sulfonamides, radiation, HIV, chemotherapeutic agents, hepatitis, parvovirus B 1 9, EBV

How to distinguish polycythemia vera from secondary polycythemia?

Both have i hematocrit and RBC mass, but polycythemia vera should have normal 02 saturation and low erythropoietin levels

TIP pentad

LMNOP-Low platelet count (thrombocytopenia), Microangiopath ic

hemolytic a nemia, Neurologic changes, "Obsolete" renal fu nction, Pyrexia Treatment forTTP

Emergent large-volume plasmapheresis, corticosteroids, antiplatelet drugs

HUS triad

Anemia (microa ngiopathic hemolytic anemia), thrombocytopenia, and acute renal failure

ITP treatment?

Children: Usually resolves spontaneously Adults: May require IVIG and/or corticosteroids

Diagnostic tests in DIC?

Fibrin split products and o-dimer are i; platelets, fibrinogen, and hematocrit are J,,

8-year-old boy presents with hemarthrosis and i PTT with normal PT and bleeding time. Diagnosis? Treatment?

Hemophilia A or B; consider desmopressin (for hemophilia A) or factor

1 4-year-old girl presents with prolonged bleeding after dental su rgery and with menses, normal PT, normal or i PTT, and i bleeding time. Diagnosis? Treatment?

von Willebrand disease; treat with desmopressin, FFP, or cryoprecipitate

VI I I or IX supplements

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Findings in multiple myeloma

Monoclonal gammopathy, Bence Jones proteinuria, and "punched out" lesions on radiographs of the skull and long bones

Reed-Sternberg cells

Hodgkin lymphoma

Microcytic anemia with J,, serum iron, J,, ferritin, and i TIBC

Iron-deficiency anemia

Microcytic anemia with J,, serum iron, J,, TI BC, and normal or iferritin

Anemia of chronic disease

80-year-old man presents with fatigue, lymphadenopathy, spleno­ mega ly, and isolated lymphocytosis. Diagnosis?

CLL

Causes of J,, hemoglobin and i mean corpuscular volume?

Vitamin B, 2 deficiency (pernicious anemia, vegetarian diet, Crohn/GI disorders) or folate deficiency (alcohol use disorder)

Late, life-threatening complication of CML

Blast crisis (fever, bone pain, splenomega ly, pancytopenia)

Auer rods on blood smear

AML

AML subtype associated with DIC? Treatment?

APL (M3); All-trans retinoic acid

Electrolyte changes in tumor lysis syndrome

J,, Ca 2•, J,, K•, J,, phosphate, J,, uric acid

CML cytogenetics

t(9,22)

CML treatment?

BCR-ABL tyrosine kinase inhibitors-imatinib

Neutropenic fever. Diagnosis? Treatment?

ANC < 1 500 cells/mm'; broad-spectru m antibiotics

Virus associated with aplastic anemia in patients with sickle cell anemia

Parvovirus B 1 9

Treatment of bone crisis i n sickle cell anemia?

02, analgesia, hydration, and, i f severe, transfusion

Significant ca use of morbidity i n thalassemia patients. Treatment?

Iron overload; treat with deferoxamine

Aplastic crisis in sickle cell disease

Parvovirus B 1 9

MUSCULOSKELETAL Fractures that occur during fall onto an outstretched hand

Colles fracture, Smith fracture, scaphoid fracture

Most common hip dislocation. Clinical presentation? Complication?

Posterior hip dislocation; painful hip in a position of flexion, internal rotation and adduction; sciatic nerve injury

Knee injury due to noncontact twisting mecha nism or direct impact

Anterior cruciate ligament i njury

on a hyperextended knee Pain at base of posterior foot reproducible with compression of the

Calca neal stress fracture

calcaneum Pain in the heel and sole of foot that worsens on prolonged weight

Plantar fasciitis

bearing Paresthesia in the sole of the foot and pain upon foot dorsiflexion and eversion

Tarsal tunnel syndrome

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X-ray fi ndings in bone tumor associated with Li-Fraumeni syndrome and familial retinoblastoma

Osteosarcoma; medullary and cortical bone destruction with sun­ bu rst appearance and Codman triangle

Septic arth ritis synovial fluid findings and empiric antibiotic treatment.

WBC count >S0,000/mm 3, PMN >90% and EEl Gram stain; ceftriaxone and vancomycin until cultu re test results

Adult with fever and loca lized bone pain with MRI showing bone

Osteomyelitis

marrow inflammation and soft tissue i nfection Joint pain and stiffness that worsen over the course of the day and are

Osteoarthritis

relieved by rest Joints in the hand affected in rheu matoid arthritis

MCP and PIP joints; DIP joints are spared

Arthritis, conjunctivitis, and u rethritis in young adu lts. Associated

Reactive arth ritis; most commonly associated with Chlamydia, also

organisms? Young woman presents with fever, malaise, malar rash and arthritis. Associated antibodies? Shoulder pain with wea k abduction and external rotation of the

consider Campylobacter, Shigella, Salmonella, and Ureaplasma Systemic lupus erythematosus; EEl ANA, a nti-dsDNA and anti-Smith antibodies Rotator cuff tear

humerus 55-year-old man has sudden, excruciating fi rst MTP joint pain after a night of drinking red wine. Diagnosis, work-up, and chronic treatment?

Gout; needle-shaped, negatively birefringent crystals are seen on joint fluid aspirate; chronic treatment with allopurinol or probenecid

Rhomboid-shaped, positively birefringent crystals on joint fluid aspirate

Calciu m pyrophosphate deposition disease

Name the organism: ■ Raw pork and skeletal muscle cysts

■ Osteomyelitis from a foot wound punctu re ■ Osteomyelitis i n a sickle cel l patient

Trichinella spiralis Pseudomonas Salmonella

NEUROLOGY Unilateral, severe periorbital headache with tea ring and conjunctiva! erythema

Cluster headache

Prophylactic treatment for migraine

Antihypertensives, antidepressants, anticonvulsants, dietary cha nges

Treatment of stroke if presentation within 3-4.5 hours?

Th rombolytics (tissue plasminogen activators tPA)

Most common pituitary tumor. Treatment?

Dopamine agonists (eg, bromocriptine, cabergoline)

55-year-old patient presents with acute "broken speech:' ■ Type of aphasia?

Broca aphasia

Most common cause of SAH

Trauma (second most com mon is berry aneurysm)

CSF fi ndings with SAH

i ICP, RBCs, xanthochromia

Lens-shaped hypedensity on CT head; patient has a lucid interval

Epidural hematoma; middle meningeal artery

■ Lobe? ■ Vascular distribution?

Frontal lobe Left MCA distribution

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Crescent-shaped hyperdensity on CT that does not cross the mid line

Subdural hematoma-bridging veins torn (seen in elderly and young children)

Albuminocytologic dissociation

Guillain-Ba rre syndrome (i protein i n CSF without a significant increase i n cell count)

Most common cause of brain neoplasm

Metastases, primary neoplasms are m uch less com mon

Most common primary sources of metastases to the brain

Lung, breast, skin (melanoma), kidney, GI tract

Most common cause of seizures in children (2-1 O years of age)

I nfection, febrile seizu res, trauma, idiopathic

Most common cause of seizures in young adults ( 1 8-35 years of age)

Trauma, alcohol withdrawal, brain tumor

Classic EEG finding of absence seizures?

3-per-second spike and wave discha rges

First-line medication for status epilepticus

IV benzodiazepine

Hearing loss in presbycusis

High frequency

Ring enhancing lesions in patients with AIDS

Toxoplasmosis, CNS lymphoma

Differences between myasthenia gravis and Lambert-Eaton myas­ thenic syndrome on nerve stimulation

Decremental response i n MG, I ncremental response in LEMS

Symptoms seen i n normal pressure hydrocephalus?

Gait ataxia (wobbly), urinary i ncontinence (wet), dementia (wacky)

Risk factors for intracranial hypertension?

Obesity, Tetracycline, growth hormone, excess vitamin A

Wernicke encephalopathy

Confusion, ophthalmoplegia, ataxia caused by a deficiency of thia­ mine (B 1 )

Most common causes of dementia

Alzheimer disease and vascular

Combined UMN and LMN disorder

ALS

Rigidity and stiffness with unilateral resting tremor and masked facies

Parkinson disease

Treatment for Parkinson disease

Levodopa/carbidopa

Treatment for Guillain-Barre syndrome

IVIG or plasmapheresis; avoid steroids

Rigidity and stiffness that progress to choreiform movements, accompanied by moodiness and altered behavior

Huntington disease; a utosomal dominant

Port-wine stain in the V, distribution as well as with i ntellectual dis­ ability, seizu res, and ipsilateral leptomeningeal angioma

Sturge-Weber syndrome; treat symptomatically; possible focal cere­ bral resection of affected lobe

Multiple cafe-au-lait spots on skin

Neurofibromatosis type 1

Hyperphagia, hypersexuality, hyperora lity, and hyperdocility

Kluver-Bucy syndrome (amygdala)

Name the orga nism: Meningitis in adults Meningitis in elderly ■ Meningoencephalitis in AIDS patients Causes of ring-enhancing brain lesions

Neisseria meningitidis

Streptococcus pneumoniae Cryptococcus neoformans

Abscess, toxoplasmosis, metastasis, lymphoma, AIDS, neurocysticercosis

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Causes of meningitis in neonates. Treatment?

GBS, E coli, Listeria; treat with ampicillin + cefotaxime or gentamicin

Causes of meningitis in infants. Treatment?

S pneumoniae, N meningitidis, H influenzae type B; treat with vanco­

mycin + cefotaxime

What must always be done before LP?

Check for i ICP; look for papilledema

CSF findings:

■ Low glucose, PMN predomina nce ■ Normal glucose, lymphocytic predomina nce ■ N u merous RBCs in serial CSF samples ■ Gamma globu l i n s

Bacterial mening itis Aseptic (vi ral) meningitis SAH MS

OBSTETRICS Screening time for GBS. l ntrapa rtum prophylaxis administered if EB

Rectovaginal swab at 36-38 weeks; IV penicillin

GBS Quadruple screening findings in Trisomy 2 1 gestation

..1- MSAFP, ..1- estriol, i inhibin A, i (3-hCG

I ntellectual disability, midfacia l hypoplasia, smooth philtrum, cardiac defects

Fetal alcohol syndrome

Fetal growth restriction, microcephaly, cleft palate, fingernail hypo­

Fetal hydantoin syndrome

plasia, coarse hair Chorioretinitis, hydrocephalus, diffuse intracranial calcifications, ring

Congenital toxoplasmosis

enhancing lesions in newborn Petechial rash, sensorineural hearing loss, periventricular calcifica­

Congenital CMV i nfection

tions in newborn Uterine bleeding at 2,000/mm', or > 1 0% bands

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Neutropenic nadir postchemotherapy

7-1 0 days

Characteristics of primary Lyme disease

Erythema migrans

Characteristics of secondary Lyme disease

Arthralgias, migratory polyarth ropathies, facial nerve palsy, myocarditis, third-degree heart block

Middle-aged man presents with acute-onset monoa rticular joint pain

Lyme disease, lxodes tick bite, doxycycline

and bilateral facial nerve palsy. What is the likely diagnosis, and how did he get it? Treatment? AI DS-defining ill nesses

Esophageal candidiasis, CMV retinitis, Kaposi sarcoma, CNS lym­ phoma, PML, toxoplasmosis, PCP, invasive cervical/a nal cancer, HIV encephalopathy

At what CD4+ cell count should Pneumocystis jirovecii pneumonia prophylaxis be i nitiated in an H IV patient? Mycobacterium avium

:S200 cells/mm 3 for P jirovecii (with TMP-SMX); :sS0-1 00 cells mm 3 for MAC (with clarith romycin/azith romycin)

ICU patient has fever and mild discomfort around central line. Culture

Candidemia may cause central line associated infection (~ 1 0%) and

complex (MAC) prophylaxis?

shows budding yeast. What is the likely cause of the fever? Most frequent cause of bloodstream i nfections in patients with i ntra-

should not be considered a contaminant Coagulase negative staphylococci

vascular devices? Most common organism in burn-related infections

Pseudomonas

Method of calculating fluid repletion in burn patients

Parkland formula: 24-hour fluid (ml) = 4 x kg x % BSA

Name the organism: ■ Dog or cat bite ■ I nfection in burn victims

Pasteurella multocida Pseudomonas

Class of drugs that may cause syndrome of muscle rigidity, hyper­ thermia, autonomic instability, and extrapyramidal symptoms

Antipsychotics (neuroleptic malignant syndrome)

Side effects of corticosteroids

Acute mania, immunosu ppression, thin skin, osteoporosis, easy bruising, myopathies

Treatment for delirium tremens

Benzodiazepi nes

Treatment for aceta minophen overdose

N-acetylcysteine

Treatment for opioid overdose

Naloxone

Treatment for benzodiazepine overdose

Flumazenil (monitor for withdrawal and seizures)

Treatment for neuroleptic malignant syndrome and malignant hyperthermia

Dantrolene

Causes of drug-induced SLE

I N H, penicillamine, hydralazine, procainamide, chlorpromazine, meth­ yldopa, quinidine

Burn patient presents with cherry-red, flushed skin and coma. Sao, is

Treat CO poisoning with 1 00% 02 or with hyperbaric 02 if poisoning

normal, but carboxyhemoglobin is elevated. Treatment? Macrocytic, megaloblastic anemia with neurologic symptoms

is severe or the patient is pregnant Vitamin B,, deficiency

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Macrocytic, megaloblastic anemia without neurologic symptoms

Folate deficiency

The following are associated with which malignancy: 1 . Acanthosis nigricans and seborrheic keratoses 2. AIDS

1 . Maligna ncy 2. Kaposi sarcoma and non-Hogkin lymphoma

3. Neurofi bromatosis type 1 4. Neurofi bromatosis type 2 5. Tuberous sclerosis

3. Pheochromocytoma, neurofibroma, optic glioma 4. Acoustic schwannoma 5. Astrocytoma, cardiac rhabdomyoma

Signs of cardiac tamponade

Distended neck veins, hypotension, diminished heart sounds (Beck triad), pulsus paradoxus

Absent breath sounds, dullness to percussion, shock, flat neck veins

Massive hemothorax

Absent breath sounds, tracheal deviation, shock, distended neck veins

Tension pneumothorax

Best next step in patient with recent neck surgery, expanding neck mass/deviated trachea, and airway compromise (noisy breathing)

Wound exploration/evacuation of hematoma

Blood in urethra l meatus or high-riding prostate

Bladder ruptu re or urethral injury

Test to rule out urethral injury

Retrograde cystourethrogram

Radiographic evidence of aortic disruption or dissection

Widened mediastinum (>8 cm), loss of aortic knob, pleural cap, tra­ cheal deviation to the right, depression of left main stem bronchus

Radiographic indications for surgery in patients with acute abdomen

Free air under the diaphragm, extravasation of contrast, severe bowel distention, space-occupying lesion (CT), mesenteric occlusion (angiography)

Treatment for blunt or penetrating abdominal trauma in a hemody­ namica lly unstable patient

Exploratory laparotomy

ICP in alcoholics or the elderly following head trauma. Can be acute or

Subdural hematoma

chronic. Crescent-shaped lesion on CT Head tra uma with immediate loss of consciousness followed by a lucid interval and then rapid deterioration. Convex-shaped lesion on CT

Epidural hematoma

SECTION 3

TOP- RATED REVI EW RESOU RCES "Some books are to be tasted, others to be swallowed, and some few to be chewed and digested. " - Sir Francis Bacon

"Always read something that will make you look good if you die in the middle of it."

-P.J. O'Rourke

"So many books, so little time." -Frank Zappa

"If one cannot enjoy reading a book over and over again, there is no use in reading it at all. "

-Oscar Wilde

"Start where you are. Use what you have. Do what you can." -Arthur Ashe

► How to Use the Database

792

► Com prehensive

794

► Question Banks

794

► I nternal Medicine, Emergency Medicine, 795 Family Medicine ► Neurology

795

► OB/GYN

795

► Pediatrics

796

► Psychiatry

796

► Surgery

796

► Commercial Review Cou rses

797

791

792

SECT I O N 3

TOP- RATED REVIEW RESOURCES

HOW TO USE THE DATABASE This section is a database recommended clinical science review resources, question banks, and other test preparation tools marketed to medical students studying shelf exams and the USMLE Step 2 CK For each resource, we list the Title, the First Author (or editor), the Current Publisher, the Copyri ght Year, the Edition, the Number of Pages, the ISBN, the Approximate List Price, the Format of the resource, and the Number of Test Questions. Finally, each resource receives a Ratin g. The resources are sorted into a com­ prehensive section as well as into sections corresponding to the six clinical disciplines (internal medicine, neurology, OB/GYN, pediatrics, psychiatry, and surgery). Within each section, resources are arranged first by Rating, then by Author, and finally by Title. For this edition of First Aid for the USMLE Step 2 CK, the database of review resources has been completely revised, with in-depth summary com­ ments on more than 100 books and online and mobile applications. A letter rating scale with six different grades reflects the detailed student evaluations. Each resource receives a rating as follows:

A+ A A-

Excellent for boards review Very good for boards review; choose among the group

B+ B

Good, but use only after exhausting better resources

B-

Fair, but there are many better resources in the discipline; or low­ yield subject material

The Ratin g is meant to reflect the overall usefulness of the resource in preparing for the USMLE Step 2 CK exam. This is based on a number of fac­ tors, including the following: ■ ■ ■ ■ ■ ■ ■

Cost of the resource Readability of the resource Appropriateness and accuracy of the resource Quality and number of sample questions Quality of written answers to sample questions Quality and appropriateness of the illustrations (eg, graphs, diagrams, photographs) Length of the text (longer is not necessarily better) Quality and number of other resources available in the same discipline Importance of the discipline on the USMLE Step 2 CK exam

Please note that the ratin g does not reflect the quality of the resource for pur poses other than reviewin g for the USMLE Ste p 2 CK exam. Many resources with low ratings are well written and informative but are not ideal for USMLE Step 2 CK preparation. We have also avoided listing or com­ menting on the wide variety of general textbooks available in the clinical sciences. Evaluations are based on the cumulative results of formal and informal surveys of hundreds of medical students from medical schools across the country. The summary comments and overall ratings represent a consensus

TOP-RATED REVIEW RESOURCES opinion, but there may have been a large range of opinions or limited student feedback on any particular resource. Please note that the data listed are sub­ ject to change. We actively encourage medical students and faculty to submit their opin­ ions and ratings of these clinical science review books so that we can update our database (see "How to Contribute," p. xi). In addition, we ask that pub­ lishers and authors submit review copies of clinical science review books, including new editions, and books not included in our database, for evalua­ tion. We also solicit reviews of new books or suggestions for alternate modes of study that may be useful in preparing for the exam, such as flash cards, tutorials, commercial review courses, online resources.

DISCLAIMER/CONFLICT -OF - INTEREST STATEMENT No material in this book, including the ratings, reflects the opinion or influ­ ence of the publisher. All errors and omissions will gladly be corrected if brought to the attention of the authors through our bloc at firstaidteam.com. Please note that USMLE-Rx and the entire First Aid for the USMLE series are publications by the senior authors of this book; their ratings are based solely on recommendations from the student authors of this book as well as data from the student survey and feedback forms.

S ECTI O N 3

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S E CT I O N 3

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Boards a n d Beyond

boardsbeyond.com

Review/Test

$ 24-$399

A

SketchyMedical Clinical

SketchyMedical

sketchy.com/explore/med ical-c l i n ical

Review

$ 300-$600

A· AMBOSS Medical Knowledge:

AM BOSS

am boss.com

Test

$ 8-$99

A·

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divi n e i ntervention pod casts.com

Podcast

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Fischer

Ka p l a n Test Prep, 6th ed., 744 pages, I S B N 978 1 506254586

Review

$ 60

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MediQ Lea rn ing

u s m le-rx.com/products/rx-bricks

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Lippi ncott Wi l l iams & Wi l ki n s, 5th ed., 592 pages, ISBN 978 1 975 1 036 1 3

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Lectu rio

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Lecturio

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Na heedy

McG raw H i l l Medical, 3 rd ed., 3 84 pages, I S B N 978 1 260464269

Review

$27

e+

USMLE Step 2 Secrets

O'Co n n e l l

El sevie r, 6th e d . , 352 pages, I S B N 9780323824347

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B

USMLE Step 2 Made Ridiculously Simple

Ca rl

MedMaster, 6th ed., 404 pages, I S B N 978 1 93566023 1

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$30

AUTHOR

P U B LISHER

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UWorld Step 2 CK Qbank

UWorld

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B

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Question Banks

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Internal Medicine, Emergency Medicine, Family Medicine AUTHOR

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McGraw-Hi l l Ed ucation, 2 0 1 7, 4th ed., 6 7 2 pages, I S B N 9781 259640827

Review

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A· First Aid for the Medicine Clerkship

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McGraw-H i l l, 202 1 , 4th e d . , 5 9 2 pages, ISBN 978 1 260460629

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A· Emergency Medicine: PreTest Self-

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McGraw-H i l l, 2 0 1 6, 1 4th ed., 5 1 2 pages, I S B N 978007 1 850056

Test/500 q

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A

Case Files: Emergency Medicine

Assessment & Review

e+

Step-Up to Medicine

Ag a begi

L i p p i n cott Wi l l i a m s & Wi l k i n s, 2 0 1 9, 5th ed., 592 pag es, I S B N 978 1 975 1 03 6 1 3

Review

$66

e+

Medical Secrets

H a rward

Elsevie r, 2 0 1 9, 6th ed., 592 pages, ISBN 9780323478724

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e+

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Toy

McGraw-H i l l, 2020, 5th ed., 752 pages, ISBN 978 1 260468595

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Toy

McGraw-H i l l. 2020, 6th ed., 688 pages, I S B N 9781 260469967

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B

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S m a l l igan

McGraw-H i l l, 2 0 1 6, 1 4th ed., 5 1 2 pages, I S B N 978-007 1 850056

Test/500 q

$35

AUTHOR

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A· Neurology: PreTest Self-Assessment &

Anschel

McGraw-H i l l, 2 0 1 7, 9th ed., 356 pages, ISBN 978-1 2595869 1 0

Test/500 q

$27

A·

Blueprints Neurology

Drislane

L i p p i n cott Wi l l ia m s & Wi l k i ns, 201 9, 5th ed , 296 pag es, ISBN 978 1 496387394

Review/ Test/ 1 00 q

$60

B

Neurology Secrets

Kass

E lsevier, 201 7, 6th ed., 552 pages, ISBN 978032335948 1

Review

$48

AUTHOR

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A· First Aid for the Obstetrics & Gynecology

Kaufm a n

McGraw-H i l l, 2 0 1 8, 4th ed., 384 pages, I S B N 978 1 25964406 1

Review

$43

e+

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Ca l l a h a n

L i p p i n cott Wi l l ia m s & Wi l k i n s, 2 0 1 8, 7th ed , 529 pag es, I S B N 978 1 975 1 34877

Review/ Test/ 1 50 q

$62

e+

Obstetrics and Gynecology: PreTest SelfAssessment & Review

Schneider

McGraw H i l l, 201 6, 1 4th ed , 3 58 pages, I S B N 978 1 25 9588723

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$42

e+

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McGraw-H i l l, 202 1 , 6th ed., 758 pages, I S B N 978 1 260468786

Review

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Neurology

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OB/GYN

Clerkship

796

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McGraw-H i l l, 2 0 1 7, 4th ed., 5 76 pages, ISBN 978 1 25 98343 1 8

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$55

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McGraw-H i l l, 202 1 , 6th ed., 640 pages, ISBN 978 1 260474954

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Yetman

McGraw-H i l l, 2020, 1 5th ed., 544 pages, I S B N 9781 26044033 1

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B

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Po l i n

El sevier, 202 1 , 7 t h ed., 688 pages, I S B N 9780323636650

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$51

AUTHO R

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Psychiatry

A

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Klamen

McGraw-H i l l, 202 1 , 1 5th ed., 320 pages, I S B N 9781 2604674 1 3

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McGraw-H i l l, 20 1 9, 5th ed., 240 pages, ISBN 978 1 260 1 43393

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M u rphy

Lippincott Wi l l i a m s & Wi l ki n s, 20 1 9, 6th ed , 240 pages, I S B N 978 1 49638 1 347

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McG raw-H i l l, 202 1 , 6th ed., 608 pages, I S B N 978 1 26046873 1

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McG raw-H i l l, 2 0 1 7, 1 1 th ed., 304 pages, I S B N 978 1 259643 94 1

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$50

AUTHO R

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Surgery A

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McG raw-H i l l, 2022, 6th ed , 688 pages, I S B N 9781 260468809

Review

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Pesta na

Ka p l a n , 202 1 , 6th ed , 264 pag es, ISBN 978- 1 5 06235 9 1 2

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McG raw-H i l l, 2 0 1 6, 3 rd ed., 5 1 2 pages, ISBN 978007 1 842099

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Blac kbo u rne

Lippincott Wi l l i a m s & Wi lki ns, 202 1 , 9th ed , 624 pages, I S B N 978 1 975 1 52949

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McG raw-H i l l, 2020, 1 4th ed., 336 pages, I S B N 978 1 260 1 436 1 4

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TOP-RATED REVIEW RESOURCES COMMERCIAL REVIEW COURSES Although commercial preparation courses can be helpful for some students, such courses are typically costly and require significant time commitment. They are usually most effective as an organizing tool for students who feel overwhelmed by the sheer volume of material involved in Step 2 CK prepara­ tion. Note, too, that multiweek courses may be quite intense and may thus leave limited time for independent study. Also note that some commercial courses are designed for first-time test takers, while others focus on students who are repeating the exam. In addition, some courses are geared toward IMGs who want to take all three Steps in a limited amount of time. Student experience and satisfaction with review courses are highly vari­ able. We suggest that you discuss options with recent graduates of the review courses you are considering. In addition, course content and structure can change rapidly. Some student opinions can be found in online discussion groups.

S ECTI O N 3

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798

SECT I O N 3

TOP- RATED REVIEW RESOURCES NOTES

AB BREVIATIONS AND SYM BOLS ABBREVIATION

M

A-a

AM AB Ab ABC ABC AB! ABPA AC ACE ACh AChR AChR-Ab ACL ACR ACTH AD ADA ADH ADLs ADP AEDs AF AF! AFP Ag ACCs AH AHA AHi Al All- IA AJN AION AJ S

MEANING

ABBREVIATION

MEANING

2 ° amyloidosis alveolar-arterial abdominal aortic aneurysm abortion

AMA

American Medical Association age-related macular degeneration acute myelogenous leukemia, acute myeloid leukemia

antibody

ANA ANC ANCA ANOVA

abacavir arterial blood gas ankle-brachia! index allergic bronchopulmonary aspergillosis abdominal circumference angiotensin-converting enzyme acetylcholine acetylcholine receptor acetylcholine receptor autoantibodies anterior cruciate ligament American College of Rheumatology adrenocorticotropic hormone Alzheimer dementia, Alzheimer disease American Diabetes Association antidiuretic hormone activities of daily living adenosine diphosphate automated external defibrillators atrial fibrillation amniotic fluid index a-fetoprotein antigen atypical glandular cells atypical hyperplasia American Heart Association apnea-hyp opnea index adrenal insufficiency autoimmune hemolytic anemia acute interstitial nephritis anterior ischemic optic neuropathy

AKI AL

androgen insensitivity syndrome acute kidney injury I O amyloidosis

ALC ALL ALS ALT ALTE

absolute lymphocyte count acute lymphocytic leukemia amyotrophic lateral sclerosis alanine aminotransferase apparent life-threatening event

Al\110 AML AMS

ANS anti-CCP anti-CTLA-4 anti-TPO APC APL APS aPTT AR ARB ARDS

ARNI AR.PKO ART AR.VD AS ASA

altered mental status antinuclear antibody absolute neutrophil count antineutrophil cytoplasmic antibody analysis of variance autonomic nervous system anticyclic citrullinated peptide anti-cytotoxic T-lymphocyte-associated antigen 4 antithyroid peroxidase activated protein C acute promyelocytic leukemia antiphospholipid syndrome activated partial thromboplastin time aortic regurgitation angiotensin receptor blocker acute respiratory distress syndrome angiotensin receptor neprilysin inhibitor autosomal recessive polycystic kidney disease antiretroviral therapy arrhythmogenic right ventricular dysplasia ankylosing spondylitis, aortic stenosis

aPTT

acetylsalicylic acid activated partial thromboplastin time

ASC-H

atypical squamous cells suspicious for high-grade dysplasia

ASC-US ASCVD

atypical squamous cells of undetermined significance atherosclerotic cardiovascular disease autism spectrum disorder, atrial septa] defect antistreptolysin 0 aspartate aminotransferase antithrombin

ASD ASO AST AT ATC ATLS ATN

antithymocyte globulin

ATRA

all-trans-retinoic acid abnormal uterine bleeding atrioventricular

AUB AV aVF aVL AVM AVN

dvanced trauma life support acute tubular necrosis

augmented vector foot augmented vector left arteriovenous malformation avascular necrosis

799

APPENDIX I

800

A B B R EVIATI O N S A N D SYM B OLS

ABBREVIATION

MEA N I N G

ABBREVIATION

MEANING

AVNRT aVR AVRT AZT

atrioventricular nodal reentry tachycardia augmented vector right

carpometacarpal (joint)

atrioventricular reentrant tachycardia zidovudine

CMC CML CMP CMR

BAC BBB

bronchioalveolar carcinoma blood-brain barrier

CMV CN

cytomegalovirus cranial nerve

BCC BCG

basal cell carcinoma

CNS CO2

central nervous system carbon dioxide cardiac output

�-hCG biPAP BMD BM! BMT BP BPD BPH bpm BPP BRUE BSA BSO BUN

bacillus Calmette-Guerin �-human chorionic gonadotropic bilevel positive airway pressure bone mineral density body mass index bone marrow transplantation blood pressure biparietal diameter benign prostatic h yp erplasia beats per minute biophysical profile brief resolved unexplained event body surface area bilateral salpingo-oophorectomy

CA

blood urea nitrogen birth weight CI inhibitor cancer antigen

CABG CAD

coronary artery bypass graft surgery coronary artery disease

CADherins CAH

Ca'•-dependent adhesion proteins congenital adrenal hyperplasia Confusion Assessment Method

BW C l INH

CAM CaNa 2EDTA CBC CBD CCB CCP CEA CEP CF CFTR CFU CGD cGMP CGRP CHADSVASc CHD CHF Cl CIN CJD CK C KD CLASB!s CLL

co COMT COPD cox CP CPAP CPPD CPR Cr CREEP CREST CRH CRL CRP CRVO CSA CSF

chronic myelogenous leukemia comprehensive metabolic panel cardiovascular magnetic resonance imaging

catechol-O-methyltransferase chronic obstructive pulmonary disease cyclooxygenase cerebral palsy continuous positive airway pressure calcium pyrophosphate deposition disease cardiopulmonary resuscitation creatine colitis, retinitis, esophagitis, encephalitis, pneumonitis calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia corticotropin-releasing hormone crown-rump length c-reactive protein central retinal vein occlusion central sleep apnea cerebrospinal fluid

CSM

css

carotid sinus massage cytokine storm syndrome

edetate calcium disodium

CST CTA CVA

complete blood cell count common bile duct calcium channel blocker cyclic citrullinated peptide

contraction stress test computed tomography angiography, CT angiography cerebrovascular accident, coslovertebral angle costovertebral angle tenderness

CVS CXR

carcinoemb ry onic antigen, carotid endarterectomy chronic eosinophilic pneumonia cystic fibrosis cystic fibrosis transmembrane conductance regulator colony forming unit chronic granulomatous disease cyclic guanosine monophosphate calcitonin gene-related peptide CHF, HTN, Age ?:. 7 5 , diabetes, stroke or TIA history, vascular disease, age 6 5-74, sex category congenital heart disease congestive heart failure confidence interval cervical intraepithelial neoplasia Creutzfeldt-Jakob disease creatine kinase chronic kidney disease central line-associated bloodstream infections chronic lymphocytic leukemia

CVAT CVID

CXVD D&C d4T D 5W

common variable immunodeficiency chorionic villus sampling x-ray of the chest cardiovascular disease dilation and curettage stavudine

DA DAAs DAI

dextrose 5% waler dopamine, emb ry onic age direct-acting antivirals diffuse axonal injury

DAP

diaminopyridine

DBP

diastolic blood pressure ductal carcinoma in situ dilated cardiomyopathy

DCIS DCM ddC DDD ddl DES DEXA DFA DH DHEA

zalcitabine depersonalization/derealization disorder didanosine diethylstilbestrol dual-energy x-ray absorptiometry direct fluorescent antibody dermatitis herpetiformis dehydroepiandrosterone

ABBREVIATIONS AND SYMBOLS

A P P E N D IX I

801

ABBREVIATION

MEANING

ABBREVIATION

MEANING

DHEAS OHR

dehydroepiandrosterone sulphate dihydrorhodamine

ETEC EtOH

enteropathogenic E coli ethanol

DI

diabetes insipidus disseminated intravascular coagulation distal interphalangeal (joint) diiodotyrosine

EUS FA FAB

endoscopic ultrasound fanconi anemia fragment antigen binding antibodies

FAP FAS

familial adenornatous polyposis fetal alcohol syndrome

FAST FDG-PET

focused abdominal sonography for trauma fluorodeoxyglucose -positron emission tomography FDPs fibrin degradation products

DIC DIP DIT OKA

diabetic ketoacidosis

DLB

dementia with Lewy bodies

DLCO DM

diffusing capacity of the lung dermatomyositis, diabetes mellitus; (may be type 1 [type 1 DM] or type 2 [type 2 DM]) disease-modifying antirheumatic drug

DMARD DMDD

disruptive mood dysregulation disorder

DNase

deoxyribonuclease

DNI DNR

do not intubate do not resuscitate

DOAC DPL

direct oral anticoagulant diagnostic peritoneal lavage

DPLD OPP DRE

diffuse parenchymal lung disease dipeptidyl peptidase

DRESS ds DTRs DTs

digital rectal exam

fractional excretion of sodium FEV l FFP FGR FHR Fio, FISH FIT FL FLAIR FNA FNH FOBT

DVT

drug reactions with eosinophilia and systemic symptoms double-stranded deep tendon reflexes delirium tremens deep venous thrombosis

eADA EBV

erythrocyte adenosine deaminase Epstein-Barr virus

FSH FTA-ABS

EC ECF

emergency contraception extracellular fluid

ECMO ECT ED EDTA

extracorporeal membrane oxygenation electroconvulsive therapy erectile dysfunction

FTC FTD FTT

EEG EF EFW

electroencephalography erythema multiforme

EGO eGFR EGPA EI-IEC ELISA EMC ENT Epi EPO EPSs ER ERCP ERV ESAs ESR ESRD ET

forced expiratory volume in I second fresh frozen plasma fetal growth restriction fetal heart rate fraction of inspired oxygen fluorescence in situ hybridization fecal imrnunochemical test femur length fluid-attenuated inversion recovery fine-needle aspiration focal nodular hyperplasia fecal occult blood test

FOOSH FPIAP

fall onto an outstretched hand food protein-induced allergic proctocolitis

FRC

functional residual capacity follicle-stimulating hormone fluorescent treponemal antibody absorption emtricitabine frontoternporal dementia failure to thrive

5-FU FUO FVC FXN

fluorouracil fever of unknown origin forced vital capacity frataxin

estimated fetal weight

G6P G6PD

gl ucose-6-phosphate glucose-6-phosphate dehydrogenase

esophagogastroduodenoscopy estimated glornerular filtration rate

GA GABA

gestational age garnrna-aminobutyric acid

eosinophilic granulornatosis with polyangiitis enterohemorrhagic Escherichia coli

GAD GALT

generalized anxiety disorder, glutamic acid decarboxylase galactose-1-phosphate uridyl transferase

enzyme-linked imrnunosorbent assay electromyography ears, nose, and throat epinephrine

GAS GBM

group A Streptococcus glioblastoma

CBS GCS

erythropoietin

G-CSF GERO GFAP GFR

group B streptococcus Glasgow Corna Scale granulocyte colony stimulating factor gastroesophageal reflux disease

ethylenediaminetetraacetic acid

extrapyrarnidal symptoms estrogen receptor endoscopic retrograde cholangiopancreatography expiratory reserve volume

GGT

erythropoiesis-stimulating agents erythrocyte sedimentation rate end-stage renal disease endotracheal

gamma-glutamyl transferase, garnma-glutamyl transpeptidase

GHB Cl CLP

gamrna-hydroxybutyric acid gastrointestinal

glial fibrillary acid protein glomerular filtration rate

gl ucagon-like peptide

802

A P P E N DIX I

ABBREVIATIONS AND SYMBOLS

ABBREVIATION

MEA N I N G

ABBREVIATION

MEANING

CLP !

GPA

granulomatosis with polyangiitis

I--ISV I--IAART 5 1-IT I- IT

herpes simplex virus

GM-CSF GnRH

glucagon-like peptide-I granulocyte-macrophage colony stimulating factor gonadotropin-releasing hormone

GTD

gestational trophoblastic disease glucose tolerance test genitourinary

CTI GU GVI--10 I- I H&E I--IAART I--IAV

graft-versus-host disease hemagglutinin, histamine hematoxylin and eosin highly active antiretroviral therapy hepatitis A virus

Hb HbA l c

hemoglobin hemoglobin A l e

I--!BsAg

hepatitis B surface antigens hepatitis B virus

I--IBV hCG HCM I--ICT I -l et I--ICV I- I D HDL I- ION HOV I--IER2

human chorionic gonadotropin hypertrophic cardiomyopathy hematopoietic cell transplantation hematocrit hepatitis C virus Huntington disease high-density lipoprotein hemolytic disease of the newborn hepatitis D virus

HES I--IEV I- IF

human epidermal growth factor 2 hypereosinophilic syndrome hepatitis E virus heart failure

HFmrEF HFpEF

heart failure with moderately reduced ejection fraction heart failure with preserved ejection fraction

I--!FrEF

heart failure with reduced ejection fraction hypoxanthine-guanine phosphoribosyltransferase

I--IGPRT I- I I- I S HI- IV 5-I--IIAA I- lib HIDA I--IIF HIPPA H IT I- IL I--ILA I--IMG-CoA I--IMWK I--INPCC I--IOCM HPV HR I--IRT HS H SIL I- ISP

hyperglycemic hyp erosmolar syndrome human herpes virus 5-hydroxyindoleacetic acid Haemophilus influenzae type b hydroxy iminodiacetic acid hypoxia-inducible factor Health Insurance Portability and Accountability Act heparin-induced thrombocytopenia Hodgkin lymphoma human leukocyte antigen 3-hydroxy-3-methylglutaryl coenzyme A-reductase inhibitor (statin) high-molecular-weight kininogen hereditary nonpolyposis colorectal cancer hypertrophic obstructive cardiomyopathy human papilloma virus hazard ratio, heart rate hormone replacement therapy hereditary spherocytosis high-grade squamous intraepithelial lesion I--l e noch-Schonlein purpura

I--ITLV

highly active antiretroviral therapy 5-hydrOA')'tryptamine hydroxytryptamine human T-cell lymphotropic virus

I--ITN

hypertension

HUS

hemolytic uremic syndrome

1

iodide iodine instrumental activities of daily living

,-

IADLs IBO !BI

inflammatory bowel disease, inflammatory bowel disorder invasive bacterial infection

JBS IBS-C IBS-0

irritable bowel syndrome JBS that is constipation predominant

IC !CA !CD !CF !CI-I

inspiratory capacity internal carotid artery implantable cardiac defibrillator intracellular fluid

!CI-ID ICP

International Classification of Headache Disorders

!CS ICSs ICU IE IFN IFN-a lgE !CF IgG IGRAs III- I IL LO IM !NH !NO INR !OP !PF IRV ltp IUD IUGR IUI IV IVC !VDU !VF !VIG )IA JPS

JRA

JBS that is diarrheal predominant

intracranial hemorrhage intracranial pressure intercostal space inhaled corticosteroids intensive care unit infective endocarditis interferon interferon-a immunoglobin E insulin-like growth factor immunoglobin G interferon gamma release assays idiopathic intracranial hypertension interleukin interstitial lung disease intramuscular isoniazid internuclear ophthalmoplegia International Normalized Ratio intraocular pressure idiopathic pulmonary fibrosis inspiratory reserve volume idiopathic thrombocytopenic purpura intrauterine device intrauterine growth restriction intrauterine insemination intravenous, intravenously inferior vena cava intravenous drug use in vitro fertilization intravenous immunoglobulin juvenile idiopathic arthritis juvenile polyposis syndrome j uvenile rheumatoid arthritis

ABBREVIATIONS AND SYMBOLS

ABBREVIATION JVD )VP

M EAN ING jugular venous distention

ABBREVIATION MDS MELD

MEANING myelodysplastic syndromes

MEN ! MENZA

multiple endocrine neoplasia type I

KOH

jugular venous pressure potassium hydroxide

KS KSHV L

Kaposi sarcoma Kaposi sarcoma-associated herpesvirus lumbar

MENZB

LAA

left atrial appendage left atrial enlargement lymphangioleiomyomatosis long-acting muscarinic antagonist

MGUS MI--IA-TP

LAE LAM LAMA LAP LBBB LBO LBP LCIS LCL LDH LEEP LES LFT LGV LH LIP LLQ LLSB LM LMN LMP LMWI--1 LP LQTS LR LSD LSIL LTOT

leukocyte alkaline phosphatase

metaiodobenzylguanidine (scan)

lactate dehydrogenase loop electrosurgical excision procedure lower esophageal sphincter

MMR MMSE MOA

measles, mumps, and rubella (vaccine) Mini-Mental State Examination mechanism of action

liver function test lymphogranuloma venereum

MoCA MODY

luteinizing hormone lymphoid interstitial pneumonia left lower quadrant

MoM 6-MP MPGN MPTP MR MR.A

Montreal Cognitive Assessment maturity-onset diabetes of the young multiple of the median

lower left sternal border lateral meniscus lower motor neuron last menstrual period low-molecular-weight heparin lumbar puncture long QT syndrome lactated Ringer's, likelihood ratio lysergic acid diethylamide low-grade squamous intraepithelial lesion long-term oxygen therapy

left ventricular ej ection fraction left ventricular hypertrophy

MAP

mean arterial pressure

MART MCA

maintenance and reliever therapy middle cerebral artery mean corpuscular hemoglobin mean corpuscular hemoglobin concentration medial collateral ligament

MCV MDD MDE

MIBG MIT

methylmalonic acid mycophenolate mofetil

left ventricular outflow tract obstruction monoclonal membrane attack complex, mycobacterium aviwn complex mucosa-associated lymphoid tissue monoamine oxidase

MCI- I MCI-IC MCL MCP

MI

monoclonal gammopathy of undetermined significance microhemagglutination assay-Treponema pallidum myocardial infarction

MMF

low back pain lobular carcinoma in situ lateral collateral ligament

LVOT M

MAO

MG

multiple endocrine neoplasia type ZA multiple endocrine neoplasia type ZB Middle East respiratory syndrome myasthenia gravis

monoiodotyrosine medial longitudinal fasciculus medial meniscus, multiple myeloma

left upper quadrant left ventricle, left ventricular

MALT

MERS

metacarpophalangeal (j oint) mean corpuscular volume maj or depressive disorder major depressive episode

MMA

803

model for end-stage liver disease

MLF MM

left bundle branch block large bowel obstruction

LUQ LV LVEF LVI--1

MAC

A P P EN D IX I

mercaptopurine membranoproliferative glomerulonephritis I-methyl-4-phenyl- I , 2 , 3 ,6- tetrahydropyridine magnetic resonance, mitral regurgitation magnetic resonance angiography

MRCP MR.SA

magnetic resonance cholangiopancreatography methicillin-resistant Staphylococcus aureus, methicillinresistant S aureus

MS MSAFP msec

multiple sclerosis maternal serum a-fetoprotein millisecond

MSM MTB mTOR

men who have sex with men

MTP

mycobacterium tuberculosis mechanistic target of rapamycin metatarsophalangeal (joint)

MUA

manual uterine aspiration

MuSK MVC MVP

muscle-specific kinase motor vehicle collision

N NMT NAFLD NAPLLR NASH

mitral valve prolapse neuraminidase nucleic acid amplification testing

NAT

nonalcoholic fatty liver disease nephritis-associated plasmin receptor nonalcoholic steatohepatitis nonaccidental trauma

NCCT NE

noncontrast computed tomography norepinephrine

NEC NEXU S

necrotizing enterocolitis National Emergency X-Radiography Utilization Study

NF

neurofibromatosis nasogastric non-Hodgkin lymphoma

NG NHL

804

A P P E N DIX I

ABBREVIATIONS AND SYMBOLS

ABBREVIATION

MEA N I N G

ABBREVIATION

MEANING

NIF

negative inspiratory force National Institutes of Health Stroke Scale natural killer (cell) N-methyl-D-aspartate

PCP

phencyclidine hydrochloride, Pnewnocystis carinii pneumonia

PCR

polymerase chain reaction proprotein convertase subtilisin/kexin type 9 polycythemia vera

NII-ISS NK NMDA NM) NMS NNT NOAC NPD NPH NPO NPPV

neuromuscular junction neuroleptic malignant syndrome number needed to treat novel oral anticoagulant nasal potential difference neutral protamine Hagedorn insulin, normal pressure hydrocephalus nil per os ( nothing by mouth) noninvasive positive-pressure ventilation

NPV NRDS NRT!s NS

negative predictive value neonatal respiratory distress syndrome nucleoside/nucleotide reverse transcriptase inhibitors normal saline

NNRTI

non-nucleoside reverse transcriptase inhibitor nonsteroidal anti-inflammatory drug

NSAID NSCLC NSGCT NST

non-small cell lung cancer nonseminomatous germ cell tumor

NTD NYI-IA

neural tube defect New York Heart Association

O&P OCD OCP

ova and parasite exam obsessive-compulsive disorder, osteochondritis dissecans oral contraceptive pill

OCPD ODD OE

obsessive-compulsive personality disorder

OGTT OH

nonstress test

oppositional defiant disorder oti tis e,d:erna oral glucose tolerance test

01

hydroxy obesity hypoventilation syndrome osteogenesis imperfecta

ONSF OR

optic nerve sheath fenestration odds ratio, operating room

01 ORIF

opportunistic infection open reduction and internal fixation

OSA

obstructive sleep apnea over the counter posteroanterior, pulmonary artery

OHS

OTC PA Paco, PAD PAH Pao, PAP PAPP-A PAS PBC PBF

PCSK9 PCV PCWP PD PDA PDD PDE PDE 5 PDGF PD-L l PEEP PET PF PFT PG PCB PGE 1 PG!, PH PICA PID PIP

pulmonary capillary wedge pressure Parkinson disease, programmed death patent ductus arteriosus Parkinson disease dementia, pervasive developmental disorder, premenstrual dysphoric disorder phosphodiesterase phosphodiesterase type 5 platelet-derived growth factor programmed death-ligand I positive end-expiratory pressure positron emission tomography platelet factor pulmonary function test prostaglandin porcelain gallbladder, porphobilinogen prostaglandin E 1 prostacyclin pulmonary hypertension posterior inferior cerebral artery pelvic inflammatory disease peak inspiratory pressure, proximal interphalangeal (joint)

PKU PLA PLCI--1

phenylketonuria phospholipase

PM PM! PMN PNET PNI-1

polymyositis point of maximal impulse

PO, PO POC POD POEM POLST PPD PPE

pulmonary Langerhans cell histiocytosis

polymorphonuclear (leukocyte) primitive neuroectodermal tumor parm,'Y smal nocturnal h emoglobinuria partial pressure of oxygen per os (by mouth, oral) products of conception postoperative day peroral endoscopic myotomy physician orders for life-sustaining treatment

Preak

purified protein derivative [ of tuberculin] personal protective equipment peak inspiratory pressure

pulmonary arterial hypertensin

PP!s

proton pump inhibitors

partial pressure of o,,'Y gen positive airway pressure

Prial

plateau pressure paramedian pontine reticular formation preterm primary rupture of membranes

partial pressure of carbon dioxide peripheral arterial disease

pregnancy-associated plasma protein A periodic acid-Schiff primary biliary cholangitis peripheral blood film

PC

pressure control

PCC PCL PCom

prothrombin complex concentrate posterior cruciate ligament posterior communicating

PCOS

polycystic ovarian syndrome

PPRF PPROM PPV PPV2 3 PR

positive predictive value, positive pressure ventilation 2 3-polyvalent pneumococcus vaccine

pRBC PrEP PT

per rectum, progesterone receptor, proteinase packed red blood cell preexposure prophylaxis prothrombin time

PCWP PROM

pulmonary capillary wedge pressure premature rupture of membranes

ABBREVIATIONS AND SYMBOLS

A P P E N D IX I

805

ABBREVIATION

MEANING

ABB REVIATION

MEANING

PS PSA

pronator syndrome

SARS SBFT

severe acute respiratory syndrome small bowel follow-through

SBO SBP

small bowel obstruction

SCD

subacute combined degeneration, sudden cardiac death

SCDs SCFE SCIO SD SOB

sequential compression socks slipped capital femoral epiphysis severe combined immunodeficiency

SERM

selective estrogen receptor modulator socioeconomic status

PSC PSGN PSP PT PTI--1 PTI--lrP PTSD

PTT

PTU PUD PVC PVR

prostate-specific antigen primary sclerosing cholangitis post-streptococcal glomerulonephritis progressive supranuclear palsy prothrombin time parathyroid hormone parathyroid hormone-related protein post-traumatic stress disorder partial thromboplastin time propylthiouracil peptic ulcer disease premature ventricular contraction peripheral vascular resistance

QTc RA RAAS RA! RAI U

QT interval corrected for extremes in heart rate rheumatoid arthritis

RAS RAST RBBB R-CI--IOP RCTs RDS ROW REM RERAs RF RI-ID RLQ

RNP

ROC ROM RPR RR rRNA RSV RTA RT-PCR RT-Qu!C RUQ RV RVH RVOT

s

SES

standard deviation sleep-disordered breathing Shwachman-Diamond syndrome

renin-angiotensin-aldosterone system radioactive iodine radioactive iodine uptake

SIBO SIMV

sodium-glucose transporter ( SGLT) sodium-glucose transporter 2 syndrome of inappropriate secretion of ADH small intestinal bacterial overgrowth synchronized intermittent mandatory ventilation

renal artery stenosis serum radio-allergosorbent test

SIRS

systemic inflammatory response syndrome

SJ S SLE

Stevens-Johnson syndrome systemic lupus erythematosus Smith

right bundle branch block rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone randomized controlled trials respiratory distress syndrome red cell distribution width rapid eye movement respiratory effort-related arousals rheumatoid factor rheumatic heart disease right lower quadrant ribonucleoprotein receiver operating characteristic range of motion, rupture of membranes rapid plasma reagin relative risk, risk ratio, respiratory rate ribosomal RNA respiratory syncytial virus renal tubular acidosis reverse transcription quantitative polymerase chain reaction real-time quaking-induced conversion right upper quadrant residual volume, right ventricle right ventricular hypertrophy right ventricular outflow tract

SMC SAB SABA SAH

short-acting �,-agonists subarachnoid hemorrhage

SAMA

short-acting muscarinic antagonist oxygen saturation

SaO,

sos

spontaneous bacterial peritonitis, systolic blood pressure squamous cell carcinoma

SGLT SGLT-2 SIADH

sacral sinoatrial serum-ascites albumin gradient spontaneous abortion

SA

sec

Sm SMA SNRI

spinal muscular atrophy, superior mesenteric artery serotonin-norepinephrine reuptake inhibitor

SNS SOB

sympathetic nervous system shortness of breath

SOFA sOsm SPF

sequential organ failure assessment serum osmolality

SSRI

sun protection factor saturation of peripheral oxygen somatostatin selective serotonin reuptake inhibitor

STD

staphylococcal scalded-skin syndrome sexually transmitted disease

STE ST!

