Pediatric Board Study Guide: A Last Minute Review [2nd ed. 2020] 978-3-030-21266-7, 978-3-030-21267-4

Table of contents :
Front Matter ....Pages i-xvi
General Pediatrics (Osama I. Naga)....Pages 1-34
Neonatology (Mamta Fuloria)....Pages 35-79
Adolescent Medicine (Jessica Addison)....Pages 81-101
Genetic Disorders (Golder N. Wilson, Osama I. Naga, Vijay S. Tonk)....Pages 103-142
Metabolic Disorders (Osama I. Naga)....Pages 143-166
Mental and Behavioral Health (Mohamed Hamdy Ataalla)....Pages 167-195
Emergency Medicine (Jo-Ann O. Nesiama, Jennifer McConnell, Kenneth Yen)....Pages 197-245
Critical Care (Manpreet K. Virk, M. Hossein Tcharmtchi)....Pages 247-266
Infectious Diseases (Matthew B. Laurens)....Pages 267-343
Hematology/Oncology (Arpan A. Sinha, Scott Moerdler, Ellen Fraint, Adit Tal, Nora E. Rahmani, Kerry Morrone)....Pages 345-390
Allergy and Immunology (Maria I. Garcia Lloret, Caroline Y. Kuo)....Pages 391-416
Endocrinology (Amr Morsi)....Pages 417-464
Orthopedics (Amr Abdelgawad, Marwa Abdou)....Pages 465-506
Sports Medicine (Daniel Murphy)....Pages 507-523
Rheumatology (Amanda G. Brown, William Blaine Lapin, Andrea A. Ramirez, Jennifer L. Rammel)....Pages 525-550
Neurology (Ivet Hartonian, Jong W. Yoo, Jason T. Lerner)....Pages 551-584
Ophthalmology (Kyle E. Miller, Shira L. Robbins)....Pages 585-609
Ear, Nose, and Throat (Kara D. Meister, Anna H. Messner)....Pages 611-642
Cardiology (Grace Kung, Allison Hill, Jennifer Su)....Pages 643-689
Pulmonology (Tanya Martínez-Fernández, Yadira Rivera-Sánchez, Preeti Sharma)....Pages 691-730
Nutrition (Susan S. Baker)....Pages 731-756
Gastroenterology (Robert D. Baker)....Pages 757-798
Nephrology (Beatrice Goilav)....Pages 799-824
Fluids and Electrolytes (Benjamin Steinman, Beatrice Goilav)....Pages 825-839
Urology (Catherine J. Chen, Micah A. Jacobs)....Pages 841-853
Dermatology (Megan Craddock, Jennifer Ruth)....Pages 855-886
Psychosocial Issues and Child Abuse (Sitratullah O. Kukoyi-Maiyegun)....Pages 887-901
Ethics (Marwa Abdou)....Pages 903-912
Research and Statistics (Sitratullah O. Kukoyi-Maiyegun)....Pages 913-920
Patient Safety and Quality Improvement (Osama I. Naga)....Pages 921-926
Pharmacology and Pain Management (Osama I. Naga)....Pages 927-945
Radiology Review (Wendy G. Kim, Michael George)....Pages 947-1014
Last-Minute Review (Osama I. Naga)....Pages 1015-1135
Back Matter ....Pages 1137-1180

Citation preview

Osama I. Naga Editor

Pediatric Board Study Guide A Last Minute Review Second Edition

123

Pediatric Board Study Guide

Osama I. Naga Editor

Pediatric Board Study Guide A Last Minute Review Second Edition

Editor Osama I. Naga, MD Department of Pediatrics Paul L. Foster School of Medicine Texas Tech University Health Sciences Center El Paso, TX USA

ISBN 978-3-030-21266-7    ISBN 978-3-030-21267-4 (eBook) https://doi.org/10.1007/978-3-030-21267-4 © Springer Nature Switzerland AG 2020 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

I owe my deepest gratitude to the contributors whose expertise helped bring this pediatric resource to life. To all the pediatricians who keep on learning for the sake of the children!

Preface

The Pediatric Board Study Guide: A Last Minute Review provides the core material needed to pass the General Pediatrics Certifying Examination by the American Board of Pediatrics (ABP) and to meet the requirements required for the ABP Maintenance of Certification. This book contains a total of 33 chapters; the first 31 chapters discuss all aspects of pediatric medicine and are all updated according to the most recent content specifications provided by the ABP. Chapter 32 reviews common pediatric radiology cases, while Chapter 33, the final chapter, consists of high-yield last minute review cases. The second edition is notably more comprehensive and detailed than the first. Improvements over the previous edition include more illustrations and added chapters. Each chapter has been either written or reviewed by an expert in that specific field from a top academic institution in the United States. New chapters include Sports Medicine, Nutrition, Fluids and Electrolytes, Ethics, Patient Safety and Quality Improvement, and Pharmacology. Finally, to make the chapters even more incisive, “Pearls and Pitfalls” have been added at the end of each chapter. The 80 clinical case scenarios in the Radiology Review (Chap. 32), with its distinct images and radiological findings, should not only improve exam performance but also help the general pediatrician to identify common radiological findings. The final chapter, Last Minute Review, has been expanded in this new edition. These high-­ yield cases are arranged in the same sequence as the book chapters and placed in a way that allows the reader to discriminate among diseases and conditions, helping the test taker to distinguish between similar presenting cases on the exam. The final chapter allows the reader to review in the shortest time possible more than 1700 critical facts for the pediatric board exam, making it the ideal resource for the week prior to the exam. This book is of particular interest to pediatricians, fellows, pediatric subspecialists preparing for the board examination or certification maintenance, pediatric residents preparing for the in-service exam, daily rounds, and real-life clinical encounters. About the ABP board-certifying exam • The board exam is offered once a year, usually in October. • The exam consists of four sections. • There is a total of 330–350 multiple-choice questions with normally five answer choices for each question. • There are currently four sections with each section lasting 105 minutes. There is a 15-­minute break between the first two sections. • After the second section, there is a 60-minute lunch break. • There is another 15-minute break between section 3 and section 4 • The exam is scored from 0 to 300 with 180 being a passing score. How to study for the ABP board-certifying exam • Read the text of the Pediatric Board Study Guide: A Last Minute Review thoroughly multiple times throughout your residency. • Chapters can be read sequentially or can be read in conjunction with a rotation.

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• For example, a resident doing a 1-month pediatric cardiology rotation could usefully read in Chap. 19, Cardiology. • After finishing a chapter, turn to Chap. 33 to review the Last Minute Review cases for that particular specialty. • Make sure to study the most recent self-assessment curricula of the Pediatric Review and Education Program (PREP): https://shop.aap.org/professional-education/self-assessments. • Simulate the test-taking experience by answering timed questions. • Read the critiques/explanations after each question, including the PREP Pearls at the end of each question. • Read the new articles in Pediatrics in Review and answer their CME questions. • Two to three months before the exam, read the Pediatric Board Study Guide one more time. • One week before the exam, read for one final time Chap. 33, the Last Minute Review. • Rest at least 24 hours before the exam. The day of the exam • Have a good breakfast. • Arrive early to the testing center. • Make sure to have all the documents with you (ID, exam ticket, etc.). • Make sure to dress appropriately; sometimes the testing room may be cold. • During the exam, make sure to answer all questions. Do not leave any question unanswered even if you do not know the answer. There is no penalty for guessing. • Pace yourself: once the time has expired in a section, you cannot go back.

El Paso, TX, USA 

Osama Naga

Contents

1 General Pediatrics�����������������������������������������������������������������������������������������������������    1 Osama I. Naga 2 Neonatology���������������������������������������������������������������������������������������������������������������   35 Mamta Fuloria 3 Adolescent Medicine�������������������������������������������������������������������������������������������������   81 Jessica Addison 4 Genetic Disorders�����������������������������������������������������������������������������������������������������  103 Golder N. Wilson, Osama I. Naga, and Vijay S. Tonk 5 Metabolic Disorders�������������������������������������������������������������������������������������������������  143 Osama I. Naga 6 Mental and Behavioral Health���������������������������������������������������������������������������������  167 Mohamed Hamdy Ataalla 7 Emergency Medicine �����������������������������������������������������������������������������������������������  197 Jo-Ann O. Nesiama, Jennifer McConnell, and Kenneth Yen 8 Critical Care �������������������������������������������������������������������������������������������������������������  247 Manpreet K. Virk and M. Hossein Tcharmtchi 9 Infectious Diseases ���������������������������������������������������������������������������������������������������  267 Matthew B. Laurens 10 Hematology/Oncology�����������������������������������������������������������������������������������������������  345 Arpan A. Sinha, Scott Moerdler, Ellen Fraint, Adit Tal, Nora E. Rahmani, and Kerry Morrone 11 Allergy and Immunology�����������������������������������������������������������������������������������������  391 Maria I. Garcia Lloret and Caroline Y. Kuo 12 Endocrinology�����������������������������������������������������������������������������������������������������������  417 Amr Morsi 13 Orthopedics���������������������������������������������������������������������������������������������������������������  465 Amr Abdelgawad and Marwa Abdou 14 Sports Medicine���������������������������������������������������������������������������������������������������������  507 Daniel Murphy 15 Rheumatology�����������������������������������������������������������������������������������������������������������  525 Amanda G. Brown, William Blaine Lapin, Andrea A. Ramirez, and Jennifer L. Rammel 16 Neurology�������������������������������������������������������������������������������������������������������������������  551 Ivet Hartonian, Jong W. Yoo, and Jason T. Lerner

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17 Ophthalmology���������������������������������������������������������������������������������������������������������  585 Kyle E. Miller and Shira L. Robbins 18 Ear, Nose, and Throat�����������������������������������������������������������������������������������������������  611 Kara D. Meister and Anna H. Messner 19 Cardiology�����������������������������������������������������������������������������������������������������������������  643 Grace Kung, Allison Hill, and Jennifer Su 20 Pulmonology �������������������������������������������������������������������������������������������������������������  691 Tanya Martínez-Fernández, Yadira Rivera-Sánchez, and Preeti Sharma 21 Nutrition���������������������������������������������������������������������������������������������������������������������  731 Susan S. Baker 22 Gastroenterology�������������������������������������������������������������������������������������������������������  757 Robert D. Baker 23 Nephrology�����������������������������������������������������������������������������������������������������������������  799 Beatrice Goilav 24 Fluids and Electrolytes���������������������������������������������������������������������������������������������  825 Benjamin Steinman and Beatrice Goilav 25 Urology�����������������������������������������������������������������������������������������������������������������������  841 Catherine J. Chen and Micah A. Jacobs 26 Dermatology �������������������������������������������������������������������������������������������������������������  855 Megan Craddock and Jennifer Ruth 27 Psychosocial Issues and Child Abuse ���������������������������������������������������������������������  887 Sitratullah O. Kukoyi-Maiyegun 28 Ethics�������������������������������������������������������������������������������������������������������������������������  903 Marwa Abdou 29 Research and Statistics���������������������������������������������������������������������������������������������  913 Sitratullah O. Kukoyi-Maiyegun 30 Patient Safety and Quality Improvement���������������������������������������������������������������  921 Osama I. Naga 31 Pharmacology and Pain Management �������������������������������������������������������������������  927 Osama I. Naga 32 Radiology Review�����������������������������������������������������������������������������������������������������  947 Wendy G. Kim and Michael George 33 Last-Minute Review������������������������������������������������������������������������������������������������� 1015 Osama I. Naga Index����������������������������������������������������������������������������������������������������������������������������������� 1137