ST elevation sexually transmitted infection

SVC

superior vena cava supraventricular tachycardia triiodothyronine

Spo,

ss

ssss

SVT

Ti T4

TACE TAH/BSO TAF TAPVR TAR TAVR TB TBG TB! TBW 3TC TCA TD

thyroxine transarterial chemoembolization total abdominal hysterectomy/bilateral salpingooophorectomy tenofovir alafenamide total anomalous pulmonary venous return thrombocytopenia absent radius transcatheter aortic valve replacement tuberculosis thyroxine-binding globulin traumatic brain injury total body water lamivudine tricyclic antidepressant tardive dyskinesia

806

A P P E N DIX I

ABBREVIATIONS AND SYMBOLS

ABBREVIATION

MEAN I N G

ABBREVIATION

MEANING

TDF TdT TEN TFT

tenofovir disoproxil fumarate

UAG

urine anion gap

terminal deoxynucleotidyl transferase toxic epidermal necrolysis

UC UFH UMN

ulcerative colitis unfractionated heparin

URI

upper respiratory infection

TGF Th cells THC THI TIA TIBC TIPS TLC TM TMP-SMX TNF TNM TOA TOF TORCH

tPA TPAL TP-EIA TPN TPO TP-PA TPR TR TRH TSC TSI-1 TS! TSS TSST- 1 TST TrE

thyroid function test transforming growth factor T-helper cells tetrahydrocannabinol transient hypogammaglobulinemia of infancy transient ischemic attack total iron-binding capacity transjugular intrahepatic portosystemic shunt total lung capacity tympanic membrane trimethoprim-sulfamethoxazole tumor necrosis factor tumor, node, metastasis (staging) tubo-ovarian abscess tetralogy of Fallot toxoplasmosis, other agents, rubella, cytomegalovirus, herpes simplex tissue plasminogen activator

us

USPSTF UT! UV V /Q VACTERL-H

vc VC UG VDRL VEGF

upper motor neuron ultrasound United States Preventive Services Task Force urinary tract infection ultraviolet ventilation/perfusion vertebral abnormalities, anal atresia, cardiac (heart) defects, tracheoesophageal fistula, esophageal atresia, renal (kidney) and radial abnormalities, limb abnormalities, hydrocephalus vital capacity, volume control voiding cystourethrogram Venereal Disease Research Laboratory vascular endothelial growth factor ventricular fibrillation

VF VGCC VIN

voltage-gated calcium channel vulvar intraepithelial neoplasia

YMA VMAT VMAT2

vanillylmandelic acid vesicular monoamine transporter vesicular monoamine transporter 2

voe VP YR VRSA

vaso-occlusive crisis ventriculoperitoneal

thyroid-stimulating hormone thyroid-stimulating immunoglobulin

VSD VT VTE VUR vWD

ventricular septa! defect ventricular tachycardia venous thromboembolism vesicoureteral reflux von Willebrand disease

toxic shock syndrome toxic shock syndrome toxin I

vWF

vzv

von Willebrand factor varicella zoster virus World Health Organization

(number of) term deliveries, preterrn deliveries, abortuses, and living children treponema pallidum enzyme immunoassay total parenteral nutrition thrombopoietin, thyroid peroxidase Treponema pallidum particle agglutination total peripheral resistance tricuspid regurgitation thyrotropin-rel easing hormone tuberous sclerosis complex

vascular resistance vancomycin-resistant S aureus

transthoracic echocardiogram

WHO WMA

World Medical Association Wolff-Parkinson-White

TXA,

thrombotic thrombocytopenic purpura transurethral resection of the prostate thromboxane A,

WPW XR ZDV

x-ray zidovudine

UA

urinalysis

TTP

TURP

tuberculin skin test

COMMON LABORATORY VALU ES * = Included in the Biochemical Profile (SMA- 1 2 ) Blood, Plasma, Serum

Reference Range

SI Reference Intervals

* Alanine aminotransferase (ALT, GPT at 30 C)

10-40 U/L

10-40 U/L

* Alkaline phosphatase

25-100 U/L

25-100 U/L

25-125 U/L

25-125 U/L

12-38 U/L

12-38 U/L

0.1-1.0 mg/dL // 0.0-0.3 mg/dL

2-17 µmol/L // 0-5 µmol/L

* Calcium, serum (Total)

8.4-10.2 mg/dL

2 . 1-2 .6 mmol/L

* Cholesterol, serum (Total)

Rec: < 200 mg/dL

< 5.2 mmol/L

* Creatinine, serum (Total)

0.6-1.2 mg/dL

53-106 µmol/L

Electrolytes, serum Sodium (Na+) Chloride (CJ-) * Potassium (K+) Bicarbonate (HCO 3 -) Magnesium (Mg2+)

1 36-146 mEq/L 95-105 mEq/L 3. 5-5.0 mEq/L 22-28 mEq/L 1.5-2 mEq/L

136-146 mmol/L 95-105 mmol/L 3. 5-5.0 mmol/L 22-28 mmol/L 0.75-1.0 mmol/L

75-105 mm Hg 33-45 mm Hg 7.35-7.45

10.0-14.0 kPa 4.4-5.9 kPa [H+ ] 36-44 nmol/L

Fasting: 70-100 mg/dL

3. 8-6. 1 mmol/L

Growth hormone - arginine stimulation

Fasting: < 5 ng/mL Provocative stimuli: > 7 ng/mL

< 5 µg/L > 7 µg/L

Osmolality, serum

275-295 mOsmol/kg HzO 3.0-4. 5 mg/dL

275-295 mOsmol/kg H 2 O 1 .0-1 . 5 mmol/L

Male: < 17 ng/mL Female: < 25 ng/mL

< 17 µg/L < 25 µg/L

6.0-7.8 g/dL 3. 5-5. 5 g/dL 2.3-3. 5 g/dL

60-78 g/L 35-55 g/L 23-35 g/L

0.4-4.0 µU/mL

0.4-4.0 mlU/L

* Urea nitrogen, serum (BUN)

7-18 mg/dL

25-64 nmol/L

* Uric acid, serum

3.0-8.2 mg/dL

0.18-0.48 mmol/L

°

Amylase, serum °

* Aspartate aminotransferase (AST, GOT at 30 C) Bilirubin, serum (adult) Total // Direct

Gases, arterial blood (room air) Po, Pco, pH * Glucose, serum

* Phosphorus (inorganic), serum Prolactin, serum (hPRL) * Proteins, serum Total (recumbent) Albumin Globulins Thyroid-stimulating hormone, serum or plasma

807

808

APPENDIX II

COM M O N LABO RATO RY VA LU ES

Cerebrospinal Fluid

Reference Range

SI Reference Intervals

Cell count

0-5/mm 3

0-5 x 10 6/L

Glucose

40-70 mg/dL

2 .2-3.9 mmol/L

Proteins, total

< 40 mg/dL

< 0.40 g/L

Erythrocyte count

Male: 4.3-5.9 million/mm 3 Female: 3. 5-5. 5 million/mm 3

4.3-5.9 x 10 1 2 /L 3. 5-5. 5 x 10 1 2 /L

Erythrocyte sedimentation rate (Westergen)

Male: 0-1 5 mm/hr Female: 0-20 mm/hr

0-1 5 mm/hr 0-20 mm/hr

Hematocrit

Male: 41-5 3% Female: 36-46%

0.41-0. 5 3 0.36-0.46

Hemoglobin, blood

Male: 13. 5-17. 5 g/dL Female: 12 .0-16.0 g/dL

1 35-175 g/L 120-160 g/L

Hemoglobin, plasma

< 4 mg/dL

< 0.62 µmol/L

Leukocyte count and differential Leukocyte count Segmented neutrophils Band forms Eosinophils Basophils Lymphocytes Monocytes

4,500-l l ,000/mm 3 54-62% 3-5% 1-3% 0-0.75% 25-33% 3-7%

4. 5-1 1.0 x 10 9/L 0. 54-0.62 0.03-0.05 0.01-0.03 0-0.0075 0.25-0.33 0.03-0.07

Mean corpuscular hemoglobin

25-35 pg/cell

0.39-0.54 fmol/cell

Mean corpuscular hemoglobin concentration

3 1%-36% Hb/cell

4.8-5.6 mmol Hb/L

Hematologic

µm 3

Mean corpuscular volume

80-100

Partial thromboplastin time (activated)

25-40 sec

25-40 sec

Platelet count

l 50,000-400,000/mm 3

150-400 x 10 9/L

Prothrombin time

1 1-15 sec

1 1-15 sec

Reticulocyte count

0.5-1 . 5% of RBCs

0.005-0.01 5

Creatinine clearance

Male: 97-137 mL/min Female: 88-128 mL/min

97-137 mL/min 88-128 mL/min

Osmolality

50-1200 mOsmol/kg H 2 0

50-1200 mOsmol/kg HzO

Proteins, total

< 1 5 0 mg/24 hr

< 0. 1 5 g/24 hr

Adult: 19-25 kg/m 2

19-25 kg/m 2

80-100 fL

Urine

Other

Body mass index

I N DEX Note: Page numbers followed by f and I indicate figures and tables, respectively.

A Abciximab, 2721 Abdomen, acute, 226-228, 2261, 228{ Abdominal aortic aneurysm (AAA), 7 5-76, 7 5{,

76{

ruptured, 226 screening, 1 7 51 Abdominal trauma blunt and deceleration, 762-763, 7621, 763{ penetrating, 756 ABGs. See Arterial blood gases (ABGs) ABI (ankle-brachia! index), 8 1 Abnormal uterine bleeding (AUB), 476-478, 477{, 4781 Abortion, 436-438, 4371, 4381 complete, 4371 complications, 4 38 doctor-patient professional relationship, 1 86 elective, 4 38, 4 381 incomplete, 4371 inevitable, 4371 missed, 4371 septic, 4371, 438 spontaneous, 436-438, 4371 threatened, 4371 ABPA (allergic bronchopulmonary aspergillosis), 299-300, 630, 636, 654 Abscess anal, 2401 appendiceal, 2 3 1{ Bartholin duct, 487 brain, 383-384, 3 8 3{ breast, 46 5-466 hepatic, 263-264, 263{ paravalvular, 69 peritonsillar, 5 51 I, 662 psoas, 230-2 3 1 pyelonephritis, 709 retropharyngeal, 5 5 1 /, 662 Absence seizures, 374, 3 7 5 , 3 7 5{ Absolute lymphocyte count (ALC ), 298 Absolute neutrophil count (ANC), 296-297 Absolute risk, 1 60 Absolute risk reduction (ARR), 1 60 Abstinence, alcohol use disorder, 6 1 3 Abstinence syndrome, neonatal, 527-528, 6 1 4 Acanthosis nigricans, 1 1 4, 1 1 4{ neoplasms, 7 5 1 1 Acarbose, diabetes, 1 281 ACC (adenoid cystic carcinoma), 2031 Accelerations, fetal heart rate, 4 5 5 ACEis. See Angiotensin-converting enzyme inhibitors (ACEls) Acetaldehyde dehydrogenase inhibitor, 6 1 3 Acetaminophen, toxic ingestion/overdose, 7441

Acetylcholine receptor autoantibodies (AChR­ Ab ), 3 84 Acetylsalicylic acid (ASA) congestive heart failure, 39 overdose, 682 stroke, 367 Achalasia, 208-209, 208{ Achilles reflex, 3 581 Achilles tendon rupture, 3 1 81 AChR-Ab (acetylcholine receptor autoantibodies), 384 Acid, toxic ingestion, 7441 Acid-base disorders, 682-684, 683{, 6831 Acidosis metabolic, 682, 68 3f, 68 3t renal tubular, 684, 6851 respiratory, 683{, 683/ ACL (anterior cruciate ligament) injury, 3 1 71, 3 19{ Acne, neonatal, 5281 Acne vulgaris, 1 06-1 07 Acoustic neuroma, 4051, 407 Acquired immunodeficiency syndrome (AIDS), 728. See also Human immunodefi­ ciency virus (HIV) Acral lentiginous melanoma, 1 201 Acromegalic cardiomyopathy, 451 Acromegaly, 1 44- 1 46, 1 4 5{, 1 46{ ACTH (adrenocorticotropic hormone) deficiency, 14 31 Acting out, 609/ Actinic keratosis, 1 1 8, 1 1 9{ Action stage of change, 1 8 3f, 1 8 31 Action tremor, 4021 Activated protein C (APC) resistance, 278 Active error, 1 841 Activities of daily living (ADLs) , 588 Acute abdomen, 226-228, 2261, 228{ Acute appendicitis, 230-2 3 1 , 2 3 1{ Acute chest syndrome, 568, 568{ Acute coronary syndromes, 49- 5 5 carotid artery stenosis, 5 5 ST-segment elevation myocardial infarction, 50{, 5 1-54, 52{, 5 3{, 541 unstable angina/non-ST-segment elevation myocardial infarction, 49-5 1 , 50{ Acute fatty liver, pregnancy, 445 Acute hepatic necrosis, 2 56-2 57 Acute intermittent porphyria, 2941 Acute kidney injury (AKI), 684, 6851-6861 Acute leukemias, 300-3 0 1 , 300{, 301{, 303 children, 569-570 Acute liver failure, 2 5 6-2 57 Acute lymphocytic leukemia (ALL), 300-3 0 1 , 30 If, 303 children, 569-570 Acute mesenteric ischemia, 227 Acute mountain sickness, 740 Acute myelogenous leukemia (AML), 300-30 1 , 300{, 3 0 1 {, 303 children, 569-570

Acute necrotizing mediastinitis, 662 Acute non-suppurative sialadenitis, 2021 Acute otitis media, 42 3 Acute peripheral vestibulopathy, 378 Acute renal failure, 684, 6851-686/ Acute respiratory distress syndrome (ARDS), 638-639, 638{ Acute respiratory failure, 637-642 acute respiratory distress syndrome, 638-639, 638{ coronavirus and COYID- 1 9, 64 1-642, 642{ hypoxemia, 637-638, 637{, 638/ mechanical ventilation, 639-64 1 , 6401-64 21 Acute suppurative sialadenitis, 2021 Acute transplant rejection, 309-3 1 0, 31 Ot Acyanotic left-to-right shunts, 528, 529-5 3 2 Acyclovir, adverse effects, 7471 AD (Alzheimer disease), 390-392, 3901 ADAMTS- 1 3 deficiency, 28 1-283, 282{, 2831, 284{ Adaptive functioning deficits, 588 Adenocarcinoma esophageal, 2 1 1 gastric, 2 1 6, 2 1 6{ lung, 6481, 649{ pancreatic, 267 Adenoid(s), 672 Adenoid cystic carcinoma (ACC), 203/ Adenoid h ypertrophy, 672 Adenoma(s) hepatic (hepatocellular), 260, 263 pituitary, 4051 growth hormone-secreting, 1 44-1 46, 145{ prolactin-secreting, 146- 1 47, 147{ pleomorphic, salivary glands, 203/ Adenomatous polyps, 2361, 2 371 Adenomyosis, 474-47 5, 4751 Adenosis, sclerosing, 493 Adenotonsillar hypertrophy, 672 Adenovirus, conjunctivitis, 4 1 51 ADH (antidiuretic hormone) deficiency, 142, 1431, 1 44, 1 441 resistance, 142, 1 44, 1 441 syndrome of inappropriate secretion, 14 7 ADHD (attention-deficit/hyp eractivity disorder), 586 Adhesions, small bowel obstruction, 227 Adhesive capsulitis, 3 39 Adjustable gastric band, 2 1 9 Adjustment disorder, 6021, 60 5 ADLs (activities of daily living), 588 Adnexal mass, 504, 5041 Adolescent psychiatric disorders, 586-590 attention-deficit/hyperactivity disorder, 586 autism spectrum disorder, 587 diagnostic criteria by symptom duration,

607{

disruptive behavioral disorders, 587-588 intellectual developmental disorder/intellec­ tual disability, 588

INDEX Adolescent psychiatric disorders (Continued) separation anxiety disorder, 589-590 Tourette syndrome, 589 ADPKD (autosomal dominant polycystic kidney disease), 697-698, 697{ Adrenal crisis, acute, 1 49 Adrenal gland, anatomy, regulatory control, and secretory products, 1 48, 1 48{ Adrenal gland disorders, 148- 1 54 adrenal anatomy, regulatory control, and secretory products, 1 48, 1 48{ adrenal insufficiency, 1 48-1 49, 1 49(, l 49t congenital adrenal hyperplasia, 1 54, l 54t Cushing syndrome, 1 50-1 5 1 , 1 5 1(, 1 52(, l 5 3t hyp eraldosteronism, 1 5 3 pheochromocytoma, 1 50, 1 50{ Adrenal hyperplasia, congenital, 1 54, l 54t, 48 1 -483, 482(, 483t precocious puberty, 4 70 primary amenorrhea/delayed puberty, 47 1 Adrenal insufficiency, 148- 1 49, 1 49(, 1 49t Adrenal tumor, precocious puberty, 470 Adrenarche, 5 1 5{ Adrenocorticotropic hormone (ACTH) deficiency, 14 3t Adult(s), recommended vaccinations, 1 72{ Adult T-cell lymphoma, 305t Advance directives, 1 89 Advanced trauma life support (ATLS) algo­ rithm, 7 5 1 primary survey, 7 5 2-7 5 3 , 7 5 2(, 7 52t, 7 5 3{ secondary survey, 7 5 3-7 54 Adverse events, 1 84 AF. See Atrial fibrillation (AF) Affective disorders. See Mood disorders AF! (amniotic fluid index), 4 5 3 Agammaglobulinemia, Bruton, 543t, 545 Agenda, interview, 1 79 Age-related macular degeneration (AMO), 4 1 2(, 4 1 8-4 1 9, 4 1 9{ Age-related skin changes, 1 1 7 Agglutinins, cold vs. warm, 292, 308 Aging vs. dementia, 388, 389t sexual changes, 6 1 6 Agoraphobia, 596 AH (atypical h yp erplasia), 494 AIDS (acquired immunodeficiency syndrome), 728. See also Human immunodefi­ ciency virus (HIV) AIHA (autoimmune hemolytic anemia), 292, 292{ infectious mononucleosis, 72 5 AION (anterior ischemic optic neuropathy), 337 Airway advanced trauma life support, 7 5 2 emergency, 7 5 2 Airway pressure/time curve, 640t AIS (androgen insensitivity syndrome), 472(, 5 14 Akathisia, 593t AKI (acute kidney injury), 684, 685t-686t Akinesia, Parkinson disease, 397

Alanine aminotransferase (alanine transaminase, ALT), 3 3 3 hepatitis, 2 5 0 ALC (absolute lymphocyte count), 298 Alcohol pregnancy, 4 34t prenatal exposure, 529t Alcohol use disorder, 61 l t, 6 1 3, 6 1 3(, 6 1 3t, 6 1 4t Alcoholic hallucinosis, 6 1 3, 6 1 3t Alcoholic hepatitis, 2 50, 2 5 7 Alcoholism, vaccines, 1 7 3{ Aldolase, 3 3 3 Aldosterone, 1 48{ excess, 1 5 3 Alkali, toxic ingestion, 744t Alkalosis metabolic, 683(, 683t respiratory, 68 3(, 68 3t ALL (acute lymphocytic leukemia), 300-3 0 1 , 3 0 1 (, 3 0 3 children, 569-570 Allergic bronchopulmonary aspergillosis (ABPA), 299-300, 630, 636, 654 Allergic conjunctivitis, 4 1 4 Allergic proctocolitis, food protein-induced, 5 3 8-539 Allergic reaction, blood transfusion, 296t Allergic rhinitis, 669 Allergic skin disorders, 89-98 atopic dermatitis (eczema), 90-9 1 , 90(, 9 1 { bullous pemphigoid/pemphigus vulgaris, 96, 97(, 97t contact dermatitis, 9 I, 9 lf drug eruption, 94, 94{ erythema multiforme, 95, 9 5{ erythema nodosum, 96, 96{ hyp ersensitivity reactions, 89, 89t, 90{ nummular eczema, 98, 98{ psoriasis, 92-93, 93{ pyoderma gangrenosum, 98, 98{ seborrheic dermatitis, 9 1 -92, 92{ Stevens-Johnson syndrome/toxic epidermal necrolysis, 9 5-96, 9 5{ urticaria (hives), 93-94, 94{ Allis sign, 5 7 5 Allogeneic transplantation, 309 a (type I error), 168 a 1 -antagonists, h yp ertension, 62t et i-agonists, hyp ertension, 62t a-fetoprotein maternal serum, 432, 432t ovarian tumor, 505t Alport syndrome, 69 l t ALS (amyotrophic lateral sclerosis), 3 59t, 398, 399-400 ALT (alanine aminotransferase), 3 3 3 hepatitis, 2 5 0 ALTE (apparent life-threatening event), 579 Alteplase, 367 Altitude sickness, 740 Altruism, 609t Alzheimer disease (AD), 390-392, 390t Amantadine, adverse effects, 747t

Amblyopia, 580 AMO (age-related macular degeneration), 4 1 2(, 4 1 8-4 1 9, 4 1 9{ Amelanotic melanoma, 1 20t Amenorrhea primary, 470-47 1 , 472t secondary, 47 1-474, 473{ Amikacin, adverse effects, 747t Aminocaproic acid, 277 Aminoglycosides, adverse effects, 747t Amiodarone, adverse effects, 747t AML (acute myelogenous leukemia), 300-3 0 1 , 300(, 3 0 1 (, 3 0 3 children, 569-570 Amnesia, dissociative, 593, 594t Amniocentesis, 4 3 3 , 4 3 3 t Amniotic fluid index (AF!), 4 5 3 Amniotic fluid volume, 4 5 3t Amphetamines abuse, 61 l t pregnancy, 4 34t withdrawal, 6 1 4t Amphotericin, adverse effects, 747t Amputation, pain after, 7 5 7 Amyloidosis, 308-309, 309t cardiomyopathy due to, 44t renal, 694t Amyotrophic lateral sclerosis (ALS), 3 59t, 398, 399-400 ANA antibody, 3 3 3t Anal abscess, 240t Anal fissure, 240t Anal wink reflex, 3 58t Analgesia, obstetric, 45 5-456 Analysis of variance (ANOVA), 1 69 Anaphylactic hypersensitivity reaction, 89t blood transfusion, 296t Anaphylactic shock, 7 l 8t Anaplastic thyroid carcinoma, l 3 7t ANC (absolute neutrophil count), 296-297 Androgen(s), pregnancy, 434t Androgen insensitivity, complete, 47 1 Androgen insensitivity syndrome (AIS), 472(, 5 14 Anemia(s), 28 5-294 aplastic, 289-290 autoimmune hemolytic, 292, 292{ infectious mononucleosis, 72 5 of chronic inflammation/disease, 286t, 287 classification, 285, 285t Cooley, 289t Diamond-Blackfan, 564-565, 564t end-stage renal disease, 287 Fanconi, 289-290, 564t, 565-566, 565t due to G6PD deficiency, 291 hemolytic, normocytic, 290-292, 290(, 292{ hereditary spherocytosis, 290(, 291-292 iron-deficiency, 2 36, 286-287, 286(, 286t, 288 due to lead poisoning, 287 megaloblastic, macrocytic, 293-294, 293{ microcytic, 286-289, 286(-288(, 286t, 289t nonhemolytic, normocytic, 289-290

INDEX paroxysmal nocturnal hemoglobinuria, 291 pernicious (vitamin B 1 2 deficiency, folate deficiency), 293-294, 293f neoplasms, 75 I t type A gastritis, 2 1 4-2 1 5 sickle cell disease, 567-569, 568(, 569f sideroblastic, 287, 287f thalassemias, 286t, 288, 288t, 289t Anesthesia, obstetric, 45 5-456 Aneurysm(s) aortic, 7 5-76, 7 5(, 76f intracranial, 3 54 ruptured saccular (berry), 368 Angina "intestinal," 229 Ludwig, 662 Prinzmetal (variant), 49 stable, 47-49, 48(, 50f unstable, 49-5 1 , 50f Angina pectoris, 47-49, 48(, 50f Angioedema, hereditary, 545t Angiofibromas, 407, 407f Angioma(s) cherry, 1 22, 1 22f tufted, 567 Angiomatosis, bacillary, 7 30t Angiotensin receptor blockers (ARBs) congestive heart failure, 39 hyp ertension, 6 l t Angiotensin receptor-neprilysin inhibitors (AR­ Nls), congestive heart failure, 39 Angiotensin-converting enzyme inhibitors (ACE!s) congestive heart failure, 39 hypertension, 6 l t pregnancy, 4 34t adverse effects, 747t Anion gap, urine, 682, 683-684 Ankle common adult orthopedic injuries, 3 1 St zones, 3 I 9, 3 l 9f Ankle fracture, 3 I St Ankle pain, 3 1 9, 3 1 9f Ankle-brachia! index (AB!), 8 1 Ankylosing spondylitis (AS), 3 30-3 32, 3 3 lf Anopia, 4 l 2f Anorectal disease, 240t, 241 Anorectal fistula, 240t Anorexia nervosa, 6 1 4-6 1 5, 6 1 5t ANOVA (analysis of variance), 1 69 Anovulatory bleeding, 477 Anovulatory problem, 472t Anserine bursitis, 345-346 Antepartum fetal surveillance, 4 5 1 -454, 452(, 452t, 453t Antepartum hemorrhage, 446-448, 447t, 448f Anterior cerebral artery, 3 54, 3 54(, 3 5 5f stroke, 365t Anterior communicating artery, 3 5 5f Anterior cord syndrome, 360t Anterior corticospinal tract, 36 If Anterior cruciate ligament (ACL) injury, 3 l 7t, 3 1 9f Anterior duodenal ulcers, 2 1 7 Anterior inferior cerebellar artery, 3 5 5f

Anterior ischemic optic neuropathy (AION), 3 37 Anterior spinal artery, complete occlusion, 360t Anterior spinothalamic tract, 36 If Anterior wall myocardial infarction, 52, 5 3f Anthrax, 660, 660f Anti-beta-2-glycoprotein, 279, 280 Antibiotic prophylaxis, endocarditis, 7 l t, 72 Antibodies, autoimmune diseases, 3 3 3t Anticardiolipin, 279, 280 Anti-CCP antibody, 3 3 3t Anticentromere antibody, 3 3 3t Anticholinergic drugs, toxic ingestion, 744t Anticholinergic adverse effects, 399 Anticholinesterase drug toxicity, 3 8 5 , 744t Anticipatory guidance, 579 Anticoagulation atrial fibrillation, 32, 3 3 deep venous thrombosis, 79, 80 Anti-D immunoglobulin, 285 Antidepressants, 603, 604t atypical, 605t tricyclic, 603, 604t adverse effects, 749t toxic ingestion/overdose, 745t Antidiuretic hormone (ADH) defi c iency, 1 42, 143t, 1 44, 1 44t resistance, 1 42, 1 44, 1 44t syndrome of inappropriate secretion, 1 47 Antidopaminergic agents, Tourette syndrome, 589 Anti-dsDNA antibody, 3 3 3t Antifibrinolytic agents, 277 Antifreeze, toxic ingestion, 745t Antigenic drift, 652 Antigenic shift, 652 Anti-glomerular basement membrane (antiGBM) disease, 3 3 7(, 690t-69 l t Antihistamines, adverse effects, 7 47t Anti-histone antibody, 3 3 3t Antihypertensive agents, 60t-62t Anti-lgE, asthma, 628t Anti-lL-4R, asthma, 628t Anti-lL-5, asthma, 628t Anti-lL-5 R, asthma, 628t Anti-Jo-I antibody, 3 3 3t Anti-La antibody, 3 3 3t Antileukotrienes, asthma, 628t Antimalarial drugs, 722 Antimuscarinic agents, toxic ingestion, 744t Antiphospholipid syndrome (APS), 279-280 Antiplatelet drugs chronic stable angina, 49 STEM!, 5 3 unstable angina/NSTEMI, 50 Anti psychotic medications, 59 I, 592t, 593t adverse effects, 747t Antiretroviral therapy (ART), HIV, 7 3 1 , 732(, 7 32t-73 3t Anti-RNA polymerase lll antibody, 3 3 5 Anti-Ro/anti-La antibody, 3 3 3 t Anti-Sci-70 antibody, 3 3 3t Anti-Sm antibody, 3 3 3t Anti-smooth muscle antibody, 3 3 3t Antisocial personality disorder, 588, 608t

Antitropoisomerase- 1 antibody, 3 3 3t n 1 -Antitrypsin deficiency, 630 Anxiety angina pectoris due to, 48 separation, 589-590 Anxiety disorders, 594-597 diagnostic criteria by symptom duration,

607t

generalized, 594-595, 595t panic disorder, 59 5-596 phobias, 596-597 Anxiolytic medications, 595t Aortic aneurysm, 7 5-76, 7 5(, 76f Aortic coarctation, 5 3 1-532, 5 32f Aortic disruption, 762, 762f Aortic dissection, 76-78, 77f angina pectoris due to, 48 Aortic insufficiency, 73t Aortic regurgitation (AR), 2 3(, 73t Aortic stenosis (AS), 2 3(, 72t APC (activated protein C) resistance, 278 Apgar scoring, 520, 5 2 l t Aphasia, 372-373 Broca/expressive, 372-373, 373f Wernicke/receptive, 373, 3 7 3f Aphthous stomatitis, recurrent, l 99t Apixaban, 272t Aplastic anemia, 289-290 Aplastic crisis, sickle cell disease, 568, 569 Apnea of prematurity, 526 Apology, rapport, l 80t Apoplexy, pituitary, 1 4 1 Apparent life-threatening event (ALTE), 579 Appendiceal abscess, 2 3 If Appendicitis acute, 230-2 3 1 , 2 3 lf perforation, 2 3 1 uncomplicated, 2 3 I Appendicolith, 2 3 lf APS (antiphospholipid syndrome), 279-280 AR (aortic regurgitation), 2 3(, 73t Arachnoid granulation, 3 5 5 Arachnoid mater, 3 5 5, 3 5 5f Arboviruses, encephalitis, 382 ARBs (angiotensin receptor blockers) congestive heart failure, 39 Arcuate fasciculus, 3 5 3f ARDS (acute respiratory distress syndrome), 638-639, 638f Argatroban, 272t Argyll Robertson pupil, 71 3 ARN!s (angiotensin receptor-neprilysin inhibi­ tors), congestive heart failure, 39 Aromatase deficiency, congenital, 1 54 ARPKD (autosomal recessive polycystic kidney disease) , 697-698 ARR (absolute risk reduction), 1 60 Arrhythmias, 2 5-34 bradyarrhythmias and conduction abnormalities, 2 5 , 26t-27t management, 3 3t, 34, 34t after STEM!, 54 tachyarrhythmias, 2 5-33, 2 5(, 28t-32t, 30f Arrhythmogenic right ventricular dysplasia (ARVD), 42-43, 43f

INDEX Arsenic, toxic ingestion, 744t, 745t ART (antiretroviral therapy), HIV, 7 3 1 , 7 3 2(. 7321-733t Arterial blood gases (ABGs) asthma, 627 hypoxemia, 637 pulmonary thromboembolism, 644 ventilator settings, 639, 642t Arteriolosclerosis, hyaline, 58, 59( Arteriovenous malformation (AVM), 368 Arteritis giant cell (temporal), 3 36-3 37, 3 3 7( granulomatous, 3 37( necrotizing, 3 3 7( Takayasu, 3 3 7-3 3 8 Arthritis juvenile idiopathic, 547-548, 5471 osteo-, 327-329, 328t, 329( psoriatic, 93, 3 3 1 , 3 3 1( juvenile, 5471 reactive, 3 3 I rheumatoid, 328t, 329-3 30, 3 3 6 juvenile, 547-548, 547t septic, 3 2 5 , 32 51, 326{, 326t ARVD (arrhythmogenic right ventricular dysplasia), 42-43, 43( AS (ankylosing spondylitis), 3 30-3 3 2, 3 3 1( AS (aortic stenosis), 2 3{, 72t ASA (acetylsalicylic acid) congestive heart failure, 39 overdose, 682 stroke, 367 Asbestosis, 6 3 51 ASC-H (atypical squamous cells suspicious for high-grade dysplasia), 5001 Ascites, 252, 2 54t, 2 5 5t ASC-US (atypical squamous cells of undeter­ mined significance), 500t, 50 I ASCVD (atherosclerotic cardiovascular disease), dyslipidemia and, 5 5-57, 561, 571 ASD (atrial septa! defect), 529-5 30, 5 301 ASD (autism spectrum disorder), 5 1 1 , 587 Aseptic meningitis, 379, 380t "Ash-leaf' macules, 407, 407( Aspartate aminotransferase (aspartate transami­ nase, AST), 3 3 3 hepatitis, 2 5 0 Aspergilloma, 654 Aspergillosis, 6 54, 654( allergic bronchopulmonary, 299-300, 630, 636, 654 chronic necrotizing pulmonary, 654 invasive pulmonary, 654 Aspergillus fumigatus, endocarditis, 69, 7 1 1 Aspiration, foreign body, 549 Aspirin-exacerbated respiratory disease, 627 Asplenia, vaccines, 1 7 3( AST (aspartate aminotransferase), 3 3 3 hepatitis, 2 5 0 Asterixis, 4021 Asthma, 626-628, 628t, 629t Astigmatism, 4 1 6 Astrocytoma, 4041 pilocytic, 560t Asymptomatic bacteriuria, pregnancy, 444, 7 1 1

Asystole, 3 3t Ataxia cerebellar, 398, 4 1 0 Friedrich, 5 l 9t Ataxia-telangiectasia, 4 1 0, 5441 Atelectrauma, 640 Atheroembolism, cholesterol, 8 1 Atherosclerotic cardiovascular disease (ASCVD), dyslipidemia and, 5 5-57, 56t, 57t Athlete's heart, 42 ATLS (advanced trauma life support) algorithm, 751 primary survey, 7 5 2-7 5 3 , 7 5 2(. 7 521, 7 5 3( secondary survey, 7 5 3-7 54 ATM gene, 4 1 0 Atonic seizures, 3 7 5( Atopic dermatitis, 90-9 1 , 90{, 9 1( Atopic h yp ersensitivity reaction, 89t Atovaquone/proguanil, 722 Atrial enlargement, 2 1 Atrial fibrillation (AF), 2 5-3 3 classification, 2 5 history/physical examination, 30 investigations, 30, 30( pathophysiology, 2 5 risk factors, 2 5 treatment, 30-3 3, 3 3t Atrial flutter, 281, 3 31 Atrial myxoma, 24 Atrial septa! defect (ASD), 529-5 30, 5 301 Atrial tachycardia, 291 multifocal, 30t Atrioventricular (AV) block first--clegree, 26t second-degree (Mobitz type 1/Wenckebach),

26t

second--clegree (Mobitz type II), 27t third--clegree (complete), 27t Atrioventricular (AV) nodal ablation, atrial fibril­ lation, 32, 3 3 Atrioventricular nodal reentry tachycardia (AVNRT), 2 5 , 2 5{, 281 Atrioventricular (AV) node, re-entry at, 25, 2 5(. 29t Atrioventricular reentrant tachycardia (AVRT), 2 5 , 29t Atrophic rhinitis, 670 Attention-deficit/hyperactivity disorder (ADHD), 586 Attributable risk, 1 60 Atypical antipsychotics, 59 1 , 592t Atypical hyperplasia (AH), 494 Atypical squamous cells of undetermined signifi­ cance (ASC-US), 500t-501 Atypical squamous cells suspicious for high­ grade dysplasia (ASC-H), 500t AUB (abnormal uterine bleeding), 476-478, 477{, 478t Auer rods, 300, 300{, 302t Auricular hematoma, 7 5 5 Auscultation, 22( Autism spectrum disorder (ASD), 5 1 1 , 587 Autoimmune diseases antibodies, 3 3 3t neoplasms, 7 5 1 I

Autoimmune hemolytic anemia (AIHA), 292, 292( infectious mononucleosis, 725 Autoimmune hepatitis, 2 5 0 Autoimmune hypothyroidism, 1 34 Autologous transplantation, 309 Autonomy, 1 8 5 Autosomal chromosome abnormalities, 5 1 61 Autosomal dominant polycystic kidney disease (ADPKD), 697-698, 697( Autosomal recessive polycystic kidney disease (ARPKD), 697-698 AV. See Atrioventricular (AV) Avascular necrosis (AVN), 343-344, 343( Aversion, alcohol use disorder, 6 1 3 AVM (arteriovenous malformation), 368 AVNRT (atrioventricular nodal reentry tachycardia), 2 5 , 2 5{, 281 Avoidant personality disorder, 609t AVRT (atrioventricular reentrant tachycardia), 25, 29t Axillary nerve injury, 3 2 l t Axonal injury, diffuse, 7 5 8, 7 5 9 Azathioprine, adverse effects, 747t Azoles, adverse effects, 747t

B Babesiosis, 72 3 Bacillary angiomatosis, HIV, 730t Bacillus anthracis, 660, 660( Bacillus Calmette-Guerin (BCG) vaccine, 657 Back pain low, 348-3 50, 349{, 3 50t motor, reflex, and sensory deficits, 3 501 Bacterial conjunctivitis, 4 1 4, 4 l 5t Bacterial meningitis, 379-38 1 , 3 79{, 3791-38 l t Bacterial skin infections, 1 0 3- 1 07 acne vulgaris, 1 06- 107 cellulitis, 1 03{, 1 04- 1 0 5 , 1 04( erysipelas, 1 0 3{, 1 04( folliculitis, 1 0 3{, 1 0 5-1 06, 1 06( impetigo, 1 03- 1 04, 1 0 3{, 1 04( leprosy, I 07 necrotizing fasciitis, 1 0 3{, 1 0 5 , 1 0 5( pilonidal cysts, 1 07, 1 07( Bacterial vaginosis, 488t, 489, 489( Bacteriuria, asymptomatic, pregnancy, 444, 7 1 1 Baker Act, 1 8 5 Baker cyst rupture, 3 1 81 Balancing, assessment, 1 84 Balanitis, circinate, 3 3 1 Bamboo spine, 3 3 1 , 3 3 1( Barbiturates abuse, 6 1 1 1 toxic ingestion/overdose, 745t withdrawal, 6 1 4t Bariatric surgery, 2 1 9-220 Barium swallow, 208, 208( Barlow maneuver, 5 7 5 , 5 7 5( Barotrauma, 640 Barrett esophagus, 2 1 0, 2 1 0( neoplasms, 75 I t Bartholin duct cyst and abscess, 487 Bartonella, 7 30t

INDEX Bart's hydrops, 289t Basal cell carcinoma (BCC), 1 1 9, 1 20( Basilar artery, 3 5 5( Basilar skull fractures, 7 5 8 Basophilic stippling, 2 8 7 , 287{, 5 8 1 , 5 8 1 ( BAT (blunt abdominal trauma), 762-763, 762t, 763( Bath salts, substance abuse, 6 1 2t Battle sign, 7 5 8 B C C (basal cell carcinoma), 1 1 9, 1 20( B-cell disorders, pediatric, 543t, 545 B-cell neoplasms, 305t BCG (bacillus Calmette-Guerin) vaccine, 657 BCR-ABL tyrosine kinase inhibitor, 304 Becker muscular dystrophy (BMD), 5 74, 574t Bed bugs, 1 1 2 Bee stings, 74 l t Behavioral counseling, 1 82, 1 83{, 1 8 3t Beh,;et syndrome, 3 3 8 Beneficence, 1 8 5 "Benign familial tremor," 402t, 4 1 0 Benign paroxysmal positional vertigo (BPPV), 377 Benign prostatic hyperplasia (BPH), 702-703, 702t Benzodiazepines, 595t, 597 abuse, 61 l t adverse effects, 747t toxic ingestion/overdose, 745t withdrawal, 6 1 4t Bereavement, 1 79, 602t Berger disease, 689t Berry aneurysm, ruptured, 368 Berylliosis, 6 3 5 t 13 (type II error), 1 68 13 2 agonists, asthma, 628t 13-blockers anxiety, 595t chronic stable angina, 49 congestive heart failure, 39 hypertension, 6 l t hypertrophic cardiomyopathy, 42 adverse effects, 747t toxic ingestion/overdose, 745t unstable angina/NSTEMI, 50 13-lactams, adverse effects, 748t Bezoar, gastric, 2 1 8-2 1 9 Bias, 1 6 5- 1 67, 1 67( Biceps reflex, 3 5 8t Bile acid diarrhea, 24 7 Bile acid resins, 57t adverse effects, 747t Bile salt deficiency, malabsorption, 22 3 Biliary cholangitis, primary, 2 59 Biliary colic, 244, 244{, 245t Biliary cyst, 247 Biliary disease, 244-248 biliary cyst, 247 cholangiocarcinoma, 247-248 cholangitis, 245t, 246 cholecystitis, 244-246, 245{, 245t choledocholithiasis, 245t, 246 cholelithiasis and biliary colic, 244, 244{, 245t gallstone ileus, 246---247 postcholecystectomy syndrome, 244, 247

Biliary obstruction, acute abdomen, 227 Binge eating/purging, 6 1 4, 6 1 5t, 6 1 6 Biophysical profile (BPP), 4 5 3 , 45 3t, 454 Biotin deficiency, 7 50t Biotrauma, 640 Bioweapons, reportable, I 76t Bipolar and related disorders, 60 3, 60 5-607, 605t, 606t Birth control, 478, 4791-4 8 1 1 Bisphosphonates menopause, 486 osteoporosis, 1 3 8 Paget disease of bone, 1 39 Bite wounds, 344-345 Bites and stings, 740, 741{, 74 1 1-7421 Bivalirudin, 2721 Bladder, painful, 70 I Bladder cancer, 704-705, 704( Bladder injuries, 764 Bladder pain syndrome, 7071 Blast crisis, 303 Blastomycosis, 65 51, 656-6 57 Bleeding. See Hemorrhage(s) Bleeding disorders, 270-277 hemophilia, 274-276, 275{, 284t and normal hemostasis, 270, 271{, 2 7 1 1-2731 and transfusion products, 274, 274t von Willebrand disease, 276-277, 276{, 2841 Blepharitis, 4 1 3, 4 1 3( Blistering dermatosis, 97{, 97t Blood glucose screening, 1 741, l 75t Blood pressure (BP) pregnancy, 4 291 screening, 1 741, 1 7 51 Blood pressure (BP) control, aortic dissection, 78 Blood replacement products, 274, 2741 Blood transfusion(s), refusal of, 1 8 1 Blood transfusion products, 274, 2741 Blood transfusion reactions, 296, 2961 Blood transmission, HIV, 726t Blood volume, pregnancy, 429t Bloodstream infections, central line-associated, 73 5-736 "Blown pupil," 3 5 3 "Blue babies," 528-529 "Blue bloater," 630 "Blue toe syndrome," 81 Blunt abdominal trauma (BAT), 762-763, 7621, 763( Blunt trauma, 7 57-764 abdomen, 762-763, 7621, 763( cardiac injury, 76 1-762, 762( chest, 760-76 1 , 760{, 761( head and face, 7 5 7-7 59, 7 5 8( pelvis, 763-764 BMD (Becker muscular dystrophy), 574, 5741 BMT (bone marrow transplantation), aplastic anemia, 290 Body dysmorphic disorder, 5981 Body lice, 1 1 0- 1 1 1 Boerhaave syndrome, 2 1 3 , 226 Bone and mineral disorders, 1 37-1 4 1 calcium and phosphate regulation and, 1 3 7, 1 3 8(

hyperparathyroidism, 1 39-1 4 1 , 1 401 osteoporosis, 1 37-1 39, 1 3 8( Paget disease of bone, 1 39, 1 39{, 1 40( Bone marrow failure, 565, 5651 Bone marrow transplantation (BMT), aplastic anemia, 290 Bone tumors, 3 2 3-324, 3241, 3 2 5( childhood, 5 7 1 , 5721 Boosting agents, HIV, 7 3 31 Borderline personality disorder, 6081 Bordelella pertussis, 5 54-5 5 5 Borrelia burgdorferi, 722-72 3, 723( Botulism, 3 86, 3871 infantile hyp otonia, 5 591 Boutonniere deformity, 3 30 Bowen disease, 1 1 8 Boxer's fracture, 3 1 6t BP (blood pressure) pregnancy, 4 291 screening, 1 741, 1 7 51 BP (blood pressure) control, aortic dissection, 78 BPH (benign prostatic h yp erplasia), 702-703, 7021 BPP (biophysical profile), 4 5 3 , 4 5 31, 454 BPPV (benign paroxysmal positional vertigo), 377 Brachioradialis reflex, 3 581 Bradyarrhythmias, 25, 261-271 Bradycardia management, 3 3 t sinus, 261 Bradykinesia, Parkinson disease, 397 Brain anatomy, 3 5 3, 3 5 3{, 3 54( arterial supply/venous drainage, 3 54, 3 54{, 3 5 5( herniation, 3 5 3 , 3 54{, 3 84 Chiari malformations, 563, 563( Brain abscess, 38 3-3 84, 383( Brain death, 1 89, 372, 3 721 Brain injury, traumatic, pediatric, 7 5 8-7 59 Brain tumors, 403, 4041-4061 pediatric, 560, 5601-56 1 1 Braxton Hicks contractions, 454 BRCA1 /BRCA2, 494, 503, 505 "Breakbone fever," 722 Breast benign disorders, 492-494 atypical hyperplasia, 494 intraductal papilloma, 493 nonproliferative lesions, 492-493 phyllodes tumor, 494, 494( proliferative lesions without atypia, 493 workup of breast mass, 4931 fat necrosis, 492 fibrocystic changes, 492 Breast abscess, 46 5-466 Breast biopsy, 495 Breast cancer, 494-497, 495{, 4961 Breast development, 468 Breast engorgement, 466 Breast mass, workup, 492, 493( Breast milk jaundice, 524 Breast screening, 1 741 Breast-conserving surgery, 496