Contents

Editor

Osama  I.  Naga, MD  Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA

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Contributors

Amr Abdelgawad, MD, MBA  Department of Orthopaedic Surgery and Rehabilitation, Texas Tech University Health Sciences Center, El Paso, TX, USA Marwa Abdou, MD  Department of Pediatrics, Texas Tech University Health Sciences Center, El Paso, TX, USA Jessica  Addison, MD, MS  Division of Adolescent/Young Adult Medicine, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA Mohamed Hamdy Ataalla, MD  Department of Child and Adolescent Psychiatry, Texas Tech University Health Sciences Center, El Paso, TX, USA Robert  D.  Baker, MD, PhD Department of Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, Amherst, NY, USA Susan S. Baker, MD, PhD  Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, State University of New York, Buffalo, NY, USA Amanda  G.  Brown, MD Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA Catherine J. Chen, MD  Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX, USA Megan  Craddock, MD Department of Dermatology and Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA Ellen  Fraint, MD Division of Pediatric Hematology, Oncology, Marrow and Blood Cell Transplantation, Department of Pediatrics, Children’s Hospital at Montefiore, Bronx, NY, USA Mamta  Fuloria, MD Division of Neonatology, Department of Pediatrics, The Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA Maria  I.  Garcia  Lloret, MD, MSc Division of Pediatric Allergy/Immunology and Rheumatology, Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA, USA Michael  George, MD Department of Radiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA Beatrice  Goilav, MD Pediatric Nephrology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA Ivet Hartonian, MD, MS  Pediatric Neurology, Department of Pediatrics, Adventist Health White Memorial, Los Angeles, CA, USA Allison Hill, MD  Division of Cardiology, Department of Pediatrics, Children’s Hospital of Los Angeles, USC Keck School of Medicine, Los Angeles, CA, USA

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Micah  A.  Jacobs, MD, MPH Department of Urology, University of Texas Southwestern Medical Center, Dallas, TX, USA Wendy G. Kim, MD  Department of Radiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA Sitratullah O. Kukoyi-Maiyegun, MD  Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Science Center, El Paso, TX, USA Grace Kung, MD  Division of Cardiology, Department of Pediatrics, Children’s Hospital of Los Angeles, USC Keck School of Medicine, Los Angeles, CA, USA Caroline  Y.  Kuo, MD Division of Pediatric Allergy/Immunology and Rheumatology, Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA, USA William  Blaine  Lapin, MD  Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA Matthew B. Laurens, MD, MPH  Division of Infectious Diseases and Tropical Pediatrics, Department of Pediatrics, Center for Vaccine Development and Global Health, University of Maryland School of Medicine, Baltimore, MD, USA Jason T. Lerner, MD  Pediatric Neurology, Department of Pediatrics, UCLA Mattel Children’s Hospital, Los Angeles, CA, USA Tanya Martínez-Fernández, MD  Division Respiratory Medicine, Department of Pediatrics, University of Texas Southwestern Medical Center/Children’s Medical Center Dallas, Dallas, TX, USA Jennifer  McConnell, MD Division of Pediatric Emergency Medicine, Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX, USA Kara  D.  Meister, MD Department of Otolaryngology-Head & Neck Surgery, Pediatrics Division, Lucile Packard Children’s Hospital, Stanford University, Stanford, CA, USA Anna H. Messner, MD, FACS, FAAP  Professor of Otolaryngology/Head & Neck Surgery, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA Kyle  E.  Miller, MC(FS) USN, MD  Department of Ophthalmology, Naval Medical Center Portsmouth, Portsmouth, VA, USA Scott  Moerdler, MD  Pediatric Hematology/Oncology Program, Department of Pediatrics, Rutgers Cancer Institute of New Jersey, Robert Wood Johnson Medical School, Rutgers University, New Brunswick, NJ, USA Kerry Morrone, MD  Division of Pediatric Hematology, Oncology, Marrow and Blood Cell Transplantation, Department of Pediatrics, Children’s Hospital at Montefiore, Bronx, NY, USA Amr  Morsi, MD  Division of Endocrinology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA Daniel  Murphy, MD, CAQ-Sports Medicine Department of Family and Community Medicine, Paul L. Foster School of Medicine, Texas Tech University Health Center, El Paso, TX, USA Osama  I.  Naga, MD  Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA

Contributors

Contributors

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Jo-Ann O. Nesiama, MD, MS  Division of Pediatric Emergency Medicine, Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX, USA Violeta Radenovich, MD, MPH  Children’s Eye Center of El Paso, Pediatric Ophthalmology, Texas Tech University Health Science Center, El Paso, TX, USA Nora  E.  Rahmani, MD  Division of Pediatric Hematology, Oncology, Marrow and Blood Cell Transplantation, Department of Pediatrics, Children’s Hospital at Montefiore, Bronx, NY, USA Andrea  A. Ramirez, MD Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA Jennifer  L.  Rammel, MD Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA Yadira  Rivera-Sánchez, MD Division Respiratory Medicine, Department of Pediatrics, University of Texas Southwestern Medical Center/Children’s Medical Center Dallas, Dallas, TX, USA Shira L. Robbins, MD, FAAO, FAAP  Department of Ophthalmology, Ratner Children’s Eye Center, University of California San Diego, La Jolla, CA, USA Jennifer  Ruth, MD Department of Dermatology and Pediatrics, Dell Children’s Medical Group, Austin, TX, USA Preeti Sharma, MD  Division Respiratory Medicine, Department of Pediatrics, University of Texas Southwestern Medical Center/Children’s Medical Center Dallas, Dallas, TX, USA Arpan A. Sinha, MBBS, MD  Jimmy Everest Center of Pediatric Hematology and Oncology, Department of Pediatrics, The University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA Benjamin  Steinman, DO Department of Pediatrics, The Children’s Hospital at SUNY Downstate, SUNY Downstate Medical Center, Brooklyn, NY, USA Jennifer Su, MD  Division of Cardiology, Department of Pediatrics, Children’s Hospital of Los Angeles, USC Keck School of Medicine, Los Angeles, CA, USA Adit  Tal, MD Division of Pediatric Hematology, Oncology, Marrow and Blood Cell Transplantation, Department of Pediatrics, Children’s Hospital at Montefiore, Bronx, NY, USA Hina J. Talib, MD  Division of Adolescent Medicine, Department of Pediatrics, Children’s Hospital at Montefiore, Bronx, NY, USA M.  Hossein  Tcharmtchi, MD  Department of Pediatrics, Section of Critical Care, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA Vijay S. Tonk, PhD  Department of Pediatrics, Texas Tech University Health Sciences Center, Lubbock, TX, USA Manpreet K. Virk, MD  Department of Pediatrics, Section of Critical Care, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX, USA Dawn  M.  Wahezi, MD, MS Pediatric Rheumatology, Children’s Hospital at Montefiore, Bronx, NY, USA Golder  N.  Wilson, MD, PhD Department of Pediatrics, Texas Tech University Health Sciences Center and KinderGenome Medical Genetics, Dallas, TX, USA

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Kenneth Yen, MD, MS  Division of Pediatric Emergency Medicine, Department of Pediatrics, UT Southwestern Medical Center, Dallas, TX, USA Jong W. Yoo, MD  Pediatric Neurology, Department of Pediatrics, UCLA Mattel Children’s Hospital, Los Angeles, CA, USA Mohamed A. Zebda, DO, MPH  Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, Galveston, TX, USA

Contributors

1

General Pediatrics Osama I. Naga

GROWTH

• 3–6  months: weight gain is approximately 15 g/day • 6–12  months: weight gain is approximately 10 g/day • Birth weight is expected to double by 5–6 months

Background • Growth is affected by maternal nutrition and uterine size • Genetic growth potential is inherited from parents and also depends on nutrition throughHeight [1] out childhood • The height of a newborn increases by 50% or • Growth is affected by growth hormone, thyby 25.4 cm (10 in.) in the 1st year roid hormone, insulin, and sex hormones, all • The height of a newborn doubles within of which have varying influence at different 3–4 years stages of growth • After 2 years the height increases by an aver• Deviation from normally expected patterns of age 5–6 cm/year growth often can be the first indication of an • There is a range of pubertal peak growth underlying disorder velocities of around 7–12 cm per year in boys • Carefully documented growth charts serve as and 6–10.5 cm per year in girls powerful tools for monitoring the overall health and well-being of patients Measurements [2] • Key to diagnosing abnormal growth is the • The length or supine height should be meaunderstanding of normal growth, which can sured in infants and toddlers  3 years • For children between 2 and 3 years of age, it is Weight best to measure both supine length and stand• Healthy term infants may lose up to 10% of ing height and compare the 2 measurements birth weight within the first 10 days after birth • Plot gestational age rather than chronological • Newborns quickly regain this weight by age for preterm infants 2 weeks of age • Specific growth charts are available for special • 0–3  months: weight gain is approximately populations, e.g., trisomy 21, Turner syndrome, 30 g/day Klinefelter syndrome, and achondroplasia O. I. Naga (*) Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 O. I. Naga (ed.), Pediatric Board Study Guide, https://doi.org/10.1007/978-3-030-21267-4_1

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O. I. Naga

Table 1.1  Difference between benign familial macrocephHead circumference • Head circumference will increase by 12.7 cm aly and hydrocephalus Benign familial (5 in.) in the 1st year macrocephaly Family history of macrocephaly Normal growth and development

Hydrocephalus History of prematurity, IVH, trauma, or CNS infection Spasticity, gait disturbance, cognitive deterioration, hypertonia Bulging AF, ocular globes deviate downward (sun-setting sign), headaches, vomiting, irritability Referral to neurosurgeon

Growth curve reading • Shifts across 2 or more percentile lines may indicate an abnormality in growth • Weight loss is one of the first signs of malabsorption and of cases of malnourishment or No signs of increased ICP neglect • Primary linear growth problems often have Reassurance some congenital, genetic, or endocrine CNS Central nervous system, IVH Intraventricular hemorabnormalities (see also Chap. 12 rhage, ICP Intracranial pressure, AF Anterior fontanelle Endocrinology) • Genetic channeling: downward percentile –– Full history, including prenatal, birth, past crossing of a large baby born to short parents medical, and family or upward percentile crossing of a small baby –– Head ultrasound is the study of choice if born to tall parents typically accomplished by anterior fontanelle is still open 9–12 months –– Skull radiography –– Brain magnetic resonance imaging (MRI) if the anterior fontanelle is closed

Macrocephaly

Definition • Head circumference 2 standard deviations above the mean

Hydrocephalus (see Table 1.1) • Referral to a pediatric neurosurgeon

Causes  icrocephaly (See Also Chap. 16 M • Benign familial macrocephaly with enlarge- Neurology) ment of subarachnoid space • Hydrocephalus Definition • Intracranial hemorrhage or mass • Head circumference 2 standard deviations • Genetic causes e.g., Soto syndrome, or below the mean ­“cerebral gigantism” Causes Benign familial macrocephaly (Table 1.1) • Congenital infections, e.g., TORCH (Toxo­ • Most common cause (50%) plasmosis, Others, Rubella, Cytomegalovirus • Autosomal dominant; usually seen in one or [CMV]), Herpes simplex), Zika virus both parents • Maternal deprivation (folate deficiency, mal• Document parental head size nutrition, hypothyroidism) • Reassure parents and child if child’s head size • Maternal hyperphenylalaninemia is congruent with familial sizes • Toxic or metabolic disorders • Periodic monitoring of the head size • Genetic conditions, e.g., trisomy 21, Cornelia • Periodic monitoring of physical growth and de Lange syndrome neurological development • Acquired or postnatal onset of microcephaly, • If the child’s head size is not congruent with e.g., hypoxic-ischemic encephalopathy familial sizes:

1  General Pediatrics

Plagiocephaly (Fig. 1.1) [3]

3

• If more than one suture is involved, it is commonly associated with genetic disorders • Asymmetric skull (head growth is limited in the plane perpendicular to the fused suture) • Bony ridging overlying the fused suture • Commonly associated with an increase in head size asymmetrically • Scaphocephaly (elongated head) is the most common type of craniosynostosis, due to early fusion of the sagittal suture; ridging of the sagittal suture is palpable

Background • Deformational flattening from lack of changes in head positions is the most common cause of asymmetric head shape Causes • Positional or supine sleeping is the most common cause of plagiocephaly • Causes of persistent head tilt, e.g., congenital muscular torticollis, ocular torticollis, Klippel-Feil syndrome (See also Chap. 13 Secondary craniosynostosis Orthopedics.) • Primary failure of brain growth leads to early • Craniosynostosis fusion of sutures and microcephaly Craniosynostosis (See Also Chap. 4 Genetic Deformational plagiocephaly (Fig. 1.2, Table 1.2) Disorders) • Anterior displacement of the occiput and the frontal region on the same side (parallelogram) Primary craniosynostosis • Ear position is more anterior on the side of • One or more sutures fuse prematurely while flattening in positional plagiocephaly the brain still increasing in size • Supine sleeping recommendations (“baby on • If one suture is involved, it is usually isolated back”) have increased the prevalence of posterior plagiocephaly

Diagnosis • Neonatal examination to exclude syndromes with cranial and brain anomalies • Careful examination alone can make the diagnosis • Referral to a pediatric neurosurgeon if craniosynostosis is clinically suspected • Plain skull radiography or CT scan can confirm the diagnosis of craniosynostosis if the diagnosis is not clear • Cranial CT scan with 3-dimensional reconstruction is not required to make the diagnosis of craniosynostosis in most cases Treatment • Deformational plagiocephaly –– Observation; usually resolves spontaneously –– The helmet may be beneficial in severe cases of deformational plagiocephaly Fig. 1.1 A 5-month-old-boy with deformational plagiocephaly, flattening on the left side, and ipsilateral frontal bossing

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a

b

Fig. 1.2  (a) Deformational plagiocephaly; (b) Unilambdoid synostosis

Table 1.2  Difference between deformational plagiocephaly and plagiocephaly due to unilambdoid synostosis Deformational plagiocephaly Parallelogram-shaped head Occipital flattening on one side Frontal bossing on the same side Anterior displacement of the ear on the same side Palpable suture, no palpable ridging

Plagiocephaly due to unilambdoid synostosis Trapezoid-shaped head Occipital flattening on one side Frontal bossing on the contralateral side Posterior displacement of the ear on the same side Absence of suture or palpable ridging of fused lambdoid suture

DEVELOPMENTAL MILESTONES [4, 5] The American Academy of Pediatrics (AAP) recommends that clinicians screen children for general development using standardized, validated tools at 9, 18, and 30 months and for autism at 18 and 24 months or at any point when a caregiver or the clinician has a concern

Tools for screening • Denver Developmental Screening Test • Ages & Stages Questionnaires (ASQs) • Modified Checklist for Autism in Toddlers –– Treatment of underlying causes, e.g., con(M-CHAT) genital muscular torticollis or other causes of head tilt –– Emphasis on floor (“tummy”) time, occu- Newborn pational/physical therapy • Craniosynostosis Gross motor –– Surgery, usually between 6 and 12 months • Lies in flexed position

1  General Pediatrics

• Turns head from side to side; head sags on ventral suspension

5

• Begins to push up when lying on tummy • Head lags when pulled to sitting position

Fine motor • Hands unfisted 50% of the time • Retains an object or finger if placed in the Visual hand • Able to fixate face on light in line of vision; • Brings hands to mouth, sucks on hand, and “doll’s-­eye” movement of eyes on turning the may hold hands together body Social/communication/problem solving • Responds to visual threats by blinking • Follows moving object 180° • Visual acuity is 20/400 • Able to fixate on the face and follow it briefly Reflex • Tries to look at parents • Moro, stepping, placing, and grasp reflexes are all active • Stares momentarily where object disappeared • Smiles on social contact; listen to voice and coos 1 Month • Turns toward sounds Social/communication • Visual preference for human face

Gross motor Language • Legs more extended • Coos and makes gurgling sounds • Head lifted momentarily to plane of body on • Begins to act bored (crying, fussy) ventral suspension • Chin up in the prone position • Turns head in the supine position 3 Months Fine motor • Hands fisted near the face • Sucks well

Gross motor • Lifts head and chest with arms in prone position • May roll to the side

Social/communication/problem-solving • Begins to smile Fine motor • Gazes at black-white objects • Brings hands together in the midline and to • Watches person; follows moving object the mouth • Body movements following the sound of • Inspects their own fingers others • Bats at objects or toys Language • Startles to voice or sound

2 Months

Social/communication/problem solving • Follows parents across the room • Expression of dislike for a taste or a loud sound • Regards hands and toys

Gross motor Language • Raises head slightly farther in prone position • Regards and vocalizes to parents when • Head sustained in plane of body on ventral talking suspension • Chuckles

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• Pushes down on legs when feet are on a hard surface

Gross motor • Holds head steady and no head lag when Fine motor • Brings hands to mouth pulled from lying down to sitting position • Uses hands and eyes together, such as seeing (Fig. 1.3) a toy and reaching for it • May be able to roll over from front to back • Shakes rattle • Pushes tummy, with elbows lifting the head and chest (Fig. 1.4) Social/communication/problem solving • Responds to affection • Begins to babble • Laughs out loud • Excited at sight of a bottle • Recognizes familiar people and things at a distance • Likes to play with people and might cry when playing stops Language • Vocalizes when alone Reflexes • Asymmetric tonic reflex is gone • Palmar grasp is gone

6 Months Fig. 1.3  Holds head steady and no head lag when pulled from lying down to sitting position

Gross motor • Begins to sit with minimal support • Rolls over from back to front and front to back • Supports weight on legs and might bounce Fine motor • Transfers objects from one hand to another • Brings objects or food to the mouth • Places hands on the bottle • Bangs and shakes toys • Rakes pellets • Removes cloth on face

Social/communication/problem-solving • Stranger anxiety • Responds to own name • Responds to sounds by making sounds showFig. 1.4  Developmental milestone at 4  months: Pushes ing joy and displeasure tummy, with elbows lifting the head and chest

1  General Pediatrics

Language • Monosyllabic babble • Looks at self in mirror and smiles

7 Months Gross motor • Sits steady without support (Fig. 1.5) • Bounces when held upright • Puts arms out to the side for balance Fine motor • Radial palmar grasp

7

• Attends to sounds and music • Prefers mother • Inhibits to “no” Language • More vowels and more variety of sounds

9 Months Gross motor • Can get into sitting position from lying down • Pulls to stand • Begins to crawl (Fig. 1.6) • Bears walk with all limbs straight

Social/communication/problem-solving Fine motor • Explores different aspects of toy and observes • Radial-digital grasps of a block toy block in each hand • Bangs 2 blocks together • Finds partially hidden toys or objects • Bites and chews cookie • Looks from object to parents and back when • Inspects and rings a bell wanting help • Pulls string to obtain a ring • Looks at familial objects or toys Social/communication/problem-solving • Separation anxiety • Recognizes familiar people • May be afraid of strangers

Fig. 1.5  Developmental milestone at 7 months: Sits steady without support

Fig. 1.6  Developmental milestone at 9  months: Begins to crawl

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• Uses sound to get attention • Plays peek-a-boo • Orients to name well Language • Says “mamama” and “bababa” nonspecific • Copies sounds and gestures of others

12 Months Gross motor • Walks with one hand held • Pulls up to stand, walks holding on to furniture (“cruising”) • May stand alone and make a few steps without holding (Fig. 1.7) Fine motor • Fine pincer grasps of pellet • Holds crayon and scribbles after demonstration • Attempts tower of 2 blocks • Finger feeds part of a meal • Takes off a hat • Puts out arm or leg to help with dressing • Rattles spoon in a cup • Puts a toy in a container, takes it out of the container Social/communication/problem-solving • Shows parents object to share interest • Follows one-step command with a gesture • Looks at the right picture or thing when it is named • Points to get desired object (proto-imperative pointing) and to share interest • Uses several gestures when vocalizing (e.g., waving, reaching) Language • Says a few words, including “mama,” “dada,” and exclamations like “uh-oh!”

Fig. 1.7  Developmental milestone at 12 months: May stand alone and make a few steps without holding

14 Months Gross motor • Walks well • Stands without pulling

1  General Pediatrics

9

• Gets an object from another room upon Fine motor demand • Imitates back and forth scribbling • May add the third block to a 2-block tower Language • Puts round peg in and out of a hole • Uses 3–5 words • Removes socks and shoes • Mature jargoning with real words • Chews well • Puts a spoon in the mouth upside down • Dumps pellet out of a bottle after a  8 Months 1 demonstration Gross motor Social/communication/problem-solving • Runs well • Points at an object to express interest (proto-­ • Creeps downstairs declarative pointing) • Gets onto a chair without assistance • Purposeful exploration of toys through trial and error Fine motor • Follows one-step commands without gesture • Throws a ball while standing • Makes 4-block tower Language • Imitates vertical stroke • Names one object • Can help undress him/herself • Eats with a spoon • Matches pairs of objects 15 Months Social/communication/problem-solving Gross motor • Normal M-CHAT • Stoops to pick up an object from the floor • Plays simple pretend, such as feeding a doll • Runs stiff-legged • Begins to have temper tantrum and shows • Climbs on furniture and may be able to creep shame when does wrong upstairs • Points to 2 of 3 objects when named and 3 body parts Fine motor • Points to familiar people with the name • Builds 3- to 4-block tower • Understands “mine” • Places 10 blocks in a cup • Drinks from a cup Language • Releases pellet into a bottle • Uses 10–25 words • Eats with a spoon with some spilling • Imitates animal sounds • Places circle in a single-shape puzzle • Names object in one picture on demand • Turns pages in a book Social/communication/problem-solving  4 Months 2 • Hugs parents in reciprocation • Shows empathy (may cry when someone else Gross motor is crying) • Walks upstairs and downstairs holding rail • Recognizes without demonstration that a toy • Kicks a ball requires activation, then hands it to an adult if • Throws ball overhand it cannot operate • Stands on tiptoes • Points to one body part

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Fine motor • Makes a single line of blocks • In drawing, imitates horizontal line • Begins to sort shapes and colors • Opens door using the knob • Takes off clothes without buttons • Pulls off pants • Builds a tower of 6 blocks • Parallel play

• Knows own gender and age • Follows 3-step instructions or commands • Fears imaginary things

Language • Uses words to describe what someone else is thinking (“Dad thought I was crying”) • Names body parts with function • Uses 3-word sentences • Says words like “I,” “me,” “we,” and “you” and some plurals (“cars”, “dogs,” “cats”) Social/communication/problem-solving • Names body parts by use • Begins to mask emotions for social etiquette • 75% intelligible speech • Follows 2-step instructions or commands such as “Sit on your chair and eat your food” • Points to 5–10 objects in pictures Language • Uses 2-word sentence • Uses 50 or more words • 50% intelligible speech

4 Years

Gross motor • Balances on 1 foot for 8 seconds • Hops and stands on 1 foot up to 2 seconds

Fine motor • Throws ball overhand more than 3 yards 3 Years • Catches a bounced ball most of the time • Copies a square Gross motor • Goes to the toilet alone • Walks up and down stairs, 1 foot on each step, • Wipes after a bowel movement no rails • Draws man with 4 to 6 parts • Climbs well • Pedals a tricycle (3-wheeled bike) Social/communication/problem solving • Balances on 1 foot for 3 seconds • Group play • Follows 3-steps commands and instructions Fine motor • Tells story and accurately counts 4 pennies • Copies a circle with pencil or crayon • Can work toys with buttons, levers, and movLanguage ing parts • Knows some basic rules of grammar, such • Screws and unscrews jar lids and turns door as correctly using “he,” “she,” “his,” “her” handle • 100% intelligible speech • Understands what “2” means • Imitates bridge of blocks • Independent eating • Puts on shoes without laces and able to unbut- 5 Years ton clothing Gross motor • Draws man with 2 to 3 parts • Walks downstairs with rail, alternating feet • Skips Social/communication/problem-solving • Understands long/short, big/small, more/less

1  General Pediatrics

Fine motor • Copies a triangle • Cuts with scissors • Builds stairs with blocks from model • Dresses and undresses

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Following objects • 1 month: Follows to the midline • 2 months: Follows past midline • 3 months: Follows 180° • 4 months: Circular tracking 360°

Speech intelligibility Social/communication/problem-solving • 50% intelligible at 2 years • Apologizes for mistakes • 75% intelligible at 3 years • Draws man with 8 to 10 parts • 100% intelligible at 4 years • Names 10 colors and counts to 10, counts 10 pennies correctly Language: receptive • 1 month Language –– Startles to voice or sound • Knows right from left • 2 months • Asks questions about the meanings of words –– Alerts to voice or sound and responds to questions • 4 months • Repeats 6 to 8 words in sentences –– Orients head to the direction of a voice or sound 6 Years • 8 months –– Responds to parents Gross motor • 9 months • Tandem gait (heel-to-toe walks) –– Orients attentively to his or her name • 10 months Fine motor –– Waves “bye-bye” in return • Builds stairs from memory • 12 months • Copies a diamond shape –– Follows one-step command with a gesture • Writes first and the last name • 14 months –– Follows one-step command without a gesture Social/communication/problem-solving • • • •

Has a best friend of same gender Looks both ways at street when crossing Draws man with 12–14 parts Able to do simple additions and subtractions

Language: expressive • Coos –– 2 months (2–4 months) • Laughs out loud –– 4 months Language • Babbles • Knows days of the week –– 6 months • Able to describe events in sequence • “Mama” or “dada” nonspecific –– 9 months • “Mama” and “dada” specific, plus a few words Key Points to Developmental –– 12 months Milestones • Vocabulary of 10–25 words –– 18 months Primitive reflexes • Two-word sentences • Moro is absent around 3–4 months of age –– 2 years (18–24 months) • Palmar grasp absent around 2–3 months of age • Parachute starts around 6–9 months of age

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Fig. 1.8  Fine motor developmental milestones and ability to draw at different ages

• Three-word sentences –– 3 years (2–3 year) • Four-word sentences –– 4 years (3–4 year) Drawing (Fig. 1.8) • Scribbles spontaneously –– 16 months • Imitates vertical lines –– 18 months • Imitates horizontal lines –– 2 years • Circle –– 3 years • Cross –– 4 years • Square –– 4.5 years • Triangle –– 5 years • Diamond –– 6 years

16 months

18 months

4 years

41/2 years

2 years

5 years

3 years

6 years

• Smiles spontaneously at a pleasurable sight/ sound –– 4 months • Recognizes caregiver socially –– 5 months • Stranger anxiety –– 6 months • Separation anxiety; gaze follows caregiver’s pointing to object, “Oh, look!” –– 9 months • Waves “bye-bye” in return –– 10 months • Shows objects to parents to share interests –– 12 months • Parallel play –– 2 years • Reduction in separation anxiety –– 28 months • Cooperative play –– 3–4 years • Ties shoelaces –– 5 years • Distinguishes fantasy from reality –– 6 years

Social skills • Reciprocal smiling –– 2 months • Follows the person who is moving across the Block play (Fig. 1.9) • Passes blocks room –– More than 6 months –– 3 months

1  General Pediatrics

15 mo

13

18 mo

2 years

3 years (bridge)

4 years (gate)

5 years (stairs)

Fig. 1.9  Fine motor skills and ability to use blocks at different ages

• Bangs blocks –– 9 months • Block in a cup –– 12 months • Tower 3 blocks –– 15 months • Tower 4 blocks –– 18 months • Tower 6 blocks –– 24 months • Builds bridge with blocks –– 3 years • Builds gate with blocks –– 4 years • Builds stairs from model –– 5 years Catching objects (Fig. 1.10) • Rakes –– 5–6 months • Radial-palmar grasp of pellet –– 7–8 months • Inferior pincer grasp of pellet –– 10 months • Fine pincer grasp of pellet –– 12 months Walking and running • Independent steps –– 12 months

Rake

Inferior pincer

Fine pincer

Fig. 1.10  Fine motor skills of catching an object at different ages

• Walks well –– 14 months • Runs stiff-legged –– 15 months • Runs well –– 18 months • Kicks ball without demonstration –– 2 years • Skips and walks backward heel-toe –– 5 years • Heel to toe walks (tandem gait) –– 6 years

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Table 1.4  Developmental red flags for language and social Climbing stairs skills by age [4] • Creeps upstairs Age Language and social red flags –– 15 months Newborn Does not respond to loud sounds • Creeps downstairs 2 months Does not alert to voice, lack of looking at faces –– 18 months Does not watch things as they move • Walks downstairs holding rail, both feet on 4 months Does not coo or make sounds each step Does not smile at people 6 months Does not turn toward sounds; no smiling, –– 2 years laughing, or expression • Goes up stairs, alternating feet, no rail 9 months Does not babble (“mama,” “baba,” “dada”) –– 3 years 12 Does not respond to name • Walks downstairs with rail, alternating feet months Does not understand “no” –– 5 years Indifferent or resistant attachment to the caregiver Developmental red flags (Tables 1.3 and 1.4) [4]

Table 1.3  Developmental red flags for motor skills by age [4] Age Newborn 2 months 4 months

6 months 9 months 12 months 15 months 18 months 24 months 36 months 4 years 5 years 6–12 years All ages

Motor red flags Hypotonia and feeding difficulty Unable to hold head up when pushing up when on tummy Unable to hold head steady Unable to bring things to the mouth Persistent fisting (a predictor of neurological dysfunction) Unable to pass an object from one hand to another and does not try to reach an object Floppy like a rag doll Unable to sit, not rolling Unable to stand or bear weight on legs when supported Unable to crawl Unable to do pincer grasps Unable to walk Unable to walk well Unable to climb stairs well and frequent falling Unable to jump in place Unable to draw pictures, a cross, or a square Poor balance Unable to skip or hop on one foot Unable to write name Loss of skills they once had

15 months 18 months 24 months

36 months 4 years

5 years

6–12 years All ages

Does not look where caregiver points Does not use words “mama,” “papa,” “dada” Does not point to the desired object Does not gain new words Does not have at least 6 words Does not point to show things to other or share interest Unable to use two-word phrases (e.g., “drink water”) Unable to follow simple instructions Unable to imitate actions or words Unable to maintain eye contact Unable to use a three-word sentence Unable to pretend, play, or make-believe Unable to speak clearly Unable to answer simple questions Unable to use pronouns (“I”, “me”, “you”, “he”, and “she”) correctly Ignores other children or does not respond to people outside the family Unable to use plurals or past tense properly Unable to recognize shapes, letters, colors Unable to brush teeth, use toilet, wash and dry hands, or get undressed without help Unable to distinguish between reality and fantasy Shows extreme behavior (unusually fearful, aggressive, shy, or sad) Unable retell or summarize a story Unable to name friends Unable to recognize the feelings of others Loss of skills they once had

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15

IMMUNIZATION [6, 7] (TABLE 1.5) Responding to parents who refuse immunization for their children • Listen to parents and address all their concerns about vaccines • Explain all risks and benefits of the vaccinations: –– Unimmunized, delay in vaccination, and use of alternative immunization schedules have caused a resurgence of many infectious diseases due to the loss of herd immunity, which puts many communities at risk –– Vaccines are very safe, but they are not risk-free, nor are they 100% effective

• • •

•

• •

Table 1.5  Immunization schedule summary Immunization schedule Birth 2 months 4 months 6 months 12 months 15–18 months 18 months 4–6 years 11–12 years 16 years High risk

Vaccine HepB DTap, IPV, HepB, Hib, PCV, RV DTap, IPV, HIB, PCV, RV DTap, IPV, HepB, Hiba, PCV, RVb, Influenzac MMR, Varicella, Hib, PCV, HepA DTap HepA (1st and 2nd dose must be 6 months apart) MMR, Varicella, DTap, IPV Tdap, MCV4 HPV (2-dose series 6–12 months apart)d Second dose of MCV4 PPSV23 2–18 years MCV4 2–10 years Meningococcal B (10 years and older)

DTaP Diphtheria and tetanus toxoids and acellular pertussis vaccine, DTP Diphtheria, pertussis, and tetanus, HepA Hepatitis A, HepB Hepatitis B, Hib Haemophilus influenzae type b (Hib) conjugate, HPV Human papillomavirus vaccine, IPV Inactivated poliovirus vaccine, MCV4 Meningococcal conjugate ACWY vaccine, MMR Measles, mumps, and rubella, PCV Pneumococcal conjugate vaccine, PPSV23 Pneumococcal polysaccharide vaccine, Tdap Tetanus and diphtheria toxoids and acellular pertussis vaccine, RV Rotavirus vaccine a Hib dose at 6 months is not required if using PedvaxHIB (Merck) b Dose at 6 months is not required if using Rotarix (GSK) c Influenza every year beginning at 6 months d 3 doses of HPV for persons initiating vaccination at age 15 years or older

•

–– Compare the risks of vaccines to the risks of diseases they protect against, some of which can cause death or permanent disability Refer parents to reputable sources and database about vaccines Discuss risks and benefits in each subsequent visit along with proper documentation Unimmunized children may be prevented from attending schools during outbreaks of vaccine-preventable diseases Advise parents to inform health-care providers that their child is not immunized during acute illness (e.g., emergency room visit) Have parents sign vaccine refusal form in every subsequent well visit Continued refusal after adequate discussion should be respected unless the child is put at significant risk of serious harm, e.g., refuses rabies vaccines after the child was bitten by a stray dog In general, the pediatrician should not refuse a child care because caregivers reject vaccines unless a strong sense of distrust develops that impacts the child’s overall care