INDEX Breastfeeding, 464-466, 464{, 465t Breastfeeding jaundice, 524 Breath, advanced trauma life support, 7 52-7 5 3 , 7 5 2( Breath-holding spells, 562 Breech presentation, 460-46 1 , 460( Brief psychotic disorder, 5 9 l t Brief resolved unexplained event (BRUE), 5 79 Broca aphasia, 372-373, 373( Broca area, 3 5 3{, 373( "Broken heart syndrome," 46t Bronchial carcinoid tumor, 648t Bronchiectasis, 628-630, 629( Bronchiolitis, 549, 5 52( Bronchitis, chronic, 630-63 1 , 630t, 63 1{, 6 3 l t Brown-Sequa rd hemisection, 3 59t Brudzinski sign, 5 5 3 BRUE (brief resolved unexplained event), 579 Bruises, child abuse, 578t Bruton agammaglobulinemia, 543t, 545 Buccal mucosa! laceration, 3 5 5( Bulimia nervosa, 6 1 5t, 6 1 6 Bulla, 88t Bullous impetigo, 1 04 Bullous pemphigoid, 96, 97{, 97t Bundle-branch block, 20, 20( Bundled payment, 1 78 Burkitt lymphoma, 305t Burn(s), 7 3 7-740 chemical and electrical, 737, 738t child abuse, 578t classification, 7 3 8-7 39, 738( diagnosis, 739, 739( history/physical examination, 739 treatment, 739-740 Burnout, healthcare personnel, 1 84- 1 8 5 Bursa infection of deep, 346 infection of superficial, 346 Bursitis, 345 anserine, 345-346 Buspirone, 595, 595t C C l esterase inhibitor deficiency, 545t CA (cancer antigen) 1 5-3, 495 CA (cancer antigen) 27-29, 49 5 CA- 1 2 5 , 504, 505, 505t Cafe au lait spots, 406, 583t Caffeine abuse, 6 l 2 t CAGE questionnaire, 6 1 3 CAH (congenital adrenal hyperplasia), 1 54, l 54t, 481-483, 482{, 483t precocious puberty, 4 70 prima ry amenorrhea/delayed puberty, 4 7 1 Calcaneal stress fracture, 3 l 8t Calcium channel blockers (CCBs) hypertension, 6 l t adverse effects, 747t Calcium oxalate stones, 695t Calcium phosphate stones, 695t Calcium pyrophosphate deposition disease (CPPD), 347t, 348, 348( Calcium regulation, 1 37, 1 3 8(

Calcium supplements, menopause, 486 Calculi, renal, 694-697, 695t-696t, 696( CAM (Confusion Assessment Method), 60 1 CAM (complementa ry and alternative medicine) therapy, 1 9 1 Campylobacter jejuni, diarrhea, 22 1 t c-ANCA, 3 3 3t Cancer bladder, 704-705, 704( breast, 494-497, 495{, 496t cervical, 499-502, 5 0 1 {, 500t, 502( colorectal, 2 36-2 37, 236{, 2 3 6t, 2 37t endometrial, 498-499, 499{, 499t esophageal, 2 I 1 gastric, 2 1 6, 2 1 6( lung, 647-6 50, 648t, 649{, 650t ovarian, 503-505, 504t, 505t pancreatic, 267 pediatric, 569-572 bone tumors, 5 7 1 , 572t Langerhans histiocytosis, 5 7 1 leukemia, 569-570 neuroblastoma, 570-57 1 , 5 70( Wilms tumor, 5 7 1 prostate, 702t, 70 3-704, 703( testicular, 70 5-706, 706t urologic, 70 3-706 vaginal, 503 vulvar, 502-503 Cancer antigen (CA) 1 5-3, 49 5 Cancer antigen (CA) 27-29, 49 5 Cancers, oral, 201 Candida albicans, 1 08- 1 09, 1 09( HIV, 727, 727{, 729{, 730( Candida diaper dermatitis, 582t Candida endocarditis, 69, 7 l t Candida esophagitis, 1 09, 204, 2 0 5 , 205t HIV, 730t Candida spp, 1 08- 1 09, 1 09( Candidiasis oral, 1 08-1 09, 200, 203 HIV, 727, 727{, 729t vulvovaginal, 488t, 489( HIV, 727 pediatric, 49 1 Cannabis abuse, 6 1 2t Capitation, 1 78 Caplan syndrome, 3 30 Capsulitis, adhesive, 3 39 Caput succedaneum, 527t Carbamazepine, 606t pregnancy, 434t adverse effects, 747t Carbohydrate maldigestion, 224 Carbon monoxide poisoning, 743 Carbonic anhydrase inhibitors, 38t, 688t Carbuncle, 1 0 5 , 106( Carcinoemb ryonic antigen (CEA), breast cancer, 495 Carcinoid syndrome, 224 Carcinoid tumor, bronchial, 648t Cardiac amyloidosis, 44t Cardiac arrhythmias, 2 5-34 bradyarrhythmias and conduction abnormali­ ties, 2 5 , 26t-27t

management, 3 3t, 34, 34t after STEM!, 54 tachyarrhythmias, 2 5-33, 2 5{, 28t-32t, 30( Cardiac axis, 1 8, 1 9{, l 9t Cardiac enzymes angina pectoris, 47 STEM!, 5 1-52 unstable angina/NSTEMI, 50 Cardiac hemochromatosis, 45t Cardiac inju ry, blunt, 76 1-762, 762( Cardiac intervals, 20, 20( Cardiac life support basics, 3 3t, 34, 34t Cardiac murmurs, 22, 22(-24( Cardiac output, pregnancy, 429t Cardiac physical exam, 22-24, 2 2(-24{, 2 3t Cardiac resynchronization therapy (CRT), congestive heart failure, 39 Cardiac rhythm, 18 Cardiac sarcoidosis, 45t Cardiac syncope, 82 Cardiac tamponade, 67 advanced trauma life support, 753, 7 5 3( Cardiogenic shock, 7 l 8t Cardiomyopathy, 40-46 acromegalic, 45t arrhythmogenic right ventricular dysplasia, 42-43, 43( chemotherapy-related, 45t diabetic, 45t differential diagnosis, 40, 40t, 4 1 ( dilated, 40t, 4 1 , 41( h ypertrophic, 40t, 4 1 -42, 41( other, 46, 46t peripartum, 46t, 464 restrictive, 40t, 4 3 secondary, 44, 44t-45t Takotsubo, 46t Cardiovascular changes, pregnancy, 429, 429t Cardiovascular medicine, 1 7-8 5 acute corona ry syndromes, 49- 5 5 carotid arte ry stenosis, 5 5 ST-segment elevation myocardial infarc­ tion, 5 1-54, 52{, 5 3{, 54t unstable angina/non-ST-segment eleva­ tion myocardial infarction, 49-5 1 , 50( arrhythmias, 2 5-34 bradyarrhythmias and conduction abnor­ malities, 2 5 , 26t-27t management, 3 3 t, 34, 34t tachyarrhythmias, 2 5-3 3, 2 5{, 28t-32t, 30( cardiac life support basics, 3 3t, 34, 34t cardiac physical exam, 22-24, 22(-24{, 23t cardiomyopathy, 40-46 arrhythmogenic right ventricular dysplasia, 42-43, 43( differential diagnosis, 40, 40t, 4 1( dilated, 40t, 4 1 , 4 1 ( hypertrophic, 40t, 4 1 -42, 4 1 ( other, 4 6 , 46t restrictive, 40t, 4 3 secondary, 44, 44t-4 5t congestive heart failure, 34-40 classification, 34- 3 5 , 3 5t with preserved ejection fraction, 39-40

INDEX systolic dysfunction/with reduced ejection fraction, 36---39, 36{, 37{, 37t, 38t corona ry arte ry disease, 47-49 angina pectoris, 4 7-49, 48( Prinzmetal (variant) angina, 49 dyslipidemia, 5 5-57, 56t, 571 electrocardiogram, 1 8-22 axis, 1 8, 1 9{, l 9t chamber enlargement, 2 1-22, Z if intervals in, 20, 20( ischemia/infarction, 2 1 , 2 1( normal, 1 8, 1 8( rate, 1 8 rhythm, 1 8 endocarditis, 67-7 1 , 68t, 69{, 70t, 7 1 1 hypertension, 57-63 classification, 57, 58t hyp ertensive emergency/urgency, 63, 63t prima ry (essential), 57-59, 59{, 60t-62t seconda ry, 62t, 6 3 pericardia! disease, 64-67 acute pericarditis, 64-6 5, 64{, 65( cardiac tamponade, 67 constrictive pericarditis, 6 5-66 pericardia! effusion, 66-67, 66( syncope, 82-84, 83{, 84t-8 5t valvular heart disease, 72, 72t-74t vascular diseases, 7 5-82 aortic aneu rysm, 7 5-76, 7 5{, 76( aortic dissection, 76-78, 77( deep venous thrombosis, 78-80, 781, 79( lymphedema, 82 peripheral arterial disease, 8 1 -82 postthrombotic (postphlebitic) syndrome, 80 Cardiovascular screening, l 74t, 1 7 51 Carclioversion atrial fibrillation, 32-3 3 synchronized vs. unsynchronized, 341 Carnett sign, 764 Carotid arte ry dissection, 3651 Carotid arte ry stenosis, 5 5 Carotid endarterectomy, 367, 367( Carotid sinus syndrome, 84t Carpal tunnel syndrome, 342, 342( Case-control study, 163-164, 1 64{, 1 6 5 t Cat bites, 742t Cataplexy, 6 1 8 Catatonia, 592 Cathinones, synthetic, substance abuse, 6 1 21 Caucla equina syndrome, 349, 360t Cavernocapilla ry hemangioma, 567( Cavernous sinus syndrome, 422 Cavernous sinus thrombosis, 369-37 1 CBD (common bile duct), gallstones, 2451, 246 CCBs (calcium channel blockers) hypertension, 6 l t adverse effects, 747t CD4 + cell count, HIV, 727, 7 301 COM (congenital dermal melanocytosis), 5831 CEA (carcinoemb ryonic antigen), breast cancer, 495 Celiac disease, 22 3-224, 223( Cell-free fetal DNA, 433t Cell-mediated type hyp ersensitivity reaction, 89t

Cellulitis, 1 0 3{, 1 04- 1 0 5 , 1 04( orbital (postseptal), 4 1 3-4 1 4 preseptal (periorbital), 4 1 3 Centor criteria, 66 1 , 66 l t Central cord syndrome, 360t Central diabetes insipiclus, 14 2, 1 44, l 44t Central hypogonaclism, 470 Central line-associated bloodstream infections (CLASB!s), 7 3 5-7 36 Central nervous system (CNS) infections, 379-384 brain abscess, 383-3 84, 383( c ryptococcal meningitis, 3 8 1 cytomegalovirus, 7 3 5 encephalitis, 382-383, 383( meningitis, 379-3 8 1 , 379{, 379t-3 8 l t toxoplasmosis, 3 8 1-382, 382( Central nervous system (CNS) lymphoma, 382 Central nervous system (CNS) tumors, 40 3, 4041-406/ Central precocious puberty, 469-4 70, 469t Central retinal artery occlusion, 420t Central retinal vein occlusion, 420t Central sleep apnea (CSA), 6 1 9 Central sulcus, 3 5 3( Central transtentorial herniation, 3 5 3 CEP (chronic eosinophilic pneumonia), 299-300 Cephalhematoma, 5271 Cephalic pustulosis, neonatal, 528t Cephalosporins, penicillin allergy, 327 Cerebellar ataxia, 4 1 0 parkinsonism, 398 Cerebellar tonsillar herniation, 3 5 3, 563, 563( Cerebral edema, high-altitude, 740 Cerebral palsy (CP), 5 57-5 58 Cerebrospinal fluid (CSF) leak, basilar skull fracture, 7 5 8 Cerebrospinal fluid (CSF) profiles, 3 80t Cervical cancer, 499-502, 50 1{, 5001, 502( Cervical factors, infertility, 4851 Cervical intraepithelial neoplasia (CIN), 499, 5001, 5 0 1 Cervical spine (C-spine) CT, advanced trauma life support, 754 Cervicitis, 489 Cesarean section (C-section), indications, 457, 4571 CF ( cystic fibrosis), 5 14-520 CFTR (cystic fibrosis transmembrane conductance regulator) gene, 5 1 4 CGD ( chronic granulomatous disease), 5441 Chalazion, 1 1 6, 4 1 2 Challenging conversations, 1 8 1 Chamber enlargement, electrocardiogram with, 2 1-22, 2 1( Chancre, 7 1 3, 7 1 3( Chancroid, 7 1 5 1-7 1 61 Change, stages of, 1 8 3{, 1 8 31 Charcot triad, 246 CHO. See Congenital heart disease (CHO) Chediak-Higashi syndrome, 5451 Chemical burns, 7 3 7, 7381 Chemotherapy nausea clue to, 307 neoadjuvant, breast cancer, 496

Chemotherapy-related cardiomyopathy, 451 Cherry angiomas, 1 22, 1 22( Chest, flail, 639, 760, 761( Chest trauma blunt and deceleration, 760-76 1 , 760{, 76 1( penetrating, 756 Chest x-ray (CXR) ARDS, 638, 638( bronchiectasis, 629 COPD, 63 1 , 63 1( hypoxia, 637 lung cancer, 649, 649( pneumonia, 6 5 1 , 6 5 1 ( systemic sarcoidosis, 634, 634( tuberculosis, 6 5 8, 659( Chiari malformations, 563, 563( Chickenpox, 5 561 Chikugunya virus, 722 Child abuse/neglect, 577-579, 5781 confidentiality, 193 Child development, 5 1 1-5 1 4 developmental milestones, 5 1 1 , 5 1 21, 5 1 31 growth, 5 I 1-5 1 4 primitive reflexes, 5 1 1 , 5 1 31 sexual, 5 1 4, 5 1 5( Childhood psychiatric disorders, 586---590 attention-deficit/hyperactivity clisorcler, 586 autism spectrum disorder, 587 diagnostic criteria by symptom duration, 6071 disruptive behavioral disorders, 587-588 intellectual developmental disorder/intellectual disability, 588 separation anxiety disorder, 589-590 Tourette syndrome, 589 Chilclhoocl-onset fluency disorder, 5 l 3t Chilclhoocl-onset genetic disease, 5 1 4-520 autosomal chromosome abnormalities (trisomies), 5 1 61 cystic fibrosis, 5 I 4-520 inherited metabolic disorders, 5 1 71-5 1 81 other, 5 1 91 sex chromosome abnormalities, 5 1 71 Children. See also Pediatrics avascular necrosis, 343 clinical research, 1 94 hearing, 579 infantile spasms, 408 osteochondritis dissecans, 345, 345( recommended vaccinations, 1 7 lf Salter-Harris pediatric fracture classification, 3 1 9-320, 320( seizure disorders, 3731, 3 7 5 vaginal discharge, 49 1 Chi-square ( x 2 ) test, 1 69 Chlamydia, 7 1 1-7 1 2, 7 1 1{, 7 1 2( Chlamydia test, 1 741 Chlamydia lrachomalis, 7 1 1-7 1 2, 71 lf, 7 1 2( cervicitis, 489 conjunctivitis, 4 1 51 neonatal ocular infections, 5 5 3-5 54, 5 54t pediatric, 578 pelvic inflammato ry disease, 489-490 prostatitis, 7 1 0 vaginitis, 487, 489 Chloasma, 4291

INDEX Chloramphenicol, adverse effects, 7471 Chloroquine, 722 Choanal atresia, 5 2 1 1 Cholangiocarcinoma, 247-248 Cholangitis, 2451, 246 primary biliary, 2 5 9 primary sclerosing, 2 59 Cholecystitis, 244-246, 245(, 245t acute abdomen, 227 emphysematous, 244 Choledocholithiasis, 2451, 246 Cholelithiasis, 244, 244(, 2451 Cholestasis, 248, 248f intrahepatic, pregnancy, 444-445 Cholesterol, 5 5-57, 561, 5 7t screening, 1 741, 1 7 51 Cholesterol absorption inhibitors, 571 Cholesterol atheroembolism, 8 1 Cholesterol embolism, 230 Chondrocalcinosis, 348, 348f Chorea, 396--397 Choriocarcinoma, 5051, 706t Chorionic villus sampling (CVS), 432, 4 3 3(, 4331 Chromosomal abnormalities autosomal, 5 1 61 sex, 5 1 7t spontaneous abortions, 436 Chronic atrophic gastritis, neoplasms, 7 51 I Chronic bronchitis, 630-63 1 , 6301, 63 1(, 6 3 1 1 Chronic disease, anemia of, 2861, 287 Chronic eosinophilic pneumonia (CEP), 299-300 Chronic granulomatous disease (CGD), 5441 Chronic inflammation, anemia of, 2861, 287 Chronic kidney disease (CKD), 684-687 Chronic limb-threatening ischemia, 8 1 Chronic lymphocytic leukemia (CLL), 30 1-302, 302(, 3021, 303 Chronic mesenteric ischemia, 2 301 Chronic myelogenous leukemia (CML), 302-303, 3021, 303, 303t Chronic necrotizing pulmonary aspergillosis, 654 Chronic obstructive pulmonary disease (COPD), 630-63 1 , 630t-6321, 63 lf Chronic pelvic pain syndrome, 700 Chronic suppurative otitis media, 423 Chronic transplant rejection, 309-3 1 0, 3 1 01 Churg-Strauss syndrome, 3 3 7(, 688, 690t CI (confidence interval), 1 68 Cimex leclularius, 1 1 2 CIN (cervical intraepithelial neoplasia), 499, 5001, 5 0 1 Cingulate herniation, 3 5 3 Circadian rhythm sleep disorder, 6 1 9 Circinate balanitis, 3 3 1 Circle o f Willis, anatomy, 3 54, 3 54(, 3 5 5f Circulation, advanced trauma life support, 7 5 2(, 7 5 3 , 7 5 3f Circulatory changes, pregnancy, 4291 Circumcision, 542 Cirrhosis, 2 5 2-2 54 ascites, 2 5 2 , 2 541, 2 5 5t complications, 2 54, 2 5 51

diagnosis, 2 5 2-2 5 3 encephalopathy, 2 52, 2 5 31, 2 54, 2 5 51 etiology, 2 5 2 history/physical examination, 2 5 2 neoplasms, 7 5 1 1 presentation, 2 52, 2 5 3f progression, 2 5 2 , 2 5 2(, 2 5 3f treatment, 2 54 Cisplatin, adverse effects, 747t CJD (Creutzfeldt-Jakob disease), 390t, 394-39 5 CKD (chronic kidney disease), 684-687 CK-MB angina pectoris, 4 7 STEMI, 5 1-52 unstable angina/NSTEMI, 50 CLASB!s ( central line-associated bloodstream infections), 73 5-736 Claudication intermittent, 8 1 neurogenic, 3 50 Clavicular fracture, 3 I 41 pediatric, 5731 Clinical research, 1 94- 1 9 5 core principles, 1 94 ethical concerns, 1 94- 1 9 5 Clinical studies evaluating, 1 6 5- 1 69 bias, 1 6 5- 1 67, 1 67f commonly used statistical tests, 1 69, 1 69f scenarios, 1 67-168 statistical testing, 167 types, 1 6 1- 1 6 5 case-control study, 163-164, 1 64(, 1 6 5 t cohort study, 1 60, 1 62- 1 6 3 , 1 64(, 1 651 cross-sectional study, 1 62, 1 64(, 165t phases of clinical trials, 1 64, 1 661 randomized controlled trial, 1 64, 1 651 and strength of evidence, 1 6 1 - 1 62, 1 62f Clinical trials, phases, 1 64, 1 661 Clinician-assisted suicide, 1 90- 1 9 1 CLL (chronic lymphocytic leukemia), 30 1-302, 302(, 3021, 303 Clonidine, adverse effects, 7471 Closed-angle glaucoma, 4 1 7, 4 1 7(, 4 1 8t Closlridioides difficile colitis, 2 3 1 -2 3 2 diarrhea, 22 1 I Closlridium bolulinum, 3 86, 3871 Closlridium difficile. See Closlridioides difficile Closlridium seplicum, endocarditis, 68, 681 Clotting factor deficiencies, 274-277, 275(, 276(, 2841 Clozapine, adverse effects, 7471 Clubbing, 2 3 Clubfoot, 5 7 5 Cluster headache, 3 6 l t, 362 CML (chronic myelogenous leukemia), 302-303, 3021, 303, 303t CMV. See Cytomegalovirus (CMV) CN(s) (cranial nerves), 3 56-3 58, 3 5 61, 3 571 CNS. See Central nervous system (CNS) Coagulase-negative staphylococcus, osteomyelitis, 3271 Coagulation, disseminated intravascular, 280-2 8 1 , 2801, 2831, 2841

Coagulation cascade, 270, 271f Coagulation disorders, 270-277 hemophilia, 274-276, 275(, 2841 and normal hemostasis, 270, 271(, 27 1 1-2731 and transfusion products, 274, 2741 von Willebrand disease, 276-277, 276(, 2841 Coagulation phase, hemostasis, 270 Coagulopathy, cirrhosis, 2 5 5t Coal worker's disease, 6 3 5t Coarctation of aorta, 5 3 1-532, 5 3 2f Cobalamin deficiency, 4 1 1 1, 7 501 Cobb angle, 577 Cocaine abuse, 6 1 21 pregnancy, 4 341 withdrawal, 6 1 41 Coccidioides immilis, 656 Coccidioidomycosis, 65 5t, 656 Cadman triangle, 324 Cognitive development, 5 1 21 Cognitive impairment, communication, 1 82 Cohort studies, 1 60, 162- 1 6 3 , 1 64(, 165 1 Colchicine, 348 Cold agglutinins, 292, 308 Colic, biliary, 244, 244(, 2451 Colitis Clostridium difficile, 2 3 1-2 3 2 ischemic, 2 3 8-2 39, 2 39f abdominal pain, 2 301 acute abdomen, 227 microscopic, 240-24 1 ulcerative, 24 1 , 24 lf, 242(, 242t neoplasms, 7 5 1 t Calles fracture, 3 l 5t Colorectal cancer, 2 36-2 37, 236{, 2361, 2 3 71 conditions associated with, 2 3 7-2 38, 238f diagnosis, 236 hereditary nonpolyp osis, 237, 2 3 71, 503 history/physical examination, 2 36, 236f risk factors, 2 36, 2 361 screening, 1 741, 1 7 51, 2 36, 2 3 71 treatment, 2 3 7 Colostrum, 464 Colposcopy, 5 0 1 Coma, 37 1-372, 372t Glasgow Coma Scale, 752, 7 52t myxedema, 1 3 5 Combined immunodeficiency disorders, pediatric, 5441 Comedones, 1 06 Common bile duct (CBD), gallstones, 2451, 246 Common pathway, 270, 27lf Common peroneal nerve injury, 3 2 1 1 Common variable immunodeficiency (CVID), 5431, 545 Communication, 1 79-1 82 behavioral counseling, 1 82, 1 83(, 1 831 challenging conversations, 1 8 1 culturally inclusive history taking, 1 8 1 gender- and sexuality-inclusive history taking, 181 interpreters, 1 82 motivational interviewing, 1 82 patient-centered, evidence-based interviewing, 1 79-1 80

INDEX patients with disabilities, 1 82 rapport, 1 80, l 80t Compartment syndrome, 340-341 Competence, 1 86--- 1 87 Complement, nephritis syndrome, 688, 688{ Complement deficiencies pediatric, 545t, 546 vaccines, 1 7 3{ Complementa ry and alternative medicine (CAM) therapy, 1 9 1 Complete androgen insensitivity, 4 7 1 Complete AV block, 27t Complete breech presentation, 460, 460{ Complex partial seizures, 374, 3 7 5{ Complex regional pain syndrome, 322-3 2 3 Computed tomography (CT), advanced trauma I ife support cervical spine, 7 54 head, 7 54 Computed tomography angiography (CTA) angina pectoris, 48 neck, 7 5 5 Condoms female, 480t male, 480t Conduct disorder, 5 87-588 Conduction abnormalities, 2 5, 26t-27t Conduction aphasia, 373{ Conductive hearing loss, 42 5{, 426 Condylomata lata, 7 1 3 , 7 1 3{ Confidence interval (CI), 1 68 Confidentiality, 193-194 Conflict of interest, 1 9 5- 1 96 gifts from drug companies, 1 9 5- 1 96 gifts from patients, 1 9 5 Confounding bias, 1 66 Confounding variables, 1 67 Confusion Assessment Method (CAM), 601 Congenital adrenal hyperplasia (CAH), 1 54, l 54t, 48 1 -483, 482{, 483t precocious puberty, 470 prima ry amenorrhea/delayed puberty, 47 1 Congenital dermal melanocytosis (COM), 583t Congenital diaphragmatic hernia, 5 2 l t Congenital heart disease (CHO), 528-534 acyanotic left-to-right shunts, 528, 529-5 32 coarctation of aorta, 5 3 1-532, 5 3 2{ cyanotic right-to-left shunts, 528-529, 5 3 2-534 genetic syndromes, 528, 529t patent ductus arteriosus, 5 3 1 , 5 3 1{ septa! defects, 529-530, 5 30t tetralogy of Fallot, 529t, 5 3 3-5 34, 5 3 3{ transposition of the great vessels, 5 32-5 3 3, 53 3( Congenital infections, 43 5, 435t-436t Congenital malformations, 520, 5 2 l t-5221 Congenital melanocytic nevus, 5831 Congestive heart failure, 34-40 acute decompensated, 3 5 treatment, 37-38, 371, 381 chronic, 3 5 treatment, 39 classification, 34- 3 5 , 3 5 1 hemodynamic profiles, 3 7 , 3 71

left-sided vs. right-sided, 34, 3 51 with preserved ejection fraction, 34, 3 51, 39-40 systolic dysfunction/with reduced ejection fraction, 34, 36---39 diagnosis, 36-37, 37{ etiology, 36, 36{ histo ry/physical examination, 36 symptoms, 3 5( treatment, 37-39, 371, 381 systolic vs. diastolic, 34, 3 51 Conjugate vaccine, 1 701 Conjunctivitis, 4 1 4, 4 1 4{, 4 1 51 Consciousness, transient loss of, 82-84, 83{, 841-8 51 Consent, informed, 1 87-1 89 clinical research, 1 9 5 Constipation, pediatric, 5 3 9-540 Constitutional growth delay, 47 1 , 472t, 5 1 2 Constrictive pericarditis, 65-66 Contact burns, child abuse, 5781 Contact dermatitis, 9 1 , 9 1{ infant, 5821 Contact lens keratitis, 4 1 5-4 1 6 Contemplation stage o f change, 1 83{, 1 831 Contraception, 478, 4 791-48 1 I Contraction stress test (CST), 4 5 3 , 4 5 3{ Contusion myocardial, 76 1 -762, 762{ pulmonary, 638, 638{, 760 Conus medullaris, 3601 Conversations, challenging, 1 8 1 Conversion disorder, 620-62 1 Cooley anemia, 2891 Coombs test, direct and indirect, 292, 292{ COPD (chronic obstructive pulmona ry disease), 630-63 1 , 6301-6321, 63 1{ Copper, toxic ingestion, 7451 Copper intrauterine device, 4791, 48 1 1 Coprolalia, 589 Cor pulmonale, 643 Corneal abrasion, 4 1 3 , 4 1 3{ Corona ry angiography, angina pectoris, 48 Corona ry arte ry disease, 47-49 angina pectoris, 4 7-49, 48{ Prinzmetal (variant) angina, 49 Corona ry steal syndrome, 48 Coronavirus, 64 1 -642, 642{ Corpus albicans, 468{ Corpus luteal cyst, 4981 Corpus luteum, 468{ Correlation, 1 69 Cortical blood supply, 3 54, 3 54{ Corticobasal degeneration, 398 Corticosteroids asthma, 628, 6281 adverse effects, 7471 Costochondritis, angina pectoris, 48 Counseling, behavioral, 1 82, 1 8 3{, 1 8 3t COVID- 1 9, 64 1 -642, 642{ vaccines, 1 7 3- 1 74 Coxsackie A virus, 5 571 CP (cerebral palsy), 5 5 7- 5 5 8 CPPD (calcium pyrophosphate deposition disease), 3471, 348, 348{ "Crabs," 1 1 0- 1 1 1

Cradle cap, 9 1 -92, 92{ Cranial nerves (CNs), 3 56-3 58, 3 5 61, 3 5 71 Craniopharyngioma, 5601 Cranium, penetrating trauma, 754 Cremasteric reflex, 3 581 CREST syndrome, 3 34 Creutzfeldt-Jakob disease (CJD), 3901, 394-39 5 Cricothyroidotomy, emergency, 7 5 2 Crigler-Najjar syndrome, 5231, 524 Crime, intent to commit, 193 Critical limb ischemia, 8 1 Crohn disease, 24 1 , 241{, 242{, 2421 Cromolyn, asthma, 6281 Cross-sectional study, 1 62, 1 64{, 1 6 5 1 Croup, 5 50, 5 50{, 5 5 01, 5 52{ CRT (cardiac resynchronization therapy), congestive heart failure, 39 Crust, 88t C ryoglobulin, 337{ C ryoglobulinemia, 308 mixed, 692 Cryoprecipitate, 2741, 275 Cryptococcal meningitis, 381 Cryplococcus neofonnans, HIV, 7301 Cryptogenic organizing pneumonia, 6 3 3 C ryptorchidism, 541 Cryplosporidium diarrhea, 220 HIV, 7301 CSA (central sleep apnea), 6 1 9 C-section (cesarean section), indications, 457, 4571 CSF ( cerebrospinal fluid) leak, basilar skull fracture, 7 5 8 CSF (cerebrospinal fluid) profiles, 3801 C-spine ( cervical spine) CT, advanced trauma life support, 7 54 CSS (cytokine storm syndrome), 64 1 CST (contraction stress test), 4 5 3 , 4 5 3{ CT (computed tomography), advanced trauma life support cervical spine, 7 54 head, 754 CTA (computed tomography angiography) angina pectoris, 48 neck, 7 5 5 Culturally-inclusive histo ry taking, 1 8 1 Culture-negative organisms, endocarditis, 681, 69 CURB-65 Score, pneumonia, 6 5 1 Curling ulcers, 2 1 5 "Currant jelly stool," 5 3 5 Cushing disease, 1 50, 1 5 1{ Cushing syndrome, 1 50-1 5 1 , 1 5 1{, 1 52{, 1 5 31 Cushing ulcers, 2 1 5 Cutaneous candidiasis, 1 09, 1 09{ Cutaneous larva migrans, I I 2 Cutaneous mycoses, 1 09- 1 10, I 09{, 1 1 Of Cutaneous squamous cell carcinoma, 1 1 8- 1 19, I 1 9{ Cutaneous T-cell lymphoma, 1 2 1 - 1 22, 1 2 1{ Culibaclerium acnes, I 06 Cutoff values, and sensitivity and specificity, 1 59, 1 59{ CVID (common variable immunodeficiency), 5431, 545

INDEX CVS (chorionic villus sampling), 432, 4 3 3{, 433/ CXR. See Chest x-ray (CXR) Cyanide poisoning, 744, 7451 Cyanosis, Raynaud phenomenon, 344 Cyanotic right-to-left shunts, 528-529, 5 32-534 Cyclic neutropenia, 566 Cyclophosphamide, adverse effects, 7471 Cyst(s), 88/ Bartholin duct, 487 biliary, 24 7 corpus luteal, 4981 epidermal inclusion, 1 1 6, l 1 6f ganglion, 342-343, 343f hydatid, 264 milk retention, 466 ovanan nonneoplastic, 498, 498/ precocious puberty, 4 70 ruptured, 227 pancreatic, 264 pilonidal, 1 07, 1 07f popliteal (Baker), 3 1 8/ theca lutein, 498t Cystic fi b rosis (CF), 5 1 4-520 Cystic fibrosis transmembrane conductance regulator (CFTR) gene, 5 1 4 Cystine stones, 696/ Cystitis, 706, 708, 7 1 0 interstitial, 70 1 Cystourethrogram, voiding, 540, 54 lf Cytokine storm syndrome (CSS), 64 1 Cytomegalovirus (CMV), 7 3 5 C N S involvement, 7 3 5 congenital, 4 3 5t encephalitis, 383 esophagitis, 2051, 7 3 5 G I and hepatobiliary involvement, 7 3 5 HIV, 7301 pneumonitis, 73 5 retinitis, 7 3 5 Cytotoxic hypersensitivity reaction, 89/

D Dabigatran, 2721 Dacryocystitis, acute, 4 1 4, 4 l 4f Dactylitis, sickle cell disease, 568 DAI (diffuse axonal injury), 758, 759 Dalteparin, 2721 Danger, confidentiality, 1 94 Dapagliflozin, congestive heart failure, 39 Dark-field microscopy, syp hilis, 7 1 4/ Database of High-Yield Facts, how to use, 1 6 DCIS (ductal carcinoma i n situ), 493, 494 DCM (dilated cardiomyopathy), 40t, 4 1 , 4 lf De Quervain tenosynovitis, 3 1 6/ Deceleration(s), fetal heart rate, 4 5 5 , 456t Deceleration trauma, 7 5 7-764 abdomen, 762-763, 762t, 763f cardiac injury, 761-762, 762f chest, 760-76 1 , 760{, 761f head and face, 7 5 7-7 59, 758f pelvis, 763-764 Decision making capacity, 1 87

to save life of child, 1 8 1 surrogate, 1 90 Decontamination, 742-743 Decubitus ulcers, 1 1 2-1 1 3 , l 1 2f Deep venous thrombosis (DVf), 78-80, 781, 79f postamputation, 7 57 Deep venous thrombosis (DVf) prophylaxis, pulmonary embolism, 645 Defeminization, 474 Defense mechanisms, 609, 609/ Defibrillation, 34t Deficits, advanced trauma life support, 7 5 3 Deformities, advanced trauma l i fe support, 753 Delayed primary closure, 2 3 1 Delayed puberty, 470-47 1 , 4721, 5 1 4 Delayed type hyp ersensitivity reaction, 891 Delirium, 600t, 60 1 -602, 60 1 1 Delirium tremens (DTs), 6 1 3, 6 1 3t Delusion, 5 9 1 Delusional disorder, 5 9 l t Dementia, 3 88-395, 389/, 599-60 I Alzheimer disease, 390-392, 390t Creutzfeldt-Jakob disease, 390/, 394-395 defined, 390 vs. delirium, 600, 600t, 602 diagnosis, 600, 600/ frontotemporal (Pick disease), 390t, 393 history/physical examination, 599-600 Korsakoff, 4 1 1 t Lewy body, 3 9 l t, 395 major, 3 89t mild, 3891 vs. normal aging, 388, 389/ normal-pressure hydrocephalus, 3901, 392, 393-394, 394{, 397 Parkinson disease, 3 9 l t, 395 reversible vs. irreversible causes, 389t treatment, 60 I types, 390, 3901-39 I t vascular, 3901, 392 Dementia with Lewy bodies (DLB), 3 9 1 1, 395 Demyelinating disorders, 387-3 88 Guillain-Barre syndrome, 380/, 388 multiple sclerosis, 3 591, 3 80/, 387-3 88 Dendritic ulcer, 4 1 5 , 4 l 5f Dengue virus, 722 Denial, 609/ Dependent personality disorder, 609/ Depersonalization/derealization disorder, 593, 594t Depressants, substance abuse, 6 1 l t Depression bipolar, 60 3, 607 double, 604 major depressive disorder, 602-60 3, 602t604t persistent depressive disorder (dysthymia), 602t, 604-605 postpartum, 602, 603/ Derealization disorder, 593, 594t Dermal melanocytosis, congenital, 5831 Dermatitis atopic, 90-9 1 , 90{, 9 lf

Candida diaper, 5821 contact, 9 1 , 9lf infant, 5821 perianal, 582, 5821 seborrheic, 9 1 -92, 92f HIV, 727 stasis, 1 1 4, l l 4f Dermatitis herpetiformis (DH), 98, 1 00{, 223, 223{, 224 Dermatofibroma, 1 1 6, l 1 6f Dermatology, 87- 1 22 allergic and immune-mediated skin disorders, 89-98 atopic dermatitis (eczema), 90-9 1 , 90{, 9lf bullous pemphigoid/pemphigus vulgaris, 96, 97{, 971 contact dermatitis, 9 1 , 9 lf drug eruption, 94, 94f erythema multiforme, 95, 9 5f erythema nodosum, 96, 96f hypersensitivity reactions, 89, 891, 90f nummular eczema, 98, 98f psoriasis, 92-93, 93f pyoderma gangrenosum, 98, 98f seborrheic dermatitis, 9 1 -92, 92f Stevens-Johnson syndrome/toxic epidermal necrolysis, 95-96, 9 5f urticaria (hives), 93-94, 94f infectious disease manifestations, 98- 1 1 2 bacterial, 1 03-1 07, 1 0 3f- 1 07f fungal, 1 08-1 1 0, 1 08f- 1 1 Of parasitic, 1 1 0- 1 1 2, 1 1 lf viral, 98- 1 0 3 , 99t- 1 00t, 1 00f- 1 02f ischemic skin disorders, 1 1 2- 1 1 3 decubitus ulcers, 1 1 2-1 1 3 , 1 1 2f gangrene, 1 1 3, l l 3f layers of skin, 88, 1 0 3 , 1 03f macroscopic terms, 88, 88/ miscellaneous skin disorders, 1 1 4- 1 1 7 acanthosis nigricans, 1 14, l l 4f age-related skin changes, 1 1 7 dermatofibroma, 1 1 6, 1 1 6f epidermal inclusion cysts, 1 1 6, l 1 6f eyelid lesions, 1 1 6, l 1 6f hidradenitis suppurativa, 1 1 6, l l 7f ichthyosis vulgaris, 1 1 7, l l 7f lichen planus, 1 1 4, l l 4f pityriasis rosea, 1 1 5 , l l 5f rosacea, 1 1 4-1 1 5, 1 1 5f stasis dermatitis, 1 1 4, l l 4f and sun protection, 1 1 7 sunburn, 1 1 7 vitiligo, 1 1 5-1 1 6, l l 5f neoplasms of skin, 1 1 7- 1 2 2 actinic keratosis, 1 1 8, l l 8f basal cell carcinoma, 1 1 9, l 20f cherry angiomas (hemangiomas), 1 22, 1 22f cutaneous squamous cell carcinoma, 1 1 8- 1 1 9, 1 1 9f Kaposi sarcoma, 1 2 1 , ! Z if melanoma, 1 1 9- 1 20, 1 20/ mycosis fungoides ( cutaneous T-cell lymphoma), 1 2 1 - 1 22, ! Z if

INDEX necrobiosis lipoidica, 1 22, 1 22( pyogenic granuloma, 1 22, 1 22( seborrheic keratosis, 1 1 7-1 1 8, 1 1 8( Dermatomyositis (OM), 3 32-3 3 3 , 3 3 21, 3 3 3 1 Dermatophyte infections, I 09- 1 10, I 09{, I ! Of Dermatosis( es) blistering, 97{, 971 perianal, 582, 5821 Dermis, 1 03( DES (diethylstilbestrol), pregnancy, 4341 Descriptive statistics, 1 69 Desmopressin, 277 Desmopressin acetate replacement test, 1 44 Developing follicle, 468( Developmental disorder, intellectual, 588 Developmental dysplasia of the hip, 57 5-576, 575( Developmental milestones, 5 1 1 , 5 1 21, 5 1 31 DEXA (dual-energy x-ray absorptiometry) scan, 1 3 8, 1 741 DH (dermatitis herpetiformis), 98, 1 00{, 223, 223{, 224 Diabetes insipidus (DI), 142- 1 44 central, 142, 1 44, 1 441 diagnosis, 14 3-144, 1 44{, 1 441 history/physical examination, 14 3 hypothalamic-pituitary axis, 142, 1 42( nephrogenic, 142, 1 44, I 441 treatment, 1 44 Diabetes mellitus (OM), 1 24- 1 2 7 chronic complications, 1 24, 1 2 5{, 1 2 5 1 classification, 1 24, 1 26 diagnosis, 12 5 general health maintenance, 1 261 gestational, 439, 440-44 1 history, 1 24 management, 1 26--1 27, 1 27{, 1 281 maturity-onset diabetes of the young, 1 26 polycystic ovarian syndrome, 48 3 pregestational, 439-440, 4401 pregnancy, 439-44 1 , 4401 congenital heart disease, 5291 screening, 1 741, 1 7 51 vaccines, 1 7 3( Diabetic cardiomyopathy, 451 Diabetic ketoacidosis (OKA), 1 27-1 29, 1 291 Diabetic nephropathy, 1 2 51, 6941 Diabetic neuropathy, 1 2 51 Diabetic retinopathy, 1 2 5{, 1 2 51, 42 1 , 42 1( Diagnostic studies, assessment, 1 5 8- 1 60 likelihood ratio, 1 60 positive and negative predictive values, 1 59-1 60 sensitivity and specificity, 1 58-1 59, 1 5 8{, 1 59( Dialysis, 684, 686 Diamond-Blackfan anemia, 564-565, 5641 Diaper dermatitis, Candida, 5821 Diaper rash, 1 09 Diaphragm blunt abdominal trauma, 7621 contraceptive, 4801 rupture, 76 1 unilateral paralysis, 760, 76 1( Diaphragmatic hernia, congenital, 5 2 1 1

Diarrhea, 220-223, 22 1 1-2221 acute, 220, 223 bile acid, 247 bloody, 220 chronic, 220, 22 3 diagnosis, 220 history/physical examination, 220 infectious agents, 220, 22 1 1-2221, 223 mechanisms, 220, 2 2 1 t-2221 treatment, 223 watery, 220 Diastolic heart failure, 34, 3 51 Diastolic murmurs, 22, 22(-24( DIC (disseminated intravascular coagulation), 280-2 8 1 , 2801, 28 31, 2841 Diethylstilbestrol (DES), pregnancy, 4341 Dieulafoy lesion, 2 1 3 , 2 1 3( Diffuse axonal injury (DAI), 7 5 8, 759 Diffuse large B-cell lymphoma, 3051 Diffuse parenchymal lung disease (DPLD), 632-63 3, 6 3 3( DiGeorge syndrome, 5 3 3 , 5431 Digital rectal exam (DRE), 1 7 51 Digitalis, toxic ingestion/overdose, 745t Digoxin congestive heart failure, 39 adverse effects, 7481 toxicity, 39 Digoxin effect, 39 Dilated cardiomyopathy (DCM), 40t, 4 1 , 4 lf Dipeptidyl peptidase (DPP)-4 inhibitors, diabetes, 1 281 Diphenhydramine, adverse effects, 748t Diphtheria, tetanus, and acellular pertussis (DTaP) vaccine, 1 7 1{, 5 54 Direct Coombs test, 292, 292( Direct hernia, 244, 24 31 Direct thrombin inhibitors, 272t Direct-acting oral anticoagulants (DOACs), 270, 2731 deep venous thrombosis, 79 Disabled patients, communication, 1 82 Disclosure, full, 1 9 1 - 1 92 Discounted fee-for service insurance, 1 78 Disease frequency, assessment, 1 5 8 Disease-modifying antirheumatic drugs (DMARDs), 3 3 0 Disk herniation, 349, 349( Diskitis, 327( Dislocation hip, 3 1 4, 3 1 61 shoulder, 3 1 41 Displacement, 6091 Disruptive behavioral disorders, 587-588 Disruptive mood dysregulation disorder (DMDD), 5 87-588 Disseminated gonococcal infection, 7 1 2, 7 1 2( Disseminated intravascular coagulation (DIC), 280-28 1 , 2801, 2831, 284t Dissociative amnesia, 593, 594t Dissociative disorders, 593, 594t Dissociative identity disorder, 593, 594t Distal esophageal spasm, 207-208, 208( Distributive shock, 7 1 81 Disulfiram, 6 1 3

Diuretics, 687, 687{, 6881 congestive heart failure, 38, 3 81, 39 hyp ertension, 60t-6 l t valvular heart disease, 72 Diverticular bleeding, 2 3 3 Diverticular disease, 2 3 2-2 3 3 , 2 3 3( Diverticulitis, 2 3 2-2 3 3 acute, 2 3 3( acute abdomen, 227 Diverticulosis, 2 3 2-2 3 3 , 2 3 3( Diverticulum(a), 2 3 2 esophageal, 209, 209( Meckel, 5 36-5 37, 5 3 6( Dix-Hallpike maneuver, 377 Dizygotic twins, 448 OKA (diabetic ketoacidosis), 1 27- 1 29, 1 291 DLB (dementia with Lewy bodies) , 3 9 1 1, 395 OM (dermatomyositis), 3 3 2-3 3 3 , 3 3 21, 3 3 31 OM (diabetes mellitus) . See Diabetes mellitus (OM) DMARDs (disease-modifying antirheumatic drugs), 3 3 0 D M D (Duchenne muscular dystrophy), 572-574, 574t DMDD (disruptive mood dysregulation disorder), 5 87-588 Do not intubate (DNI) orders, 1 90 Do not resuscitate (DNR) orders, 1 90 DOACs (direct-acting oral anticoagulants), 270, 273t deep venous thrombosis, 79 Doctor-patient professional relationship, 1 86 Dog bites, 742t DOPAMINE RASH mnemonic, 3 3 5 Dopamine-blocking agents, Tourette syndrome, 589 Dopamine-depleting agents, Tourette syndrome, 589 Dorsal column, 3 5 8t, 3 6 1( Double depression, 604 Double Y males, 5 l 7t Double-blind studies, 1 64 Down syndrome, 5 1 6t congenital heart disease, 5291 intellectual disability, 588 neoplasms, 7 5 lt Downward transtentorial herniation, 3 5 3 Doxorubicin, adverse effects, 7481 DPLD (diffuse parenchymal lung disease), 632-63 3, 6 3 3( OPP ( dipeptidyl peptidase )-4 inhibitors, diabetes, l 28t DRE (digital rectal exam), 1 7 51 Driving pressure, 640t Drop arm sign, 340, 340t Drowning, 740 Drug abuse. See Substance use disorders Drug companies, gifts from, 1 9 5- 1 96 Drug eruption, 94, 94( Drug adverse effects, 7471-7491 Drug-drug interactions, 746, 7461 Drug-induced hepatitis, 2 5 1 Drug-induced systemic lupus erythematosus, 335 Drusen, 4 1 9, 4 1 9(

INDEX Dry gangrene, 1 1 3 DT(s) (delirium lremens), 6 1 3, 6 1 3t DTaP (diphtheria, tetanus, and acellular pertus­ sis) vaccine, 1 7 1{, 5 54 Dual-energy x-ray absorptiometry (DEXA) scan, 1 3 8, 1 741 Dubin-Johnson syndrome, 523t Duchenne muscular dystrophy (DMD), 572-574, 574t Ductal carcinoma in situ (DCIS), 493, 494 Ductal hyperplasia, usual, 493 Ductus arleriosus, patent, 2 3{, 5 3 1 , 53 lf myocardial infarction involving, 52, 52f Duke criteria, endocarditis, 70, 70t Dumping syndrome, 220 Duodenal alresia, 522t Duodenal hemaloma, 228-229 Duodenal ulcers, 2 1 5 , 2 1 5t, 2 1 6-2 1 7, 2 1 6f Duodenum, blunt abdominal trauma, 7621 Dupuytren conlracture, 343 Dura maier, 3 5 5, 3 5 5f Durable power of attorney for healthcare, 1 90 Dure! hemorrhage, 3 54f DVT (deep venous thrombosis), 78-80, 78t, 79f poslamputation, 7 5 7 DVT (deep venous thrombosis) prophylaxis, pulmonary embolism, 64 5 Dysaulonomia, parkinsonism, 398 Dysgerminoma, 5051 Dyskinesia, 5931 lardive, 593/ Dyslipidemia, 5 5-57, 561, 57t Dysmenorrhea primary, 474 secondary, 474-47 5, 47 5t Dyspepsia, 2 1 4 Dysphagia, 203-204, 204f Dysplastic nevi, multiple, neoplasms, 7 5 1 1 Dysraphism, spinal, 560-562 Dysthymia, 6021, 604-60 5 Dystonia, acute, 593t E Ear(s) glue, 42 3-424 penetrating trauma, 7 5 5 "swimmer's," 424 "Ear stones," 377 Eating disorders, 6 1 4-6 1 6 anorexia nervosa, 6 1 4-6 1 5, 6 1 51 bulimia nervosa, 6 1 5t, 6 1 6 EBY. See Epstein-Barr virus (EBV) EC (emergency contraception), 478, 48 1 1 sexual assault, 622 ECG. See Electrocardiogram (ECG) Echinococcus granulosus diarrhea, 2 2 1 t hydatid cyst, 264 Echolalia, 589 Eclampsia, 443-444, 443t Ecstasy, substance abuse, 6 1 2t ECT (electroconvulsive therapy), depression, 603 Ecthyma, 1 04