Recommended immunization schedule for children and adolescents ages 18  years or younger, United States, 2018 (US Department of Health and Human Services, Centers for Disease Control and Prevention) https://www.cdc.gov/vaccines/schedules/hcp/imz/child-adolescent.html

Hepatitis B Vaccine (HepB) First dose of HepB is at birth • Birth dose (monovalent HepB vaccine only) Doses following birth dose • Administer the second dose 1–2 months after the first dose (minimum interval of 4 weeks) • Administration of four doses of HepB is permissible if the combination is used after birth dose

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• The final third or fourth dose in the HepB series should not be administered before 6 months of age

• If RotaTeq (RV5; Merck & Co.) is used, administer a 3-dose series at age 2, 4, and 6 months • If any dose in the series was RotaTeq or vaccine product is unknown for any dose in the series, a total of 3 doses of RV vaccine should be administered

Mother is HBsAg-negative • One dose within 24 h of birth for medically stable infants ≥ 2000 g. For infants  500 mg/kg is potentially fatal

Ibuprofen • Inhibit prostaglandin synthesis

Clinical presentation • Acute salicylism –– Nausea, vomiting, diaphoresis, and tinnitus

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• Moderate toxicity Beta-Blockers –– Tachypnea, hyperpnea, tachycardia, and AMS • Acebutolol, atenolol, bisoprolol, metoprolol, • Severe toxicity nadolol, sotalol, and propranolol –– Hyperthermia and coma Clinical presentation Management • Hypotension, bradycardia, atrioventricular • Consider activated charcoal (AV) block, heart failure • Check blood gas (respiratory alkalosis, meta• Bronchospasm bolic acidosis, and high anion gap) • Hypoglycemia, hyperkalemia • Check serum level every 2 h until it is consis• Stupor, coma, seizures tently down trending • IV fluids Management tailored to the symptoms • Sodium bicarbonate therapy in the symptom• Consider dose of activated charcoal atic patients • Hypotension/bradycardia/AV block: Fluid • Goal of therapy includes a urine pH of 7.5– boluses, beta-agonists, vasopressors, atro8.0, a serum pH of 7.5–7.55, and decreasing pine, possibly pacing salicylate levels • Hypoglycemia: Glucose • Hyperkalemia: Calcium gluconate, dextrose and insulin, sodium bicarbonate, possibly Anticholinergics dialysis • Two special cases • Diphenhydramine, atropine, scopolamine, –– Propranolol → causes sodium channel hyoscyamine, jimsonweed (Datura stramoblockade → QRS widening → treat with nium), and deadly nightshade (Atropa sodium bicarbonate belladonna) –– Sotalol → causes potassium efflux blockade → prolonged QT  →  monitor for Clinical presentation (anticholinergic symptoms) torsades • Dry as a bone: Dry mouth, decreased sweating, and urination • Red as a beet: Flushing  arbamazepine C • Blind as a bat: Mydriasis, blurred vision • Mad as a hatter: Agitation, seizures, Clinical presentation hallucinations • Mild ingestion: • Hot as a hare: Hyperthermia –– CNS depression • Bloated as a toad: Ileus, urinary retention –– Drowsiness • Heart runs alone: Tachycardia –– Vomiting –– Ataxia Management –– Slurred speech • Consider activated charcoal –– Nystagmus • Supportive care • Severe intoxication: • Physostigmine can be considered for severe –– Seizures or persistent symptoms

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227

• Bradycardia • Hypotension –– Can cause transient initial hypertension • Apnea

–– Coma –– Respiratory depression Management • Activated charcoal • Supportive measures • Charcoal hemoperfusion intoxication

for

Cardiac Glycosides (Digitalis)

severe

Management • Supportive care, e.g., intubation, atropine, dopamine as needed • Charcoal usually not recommended due to CNS depression

• Digoxin, foxglove plants, oleander, lily of the Opiates valley (Convallaria) • Morphine, heroin, methadone, propoxyphene, codeine, meperidine Clinical presentation • Most cases are drug abuse • Nausea and vomiting • CNS depression (confusion) • Blurry vision Clinical presentation • Cardiac conduction abnormalities (irregular • Common triad of opiate poisoning: pulse, bradycardia or tachycardia) –– Pinpoint pupil Management –– Mental depression (lethargy to coma) • Electrocardiogram (ECG) and digoxin levels –– Respiratory depression • Activated charcoal • Hypotension with no change in heart rate • Atropine, cardiac pacing (for severe • Decreased GI motility, nausea, vomiting, bradycardia) abdominal pain • Magnesium sulfate (for premature ventricular Management contractions [PVCs]) • Supportive care, e.g., airway, breathing, and • Management of hyperkalemia, hypokalemia, circulation; intubation; fluids as necessary hypomagnesemia • Naloxone as needed • If severe, digoxin-specific antibody –– Half-life of naloxone is short fragments –– Repeat doses and continuous infusions may be necessary –– Cautious use in known opioid-dependent Clonidine patients ◦◦ Can induce withdrawal • Antihypertensive medication with α-2 adrenergic receptor blocking ability • Commonly used in children with attentionPhenothiazines deficit hyperactivity disorder (ADHD) • Dose as small as 0.1 mg can cause toxicity in • Promethazine, children chlorpromazine

Clinical presentation • Lethargy • Miosis

Clinical presentation • Hypertension

prochlorperazine,

and

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• Cogwheel rigidity Prevention of CO poisoning • Dystonic reaction (spasm of the neck, tongue • CO detectors thrusting, oculogyric crisis) • Maintenance of fuel-burning appliances • CNS depression • Yearly inspection of furnaces, gas pipes, and chimneys Management • Car inspection for exhaust system • Activated charcoal • No running engine in a closed garage • Manage high BP • Avoid indoor use of charcoal and fire sources • Diphenhydramine for dystonic reaction Clinical presentation • Headache, malaise, nausea, and vomiting are Tricyclic Antidepressants (TCAs) the most common flu- or food poisoning-like early symptoms • TCAs can cause significant toxicity in chil• Confusion, ataxia, syncope, tachycardia, and dren even with ingestion of 1–2 pills (10– tachypnea at higher exposure 20 mg/kg) • Coma, seizure, myocardial ischemia, acidosis, cardiovascular collapse, and potentially Clinical presentation death in severe cases • Anticholinergic toxidrome: Delirium, mydriasis, dry mucous membrane, tachycardia, Management hyperthermia, hypotension, and urinary • Evaluate for COHb level in symptomatic retention patients • Cardiovascular and CNS symptoms dominate • Arterial blood gas with CO level the clinical presentation • Check creatine kinase in severe cases • Most common cardiac manifestations: • ECG in any patient with cardiac symptoms Widening of QRS complex, PVCs, ventricu• 100% oxygen to enhance elimination of CO, lar arrhythmia use until CO  25%, significant CNS symptoms, or cardiac dysfunction Management • Supportive measures, ABCs • ECG: Cyanide –– A QRS duration > 100 ms identifies patients at risk for seizures and cardiac arrhythmia • Seeds (cherries, apricots, peaches, apples, –– An R wave in lead aVR of > 3 mm is an plums); cassava; burning plastics (nitrile-­ independent predictor of toxicity containing products); nitroprusside; some • Start sodium bicarbonate therapy: QRS durapesticides tion >  100  ms, ventricular dysrhythmias, • Amygdalin is contained in seeds and produces hypotension, and seizures hydrogen cyanide, which is a potent toxin • Inhibition of cellular respiration (cytochrome c oxidase) stops ATP production

Carbon Monoxide (CO)

• Wood-burning stove, old furnaces, and Clinical presentation automobiles • Decreased level of consciousness

7  Emergency Medicine

• Low exposures—weakness, headache, dizziness, confusion • Severe exposures—seizure, apnea, cardiac arrest • Cherry red skin Management • Supportive measures • Hydroxocobalamin (vitamin B12), nitrites

 thylene Glycol Ingestion E (Antifreeze) Clinical presentation • Nausea, vomiting, CNS depression, anion gap metabolic acidosis • Hypocalcemia, renal failure due to deposition of calcium oxalate crystals in the renal tubules Management • Osmolar gap can be used to estimate ethylene glycol level • IV fluids, glucose, and bicarbonate as needed for electrolyte imbalances and dehydration • Fomepizole • Ethanol can be used if fomepizole is unavailable

Methanol • Toxicity primarily caused by formic acid

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• Ethanol can be used if fomepizole is unavailable • Hemodialysis (consider if > 30 ml methanol ingested)

Iron • Ingestion of > 60 mg/kg/dose is toxic Clinical presentation • Gastrointestinal stage (30 min−6 h) –– Nausea, vomiting, and abdominal pain –– Hematemesis and bloody diarrhea in severe cases • Stability stage (6–24 h) –– No symptoms: Patient must be observed during this stage • Systemic toxicity within (48 h) –– Cardiovascular collapse –– Severe metabolic acidosis • Hepatotoxicity and liver failure (2–3 days) • Gastrointestinal and pyloric scarring (2–6 weeks) Management • Abdominal radiograph (may show pills and need for GI decontamination) • Serum iron  500 mcg/dl

Clinical presentation • Drowsiness, nausea, and vomiting Mushrooms • Metabolic acidosis • Visual disturbances: blurred and cloudy • Ingestion of mushrooms can have fatal consevision, feeling of being in a snowstorm, and quences in species that harbor amatoxins untreated cases can lead to blindness (e.g., Amanita) and related compounds Management Clinical presentation • Osmolar gap (may be used as surrogate • Nausea, vomiting, and diarrhea; delayed marker until methanol blood level is onset (6 h) available) • A second latent period is followed by acute • IV fluids, glucose and bicarbonate as needed and possibly fulminant hepatitis beginning for electrolyte imbalances and dehydration 48–72 h after ingestion • Fomepizole

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Management • Activated charcoal • Whole bowel irrigation • Supportive care, including liver transplant, if necessary, is the mainstay of therapy

Caustic Ingestion • Strong acid and alkalis   12 pH can produce severe injury even in small-volume ingestion • Patient can have significant esophageal injury without visible oral burns

J.-A. O. Nesiama et al.