Ectopic pregnancy, 445, 445f ruptured, 227 Eczema, 90-9 1 , 90{, 9 If nummular, 98, 98f ED (erectile dysfunction), 70 1 -702 Edema, 2 3 cerebral, high-altitude, 740 pulmonary, 2 3 high-altitude, 740 EDTA (ethylenediaminelelraacetic acid), pseudothrombocytopenia, 284 Edwards syndrome, 5 1 61 Effect, measures of, 1 60- 1 6 1 , 1 6 lf Effect modification, 1 67 EGPA (eosinophilic granulomalosis with poly­ angiitis), 299-300, 3 3 7{, 690/ Ehrlichiosis, 383, 722 Ejection fraction heart failure with preserved, 34, 3 51, 39-40 heart failure with reduced, 34, 3 5f-37f, 36-39, 3 7t, 38/ Elastic pressure, 6401 Elastosis, solar, 1 1 7 Elbow, nursemaid's, 5731 Elder abuse/neglect, confidentiality, 1 9 3 Electrical alternans, 6 6 , 66f Electrical burns, 737, 738/ Electrocardiogram (ECG), 1 8-22 axis, 1 8, 1 9{, I 9t chamber enlargement, 2 1-22, 2 lf intervals in, 20, 20f ischemia/infarction, 2 1 , 2 lf normal, 1 8, I Sf rate, 1 8 rhythm, 1 8 Electroconvulsive therapy (ECT), depression, 603 Electrolyte disorders, 676-682 h yp ercalcemia, 680-68 1 , 68lf h yp erkalemia, 678-679, 679{, 680 h yp ernatremia, 676 h yp ocalcemia, 681 -682 h yp okalemia, 679, 679{, 680f h yp omagnesemia, 682 h yp onatremia, 676-678, 677{, 678f EM (erythema multiforme), 95, 9 5f Embolism cholesterol, 230 pulmonary, 644-645 angina pectoris, 48 diagnosis, 644-64 5, 644{, 64 51 history/physical examination, 279, 644, 644t treatment, 645 , 646f Embolus(i) saddle, 644f septic, endocarditis, 69 Embryonal carcinoma, 5051 Embryonic age, 428 Emergency airway, 7 5 2 Emergency contraception ( E C ) , 47 8 , 48 1 t sexual assault, 622 Emotion(s) challenging conversations, 1 8 1 delivering news, 1 92 Emotion-seeking skills, interviewing, 1 80

EmpagliAozin congestive heart failure, 39 diabetes, 1 281 Empathy interviewing, 1 80 rapport, 1 80/ Emphysema, 630-63 1 , 630t, 63 1{, 6 3 l t Emphysematous cholecystitis, 244 Emphysematous pyelonephritis, 709, 709f Empyema, 666, 6671 Encephalitis, 3 82-3 83, 383f Encephalopathy, 3 7 1-372, 372t hepatic, 2 52, 2 5 3t, 2 54, 2 5 5t Wernicke, 4 1 1 I Enchondroma, 324/ Endocarditis, 67-7 1 infective, 2 3 , 67-7 1 antibiotic prophylaxis, 7 1 t, 72 diagnosis, 70, 70t etiologies, 68-69, 68t history/physical examination, 69, 69f treatment, 70, 71 I Libman-Sacks, 3 36 non-bacterial thrombotic, 67, 68/ Endocervical curettage, 50 I Endocrine changes, pregnancy, 4 30{, 4 30/ Endocrinology, 1 2 3- 1 5 5 adrenal gland disorders, 148- 1 54 adrenal anatomy, regulatory control, and secretory products, 1 48, l 48f adrenal insufficiency, 148- 1 49, 1 49{, 1 491 congenital adrenal hyperplasia, 1 54, 1 541 Cushing syndrome, 1 50-1 5 1 , 1 5 1{, 1 52{, 1 5 31 hyperaldosleronism, 15 3 pheochromocytoma, 1 50, l 50f bone and mineral disorders, 1 3 7-1 4 I calcium and phosphate regulation and, 1 37, 1 3 8f hyperparathyroidism, 1 39-1 4 1 , 140/ osteoporosis, 1 37-1 39, l 38f Paget disease of bone, 1 39, 1 3 9{, l 40f disorders of glucose metabolism, 1 24-1 30 diabetes mellitus, 1 24-1 27, 1 2 5{, 1 2 51, 1 2 61, 1 27{, l 28t diabetic ketoacidosis and hyperglycemic hyperosmolar syndrome, 1 27-1 29, 1 291 hypoglycemia, 1 29- 1 3 0 metabolic syndrome, 1 24 multiple endocrine neoplasias, 1 5 5, l 5 5f pituitary and hypothalamic disorders, 1 4 1 - 1 47 acromegaly, 1 44-1 46, 145{, l 46f deficiency of pituitary hormones, 1 4 1 - 1 42, 143t diabetes insipidus, 1 42- 1 44, 1 42{, 1 44{, 1 441 excess of pituitary hormones, 1 44- 1 47 hyperprolactinemia, 1 46-1 47, 1 47f hypothalamic-pituitary axis and, 1 4 1 , 14 lf syndrome of inappropriate antidiuretic hormone secretion, 14 7 reproductive, 48 1-485 congenital adrenal hyperplasia, 48 1-483, 482{, 483/

INDEX polycystic ovarian syndrome, 483-484, 484{ thyroid disorders, 1 30-1 3 7 hyperthyroidism and thyrotoxicosis, 1 30-1 34, 1 32(, 1 3 2t-l 34t, 1 3 3{ hyp othyroidism, 1 32t, 1 34-1 3 5 thyroid neoplasms, 1 36, 1 36(, I 3 7t thyroid physiology and, 1 30, 1 3 If, I 32t thyroiditis, 13 5 Endodermal sinus tumor, 706t End-of-life issues, 1 89-1 9 1 advance directives, 1 89 euthanasia and clinician-assisted suicide, 1 90-1 9 1 futile treatment, 1 9 1 hospice care, 1 9 1 surrogate decision making, 1 90 withdrawal of life-sustaining treatment, 1 90 Endometrial biopsy, 476, 478{ Endometrial cancer, 498-499, 499(, 499t Endometrial sinus tumor, 505t Endometriosis, 474-47 5, 47 5t Endometritis, 7 1 9 postpartum, 462-463 Endoscopic biopsy, Helicobacter pylori, 2 l 5t Endotracheal intubation, emergency airway, 7 52, 7 52(, 7 5 3 End-stage renal disease (ESRD), 684 anemia, 287 vaccines, 1 7 3{ Enoxaparin, 272t unstable angina/NSTEMI, 50 Entamoeba histolytica, diarrhea, 222t Enterobacteriaceae, osteomyelitis, 327t Enterobius vermicularis, 5 5 5 Enterococcus spp endocarditis, 68, 68t, 7 1 t urina ry tract infection, 708t Enterocolitis, necrotizing, 5 38, 5 3 8{ Enteropathic spondylitis, 3 3 1 Entry inhibitors, HIV, 7 3 3t Enuresis, 542 Environmental conditions, 737-742 bites and stings, 740, 741(, 74 l t-742t burns, 73 7-740, 738(, 738t, 739{ drowning, 740 high-altitude sickness, 740 Eosinopenia, 298, 298t Eosinophilia, 299-300, 299t Eosinophilic esophagitis, 206, 206{ Eosinophilic granulomatosis with polyangiitis (EGPA), 299-300, 3 3 7(, 690t Eosinophilic pneumonia, chronic, 299-300 Eosinophilic pulmona ry syndromes, 63 5-636 Ependymoma, 560t Epidemiology, 1 57-1 76 assessment of diagnostic studies, 1 5 8- 1 60 likelihood ratio, 1 60 positive and negative predictive values, 1 59-1 60 sensitivity and specificity, 1 5 8-1 59, 1 5 8(, 1 59{ assessment of disease frequency, 1 5 8 evaluating clinical studies, 1 65-169 bias, 1 6 5-167, 1 67{

commonly used statistical tests, 1 69, 1 69{ scenarios, 1 6 7-1 68 statistical testing, 1 67 measures of effect, 1 60- 1 6 1 , 161{ person-time estimate, 1 5 8 prevention, 1 70 reportable diseases, 1 7 5 , l 75t screening recommendations, 1 74, I 74t, I 75t survival curves, 161, 1 6 1{ types of clinical studies, 1 6 1 - 1 6 5 case-control study, 1 63-164, 1 64(, 165t cohort study, 1 60, 1 62-163, 1 64(, 165t cross-sectional study, 1 62, 1 64(, 165t phases of clinical trials, 1 64, 1 66t randomized controlled trial, 1 64, 165t and strength of evidence, 16 1-1 62, 1 62{ vaccination, 1 70-1 74 COVID- 19, 1 7 3- 1 74 recommended schedules, 1 70- 1 7 3 , I 7 1 f-l 73f types of vaccines, 1 70, l 70t Epidermal inclusion cysts, 1 1 6, 1 1 6{ Epidermis, 1 0 3{ Epididymitis, 699, 700 Epidural block, 45 5-456 Epidural hematomas, 369, 7 5 8, 758{ Epidural hemorrhage, 369, 370t Epigastric hernia, 243, 243t Epiglottitis, 5 50t, 5 5 1-552, 5 5 1 t, 5 52(, 5 5 3{ Epilepsy acquired, 373-374 idiopathic, 373 Episiotomy, 46 1 Epispadias, 54 If, 542, 542t Epistaxis, 67 1 , 67 1{ Epley maneuver, 377 Epstein-Barr virus (EBV) HIV, 729t, 730t Hodgkin lymphoma, 306 infectious mononucleosis, 724-725 lymphocytosis, 724 Eptifibatide, 272t ER ( estrogen receptor) status, breast cancer, 495, 496 Erectile dysfunction (ED), 70 1-702 Errors, 1 84, I 84t analyzing, 18 5 ERV (expiratory reserve volume), 626{ E rysipelas, 1 03(, 1 04-105, 1 04{ E rythema infectiosum, 5 56t Erythema migrans, 722, 723, 723{ Erythema multiforme (EM), 95, 95f Erythema nodosum, 96, 96{ Erythema toxicum, 90 Erythema toxicum neonatorum, 528t Erythematotelangiectatic rosacea, 1 14 E rythrocytosis, relative, 295 E rythroplakia, 1 99(, 200 E rythroplasia of Queyrat, 1 1 8

Escherichia coli

diarrhea, 222t meningitis, 379t prostatitis, 7 1 0 urinary tract infection, 708t Esophageal cancer, 2 1 1

Esophageal candidiasis, 1 09 Esophageal disease, 203-2 1 1 achalasia, 208-209, 208{ distal esophageal spasm, 207-208, 208{ dysphagia/odynophagia, 203-204, 204{ eosinophilic esophagitis, 206, 206{ esophageal cancer, 2 1 1 esophageal diverticula, 209, 209{ esophageal rings, 207, 207{ gastroesophageal reflux disease (GERO), 209-2 1 0, 209(, 2 1 0{ hiatal hernia, 2 1 lf, 2 1 1 infectious esophagitis, 204-205, 205t pill (medication-induced) esophagitis, 20 5-206, 206{ Plummer-Vinson syndrome, 207 Esophageal diverticula, 209, 209{ Esophageal dysphagia, 204 Esophageal perforation acute abdomen, 226 angina pectoris due to, 48 Esophageal rings, 206(, 207 Esophageal spasm, distal, 207-208, 208{ Esophageal varices, 2 1 2(, 2 1 2-2 1 3 cirrhosis, 2 5 5t Esophageal webs, 204, 207{ Esophagitis Candida, 109, 204, 205, 205t HIV, 730t cytomegalovirus, 205t, 7 3 5 eosinophilic, 206, 206{ infectious, 204-205, 205t pill (medication-induced), 20 5-206, 206{ Esophagus, Barrett, 2 1 0, 2 1 Of neoplasms, 7 5 l t ESRD ( end-stage renal disease), 684 anemia, 287 vaccines, 1 7 3{ Essential thrombocytopenia, 295 Essential tremor, 402t, 4 1 0 Estriol, pregnancy, 432t Estrogen, topical, menopause, 486 Estrogen receptor (ER) status, breast cancer, 495, 496 Ethics, 1 8 5- 1 86 doctor-patient professional relationship, 1 86 doctor-patient sexual relationship, 1 86 general/core principles, 1 8 5- 1 86 Ethylene glycol presentation, 682 toxic ingestion, 745t Ethylenediaminetetraacetic acid (EDTA), pseudothrombocytopenia, 284 Euthanasia, 1 90- 1 9 1 Euthyroid sick syndrome, I 3 2 t Evidence, strength of, 1 6 1-162, 1 62{ Evidence-based interviewing, 1 79-1 80 Ewing sarcoma, 3 2 3 , 324, 3 2 5(, 5 7 1 , 572t Exanthems, viral, 5 5 5, 5 5 6t-5 57t Excoriation disorder, 598t Exenatide, diabetes, 1 28t Exercise, pregnancy, 4 30t Exhibitionistic disorder, 6 1 7t Expirato ry reserve volume (ERV), 626{ Exposure, advanced trauma life support, 7 5 3

INDEX Expressive aphasia, 372-37 3 , 373{ External cephalic version, 460 External hemorrhoids, 240t External validity, 1 6 3 Extracranial injuries, neonatal, 5 2 6 , 527t Extraperitoneal bladder inju ry, 764 Extrapyramidal symptoms, 592t, 593t Extremities, penetrating trauma, 757 Extrinsic pathway, 270, 271{ Exudate, 664, 665(, 665t, 666 Eye(s), penetrating trauma, 7 54 Eyelid lesions, 1 1 6, 1 1 6{ F Fab ry disease, 5 I St Facial lacerations, 754 Facial nerve palsy, 3 57, 3 5 7(, 3 5 7t Facial trauma blunt and deceleration, 7 5 7-759, 7 5 8{ penetrating, 7 54 Facilitation, interviewing, 1 80 Factitious disorders, 62 1 Factor V Leiden, 278 Factor Xa inhibitors, 272t Factorial design, 1 64 Failure mode and effects analysis, 1 8 5 Failure to progress, labor and delive ry, 459, 459t Failure to thrive (FTI), 5 1 3- 5 1 4 Faix cerebri, 3 54{ Familial adenomatous polyposis, 2 3 7t Familial short stature, 5 1 3 Fanconi anemia, 289-290, 564t, 565-566, 565t Farsightedness, 4 1 6 FA S (fetal alcohol syndrome), 434t, 588 Fasciculus cuneatus, 361{ Fasciculus gracilis, 36 1{ Fasciitis, necrotizing, 1 0 3(, 1 0 5 , 1 0 5{ FAST (focused abdominal sonography for trauma), 7 5 3 , 762 Fat necrosis, breast, 492 Fatigue, healthcare personnel, 1 84- 1 8 5 Fatty liver disease nonalcoholic, 2 5 9 pregnancy, 445 Febrile nonhemolytic reaction, blood transfu­ sion, 296t Febrile seizures, 5 5 8-559 Fee-for service insurance, 1 78 discounted, 1 7 8 Felty syndrome, 297, 3 30 Female condom, 480t Female development normal, 468 sexual, 5 1 5{ Female sexual interest/arousal disorder, 507 Femoral fracture, 3 1 7t Femoral head necrosis, 343, 343(, 344 Femoral hernia, 24 3t Femoral nerve inju ry, 32 l t Fertility awareness methods, 480t Fetal alcohol syndrome ( FAS), 434t, 588 Fetal aneuploidy, screening, 43 1 t, 432t

Fetal breathing movements, 4 5 3 t Fetal growth restriction, 449 Fetal heart rate (FHR) monitoring, 452, 452(, 452t, 4 5 5 , 456t Fetal heart tones, 4 28 Fetal macrosomia, 449 Fetal malpresentation, 460-46 1 , 460{ Fetal movement, 428, 452, 4 5 3 t Fetal surveillance, antepartum, 4 5 1 -454, 452(, 452t, 4 5 3t Fetal thyrotoxicosis, 1 32 Fetal tone, 4 5 3 t Fetishistic disorder, 6 1 7t FEY 1 (forced expiratory volume in I second) , 626, 626t Fever, 7 1 9-72 1 "breakbone," 722 neutropenic, 297-298 postoperative, 7 I 9, 7 I 9f, 7 I 9t postpartum, 464 rheumatic, 662 Rocky Mountain spotted, 3 83 , 72 3-724, 724{ sepsis, 720-72 1 of unknown origin, 7 1 9-720 FFP (fresh frozen plasma), 274t FHR (fetal heart rate) monitoring, 452, 452(, 452t, 4 5 5 , 456t Fibrates, 57t Fibrillation atrial, 2 5-3 3, 3 3t ventricular, 3 l t, 3 3t Fibrin mesh, 270 Fibroadenoma, 492 Fibrocystic changes, breast, 492 Fibroids, 497-498 Fibromyalgia, 3 38, 3 38t Fifth disease, 5 56t Fine motor development, 5 I 2t Finger clubbing, 2 3 FiO 2 (fraction o f inspired oxygen), ventilator setting, 639 First-degree AV block, 26t Fisher's exact test, 1 69 Fistula anorectal, 240t tracheoesophageal, 5 2 1 t Fitz-Hugh-Curtis syndrome, 7 1 2{ Flail chest, 639, 760, 761{ "Flapping tremor," 402t Flow murmur, 22 Fluconazole, adverse effects, 747t Fluency disorder, childhood-onset, 5 l 3t Fluid restriction, congestive heart failure, 38 Fluorescent treponemal antibody absorption (FTA-ABS), 7 1 4t Fluoroquinolones, adverse effects, 748t Flutter, atrial, 28t, 3 3t Focal nodular hyperplasia (FNH), 262, 262{ Focal segmental glomerulosclerosis (FSGS), 693t Focal seizures, 374, 3 7 5 , 3 7 5{ complex, 374, 3 7 5{ simple, 374, 3 7 5{ Focused abdominal sonography for trauma (FAST), 7 5 3 , 762 Focused expression, interviewing, 1 80

Folate deficiency, 293-294, 293(, 4 1 I t, 7 50t neoplasms, 7 5 I t type A gastritis, 2 1 4-2 1 5 Folie a deux, 5 9 1 t Follicle-stimulating hormone (FSH) deficiency, 143t Follicular lymphoma, 305t Follicular ovarian cysts, 498t Follicular phase of menstrual cycle, 468-469, 468{ Follicular thyroid carcinoma, I 37t Folliculitis, 1 0 3(, 1 0 5- 1 06, 1 06{ "hot tub," I 0 5 Food protein-induced allergic proctocolitis (FPIAP), 5 3 8- 5 3 9 Food-borne disease, reportable, I 76t Foot common adult orthopedic injuries, 3 1 St zones, 3 I 9, 3 I 9{ Footling breech presentation, 460, 460{ Forced expirato ry volume in I second (FEY 1 ) , 626, 626t Forced vital capacity (FVC), 626, 626t Forearm, common adult orthopedic injuries, 3 I 5t Foreign body aspiration, 549 Foreign objects, pediatric vaginal discharge, 49 1 Fothergill sign, 764 4Ts score, 279 Fournier gangrene, I 0 5 FPIAP (food protein-induced allergic proctocoli­ tis), 5 3 8-539 Fraction of inspired oxygen (FiO2 ) , ventilator setting, 6 3 9 Fracture(s) ankle, 3 1 8t boxer's, 3 1 6t calcaneal stress, 3 1 St child abuse, 578t clavicular, 3 l 4t pediatric, 573t Colles, 3 1 5t compartment syndrome, 341 femoral, 3 l 7t Galeazzi, 3 1 5t greenstick, 573t hip, 3 1 4, 3 1 7t humerus, 3 1 4t supracondylar, 573t Le Fort, 759 metatarsal stress, 3 I St Monteggia, 3 l 5t "nightstick," 3 I 5t orbital, 754 blowout, 422 pediatric, 572, 573t Salter-Harris classification, 3 I 9-320, 320(, 573t pelvic, 763 posterior rib, child abuse, 578t scaphoid, 3 I 6t skull basilar, 7 5 8 linear, 7 5 8 spiral, child abuse, 578t

INDEX tibial stress, 3 l 8t torus, 573t Fragile X syndrome, 5 l 9t, 587, 588 Frank breech presentation, 460, 460f Fraternal twins, 448 FRC (functional residual capacity), 626{, 626t Fresh frozen plasma (FFP), 2741 Friedrich ataxia, 5 l 9t Frontal eye field, 3 5 3f Frontal lobe, 3 5 3f Frontotemporal dementia (FTD), 390t, 393 Frotteuristic disorder, 6 1 71 "Frozen shoulder," 3 3 9 Fructose intolerance, 224 hereditary, 5 l 9t FSGS (focal segmental glomerulosclerosis), 693t FSH (follicle-stimulating hormone) deficiency, 143t FTA-ABS (fluorescent treponemal antibody absorption), 7 14t FTD (frontotemporal dementia), 390t, 393 FIT (failure to thrive), 5 1 3-5 1 4 Fuels, substance abuse, 6 1 l t Fulminant hepatic failure, 2 5 6-2 57 Fulminant hepatic necrosis, 2 56-2 57 Fulminant hepatitis, 2 56-2 57 Functional neurologic symptom disorder, 620-62 1 Functional residual capacity (FRC), 626{, 626t Functional tremor, 402t Fundal height, 428 Fungal infections geographic distribution of systemic, 6 5 5f opportunistic pulmonary, 65 5t skin, 1 08-1 1 0 candidiasis, 1 08-1 09, 1 09f dermatophyte infections, 1 09- 1 10, 1 09{, I !Of sporotrichosis, 1 1 0 tinea versicolor, I 08, 108f Fungal sinusitis, 662 Furosemide, adverse effects, 748t Furuncles, 1 0 5 Futile treatment, 1 9 1 FVC (forced vital capacity), 626, 626t G G6PD (glucose-6-phosphate dehydrogenase) deficiency, 291 GA (gestational age), 428 GAD (generalized anxiety disorder), 594-595, 595t Gait ataxia, normal-pressure hydrocephalus, 394, 397 Galactocele, 466 Galactosemia, classic, 5 l 9t Galan! reflex, 5 l 3t Galeazzi fracture, 3 l 5t Galeazzi sign, 5 7 5 Gallbladder, porcelain, 244 Gallops, 22 Gallstone(s), 244-248 cholangitis, 245t, 246

cholecystitis, 244-246, 245{, 245t choledocholithiasis, 245t, 246 cholelithiasis and biliary colic, 244, 244{, 245t Gallstone ileus, 225, 246-247 Gamma-hydroxybutyric acid (GHB), substance abuse, 6 1 2t Ganglion cyst, 342-343, 343f Gangrene, 1 1 3 , l I 3f Fournier, 1 0 5 Gardner syndrome, 2 3 8 GAS (group A Streptococcus) acute pharyngitis, 66 1 -662, 66 1{, 66 l t pediatric vaginal discharge, 4 9 I Gas gangrene, 1 1 3, I 1 3f Gastrectomy, sleeve, 2 1 9 Gastric band, adjustable, 2 1 9 Gastric bezoar, 2 1 8-2 1 9 Gastric bypass, roux-en-Y, 2 1 9 Gastric cancer, 2 1 6, 2 I 6f Gastric remnants, postsurgical, neoplasms, 7 5 l t Gastric ulcers, 2 1 5, 2 1 5t, 2 1 6-2 1 7, 2 1 6f Gastric varices, 2 1 2-2 1 3 Gastrinoma, 267 Gastritis, 2 1 4-2 1 6, 2 1 5{, 2 1 5t chronic atrophic, neoplasms, 75 l t Gastroenterologic disease, pediatric, 5 34-540 constipation, 5 3 9-540 food protein-induced allergic proctocolitis, 5 3 8-5 3 9 Hirschsprung disease, 5 37, 5 3 7f intussusception, 5 3 5-536, 5 3 5f malrotation with volvulus, 5 36, 5 3 6f Meckel diverticulum, 5 3 6-537, 5 3 6f necrotizing enterocolitis, 5 3 8, 5 3 8f pyloric stenosis, 5 34-5 3 5 , 5 34f Gastroesophageal reflux disease (GERO), 209-2 1 0, 209{, 2 1 0f angina pectoris, 48 Gastrointestinal (GI) bleeding, 2 1 1-2 1 3, 2 1 2{,

2 1 2t, 2 1 3f

Gastrointestinal (GI) changes, pregnancy, 429t Gastrointestinal (GI) medicine, 1 97-268 anorectal disease, 240t, 241 biliary disease, 244-248 biliary cyst, 24 7 cholangiocarcinoma, 247-248 cholangitis, 245t, 246 cholecystitis, 244-246, 245{, 245t choledocholithiasis, 245t, 246 cholelithiasis and biliary colic, 244, 244{, 245t gallstone ileus, 246-247 postcholecystectomy syndrome, 244, 24 7 cytomegalovirus, 73 5 esophageal disease, 203-2 1 1 achalasia, 208-209, 208f distal esophageal spasm, 207-208, 208f dysphagia/odynophagia, 20 3-204, 204f eosinophilic esophagitis, 206, 206f esophageal cancer, 2 1 1 esophageal diverticula, 209, 209f esophageal rings, 207, 207f gastroesophageal reflux disease, 209-2 1 0, 209{, 2 1 0f

hiatal hernia, 2 1 1{, 2 1 1 infectious esophagitis, 204-205, 205t pill (medication-induced) esophagitis, 20 5-206, 206f Plummer-Vinson syndrome, 207 gastrointestinal bleeding, 2 1 1 -2 1 3 , 2 1 2{, 2 1 2t, 2 1 3f hernias, 243-244, 243t inflammatory bowel disease, 24 1 , 241{, 242{, 242t large bowel disorders, 2 3 1-2 4 1 Clostridium difficile colitis, 2 3 1 -2 3 2 colorectal cancer, 2 36-2 37, 236{, 236t, 2 3 7t colorectal cancer-associated conditions, 2 3 7-2 38, 2 3 8f diverticular disease, 2 3 2-2 3 3 , 2 3 3f irritable bowel syndrome, 2 34-2 36 ischemic colitis, 2 3 8-2 39, 239f large bowel obstruction, 2 34, 234t, 2 3 5f microscopic colitis, 240-241 liver disease, 248-264 and abnormal liver function tests, 248, 248{, 249f acute liver failure, 2 56-2 5 7 benign lesions, 262-264, 262{, 26 3f cirrhosis, 2 52-2 54, 2 52{, 2 5 3{, 2 5 3 t-2 5 5t hemochromatosis, 260-261 hepatic hydrothorax, 2 5 8 hepatitis, 249-2 52, 2 5 0t, 2 5 1{, 2 5 l t hepatocellular carcinoma, 2 59-260 hepatopulmonary syndrome, 2 5 7-2 58 hepatorenal syndrome, 2 5 5t, 2 57 ischemic hepatitis, 256 and liver transplantation, 261-262 nonalcoholic fatty liver disease, 2 59 primary biliary cholangitis, 2 59 primary sclerosing cholangitis, 2 59 spontaneous bacterial peritonitis, 2 52, 2 54-2 56, 2 5 5t, 2 5 6 transjugular intrahepatic portosystemic shunt procedure, 2 5 8 Wilson disease (hepatolenticular degen­ eration), 26 1 , 261f oral and salivary gland disease, 1 99-203 oral cancers, 20 I oral lesions, 1 99-20 I , 1 99{, I 99t salivary gland disease, 20 1 -203, 202t, 203{, 203t pancreatic disease, 264-267 pancreatic cancer, 267 pancreatic cysts, 264 pancreatic neuroendocrine tumors (PNETs), 266-267 pancreatitis, 264, 265t small bowel disorders, 220-2 3 1 acute abdomen, 226-228, 226t, 228f acute appendicitis, 2 30-2 3 1 , 2 3 1f carbohydrate maldigestion, 224 carcinoid syndrome, 224 diarrhea, 220-223, 22 1 t-222t duodenal hematoma, 228-229 ileus, 22 5-226 malabsorption/maldigestion, 22 3-224, 223f mesenteric ischemia, 229-2 30, 230t

INDEX Gastrointestinal (GI) medicine (Continued) small bowel obstruction, 224-22 5, 22 5(, 234( stomach and duodenal disorders, 2 1 4-220 bariatric surgery, 2 1 9-220 dyspepsia, 2 I 4 gastric bezoar, 2 I 8-2 1 9 gastric cancer, 2 1 6, 2 1 6( gastritis, 2 1 4-2 I 6, 2 1 5(, 2 1 51 gastroparesis, 2 1 7-2 1 8 Menetrier disease, 2 1 8 peptic ulcer disease, 2 1 6-2 1 7, 2 1 6( Zollinger-Ellison syndrome, 2 1 7 Gastrointestinal (GI) perforation, 226 Gastroparesis, 2 1 7-2 1 8 Gastroschisis, 520, 522t Gaucher disease, 5 1 8t CBS (group B streptococcus) meningitis, 379t pregnancy, 4 3 1 G C S (Glasgow Coma Scale), 7 5 2 , 7 521 Gemfibrozil, adverse effects, 7481 Gender dysphoria, 6 1 6-6 1 7 Gender-inclusive history taking, 1 8 1 Generalizability, 1 63 Generalized anxiety disorder (GAD), 594-595, 5951 Generalized seizures, 374, 3 7 5( Genetic disease, childhood-onset, 5 1 4-520 autosomal chromosome abnormalities (trisomies), 5 1 6t cystic fibrosis, 5 I 4-520 inherited metabolic disorders, 5 1 7t-5 1 8t other, 5 1 91 sex chromosome abnormalities, 5 1 71 Genital herpes, 98- 1 00, 99t, 1 00(, 7 1 5 , 7 1 5t-

7 1 6t

Genital lesions, 7 1 5 , 7 1 5t-7 1 6t Genital tract trauma, postpartum hemorrhage, 4631 Genital ulcers, 489 Genitopelvic pain disorder, 507 Genitourinary disease, 698-7 1 6 benign prostatic hyp erplasia, 702-703, 702t erectile dysfunction, 70 1-702 hydronephrosis, 698, 698( infectious, 706-7 1 1 prostatitis, 7 1 0-7 1 1 pyelonephritis, 708-7 1 0, 709( urinary tract infections, 706-7 1 1 , 707t, 7081 interstitial cystitis (painful bladder syn­ drome), 70 1 pediatric, 540-542 cryptorchidism, 541 hypospadias and epispadias, 54 If, 542, 5421 inguinal hernia, 541 vesicoureteral reflux, 540, 54 If scrotal pain and swelling, 698-700, 699( sexually transmitted, 7 1 1 -7 1 6 chlamydia, 7 1 1-7 1 2, 7 1 1(, 7 1 2( genital lesions, 7 1 5, 7 1 5t-7 1 6t gonorrhea, 7 1 2, 7 1 2( syphilis, 7 1 3-7 1 5 , 7 1 3(, 7 1 4(, 7 1 41

urinary incontinence, 700, 700t urologic cancer, 703-706 bladder, 704-705, 704( prostate, 702t, 703-704, 703( renal cell carcinoma, 705, 70 5( testicular, 70 5-706, 706t GERO (gastroesophageal reflux disease), 209-2 1 0, 209(, 2 1 0( angina pectoris, 48 Germ cell tumors, 706, 7061 Germinal matrix hemorrhage, 526 Gestation, multiple, 448 Gestational age (GA), 428 Gestational diabetes, 439, 440-441 Gestational h ypertension, 44 1-442 Gestational trophoblastic disease (GTD), 446, 446(, 4461 GFR (glomerular filtration rate), pregnancy, 4291 CH (growth hormone) deficiency, 1431 excess, 45t, 1 44- 1 46, 1 4 5( GHB (gamma-hydroxybutyric acid), substance abuse, 6 1 21 GI. See Gastrointestinal (GI) Giant cell arteritis, 3 36-3 3 7, 3 3 7( Giant cell tumor of bone, 3 2 3 , 324, 3241, 3 2 5( Gifts from drug companies, 1 9 5- 1 96 from patients, 1 9 5 Gilbert syndrome, 5231, 524 Gingival mucosa! laceration, 3 5 5( Glasgow Coma Scale (GCS), 752, 7521 Glaucoma, 4 1 7, 4 1 7(, 4 1 81 Glenohumeral osteoarthritis, 329 Glimepiride, diabetes, 1 281 Glioblastoma, 4041 Glipizide, diabetes, 1 281 Global payment, 1 78 Globe laceration, 7 54 Glomerular disease, 688-694 nephritic syndrome, 688-69 1 , 688(, 689t69 I t, 692( nephrotic syndrome, 692, 693t-694t Glomerular filtration rate (GFR), pregnancy, 4291 Glomerulonephritis, postinfectious, 688, 689t Glomerulosclerosis, focal segmental, 6931 Glucocorticoid biosynthesis pathway, 482( Glucose metabolic disorders, 1 24-1 30 diabetes mellitus, 1 24-1 27, 1 2 5(, 1 2 51, 1 26t, 1 2 7(, 1 281 diabetic ketoacidosis and hyperglycemic hy­ perosmolar syndrome, 1 27-1 29, 1 29t hyp oglycemia, 1 29- 1 30 metabolic syndrome, 1 24 Glucose-6-phosphate dehydrogenase (G6PD) deficiency, 29 1 a-Glucosidase inhibitors, diabetes, 1 281 Glue, substance abuse, 6 1 1 t Glue ear, 42 3-424 Glyburide, diabetes, 1 281 Glycoprotein Ilb/Illa inhibitors, 2721 GNAQ gene, 408 Goiter, toxic multinodular, 1 30 Gold, toxic ingestion, 7451

Gonadotropin-releasing hormone (GnRH), precocious puberty, 469-470, 4691 Gonadotropin-releasing hormone (GnRH) agonist stimulation test, 470 Gonococcal infection, disseminated, 7 1 2, 7 1 2( Gonococcal septic arthritis, 326, 3261 Gonorrhea, 7 1 2, 7 1 2( "Good Samaritan" laws, 1 96 Goodpasture syndrome, 3 3 7(, 6901 Gout, 346-348, 347(, 3471 GPA (granulomatosis with polyangiitis), 3 3 7(, 688, 6901 eosinophilic, 299-300, 3 3 7(, 5901 Graafian follicle, 468( Graft-vs.-host disease (GVHD), 309-3 1 0 Graft-vs.-leukemia effect, 3 1 0 Grand ma! seizures, 3 74, 3 7 5 , 3 7 5( Granuloma, pyogenic, 1 22, 1 22( Granuloma inguinale, 7 1 5t-7 1 6t Granulomatosis with polyangiitis (GPA, Wegener), 3 37(, 688, 690t eosinophilic, 299-300, 3 3 7(, 5901 Granulomatous arteritis, 3 3 7( Granulomatous disease, chronic, 5441 Granulosa cell tumors, 504, 5051 Graves disease, 1 30, 1 32, 1 32( Gravidity, 428 Greenstick fracture, 5731 Gross motor development, 5 1 21 Group A Streptococcus (GAS) acute pharyngitis, 66 1-662, 661(, 66 1 1 pediatric vaginal discharge, 49 1 Group A J3-hemolytic Streptococcus pyogenes, acute pharyngitis, 66 1 -662, 66 1(, 66 1 1 Group B streptococcus (CBS) meningitis, 3 791 pregnancy, 4 3 1 Growth, 5 1 1-5 1 4 Growth delay, constitutional, 47 1 , 4721, 5 1 2 Growth hormone (CH) deficiency, 1 4 31 excess, 451, 1 44-1 46, 145( GTD (gestational trophoblastic disease), 446, 446(, 4461 Guillain-Barre syndrome, 3 801, 388 Gummas, 7 1 3 , 7 1 3( Gunshot wound abdomen, 756 confidentiality, 1 94 GVHD (graft-vs.-host disease), 309-3 1 0 Gynecology, 467-507 abnormalities of menstrual cycle, 469-478 abnormal uterine bleeding, 476-478, 477(, 4781 precocious puberty, 469-4 70, 4691 primary amenorrhea/delayed puberty, 470-47 1 , 4721 primary dysmenorrhea, 474 secondary amenorrhea, 47 1 -474, 473( secondary dysmenorrhea, 474-47 5, 47 51 benign breast disorders, 492-494 atypical hyperplasia, 494 intraductal papilloma, 493 nonproliferative lesions, 492-493 phyllodes tumor, 494, 494(

INDEX proliferative lesions without atypia, 49 3 workup of breast mass, 493t cancer, 498-505 breast, 494-497, 495(, 496t cervical, 499-502, 50 1 (, 500t, 502( endometrial, 498-499, 499(, 499t ovarian, 503-505, 504t, 505t vaginal, 503 vulvar, 502-503 contraception, 478, 479t-48 l t gynecologic disorders, 487-492 Bartholin duct cyst and abscess, 487 cervicitis, 489 nonneoplastic ovarian cysts, 498 ovarian torsion, 490-49 1 pediatric vaginal discharge, 49 1 pelvic inflammato ry disease, 489-490 toxic shock syndrome, 49 1-492 uterine leiomyomas (fibroids) , 497-498 vaginitis, 487-489, 488t, 489( infertility, 484, 485(, 485t menarche and normal female development, 468 menopause, 486 normal menstrual cycle, 468-469, 468( pelvic organ prolapse, 506, 506( reproductive endocrinology, 48 1-485 congenital adrenal hyperplasia, 48 1-483, 482(, 483t polycystic ovarian syndrome, 483-484, 484( sexual disorders, 507

H HMRT (highly active antiretroviral therapy), HIV, 7 3 1 , 732(, 73 2t-7 3 3t HACEK, endocarditis, 68, 68t, 7 l t Haemophilus ducreyi, 7 1 5, 7 1 5t-7 1 6t Haemophilus influenzae, septic arthritis, 326t Haemophilus influenzae type B (Hib) meningitis, 3 79, 3 79t vaccine, l 7 l f-l 73f Haemophilus spp conjunctivitis, 4 l 5t endocarditis, 68, 68t, 71 t Hair-pulling disorder, 598t Hai ry cell leukemia (HCL), 304, 304( Hai ry leukoplakia, oral, 200 Hallucination(s), 5 9 1 hypnagogic, 6 1 8 hypnopompic, 6 1 8 Hallucinogens, substance abuse, 6 1 2t Hallucinosis, alcoholic, 6 1 3 , 6 1 3t Halothane, adverse effects, 748t Hand, common adult orthopedic injuries, 3 1 5t-3 1 6t HAND (HIV-associated neurocognitive disorder), 390 Hand infections, 344-345 Hand-foot-and-mouth disease, 5 57t Harmful substances, pregnancy, 4 30t Hashimoto thyroiditis, 1 34 HAY (hepatitis A virus), 249, 2 50t Hawkins test, 340, 340t Hawthorne effect, 1 67

Hazard ratio (HR), 1 6 1 HbA 1 c (hemoglobin Ai c) screening, l 74t, l 75t HbAS (sickle cell trait), 568 HbS (hemoglobin S), 567-569, 568(, 569( HBV (hepatitis B virus), 249, 2 50t chronic, 2 5 1 J3-HCG (J3-human chorionic gonadotropin), 428, 432t HCL (hai ry cell leukemia), 304, 304( HCM (h ypertrophic cardiomyopathy), 40t, 4 1 -42, 41( HCV (hepatitis C virus), 249, 2 50t chronic, 2 5 2 HD (Huntington disease) , 396-397, 396( HOV (hepatitis D virus), 249, 2 50t Head circumference, 5 1 1 Head CT, advanced trauma life support, 7 54 Head lice, 1 1 1 Head trauma blunt and deceleration, 7 5 7-7 59, 7 5 8( child abuse, 578t penetrating, 7 54-7 5 5 Headaches, 36 1-364, 3 6 l t cluster, 3 6 l t, 362 migraine, 3 6 1 -362, 3 6 l t "red flags," 36 3 secondary, 363-364 tension-type, 3 6 l t, 363 "thunderclap," 368 trigeminal neuralgia, 364 Healthcare personnel burnout and fatigue, 1 84- 1 8 5 vaccines, 1 7 3( Health insurance plans, 1 78 Health Insurance Portability and Accountability Act (HIPM), 1 9 3 Health system delive ry, 1 78-1 79 health insurance plans, 1 7 8 Medicare and Medicaid, 1 78, l 78t palliative care, 1 79 Health systems science, 1 77-1 96 clinical research, I 94- 1 9 5 core principles, I 94 ethical concerns, 1 94- 1 9 5 communication, 1 79-1 82 behavioral counseling, 1 82, 1 8 3(, 1 8 3t challenging conversations, 1 8 1 culturally inclusive histo ry taking, 1 8 1 gender- and sexuality-inclusive histo ry taking, 1 8 1 interpreters, 1 82 motivational interviewing, 1 82 patient-centered, evidence-based interviewing, 1 79-1 80 patients with disabilities, 1 82 rapport, 1 80, l 80t competence and decision-making capacity, 1 86-1 89 competence, 1 86-1 87 decision-making capacity, 1 87 informed consent, 1 87-1 89 complementa ry and alternative medicine therapy, I 9 I confidentiality, 1 93-194 conflict of interest, I 95-196

gifts from drug companies, 1 95-196 gifts from patients, 1 9 5 disclosure, 1 9 1- 1 9 3 fu l l disclosure, 1 9 1 - 1 92 setting for delivering news, 1 92-193 end-of-life issues, 1 89- 1 9 1 advance directives, 1 89 euthanasia and clinician-assisted suicide, 1 90- 1 9 1 futile treatment, I 9 I hospice care, 19 I surrogate decision making, 1 90 withdrawal of life-sustaining treatment, 1 90 ethics and legal issues, 1 8 5- 1 86 doctor-patient professional relationship, 1 86 doctor-patient sexual relationship, 1 86 general/core principles, 1 8 5- 1 86 health system delivery, 1 78-1 79 health insurance plans, 1 78 Medicare and Medicaid, 1 78, l 78t palliative care, 1 79 malpractice, 1 96 defined, 1 96 impaired practicing clinician, 1 96 patient safety and quality, 1 82-1 8 5 analyzing medical errors, 1 8 5 errors, 1 84, 1 84t health worker burnout and fatigue, 1 84- 1 8 5 measuring quality outcomes, 1 8 3- 1 84 PDSA cycle, 1 8 3 , 1 83( safety culture, 1 82- 1 8 3 Swiss cheese model, 1 8 3 , 1 83( "Healthcare proxy," 1 90 Hearing loss communication, 1 82 conductive, 42 5(, 426 presbycusis, 377 sensorineural, 42 5, 42 5( Hearing screening, children, 5 79 Heart disease, vaccines, 1 7 3( Heart failure (HF). See Congestive heart failure Heart failure with preserved ejection fraction (HFpEF), 34, 3 5t, 39-40 Heart failure with reduced ejection fraction (HFreF), 34, 3 5(-37(, 36-39, 3 7t, 3 8t Heart murmurs, 22, 22f-24f Heart rate (HR), 1 8 fetal, 452, 452(, 452t, 4 5 5 , 456t pregnancy, 4 29t Heat rash, 528t Height, child development, 5 1 1 Helicobacter pylori, 2 1 5 , 2 1 5t HELLP syndrome, 442 Hemangioblastoma, 404t Yon Hippel-Lindau syndrome, 409 Hemangioendothelioma, kaposiform, 567 Hemangioma(s), 1 22, 1 22( cavernocapillary, 567( hepatic, 263 infantile, 122 Hematemesis, 2 1 1 , 2 1 2t

INDEX Hematochezia, 2 1 1 , 2 1 2t Hematologic infections, 72 1-72 5 mosquito-borne, 721-722 malaria, 721-722, 721( other, 722 Hematology, 269-3 1 2 coagulation (bleeding) disorders, 270-277 hemophilia, 274-276, 275(, 284t and normal hemostasis, 270, 27 1(, 2 7 l t273t and transfusion products, 274, 274t von Willebrand disease, 276-277, 276(, 284t hyp ercoagulable states, 277-2 8 1 activated protein C (APC) resistance/fac­ tor V Leiden, 278 antiphospholipid syndrome (APS), 279-280 diagnosis, 278 disseminated intravascular coagulation (DIC), 280-2 8 1 , 280t, 283t, 284t etiology, 277, 277t heparin-induced thrombocytopenia (HIT), 279 history/physical examination, 278 treatment, 278 multisystem, 3 1 0-3 1 2 hemophagocytic lymphohistiocytosis, 3 1 0-3 1 1 Langerhans cell histiocytosis, 3 1 1-3 1 2 mastocytosis, 3 1 1 pediatric, 564-569 cyclic neutropenia, 566 Diamond-Blackfan anemia, 564-565, 564t Fanconi anemia, 564t, 565-566, 565t Kasabach-Merritt syndrome, 567, 567( sickle cell disease, 567-569, 568(, 569( thrombocytopenia absent radius syndrome, 566 plasma cell disorders, 307-309 amyloidosis, 308-309, 309t multiple myeloma, 307-308, 307( Waldenstrdm macroglobulinemia, 308 platelet disorders, 28 1-285 characteristics, 27 1 t hemolytic uremic syndrome (HUS), 282-283, 283t, 284t idiopathic thrombocytopenic purpura (ITP, immune thrombocytopenia), 284-28 5 thrombotic thrombocytopenic purpura (TIP), 28 1-282, 282(, 283t red blood cell disorders, 28 5-296 anemias, 28 5-294, 285(-288(, 286t, 289t, 290(, 292(, 293( G6PD deficiency, 291 hereditary spherocytosis, 290(, 291-292 paroxysmal nocturnal hemoglobinuria, 291 polycythemias, 295 porphyria, 294, 294t thalassemias, 288, 289t transfusion reactions, 296, 296t

transplant medicine, 309-3 1 0, 3 1 0t white blood cell disorders, 296-307 eosinophilia, 299-300, 299t leukemias, 300-304, 300f-302f, 302t, 303t lymphomas, 304-307, 304t, 305t, 306( lymphopenia and eosinopenia, 298, 298t neutropenia, 296-298 Hematoma(s) auricular, 7 5 5 duodenal, 228-229 epidural, 369, 7 5 8, 758( rectus sheath, 763-764 septa!, 3 5 5f subdural, 7 58, 7 5 8( Hematuria, 704 Hemianopia, 4 1 2( Hemochromatosis, 2 50, 260-261 cardiomyopathy due to, 45t Hemodialysis, vaccines, 1 7 3( Hemoglobin A 1 c (HbA1 c) screening, I 74t, l 75t Hemoglobin H disease, 289t Hemoglobin S (HbS), 567-569, 568(, 569( Hemoglobinuria, 295 paroxysmal nocturnal, 29 I Hemolytic anemias autoimmune, 292, 292( infectious mononucleosis, 725 extrinsic, 293 intrinsic, 293 normocytic, 290-292, 290(, 292( Hemolytic transfusion reaction, 296t Hemolytic uremic syndrome (HUS), 282-283, 283t, 284t Hemophagocytic lymphohistiocytosis, 3 1 0-3 1 1 Hemophilia, 274-276, 275(, 284t Hemoptysis, 663-664, 664( Hemorrhage(s) advanced trauma life support, 7 5 3 antepartum, 446-448, 447t, 448( gastrointestinal, 2 1 1-2 1 3, 2 1 2(, 2 1 2t, 2 1 3( germinal matrix, 526 intracerebral, 369, 369( intracranial, advanced trauma life support, 753 postpartum, 462, 463t splinter, endocarditis, 69, 69f subarachnoid, 363, 368-369, 368(, 380t subdural and epidural, 369, 370t subgaleal, 527t Hemorrhagic shock, 7 1 8 Hemorrhoids, 240t, 241 Hemostasis, normal, 270, 271(, 2 7 l t-273t Hemothorax, penetrating chest trauma, 756 Henoch-Schonlein purpura, 3 3 7( HepA (hepatitis A) vaccine, I 7 l f-l 73f Heparin low-molecular-weight, 272t deep venous thrombosis, 79 pulmonary embolism, 645 unstable angina/NSTEMI, 50 toxic ingestion/overdose, 745t unfractionated, 272t Heparin-induced thrombocytopenia (HIT), 279

Heparin-to-warfarin bridge, 270 Hepatic abscess, 263-264, 263( Hepatic adenomas, 260, 263 Hepatic encephalopathy, 252, 2 5 3t, 2 54, 2 5 5t Hepatic failure, fulminant, 2 5 6-2 57 Hepatic hemangioma, 263 Hepatic hydrothorax, 2 5 8 Hepatic necrosis, acute (fulminant), 2 5 6-2 57 infectious mononucleosis, 725 Hepatitis, 249-2 52 acute, 249, 250 alcoholic, 2 50, 2 57 autoimmune, 250 chronic, 249, 2 50, 2 5 1 complications, 2 5 2 diagnosis, 249-2 50, 2 5 1(, 2 5 l t drug-induced, 2 5 1 fulminant, 2 5 6-2 57 history/physical examination, 249 ischemic (hypoxic), 2 5 6 serologic markers, 2 50, 2 5 1(, 2 5 1 t treatment, 2 50-2 52 types, 249, 2 50t Hepatitis A (HepA) vaccine, l 71f-l 73f Hepatitis A virus (HAY), 249, 2 50t Hepatitis B postexposure prophylaxis, 2 5 2 Hepatitis B (HepB) vaccine, 1 7 lf- 1 7 3f Hepatitis B virus (HBV), 249, 2 50t chronic, 2 5 I Hepatitis C virus (HCV), 249, 2 50t chronic, 2 5 2 Hepatitis D virus (HOV), 249, 2 50t Hepatitis E virus (HEY), 249, 2 50t Hepatobiliary involvement, cytomegalovirus, 735 Hepatocellular adenoma, 260, 263 Hepatocellular carcinoma, 259-260 Hepatocellular injury, 248 Hepato-iminodiacetic acid (HIDA) scan, 246 Hepatojugular reflux, 22 Hepatolenticular degeneration, 2 50, 2 6 1 , 261(, 40 1-402 Hepatopulmonary syndrome, 2 5 7-2 58 Hepatorenal syndrome, 2 5 5t, 257 HepB (hepatitis B) vaccine, l 71f-l 73f HERZ (human epidermal growth factor 2) status, breast cancer, 495, 496 Hereditary nonpolyposis colorectal cancer (HNPCC), 2 3 7, 2 37t, 503 Hereditary polyposis syndromes, 2 36t Hereditary spherocytosis, 290(, 291-292 Hernia(s), 243-244, 243t congenital diaphragmatic, 5 2 1 t direct, 244, 243t epigastric, 243, 243t femoral, 24 3t hiatal, 2 1 1(, 2 1 1 incisional, 243, 243t indirect, 244, 243t inguinal, 24 3 pediatric, 541 Spigelian, 24 3t strangulated, acute abdomen, 227 umbilical, 243, 243t ventral, 24 3

INDEX Herniated disk, 349, 349{ Herniation, brain, 3 5 3 , 3 54{ Chiari malformations, 563, 563{ Herpes, genital, 98---1 00, 99t, 1 00(, 7 1 5 , 7 1 5t7 1 6t Herpes labialis, 1 00{ Herpes simplex keratitis, 4 1 5, 4 1 5{ Herpes simplex virus (HSV), 98- 1 00, 99t,

1 00{

cervicitis, 489 congenital, 4 3 5t encephalitis, 368, 3 82-3 8 3 , 3 8 3{ esophagitis, 20 5t neonatal ocular infections, 5 5 3-5 54, 5 54t Herpes zoster, 99t, 1 0 1 - 1 02, I O I{ Herpes zoster ophthalmic us, I O I Herpes zoster oticus, 1 0 I Herpesvirus(es) human, 98- 1 02, 99t-1 00t Kaposi sarcoma-associated, 1 2 1 Herpetic neuralgia, subacute, I O I Hesselbach triangle, 24 3 HEV (hepatitis E virus), 249, 2 50t Heyde syndrome, 276 HF (heart failure). See Congestive heart failure HCPRT (hypoxanthine-guanine phosphoribosyltransferase) deficiency, 346 HHS (hyperglycemic hyp erosmolar syndrome), 1 27-1 29, 1 29t HHVs. See Human herpesviruses (HHVs) Hiatal hernia, 21 If, 21 I Hib (Haemophilus influenzae type B) meningitis, 379, 3 79t vaccine, l 7 1 f- l 73f HIDA (hepato-iminodiacetic acid) scan, 246 Hidradenitis suppurativa, 1 1 6, 1 1 7{ High-altitude cerebral edema, 740 High-altitude pulmonary edema, 740 High-altitude sickness, 740 High-grade squamous intraepithelial lesion (HSIL), 500t, 5 0 1 Highly active antiretroviral therapy (HMRT), HIV, 7 3 1 , 732(, 732t-733t Hip, common adult orthopedic injuries, 3 I 6t3 l 7t Hip dislocation, 3 l 6t anterior, 3 1 4, 3 1 6t congenital, 5 7 5-576, 5 7 5{ posterior, 3 1 4, 3 1 6t Hip dysplasia, developmental, 57 5-5 76, 5 7 5{ Hip fracture, 3 1 4, 3 1 7t HIPM (Health Insurance Portability and Accountability Act), 1 9 3 Hirschsprung disease, 5 37, 5 3 7{ Hirsutism, 474, 484 Histiocytosis, Langerhans cell, 3 1 1-3 1 2, 5 7 1 Histoplasma capsulatum, 65 5f HIV, 729t Histoplasmosis, 654-6 56, 6 5 5 , 6 5 5(, 6 5 5 t History taking culturally-inclusive, 1 8 1 gender- and sexuality-inclusive, 1 8 I Histrionic personality disorder, 608t HIT (heparin-induced thrombocytopenia), 279

HIV. See Human immunodeficiency virus (HIV) Hives, 93-94, 94{ HL (Hodgkin lymphoma), 304t, 306--307, 306{ HLA-DRB I locus, 387 HMC-CoA reductase inhibitors. See Stalins HNPCC (hereditary nonpolyposis colorectal cancer), 237, 2 37t, 503 Hoarding disorder, 598t HOCM (hypertrophic obstructive cardiomyopathy), 4 1 -42 Hodgkin lymphoma (HL), 304t, 306-307, 306{ Homans sign, 79 Homocystinemia, 5 I 8t Homunculus, 3 5 3, 3 54{ Hordeolum, 1 1 6, 4 1 2, 4 1 2{ Hormone replacement therapy (HRT), menopause, 486 Horner syndrome, 422, 42 3{ Hospice care, 1 9 1 Hospitalization, involuntary, 1 8 5 "Hot tub folliculitis," 1 0 5 HPV. See Human papillomavirus (HPV) HR (hazard ratio), 1 6 1 HR (heart rate), 1 8 fetal, 452, 452(, 452t, 4 5 5 , 456t pregnancy, 4 29t HRT (hormone replacement therapy), meno­ pause, 486 HSIL (high-grade squamous intraepithelial lesion), 500t, 5 0 1 HSV. See Herpes simplex virus (HSV) 5-HT partial agonist, 595t HTN . See Hypertension (HTN) Human bites, 742t 13-Human chorionic gonadotropin (!3-HCC), 428, 432t ovarian tumor, 505t Human epidermal growth factor 2 (HER2) status, breast cancer, 49 5, 496 Human herpesviruses (HHVs), 98- 1 02, 99t-1 00t cytomegalovirus, 99t Epstein-Barr virus, 99t herpes simplex virus, 98---1 00, 99t, 1 00{ HIV, 729t roseola infantum, 5 56t roseoloviruses, l OOt varicella-zoster virus, 99t, 1 0 1-1 02, 1 0 1{ Human immunodeficiency virus (HIV), 725-7 3 5 congenital, 4 3 5t diagnosis, 728, 728{ history/physical examination, 727-728, 727{ immune reconstitution inflammatory syndrome, 73 1 management and prevention, 7 3 1 , 732(,

732t-733t

neoplasms, 7 5 1 t opportunistic infections, 729, 729t-730t,

730{

pregnancy, 734 prophylaxis for opportunistic infections and vaccinations, 73 1-734, 734{ screening, 729 serology to monitor disease progression, 727-729

transmission, 726, 726t vaccines, 1 7 3{ viral anatomy and physiology, 72 5(, 726 Human immunodeficiency virus (HIV)-associ­ ated neurocognitive disorder (HAND), 390 Human papillomavirus (HPV), 102- 1 0 3 , 1 02{ cervical cancer, 499 genital lesions, 7 1 5t-7 1 6t HIV, 729t recurrent respiratory papillomatosis, 674 vaccine, 17 lf- 1 7 3{ Humerus fracture, 3 l 4t Humor, 609t Hunter syndrome, 5 1 8t Huntington disease (HD), 396--397, 396{ Hurler syndrome, 5 1 8t HUS (hemolytic uremic syndrome), 282-283, 283t, 284t Hutchinson teeth, 7 1 3{ Hyaline arteriolosclerosis, 58, 59{ Hydatid cyst, 264 Hydralazine congestive heart failure, 39 adverse effects, 748t Hydrocele, 699 Hydrocephalus, normal-pressure, 390t, 392, 393-394, 394(, 397 Hydrochlorothiazide, adverse effects, 748t Hydronephrosis, 698, 698{ Hydrops fetalis, 289t Hydrothorax, hepatic, 2 5 8 Hydroxychloroquine, 3 30 adverse effects, 748t I l !3-Hydroxylase deficiency, I 54t, 48 1 , 482, 483t 1 70'.-Hydroxylase deficiency, I 54t, 482, 483t 2 1-Hydroxylase deficiency, 1 54t, 48 1 , 482, 483t Hymen, imperforate, 47 1 , 472{ H yperactivity, 586 H yperacute transplant rejection, 309-3 1 0, 31 Ot H yperaldosteronism, 1 5 3 H yperandrogenemia, polycystic ovarian syndrome, 484 Hyperandrogenism, 47 1 secondary amenorrhea, 474 Hyperbilirubinemia, 248, 249{ conjugated (direct) vs. unconjugated (indi­ rect), 520-524, 523t, 524{ H ypercalcemia, 1 40, 680-68 1 , 68 If H ypercoagulable states, 277-2 8 1 activated protein C resistance/factor V Leiden, 278 antiphospholipid syndrome, 279-280 diagnosis, 278 disseminated intravascular coagulation, 280-2 8 1 , 280t, 283t, 284t etiology, 277, 277t heparin-induced thrombocytopenia, 279 history/physical examination, 278 spontaneous abortions, 4 3 7 treatment, 278 H yperemesis gravidarum, 438-439 H ypereosinophilia, 299-300, 299t

INDEX Hyperglycemia pregnancy, 440 secondary amenorrhea, 4 73 Hyperglycemic hyperosmolar syndrome (HHS), 1 27-1 29, 1 29t H ypergonadotropic hypogonadism, 472{,

472t

H yperimmunoglobulin E (hyp er-IgE) syn­ drome, 545t Hyper-immunoglobulin M (hyp er-lgM) syndrome, 543t Hyperkalemia, 678-679, 679{, 680 Hyperlipidemia, 5 5-57, 56t, 57t Hypernatremia, 676 Hyperopia, 4 1 6 Hyperparathyroidism, 1 39- 1 4 1 classification, 1 39-1 40 complications, 1 4 1 diagnosis, 1 40, I 40t history/physical examination, 1 40 primary, 1 39, l 40t pseudo-, 1 40 secondary, 1 40, I 40t tertiary, 1 40, I 40t treatment, 1 40- 1 4 1 Hyperpituitarism, 1 44-147 Hyperprolactinemia, 146-147, 147( Hypersegmentation, 293, 293( Hypersensitivity pneumonitis, 634 H ypersensitivity reactions, 89, 89t, 90( H ypersomnia, primary, 6 1 8 H ypertension (HTN), 57-63 classification, 57, 58t gestational, 44 1-442 hypertensive emergency/urgency, 63, 63t idiopathic intracranial, 380t, 4 1 0-4 1 2 , 4 1 1( portal, 2 5 3 pregnancy, 44 1 -444, 443t primary (essential), 57-59, 59{, 60t-62t diagnosis, 59 history/physical examination/complica­ tions, 58, 59( risk factors, 57 treatment, 59, 60t-62t pulmonary, 643 secondary, 62t, 63 severe, 58t, 63t stage I, 58t stage II, 58t Hypertensive disease, pregnancy, 44 1 -444, 443t Hypertensive emergency, 62t, 63, 63t Hypertensive retinopathy, 58, 59{, 42 1 , 42 1( H ypertensive urgency, 63, 63t H yperthermia, 737 malignant, 7 1 9, 737 medication-induced, 737 Hyperthyroidism, 1 30- 1 34 diagnosis, I 3 2, 1 3 2{, 1 3 2t, 1 3 3{, 1 3 3t etiologies, 1 30- 1 32 history, I 3 2 primary, l 32t secondary, l 32t treatment, 13 3-1 34, I 34t Hypertrophic cardiomyopathy (HCM), 40t, 4 1 -42, 4 1 (

Hypertrophic obstructive cardiomyopathy (HOCM), 4 1 -42 Hyperuricemia, 347 Hypnagogic hallucinations, 6 1 8 Hypnopompic hallucinations, 6 1 8 Hypocalcemia, 68 1 -682 Hypochondria, 620 Hypoglycemia, 1 29-1 30 Hypoglycemia unawareness, 1 2 5t Hypogonadism central, 4 70 hyp ergonadotropic, 4 72{, 4 72t hyp ogonadotropic, 4 72{, 4 72t primary amenorrhea/delayed puberty, 47 1 Hypogonadotropic hypogonadism, 472{, 472t Hypokalemia, 679, 679{, 680( Hypomagnesemia, 682 Hypomania, 60 5, 60 5t Hyponatremia, 676-678, 677{, 678( Hypophyseal portal system acromegaly, 145( Cushing disease, 1 5 lf prolactin regulation, 14 7( Hypopituitarism, 1 4 1 - 1 42, 143t Hypopyon, 4 1 6 Hypospadias, 5 4 1 {, 542, 542t Hypotension, ventilator induced acute, 640 Hypothalamic disorders. See Pituitary and hypothalamic disorders Hypothalamic-anterior pituitary axis acromegaly, I 4 5( Cushing disease, 1 5 lf prolactin regulation, 147( Hypothalamic-pituitary axis, 1 4 1 , 14 lf Hypothalamic-pituitary-thyroid axis, 1 30, 1 3 1( Hypothermia, 736-7 37 Hypothesis testing, 1 68-1 69 Hypothyroidism, 1 34- 1 3 5 autoimmune, I 34 congenital, 1 34, 1 3 5 , 526-527 diagnosis, 1 3 2t, 1 3 5 etiology, 1 34 history/physical examination, I 34- 1 3 5 primary, 1 32t secondary, 1 32t, 1 34 subclinical, 1 32t treatment, 13 5 Hypotonia, infantile, 5 59, 5 59t Hypovolemic shock, 7 1 8t Hypoxanthine-guanine phosphoribosyltransferase (HGPRT) deficiency, 346 Hypoxemia, 637-638, 637{, 638t Hypoxic hepatitis, 2 5 6 Hypoxic liver injury, 2 56

IBO (inflammatory bowel disease), 24 1 , 241{, 242{, 242t colorectal cancer screening, 2 37t !CA (internal carotid artery), 3 5 5( penetrating trauma, 7 5 5 ! C D (implantable cardioverter-defibrillator), congestive heart failure, 39

!CH (intracranial hemorrhage), advanced trauma life support, 75 3 lchthyosis vulgaris, 1 I 7, I 1 7( ICP (intracranial pressure), idiopathic intracranial hypertension, 380t, 4 1 0-4 1 2, 41 lf ICSs (inhaled corticosteroids), asthma, 628, 628t Identical twins, 448 Idiopathic intracranial h yp ertension, 3 80t, 4 1 0-4 1 2, 4 1 1( Idiopathic pulmonary fibrosis (!PF), 632-63 3, 6 3 3( Idiopathic thrombocytopenic purpura (]TP), 284-28 5 I E . See Infective endocarditis (IE) l:E (inspiratory-to-expiratory time) ratio, 639 lgA (immunoglobulin A), 3 3 7( lgA (immunoglobulin A) deficiency, 543t lgA (immunoglobulin A) nephropathy, 689t IIV (inactivated influenza vaccine), 1 7 1 (- 1 7 3( !LO (interstitial lung disease) , 632-6 3 3 , 6 3 3( lieus, 22 5-226 gallstone, 246-247 Illness anxiety disorder, 620 Illusion, 5 9 1 lmatinib, 304 Immersion burns, child abuse, 5 78t Immune complex hypersensitivity reaction, 89t Immune reconstitution inflammatory syndrome, 731 Immune thrombocytopenia, 284-285 Immune-mediated skin disorders, 89-98 atopic dermatitis (eczema), 90-9 1 , 90{, 9 1( bullous pemphigoid/pemphigus vulgaris, 96, 97{, 97t contact dermatitis, 9 1 , 9 1 ( drug eruption, 94, 94( erythema multiforme, 9 5 , 9 5( erythema nodosum, 96, 96( h ypersensitivity reactions, 89, 89t, 90( nummular eczema, 98, 98( psoriasis, 92-93, 9 3( pyoderma gangrenosum, 98, 98( seborrheic dermatitis, 9 1 -92, 92( Stevens-Johnson syndrome/toxic epidermal necrolysis, 9 5-96, 9 5( urticaria (hives), 93-94, 94( lmmunocompromised individuals, vaccines,

1 73(

Immunodeficiency disorders congenital, 542-546, 543t, 545t neoplasms, 7 5 It Immunoglobulin A (IgA), 3 3 7( Immunoglobulin A (lgA) deficiency, 543t Immunoglobulin A (IgA) nephropathy, 689t Immunology, pediatric, 542-548 B-cell disorders, 543t, 545 combined disorders, 544t complement disorders, 545t, 546 immunodeficiency disorders, 542-546, 543t, 545t juvenile idiopathic arthritis, 547-548, 547t Kawasaki disease, 546 phagocytic deficiencies, 542{, 544t-545t, 546 T-cell disorders, 543t, 546 Impaired practicing clinician, 1 96

INDEX Impaled object, 756 lmperforate hymen, 47 1 , 472( Impetigo, 1 03- 1 04, ! 03(, ! 04( Impingement, shoulder, 340, 340t Implantable cardioverter-defibrillator (!CD), congestive heart failure, 39 lmpulsivity, 586 Inactivated influenza vaccine (IIV), l 7 lf- 1 7 3( Inactivated poliovirus (IPV) vaccine, 1 7 lf Inactivated vaccine, l 70t Inattention, 586 Incapacitated individuals, informed consent, 1 88 Incarcerated patients, clinical research, 1 9 5 Incidence, I 58 lncision(s), closure, 2 3 1 lncisional hernia, 243, 243t Incontinence, urinary, 700, 700t lncretins, diabetes, 1 28t Incurable illness, clinical research, 1 94 Indirect Coombs test, 292( Indirect hernia, 244, 243t Infant contact dermatitis, 582t Infantile hemangiomas, 1 2 2 Infantile hypotonia, 5 5 9 , 5 59t Infantile spasms, 408 Infarction, electrocardiogram with, 2 1 , 2 1{ Infections central line-associated bloodstream, 73 5-736 CNS, 379-384 brain abscess, 38 3-3 84, 383( cryptococcal meningitis, 3 8 1 encephalitis, 382-3 8 3 , 383( meningitis, 3 79-38 1 , 379(, 379t-3 8 l t toxoplasmosis, 3 8 1-382, 382( congenital, 4 3 5 , 4 3 5 t-436t genitourinary, 706-7 1 1 prostatitis, 7 ! 0-7 1 1 pyelonephritis, 708-7 10, 709( urinary tract, 706--7 1 1 , 7071, 708/ hematologic, 7 2 1 -722 mosquito-borne, 721-722 intra-amniotic, 450 opportunistic HIV, 727-729, 729t-730t, 730( pulmonary fungal, 65 5t pediatric, 548- 5 5 7 acute otitis media, 548 bronchiolitis, 549, 5 5 2( croup (laryngotracheobronchitis), 5 50, 5 50(, 5 50t, 5 52( epiglottitis, 5 50t, 5 5 1-552, 5 5 1 1, 5 5 2(, 5 5 3( meningitis, 5 52-5 5 3 ocular, 5 5 3-5 54, 5 541 pertussis (whooping cough), 5 54-5 5 5 pinworm, 5 5 5 TORCH infections, 4 3 5 , 4 3 5 t-436t tracheitis, 5 50t viral exanthems, 5 5 5, 5 56t-5 57t postamputation, 7 5 7 postpartum, 462-464 respiratory tract, 650-663 acute pharyngitis, 66 1 -662, 66 1(, 66 1 1 anthrax, 660, 660(

aspergillosis, 6 54, 654( blastomycosis, 6 5 51, 656-6 57 coccidioidomycosis, 65 5t656 histoplasmosis, 654-6 56, 655, 6 5 5(, 6 5 51 influenza, 652-654 mycobacterial, 657-6 59, 6 57(-659( Nocardia, 656 opportunistic, 6 5 5t oral, 662 Pneumocystis jirovecii pneumonia, 659-660, 659( pneumonia, 650-652, 65 1(, 6 5 l t-6 5 3 t, 652( sinusitis, 662-663, 663( skin, 98- 1 1 2 bacterial, 103-107, 1 0 3(- 1 07( fungal, 108-1 1 0, I 08f- l 1 0f parasitic, 1 1 0- 1 1 2 , 1 1 lf viral, 98- 1 0 3 , 99t- l 00t, 1 00f- i 02f urinary tract, 706--7 1 1 , 707t, 708t complicated, 707t, 708-7 1 I cystitis, 706, 708, 7 I O microbiology, 706-708, 708t mimics, 707t pregnancy, 444 prophylaxis, 707t prostatitis, 7 1 0-7 1 1 pyelonephritis, 706, 708-7 10, 709( types, 706, 7071 uncomplicated, 7071 upper, 708-7 1 0, 709( vesicoureteral reflux, 540 Infectious esophagitis, 204-205, 205t Infectious mononucleosis, 724-72 5 Infective endocarditis (IE), 2 3 , 67-7 1 antibiotic prophylaxis, 7 1 /, 72 diagnosis, 70, 70t etiologies, 68-69, 68/ history/physical examination, 69, 69( treatment, 70, 7 1 t Inferior vena cava (IVC) filter, pulmonary embolism, 645 Inferior wall myocardial infarction, 52, 52( Infertility, 484, 485(, 485t Inflammatory bowel disease (IBO), 24 1 , 24 If, 242(, 242t colorectal cancer screening, 2 3 7t Inflammatory breast carcinoma, 497 lnfluenza, 652-654 Influenza vaccine, 1 7 1(- 1 7 3( Informed consent, 1 87- 1 89 clinical research, 1 9 5 Inguinal hernias, 243 pediatric, 541 INH (isoniazid), adverse effects, 748t Inhalants, abuse, 6 1 1 1 Inhalation injury, advanced trauma life support, 752 Inhaled corticosteroids (ICSs), asthma, 628, 628t Inherited metabolic disorders, 5 I 7t-5 1 8t Inhibin, ovarian tumor, 505t Inhibin A, pregnancy, 4321 Inotropic agents, congestive heart failure, 38

INR ( international normalized ratio) , 270, 273t Insomnia, primary, 6 1 8 lnspiratory reserve volume (IRV), 626( lnspiratory-to-expiratory time (l:E) ratio, 639 Insulin, diabetes mellitus, 1 27, 1 27(, 1 281 lnsulinoma, 266 lntegrase strand transfer inhibitors, HIV, 7 3 3/ Intellectual deficits, 588 Intellectual developmental disorder, 588 Intellectual disability, 588 lntellectualization, 609t Intention tremor, 402t lntermenstrual bleeding, 476 Intermittent claudication, 8 1 Internal carotid artery (ICA), 3 5 5f penetrating trauma, 7 5 5 Internal hemorrhoids, 240/ International normalized ratio (INR), 270, 273t lnterosseous line, 7 5 3 Interpreters, I 82 Interstitial cystitis, 70 1 Interstitial lung disease (ILD), 632-6 3 3 , 6 3 3( Interviewing motivational, 1 82 patient-centered, evidence-based, 1 79- 1 80 "Intestinal angina," 229 Intimate partner violence, mandatory reporting, 1 94 Intra-amniotic infection, 450 lntracerebral hemorrhage, 369, 369( lntracranial aneurysms, 3 54 lntracranial hemorrhage (ICH), advanced trauma life support, 7 5 3 lntracranial hyp ertension, idiopathic, 380t, 4 1 0-4 1 2, 4 1 1( lntracranial neoplasms, 403, 404t-406t lntracranial pressure (ICP), idiopathic intracranial h yp ertension, 3 80t, 4 1 0-4 1 2 , 41 lf lntraductal papilloma, 493 lntrahepatic cholestasis, pregnancy, 444-445 lntranuclear ophthalmoplegia, 42 3, 423( lntraoral laceration, 7 5 5 lntraperitoneal bladder injury, 764 Intrauterine devices (lUDs), 478, 479t-48 l t Intrauterine fetal demise, 4371 Intrauterine growth restriction (IUGR), 449 Intravenous drug users (IVDUs), endocarditis, 68t Intrinsic pathway, 270, 271( lntussusception, 5 3 5-536, 5 3 5( Invasive pulmonary aspergillosis, 654 Invitation challenging conversations, 1 8 1 delivering news, 1 92 Involuntary hospitalization, 1 8 5 !PF (idiopathic pulmonary fibrosis), 632-6 3 3 , 6 3 3( IPV (inactivated poliovirus) vaccine, 1 7 1( Iron, toxic ingestion/overdose, 7451 Iron-deficiency anemia, 2 36, 286--287, 286(, 286t IRV (inspiratory reserve volume), 626( Ischemia electrocardiogram with, 2 1 , 2 1( Raynaud phenomenon, 344

INDEX Ischemic colitis, 2 3 8-239, 239f acute abdomen, 227, 2 30t Ischemic hepatitis, 2 5 6 Ischemic necrosis, 343-344, 343f Ischemic skin disorders, 1 1 2- 1 1 3 decubitus ulcers, 1 1 2-1 1 3 , l 1 2f gangrene, 1 1 3 , l l 3f Isoniazid (INH), adverse effects, 748t Isosorbide dinitrate, congestive heart failure, 39 Isospora, diarrhea, 220 Isotretinoin, 107 ITP (idiopathic thrombocytopenic purpura), 284-285 IUDs (intrauterine devices), 478, 479t-48 l t IUGR (intrauterine growth restriction), 449 Ivabradine, congestive heart failure, 39 IVC (inferior vena cava) filter, pulmonary embolism, 645 IVDUs (intravenous drug users), endocarditis, 681

JAK2 gene, 295 Janeway lesions, endocarditis, 69, 69f Janssen/J&J vaccine, 1 74 Jaundice, 248, 248{, 249f breast milk vs. breastfeeding, 524 neonatal, 520-524, 523t, 524f physiologic vs. pathologic, 520, 523 1 JC virus, HIV, 729t Jehovah's Witnesses, 1 8 1 , 1 89 Jejuna! atresia, 522t Jervell and Lange-Nielsen syndrome, 20 Jet-lag, 6 1 9 JIA (juvenile idiopathic arthritis), 547-548, 5471 Job syndrome, 5451 Jock itch, I 09, 1 1 Of JPS (juvenile polyposis syndrome), 2 3 8 JRA (juvenile rheumatoid arthritis), 547-548, 5471 Jugular venous distention (JVD), 22 Justice, 1 86 Juvenile idiopathic arthritis (JlA), 547-548, 5471 Juvenile polyposis syndrome (JPS) , 2 3 8 Juvenile rheumatoid arthritis (JRA), 547-548, 5471 JVD ( jugular venous distention), 22 K

K + (potassium)-sparing diuretics, 381, 6881 hypertension, 6 l t Kallmann syndrome, 47 1 Kanamycin, pregnancy, 434t Kaplan-Meier curve, 1 6 1 , 1 6 lf Kaposi sarcoma, 1 2 1 , 1 2 1{, 729t Kaposi sarcoma-associated herpesvirus ( KSHV), 121 Kaposiform hemangioendothelioma, 567 Kasabach-Merritt syndrome, 567, 567f Kawasaki disease, 3 3 7{, 546 Kayser-Fleischer rings, 2 6 1 , 261{, 40 1

Keratitis contact lens, 4 1 5-4 1 6 herpes simplex, 4 1 5, 4 l 5f Keratoconjunctivitis sicca, 329 Keratoderma blennorrhagica, 3 3 1 Keratosis actinic, 1 1 8, l l 9f seborrheic, 1 1 7-1 1 8, 1 1 8f neoplasms, 7 5 1 I Kernicterus, 520 Kernig sign, 5 5 3 Kernohan notch, 3 54f Ketoacidosis, diabetic, 1 27-1 29, 1 291 Kidney, blunt abdominal trauma, 7621 Kidney disease. See also Renal disease chronic, 684-687 polycystic, 697-698, 697{ Kidney injury, acute, 684, 6851-6861 Kidney stones, 694-697, 695 1-6961, 696f Killed vaccine, 1 701 Killip classification, congestive heart failure, 3 5 Kinetic tremor, 4021 Klebsiella granulomalis, genital lesions, 7 l 5t7 1 6t Klebsiella pneumoniae, urinary tract infection, 708t Kleptomania, 598t Klinefelter syndrome, 5 1 71 Knee, common adult orthopedic injuries, 3 l 7t-3 1 81 Knee injury, unhappy triad, 3 1 0, 3 1 9{ Knee ligament injuries, 3 1 71 Knife wound abdomen, 756 confi d entiality, 1 94 Knowledge challenging conversations, 1 8 1 delivering news, 1 92 Koebner phenomenon, 92, 1 1 4 Koilonychia, 286, 286f Korsakoff dementia, 4 1 1 I Krabbe disease, 5 1 81 Krukenberg tumor, 2 1 6 KSHV (Kaposi sarcoma-associated herpesvirus), 121 Kussmaul sign, 22 constrictive pericarditis, 65

L La belle indifference, 620 LAA (left atrial appendage), occlusion, 32 Labor and delivery abnormal, 457-462 episiotomy, 46 1 failure to progress, 4 59, 4591 fetal malpresentation, 460-46 1 , 460f indications for C-section, 457, 457t intra-amniotic infection, 460 preterm labor, 457-458 rupture of membranes, 4 5 8-459 shoulder dystocia, 46 1 , 46 lf umbilical cord prolapse, 46 1 uterine inversion, 462 uterine rupture, 462

normal, 454-456 analgesia and anesthesia, 45 5-456 definition and stages of labor, 454, 454t fetal heart rate monitoring, 45 5, 4561 obstetric examination, 454-4 5 5 Labyrinthitis, 378 Lactase deficiency, 224 Lactate dehydrogenase (LDH), 3 3 3 ovarian tumor, 505t Lactation, 464-466, 464{, 465t Lacunar stroke, 3651 LAD (left anterior descending artery), myocar­ dial infarction involving, 52, 5 3f Ladd bands, 5 36, 5 3 6f LAIV4 (live attenuated influenza vaccine), 1 7 1f-1 73f LAM (lymphangioleiomyomatosis), 407-408 Lambert-Eaton myasthenic syndrome (LEMS), 3 8 5-3 86, 3 8 5t Lamotrigine, 6061 Langerhans cell histiocytosis, 3 1 1-3 1 2 , 5 7 1 Language development, 5 l 2 t Language disorder, 5 1 1 , 5 1 3t Large bowel disorders, 2 3 1-24 1 Closlridium difficile colitis, 2 3 1-2 3 2 colorectal cancer, 2 36-2 37, 236{, 2361, 2 371 colorectal cancer-associated conditions, 2 3 7-2 38, 2 3 8f diverticular disease, 2 3 2-2 3 3 , 2 3 3f irritable bowel syndrome, 2 34-2 36 ischemic colitis, 2 3 8-2 39, 239f large bowel obstruction, 2 34, 2341, 23 5f microscopic colitis, 240-24 1 Large bowel obstruction (LBO), 2 34, 2 341, 2 3 5f Large cell carcinoma, lung, 648t Large vessel vasculitis, 3 3 7f Laryngeal lesions, 673-674 Laryngitis, 672-673 Laryngopharyngeal reflux, 673 Laryngotracheobronchitis, 5 50, 5 50{, 5 50t, 5 5 2f Latent error, 1 841 Lateral corticospinal tract, 3 5 8t, 36 lf Lateral femoral cutaneous nerve injury, 3221 Lateral myocardial infarction, 52 Lateral spinothalamic tract, 36lf Lateral ventricles, 3 54f LBBB (left bundle-branch block), 20, 20f LBO (large bowel obstruction), 2 34, 2341, 2 3 5f LBP (low back pain), 348-3 50, 349{, 3 501 LCA (left coronary artery), myocardial infarction involving, 52 LDH (lactate dehydrogenase), 3 3 3 ovarian tumor, 5051 LDl.rC (low-density lipoprotein cholesterol), 5 5-57, 561, 57t Le Fort fractures, 759 Lead, pregnancy, 4 34t Lead poisoning, 287, 580-582, 581{, 74 51 Lead-time bias, 1 67 Learning disorder, 588 specifi c , 5 1 31 Left anterior descending artery (LAD), myocar­ dial infarction involving, 52, 5 3f Left atrial abnormality, 2 1 Left atrial appendage (LAA), occlusion, 32

INDEX Left bundle-branch block (LBBB), 20, 20{ Left coronary arte ry (LCA), myocardial infarc­ tion involving, 52 Left ventricular assist device (LVAD), congestive heart failure, 39 Left ventricular ejection fraction (LVEF), in heart failure, 34, 3 51 Left ventricular hypertrophy (LVH), 2 1 , 2 lf Left-sided heart failure, 34, 3 51 Left-to-right shunts, acyanotic, 528, 529-53 2 Leg, common adult orthopedic injuries, 3 1 713 1 81 Leg elevation, shoulder dystocia, 46 1 , 46 1{ Legal emancipation, 1 8 8 Legal issues, 1 8 5- 1 86 doctor-patient professional relationship, 1 86 doctor-patient sexual relationship, 1 86 general/core principles, 1 8 5- 1 86 Legg-Calve-Perthes disease, 576, 5 76{ Legitimization, rapport, 1 801 Leiomyomas, uterine, 497-498 LEMS (Lambert-Eaton myasthenic syndrome), 3 8 5-386, 38 51 Length bias, 1 67 Lentigo maligna melanoma, 1 201 Leprosy, 1 07 Leptomeninges, 3 5 5 Lesch-Nyhan syndrome, 346 Leser-Trelat sign, 1 1 8, I 1 8{ Leukemia(s), 300-304 acute, 300-3 0 1 , 300(, 3 0 1 (, 303 children, 569-570 chronic lymphocytic, 30 1-302, 302(, 3021, 303 chronic myelogenous, 302-303, 3021, 303, 3031 hai ry cell, 304, 304{ Leukemoid reaction, vs. chronic myelogenous leukemia, 303, 3031 Leukocoria, 422, 422(, 580 Leukocyte adhesion deficiency, 5451 Leukodystrophy, metachromatic, 5 1 81 Leukoencephalopathy, progressive multifocal, HIV, 7291 Leukoplakia oral, I 99(, 200 oral hairy, HIV, 7291 Leuprolide stimulation test, 470 Level of evidence pyramid, 1 6 1- 1 62, 1 62{ Levodopa-carbidopa, Parkinson disease, 398-399, 399{ Levonorgestrel, 48 I I Levothyroxine, hypothyroidism, 1 3 5 Lewy body dementia, 3 9 1 1, 395 Leydig cell tumor, 706t LFTs (liver function tests), abnormal, 248, 248(, 249{ LCV (lymphogranuloma venereum), 7 1 1 LH (luteinizing hormone) deficiency, 1431 LH (luteinizing hormone) surge, 469 Libman-Sacks endocarditis, 3 36 Lice, 1 1 0-1 1 1 Lichen planus, 1 14, 1 1 4{ oral, 20 1 Lichen sclerosus, 503 Lichenification, 881

Lifestyle modifications chronic heart failure, 39 dyslipidemia, 56 Life-sustaining treatment, withdrawal, 1 90 Life-threatening emergencies, informed consent for minors, 1 8 8 Light criteria, pleural effusion, 666, 6661 Likelihood ratio (LR), 1 60 Limb ischemia, 8 1 Limbic association area, 3 5 3{ Limp, pediatric, 576 Linagliptin, diabetes, 1 281 Linear regression, 1 69 Linear skull fractures, 7 5 8 Lipid-lowering agents, 56, 571 Lipoprotein lipase stimulators, 571 Liraglutide, diabetes, 1 281 Listeria, meningitis, 379(, 3791 Lithium, 6061 pregnancy, 4 341 prenatal exposure, 5 291 Live attenuated influenza vaccine (LAIV4), 1 7 1(- 1 7 3{ Live attenuated vaccine, 1 701 Liver blunt abdominal trauma, 762t fatty, 2 5 9 shock, 2 5 6 Liver abscess, 263-264, 263{ Liver disease, 248-264 and abnormal liver function tests, 248, 248(, 249{ acute liver failure, 2 5 6-2 57 benign lesions, 262-264, 262(, 263{ bleeding disorders, 2841 chronic, vaccines, 1 7 3{ cirrhosis, 2 5 2-2 54, 2 5 2(, 2 5 3(, 2 5 3 t-2 5 5t hemochromatosis, 260-26 1 hepatic hydrothorax, 2 58 hepatitis, 249-2 52, 2 5 01, 2 5 1(, 2 5 1 1 hepatocellular carcinoma, 2 5 9-260 hepatopulmonary syndrome, 2 5 7-2 5 8 hepatorenal syndrome, 2 5 51, 2 5 7 ischemic hepatitis, 2 5 6 and liver transplantation, 261-262 nonalcoholic fatty liver disease, 2 5 9 primary biliary cholangitis, 2 5 9 primary sclerosing cholangitis, 2 5 9 spontaneous bacterial peritonitis, 2 52, 2 54-2 56, 2 5 51, 256 transjugular intrahepatic portosystemic shunt procedure, 2 5 8 Wilson disease (hepatolenticular degenera­ tion), 2 50, 2 6 1 , 261{ Liver failure, acute, 2 56-2 57 Liver function tests (LFTs), abnormal, 248, 248(, 249{ Liver injury, hypoxic, 2 5 6 Liver lesions, benign, 262-264, 262(, 263{ Liver transplantation, 261-262 Living will, 1 89, 1 90 LMN (lower motor neuron) lesion, 3 57, 3 571 LMWH. See Low-molecular weight heparin (LMWH)

Lochia, 462 "Locked-in" syndrome, 372, 3721 Loffler syndrome, 636 Lofgren syndrome, 63 3-634 Logistic regression, 1 69 Long thoracic nerve injury, 3201 Loop diuretics, 6881 congestive heart failure, 38, 3 81, 39 hypertension, 60t Lou Gehrig disease, 3 591, 398, 399-400 Low back pain (LBP), 348-3 50, 349(, 3 501 Low-density lipoprotein cholesterol (LDL-C), 5 5-57, 561, 571 Lower extremity common adult orthopedic injuries, 3 1 613 1 81 musculoskeletal disorders, 345-348 bursitis, 345 gout, 346-348, 347(, 3471 Morton neuroma, 346 osteochondritis dissecans, 345, 345{ patellofemoral pain syndrome, 346 pes anserinus pain syndrome, 345-346 pseudogout, 34 7f, 3471, 348, 348{ Lower gastrointestinal bleeding, 2 1 21, 2 1 3,

2 1 3{

Lower motor neuron (LMN) lesion, 3 57, 3 571 Low-grade intraepithelial lesion (LSIL), 5001, 501 Low-molecular weight heparin (LMWH), 2721 deep venous thrombosis, 79 pulmonary embolism, 645 unstable angina/NSTEMI, 50 LP (lumbar puncture), 3 56, 3 56(, 384, 5 5 8 L R (likelihood ratio), 1 60 LSD (lysergic acid diethylamide ), abuse, 6 1 21 LSIL (low-grade intraepithelial lesion), 5001, 501 Ludwig angina, 662 Lumbar puncture (LP), 3 56, 3 56(, 384, 5 5 8 Lumbar spinal stenosis, 349-3 5 0 , 3 501 Lumpectomy, 496 Lung cancer, 647-6 50, 6481, 649(, 6501 screening, 1 7 51 Lung capacities, 626(, 627{ Lung compliance, decreased, 64 I I Lung consolidation, 6651 Lung disease acute respirato ry failure, 637-642 acute respiratory distress syndrome, 638-639, 638{ coronavirus and COVID- 19, 64 1-642, 642{ h ypoxemia, 637-638, 637(, 6381 mechanical ventilation, 639-64 1 , 64016421 hemoptysis, 663--064, 664{ infectious, 6 50-663 acute pharyngitis, 66 1-662, 661(, 66 1 1 anthrax, 660, 660{ aspergillosis, 6 54, 654{ blastomycosis, 65 51, 656-6 57 coccidioidomycosis, 6 5 51656 histoplasmosis, 654-6 56, 65 5, 6 5 5f, 6 5 51 influenza, 652-654 mycobacterial, 657-6 59, 6 57(-6 59{

INDEX Lung disease (Continued) Nocardia, 656 opportunistic, 655t oral, 662 Pneumocystis jirovecii pneumonia, 659-660, 659( pneumonia, 650-652, 65 1(, 6 5 l t-65 3 t, 652( sinusitis, 662-663, 663( neoplasms, 646-6 50 lung cancer, 647-6 50, 648t, 649(, 650/ solitary pulmonary nodule, 646-647, 647(, 6471 obstructive, 626-63 1 asthma, 626-628, 628t, 629t bronchiectasis, 628-630, 629( chronic, 630-63 1 , 630t-632t, 6 3 1( lung volumes, 626, 626( restrictive vs. , 626, 626t, 627( pleural disease, 664-667 pleural effusion, 664-666, 66 5t-667t pneumothorax, 665t, 666-667, 667( restrictive, 63 1-636 allergic bronchopulmonary aspergillosis, 636 cryptogenic organizing pneumonia, 63 3 eosinophilic pulmonary syndromes, 63 5-636 h ypersensitivity pneumonitis, 634 interstitial (diffuse parenchymal), 632-63 3, 6 3 3( obstructive vs., 626, 626t, 627( pneumoconiosis, 634-6 3 5 , 6 3 5( systemic sarcoidosis, 63 3-634, 634( vaccines, 1 73( Lung injury, ventilator-induced, 640 Lung volumes, 626(, 627( Lupus anticoagulant, 279, 280, 3 36 Lupus erythematosus, systemic, 3 3 5-336, 3 3 5(, 3 3 7( Lupus nephritis, 690t Luteal phase of menstrual cycle, 468-469, 468( Luteinizing hormone (LH) deficiency, 14 31 Luteinizing hormone (LH) surge, 469 Luteoma, 4981 LVAD (left ventricular assist device), congestive heart failure, 39 LVEF (left ventricular ejection fraction), in heart failure, 34, 3 5t LVH (left ventricular hyp ertrophy), 2 1 , 2 1( Lyme disease, 722-72 3, 723( Lymphadenitis, acute, 662 Lymphangioleiomyomatosis (LAM), 407-408 Lymphedema, 82 Lymphoblasts, 302( Lymphocyte count, absolute, 298 Lymphocytosis, 303 Epstein-Barr virus, 724 Lymphogranuloma venereum (LGV), 7 1 1 Lymphohistiocytosis, hemophagocytic, 3 1 0-3 1 1 Lymphoma(s), 304-307 adult T-cell, 305t Burkitt, 3051 CNS, 382 cutaneous T-cell, 1 2 1 - 1 22, 1 2 1(

diffuse large B-cell, 3051 follicular, 305t Hodgkin, 304t, 306-307, 306( mantle cell, 305t mucosa-associated lymphoid tissue (MALT), 216 mycosis fungoides/Sezary syndrome, 305t non-Hodgkin, 304-306, 3041, 3051 primary CNS, 305t testicular, 7061 Lymphopenia, 298, 2981 Lynch syndrome, 237, 2 371, 503 Lysergic acid diethylamide (LSD), abuse, 6 1 2t

M MAC (Mycobacterium avium complex), 658-659 HIV, 730t Macrocephaly, benign familial, 563 Macrocytic anemias, megaloblastic, 293-294, 293( Macroglobulinemia, Waldenstriim, 308 Macrosomia, fetal, 449 Macrovascular complications, diabetes mellitus, 1 2 5t, 1 26t Macular degeneration, age-related, 4 1 2(, 4 1 8-4 1 9, 4 1 9( Macule, 88t Magnesium toxicity, 443 , 444 Maintenance stage of change, 1 8 3f, 1 8 3t Major depressive disorder (MOD), 602-603, 602t-6041 Major depressive episodes (MDEs), 602 Major neurocognitive disorder. See Dementia Malabsorption, 22 3-224, 223( Malaria, 72 1 -722, 7 2 1( Malassezia furfur, seborrheic dermatitis, 9 1 Malassezia spp, 1 08 Maldigestion, 22 3-224, 223( carbohydrate, 224 Male condoms, 480t Male development normal, 468 sexual, 5 1 5( Male factor infertility, 48 51 Malignant h yp erthermia, 7 1 9, 7 3 7 Malignant melanoma, 1 1 9- 1 20, 1 20t Malignant neoplasms. See Cancer Malignant otitis externa, 424 Malingering, 62 1 Malleolar zone, 3 1 9, 3 I 9f Mallory-Weiss tear, 2 1 3 Malpractice, 1 96 Malrotation with volvulus, 5 36, 5 3 6( MALT (mucosa-associated lymphoid tissue) lymphoma, 2 1 6 Maltase-glucoamylase deficiency, 224 Mammography, l 74t, 49 5, 49 5( Mania, 60 5-606, 60 5t, 607 Mantle cell lymphoma, 3051 Mantoux tuberculin test, 657, 657( MAO (monoamine oxidase) inhibitors depression, 60 3, 604t Parkinson disease, 398-399, 399( adverse effects, 7481 Marcus-Gunn pupil, 422

Marijuana, abuse, 6 1 2t Masochism, sexual, 6 1 7t Mastitis, 465-466 Mastocytosis, 3 1 1 "Matching," 1 6 3 Maternal serum cx-fetoprotein (MSAFP), 432, 4321 Mature follicle, 468( Maturity-onset diabetes of the young (MODY), 126 Mc Burney point, 2 3 1 McCune-Albright syndrome, 470 MCHC (mean corpuscular hemoglobin con­ centration), 286 MCL (medial collateral ligament), knee injury, 3 1 9( McRoberts maneuver, 46 1 , 46 1( MCS (mechanical circulatory support), conges­ tive heart failure, 3 8 MCV (mean corpuscular volume), 285 MOD (major depressive disorder), 602-603, 6021-6041 MDEs (major depressive episodes), 602 MOMA, substance abuse, 6 1 2t Mean corpuscular hemoglobin concentration (MCHC), 286 Mean corpuscular volume (MCV), 2 8 5 Measles, 5 56t mumps, rubella (MMR) vaccine, 1 7 1(-1 73( Measurement bias, 1 66 Mechanical circulatory support (MCS), conges­ tive heart failure, 3 8 Mechanical ventilation, 639-64 1 complications, 639-64 1 indications, 639 mechanics, 639, 640t pathologic waveforms, 639, 64 l t settings, 639, 642t Meckel diverticulum, 5 3 6-537, 5 3 6( Meconium aspiration, 525t MECP2 gene, 562 Medial collateral ligament (MCL), knee injury, 3 1 9( Medial longitudinal fasciculus (MLF) lesions, 387 Medial meniscus (MM), knee injury, 3 1 9( Medial temporal lobe herniation, 3 5 3 Median nerve injury, 3 2 l t, 322, 322(, 3 2 3( Mediastinitis, acute necrotizing, 662 Medicaid, 1 78, I 78t Medical errors, 1 84, 1 84t analyzing, 1 8 5 Medical malpractice, 1 96 Medical records, access, 1 9 3 Medicare, 1 78, 1 781 Medication-induced esophagitis, 20 5-206, 206( Medication-induced hyperthermia, 737 Medium vessel vasculitis, 3 3 7( Medroxyprogesterone, 479t Medullary thyroid carcinoma, l 3 7t Medulloblastoma, 560t Mefloquine, 722 Megaloblastic, macrocytic anemias, 293-294, 293( Melanocytic nevus, congenital, 583t Melanocytosis, congenital dermal, 583t

INDEX Melanoma, 1 1 9-1 20, 1 20t Melanosis, neonatal pustular, 528t Melasma, 429t Melatonin, 6 1 9 MELD (Model fo r End-stage Liver Disease) score, spontaneous bacterial peritonitis, 2 54 Melena, 2 1 1 , 2 1 2t Membranoproliferative nephropathy, 6891 Membranous nephropathy, 6931 MEN. See Multiple endocrine neoplasias (MEN) Men who have sex with men (MSM) HlV, 725 vaccines, 1 7 3( MenACWY (meningococcal A, C W, Y) vaccine, 1 7 1{, 1 72{, 1 7 3( Menarche, 468, 5 1 5( MenB (meningococcal B) vaccine, l 7 l f- l 73f Menetrier disease, 2 1 8 Meniere disease, 378-379 Meninges, anatomy, 3 5 5, 3 5 5( Meningioma, 404t Meningitis, 3 79-3 8 1 bacterial, 379-3 8 1 , 379{, 3 791-3 8 l t complications, 3 8 1 cryptococcal, 3 8 1 defined, 3 5 5 diagnosis, 380, 3 80t history/physical examination, 379 pediatric, 5 52-5 5 3 treatment, 3 80-3 8 1 , 3 8 l t viral (aseptic), 3 79, 3801 Meningococcal A, C W, Y (MenACWY) vaccine, 1 72( Meningococcal B (MenB) vaccine, l 7 lf-l 73f Meningococcal meningitis, 379, 3791 Meningococcal (MenACWY) vaccine, 1 7 lf, 1 72{, 1 7 3( Meniscal tears, 3 l 7t Menometrorrhagia, 476 Menopause, 486 Menorrhagia, 476, 477 Menses, 468( Menstrual bleeding heavy, 476 heavy prolonged, 476 Menstrual cycle abnormalities, 469-478 abnormal uterine bleeding, 476-478, 477{, 478t precocious puberty, 469-470, 4691 primary amenorrhea/delayed puberty, 470-47 1 , 472t primary dysmenorrhea, 474 secondary amenorrhea, 47 1-474, 473( secondary dysmenorrhea, 474-47 5, 475t normal, 468-469, 468( Mentzer index, 288 Mercury pregnancy, 434t toxic ingestion, 744t Mesenteric ischemia, 229-2 30, 2 3 01 acute, 2 30t acute abdomen, 227 chronic, 2 30t