• Benzene is known to cause cancer • Inhalants, including toluene, propellants, and volatile nitrite, can cause dysrhythmias and sudden death Clinical presentation • Cough, tachypnea, respiratory distress Management • Emesis and lavage are contraindicated • Activated charcoal should be avoided due to risk of inducing vomiting • Observation and supportive care

Clinical presentation  rganophosphate and Carbamate O • Pain, drooling, vomiting, and abdominal pain Insecticides (Nerve Gas Agents) • Difficulty in swallowing, or refusal to swallow • Inhibit anticholinesterase • Stridor and respiratory distress are common presenting symptoms Clinical presentation • Esophageal stricture caused by circumferen• (DUMBBELLS) “Drowning in your own tial burn; requires repeated dilation or surgisecretions” cal correction –– Diarrhea –– Urination Management –– Miosis • Supportive measures, ABCs –– Bradycardia –– Inducing emesis and lavage are –– Bronchospasm contraindicated –– Emesis –– Endoscopy should be performed within –– Lacrimation 12–24  h in symptomatic patients, or on –– Lethargy basis of history and characteristics of –– Salivation and Seizures ingested products Management • Wash all exposed skin with soap and water Hydrocarbons and immediately remove all exposed clothing • Products contain hydrocarbon substances, • Fluid and electrolyte replacement, intubation, mineral spirits, kerosene, gasoline, turpenand ventilation if necessary tine, and others • Atropine and pralidoxime • Aspiration of even small amount can be serious and potentially life-threatening • Pneumonitis is the most important manifestation of hydrocarbon toxicity

7  Emergency Medicine

 OREIGN BODY ASPIRATION F AND INGESTION Foreign Body Aspiration • Occurs in the context of child’s play/exploration of environment • Foreign bodies may lodge in the upper or lower respiratory tract

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• Bilateral decubitus views may aid in diagnosis: Bilateral to compare which lung exhibits air trapping, which may be where the foreign body is lodged • The presence of normal chest radiographs does not exclude this diagnosis in the presence of a compelling history • For patients in severe respiratory distress, immediate bronchoscopy to remove the foreign body emergently is key to treatment

Upper airway • The most commonly implicated foods are  oreign Body Ingestion F –– Candy, meat, hot dogs, grapes • Associated symptoms include • Most children will have a history of ingested –– Choking, coughing, stridor, respiratory foreign body, often reported by a caregiver or distress playmate/sibling –– May result in complete airway obstruction • Most commonly ingested foreign bodies are • In patients with complete airway obstruction, –– Food (meat) emergency procedures may be life-saving –– Followed by coins, pins, toy parts, button –– Back blows in infants batteries, magnets –– Heimlich maneuvers in older children • Most will pass harmlessly through the GI –– If patient becomes unconscious, may need tract to initiate cardiopulmonary resuscitation • Foreign bodies may lodge in areas where (CPR) there is physiological/anatomic narrowing of ◦◦ Convert to rescue breaths and chest the lumen of the GI tract compressions –– Lodging may occur secondary to patholog–– If all of the above are unsuccessful, then ical narrowing of the lumen of the GI tract advanced airway techniques may be (e.g., from previous surgeries such as in necessary tracheoesophageal fistula, esophageal/duodenal webs) Lower airway • Symptoms, if present, may include coughing, • More common in younger children choking, foreign body sensation, throat pain, • Foreign body may lodge in the right or left lung drooling, vomiting, refusal or inability to tol• Symptoms may involve coughing, choking, erate fluids/food wheezing, or may be asymptomatic • Children may also be completely • Diagnosis may be delayed due to lack of asymptomatic symptoms Management • If the patient is stable and not in respiratory distress, neck and/or chest radiographs may help with diagnosis –– Not all inhaled foreign bodies are radiopaque and may not be visualized on radiographs even if present

Esophagus • The esophagus is the most common site for ingested foreign bodies to become lodged • Ingested foreign bodies typically become lodged in one of three sites: –– At the thoracic inlet

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–– Mid-esophagus, at the level of the carina and aortic arch –– Esophago-gastric junction

DIABETIC KETOACIDOSIS (DKA)

 KA in Pediatric Patients D Management • If the patient is asymptomatic and there is no • DKA is a severe complication of type 1 airway compromise, may do neck and/or diabetes chest radiographs • Occurs in 25–40% of new-onset type 1 • If foreign body is not visualized and patient is diabetes asymptomatic, then can do an esophagram • Inadequate relative or absolute deficit of insu• If foreign body is lodged in the esophagus, lin leads to starvation of insulin-dependent may need removal by esophagoscopy tissue (muscle, liver, fat) with resultant • Some authorities report the use of glucagon hyperglycemia with varying degrees of success • Starvation state triggers a cascade of hor• Emergent removal is indicated for two or monal release such as glucagon, catecholmore magnets, button batteries, lodged sharp amines, cortisol, cytokines objects • Results in a catabolic state with lipolysis, proteolysis, adipose tissue metabolism into free Stomach and Lower Gastrointestinal Tract fatty acids, hepatic conversion of fatty acid to • Most foreign bodies in the stomach will pass keto-acids, and anion gap metabolic acidosis harmlessly through the remaining portion of • Hyperglycemia causes osmotic diuresis with the GI tract hypovolemia and dehydration • Single, not sharp foreign bodies may be managed conservatively and observed • Parents may be advised to watch patient Diagnostic laboratory findings • Acidosis (venous pH   7.2 and  2 weeks old; most common adverse effects are nausea and vomiting, although neuropsychiatric events have been reported –– Zanamivir is inhaled, approved for treatment (age > 7) and prophylaxis (age > 5) –– Baloxavir in children ≥ 12 years administered as a single oral dose Adamantanes (amantadine and rimantadine) no longer recommended due to resistance

Avian Influenza H5N1 Background • Reported cases were in south Asia, Iraq, Turkey, and Egypt • Highly pathogenic strain in birds and poultry • Not a human strain Mode of transmission • Humans who have close contact with infected birds or poultry • Visiting market selling live infected birds Clinical presentation • Severe lower respiratory disease in infected persons Prevention • H5N1-specific vaccine (developed and approved) • Avoid visiting markets where live birds are sold • Thorough cooking inactivates the virus, but avoidance of poultry if there a concern is more appropriate

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Parainfluenza Virus (PV) Background and epidemiology • Parainfluenza viruses are paramyxoviruses distinct from the influenza family • Previous infection does not confer immunity, so reinfection can occur • Transmitted via contact with NP secretions and respiratory droplets and fomites • Seasonal patterns of transmission: PV1 and PV2 occur in fall, PV3 occurs in spring, and PV4 occurs year-round • Children shed virus for 1 week before symptoms and for 1–3 weeks after symptoms resolve • Incubation period is 2–6 days

M. B. Laurens

–– Reduces risk of lower tract disease in highrisk children –– Administered every 30  days during RSV season (max 5 doses/season) –– Considered for first 1–2 years of life –– Indicated for: 1. Preterm infants with chronic lung disease 2. Infants with hemodynamically significant congenital heart disease 3. Preterm infants  6 months in immunocompromised (66–75%) • Children typically have 2 episodes/year; Clinical presentation adults have 1 episode/year • Abrupt onset of nausea and vomiting (profuse, nonbloody, nonbilious) more common Clinical features in adults • Pharyngitis, nasal congestion, and discharge • Watery diarrhea (non-bloody) may be the that goes from clear to mucopurulent only symptom in children • Malaise, headache, myalgia, cough, fever • Abdominal cramps • Symptoms peak at 3–4  days and last 7  days • Headaches • Can cause otitis media, bronchiolitis, and • Low-grade fever is common pneumonia • Myalgias and malaise Testing • Chronic gastroenteritis in immunocompro• Not useful clinically mised • PCR preferred, usually paired with enterovi• Symptoms last 24–48 h, longer in children, rus PCR due to genetically conserved regions immunocompromised, and elderly GI Viral Infection • Norovirus (Norwalk virus) and Sapovirus • Rotavirus

Norovirus and Sapovirus

Rotavirus Background and epidemiology • Causes severe acute gastroenteritis • Shed in stool days before and after clinical illness • Transmitted fecal–oral, possibly respiratory • Late winter to early spring transmission • Stable in environment for weeks to months • Rotavirus was most common viral cause of acute gastroenteritis until vaccine introduction that reduced hospitalizations by 75% for children  4 months, at least 2 positive mothers (especially with high viral negative HIV antibody tests from separate load) decreases the risk of transmission of specimens at > 6 months and no clinical or HIV to their infants laboratory evidence of infection • Immunization of infants and children –– Immunization schedule for HIV-exposed Diagnosis: Adult and adolescent infection children is the same as for their healthy • Conduct initial serology, followed by confirpeers, with only a few exceptions: matory serology testing ◦◦ Patients who have severely symptomatic • For initial serology testing: illness –– Antibodies to HIV-1/HIV-2 and HIV-1 p24 ◦◦ Patient with CD4 percentage of less than antigen (fourth generation) 15% or CD4 counts of less than 200 –– Antibodies to either HIV-1 and HIV-2 cells/mm3 should not receive MMR, var(third generation) icella vaccines, or other live vaccines

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• Annual influenza immunization is recommended for all children older than age 6 months, but only the killed vaccine

Measles Virus

M. B. Laurens

• HIV on antiretroviral therapy and documented measles vaccination × 2: Treat as immune • Vaccinate all health-care personnel

Mumps

Background • Mode of transmission: Respiratory droplets Background (airborne) • An acute, self-limited, systemic viral illness • Infectious for 3–4 days before the onset of characterized by the swelling of one or more morbilliform rash and 4  days after the of the salivary glands, typically the parotid exanthem glands Diagnosis Mode of transmission • PCR testing, IgM detection, 4-fold rise in IgG • Contact to respiratory secretions serology, cell culture • Incubation period is 16–18 days • IgM detection is preferred test Clinical presentation Clinical presentation • Symptoms in the patient’s history consist • High fever plus coryza, cough, conjunctivitis mostly of fever, headache, and malaise • Rash develops next: Erythematous, morbilli• Within 24 h, patients may report ear pain form, maculopapular rash spread from face localized near the lobe of the ear and aggradownward and disappears the same way vated by a chewing movement of the jaw • Koplik spots (white spots on oral mucosa) • Unilateral or bilateral parotid swelling during prodrome (Fig. 9.7) • Complications in young children and immu• Orchitis may occur after puberty; rarely nocompromised include otitis media, broncauses sterility chopneumonia, croup, diarrhea, and death • Death is also more common with severe Complications • Rare: Arthritis, thyroiditis, glomerulonephrimalnutrition tis, myocarditis, transverse myelitis, enceph• Severe complication: Acute encephalitis with alitis, oophoritis, permanent hearing permanent brain damage impairment Control and prevention • Vaccinate non-immunes within 72  h of Diagnosis (Table 9.1) • PCR from buccal swabs, throat washings, exposure saliva, or CSF • Immune globulin within 6 days of exposure • Viral cell culture for non-immunes if vaccination not possible, • Serology including pregnant women, severe primary immunodeficiency, bone marrow or solid organ transplant recipient, acute lymphoblastic Treatment • Supportive care only leukemia, HIV AIDS with severe immunosup• School exclusion for 5 days from onset of pression, and infants whose mothers received parotid gland swelling immunomodulatory drugs during pregnancy

9  Infectious Diseases

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• The USA has not experienced rubella transmission since 2004; imported cases occur • The Americas have not experienced rubella transmission since 2009 • Incubation period: 16–18 days

Fig. 9.7  Child with unilateral parotitis

Table 9.1  Differences between viral and bacterial parotitis Viral parotitis (mumps) Well-appearing No fever or low-grade fever Mild tenderness Normal labs, positive mumps IgM

Bacterial parotitis Toxic-appearing or ill-looking High fever Moderate or severe tenderness Leukocytosis, shift to the left, high CRP

IgM Immunoglobulin M, CRP C-reactive protein

• Unimmunized children should stay out of school for 26 days after onset of parotitis in the last person with mumps in the affected school.