Metabolic acidosis, 682, 683{, 683t Metabolic alkalosis, 68 3{, 68 3t Metabolic disorders, inherited, 5 l 7t-5 1 8t Metabolic syndrome, 1 24 polycystic ovarian syndrome, 483 Metachromatic leukodystrophy, 5 l 8t Metastasis, breast cancer, 494, 49 5, 496 Metatarsal stress fracture, 3 l 8t Metatarsus adductus, 5 7 5 Metformin diabetes, l 28t adverse effects, 748t Methacholine challenge, 627 Methanol presentation, 682 toxic ingestion, 7451 Methemoglobinemia, 743-744 Methimazole, adverse reactions, l 34t Methotrexate, 3 30 pregnancy, 4 34t adverse effects, 7481 Methyldopa, adverse effects, 748t Methylxanthines, asthma, 628t Metoclopramide, adverse effects, 748t Metronidazole, adverse effects, 748t Metrorrhagia, 476 MG (myasthenia gravis), 384-385, 384{, 3 8 5t neoplasms, 7 5 1 I MGUS (monoclonal gammopathy of undeter­ mined significance), 307 MHA-TP (microhemagglutination assay-Treponema pallidum), 7 l 4t Ml. See Myocardial infarction (MI) Microalbuminuria, 1 24 Microcephaly, benign familial, 563 Microcytic anemias, 286-289, 286(-288{, 286t, 289t Microcytosis, 286-289, 286(-288{, 286t, 289t Microhemagglutination assay-Treponema pallidum (MHA-TP), 7 1 4t Microscopic colitis, 240-24 1 Microscopic polyangiitis, 3 37{, 688, 690t Middle cerebral artery, 3 54, 3 54{, 3 5 5f stroke, 3651 Midfoot zone, 3 1 9, 3 1 9( Miglitol, diabetes, l 28t Migraine headache, 3 6 1 -362, 3 6 l t Milia, 5281 Milia rubra, 528t Milk retention cyst, 466 Mineralocorticoids, 1 48( Minimal change disease, 693t "Minipills," progestin-only, 4 79t Minors, informed consent, 1 88- 1 89 Mitra! regurgitation (MR), 2 3{, 72, 74t Mitra! stenosis (MS), 2 3{, 24{, 72, 73t Mitra! valve prolapse (MVP), 2 3{, 24{, 74t Mittelschmerz, 476 Mixed cryoglobulinemia, 692 MLF (medial longitudinal fasciculus) lesions, 387 MM (medial meniscus), knee injury, 3 1 9( MM (multiple myeloma), 307-308, 307( MMR (measles, mumps, rubella) vaccine, l 7 lf- l 7 3f

Mobitz type I AV block, 26t Mobitz type II AV block, 27t Model for End-stage Liver Disease (MELD) score, spontaneous bacterial peritonitis, 2 54 Moderna vaccine, 1 7 3 Modified Wells score, 644, 6451 MODY (maturity-onset diabetes of the young), 1 26 Molar pregnancy, 446, 446{, 4461 Molluscum contagiosum, 1 02, 1 02( Monetary compensation, clinical research, 1 9 5 Mongolian spot, 583t Monoamine oxidase (MAO) inhibitors depression, 603, 604t Parkinson disease, 398-399, 399( adverse effects, 748t Monoclonal gammopathy of undetermined significance (MGUS), 307 Monozygotic twins, 448 Monteggia fracture, 3 l 5t Mood disorders, 602-607 adjustment disorder, 602t, 60 5 bipolar and related disorders, 603, 60 5-607, 605t, 606t diagnostic criteria by symptom duration, 6071 major depressive disorder, 602-60 3, 602t604t due to medical condition, 6021 persistent depressive disorder (dysthymia), 602t, 604-60 5 substance-induced, 602t Mood stabilizers, 606, 606t Moraxella, conjunctivitis, 4 1 51 Morbilliform rash, 94, 94f Moro reflex, 5 1 3t Morton neuroma, 346 Mosquito-borne infections, 721-722 malaria, 72 1-722, 72 1( other, 722 Mother-to-child transmission, HIV, 7261, 728, 734 Motivational interviewing, 1 82 Motor aphasia, 372-373, 373( Motor homunculus, 3 5 3 , 3 54( Motor neuron disease, 3 59t, 398, 399-400 Movement disorders, 396-402 amyotrophic lateral sclerosis, 3 59t, 398, 399-400 Huntington disease, 396-397, 396( Parkinson disease and parkinsonism, 397-399, 399( restless legs syndrome, 400-40 1 tremors, 40 1 , 40 lf, 402t Wilson disease (hepatolenticular degeneration), 40 1-402 MR (mitral regurgitation), 2 3{, 72, 74t MS (mitral stenosis), 2 3{, 24{, 72, 731 MS (multiple sclerosis), 3 59t, 3 801, 387-3 88 MSAFP (maternal serum a-fetoprotein), 432, 432t MSM (men who have sex with men) HIV, 725 vaccines, 1 73( Mucocele, 202t

INDEX Mucoepidermoid carcinoma, salivary glands, 2031 Mucor, sinusitis, 662 Mucosa-associated lymphoid tissue (MALT) lymphoma, 2 1 6 Mucosa! abnormalities, malabsorption, 2 2 3 Miillerian agenesis, 4 7 1 , 4 72f Multifocal atrial tachycardia, 30t Multiple dysplastic nevi, neoplasms, 7 5 1 1 Multiple endocrine neoplasias (MEN), 1 5 5, 15 5f insulinoma, 266 pheochromocytoma, 1 50 somatostatinoma, 266 Multiple gestation, 448 Multiple myeloma (MM), 307-308, 307( Multiple personality disorder, 593, 594t Multiple sclerosis (MS), 3 59t, 3 80t, 387-3 88 Multisystem disorders, 7 1 7-764 blunt and deceleration trauma, 7 5 7-764 abdomen, 762-76 3, 762t, 76 3( cardiac injury, 76 1 -762, 762( chest, 760-76 1 , 760{, 761( head and face, 7 5 7-7 59, 7 5 8( pelvis, 763-764 central line-associated bloodstream infec­ tions, 7 3 5-736 cytomegalovirus, 73 5 environment, 73 7-742 bites and stings, 740, 74 If, 74 1 1-7421 burns, 737-740, 7 3 8{, 7 3 8t, 739( drowning, 740 high-altitude sickness, 740 fever, 7 1 9-72 1 postoperative, 7 1 9, 7 1 9{, 7 1 91 sepsis, 720-72 1 of unknown origin, 7 1 9-720 hematologic, 3 1 0-3 1 2 hemophagocytic lymphohistiocytosis, 3 1 0-3 1 I Langerhans cell histiocytosis, 3 1 1-3 1 2 mastocytosis, 3 1 1 human immunodeficiency virus, 72 5-7 3 5 diagnosis, 728, 728( history/physical examination, 727-728,

727(

immune reconstitution inflammatory syndrome, 7 3 1 management and prevention, 7 3 1 , 732{, 732t-733t opportunistic infections, 729, 729t-7 301, 730( prophylaxis for opportunistic infections and vaccinations, 7 3 1 -7 34, 734( screening, 729 serology to monitor disease progression, 727-729 transmission, 726, 726t viral anatomy and physiology, 725{, 726 infectious mononucleosis, 724-725 mosquito-borne infections, 721-722 malaria, 72 1-722, 72 1( other, 722 neoplasm-associated, 7 5 1 , 7 5 1 t penetrating trauma, 7 54-7 57

abdomen, 756 chest, 756 extremities, 7 5 7 head, 7 54-7 5 5 neck, 7 5 5-7 56 shock, 7 1 8, 7 1 8t thermal dysregulation, 736-737 hyperthermia, 7 3 7 hypothermia, 736-7 37 tick-borne infections, 722-72 5 babesiosis, 72 3 Lyme disease, 722-723, 723( Rocky Mountain spotted fever, 72 3-724, 724( toxicology, 742-749 antidotes and management, 744t-74 51 carbon monoxide poisoning, 743 drug interactions and reactions, 746, 746t drug adverse effects, 747, 7471-7491 methemoglobinemia, 743-744 resuscitation of poisoned patient, 742-743 trauma management, 7 5 1-754 primary survey, 7 5 2-7 5 3 , 7 5 2{, 7 52t, 7 5 3( secondary survey, 7 5 3-7 54 vitamin deficiencies, 7 50, 7 501 Munchausen by proxy, 62 1 Munchausen syndrome, 62 1 Murmurs, 22, 22f-24f Murphy sign, 244 Muscarinic antagonists, asthma, 6281 Muscular dystrophy Becker, 574, 5741 Duchenne, 572-574, 5 741 Musculocutaneous nerve injury, 3 2 1 1 Musculoskeletal changes, pregnancy, 429t Musculoskeletal disorders, 3 1 3-3 50 lower extremity, 345-348 bursitis, 345 gout, 346-348, 347{, 3471 Morton neuroma, 346 osteochondritis dissecans, 345, 345( patellofemoral pain syndrome, 346 pes anserinus pain syndrome, 345-346 pseudogout, 347{, 3471, 348, 348( pediatric, 5 72-577 Becker muscular dystrophy, 574, 574t clubfoot (talipes equinovarus), 5 7 5 developmental dysplasia o f the hip, 57 5-576, 575( Duchenne muscular dystrophy, 572-574, 574t Legg-Calve-Perthes disease, 5 76, 576( metatarsus adductus, 5 7 5 myotonic dystrophy, 5 74 orthopedic injuries, 5 72, 5731 scoliosis, 577 slipped capital femoral epiphysis, 576-577, 577( spondylolisthesis, 574 trunk, 348-3 5 0 herniated disk, 349, 349( low back pain, 348-349 spinal stenosis, 349-3 50, 3 501 spondylolisthesis and spondylosis, 3 50

upper extremity, 3 39-345 adhesive capsulitis, 3 39 avascular necrosis, 343-344, 343( carpal tunnel syndrome, 342, 342( compartment syndrome, 340-34 1 Dupuytren contracture, 343 ganglion cyst, 342-343, 343( hand infections and bite wounds, 344-345 pronator syndrome, 342 Raynaud phenomenon, 344 rhabdomyolysis, 341 rotator cuff injuries, 340, 340t whole body, 3 1 4-3 39 adult orthopedic injuries, 3 1 4, 3 1 41-3 1 81 ankylosing spondylitis, 3 3 0-332, 3 3 lf Behc;et syndrome, 3 38 complex regional pain syndrome, 322-323 enteropathic spondylitis, 3 3 1 fibromyalgia, 3 3 8, 3 3 8t giant cell arteritis, 3 36-3 37, 3 3 7( myofascial pain syndrome, 3 34 osteoarthritis, 327-329, 328t, 329( osteomyelitis, 326-327, 327{, 3271 osteoporosis, 1 37-1 39, 1 3 8( osteosarcoma, 32 3-324, 324t, 3 2 5( Ottawa ankle rules, 3 1 9, 3 1 9( peripheral nerve injuries, 320, 320t-3221, 322{, 3 2 3( polymyalgia rheumatica, 3 381, 3 39 polymyositis and dermatomyositis, 3 3 2-3 3 3 , 3 3 21, 3 3 3t psoriatic arthritis, 3 3 1 , 3 3 1( reactive arthritis, 3 3 1 rheumatoid arthritis, 328t, 329-3 30 Salter-Harris pediatric fracture classification, 3 1 9-320, 320( septic arthritis, 3 2 5 , 32 5t, 326{, 3261 seronegative spondyloarthropathy, 3 3 0-332, 3 3 1( serum sickness-like reaction, 3 3 6 systemic lupus erythematosus, 3 3 5-3 36, 3 3 5( systemic sclerosis, 3 34-3 3 5 Takayasu arteritis, 3 37-3 3 8 temporomandibular joint disorders, 3 3 3-334 unhappy triad of knee injury, 3 1 9, 3 1 9( MVP (mitral valve prolapse), 2 3{, 24{, 741 Myasthenia crisis, 3 84, 3 8 5 Myasthenia gravis (MG), 384-3 8 5 , 384{, 3851 neoplasms, 7 5 1 t Myasthenic syndrome, Lambert-Eaton, 3 8 5-3 86, 3851 Mycobacterial infections, 657---059, 6 57f-6 59f nontuberculous, 6 5 8-6 59 tuberculosis, 657-6 58, 65 7f-659f Mycobaclerium avium complex (MAC), 6 5 8-6 59 HIV, 730t Mycobaclerium avium-inlracellulare, HIV, 730t Mycobaclerium leprae, 107 Mycobaclerium tuberculosis, 657-6 58, 6 57f-6 59f HIV, 7291

INDEX Mycophenolate mofetil, adverse effects, 7481 Mycosis(es), cutaneous, 1 09- 1 1 0, 1 09(, 1 1 0( Mycosis fungoides, 1 2 1-1 22, 1 2 1(, 3051 Myelitis, transverse, 3 591 Myeloblasts, 302( Myelodysplastic syndrome, 295 Myelofibrosis, 295 Myeloma, multiple, 307-308, 307( Myocardial contusion, 761-762, 762( Myocardial infarction (Ml) anterior, 52, 5 3( inferior, 52, 52( lateral, 52 non-ST-segment elevation, 49-5 1 , 50( posterior, 5 3 ST-elevation, 2 1 , 2 lf ST-segment elevation, 50(, 5 1-54, 52(, 5 3(, 541 Myocarditis, 461 Myoclonic seizures, 3 7 5( Myoclonus, "negative," 4021 Myofascial pain syndrome, 3 34 Myopia, 4 1 6 Myotonic dystrophy, 5 74 infantile hypotonia, 5 59, 5 591 Myx edema coma, 1 3 5 M yxoma, atrial, 24

N Naltrexone, 6 1 3 Narcissistic personality disorder, 6081 Narcolepsy, 6 1 8-6 1 9 Narcotic withdrawal, neonatal, 527-528 Nasal polyps, 670 Nasal trauma, 7 5 5 NASH (nonalcoholic fatty liver disease), 2 5 9 Nasopharyngeal carcinoma, infectious mononucleosis, 725 Nasotracheal intubation, emergency airway, 7 5 2 NAT (nonaccidental trauma), pediatric, 577-579, 5 781 Natalizumab, multiple sclerosis, 388 Natural family planning, 4801 Nausea, due to chemotherapy, 307 NBTE (non-bacterial thrombotic endocarditis), 67, 681 Near miss, 1 84t Nearsightedness, 4 1 6 N E C (necrotizing enterocolitis), 5 3 8, 5 3 8( Neck, penetrating trauma, 7 5 5-7 56 Necrobiosis lipoidica, 1 22, 1 22( Necrosis, avascular (ischemic), 343-344, 343( Necrotizing arteritis, 3 3 7( Necrotizing enterocolitis (NEC), 5 3 8, 5 3 8( Necrotizing fasciitis, 1 03(, 1 0 5, 1 0 5( Necrotizing mediastinitis, acute, 662 Neer sign, 340, 3401 "Negative myoclonus," 4021 Negative predictive value (NPV), 1 59- 1 60 Negative symptoms, schizophrenia, 590, 592 Negligence, 1 841 Neisseria gonorrhoeae, 7 1 2, 7 1 2( cervicitis, 489 conjunctivitis, 4 1 4, 4 1 4(, 4 1 51

neonatal ocular infections, 5 5 3-5 54, 5 541 pediatric, 578 pelvic inflammatory disease, 489-490 prostatitis, 7 1 0 septic arthritis, 326, 3261 vaginitis, 489 Neisseria meningitidis, 379, 3 791 Neoadjuvant chemotherapy, breast cancer, 496 Neonatal abstinence syndrome, 527-528, 6 1 4 Neonatal acne, 5281 Neonatal cephalic pustulosis, 5281 Neonatal jaundice, 520-524, 52 3t, 524( Neonatal pustular melanosis, 5281 Neonatal respiratory distress syndrome (NRDS), 524-526, 5 2 51 Neonatal systemic lupus erythematosus, 3 3 6 Neonate HIV, 734 ocular infections, 5 5 3-5 54, 5 541 Neonatology, 520-528 Apgar scoring, 520, 5 2 1 t apnea o f prematurity, 526 benign neonatal rashes, 527, 5281 congenital hypothyroidism, 526-527 congenital malformations, 520, 5 2 l t-5221 germinal matrix hemorrhage, 526 neonatal abstinence syndrome, 527-528 neonatal extracranial injuries, 526, 5271 neonatal jaundice, 520-524, 5231, 524( respiratory distress syndrome, 524-526, 5 2 5 1 Neoplasms diseases associated with, 7 5 1 , 7 5 1 1 lung, 646-6 50 lung cancer, 647-650, 6481, 649(, 6501 solitary pulmonary nodule, 646-647, 647(, 6471 malignant. See Cancer of skin, 1 1 7- 1 2 2 actinic keratosis, 1 1 8, 1 1 9( basal cell carcinoma, 1 1 9, 1 20( cherry angiomas (hemangiomas), 1 22, 1 22( cutaneous squamous cell carcinoma, 1 1 8- 1 19, 1 1 9( Kaposi sarcoma, 1 2 1 , 1 2 1( melanoma, 1 1 9- 1 20, 1 20t mycosis fungoid es ( cutaneous T-cell lymphoma), 1 2 1 - 1 22, 1 2 1( necrobiosis lipoidica, 1 22, 1 22( pyogenic granuloma, 1 22, 1 22( seborrheic keratosis, 1 1 7-1 1 8, 1 1 8( Nephritic syndrome, 688-69 1 , 688(, 6891-69 1 1, 692( Nephritis, lupus, 690/ Nephrogenic diabetes insipidus, 142, 1 44, 1 44t Nephrolithiasis, 694-697, 695 1-696t, 696( Nephropathy diabetic, 12 51, 6941 IgA, 6891 membranoproliferative, 6891 membranous, 6931 Nephrotic syndrome, 692, 693t-6941 Neurally mediated syncope, 83 Neuritis optic, 4 I 9-420 vestibular, 378

Neuroanatomy, 3 5 3-36 1 brain, 3 5 3, 3 5 3(, 3 54( circle of Willis and arterial supply/venous drainage of brain, 3 54, 3 54(, 3 5 5( lumbar puncture, 3 56, 3 56( meninges, 3 5 5 , 3 5 5( peripheral and cranial nerves, 3 56-3 58, 3 561, 3 571 reflexes, 3 58, 3 581 spinal cord, 3 5 8, 3 581-3601, 3 6 1( Neuroblastoma, 570-57 1 , 5 70( Neurocognitive disorders, 3 88-395, 3891, 599-602 Alzheimer disease, 390-392, 3901 Creutzfeldt-Jakob disease, 3901, 394-395 delirium, 6001, 60 1 -602, 60 1 1 frontotemporal dementia (Pick disease), 3901, 393 HIV-associated, 390 Lewy body dementia, 3 9 1 1, 395 major, 3891 major (dementia), 599-60 1 , 6001 mild, 3891 vs. normal aging, 3 891 normal-pressure hydrocephalus, 3901, 392, 393-394, 394(, 397 reversible vs. irreversible causes, 389/ types, 390, 3901-39 1 1 vascular dementia, 3901, 392 Neurocutaneous disorders, 406-4 1 0 ataxia-telangiectasia, 4 1 0 neurofibromatosis, 406-407, 406( Sturge-Weber syndrome, 408-409 tuberous sclerosis, 407-408, 407( Von Hippel-Lindau syndrome, 409 N euroendocrine tumors, pancreatic, 266-267 Neurofibroma(s), 406, 406( Neurofibromatosis (NF), 406-407, 406( neoplasms, 7 5 1 1 Neurogenic claudication, 3 50 Neurogenic shock, 7 1 81 Neuroleptic malignant syndrome, 5931 Neurology, 3 5 1-426 aphasia, 372-373 Broca/expressive, 372-373, 373( Wernicke/receptive, 373, 373( clinical neuroanatomy, 3 5 3-36 1 brain, 3 5 3 , 3 5 3(, 3 54( circle of Willis and arterial supply/venous drainage of brain, 3 54, 3 54(, 3 5 5( lumbar puncture, 3 56, 3 56( meninges, 3 5 5, 3 5 5( peripheral and cranial nerves, 3 56-3 58, 3 561, 3 57t reflexes, 3 58, 3 581 spinal cord, 3 58, 3 581-3601, 361( CNS infections, 379-384 brain abscess, 383-384, 3 8 3( cryptococcal meningitis, 3 8 1 encephalitis, 3 82-3 8 3 , 383( meningitis, 379-3 8 1 , 379(, 3 791-3 8 1 1 toxoplasmosis, 38 1-382, 382( coma and encephalopathy, 3 7 1 -372, 3721 CSF profiles, 3801 demyelinating disorders, 3 87-388

INDEX Neurology (Continued) Guillain-Barre syndrome, 380t, 388 multiple sclerosis, 3 59t, 3801, 387-3 88 headaches, 36 1-364, 3 6 1 1 cluster, 3 6 1 1, 362 migraine, 3 6 1 -362, 3 6 1 1 secondary, 363-364 tension-type, 3 6 1 1, 363 trigeminal neuralgia, 364 idiopathic intracranial hyp ertension, 3801, 4 1 0-4 1 2, 4 1 1{ intracranial neoplasms, 403, 4041-4061 movement disorders, 396-402 amyotrophic lateral sclerosis, 3 591, 398, 399-400 Huntington disease, 396--397, 396{ Parkinson disease and parkinsonism, 397-399, 399{ restless legs syndrome, 400-40 I tremors, 40 1 , 40 1(, 4021 Wilson disease (hepatolenticular degen­ eration), 40 1-402 neurocognitive disorders (dementia), 388-395, 3891 Alzheimer disease, 390-392, 3901 Creutzfeldt-Jakob disease, 3901, 394-395 frontotemporal (Pick disease), 3901, 393 Lewy body, 3 9 1 1, 395 major, 3 891 mild, 3 891 vs. normal aging, 3891 normal-pressure hydrocephalus, 3901, 392, 393-394, 394(, 397 reversible vs. irreversible causes, 3891 types, 390, 3901-39 1 1 vascular, 3901, 392 neurocutaneous disorders, 406-4 1 0 ataxia-telangiectasia, 4 1 0 neurofibromatosis, 406-407, 406{ Sturge-Weber syndrome, 408-409 tuberous sclerosis, 407-408, 407{ Von Hippel-Lindau syndrome, 409 neuromuscular junction disorders, 3 84-387 botulism, 386, 3871 Lambert-Eaton myasthenic syndrome, 38 5-386, 3851 myasthenia gravis, 384-3 8 5 , 3 84(, 3 8 5t nutritional deficiencies, 4 1 0, 4 1 1 I ophthalmology, 4 1 2-42 3 acute dacryocystitis, 4 1 4, 4 1 4{ age-related macular degeneration, 4 1 8-4 1 9, 4 1 9{ blepharitis, 4 1 3 , 4 1 3{ cataract, 4 1 7, 4 1 9{ cavernous sinus syndrome, 422 chalazion, 4 1 2 conjunctivitis, 4 1 4, 4 1 4(, 4 1 51 contact lens keratitis, 4 1 5-4 1 6 corneal abrasion, 4 1 3, 4 1 3{ diabetic retinopathy, 42 1 , 42 1{ glaucoma, 4 1 7, 4 1 7(, 4 1 81 herpes simplex keratitis, 4 1 5, 4 1 5{ hordeolum, 4 1 2, 4 1 2{ Horner syndrome, 422, 423{

hypertensive retinopathy, 42 1 , 42 1{ intranuclear ophthalmoplegia, 423, 423{ leukocoria, 422, 422{ optic neuritis, 4 1 9-420 orbital blowout fracture, 422 orbital (postseptal) cellulitis, 4 1 3-4 1 4 papilledema, 4 1 1 , 4 1 lf, 422, 422{ presbyopia, 4 1 6 preseptal (periorbital) cellulitis, 4 1 3 refractive errors, 4 1 6 relative afferent pupillary defect, 422 retinal detachment, 420, 42 1 , 42 1{ retinal vascular occlusion, 4 1 9, 4201 retinitis pigmentosa, 42 1-422, 422{ uveitis, 4 1 6, 4 1 6{ visual field defects, 4 1 2, 4 1 2{ otology, 42 3-426 conductive hearing loss, 42 5(, 426 malignant otitis externa, 424 otitis externa, 424 otitis media, 42 3-424 sensorineural hearing loss, 42 5, 42 5{ pediatric, 5 57-563 benign familial microcephaly and macrocephaly, 563 breath-holding spells, 562 cerebral palsy, 5 57- 5 58 Chiari malformations, 563, 563{ cranial neoplasms, 560, 5601-56J t febrile seizures, 5 58-5 59 infantile hypotonia, 5 59, 5 591 Rett syndrome, 562-563 spinal dysraphism, 560-562 seizure disorders, 373-376 classification, 374, 3 7 5{ diagnosis, 3 7 5 etiologies by age, 373-374, 3741 history, 374 status epilepticus, 376 treatment, 37 5-376 vascular disorders, 364-37 1 cavernous sinus thrombosis, 369-37 1 intracerebral hemorrhage, 369, 369{ stroke, 36 5-367, 3651, 366(, 367{ subarachnoid hemorrhage, 368-369, 368(, 3801 subdural and epidural hemorrhage, 369, 3701 transient ischemic attack, 364 vertigo, 3 77-379 acute peripheral vestibulopathy (labyrinthitis, vestibular neuritis), 378 benign paroxysmal positional, 377 central vs. peripheral, 377 Meniere disease, 378-379 Neuroma acoustic, 40 51, 407 Morton, 346 Neuromuscular junction disorders, 384-387 botulism, 386, 3871 Lambert-Eaton myasthenic syndrome, 38 5-386, 3851 myasthenia gravis, 384-3 8 5 , 384(, 3851 Neuropathy, diabetic, 1 2 51 N eurosyphilis, 7 1 3, 7 1 4, 7 1 5

Neurovascular injuries, penetrating trauma of extremities, 7 5 7 Neutropenia, 296-298 cyclic, 566 Neutropenic fever, 297-298 Neutrophil(s), hypersegmented, 293, 293{ Neutrophil count, absolute, 296--297 Never event, 1 841 Never-competent person, informed consent, 1 88 Nevus(i) congenital melanocytic, 5831 multiple dysplastic, neoplasms, 7 5 1 1 New York Heart Association (NYHA) classifica­ tion, congestive heart failure, 3 5 , 3 51 News, setting for delivering, 1 92- 1 9 3 NF (neurofibromatosis), 406--407, 406{ neoplasms, 7 5 1 I NHL (non-Hodgkin lymphoma), 304-306, 3041, 3051 Niacin, 5 71 adverse effects, 7481 Niacin deficiency, 7 501 Nicotine abuse, 6 1 21 Niemann-Pick disease, 5 1 81 "Nightstick fracture," 3 1 51 Nikolsky sign, 97t, 1 04 Nipple injury, 466 Nit(s), 1 1 1 Nitrates chronic stable angina, 49 STEMI, 5 3 unstable angina/NSTEMI, 5 0 Nitroglycerin, adverse effects, 7481 Nitroprusside, hypertensive emergency, 63 NNRTis (non-nucleoside/nucleotide reverse transcriptase inhibitors), HIV, 73 31 NNT (number needed to treat), 1 60 Nocardia, 656, 656{ Nodular basal cell carcinoma, 1 1 9, 1 20{ Nodular melanoma, 1 201 Nonaccidental trauma (NAT), pediatric, 5 77-579, 5781 Nonalcoholic fatty liver disease (NASH), 2 5 9 Nonallergic rhinitis, 669-670 Non-bacterial thrombotic endocarditis (NBTE), 67, 681 Nonfocused expression, interviewing, 1 79 Non-germ cell tumors, 7061 Nongonococcal urethritis, 7 1 1 Nonhemolytic, normocytic anemias, 289-290 Non-Hodgkin lymphoma (NHL), 304-306, 3041, 3051 Nonmaleficence, 1 86 Non-nucleoside/nucleotide reverse transcriptase inhibitors (NNRTis), HIV, 7 3 3 1 Non-Q-wave infarcts, 2 1 Nonseminomatous germ cell tumor (NSGCT), 706, 7061 Non-small cell lung carcinoma (NSCLC), 648t, 649 Nonsteroidal anti-inflammatory drugs (NSAIDs) pill esophagitis, 206{ ulcers, 2 1 7 Nonstress test (NST), 452, 452(, 4521, 4 5 3 1

INDEX Non-ST-segment elevation myocardial infarc­ tion (NSTEMI), 49-5 1 , 50{ Nontuberculous mycobacteria, 6 5 8-659 Normal-pressure hydrocephalus (NPH), 390t, 392, 393-394, 394{, 397 Normocytic anemias hemolytic, 290-292, 290{, 292{ nonhemolytic, 289-290 Nonnotensive, 58t Nose and throat clisorclers, 669-674 aclenotonsillar atrophy, 672 benign and malignant laryngeal lesions, 673-674 epistaxis, 67 1 , 67 1{ laryngitis, 672-673 laryngopharyngeal reflux, 673 nasal polyps, 670 rhinitis, 669-670 Nosebleed, 67 1 , 67 1{ Notifiable outbreaks, confidentiality, 1 94 NPH (normal-pressure hydrocephalus), 390t, 392, 393-394, 394{, 397 NPV (negative predictive value), 1 59- 1 60 NRDS (neonatal respiratory distress syndrome), 524-526, 5 2 5 t NRT!s (nucleosicle/nucleoticle reverse transcrip­ tase inhibitors), HIV, 732t NSA!Ds (nonsteroiclal anti-inflammatory drugs) pill esophagitis, 206{ ulcers, 2 1 7 NSCLC (non-small cell lung carcinoma), 648t, 649 NSGCT (nonseminomatous germ cell tumor), 706, 706t NST (nonstress test), 452, 452{, 452t, 453 t Nuchal translucency, 43 2 Nucleosicle/nucleoticle reverse transcriptase inhibitors (NRT!s), HIV, 7 3 2t Number needed to treat (NNT) , 1 60 Nummular eczema, 98, 98{ NURS technique, interviewing, 1 80 Nursemaid's elbow, 573t Nutrition, pregnancy, 430t Nutritional deficiencies, neurologic syndromes, 4 1 0, 4 1 1 t NYHA (New York Heart Association) classifica­ tion, congestive heart failure, 3 5, 3 5t

0 OA (osteoarthritis), 327-329, 328t, 329{ Oat cell carcinoma, 648t Obesity hypoventilation syndrome (OHS), 668-669 Observational study, 1 62, 1 64{ Observer bias, 167 Obsessive-compulsive clisorcler, 597, 597t Obsessive-compulsive personality disorder, 608t Obsessive-compulsive-related disorders, 597-598, 598t Obstetrics, 427-466 abnormal labor and delivery, 4 57-462 episiotomy, 461 failure to progress, 459, 4 59t fetal malpresentation, 460-46 1 , 460{

indications for C-section, 457, 4 5 7t intra-amniotic infection, 460 preterm labor, 457-45 8 rupture o f membranes, 4 5 8-459 shoulder clystocia, 46 1 , 46 1{ umbilical cord prolapse, 46 1 uterine inversion, 462 uterine rupture, 462 abortion, 436-438, 437t, 438t basics of pregnancy, 428 congenital infections, 4 3 5, 4 3 5t-4 36t diagnosis of pregnancy, 4 28 lactation and breastfeeding, 464-466, 464{, 465t maternal complications of pregnancy, 43 8-445 acute fatty liver, 445 diabetes, 439-44 1 , 440t h yp eremesis graviclarum, 4 3 8-439 h yp ertensive disease, 44 1-444, 443t intrahepatic cholestasis, 444-445 urinary tract infection and asymptomatic bacteriuria, 444 normal labor and delivery, 454-456 analgesia and anesthesia, 45 5-456 definition and stages of labor, 454, 4 54t fetal heart rate monitoring, 4 5 5 , 4 5 6t obstetric examination, 454-4 5 5 obstetric complications o f pregnancy, 445-454 antepartum fetal surveillance, 4 5 1-454, 452{, 452t, 4 5 3 t antepartum hemorrhage and abnormal placentation, 446-448, 447t, 448{ ectopic pregnancy, 445, 445{ fetal growth restriction, 449 fetal macrosomia, 449 gestational trophoblastic disease, 446, 446{, 446t multiple gestation, 448 oligohyclramnios, 450 polyhyclramnios, 450 Rh isoimmunization, 45 1 , 4 5 1{ physiologic changes in pregnancy, 429, 429t430t, 430{ prenatal care and diagnostic testing, 430-4 3 3 amniocentesis, 4 3 3 , 4 3 3 t cell-free fetal DNA, 43 3t chorionic villus sampling, 432, 4 3 3{, 433t Group B streptococcus, 4 3 1 nuchal translucency, 432 quadruple screening, 432, 432t recommendations, 430, 430t schedule, 43 1 , 43 l t teratology, 434, 4341 puerperium, 462-464 peripartum carcliomyopathy, 464 postpartum hemorrhage, 462, 4631 postpartum infection, 462-464 Sheehan syndrome (postpartum pituitary necrosis), 464 Obstructive lung disease, 626-63 1 asthma, 626-628, 6281, 6291 bronchiectasis, 628-630, 629{ chronic, 630-63 1 , 6301-632t, 63 1{

lung volumes, 626, 626{ restrictive vs., 626, 6261, 627{ Obstructive shock, 7 1 81 Obstructive sleep apnea (OSA), 6 1 9, 667-668 Obtunclation, 3 7 1 Obturator nerve injury, 3 2 1 1 Obturator sign, 2 3 0 Occipital lobe, 3 5 3( OCD (osteochoncl r itis cl i ssecans), 345, 345{ OCPs (oral contraceptive pills), 4791 Ocrelizumab, multiple sclerosis, 388 Ocular infections, neonate, 5 5 3-5 54, 5 541 Ocular rosacea, 1 1 5 Oculomotor deficit, parkinsonism, 398 ODD (oppositional defiant disorder), 5 87-588 Odds ratio (OR), 1 6 1 , 1 6 1 { Oclynophagia, 20 3-204, 204{ OHS (obesity hypoventilation syndrome), 668-669 OJ (osteogenesis imperfecta), 578 OI(s) (opportunistic infections) HIV, 727-729, 7291-7301, 730{ prophylaxis, 7 3 1 -734, 734{ pulmonary fungal, 65 5t Oligoclenclroglioma, 4041 Oligohyclramnios, 450 Oligomenorrhea, 476 Omphalocele, 520, 5221 Oncology. See Cancer Onclansetron, nausea clue to chemotherapy, 307 Onychomycosis, 1 1 0 Open-angle glaucoma, 4 1 7, 4 1 7{, 4 1 81 Open-ended skills, interviewing, 1 80 Ophthalmology, 4 1 2-42 3 acute clacryocystitis, 4 1 4, 4 1 4{ age-related macular degeneration, 4 1 2{, 4 1 8-4 1 9, 4 1 9{ blepharitis, 4 1 3 , 4 1 3{ cataract, 4 1 7, 4 1 9{ cavernous sinus syndrome, 4 22 chalazion, 4 1 2 conjunctivitis, 4 1 4, 4 1 4{, 4 1 51 contact lens keratitis, 4 1 5-4 1 6 corneal abrasion, 4 1 3 , 4 1 3{ diabetic retinopathy, 42 1 , 42 1{ glaucoma, 4 1 7, 4 1 7{, 4 1 81, 4 1 9{ herpes simplex keratitis, 4 1 5 , 4 1 5{ horcleolum, 4 1 2, 4 1 2{ Horner syndrome, 422, 423{ hypertensive retinopathy, 42 1 , 42 1{ intranuclear ophthalmoplegia, 423, 423{ leukocoria, 422, 422{ optic neuritis, 4 1 9-420 orbital blowout fracture, 422 orbital (postseptal) cellulitis, 4 1 3-4 1 4 papilleclema, 4 1 1 , 4 1 lf, 422, 422{ presbyopia, 4 1 6 preseptal (periorbital) cellulitis, 4 1 3 refractive errors, 4 1 6 relative afferent pupillary defect, 422 retinal detachment, 420, 42 1 , 42 1{ retinal vascular occlusion, 4 1 9, 420t retinitis pigmentosa, 42 1-422, 422{ uveitis, 4 1 6, 4 1 6{ visual field defects, 4 1 2 , 4 1 2{ Ophthalmoplegia, intranuclear, 423, 423{

INDEX Opioid(s) abuse, 6 1 0, 6 1 1 1 osteoarthritis, 329 overdose, 74 51 synthetic, abuse, 6 1 1 1 withdrawal, 6 1 41 Opportunistic infections (Ols) HIV, 727-729, 7291-7301, 730( prophylaxis, 7 3 1 -734, 734( pulmona ry fungal, 65 51 Oppositional defiant disorder (ODD), 587-588 Optic neuritis, 4 1 9-420 Optic neuropathy, anterior ischemic, 3 37 OR (odds ratio), 161, 1 6 1( Oral cancers, 20 I Oral candidiasis, 108- 1 09, 203 HIV, 727, 727{, 7291 Oral cavity, penetrating trauma, 7 5 5 Oral contraceptive pills (OCPs), 4791 Oral contraceptive taper, 48 1 1 Oral hai ry leukoplakia, 200 HIV, 7291 Oral herpes, 98-1 00, 991 Oral infections, 662 Oral lesions, 1 99-20 I, 1 99{, 1 991 Oral leukoplakia, 1 99{, 200 Oral lichen planus, 20 I Oral submucosal fibrosis, 1 99{, 200 Orbital cellulitis, 4 1 3-4 1 4 Orbital fractures, 7 5 4 blowout, 422 Organophosphates, toxic ingestion, 7441 Orogastric tube, advanced trauma life support, 754 Oropharyngeal candidiasis, 1 09 Oropharyngeal dysphagia, 204 Orthopedic injuries common adult, 3 1 4, 3 1 41-3 1 81 pediatric, 572, 5731 penetrating trauma of extremities, 757 Orthostatic syncope, 83, 851 Ortolani maneuver, 5 7 5 , 5 7 5( OSA (obstructive sleep apnea), 6 1 9, 667668 Osgood-Schlatter disease, 5731 Osler nodes, endocarditis, 69, 69f Osmotic agents, 3 81, 6881 Osmotic demyelination syndrome, 678, 678( Osmotic gap, stool, 22 1 1 Osteoarthritis (OA) , 327-329, 3281, 329( Osteoblastoma, 3241 Osteochondritis dissecans (OCD), 345, 345( Osteochondroma, 324, 3241 Osteogenesis imperfecta (01), 578 Osteoid osteoma, 3241 Osteomyelitis, 326-327, 327{, 3271 Osteonecrosis, 343-344, 343( Osteopenia, 1 3 8 Osteoporosis, 1 37-1 39, 1 3 8( postmenopausal, 486 Osteosarcoma, 3 2 3-3 24, 3241, 3 2 5( Ewing sarcoma vs. , 5 7 1 , 5721

Otitis externa, 424 malignant, 424 Otitis media, 423-424 acute, 548 with effusion, 423-424 Oto! iths, 3 77 Otology, 42 3-426 conductive hearing loss, 42 5{, 426 malignant otitis externa, 424 otitis externa, 424 otitis media, 42 3-424 sensorineural hearing loss, 42 5, 42 5( Otorrhea, basilar skull fracture, 7 5 8 Ottawa ankle rules, 3 1 9, 3 1 9( Outcome measures, 1 84 Ovarian cancer, 503-505, 5041, 5051 Ovarian cycle, normal, 468-469, 468( Ovarian cyst( s) follicular (physiologic), 4981 nonneoplastic, 498, 4981 precocious puberty, 470 ruptured, 227 Ovarian failure, premature, 472( Ovarian insufficiency, primary, 4 70 Ovarian masses, 504-505 Ovarian torsion, 490-49 1 acute abdomen, 227 Ovarian tumor, precocious puberty, 4 70 Overflow incontinence, 7001 Ovulation, 469 Ovulatory bleeding, 477 Ovulatory factors, infertility, 4851 Oxygen congestive heart failure, 3 8 unstable angina/NSTEMI, 5 0 Oxytocin, 4 5 5

p P mitrale, 2 1 P pulmonale, 2 1 P value, 1 68- 1 69 Packed red blood cells (PRBCs), 2741 Paget disease of bone, 1 39, 1 39{, 1 40( neoplasms, 7 5 1 I PAH (pulmonary arterial hypertension), 643 Painful bladder syndrome, 70 I Palatine tonsils, 672 Palliative care, 1 79 Palmar grasp reflex, 5 1 31 PALM-COEIN acronym, 476 p-ANCA, 3 3 31 Pancoast tumors, 648 Pancreas, blunt abdominal trauma, 7621 Pancreatic cancer, 267 Pancreatic cysts, 264 Pancreatic disease, 264-267 pancreatic cancer, 267 pancreatic cysts, 264 pancreatic neuroendocrine tumors (PNETs), 266-267 pancreatitis, 264, 2651 Pancreatic islet cell tumors, multiple endocrine neoplasia, 1 5 5

Pancreatic neuroendocrine tumors (PNETs), 266-267 Pancreatitis, 264, 2651 dyslipidemia, 57 Panic attacks, 596 Panic disorder, 59 5-596 Panniculitis, 96 Pantothenate deficiency, 7 501 Pap smear, 1 741, 500, 501{, 5001 Papillary muscle rupture, 72 Papillary thyroid carcinoma, 1 371 Papilledema, 4 1 1 , 4 1 1{, 422, 422( Papilloma, intraductal, 493 Papillomatosis, recurrent respiratory, 674 Papule, 881 Papulopustular rosacea, 1 1 4 Paraesophageal hiatal hernia, 2 1 1{, 2 1 1 Paralysis, tick-borne, 388 Paramethadione, pregnancy, 4341 Paramyxovirus, 5 561 Paraneoplastic syndromes, lung cancer, 6 501 Paranoid personality disorder, 6081 Paraphilic disorders, 6 1 6, 6 1 71 Parapneumonic effusion, 666, 6671 Parasitic skin infections, 1 1 0- 1 1 2 bed bugs, 1 1 2 cutaneous larva migrans, 1 1 2 lice, 1 1 0- 1 1 1 scabies, 1 1 1- 1 1 2 , 1 1 lf Parathyroid hormone (PTH) calcium and phosphate regulation, 1 3 8( excess, 1 39- 1 4 1 , 1 401 Parathyroid hormone-related protein (PTHrP), ectopic, 1 401 Paravalvular abscess, endocarditis, 69 Parietal lobe, 3 5 3( Parity, 428 Parkinson disease (PD), 397-399, 399( Parkinson disease dementia (PDD), 3 9 1 1, 395 Parkinsonian tremor, 397, 4021 Parkinsonism, 397-399, 399( pseudo-, 5931 Parkland formula, 739 Paroxysmal nocturnal hemoglobinuria (PNH), 291 Partial seizures, 374, 375, 3 7 5( complex, 3 74, 3 7 5f simple, 374, 3 7 5( Partial thromboplastin time (PTT), 270, 275{, 279 Partnership, rapport, 1 801 Parvovirus B 1 9, 5 561 Passive aggression, 6091 Patau syndrome, 5 1 61 Patch, 881 Patch testing, 9 I Patellar reflex, 3 581 Patellofemoral pain syndrome, 346 Patent ductus arteriosus (PDA), 2 3{, 5 3 1 , 5 3 1( myocardial infarction involving, 52, 52f Patient(s), gifts from, I 9 5 Patient-centered, evidence-based interviewing, 1 79- 1 80 PCC (prothrombin complex concentrate), 2741

INDEX PCL (posterior cruciate ligament) injury, 3 1 71 PCOS (polycystic ovarian syndrome), 483-484, 484{ PCP (Pneumocyslis carinii pneumonia), 659-660, 659{ HIV, 7291 PC S (postcholecystectomy syndrome), 244, 247 PC SK9 (proprotein convertase subtilisin/kexin type 9) inhibitors, 571 PCV (polycythemia vera), 295 PCVI 3, PCV I 5, PCV20 (pneumococcal conju­ gate) vaccine, 1 7 1(-1 73{ PD (Parkinson disease), 397-399, 399{ PDA (patent ductus arteriosus), 2 3(, 5 3 1 , 5 3 1{ myocardial infarction involving, 52, 52{ POD (Parkinson disease dementia), 3 9 1 1, 395 PDE-5 (phosphodiesterase-5) inhibitors, 702 PCP (phencyclidine hydrochloride), abuse, 6 1 21 PDSA cycle, 1 83, 1 8 3{ PE. See Pulmonary embolism (PE) Peak inspiratory pressure (Pib k), 6401 PEARLS model, rapport, 1 80, 1 801 Pediatrics, 509-583 child abuse, 577-579, 5 781 child development, 5 1 1-5 1 4 developmental milestones, 5 1 1 , 5 1 21, 5 1 31 growth, 5 1 1-5 14 primitive reflexes, 5 1 1 , 5 1 31 sexual, 5 1 4, 5 1 5{ congenital heart disease, 528-534 acyanotic left-to-right shunts, 528, 529-532 coarctation of aorta, 5 3 1-532, 5 3 2{ cyanotic right-to-left shunts, 528-529, 5 3 2-534 genetic syndromes, 528, 5291 patent ductus arteriosus, 5 3 1 , 53 lf septa! defects, 529-5 30, 5 301 tetralogy of Fallot, 53 3-5 34, 53 3f transposition of the great vessels, 5 32-5 3 3, 5 3 3{ gastroenterologic disease, 5 34-540 constipation, 5 3 9-540 food protein-induced allergic proctocolitis, 5 38-5 39 Hirschsprung disease, 5 3 7, 5 3 7{ intussusception, 5 3 5-536, 5 3 5{ malrotation with volvulus, 5 36, 5 3 6{ Meckel diverticulum, 5 36-5 3 7, 5 3 6{ necrotizing enterocolitis, 5 3 8, 5 3 8{ pyloric stenosis, 5 34- 5 3 5 , 5 34{ genetic disease, 5 1 4-520 autosomal chromosome abnormalities (trisomies), 5 1 61 cystic fibrosis, 5 1 4-520 inherited metabolic disorders, 5 1 71-5 1 81 other, 5 1 91 sex chromosome abnormalities, 5 1 71 hematology, 564-569 cyclic neutropenia, 566 Diamond-Blackfan anemia, 564-565, 5641 Fanconi anemia, 5641, 56 5-566, 5651 Kasabach-Merritt syndrome, 567, 567{

sickle cell disease, 567-569, 568(, 569{ thrombocytopenia absent radius syn­ drome, 566 immunology, 542-548 B-cell disorders, 5431, 545 combined disorders, 5441 complement disorders, 5451, 546 immunodeficiency disorders, 542-546, 5431, 5451 juvenile idiopathic arthritis, 547-548, 5471 Kawasaki disease, 546 phagocytic deficiencies, 542(, 5441-5451, 546 T-cell disorders, 5431, 546 infectious disease, 548- 5 5 7 acute otitis media, 548 bronchiolitis, 549, 5 5 2{ croup (laryngotracheobronchitis), 5 50, 5 50(, 5 501, 5 5 2{ epiglottitis, 5 5 01, 5 5 1-552, 5 5 1 1, 5 5 2(, 5 5 3{ meningitis, 5 52-5 5 3 ocular, 5 5 3-5 54, 5 541 pertussis (whooping cough), 5 54- 5 5 5 pinworm, 5 5 5 TORCH infections, 4 3 5 , 4 3 5 1-4361 tracheitis, 5 501 viral exanthems, 5 5 5, 5 561-5 571 musculoskeletal disorders, 572-577 Becker muscular dystrophy, 5 74, 5741 clubfoot (talipes equinovarus), 5 7 5 developmental dysplasia o f the hip, 57 5-576, 5 7 5{ Duchenne muscular dystrophy, 572-574, 5741 Legg-Calve-Perthes disease, 576, 5 76{ metatarsus adductus, 5 7 5 myotonic dystrophy, 574 orthopedic injuries, 572, 5731 scoliosis, 577 slipped capital femoral epiphysis, 576-577, 5 77{ spondylolisthesis, 5 74 neonatology, 520-528 Apgar scoring, 520, 5 2 1 1 apnea o f prematurity, 526 benign neonatal rashes, 527, 5281 congenital hypothyroidism, 526-527 congenital malformations, 520, 5 2 1 1-5221 germinal matrix hemorrhage, 526 neonatal abstinence syndrome, 527-528 neonatal extracranial injuries, 526, 5271 neonatal jaundice, 520-524, 52 31, 524{ respiratory distress syndrome, 524-526, 5251 neurologic disease, 5 57-563 benign familial microcephaly and macrocephaly, 563 breath-holding spells, 562 cerebral palsy, 5 57-5 58 Chiari malformations, 563, 563{ cranial neoplasms, 560, 5601-561 t febrile seizures, 5 5 8- 5 59 infantile hypotonia, 5 59, 5 591 Rett syndrome, 562-563 spinal dysraphism, 560-562

oncology, 569-572 bone tumors, 5 7 1 , 5 721 Langerhans histiocytosis, 5 7 1 leukemia, 569-570 neuroblastoma, 570-57 1 , 570{ Wilms tumor, 5 7 1 traumatic brain injury, 7 5 8-7 59 urology, 540-542 cryptorchidism, 541 hypospadias and epispadias, 54 If, 542, 5421 inguinal hernia, 541 vesicoureteral reflux, 540, 54 If well child care, 579-582 anticipatory guidance, 579 hearing and vision screening, 5 79-580 lead poisoning, 580-582, 5 8 1 (, 7451 perianal dermatitis, 582, 5821 pigmented lesions, 582, 5831 vaccinations, 580 Pedophilic disorder, 6 1 71 PEEP (positive end-expiratory pressure), 639, 6401 Pellagra, 224 Pelvic exam, 1 741 Pelvic factors, infertility, 4851 Pelvic fractures, 7 6 3 Pelvic inflammatory disease (PIO), 489-490, 7 1 1 , 7 1 1(, 7 1 2{ acute abdomen, 227 Pelvic masses, benign vs. malignant, 5041 Pelvic organ prolapse, 506, 506{ Pelvic pain syndrome, chronic, 700 Pelvic trauma, blunt and deceleration, 763-764 Pemphigoid, bullous, 96, 97(, 971 Pemphigus vulgaris, 96, 97(, 971 "Pencil-in-cup" deformity, 3 3 1 , 3 3 1{ Penetrating trauma, 7 54-7 57 abdomen, 756 chest, 756 extremities, 7 5 7 head, 7 54-7 5 5 neck, 7 5 5-7 56 Penicillamine, adverse effects, 7481 Penicillin, adverse effects, 7481 Penicillin allergy, 327 Peptic ulcer disease (PUD), 2 1 5 , 2 1 51, 2 1 6-2 1 7,

2 1 6{

Perception challenging conversations, 1 8 1 delivering news, 1 92 Performance anxiety disorder, 596, 597 Perianal dermatitis, 582, 5821 Perianal streptococcus, 5821 Pericardia! disease, 64-67 acute pericarditis, 64-6 5, 64(, 65{ cardiac tamponade, 67 constrictive pericarditis, 6 5-66 pericardia! effusion, 66-67, 66{ Pericardia! effusion, 66-67, 66{ Pericardia! stripping, constrictive pericarditis, 66 Pericarditis acute, 64-6 5, 64(, 6 5{ constrictive, 6 5-66

INDEX Perinatal transmission, HIV, 726t, 728, 734 Periorbital cellulitis, 4 1 3 Peripartum cardiomyopathy, 46t, 464 Peripheral arterial disease, 8 1-82 Peripheral edema, 2 3 Peripheral nerve(s), 3 56-3 58, 3 5 6t, 3 57t Peripheral nerve injuries, 320, 320t-322t, 322(, 3 2 3( Peripheral neuropathy, 4 1 l t Peripheral precocious puberty, 469-47 0 , 469t Peripheral pulses, 2 3 Peripheral vascular resistance, pregnancy, 429t Peripheral vestibulopathy, acute, 378 Peritonitis, spontaneous bacterial, 252, 2 54-2 56, 2 5 5t, 2 5 6 Peritonsillar abscess, 5 5 l t, 662 Pernicious anemia, 293-294, 293( neoplasms, 7 5 l t type A gastritis, 2 14-2 1 5 Persistent depressive disorder, 602t, 604-605 Persistent vegetative state (PVS), 372, 372t Personality disorders, 607-6 10, 608t-609t Person-time estimate, 1 5 8 Pertussis, 5 54-5 5 5 Pes anserinus pain syndrome, 345-346 Petit ma! seizures, 3 74, 375, 375( Peutz-Jeghers syndrome, 2 3 8, 2 3 8( Pfizer-BioNTech vaccine, 1 7 3 PFTs (pulmonary function tests), 626, 626(, 626t, 627( interstitial lung disease, 63 3 PCB (porcelain gallbladder), 244 PH (pulmonary hypertension), 643 Phagocytic deficiencies, pediatric, 542f, 544t545t, 546 Phalen maneuver, 342, 342( Phantom limb pain, 757 Pharyngitis, acute, 66 1-662, 66 1(, 66 l t Phase 1 clinical trial, l 66t Phase 2 clinical trial, l 66t Phase 3 clinical trial, l 66t Phase 4 clinical trial, l 66t Phencyclidine hydrochloride (PDP), abuse, 6 1 2t Phenobarbital, toxic ingestion/overdose, 745t Phenylketonuria (PKU), 5 l 7t Phenytoin pregnancy, 4 34t adverse effects, 748t Pheochromocytoma, 1 50, 1 50( Von Hippel-Lindau syndrome, 409 Philadelphia chromosome, 303, 304 Phlegmasia alba dolens, 79 Phlegmasia cerulea dolens, 79, 79( Phobias, 596-597 Phosphate regulation, 1 3 7, 1 3 8( Phosphodiesterase-5 (PDE-5) inhibitors, 702 Phototherapy, depression, 603 Phyllodes tumor, 494, 494( Phymatous rosacea, 1 1 5 , 1 1 5( Physical abuse, 62 1-622 Physical exam, cardiac, 22-24, 22(-24(, 23t Physician orders for life-sustaining treatment (POLST), 1 89 Physiologic ovarian cysts, 498t

Physiologic tremor, 402t Phytobezoars, 2 1 8 Pia mater, 3 5 5, 3 5 5( PICA (posterior inferior cerebellar artery), stroke, 365t Pick disease, 390t, 393 PIO (pelvic inflammatory disease), 489-490, 7 1 1 , 7 1 1(, 7 1 2( acute abdomen, 227 Pigmented lesions, childhood, 582, 58 3t Pill esophagitis, 20 5-206, 206( Pilocytic astrocytoma, 560t Pilonidal cysts, I 07, I 07f Pinealoma, 5 6 l t "Pink babies," 528 "Pink puffer," 630 Pinpoint pupils, 6 1 0 Pinworm infection, 5 5 5 Pioglitazone, diabetes, l 28t Pituitary adenoma, 405t growth hormone-secreting, 144-146, 145( prolactin-secreting, 146-147, 147( Pituitary and hypothalamic disorders, 141-147 acromegaly, 144-146, 145(, 146( deficiency of pituitary hormones, 141- 142, 143t diabetes insipidus, 142-144, 142(, 144(, 144t excess of pituitary hormones, 144-147 hyp erprolactinemia, 146-147, 147( hyp othalamic-pituitary axis and, 1 4 1 , 14 lf syndrome of inappropriate antidiuretic hormone secretion, 147 Pituitary apoplexy, 141 Pituitary hormones deficiency, 141-142, 143t excess of, 144- 147 Pituitary necrosis, postpartum, 464 Pituitary-thyroid axis, pregnancy, 4 30(, 4 30t Pityriasis rosea, 1 1 5, 1 1 5( PKU (phenylketonuria), 5 l 7t Placenta accreta spectrum, 448( Placenta increta, 448( Placenta percreta, 448( Placenta previa, 447t, 448f Placental abruption, 447t, 448f Placental tissue, retained, postpartum hemorrhage, 463t Placentation, abnormal, 446-448, 447t, 448( Plantar reflex, 5 l 3t Plaque, 88t Plasma, fresh frozen, 274t Plasma cell disorders, 307-309 amyloidosis, 308-309, 309t multiple myeloma, 307-308, 307( Waldenstrom macroglobulinemia, 308 Plasmodium spp, 72 1-722, 721( Plateau pressure (PPl;,,), 640t Platelet(s), 274t Platelet disorders, 28 1-285 characteristics, 27lt hemolytic uremic syndrome (HUS), 282-283, 283t, 284t idiopathic thrombocytopenic purpura (ITP, immune thrombocytopenia), 284-28 5

thrombotic thrombocytopenic purpura (TIP), 281-282, 282(, 283t Platelet phase, hemostasis, 270 Platelet plug, 270 Pleomorphic adenomas, salivary glands, 203t Pleural disease, 664-667 pleural effusion, 664-666, 665t-667t pneumothorax, 665t, 666-667, 667( Pleural effusion, 664-666, 665t-667t Pleural friction rub, 664 Plugged duct, 466 Plummer-Vinson syndrome, 207 neoplasms, 7 5 I t P M (polymyositis), 3 32-3 3 3 , 3 3 2t, 3 3 3t PMDD (premenstrual dysphoric disorder), 605 PNETs (pancreatic neuroendocrine tumors), 266-267 Pneumatosis intestinalis, 5 38, 5 3 8( Pneumococcal conjugate (PCVl 3, PCVI 5, PCV20) vaccine, 1 7 1(-173( Pneumococcal polysaccharide (PPSV23 ) vaccine, 1 7 1(- 1 7 3( Pneumococcal vaccine, 1 7 1(- 1 7 3(, 652 Pneumoconiosis(es), 634-6 3 5 , 6 3 5( Pneumocystis carinii pneumonia (PCP), 659-660, 659( HIV, 729t Pneumocystis iirovecii pneumonia, 659-660, 659( HIV, 729t Pneumomediastinum, tracheobronchial disrup­ tion, 760, 760( Pneumonia, 650-652 chronic eosinophilic, 299-300 congenital, 525t COVID- 19, 642 cryptogenic organizing, 6 3 3 CURB-65 Score, 6 5 1 diagnosis, 6 5 1 , 65 1(, 6 5 l t, 652(, 652t etiology, 65 I, 65 l t, 652(, 652t history/physical examination, 650-6 5 1 lobar, 65 l f, 665t Pneumocystis iirovecii (Pneumocystis carinii),

659-660, 659( HIV, 729t treatment, 652, 6 5 3 t Pneumonitis cytomegalovirus, 73 5 hypersensitivity, 634 Pneumoperitoneum, 2 1 6, 2 1 6(, 228, 228( Pneumothorax, 665t, 666-667, 667( penetrating chest trauma, 756 primary spontaneous, 666 secondary, 666 tension, 666-667, 667( advanced trauma life support, 752(, 7 5 3 ventilator induced, 640 tracheobronchial disruption, 760 PNH (paroxysmal nocturnal hemoglobinuria), 291 Poisoning, 742-745 antidotes and management, 744t-745t carbon monoxide, 743 cyanide, 744, 745t lead, 287, 5 80-582, 581(, 745t

INDEX methemoglobinemia, 74 3-744 resuscitation, 742-743 Poliomyelitis, 3 59t POLST (physician orders for life-sustaining treatment), 1 89 Polyangiitis granulomatosis with, 3 37{, 688, 690t eosinophilic, 299-300, 3 37{, 690t microscopic, 3 3 7{, 688, 690t Polycystic kidney disease, 697-698, 697{ Polycystic ovarian syndrome (PCOS), 483-484, 484{ Polycythemia(s), 295 Polycythemia vera (PCV), 295 Polyhydramnios, 450 Polymenorrhea, 476 Polymyalgia rheumatica, 3 3 8t, 3 3 9 Polymyositis (PM), 3 3 2-3 3 3 , 3 32t, 3 3 3t Polyp(s) adenomatous, 2 36t, 2 3 7 t nasal, 670 vocal cord, 673 Popliteal cyst rupture, 3 l 8t Porcelain gallbladder (PCB), 244 Porphyria, 294, 294t acute intermittent, 294t Porphyria cutanea tarda, 294t Portal hypertension, 2 5 3 , 2 5 3{ Positive end-expiratory pressure (PEEP), 639, 640t Positive predictive value (PPV), 1 59-1 60 Postamputation pain, 7 5 7 Postcholecystectomy syndrome ( P C S ) , 244, 247 Posterior cerebral artery, 3 54{, 3 5 5{ Posterior cerebral circulation, stroke, 365t Posterior communicating artery, 3 54, 3 54{, 3 5 5{ Posterior cruciate ligament (PCL) injury, 3 l 7t Posterior inferior cerebellar artery (PICA), stroke, 365t Posterior myocardial infarction, 52 Posterior rib fractures, child abuse, 5 78t Post-exposure prophylaxis, HIV, 734 Postherpetic neuralgia, I O 1 Postinfectious glomerulonephritis, 688, 689t Postmenopausal vaginal bleeding, 476 Postoperative fever, 7 1 9, 7 1 9{, 7 1 9t Postpartum "blues," 603t Postpartum depression, 602, 603t Postpartum disorders, 602, 603t Postpartum endometritis, 462-463 Postpartum fever, 464 Postpartum hemorrhage, 462, 463t Postpartum infection, 462-464 Postpartum pituitary necrosis, 464 Postpartum psychosis, 603t Postpartum urinary retention, 462 Postphlebitic syndrome, 80 Postseptal cellulitis, 4 1 3-4 1 4 Postsurgical gastric remnants, neoplasms, 75lt Postthrombotic syndrome, 80 Post-traumatic stress disorder (PTSD), 598-599 Postural instability, Parkinson disease, 397 Postural tachycardia syndrome (POTS), 84t Postural tremor, 402t

Potassium (K + )-sparing diuretics, 3 8t, 688t hypertension, 6 l t POTS (postural tachycardia syndrome), 84t PPD (purified protein derivative) test, tuberculosis, 657, 657{ P r,..k (peak inspiratory pressure), 640t Pri,, (plateau pressure), 640t PPROM (preterm premature rupture of mem­ branes), 458 PPSV2 3 (pneumococcal polysaccharide) vaccine, 1 7 1(-1 73{ PPV (positive predictive value), 1 59-1 60 PR interval, 20 PR (progesterone receptor) status, breast cancer, 495, 496 Prader-Willi syndrome, 5 1 9t Pragmatic communication disorder, 5 l 3t Prazosin, adverse effects, 748t PRBCs (packed red blood cells), 274t Preclinical trial, 1 66t Precocious puberty, 469-470, 469t, 5 I 4 Precontemplation stage of change, 1 8 3{, 1 8 3t Predictive values, positive and negative, 1 59- 1 60 Preeclampsia, 442-443, 443t Pre-exposure prophylaxis (PrEP), HIV, 73 1-734 Prefrontal cortex, 3 5 3{ Pregestational diabetes, 4 39-440, 440t Pregnancy basics, 428 cardiovascular conditions, 72 clinical research, 1 94 diagnosis, 428 ectopic, 445, 445{ ruptured, 227 HIV, 734 maternal complications, 4 3 8-44 5 acute fatty liver, 445 diabetes, 4 39-44 1 , 440t hyperemesis gravidarum, 4 3 8-439 hypertensive disease, 44 1-444, 443t intrahepatic cholestasis, 444-445 urinary tract infection and asymptomatic bacteriuria, 444, 707t, 7 1 1 molar, 446, 446{, 446t multiple sclerosis, 387 nonviable, 438 obstetric complications, 445-454 antepartum fetal surveillance, 4 5 1-454, 452{, 452t, 4 5 3 t antepartum hemorrhage and abnormal placentation, 446-448, 447t, 448{ ectopic pregnancy, 445, 445{ fetal growth restriction, 449 fetal macrosomia, 449 gestational trophoblastic disease, 446, 446{, 446t multiple gestation, 448 oligohydramnios, 450 polyhydramnios, 450 Rh isoimmunization, 45 1 , 45 If physiologic changes, 429, 429t-430t, 430{ prenatal care and diagnostic testing, 4 30-4 3 3 amniocentesis, 4 3 3 , 4 3 3 t cell-free fetal DNA, 433t chorionic villus sampling, 432, 4 3 3{, 433t

Group B streptococcus, 4 3 1 nuchal translucency, 432 quadruple screening, 432, 432t recommendations, 430, 430t schedule, 43 1 , 43 l t teratology, 434, 434t vaccines during, 1 73{ Prehyp ertensive, 58t Premature ovarian failure, 472{ Premature rupture of membranes (PROM), 458 Premature ventricular contraction (PVC), 31 t Prematurity, apnea, 5 26 Premenstrual dysphoric disorder (PMDD), 605 Premotor cortex, 3 5 3{ Prenatal care and diagnostic testing, 4 30-4 3 3 amniocentesis, 4 3 3, 4 3 3t cell-free fetal DNA, 4 3 3t chorionic villus sampling, 432, 4 3 3{, 433t Group B streptococcus, 43 1 nuchal translucency, 432 quadruple screening, 432, 432t recommendations, 430, 430t schedule, 43 1 , 43 l t teratology, 4 34, 4 34t PrEP (pre-exposure prophylaxis), HIV, 73 1-734 Preparation stage of change, 1 83{, 1 83t Presbycusis, 377 Presbyopia, 4 1 6 Preseptal cellulitis, 4 1 3 Preterm labor, 457-4 58 Preterm premature rupture of membranes (PPROM), 4 5 8 Prevalence, 1 5 8 Prevention, 1 70 Primaquine, 722 Primary amenorrhea, 470-47 1 , 472t Primary auditory cortex, 3 5 3{ Primary biliary cholangitis, 2 5 9 Primary closure, 2 3 1 delayed, 2 3 I Primary CNS lymphoma, 305t Primary dysmenorrhea, 4 74 Primary intent, 2 3 1 Primary motor cortex, 3 5 3, 3 5 3{ Primary ovarian insufficiency, 4 70 Primary prevention, 1 70 Primary sclerosing cholangitis (PSC), 2 59 Primary somatosensory cortex, 3 5 3, 3 5 3{ Primary survey, trauma management, 7 5 2-7 5 3 , 7 5 2{, 7 52t, 7 5 3{ Primary visual cortex, 3 5 3{ Primitive reflexes, 5 1 1 , 5 1 3t Primordial follicles, 468{ Prinzmetal angina, 49 Procainamide, adverse effects, 749t Process, assessment, 1 84 Proctalgia fugax, 240t Proctitis, radiation, 240t Proctocolitis, food protein-induced allergic, 5 3 8-539 Professional relationship, doctor-patient, 1 86 Progesterone receptor (PR) status, breast cancer, 49 5, 496 Progestin challenge, 473 Progestin-only implant, 479t

INDEX Progestin-only "mi nip ills," 4 79t Progressive multifocal leukoencephalopathy, HIV, 7291 Progressive supranuclear palsy (PSP), 398 Proguanil, 722 Projection, 609/ Prolactin deficiency, 1431 excess, 1 46-1 47, 1 47( Prolactinoma, 146- 147, 147( Proliferative phase, uterine cycle, 468( PROM (premature rupture of membranes), 458 Pronator syndrome (PS), 342 Prophylaxis endocarditis antibiotic, 7 1 1, 72 HIV opportunistic infections, 73 1-734, 7 34( Propionibacterium, 1 06 Propranolol, 5951 Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors, 5 71 Propylthiouracil adverse reactions, 1 34/ adverse effects, 7491 Prospective cohort study, 1 6 3 , 1 64( Prostate, structure, 703( Prostate cancer, 7021, 703-704, 703( Prostate-specific antigen ( PSA), 1 7 51, 70 3, 704 Prostatic hyperplasia, benign, 702-703, 7021 Prostatitis, 699, 700, 7 1 0-7 1 1 Prosthetic valve, endocarditis due to, 68/ Protease inhibitors, HIV, 7 3 3 t Protected health information, 1 9 3 Protein C, 270 Protein C deficiency, 278 Protein S, 270 Protein S deficiency, 278 Proteus mirabilis, urinary tract infection, 7081 Prothrombin complex concentrate (PCC), 2741 Prothrombin time (PT), 270 Prothrombotic states. See Hypercoagulable states PS (pronator syndrome), 342 PSA (prostate-specific antigen), 1 7 51, 703, 704 PSC (primary sclerosing cholangitis), 2 5 9 Pseudoclaudication, 3 5 0 Pseudogout, 347 (, 3471, 348 Pseudomonas conjunctivitis, 4 1 51 contact lens keratitis, 4 1 5-4 1 6 osteomyelitis, 3271 Pseudomonas aeruginosa "hot tub folliculitis," 1 0 5 urinary tract infection, 708t Pseudoparkinsonism, 5931 Pseudothrombocytopenia, 284 Psoas abscess, 2 3 0-2 3 1 Psoas sign, 230 Psoriasis, 92-93, 93( Psoriatic arthritis, 93, 3 3 1 , 3 3 1( juvenile, 5471 PSP (progressive supranuclear palsy), 398 Psychiatry, 5 8 5-62 3 anxiety disorders, 594-597 diagnostic criteria by symptom duration, 6071

generalized, 594-595, 5951 panic disorder, 59 5-596 phobias, 596-597 childhood and adolescent disorders, 586-590 attention-deficit/hyperactivity disorder, 586 autism spectrum disorder, 587 diagnostic criteria by symptom duration, 6071 disruptive behavioral disorders, 587-588 intellectual developmental disorder/intel­ lectual disability, 588 separation anxiety disorder, 5 89-590 Tourette syndrome, 589 diagnostic criteria by symptom duration, 6071 dissociative disorders, 593, 5941 eating disorders, 6 1 4-6 1 6 anorexia nervosa, 6 1 4-6 1 5, 6 1 51 bulimia nervosa, 6 1 51, 6 1 6 factitious disorders and malingering, 62 1 mood disorders, 602-607 adjustment disorder, 6021, 605 bipolar and related disorders, 60 5-607, 605t, 6061 diagnostic criteria by symptom duration, 607t major depressive disorder, 602-60 3, 602t-6041 persistent depressive disorder (dysthymia), 602t, 604-60 5 neurocognitive disorders, 599-602 delirium, 600t, 60 1-602, 60 1 1 major (dementia), 599-60 1 , 6001 obsessive-compulsive and related disorders, 597-598, 597t, 598/ obsessive-{:ompulsive personality disorder, 5971 personality disorders, 607-6 10, 608/-6091 psychotic disorders, 590-593 antipsychotic medications, 59 1 , 5921, 5931 diagnostic criteria by symptom duration, 6071 differential diagnosis, 5 9 1 I schizophrenia, 590, 59 1-592, 5 9 1 1-593t schizophreniform disorder, 591 -592, 5 9 1 1 sexual and physical abuse, 62 1-622 sexual disorders, 6 1 6-6 1 7 gender dysphoria, 6 1 6-6 1 7 paraphilic, 6 1 6, 6 1 71 sexual changes with aging, 6 1 6 sexual dysfunction, 6 1 7 sleep disorders, 6 1 7-6 1 9 circadian rhythm, 6 1 9 narcolepsy, 6 1 8-6 1 9 primary hypersomnia, 6 1 8 primary insomnia, 6 1 8 sleep apnea, 6 1 9 somatic symptom and related disorders conversion disorder, 620-62 1 illness anxiety disorder, 620 somatic symptom disorder, 620 somatic symptoms and related disorders, 620-62 1 substance use disorders, 6 1 0-6 1 4 alcohol use disorder, 6 1 1 1, 6 1 3 , 6 1 3(, 6 1 3t, 6 1 41

signs and symptoms, 6 1 1 , 6 1 l t-6 1 21 withdrawal, 6 1 0, 6 1 3 , 6 1 3(, 6 1 41 suicidality, 622-62 3 trauma and stressor-related disorders, 598-599 "Psychogenic" tremor, 402t Psychogenic/nonepileptic spells, 620 Psychosis, 590, 5 9 1 , 591 I postpartum, 603t Psychotic disorders, 590-593 antipsychotic medications, 5 9 1 , 5921, 5931 diagnostic criteria by symptom duration, 607t differential diagnosis, 5 9 1 t schizophrenia, 590, 59 1-592, 5 9 l t-593t schizophreniform disorder, 59 1-592, 5 9 1 1 P T (prothrombin time), 270 PTH (parathyroid hormone) calcium and phosphate regulation, 1 3 8( excess, 1 39- 1 4 1 , 1 401 PTHrP (parathyroid hormone-related protein), ectopic, 1 401 PTSD (post-traumatic stress disorder), 598-599 PTT (partial thromboplastin time), 270, 2 7 5(, 279 Pubarche, 5 1 5( Puberty delayed, 470-47 1 , 4721, 5 1 4 pathologic delay, 5 1 4 precocious, 469-4 70, 469t, 5 14 Pubic lice, 1 1 0- 1 1 1 PUD (peptic ulcer disease), 2 1 5, 2 1 51, 2 1 6-2 1 7, 2 1 6( Pudenda! block, 4 5 5 Puerperium, 462-464 peripartum cardiomyopathy, 464 postpartum hemorrhage, 462, 463/ postpartum infection, 462-464 Sheehan syndrome (postpartum pituitary necrosis), 464 Pulmonary arterial hypertension (PAH), 643 Pulmonary changes, pregnancy, 4291 Pulmonary contusion, 638, 638(, 760 Pulmonary disease, 625-674 acute respiratory failure, 63 7-642 acute respiratory distress syndrome, 638-639, 638( coronavirus and COVlD- 1 9, 64 1-642, 642( hypoxemia, 637-638, 637(, 638/ mechanical ventilation, 639-64 1 , 640t642t hemoptysis, 663-664, 664( neoplasms, 646-6 50 lung cancer, 647-6 50, 6481, 649(, 6501 solitary pulmonary nodule, 646-647, 647(, 6471 nose and throat disorders, 669-674 adenotonsillar atrophy, 672 benign and malignant laryngeal lesions, 673-674 epistaxis, 67 1 , 67 lf laryngitis, 672-67 3 laryngopharyngeal reflux, 673 nasal polyps, 670 rhinitis, 669-670 obstructive lung disease, 626-63 1

INDEX asthma, 626-628, 6281, 6291 bronchiectasis, 628-630, 629{ chronic, 630---03 1 , 6301-6321, 6 3 1 { lung volumes, 626, 626{ restrictive vs., 626, 6261, 627{ pleural disease, 664-667 pleural effusion, 664-666, 6651-6671 pneumothorax, 6651, 666---067, 667{ respiratory tract infections, 650-663 acute pharyngitis, 66 1-662, 66 1(, 66 1 1 anthrax, 660, 660{ aspergillosis, 654, 654{ blastomycosis, 6 5 5 1, 656-6 57 coccidioidomycosis, 65 516 5 6 histoplasmosis, 654-6 56, 6 5 5 , 6 5 5(, 6 5 51 influenza, 652-6 54 mycobacterial, 657-6 59, 657(-6 59{ Nocardia, 656 opportunistic, 6 5 5 I oral, 662 Pneumocyslis iirovecii pneumonia, 659-660, 659{ pneumonia, 650-652, 65 1(, 65 1 1-6 5 3 1, 652{ sinusitis, 662-663, 663{ restrictive, 63 1---036 allergic bronchopulmonary aspergillosis, 636 cryptogenic organizing pneumonia, 6 3 3 eosinophilic pulmonary syndromes, 63 5-636 hypersensitivity pneumonitis, 6 34 interstitial (diffuse parenchymal), 632-63 3, 6 3 3{ obstructive vs., 626, 6261, 6271 pneumoconiosis, 634-6 3 5 , 6 3 5{ systemic sarcoidosis, 63 3-634, 634{ sleep disorders, 667-669 obesity h ypoventilation syndrome, 668-669 obstructive sleep apnea, 667-668 vascular, 643-646 pulmonary h ypertension/cor pulmonale, 643 pulmonary thromboembolism, 644-64 5, 644(, 6441, 64 5I, 646{ Pulmonary edema, 2 3 high-altitude, 740 Pulmonary embolism (PE), 644-645 angina pectoris, 48 diagnosis, 644-645, 644(, 64 51 history/physical examination, 279, 644, 6441 treatment, 645, 646{ Pulmonary fibrosis, idiopathic, 632-6 3 3 , 6 3 3{ Pulmonary function tests (PFTs), 626, 626(, 6261, 627{ interstitial lung disease, 63 3 Pulmonary h yp ertension (PH), 643 Pulmonary nodule, solitary, 646-647, 647(, 6471 Pulmonary thromboembolism, 644-645, 644(, 6441, 6451, 646{ Pulse(s), peripheral, 2 3 Pulseless electrical activity, 3 31, 3 4 Pulseless ventricular tachycardia, 3 31 Pulsus alternans, 24

Pulsus bisferiens, 24 Pulsus paradoxus, 24 Pulsus parvus et tardus, 24 Pupil(s) Argyll Robertson, 7 1 3 "blown," 3 5 3 Marcus-Gunn, 422 pinpoint, 6 1 0 Pupillary defect, relative afferent, 422 Purified protein derivative (PPD) test, tubercu­ losis, 657, 657{ Purpura Henoch-Schonlein, 3 3 7{ idiopathic thrombocytopenic, 284-285 senile, 1 1 7 thrombotic thrombocytopenic, 2 8 1 -282, 282(, 2831 Pustular melanosis, neonatal, 5281 Pustule, 881 Pustulosis, neonatal cephalic, 5281 PVC (premature ventricular contraction), 3 1 1 PVS (persistent vegetative state), 372, 3721 Pyelonephritis, 706, 708-7 1 0, 709{ emphysematous, 709, 709{ pregnancy, 444 Pyloric stenosis, 5 34-5 3 5 , 5 34{ Pyoderma gangrenosum, 98, 98{ Pyogenic granuloma, 1 22, 1 22{ Pyridoxine deficiency, 7 501

Q Q waves, 2 1 , 2 If QRS axis, 1 8, 1 9(, 1 91 QRS interval, 20 QT interval, 20 Quadrantanopia, 4 1 2{ Quadruple screening, pregnancy, 432, 4321 Quality, 1 82- 1 8 5 analyzing medical errors, 1 8 5 errors, 1 84, 1 841 health worker burnout and fatigue, 1 84- 1 8 5 measuring quality outcomes, 1 83-1 84 PDSA cycle, 1 83, 1 8 3{ safety culture, 1 82-1 8 3 Swiss cheese model, 1 8 3, 1 8 3{ Quality outcomes, measuring, 1 83-1 84 Quaternary prevention, 1 70 Quetiapine, Parkinson disease, 399 Quickening, 428 Quinidine, adverse effects, 7491

R R waves, 2 1 RA (rheumatoid arthritis), 3281, 329-3 30, 3 3 6 juvenile, 547-548, 5471 RAAS (renin-angiotensin-aldosterone system), heart failure, 36, 36{ Rabies postexposure prophylaxis, 740, 74 lf Radial nerve injury, 3201, 322{ Radiation, pregnancy, 4 341 Radiation proctitis, 2401 Radioactive iodine uptake (RAJU) test, 1 3 2, 1 3 3(, 1 3 31, 1 341

Radionuclide bone scan, Paget disease of bone, 1 39, 1 40{ Ramsay Hunt syndrome, I O I Randomized controlled trial (RCT), 1 64, 1 6 5 1 Ranula, 2021 Rapport, 1 80, 1 801 Rash(es) benign neonatal, 527, 5281 heat, 5281 "Rat-bite" erosions, 347, 348{ Rate control, atrial fibrillation, 32 Rationalization, 6091 Raynaud phenomenon, 344 RBBB (right bundle-branch block), 20, 20{ RBCs. See Red blood cell(s) (RBCs) RBM8A gene, 566 RCA (right coronary artery), myocardial infarc­ tion involving, 52, 52{ RCT (randomized controlled trial), 1 64, 1 6 5 1 RDS (respiratory distress syndrome) acute, 638-6 39, 638{ neonatal, 524-526, 5 2 5 1 Reaction formation, 6091 Reactive arthritis, 3 3 1 Real-time quaking-induced conversion (RTQu!C), 395 Recall bias, 1 67, 1 67{ Recapitulation, interviewing, 1 80 Receiver operating characteristic (ROC) curves, 1 59, 1 59{ Receptive aphasia, 373, 373{ Recombinant influenza vaccine (RIV4), 1 73{ Recombinant zoster vaccine (RZV), 1 7 1(-1 73{ Recruihnent, clinical research, 1 9 5 Rectal injury, 764 Rectal prolapse, 2401 Rectus sheath hematoma, 763-764 Recurrent aphthous stomatitis, 1 991 Recurrent respiratory papillomatosis, 674 Red blood cell(s) (RBCs), packed, 2741 Red blood cell (RBC) disorders, 28 5-296 anemias, 285-294, 285(-288(, 2861, 2891, 290(, 292(, 293{ G6PD deficiency, 291 hereditary spherocytosis, 290(, 291-292 paroxysmal nocturnal hemoglobinuria, 291 polycythemias, 295 porphyria, 294, 2941 thalassemias, 288, 2891 transfusion reactions, 296, 2961 Red reflex, 580 Reentrant tachycardias, 2 5 , 2 5(, 291 Re-entry at atrioventricular node, 2 5 , 2 5(, 291 Reflection, interviewing, 1 79 Reflex(es), 3 5 8, 3 581 primitive, 5 I I, 5 1 31 Reflex syncope, 83 Reflux gastroesophageal, 209-2 10, 209(, 2 1 Of vesicoureteral, 540, 54 lf Refractive errors, 4 1 6 Refusal o f treatment, 1 89 Regression, 6091 linear, 1 69 logistic, 1 69

INDEX Relative afferent pupillary defect, 422 Relative risk (RR), 1 60, 1 6 1{ Renal amyloidosis, 6941 Renal calculi, 694-697, 6951-6961, 696{ Renal cell carcinoma, 705, 705{ Renal changes, pregnancy, 429t Renal disease, 67 5-698 acid-base disorders, 682-684, 683(. 6831 acute kidney injury, 684, 6851-6861 chronic, 684-687 and diuretics, 687, 687(. 6881 electrolyte disorders, 676-682 hypercalcemia, 680-68 1 , 68 lf hyperkalemia, 678-679, 679(. 680 hypernatremia, 676 h ypocalcemia, 681-682 h ypokalemia, 679, 679(. 680{ h ypomagnesemia, 682 h yponatremia, 676-678, 677(. 678{ end-stage, 684 anemia, 287 vaccines, 1 7 3{ glomerular, 688-694 nephritic syndrome, 688-69 1 , 688(. 689169 1 1, 692{ nephrotic syndrome, 692, 693t-694t nephrolithiasis, 694-697, 6951-6961, 696{ polycystic, 697-698, 697{ renal cell carcinoma, 705, 705{ renal tubular acidosis, 684, 6851 Renal failure, acute, 684, 6851-6861 Renal transplant patients, 686 Renal tubular acidosis (RTA), 683(. 684, 6851 Renin-angiotensin-aldosterone system (RAAS), heart failure, 36, 36{ Reportable diseases, 1 76, 1 761 confidentiality, 193 Reproductive endocrinology, 48 1-485 congenital adrenal hyperplasia, 48 1-483, 482(. 4831 polycystic ovarian syndrome, 483-484, 484{ Reproductive health, informed consent, 188 Reproductive screening, 1 741, 1 7 51 RERAs (respiratory effort-related arousals), 667 Research, 1 94-195 core principles, 194 ethical concerns, 1 94-195 Reserpine, adverse effects, 7491 Residual volume (RV), 626{ Resistive pressure, 6401 increased, 64 1 1 Respect, rapport, 1 801 Respiratory acidosis, 683(. 6831 Respiratory alkalosis, 683(. 6831 Respiratory distress syndrome (RDS) acute, 638-639, 638{ neonatal, 524-526, 5251 Respiratory effort-related arousals (RERAs), 667 Respiratory failure, acute, 637-642 acute respiratory distress syndrome, 638-639, 638{ coronavirus and COVID- 19, 64 1-642, 642{ hypoxemia, 637-638, 637(. 6381 mechanical ventilation, 639-64 1 , 640t-6421 Respiratory mechanics, 639, 6401

Respiratory syncytial virus (RSV), 549 Respiratory tract infections, 650-663 acute pharyngitis, 66 1-662, 661(. 66 1 1 anthrax, 660, 660{ aspergillosis, 6 54, 654{ blastomycosis, 6 5 5t, 656-657 coccidioidomycosis, 65 51656 histoplasmosis, 6 54-656, 655, 6 5 5(. 6 5 51 influenza, 652-6 54 mycobacterial, 657-659, 6 57(-6 59{ Nocardia, 656 opportunistic, 65 51 oral, 662 Pneumocyslis jirovecii pneumonia, 659-660, 659{ pneumonia, 6 50-652, 65 lf, 6 5 1 1-6 531, 652{ sinusitis, 662-663, 663{ Rest pain, 8 1 Resting tremor, 4021 Restless leg syndrome, 400-40 I Restrictive cardiomyopathy, 401, 4 3 Restrictive lung disease, 63 1-636 allergic bronchopulmonary aspergillosis, 636 cryptogenic organizing pneumonia, 63 3 eosinophilic pulmonary syndromes, 63 5-636 h yp ersensitivity pneumonitis, 634 interstitial (diffuse parenchymal), 632-6 3 3 , 6 3 3{ obstructive vs., 626, 6261, 6271 pneumoconiosis, 634-6 3 5 , 6 3 5{ systemic sarcoidosis, 63 3-634, 634{ Retained placental tissue, postpartum hemorrhage, 4631 Reticulocyte count, 28 5 Retinal detachment, 420, 42 1 , 42 1{ Retinal hemangioblastomas, Von Hippel-Lindau syndrome, 409 Retinal vascular occlusion, 4 1 9, 4201 Retinitis pigmentosa, 42 1-422, 422{ cytomegalovirus, 73 5 Retinoblastoma, 580 Retinopathy diabetic, 1 2 5(. 1 2 51, 42 1 , 42 1{ h yp ertensive, 58, 59(. 42 1 , 42 1{ Retropharyngeal abscess, 5 51 I, 662 Retrospective cohort study, 16 3, 1 64{ Rett syndrome, 562-563, 587 Reynolds pentad, 246 RF (rheumatoid factor), 3 30, 3 3 3t Rh immunoglobulin, 285 Rh isoimmunization, 45 1 , 45 lf Rhabdomyolysis, 341 Rhabdomyosarcoma, pediatric vaginal discharge, 49 1 Rhagades, 7 1 3{ RHO (rheumatic heart disease), valvular disease, 72, 721, 731 Rheumatic fever, 662 Rheumatic heart disease (RHO), valvular dis­ ease, 72, 72t, 73t Rheumatoid arthritis (RA), 328t, 329-3 30, 3 36 juvenile, 547-548, 5471 Rheumatoid factor ( RF), 3 30, 3 3 31 Rheumatoid nodules, 3 30

Rhinitis, 669-670 allergic, 669 nonallergic, 669-670 senile (atrophic), 670 Rhinophyma, 1 1 5, 1 1 5{ Rhizopus, sinusitis, 662 Rhythm control, atrial fibrillation, 32-3 3 Riboflavin deficiency, 7 501 Rickettsia rickettsii, 723-724, 724{ Rifampin, adverse effects, 7491 Right atrial abnormality, 2 1 Right bundle-branch block (RBBB), 20, 20{ Right coronary artery (RCA), myocardial infarction involving, 52, 52{ Right mainstem bronchus intubation, 752(. 753 Right ventricular dysplasia, arrhythmogenic, 42-43, 43{ Right ventricular (RV) heart failure, 34, 3 51 Right ventricular hypertrophy (RVH), 22 Right ventricular (RV) infarction after STEM!, 54 Right ventricular outflow tract (RVOT) obstruction, tetralogy of Fallo!, 53 3-534 Right-sided heart failure, 34, 3 5 1 Right-to-left shunts, cyanotic, 528-529, 5 3 2-534 Rigidity, Parkinson disease, 397 Ringed sideroblasts, 287, 287{ Ringworm, 1 09, 1 09{ Rinne test, 42 5, 42 5(. 426 Risk absolute, 1 60 attributable, 160 relative, 1 60, 16 If Risk assessment, Swiss cheese model, 1 83, 1 8 3{ Risk difference, 1 60 Risk ratio (RR), 1 60 Ristocetin cofactor assay, 276 Rituximab, 285 RIV4 (recombinant influenza vaccine), 1 7 3{ Rivaroxaban, 2721 RNA-based vaccine, 1 701 ROC (receiver operating characteristic) curves, 1 59, 1 59{ Rocky Mountain spotted fever, 383, 72 3-724, 724{ Rodent bites, 7421 ROM (rupture of membranes), 458-459 Romano Ward syndrome, 20 Rome IV diagnostic criteria, large bowel obstruction, 23 5 Root cause analysis, 1 8 5 Rooting reflex, 5 1 31 Rosacea, 1 14-1 1 5, 1 1 5{ "Rose-gardener disease," 1 1 0 Roseola infantum, 5 561 Rosiglitazone, diabetes, 1 281 Rotator cuff injuries, 340, 3401 Rotator cuff tear, 340, 3401 acute, 3 I 4t Rotavirus (RV) vaccine, 17 lf Roth spots, endocarditis, 69, 69{ Rotor syndrome, 5231 Roux-en-Y gastric bypass, 2 1 9 Rovsing sign, 2 3 0

INDEX RR (relative risk), 1 60, 1 6 1{ RR (risk ratio), 1 60 RSV (respiratory syncytial virus), 549 RTA (renal tubular acidosis), 684, 685t RT-Qu!C (real-time quaking-induced conversion), 395 Rubella, 5 56t congenital, 43 5t, 5291 Rubor, Raynaud phenomenon, 344 RULE acronym, oral lesions, 20 1 Rule of 9s, 739, 739{ Rupture of membranes (ROM), 4 5 8-459 RV (residual volume), 626{ RV (right ventricular) heart failure, 34, 3 5t RV (right ventricular) infarction after STEM!, 54 RV (rotavirus) vaccine, 1 7 1{ RVH (right ventricular hypertrophy), 22 RVOT (right ventricular outflow tract) obstruction, tetralogy of Fallot, 5 3 3- 5 34 RZV (recombinant zoster vaccine), 1 7 1(- 1 7 3{

s S waves, 2 1 S 1 gallop, 22 S 4 gallop, 22 SABs (spontaneous abortions), 436-438, 437t Saccular aneurysm, ruptured, 368 SAD PERSONS mnemonic, 622, 623 Saddle embolus, 644{ Sadism, sexual, 6 1 7t Safety, 1 82-1 8 5 analyzing medical errors, 1 8 5 errors, 1 84, I 84t health worker burnout and fatigue, 1 84- 1 8 5 measuring quality outcomes, 1 8 3-1 84 PDSA cycle, 1 83 , 1 8 3{ safety culture, 1 82-1 83 Swiss cheese model, 1 83 , 1 8 3{ Safety culture, 1 82-1 8 3 SAH (subarachnoid hemorrhage), 363, 368-369, 368(, 3 80t Salicylates adverse effects, 749t toxic ingestion/overdose, 745t Salivary gland disease, 20 1 , 202t, 203(, 203t Salmonella spp diarrhea, 222t, 22 3 osteomyelitis, 327t septic arthritis, 326t Salt wasting, 482 Salter-Harris pediatric fracture classification, 3 I 9-3 20, 320(, 573t Sarcoidosis cardiomyopathy due to, 45t systemic, 6 3 3-634, 634{ Sarcoma Ewing, 3 2 3 , 324, 3 2 5(, 5 7 1 , 5 721 Kaposi, 1 2 1 , 1 2 1(, 7291 Sarcoma botryoides, pediatric vaginal discharge, 49 1 Sarcoptes scabiei, 1 1 1 - 1 1 2, 1 1 If "Sausage digits," 93 S wyer syndrome, 472{ SBO. See Small bowel obstruction (SBO)

SBP (spontaneous bacterial peritonitis), 2 5 2 , 2 54-2 56, 2 5 5t, 2 5 6 Scabies, 1 1 1-1 1 2, 1 1 lf Scale, 88t Scaphoid fracture, 3 1 6t Scaphoid necrosis, 343, 344 SCC. See Squamous cell carcinoma (SCC) SCD (subacute combined degeneration), 4 1 It Scenarios, statistical testing, 1 68-1 69 SCFE (slipped capital femoral epiphysis), 3 I 9, 576-577, 5 77{ Schatzki rings, 207, 207{ Schistocytes, 28 1 , 282{ Schizoaffective disorder, 5 9 1 , 5 9 l t Schizoid personality disorder, 5 9 l t, 6081 Schizophrenia, 590, 5 9 1-592, 5 9 1 1-5931 Schizophreniform disorder, 59 1-592, 5 9 1 1 Schizotypal personality disorder, 5 9 1 t, 6081 Schwannoma, vestibular, 4051, 407 SCLC (small cell lung cancer), 6481, 649, 649{ Scleroderma, 3 34-3 3 5 Sclerosing adenosis, 493 Sclerosing cholangitis, primary, 2 5 9 Scoliosis, 5 7 7 Scorpion stings, 7421 Scotoma, central, 4 1 2{ Screening abdominal aortic aneurysm, 1 7 51 blood glucose, 1 741, 1 7 51 blood pressure, 1 741, 1 7 51 breast cancer, 49 5, 495{ cardiovascular, 1 741, 1 7 51 cholesterol, 1 741, 1 7 51 colorectal cancer, 1 741, 1 7 51, 2 36, 2 3 71 diabetes mellitus, l 74t, 1 7 51 fetal aneuploidy, 43 1 1, 4321 HIV, 729 lung cancer, 1 7 51 quadruple, 432, 4321 recommendations, 1 74, 1 741, 1 7 51 reproductive, 1 741, 1 7 51 sexually transmitted diseases, I 74t, 1 7 51 vision, 580 Scrotal pain and swelling, 698-700, 699{ SOB (sleep-disordered breathing), 672 Seasonal affective disorder, 603 Seatbelt sign, 762, 763{ Seborrheic dermatitis, 9 1 -92, 92{ HIV, 727 Seborrheic keratosis, 1 1 7-1 1 8, 1 1 8{ neoplasms, 7 5 1 I Secondary closure, 2 3 1 Secondary dysmenorrhea, 474-475, 475t Secondary intent, 2 3 1 Secondary prevention, 1 70 Secondary survey, trauma management, 7 5 3-754 Second-degree AV block, 26t-271 Secretory phase, uterine cycle, 468{ Seidel sign, 7 54 Seizure(s) absence (petit ma!), 374, 375, 375{ atonic, 3 7 5{ classification, 374, 3 7 5{ febrile, 5 5 8-5 59 focal (partial), 374, 3 7 5 , 3 7 5{

complex, 374, 375{ simple, 374, 375{ generalized, 374, 375{ intractable temporal lobe, 376 myoclonic, 3 7 5{ tonic, 3 7 5{ tonic-clonic (grand ma!), 374, 3 7 5 , 3 7 5{ unknown, 374 Seizure disorders, 3 7 3-376 children, 3731, 3 7 5 classification, 374, 3 7 5{ diagnosis, 3 7 5 etiologies b y age, 37 3-374, 3 741 history, 374 status epilepticus, 376 treatment, 37 5-376 Selection bias, 1 6 5 Selective estrogen receptor modulator (SERM), 496 Selective serotonin reuptake inhibitors ( SSR!s), 5951, 603, 6041 adverse effects, 7491 Selenium deficiency, 7 501 Seminoma, 706, 706/ Senile purpura, 1 1 7 Senile rhinitis, 670 Sensitivity, 1 58- 1 59, 1 58(, 1 59{ Sensorineural hearing loss, 42 5, 42 5{ Sensory aphasia, 373, 3 7 3{ Sensory homunculus, 3 5 3 , 3 54{ Sentinel event, 1 841 Separation anxiety disorder, 589-590 Sepsis, 720-72 1 Septa! hematoma, 3 5 5{ Septic abortion, 4371, 438 Septic arthritis, 3 2 5 , 32 51, 326(, 3261 Septic emboli, endocarditis, 69 Septic pelvic thrombophlebitis, 463-464 Septic shock, 7 1 81, 720 Sequential organ failure assessment (SOFA), 720 SERM (selective estrogen receptor modulator), 496 Seronegative spondyloarthropathy, 3 30-3 32, 3 3 1{ Serotonin norepinephrine reuptake inhibitors (SNRls), 5951, 6041 Serous otitis media, 42 3-424 Serratia marcescens, urinary tract infection, 7081 Sertoli cell tumor, 706/ Serum osmolality (sOsm), hyponatremia, 677 Serum sickness-like reaction, 3 36 Setting, challenging conversations, 1 8 1 Setting the stage, interviewing, 1 79 Setup, delivering news, 1 92 Severe combined immunodeficiency, 5441 Sex chromosome abnormalities, 5 1 71 Sexual abuse, 62 1 -622 children, 577, 578 Sexual assault, 622 Sexual changes, aging, 6 1 6 Sexual development, 5 14, 5 1 5{ Sexual disorders, 6 1 6-6 1 7 female, 507 gender dysphoria, 6 1 6-6 1 7 paraphilic, 6 1 6, 6 1 71