Rubella Virus Epidemiology • Transmitted via direct or droplet contact with respiratory secretions • Peak incidence from winter to spring • 25 to 50% asymptomatic • Lifelong immunity • Can transmit 3 days before to 7 days after rash appears • Infants with congenital rubella may shed for 1 year in nasopharyngeal secretions and urine

Clinical presentation • Congenital rubella syndrome –– Constellation of congenital anomalies ◦◦ Ophthalmologic (cataracts, microphthalmos, congenital glaucoma) ◦◦ Cardiac (patent ductus arteriosus, peripheral pulmonary artery stenosis) ◦◦ Auditory (hearing impairment) ◦◦ Neurologic (meningoencephalitis, microcephaly, mental retardation, autism) –– Neonates will have growth restriction, interstitial pneumonitis, hepatosplenomegaly, thrombocytopenia, and dermal erythropoiesis that manifests as “blueberry muffin” rash –– Neonates may also have metaphyseal lucencies (also seen in vitamin D intoxication/hypercalcemia, rickets, scurvy, arsenic and heavy metal poisoning, leukemia, congenital syphilis, sickle cell disease, congenital hypothyroidism) –– Increased risk of congenital defects if fetal infection occurs early in pregnancy • Postnatal rubella –– Subclinical or mild disease –– Erythematous maculopapular rash –– Forchheimer spots: Rose-colored spot on soft palate –– Lymphadenopathy (posterior auricular or suboccipital nodes) –– Conjunctivitis –– Adolescent females susceptible to transient arthralgia and arthritis –– Rare complications: Encephalitis, thrombocytopenia • Infants with congenital rubella may shed the virus from the nasal mucosa > 1 year to susceptible contact

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Rabies Virus

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Arboviruses (Arthropod-Borne Viruses)

• West Nile virus (WNV) Background • Dengue fever • RNA virus classified in the Rhabdoviridae family • Usually transmitted by bats and carnivores,  est Nile Virus W e.g., raccoons, foxes, and coyotes • Almost never transmitted by squirrels, chipBackground and epidemiology munks, rats, mice, rabbits, and guinea pigs • The most common neuroinvasive arboviral disease in the USA Clinical presentation • Transmitted to humans by Culex mosquitoes • Anxiety • Transmission occurs from summer to fall • Dysphagia • California, North and South Dakota,  Nebraska • Seizures and Illinois were the most common locations • Encephalitis in 2018 • In most cases, progress to death • Incubation 2–6 days Treatment • Humans also infected via transfusion and • Prompt local flushing and cleaning the wound organ transplant with soap and water • Passive and active immunization for: Clinical presentation –– All persons bitten by bats, carnivores, e.g., • Most cases are asymptomatic (70–80%) raccoon, foxes, and coyotes • May present with fever and flu-like –– Open wound or scratch contaminated with symptoms saliva of infected animals or human • Fever, headache, myalgia, arthralgia, vomit• No prophylaxis if domestic dog, cat or ferret ing, diarrhea, transient rash that can be observed for 10 days •  90% of anogenital warts Clinical features • Most infections are subclinical • Skin warts are generally painless; plantar warts can be painful • Anogenital warts (condylomata acuminata) have cauliflower-like surface and can occur in groups • Invasive cancers linked to HPV include the following locations: Oropharynx, penis, anus, cervix, vagina, and vulva • Squamous intraepithelial lesions can be low or high grade due to persistent HPV • Cervical intraepithelial neoplasia (CIN) is precancerous and linked to HPV • Adenocarcinoma in situ is another endocervical precancer

Laboratory Immunization • During febrile phase, diagnose with PCR for • 2 doses before 15th birthday viral DNA or immunoassay for nonstructural • Only 9-valent vaccine available in USA since protein 1 (NS-1) 2017 • From 3 to 5 days after onset, can test for antidengue IgM • Leukopenia, thrombocytopenia, and modest BACTERIAL PATHOGENS elevation of liver enzymes Treatment • Supportive

Gram-Positive Bacteria S. aureus

Background and epidemiology • S. aureus is the most common cause of skin and soft tissue infection and musculoskeletal Background and epidemiology infection in healthy children • Most adults will be infected at some time • Second leading cause of healthcare-associ• School-age children acquire nongenital hand ated bacteremia (coagulase-negative staphyand foot warts through minor skin trauma lococci is first) • Genital transmission occurs skin-to-skin • Leading cause of secondary bacterial pneu• HPV causes most vulvar, vaginal, penile, monia in children and anal cancers; 70% of oropharyngeal • Most common cause of healthcare-associated cancers surgical site infections • Rare transmission to infant during delivery • Coagulase positive • Incubation period is months to years • Grapelike clusters (Fig. 9.8)

Human Papillomavirus (HPV)

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• Children who undergo neurosurgical procedures, especially shunt revisions, are at high risk for staphylococcal infection • Indwelling bloodstream catheters can be associated with staphylococcal infection and must be removed if the patient develops symptoms or positive culture

Folliculitis/Furunculosis/ Carbunculosis (Fig. 9.9) Fig. 9.8  Staphylococci in blood culture (Gram stain, original magnification × 1000). The bacteria are Gram-positive cocci and grow in pairs, tetrads, and clusters (arrow). (Courtesy of M.  Nawar Hakim, MD, Department of Pathology, Texas Tech University Health Sciences Center, El Paso, Texas, USA)

• S. aureus colonizes the nares and skin in 30–50% of children • Transmitted by direct contact and indirectly from other patients in hospital settings • Can spray short distances into the air • “Vancomycin-intermediately susceptible S. aureus” related to repeat vancomycin use in individuals. Vancomycin-resistant S. aureus rare • Incubation period can be 12 h for postoperative toxic shock syndrome

Background • Folliculitis: Superficial inflammation centered around a follicle • Furuncles: Bacterial folliculitis of a single follicle that involves a deeper portion of the follicle • Carbuncle: Bacterial folliculitis that involves the deeper portion of several contiguous follicles • Bacterial folliculitis most often caused by S. aureus. • Hot tub folliculitis is usually caused by Gramnegative bacteria (most often P. aeruginosa; self-limited) • Usually the child looks healthy and does not appear ill • Abscess (  1—Exposure is associated with a higher odds of an outcome Table 29.1  Calculating the odds ratio Disease present (+) Risk factor/exposure A (+) Risk factor/exposure C (−)

Disease absent (−) B D

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• OR   1 → hypothesis of “difference” Smoking is associated with higher odds of • There is a statistically significant difference lung cancer between the 2 variables that are being studied Risk: Probability of a Disease • Example: Does exercise reduce cholesterol levels? Outcome • Alternative hypothesis: Exercising daily has 1. Absolute risk—Ratio of the number of people an effect on cholesterol level by lowering exposed to a risk factor that developed disease cholesterol levels. to all of those who were exposed to the risk Type I error (alpha) factor • A type I error (false-positive, also known as a Absolute risk = A / A + B rejection error) is rejection of a null hypothesis that is actually true in the population 2. Relative risk—Disease risk in the exposed • The investigator concludes that there is a siggroup divided by disease risk in unexposed nificant difference between the groups when, group. It can be calculated for cohort study in fact, there is no true difference (prospective study). • This risk can be reduced by setting a more stringent P value (e.g., .01 instead of .05) For a rare disease, OR approximates R • A difference was seen that truly does not exist Relative risk = A / ( A + B ) / C / ( C + D ) Type II error (beta) 3. Attributable risk—Difference in risk between • A type II error (false negative, also known as the exposed and unexposed groups an acceptance error) is failure to reject the null hypothesis that is actually false Attributable risk = A / A + B - C / C + D • The investigator concludes that there is no difference when a difference actually exists in the population Statistical Hypothesis • Increasing the sample size will reduce the (Null Hypothesis vs. Alternative risk of these errors Hypothesis) • A difference that was not seen but which truly exists Null hypothesis • The null hypothesis is referred to as the Power of a study hypothesis of “no difference” • The probability of rejecting the null hypoth• There is no statistically significant relationesis or the probability that there is a treatment ship between the 2 variables that are being effect (i.e., exercising daily reduces cholesstudied terol levels)

29  Research and Statistics

• The larger the sample size, the higher the statistical power • Power = 1 minus the probability of a type II error (beta)

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–– P ≤ 0.01: Very strong presumption against null hypothesis –– P ≤ 0.05: Strong presumption against null hypothesis –– 0.05   0.1: No presumption against the null hypothesis

Standard deviation (SD) • A measure of the variability of individual values around the mean or average value • When data are grouped closely together, the SD is small. When data are highly variable, Confidence intervals the SD is large • A confidence interval is a range of values • One standard deviation—68% of the numwith a specified probability that a given bers fall within the mean (average) parameter falls in that range • 2 standard deviations—95% of the numbers • Often 95% (P value of 0.05) or 99% (P value fall within the mean of 0.01) • 3 standard deviations—99.7% of the numbers • If the confidence interval includes a 0 or a 1, fall within the mean then the null hypothesis cannot be rejected Standard error of the mean (SEM) Intention-to-treat analysis • Describes the variability in a distribution of • In an intention-to-treat analysis, study particsample means ipants are analyzed according to their ran• Mathematically, it is the sample SD divided domized assignment, regardless of changes by the square root of the sample size that may occur after randomization • SEM = Standard deviation/sample size • Intention-to-treat analysis prevents the loss of • So as the sample size increases, the standard statistical power that may be encountered error decreases with a failure to complete study protocols • SEM is a smaller number than the SD (dropout) or noncompliance P value Number needed to treat • The P value is the probability of obtaining a • The total number of patients who need to be test statistic result at least as extreme as the treated to prevent an adverse event or bad one that was actually observed, assuming that outcome the null hypothesis is true • Number needed to treat = 1/absolute risk • A researcher will often “reject the null reduction (ARR) hypothesis” when the P value turns out to be • Example if the ARR is 20% then the number less than a predetermined significance level, needed to treat = 1/0.2, which is 5. Thus 5 often 0.05 or 0.01. Such a result indicates that people need to be treated to prevent one bad the observed result would be highly unlikely outcome under the null hypothesis • The number needed to harm is a measure that • Many common statistical tests, such as chiindicates how many persons on average need square test or Student t test, produce test stato be exposed to a risk factor over a specific tistics that can be interpreted using P values period to cause harm in an average of one • An informal interpretation of a P value, based person who would not otherwise have been on a significance level of about 10%, might be: harmed

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PEARLS AND PITFALLS • Bias is a systematic error in the design study that can decrease the ability to find a relationship between the exposure and the outcome of interest. • A randomized controlled study is the optimal way to evaluate the effect of 2 different treatments for a disease. • Statistical significance does not imply clinical significance. • A good screening test is a rapid, inexpensive assay and has a good sensitivity (i.e., a negative test is reliable). • Having a larger sample size increases the power of a study. • Standard deviation is a measure of the variability of individual values around the mean or average value. • A confidence interval is a range of values with a specified probability that a given parameter falls in that range.