INDEX Sexual disorders (Continued) sexual changes with aging, 6 1 6 sexual dysfunction, 6 1 7 Sexual dysfunction, 6 1 7 Sexual masochism, 6 1 7t Sexual relationship, doctor-patient, 1 86 Sexual sadism, 6 l 7t Sexual transmission, HIV, 726t Sexuality-inclusive history taking, 1 8 1 Sexually transmitted diseases (STDs), 7 1 1-7 1 6 chlamydia, 7 1 1-7 1 2, 7 1 1(, 7 1 2( genital lesions, 7 1 5, 7 1 5t-7 1 6t gonorrhea, 7 1 2 , 7 1 2( informed consent, 1 88-1 89 pediatric vaginal discharge, 49 1 reportable, I 76t screening, l 74t, l 75t syphilis, 7 1 3-7 1 5, 7 1 3(, 7 1 4(, 7 14t Sezary syndrome, 305t SGLT (sodium-glucose transporter)2 inhibitors, diabetes, l 28t Sheehan syndrome, 1 4 1 , 464 Shellfish stings, 742t Shift work, 6 1 9 Shigella spp, diarrhea, 222t Shingles, 99t, 1 0 1-1 02, l 0 l f Shock, 7 1 8, 7 1 8t infants, 5 3 1 Shock liver, 2 5 6 Short bowel syndrome, malabsorption, 22 3 Short stature, familial, 5 1 3 Shoulder common adult orthopedic injuries, 3 l 4t "frozen," 3 39 physical examination maneuvers, 340, 340t Shoulder dislocation, 3 l 4t Shoulder dystocia, 46 1 , 46 1( Shoulder impingement, 340, 340t Shunts

acyanotic left-to-right, 528, 529-532 cyanotic right-to-left, 528-529, 5 32-5 34 Shy-Drager syndrome, 398 SIADH (syndrome of inappropriate antidiuretic hormone secretion), 147 Sialadenitis acute non-suppurative, 202t acute suppurative, 202t Sialadenosis, 202t Sialolithiasis, 202t SIBO (small intestinal bacterial overgrowth), malabsorption, 223 Sick sinus syndrome, 27t Sickle cell crisis, 568, 569 Sickle cell disease, 567-569, 568(, 569( Sickle cell trait (HbAS), 568 Adverse effects, drugs, 747t-749t Sideroblast(s), ringed, 287, 287( Sideroblastic anemia, 287, 287( SIDS (sudden infant death syndrome), 579 Silicosis, 6 3 5t Simple partial seizures, 3 74, 3 7 5( Sinus bradycardia, 26t Sinus rhythm, 1 8 Sinus tachycardia, 28t Sinusitis, 662-663, 663(

Sipple syndrome, 1 5 5 Sister Mary Joseph node, 2 1 6 Sitagliptin, diabetes, l 28t Situational syncope, 84t Sjogren syndrome, 329 SJS (Stevens-Johnson syndrome), 9 5-96, 95f Skin layers, 88, 1 0 3 , 1 0 3( macroscopic terms, 88, 88t Skin changes, pregnancy, 429t Skin disorders. See Dermatology Skin infections, 98-1 1 2 bacterial, 1 0 3-1 07, 1 03f-1 07f fungal, 1 08-1 1 0, 1 08f-l 1 0f parasitic, 1 1 0-1 12, l l lf viral, 98-103, 99t- 1 00t, 1 00(- 1 02( Skin picking disorder, 598t Skull fractures basilar, 7 5 8 linear, 758 SLE (systemic lupus erythematosus), 3 3 5-3 36, 3 3 5(, 3 3 7( Sleep apnea, 6 1 9 central, 6 1 9 obstructive, 6 1 9, 667-668 Sleep disorders, 6 1 7-6 1 9 circadian rhythm, 6 1 9 narcolepsy, 6 1 8-6 1 9 primary hypersomnia, 6 1 8 primary insomnia, 6 1 8 pulmonary, 667-669 obesity hypoventilation syndrome, 668-669 obstructive sleep apnea, 667-668 sleep apnea, 6 1 9 Sleep hygiene, 6 1 8 Sleep paralysis, 6 1 8 Sleep-disordered breathing (SOB), 672 Sleeve gastrectomy, 2 1 9 Sliding hiatal hernia, 2 1 lf, 2 1 1 Slipped capital femoral epiphysis (SCFE), 3 1 9, 576-577, 577( Small bowel disorders, 220-2 3 1 acute abdomen, 226-228, 226t, 228( acute appendicitis, 230-2 3 1 , 23 lf carbohydrate maldigestion, 224 carcinoid syndrome, 224 diarrhea, 220-223, 22 l t-222t duodenal hematoma, 228-229 ileus, 22 5-226 malabsorption/maldigestion, 22 3-224, 223( mesenteric ischemia, 229-230, 2 30t small bowel obstruction, 224-225, 2 2 5(, 234( Small bowel obstruction (SBO), 224-225, 2 2 5( acute abdomen, 227 adhesions, 227 large vs. , 2 34t, 2 3 5f Small cell lung cancer (SCLC), 648t, 649, 649( Small intestinal bacterial overgrowth (SIBO), malabsorption, 223 Small vessel vasculitis, 3 37f SMART goals, 182 Smith-Modified Sgarbossa Criteria, 20 Smudge cells, 3 0 1 , 302, 303 Snake bites, 742t

SNR!s (serotonin norepinephrine reuptake inhibitors), 595t, 604t SNS (sympathetic nervous system), heart failure, 36, 36f "Soap bubble" appearance, 324, 3 2 5( Social anxiety disorder, 596-597 Social communication disorder, 5 l 3t Social development, 5 l 2t Sodium restriction, congestive heart failure, 38 Sodium-glucose transporter (SGLT)2 inhibitors, diabetes, l 28t SOFA (sequential organ failure assessment), 720 Solar elastosis, 1 1 7 Solid organ transplant rejection, 309-3 10, 3 I Ot Solitary pulmonary nodule, 646-647, 647(, 647t Solvents, substance abuse, 61 l t Somatic symptom disorder, 620 Somatosensory association cortex, 3 5 3f Somatostatinoma, 266-267 sOsm (serum osmolality), hyponatremia, 677 Spasms, infantile, 408 Special populations, recommended vaccinations, 1 7 3 , 1 7 3( Specific learning disorder, 5 l 3t Specificity, 1 5 8-1 59, 1 58(, 1 59( Speech difficulties, communication, 1 82 Speech sound disorder, 5 l 3t Spermicide, 480t SPF (sun protection factor), 1 1 7 Spherocytes, 290(, 291-292 Spherocytosis, hereditary, 290(, 291-292 Sphincter of Oddi dysfunction, 244 Spider bites, 74 1 t Spigelian hernia, 24 3t SPIKES model challenging conversations, 1 8 1 delivering news, 1 92-193 Spinal cord, 3 5 8, 3 58t-360t, 361( Spinal cord lesions, 3 59t-360t Spinal dysraphism, 560-562 Spinal muscular atrophy, 3 59t infantile hypotonia, 5 59t Spinal stenosis, 349-3 50, 3 50t Spinal tracts, 3 5 8t, 361( Spinothalamic tract, 3 58t Spiral fractures, child abuse, 578t Spironolactone, low-dose, congestive heart failure, 39 Spleen, blunt abdominal trauma, 762t Splenic rupture, infectious mononucleosis, 72 5 Splenic sequestration, sickle cell disease, 568 Splinter hemorrhages, endocarditis, 69, 69f Splitting, 609t Spondylitis ankylosing, 3 30-3 32, 3 3 1( enteropathic, 3 3 1 Spondyloarthropathy, seronegative, 3 30-3 32, 3 3 1( Spondylolisthesis, 3 50 pediatric, 574 Spondylosis, 3 50 Spontaneous abortions (SABs), 436-438, 437t Spontaneous bacterial peritonitis (SBP), 2 52, 2 54-2 56, 2 5 5t, 2 5 6 Sporothrix schenckii, 1 1 0

INDEX Sporotrichosis, 1 1 0 Squamous cell carcinoma (SCC) cutaneous, 1 1 8-1 1 9, 1 1 9( esophageal, 2 1 1 HIV, 729t lung, 648t, 649( oral, 20 1 SSR!s (selective serotonin reuptake inhibitors), 595t, 60 3, 604t adverse effects, 749t SSSS (staphylococcal scalded-skin syndrome), 96, 1 04 Stab wound abdomen, 756 confidentiality, 1 94 Staghorn calculi, 694 Stanford classification, aortic dissection, 77, 77( Staphylococcal conjunctivitis, 4 l 5t Staphylococcal scalded-skin syndrome (SSSS), 96, 1 04 Staphylococcus aureus acute lymphadenitis, 662 cavernous sinus thrombosis, 369 endocarditis, 68, 68t, 69{, 7 1 t hordeolum, 4 1 2 osteomyelitis, 327t pneumonia, 652( septic arthritis, 326t toxic shock syndrome, 49 1-492 Staphylococcus epidennidis endocarditis, 68 osteomyelitis, 327t Staphylococcus saprophyticus, urinary tract infection, 708t Stasis dermatitis, 90{, 1 14, 1 1 4( Stalins congestive heart failure, 39 dyslipidemia, 56, 57t adverse effects, 748t Statistical testing, 1 67-1 69, 1 69( Status epilepticus, 376 febrile, 5 5 8 STDs. See Sexually transmitted diseases (STDs) ST-elevation myocardial infarction (STEM!), 2 1 , 2 1( Stem cell research, 1 9 5 STEM!. See ST-segment elevation myocardial infarction (STEM!) Sterilization, surgical, 479t Steroid-sparing therapies, 285 Stevens-Johnson syndrome (SJS), 95-96, 9 5( Stimulants, substance abuse, 6 1 1 t-6 1 2t Stings, 740, 74 1{, 74 l t-742t Stomach disorders, 2 1 4-220 bariatric surgery, 2 1 9-220 dyspepsia, 2 1 4 gastric bezoar, 2 1 8-2 1 9 gastric cancer, 2 1 6, 2 1 6( gastritis, 2 1 4-2 16, 2 1 5{, 2 1 5t gastroparesis, 2 1 7-2 1 8 Menetrier disease, 2 1 8 peptic ulcer disease, 2 1 5, 2 1 5t, 2 1 6-2 1 7, 2 1 6( Zollinger-Ellison syndrome, 2 1 7 Stomatitis, recurrent aphthous, l 99t Stool analysis, 220

Stool antigen test, 2 l 5t Stool osmotic gap, 22 1 t STOP-Bang survey, 668 Strabismus, 580 Strangulated hernia, acute abdomen, 227 Strategy challenging conversations, 1 8 1 delivering news, 1 9 3 Streptococcal conjunctivitis, 4 I 5t Streptococcal pharyngitis, 66 1-662, 661{, 66 l t Streptococcus, group B meningitis, 3 79t pregnancy, 4 3 1 Streptococcus, perianal, 582t Streptococcus bovis type 1, endocarditis, 68, 68t Streptococcus gallolyticus, endocarditis, 68, 68t, 7lt Streptococcus mitis, endocarditis, 68, 68t Streptococcus mutans, endocarditis, 68, 68t Streptococcus pneumoniae meningitis, 379t pneumonia, 652( Streptococcus pyogenes acute lymphadenitis, 662 Group A 13-hemolytic, acute pharyngitis, 66 1 -662, 66 1{, 66 l t Streptococcus sanguinis, endocarditis, 68, 68t Streptococcus viridans, endocarditis, 68, 68t Streptokinase, toxic ingestion/overdose, 745t Streptomycin, pregnancy, 4 34t Stress, post-traumatic, 598-599 Stress fracture calcaneal, 3 l 8t metatarsal, 3 l 8t tibial, 3 l 8t Stress incontinence, 700t Stress testing, angina pectoris, 48, 48( Stress ulcers, 2 1 5 Stressor-related disorders, 598-599 Stroke, 365-367 acute treatment, 366-367 cardioembolic, 366 diagnosis, 36 5-366, 366( etiologies, 365, 366 hemorrhagic, 365, 366 history/physical examination, 365, 365t ischemic, 365, 366-367, 366( preventive and long-term treatment, 367, 367( risk factors, 365 symptoms by vessel territory, 365, 365t thrombotic, 366 Stroke prevention, atrial fibrillation, 32 Stroke volume, pregnancy, 429t Structure, assessment, 18 3 Struvite stones, 69 5t ST-segment elevation myocardial infarction (STEM!), 5 1-54, 52{, 5 3{, 54t complications, 54, 54t diagnosis, 5 1- 5 3 , 52{, 5 3( differential diagnosis, 50{, 5 3 history/physical examination, 5 1 interventions, 5 3 localization, 52- 5 3 , 52{, 5 3( long-term management, 54 treatment, 53, 5 3(

Stupor, 3 7 1 Sturge-Weber syndrome, 408-409 Stye, 1 1 6, 4 1 2 , 4 1 2( Subacute combined degeneration (SCD), 4 1 l t Subarachnoid hemorrhage (SAH). 363, 368-369, 368{, 380t Subarachnoid space, 3 5 5( Subdural hematomas, 758, 7 5 8( Subdural hemorrhage, 369, 370t Subendocardial infarcts, 2 1 Subfalcine herniation, 3 5 3 Subgaleal hemorrhage, 527t Sublimation, 609t Submucosal fibrosis, oral, 1 99{, 200 Substance abuse treatment, informed consent, 1 88- 1 89 Substance use disorders, 6 1 0-6 14 alcohol use disorder, 6 1 l t, 6 1 3 , 6 1 3{, 6 1 3t, 6 1 4t signs and symptoms, 6 1 1 , 61 l t-6 1 2t withdrawal, 6 1 0, 6 1 3, 6 1 3{, 6 1 4t Substance-induced mood disorder, 602t Subunit vaccine, l 70t Succinylcholine, adverse effects, 749t Suckling reflex, 5 l 3t Sucrase-isomaltase deficiency, 224 Sudden infant death syndrome (SIDS), 5 79 Suicidal patients, 622-62 3 confidentiality, 1 9 3 minor, 602 Suicide, clinician-assisted, 1 90- 1 9 1 Sulfasalazine, 3 30 Sulfonylureas, diabetes, l 28t Sun protection, 1 1 7 Sun protection factor (SPF), 1 1 7 Sunblock, 1 1 7 Sunburn, 1 1 7 "Sunburst" pattern, 324 Sunshine Act, 1 9 5 Superficial spreading melanoma, l 20t Superinfection, bums, 739 Superior cerebellar artery, 3 5 5( Superior gluteal nerve injury, 3 2 l t Superior sulcus tumors, 648 Superior vena cava (SVC) syndrome, 648, 649( Support, rapport, l 80t Suppression, 609t Supracondylar humerus fracture, 573t Supratentorial mass, 3 54( Supraventricular tachyarrhythmias, 28t-30t Supraventricular tachycardia, 3 3t Surgical incisions, closure, 2 3 1 Surrogate decision making, 1 90 Survival curves, 1 6 1 , 1 6 1( SVC (superior vena cava) syndrome, 648, 649( Swan-neck deformity, 3 30 "Swimmer's ear," 424 Swiss cheese model, 1 83, 1 8 3( Sylvian fissure, 3 5 3( Sympathetic nervous system (SNS), heart failure, 36, 36( Synchronized cardioversion, 34t Syncope, 82-84, 83{, 84t-85t Syndesmophytes, vertical, 3 3 1

INDEX Syndrome of inappropriate antidiuretic hor­ mone secretion ( SIADH), 1 47 Syngeneic transplantation, 309 Synovial fluid analysis, 32 5t, 328t characteristics, 326{ Synthetic opioid abuse, 6 1 I t Syphilis, 7 1 3-7 1 5 , 7 1 3{, 7 1 4{, 7 1 4t-7 1 6t congenital, 4 36t, 7 1 3{ Syringomyelia, 360t Syrinx, 360t Systemic inflammatory response system, 7 1 8t Systemic lupus erythematosus (SLE), 3 3 5-3 36, 3 3 5{, 3 3 7{ Systemic sarcoidosis, 63 3-634, 634{ Systemic sclerosis, 3 34-3 3 5 Systolic heart failure, 34, 3 5(-37{, 36-39, 3 7t, 38t Systolic murmurs, 22, 22(-24{

T T1 (triiodothyronine) , 1 32 T; (thyroxine), 1 30, 1 3 2 Tabes dorsalis, 360t, 7 1 3 Tachyarrhythmias, 2 5-3 3 , 2 5{, 28t-32t, 30{ ventricular, 3 l t-32t Tachycardia atrial, 29t multifocal, 30t postural, 84t sinus, 28t supraventricular, 3 3 t ventricular, 3 I t pulseless, 3 3t Tachycardia-bradycardia syndrome, 27t Taenia so/ium, diarrhea, 222t Takayasu arteritis, 3 37-3 38 Takotsubo cardiomyopathy, 46t Talipes equinovarus, 5 7 5 Tamoxifen, 496 Tanner staging, 5 14, 5 1 5{ TAR (thrombocytopenia absent radius) syndrome, 566 Tarasoff decision, 1 9 3 Tardive dyskinesia, 593t Tay-Sachs disease, 5 I St TB (tuberculosis), 657-6 58, 657(-659{ HIV, 729t TBG (thyroxine-binding globulin), 1 30 pregnancy, 4 30t TB! (traumatic brain injury), pediatric, 7 5 8-7 59 TCAs (tricyclic antidepressants), 603 , 604t adverse effects, 749t toxic ingestion/overdose, 745t T-cell deficiencies, pediatric, 54 3t, 546 T-cell lymphoma, cutaneous, 1 2 1-1 22, 1 2 1{ T-cell neoplasms, 305t Td (tetanus, diphtheria, and acellular pertussis) vaccine, 1 7 1 (- 1 7 3{ Tdap (tetanus, diphtheria, and acellular pertussis) vaccine, 1 7 1 (- 1 7 3{ Telangiectasia, 4 1 0 Temporal arteritis, 3 3 6-337, 3 3 7{ Temporal lobe, 3 5 3{

Temporal lobe seizure, intractable, 376 Temporomandibular joint (TMJ) disorders, 3 3 3-3 34 TEN (toxic epidermal necrolysis), 94, 9 5-96, 9 5{ Tendinitis, 340 Tendinosis, 340 Tenosynovitis, De Quervain, 3 1 6t Tension pneumothorax, 666-667, 667{ advanced trauma life support, 7 5 2{, 7 5 3 ventilator induced, 640 Tension-type headache, 3 6 l t, 363 Tentorium cerebelli, 3 54{ Teratology, 434, 434t Teratoma, 706t Terminal complement deficiency, 545t Terminal illness, palliative care, 1 79 Tertiary intent, 2 3 I Tertiary prevention, 1 70 Testes, undescended, 541 Testicular cancer, 70 5-706, 706t Testicular lymphoma, 706t Testicular torsion, 699, 699{ Tetanus, diphtheria, and acellular pertussis (Tdap or Td) vaccine, 1 7 1(- 1 7 3{ Tetanus prophylaxis, 740, 74 lf Tetracyclines pregnancy, 4 34t adverse effects, 749t Tetrahydrocannabinol (THC), abuse, 6 1 2t Tetralogy of Fallot (TOF), 529t, 5 3 3-5 34, 5 3 3{ TFTs (thyroid function tests), 1 30, l 32t Thalassemia(s), 286t, 288, 288t, 289t a-Thalassemia(s), 288, 288{, 289t J3-Thalassemia(s), 288, 288{, 289t J3-Thalassemia major, 289t J3-Thalassemia minor, 289t Thalidomide, pregnancy, 434t THC (tetrahydrocannabinol) , abuse, 6 1 2t Theca lutein cyst, 498t Thelarche, 468, 5 1 5{ Thermal dysregulation, 736-7 37 hyperthermia, 737 hypothermia, 736-73 7 Thermal injury, advanced trauma life support, 752 Thiamine deficiency, 4 1 l t, 7 50t Thiazide diuretics, 38t, 688t hyp ertension, 60t Thiazolidinediones, diabetes, 1 28t Thigh, common adult orthopedic injuries, 3 1 6t-3 1 7t Third-degree AV block, 27t Thoracotomy, penetrating chest trauma, 756 Throat disorders, 669-674 adenotonsillar atrophy, 672 benign and malignant laryngeal lesions, 67 3-674 laryngitis, 672-673 laryngopharyngeal reflux, 673 Thrombocytopenia, 284t essential, 295 heparin-induced, 279 immune, 284-285 pseudo-, 284

Thrombocytopenia absent radius (TAR) syn­ drome, 566 Thromboembolism, pulmonary, 644-645, 644{, 644t, 645t, 646{ Thrombogenesis deficiencies, 276-277, 276{ Thrombolysis, pulmonary embolism, 645 Thrombolytics, 27 lf, 367 Thrombophilias. See Hyp ercoagulable states Thrombophlebitis, septic pelvic, 463-464 Thrombosis, cavernous sinus, 369-3 7 1 Thrombotic thrombocytopenic purpura (TTP), 2 8 1 -282, 282{, 283t Thrush, 1 08-1 09, 1 09{ HIV, 727, 727{, 729t "Thumbprint sign," 5 5 2 "Thumbprinting," 229 "Thunderclap" headache, 368 Thymic aplasia, 543t Thymoma, 384, 384{ Thyroid carcinoma, l 37t Thyroid disorders, 1 30-1 37 h yperthyroidism and thyrotoxicosis, 1 30-1 34, 1 32{, 1 32t-1 34t, I 3 3{ h ypothyroidism, 1 3 2t, 1 34-1 3 5 thyroid neoplasms, 1 36, 1 36{, l 37t thyroid physiology and, 1 30, 13 lf, l 32t thyroiditis, 13 5 Thyroid function tests (TFTs), 1 30, l 32t Thyroid hormone(s) general resistance, 1 34 synthesis, 1 30, 1 3 lf Thyroid neoplasms, 1 36, 1 36{, l 3 7t Thyroid nodule, 1 36, 1 36{ Thyroid physiology, 1 30, 1 3 1{, 1 3 2t Thyroid stimulating hormone (TSH) deficiency, 143t Thyroid storm, 1 34 Thyroidectomy, adverse reactions, l 34t Thyroiditis, 1 30, 1 34, 1 3 5 Hashimoto, 1 34 Thyroid-stimulating hormone (TSH), 1 30, 1 32 Thyrotoxicosis, 1 30-1 34, 1 32{, 1 32t-l 34t, 1 3 3{ fetal, 1 3 2 Thyroxine (T;), 1 30, 1 32 Thyroxine-binding globulin (TBG), 1 30 pregnancy, 4 30t TIA (transient ischemic attack), 364 Tibial nerve injury, 3 2 l t Tibial stress fracture, 3 I St Tic(s), Tourette syndrome, 589 Tick-borne infections, 722-72 5 babesiosis, 72 3 Lyme disease, 722-723, 723{ reportable, l 76t Rocky Mountain spotted fever, 72 3-724, 724{ Tick-borne paralysis, 388 Tidal volume pregnancy, 4 29t ventilator setting, 6 39 Tinea ca pitis, 1 1 0, 1 1 Of Tinea corporis, 1 09, 1 09{ Tinea cruris, 1 09, I ! Of Tinea manuum, 1 09 Tinea pedis, 1 09, I ! Of

INDEX Tinea unguium, 1 1 0 Tinea versicolor, I 08, 108( TIPS (transjugular intrahepatic portosystemic shunt), 2 1 3, 2 5 8 Tirofiban, 272t Tissue plasminogen activators (tPAs), 270, 272t, 366, 367 toxic ingestion/overdose, 745t TLC (total lung capacity), 626(, 626t TLOC (transient loss of consciousness), 82-84, 83(, 84t-8 5t TM} (temporomandibular joint) disorders, 3 3 3-3 34 TOF (tetralogy of Fallot), 529t, 5 3 3-5 34, 5 3 3( Toilet training, 5 1 1 Tonic seizures, 375( Tonic-clonic (grand ma!) seizures, 374, 375, 375( Tonsil(s), palatine, 672 Tonsillar hypertrophy, 672 Tophaceous gout, 347, 347( Torsades de pointes, 32t Torus fracture, 573t Torus palatinus, l 99t Total lung capacity (TLC), 626(, 626t Total volume (TV), 626( Tourette syndrome, 589 Toxic adenoma, 13 3t, 1 30 Toxic epidermal necrolysis (TEN), 94, 95-96, 95( Toxic multinodular goiter, 1 30 Toxic shock syndrome (TSS), 49 1-492, 67 1 Toxic shock syndrome toxin I (TSST- 1 ) , 49 1 Toxicology, 742-749 antidotes and management, 744t-745t carbon monoxide poisoning, 743 drug interactions and reactions, 746, 746t drug adverse effects, 747, 747t-749t methemoglobinemia, 743-744 resuscitation of poisoned patient, 742-743 Toxoid vaccine, I 70t Toxoplasma gondii, HIV, 730t Toxoplasmosis, 3 8 1-382, 382( congenital, 4 3 5t tPAs (tissue plasminogen activators), 270, 272t, 366, 367 toxic ingestion/overdose, 745t TP-EIA (Treponema pallidum enzyme immuno­ assay), 7 1 4t TP-PA (Treponema pallidum particle agglutination), 7 1 4t TR (tricuspid regurgitation), 2 3(, 74t Tracheitis, 5 50t Tracheobronchial disruption, 760, 760( Tracheoesophageal fistula, 52 l t Tracheostomy, emergency, 752 Tranexamic acid, 277 Transcortical mixed aphasia, 3 7 3( Transcortical motor aphasia, 373( Transcortical sensory aphasia, 373( Transdermal patch, 479t Transfusion(s), refusal of, 1 8 1 Transfusion products, 274, 274t Transfusion reactions, 296, 296t Transient ischemic attack (TIA), 364

Transient loss of consciousness (TLOC), 82-84, 83(, 84t-85t Transient tachypnea of the newborn, 5 2 5t Transitional cell carcinoma, bladder, 704-705, 704( Transjugular intrahepatic portosystemic shunt (TIPS), 2 1 3, 2 5 8 Transplant medicine, 309-3 10, 3 1 0t Transplant rejection, 309-3 10, 3 1 0t Transposition of the great vessels, 5 3 2-53 3, 5 3 3( Transtentorial herniation, 3 5 3 Transudate, 664, 665(, 665t, 666 Transvaginal ultrasonography ovarian cancer, 504 polycystic ovarian syndrome, 483-484, 484( Transverse myelitis, 3 59t Transverse vaginal septum, 472( Transvestic disorder, 6 1 7t TRAPSS mnemonic, 397, 398 Trastuzumab, 496 Trauma blunt and deceleration, 7 5 7-764 abdomen, 762-763, 762t, 763( cardiac injury, 761-762, 762( chest, 760-76 1 , 760(, 761( head and face, 757-7 59, 7 5 8( pelvis, 763-764 management, 7 5 1-754 primary survey, 752-7 5 3 , 752(, 7 52t, 7 5 3( secondary survey, 75 3-754 penetrating, 7 54-7 57 abdomen, 756 chest, 756 extremities, 7 5 7 head, 7 54-7 5 5 neck, 7 5 5-7 56 Trauma-related disorders, 598-599 Traumatic brain injury (TB!), pediatric, 758-759 Trazodone, adverse effects, 749t Tremor(s), 40 1 , 40 1(, 402t, 4 1 0 Treponema pallidum, 7 1 3-7 1 5, 7 1 3(, 7 1 4(, 7 1 4t-7 1 6t Treponema pallidum enzyme immunoassay (TP-EIA), 7 1 4t Treponema pallidum particle agglutination (TPPA), 7 1 4t Triceps reflex, 3 58t Trichinella spiralis, diarrhea, 222t Trichobezoars, 2 1 8 Trichomonas, cervicitis, 489 Trichomoniasis, 488t, 489( Trichotillomania, 598t Tricuspid regurgitation (TR), 2 3(, 74t Tricyclic antidepressants (TCAs), 603, 604t adverse effects, 749t toxic ingestion/overdose, 745t Trigeminal neuralgia, 364 Triglycerides, 5 5-57, 56t, 57t Triiodothyronine (T1 ), 1 3 2 Trimethadione, pregnancy, 434t Trimethoprim, adverse effects, 749t Triple phosphate stones, 695t Trisomy(ies), 5 14, 5 1 6t

Trisomy 1 3 , 5 1 6t Trisomy 1 8, 432t, 5 1 6t Trisomy 2 1 , 432t, 5 1 6t Troponin angina pectoris, 47 STEM!, 5 1-52 unstable angina/NSTEMI, 50 Truncus arteriosus, 5 29t, 5 3 2 Trunk, musculoskeletal disorders, 348-350 herniated disk, 349, 349( low back pain, 348-3 50, 349(, 3 50t spinal stenosis, 349-3 50, 3 50t spondylolisthesis and spondylosis, 3 50 TSC (tuberous sclerosis complex) gene, 407 TSH (thyroid-stimulating hormone), 1 30, 1 3 2 TSH (thyroid stimulating hormone) deficiency, 143t TSS (toxic shock syndrome), 49 1-492, 67 1 TSST-1 (toxic shock syndrome toxin 1 ) , 49 1 TST (tuberculin skin test), 657, 657( t-test, 1 69, 1 69( TTP (thrombotic thrombocytopenic purpura), 281-282, 282(, 283t Tubal factors, infertility, 485t Tubal ligation, 479t Tuberculin skin test (TST), 657, 657( Tuberculosis (TB), 657-658, 657(-6 59( HIV, 729t Tuberous sclerosis, 407-408, 407( neoplasms, 7 5 1 t Tuberous sclerosis complex (TSC) gene, 407 Tufted angioma, 567 Tumor lysis syndrome, 306, 570 Tumor markers breast cancer, 495 ovarian cancer, 504, 505t Turcot syndrome, 238 Turner syndrome, 47 1 , 472(, 5 1 7t, 529t TV (total volume), 626( T-waves, 2 1 , 2 1( Twins, 448 Type I (ex) error, 1 68 Type II ([3) error, 1 68 Typical antipsychotics, 59 I , 592t, 593t

u

U I RNP antibody, 3 3 3t UA (unstable angina), 47-49, 48(, 50( UAG (urine anion gap), 682, 683-684 "Ugly duckling sign," 120 Ulcer(s), 88t anterior duodenal, 2 1 7 Curling, 2 I 5 Cushing, 2 1 5 decubitus, 1 1 2-1 1 3, 1 1 2( dendritic, 4 1 5 , 4 1 5( genital, 489 NSAID-induced, 2 1 7 peptic, 2 1 5, 2 1 5t, 2 1 6-2 1 7, 2 1 6( stress, 2 1 5 Ulcerative colitis, 24 1 , 24 1(, 242(, 242t neoplasms, 7 5 1 t Ulipristal, 48 1 t Ulnar nerve injury, 3 2 1 t, 3 2 2 , 322(, 3 2 3(

INDEX Ultrasonography pregnancy, 4 28 Ira nsvagi na I ovarian cancer, 504 polycystic ovarian syndrome, 483-484, 484( Ultraviolet (UV) radiation, 1 1 7 Umbilical artery Doppler velocimetry, 4 5 3 Umbilical cord prolapse, 46 1 Umbilical hernia, 243, 243t UMN (upper motor neuron) lesion, 3 57, 3 5 7t Uncal transtentorial herniation, 3 5 3 , 384 Unconsciousness, 37 1-372, 372t Uncus, 3 54( Unfractionated heparin, 272t Unhappy triad, knee injury, 3 1 0, 3 1 9( Unresponsiveness, 37 1-372, 372t Unstable angina (UA), 47-49, 48(, 50( Unsynchronized cardioversion, 34t Upper extremity common adult orthopedic injuries, 3 l 4t3 l 6t musculoskeletal disorders, 3 39-345 adhesive capsulitis, 3 39 avascular necrosis, 343-344, 343( carpal tunnel syndrome, 342, 342( compartment syndrome, 340-34 1 Dupuytren contracture, 34 3 ganglion cyst, 342-343, 343( hand infections and bite wounds, 344-345 pronator syndrome, 342 Raynaud phenomenon, 344 rhabdomyolysis, 34 1 rotator cuff injuries, 340, 3401 Upper gastrointestinal bleeding, 2 1 2(, 2 1 2-2 1 3 , 2 1 21 Upper motor neuron (UMN) lesion, 3 57, 3 5 71 Urea breath test, 2 1 5t Ureteric obstruction, acute abdomen, 227 Urethral injury, 764 Urethritis, nongonococcal, 7 1 1 Urge incontinence, 700t Uric acid stones, 695t Urinary catheter, advanced trauma life support, 7 54 Urinary incontinence, 700, 700t Urinary retention, postpartum, 462 Urinary tract infections (UTls), 706-7 1 1 , 7071, 7081 complicated, 7071, 708-7 1 1 cystitis, 706, 708, 7 1 0 microbiology, 706-708, 708t mimics, 7071 pregnancy, 444, 707t prophylaxis, 7071 prostatitis, 7 1 0-7 1 1 pyelonephritis, 706, 708-7 1 0, 709( types, 706, 7071 uncomplicated, 707t upper, 708-7 1 0, 709( vesicoureteral reflux, 540 Urine anion gap (UAG), 682, 683-684 Urine osmolality, hypernatremia, 676 Urologic cancer, 70 3-706 bladder, 704-705, 704( prostate, 7021, 703-704, 703(

renal cell carcinoma, 705, 705( testicular, 70 5-706, 7061 Urothelial carcinoma, 704-705, 704( Urticaria, 93-94, 94( Uterine atony, postpartum hemorrhage, 463t Uterine bleeding, abnormal, 476-478, 477(, 478t Uterine cycle, normal, 468-469, 468( Uterine factors, infertility, 48 51 Uterine inversion, 462 Uterine leiomyomas, 497-498 Uterine prolapse, 506, 506( Uterine rupture, 462 UTis. See Urinary tract infections ( UTis) UV ( ultraviolet) radiation, 1 1 7 Uveitis, 4 1 6, 4 1 6(

V Vaccination, I 70- 1 74 bacillus Calmette-Guerin (BCG), 657 childhood, 580 COYID- 1 9, 1 73- 1 74 recommended schedules, 1 70-1 73, l 7 lf-l 73f types of vaccines, 1 70, 1 701 Vaccine-preventable diseases, reportable, l 76t YACTERL-H association, 529, 565 Vaginal bleeding, postmenopausal, 476 Vaginal cancer, 503 Vaginal discharge, pediatric, 49 1 Vaginal injury, 764 Vaginal ring, 4791 Vaginal septum, transverse, 4 72( Yaginismus, 507 Yaginitis, 487-489, 4881, 489( Yaginosis, bacterial, 4881, 489, 489( Validation, interviewing, 1 80 Validity, 1 6 3 Yalproic acid, 6061 pregnancy, 4341 adverse effects, 7491 Valvular heart disease, 72, 721-741 Yancomycin, adverse effects, 7491 Variability, fetal heart rate, 4 5 5 , 4561 Yaricella, 99t, 1 0 1 - 1 02, 1 0 1 (, 5 561 Yaricella (VAR) vaccine, l 7 lf-l 7 3f Yaricella zoster, 5 571 Yaricella-zoster virus (VZV), 991, 1 0 1 - 1 02, 1 0 1(, 5 56t, 5 571 Yarices, esophageal and gastric, 2 1 2(, 2 1 2-2 1 3 cirrhosis, 2 5 51 Yaricocele, 699 Vasa previa, 44 7 I, 448( Vascular dementia, 3901, 392 Vascular disorders, 7 5-82 aortic aneurysm, 7 5-76, 7 5(, 76( aortic dissection, 76-78, 77( cerebral, 364-37 1 cavernous sinus thrombosis, 369-37 1 intracerebral hemorrhage, 369, 369( stroke, 36 5-367, 3651, 366(, 367( subarachnoid hemorrhage, 368-369, 368(, 380t subdural and epidural hemorrhage, 369, 370t transient ischemic attack, 364

deep venous thrombosis, 78-80, 781, 79( lymphedema, 82 peripheral arterial disease, 8 1 -82 postthrombotic (postphlebitic) syndrome, 80 pulmonary, 643-646 pulmonary hypertension/car pulmonale, 643 pulmonary thromboembolism, 644-645, 644(, 6441, 645t, 646( Vascular phase of hemostasis, 270 Vasculitis large vessel, 3 3 7( medium vessel, 3 3 7( small vessel, 3 3 7( Vasectomy, 4791 Yasodilators congestive heart failure, 38 h ypertension, 62t Vasa-occlusive disease (YOO), sickle cell disease, 568, 569 Vasopressors, congestive heart failure, 38 Vasovagal syncope, 84, 84t VC (vital capacity), 626( VCUG (voiding cystourethrogram), 540, 54 lf Venereal Disease Research Laboratory (VDRL) test, 7 1 4, 7 1 41 Venous return, decreased, ventilator induced, 64 1 Venous thromboembolism (VTE) prophylaxis, congestive heart failure, 3 8 Ventilation/perfusion ( V/Q) scan, 644 Ventilator-induced lung injury, 640 Ventral hernias, 24 3 Ventricular contraction, premature, 3 1 I Ventricular fibrillation (VF), 3 1 t, 3 3t Ventricular septa! defect (VSD), 2 3(, 529-530, 5 301 Ventricular tachyarrhythmias, 3 1 1-321 Ventricular tachycardia (YT), 3 lt pulseless, 3 31 Verrucae, 1 02-1 0 3 , 1 02( Vertebral artery, 3 5 5f stroke, 3651 Vertebrobasilar insufficiency, 365t Vertical syndesmophytes, 3 3 1 Vertigo, 3 77-379 acute peripheral vestibulopathy (labyrinthitis, vestibular neuritis), 378 benign paroxysmal positional, 3 77 central vs. peripheral, 377 Meniere disease, 3 78-379 Vesicle, 881 Yesicoureteral reflux (VUR), 540, 54 lf Vestibular neuritis, 378 Vestibular schwannoma, 40 5t, 407 Vestibulopathy, acute peripheral, 378 VF (ventricular fibrillation), 3 l t, 3 3 1 VGCC (voltage-gated calcium channel) autoantibodies, 3 8 5-386 VHL (Von Hippel-Lindau) syndrome, 409 Vinblastine, adverse effects, 749t Yincristine, adverse effects, 7491 VIPoma, 266 Viral conjunctivitis, 4 1 4, 4 l 5t Viral exanthems, 5 5 5 , 5 5 6t-5 57t

INDEX Viral infections, skin, 98- 1 0 3 , 99t-1 00t, 1 00(1 02( Viral meningitis, 3 79, 380t Virchow node, 2 1 6 Virchow triad, 644, 644( Viridans streptococci, endocarditis, 68, 68t,

7lt

Virilization, 474 Vision screening, children, 580 Visual field defects, 4 1 2, 4 1 2{ Vital capacity (VC), 626( Vitamin A, pregnancy, 434t Vitamin A deficiency, 7 5 0t Vitamin B 1 deficiency, 4 1 l t, 7 50t Vitamin B 2 deficiency, 7 50t Vitamin B 3 deficiency, 7 50t Vitamin B 5 deficiency, 7 50t Vitamin B6 deficiency, 7 50t Vitamin B 7 deficiency, 7 50t Vitamin B9 deficiency, 7 50t Vitamin B 1 2 deficiency, 293-294, 293{, 360t, 4 1 l t, 7 50t neoplasms, 7 5 1 t type A gastritis, 2 1 4-2 1 5 Vitamin C deficiency, 7 50t Vitamin D, calcium and phosphate regulation,

1 38(

Vitamin D deficiency, 7 50t Vitamin deficiencies, 7 50, 7 50t Vitamin E deficiency, 7 50t Vitamin K, 270 Vitamin K deficiency, 284t, 7 50t Vitiligo, 1 1 5- 1 16, 1 1 5{ Vocal cord nodule, 674 Vocal cord polyp, 673 YOO (vaso-occlusive disease), sickle cell disease, 568, 569 Voiding cystourethrogram (YCUG), 540, 54 lf Volkmann contracture, 34 1 Voltage-gated calcium channel (YGCC) auto­ antibodies, 38 5-386 Yolutrauma, 640 Yolvulus acute abdomen, 227 malrotation with, 5 36, 5 3 6( Yon Hippel-Lindau (VHL) syndrome, 409 von Willebrand disease (vWD), 276-277, 276{, 284t von Willebrand factor (vWF), 270, 276 Voyeuristic disorder, 6 1 7t Y/Q (ventilation/perfusion) scan, 644

YSD (ventricular septal defect), 2 3{, 529-5 30, 5 30t VT (ventricular tachycardia), 3 l t pulseless, 3 3t VTE (venous thromboembolism) prophylaxis, congestive heart failure, 38 Yulvar cancer, 502-503 Yulvodynia, 507 Yulvovaginal candidiasis, 1 09, 488t, 489( Yulvovaginitis, pediatric infectious, 49 1 noninfectious, 49 I VUR (vesicoureteral reflux), 540, 54 lf vWD (von Willebrand disease), 276-277, 276{, 284t vWF (von Willebrand factor), 270, 276 VZV (varicella-zoster virus), 99t, 1 0 1 - 1 02, I O I{, 5 56t, 5 57t

w

WAGR syndrome, 5 7 1 Waldenstrdm macroglobulinemia, 308 Wallenberg syndrome, 365t Warfarin, 270, 272t, 273t pregnancy, 4 34t toxic ingestion/overdose, 745t Warm agglutinin, 292 Warts, 1 02-1 0 3 , 1 02( Wasp stings, 74 1 t Water deprivation test, diabetes insipidus, 1 44, 1 44{, 1 44t Water-borne disease, reportable, l 76t "Watershed area," 2 38, 2 39( Weber test, 42 5, 425{, 426 Wegener granulomatosis, 3 3 7{, 688, 690t Weight, child development, 5 1 1 , 5 14 Weight gain, pregnancy, 430, 430t Well child care, 579-582 anticipatory guidance, 579 hearing and vision screening, 579-580 lead poisoning, 580-582, 581{, 745t perianal dermatitis, 582, 5 82t pigmented lesions, 582, 583t vaccinations, 580 Wells' criteria, deep venous thrombosis, 78, 78t, 79( Wells score, modified, 644, 645t Wenckebach AV block, 26t Wermer syndrome, 1 5 5 Wernicke aphasia, 373, 373(

Wernicke area, 3 5 3{, 373( Wernicke encephalopathy, 4 1 1 t Wet gangrene, 1 1 3 Wheal, 88t Whipple disease, 223 White blood cell disorders, 296-307 eosinophilia, 299-300, 299t leukemias, 300-304, 300(-302{, 302t, 303t lymphomas, 304-307, 304t, 305t, 306( lymphopenia and eosinopenia, 298, 298t neutropenia, 296-298 Whooping cough, 5 54-5 5 5 Wickham striae, 1 14, 1 14{ Williams syndrome, congenital heart disease, 529t Wilms tumor, 5 7 1 Wilson disease, 2 50, 26 1 , 2 6 1 {, 40 1-402 Wiskott-Aldrich syndrome, 544t Withdrawal, 6 1 0, 6 1 3 , 6 1 3{, 6 1 4t Withdrawal method, 480t Wolff-Parkinson-White (WPW) syndrome, 29t Woods screw maneuver, 461 "Word salad," 373 Wrist, common adult orthopedic injuries, 3 1 5t-3 1 6t

X Xanthelasma, 1 1 6, 1 1 6{ Xanthochromia, 368 Xeroderma pigmentosum, neoplasms, 7 5 I t

y Yeast infections, 1 08-1 09, 1 09( Yolk sac tumor, 50 5t, 706t

z Zavanelli maneuver, 461 Zenker diverticulum, 209, 209( Zidovudine, adverse effects, 749t Zika virus, 722 congenital, 436t Zinc deficiency, 7 50t Zollinger-Ellison syndrome, 2 1 7 Zoonoses, reportable, l 76t Zoster, 99t, 1 0 1-1 02, I O I{, 5 57t Zoster recombinant vaccine, 1 7 1 (- 1 73(

852

ABOUT THE AUTHORS

About the Editors Tao Le, M D, M H S Tao developed a passion for medical ed ucation a s a medical student. He has ed ited more than 1 5 titles i n the First Aid series. I n add ition, he is Founder and Ch ief Ed ucation Officer of USMLE-Rx for exam preparation and ScholarRx for sustai nable, g lobal medical ed ucation. As a medical student, he was editor-in-chief of the University of Ca l ifornia, San Francisco (UCSF) Synapse, a u niversity newspaper with a weekly circulation of 9000. Tao earned his medical degree from UCSF in 1 996 and completed his residency training in internal medicine at Yale U niversity and fellowship training at Johns Hopkins Un iversity. Tao su bsequently went on to cofound Medsn, and served as its chief medical officer. He is cu rrently chief of adult allergy and immu nology at the U niversity of Louisvil le.

Mona Ascha, M D Mona has a passion for plastic surgery, medical ed ucation, data science, research reprod ucibil ity, and providing care for marginal ized popu lations. She is cu rrently com pleti ng a gender-affi rmation surgery fel lowship at Johns Hopkins U n iversity and is earning her Master's degree i n Data Science. Mona has worked on the First Aid team si nce 20 1 5, contributing to m u ltiple projects; she has found the work to be incredi bly fu lfi l l i ng and rewardi ng. In her spare time, Mona l i kes to read about statistics, code, and build her GitH ub repo with personal projects. She is an avid tennis player and marathoner, havi ng completed nine marathons to date. She also enjoys spend ing time i n coffee shops reading and people watchi ng, brushing u p on her French, top rope rock climbi ng, taking improv classes, eating donuts, and getting crazy manicures.

Marina Boushra, M D Marina was born i n Cairo, Egypt to a family that taught her a deep love of med icine and a passion for ed ucation. She completed her residency training i n Emergency Medicine at East Carolina U niversity in G reenville, North Carolina and her fel l owship training in Critical Care Medicine at the Cleveland Clinic in Cleveland, Ohio. She is now an emergency medicine physician and i ntensivist at the Cleveland Clinic, where she hopes to instil l in learners t h e same passion for medicine a n d ed ucation with which she was fortu nate to grow up. Her medical interests include ED-ICU transitions, obstetric and toxicologic emergencies, medical education, and microbiology. Marina has been involved with the First Aid and ScholarRx Teams since 201 4. Outside the hospital, she enjoys being overly competitive at board and card games, writi ng prose, and binging TV shows with her h usband and their alarmingly growing number of cats.

Caroline Coleman, M D Caroline i s a cu rrent PGY-3 i n the J . Wi l l i s H u rst I nte rnal Med icine Residency at Emory Un iversity i n Atl a nta. She g rew up i n Florida before moving to Georgia to p u rsue her u ndergra d uate degree i n economics from the U n iversity of Georgia and medical degree at Emory U n iversity School of Med ici ne. She is i nterested i n a career as an academic hospitalist where she can pursue her i nterests of clinical reasoning, medical education, and i l l ustrations and graphic design.

Vikas Bhushan, M D Vi kas is a writer, editor, entrepre n e u r, and reti red telerad iologist. In 1 990 he conceived and authored the original First Aid for the USMLE Step 1 . His entrepreneurial endeavors i nclude a student-focused medical publ isher (S2S), an e-learning company (medschool.com), and an ER teleradiology practice (24/7 Radiology). Trained on the Left Coast, Vi kas completed a bachelor's degree at the U niversity of California Berkeley; an MD with thesis at UCSF; and a d iag nostic radiology residency at UCLA. H i s eclectic i nterests include cryptoeconomics, i nformation design, and avoiding a day job. Always fi nding the long shortcut, Vi kas is a n adventurer, knowledge seeker, and occasional innovator. He enjoys i ntermediate status as a kiteboarder and father, and strives to raise his three children as g lobal citizens.

Abhishek Bhardwaj, M D Abhishek i s a pul monologist and i ntensivist i n Southern California. He was born and raised i n I ndia and earned his medical degree from Kastu rba Medical College i n Manipal, I ndia. He completed h i s I nternal Med ici n e tra i n i n g at Mercy Catholic Medical Center i n Philadelphia and his fellowship training i n Pul monary and Critical Care Med icine at the Cleveland Clinic i n Clevela nd, Oh io. In add ition, he a l so pursued a Resuscitation Fel lowship at the U niversity of Pen nsylvania and is cu rrently earn i n g a Master's degree in C l i n i c Resea rch. He is passionate a bout improvi ng critical care accessibil ity and outcomes, medical education, and h u manism i n med icine. As a foreign medical g raduate, he understands the unique challenges of i nternational medical students and enjoys mentoring the next generation of physicians. He is also an avid d rone videographer, podcast host, com petitive ta ble tennis player, and creative writer. He strives to never miss a sunset with his two sons and prioritizes time with his fami ly.

Daniel Griffin, DO Dan is an intensivist at Saint Fra ncis Med ica l Center i n Cape Girardeau, Missouri. He was born and ra ised i n Mississippi where he attended Mississippi Col lege for his undergraduate degree and Wi l l i a m Ca rey U n iversity Col l ege of Osteopathic Medicine fo r his medica l degree. He completed internal med icine residency at Mag nolia Regional Health Center in Corinth, MS and pul monary/critical care fellowship at U niversity of Missouri at Kansas City. Over the last six years, he has been involved in various projects with the First Aid team. In his spare time, Dan enjoys exercising and spending time with his fam ily.

Stephanie Jones, P h D Stephanie is a biological i l l ustrator in Tumwater, Washington and earned her PhD in genetics and molecular biology from Emory University. During her dissertation research, Stephanie pursued her passion for visual scientific com munication and created nu merous i l l u strations that have been featu red in scientific publ ications, medical ed ucation, science outreach, and professional branding. She strives to show students and professionals how fascinati ng and accessible science can be.

Kimberly Kal l ia n os, M D Origi n a l ly fro m Atla nta, Kimberly graduated from the U n ivers ity of N orth Ca ro l i na at Chapel H i l l i n 2006 a nd from H a rva rd Med ical School in 201 1 . She completed her radiology residency and fellowship at UCSF and is cu rrently an Assistant professo r of C l i n ical Radiology at UCSF i n the cardiac and pulmonary imaging section.