Suggested Reading Chou R, Aronson N, Atkins D, Ismaila AS, Santaguida P, Smith DH, et  al. AHRQ series

S. O. Kukoyi-Maiyegun

paper 4: assessing harms when comparing medical interventions: AHRQ and the effective health-care program. J Clin Epidemiol. 2010;63(5):502–12. Copeland-Linder N.  Research and statistics: reliability and validity in pediatric practice. Pediatr Rev. 2009;30(7):278–9. Ferrante di Ruffano L, Hyde CJ, McCaffery KJ, Bossuyt PM, Deeks JJ. Assessing the value of diagnostic tests: a framework for designing and evaluating trials. BMJ. 2012;344:e686. Hernandez RG, Rowe PC. Research and statistics: cohort studies. Pediatr Rev. 2009;30(9):364–5. Moore EM, Johnson SB. Research and statistics: case reports, anecdotal evidence, and descriptive epidemiologic studies in pediatric practice. Pediatr Rev. 2009;30(8):323–4. Norris S, Atkins D, Bruening W, Fox S, Johnson E, Kane R, et al. Selecting observational studies for comparing medical interventions. In: Methods guide for effectiveness and comparative effectiveness reviews [Internet]. Rockville: Agency for Healthcare Research and Quality; 2010. Palaia A. Research and statistics: study design and data sources. Pediatr Rev. 2013;34(8):371–2. Perry-Parrish C, Dodge R. Research and statistics: validity hierarchy for study design and study type. Pediatr Rev. 2010;31(1):27–9.

Patient Safety and Quality Improvement

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Osama I. Naga

Nonpreventable medical errors • Example: Patient with no history of allergic reaction to penicillin develops a severe aller• A medical error is an act that has the potential gic reaction to amoxicillin. to cause patient harm, regardless of whether or not harm reaches the patient. Causes of medical errors • Inadequate or poor communication Preventable adverse event • Insufficient information flow • A medical error that results in harm to the • Human issues patient • Patient issues • Workflow and staffing issues Potential adverse events or near misses • Technical breakdowns/failures • Medical errors that do not cause harm to the • Policies and procedures that are inadequate patient

MEDICAL ERRORS

Types of potential adverse events or near How to address medical error • Either the medical error caused harm or not, misses: the physician must analyze the event to pre• Intercepted: An error that is recognized and vent its reoccurrence and to improve the corrected before it reaches the patient safety system. • Nonintercepted: An error that reaches the • All providers must be responsible for the patient but does not result in harm quality of patient care and safety. Preventable medical errors Example: A nurse administered the incorrect • Examples: medication. –– Failure to provide preventive treatment. • Investigate the flaw or problem within the –– Failure to provide both the hepatitis B vacsystem that allowed or facilitated the cine and hepatitis B immunoglobulin to an mistake. infant born to an HBsAg-positive mother • Try to understand how and why the event within 12 h. occurred, instead of focusing on just blaming –– Patient who is allergic to penicillin is preor punishing the nurse. scribed amoxicillin and develops a skin • Changing how medications are stored, rash after drug administration. labeled, or packaged may prevent the error O. I. Naga (*) from occurring again. Department of Pediatrics, Paul L. Foster School of Medicine, • This course of action does not negate the Texas Tech University Health Sciences Center, El Paso, TX, USA e-mail: [email protected] nurse’s personal responsibility. © Springer Nature Switzerland AG 2020 O. I. Naga (ed.), Pediatric Board Study Guide, https://doi.org/10.1007/978-3-030-21267-4_30

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Sentinel Event

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• Incorrect documentation/data • Knowledge deficit • Performance error and poor communication

• A sentinel event is an unexpected occurrence of death or serious physical or psychological Significance injury. • Harm may or may not reach the patient (e.g., • Near miss sentinel event: If the risk and mistake caught early by physician, parents, or potential consequences of a recurrence may pharmacist). lead to a serious adverse outcome. • The severity of harm varies from minimal to • A sentinel event may or may not be due to a serious adverse effects or even death. medical error.

Treatment Errors

Prevention • Avoid abbreviations of drug names (e.g., “MS” may mean “morphine sulfate” or “magnesium sulfate”). • Confirm that the patient’s weight is correct for weight-­based dosages. • Ensure that the weight-based dose does not exceed the recommended adult dose • Identify drug allergies in patients. • Write out instructions rather than using abbreviations, e.g., “Take 1 tablet twice a day” rather than “bid.” Avoid vague instructions such as “Take as directed.” • The concentration of the medication and the frequency of administration should be clearly noted on prescriptions. • Avoid use of a terminal zero to the right of the decimal point (e.g., use 5 rather than 5.0) to minimize 10-fold dosing errors. • Use a zero to the left of a dose less than 1 (e.g., use 0.1 rather than 0.1) to avoid 10-fold dosing errors. • Spell out dosage units rather than using abbreviations (e.g., milligram or microgram rather than mg or μg; units rather than “u”). • The pharmacist and nurse must verify all medication orders. • When giving a verbal order, request that the name of the medication and directions for use be read back.

Causes of medication errors • Calculation error • Dosage form confusion • Illegible handwriting

Decrease the risk of medication administration errors by parents • Liquid medications should be dosed in milliliters, not teaspoons or tablespoons.

Root Cause Analysis (RCA) Process • A process for identifying the primary or causal factors that underlie variation in performance, including the occurrence or possible occurrence of a sentinel event. • Once a sentinel event has been identified, investigation must be immediately undertaken to determine the root causes that have led to the event as well as to implement an action plan to monitor for and to minimize any future risk that this event will recur.

Diagnostic Errors • Diagnostic errors in the form of missed or incorrect diagnosis account for most pediatric malpractice cases. • The most common diseases associated with diagnostic errors: pneumonia, meningitis, appendicitis, and testicular torsion. • Most pediatricians have at least 1 diagnostic error per month, e.g., misdiagnosis of viral pharyngitis as acute streptococcal pharyngitis— a viral illness diagnosed as a bacterial illness.

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• Medications should be dosed to the nearest Voluntary error reporting • Errors reported, with little or no patient harm, 0.1, 0.5, or 1 mL. can provide information critical to improving • Appropriate-volume milliliter-based dosing patient safety. devices should be distributed with the medication. Barriers to voluntary reporting –– Syringes are the preferred dosing device. • Fear of punitive action –– Measuring cups and spoons calibrated and • Lack of feedback marked in milliliters are acceptable • The incident report takes a long time to alternatives. complete • Advanced counseling strategies to ensure • Lack of physician access to electronic inciparental understanding and adequate health dent reporting systems literacy and numeracy should be offered. • Interruption of patient care to complete an incident report Role of the institution in reducing medication errors • Provide education and training to hospital Factors that may enhance voluntary reporting • Education about which errors should be staff. reported, even if trivial or causing no harm • Computerized systems to check dose and • Encouraging a nonpunitive culture for reportdosage schedules, drug interactions, allergies, ing and reviewing adverse events and duplicate therapies. • Feedback on a regular basis about errors • Standardize equipment (such as infusion reported and about individual events pumps and weight scales) throughout the • Evidence that reporting of errors has successinstitution. fully led to system changes • Standardize measurement systems through• Electronic format for reports out the institution, for example, using only • Protecting the confidentiality of the author of kilograms for weight, rather than using the incident report pounds in 1 department and kilograms in another. • Avoid use of verbal orders whenever possible. Mandatory error reporting • Example of mandatory reporting • Have a pharmacist participate in daily clinical –– Suspected child physical or sexual abuse rounds. –– Deaths not related to anticipated disease • Adjust for look-alike and sound-alike progression medications. –– Medical equipment malfunction or misuse • Remove high-risk medications. leading to serious patient harm or death –– Discharge of a patient incapable of making medical decisions to an unauthorized REPORTING MEDICAL ERRORS person • Reporting medical error is paramount to Medical error disclosure establishing a system capable of preventing • Once a medical error has been detected, it future mistakes and improving the safety of must be disclosed to the patient and care. family. • Both voluntary and mandatory reporting sys• Providers should consider offering an apoltems will help to prevent the recurrence of a ogy, and disclosing medical errors. similar event.

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Policies

The number of staff is not appropriate for the number of patients and the size of the practice.

The fax machine is not receiving the lab and imaging study reports

Procedure

Incorrect patient demographic information

The computers are far from the exam rooms The Internet is slow

The staff is new and not well trained The staff burnout

Measurement

Plant, technology

People

The rooms are far from each other, and the communications between staff and physicians are poor

Long waiting time in a pediatric office

Environment

Fig. 30.1  A fishbone diagram as used in root cause analysis can help brainstorming by team members to identify the root cause of long wait times in a busy pediatric practice

• Patients desire and deserve disclosure of errors that have caused them harm. • Disclosure of medical errors by physicians helps build trust in the clinician. • Frank disclosure of medical errors by physicians decreases the likelihood of parents seeking legal action.

• Root cause analysis to identify problems and their solutions. • For each cause found, the team should continue to “drill down” by repeatedly asking “Why is that?” until the root causes are identified. • The fishbone diagram as used in root cause analysis can help brainstorming by team members to identify the root causes of problems, leading to improvement in the quality of care (Fig. 30.1).

Recommendations • Ensure that patient and family remain at the center of communication. • Communicate clearly about how the medical error occurred and what will be done to prevent its recurring in the future. • Support personnel to answer the family’s PATIENT SAFETY questions.  andoffs • Promptly share with the family any new H information obtained from investigation. • A handoff takes place when a patient or a • Address all patient and family concerns as patient’s medical information is transferred soon as possible. from one provider to another and/or from one healthcare venue to another. • The resident duty hour restrictions implemented several years ago have increased the QUALITY IMPROVEMENT (QI) frequency of patient handoffs. • To cultivate an effective team, encourage • The use of a mnemonic is recommended to transparency, collaboration, teamwork, and structure patient handoffs. This will decrease learning from mistakes. the risk of miscommunication and help

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ensure that all important elements are N  ever Events included. For example: I-PASS: Illness severity, Patient summary, Action list, Situation • Serious reportable hospital events that should awareness and contingency plans, and never occur Synthesis by receiver. • Serious—resulting in death or significant • Methods of communications: disability –– Printed patient summary documents (sign• Should not occur: out documents) –– Clearly identifiable and measurable –– Verbal communication –– Usually preventable • Use of both a sign-out document and verbal Examples of never events communication together are recommended. • Surgical event • Sign-out and verbal documents generally –– A surgical procedure performed on the remain accurate for only a short period wrong body part (patient’s clinical condition may change in • Product or device event 6 h or less). –– Contaminated drug or instrument provided • The ideal method of handoff is using an elecby the healthcare setting tronic summary that is continuously updated • Patient protection event to reflect clinical changes. –– An adolescent with suicide attempts or self-harm, resulting in serious disability, while being cared for in a healthcare Fostering a Culture of Safety facility • Care management event • Encourage communication and teamwork to –– Patient death or serious injury associated meet high patient safety standards. with a medication error (e.g., errors involv• Provide an open, fair, and transparent enviing the wrong drug, wrong dose, wrong ronment for the disclosure of medical errors patient, wrong time, wrong rate, wrong to better identify means to decrease or elimipreparation, or wrong route of nate them in the future. administration) Examples of fostering a culture of safety • Environmental event • Use at least 2 patient identifiers when provid–– Patient’s death due to an oxygen line ing care, treatment, and services: designed to deliver oxygen to the patient –– Check the patient’s name, hospital number, containing no oxygen and date of birth on both the wristband and • Criminal event hospital chart before obtaining blood or –– Abduction of a newborn from the newborn administering medicine. nursery • Report critical results of tests and diagnostic procedures on a timely basis. • Maintain and communicate accurate patient PEARLS AND PITFALLS medication information: –– Obtain a list of patient medications on • Punishing the provider for medical mistakes admission to a healthcare facility. is not the ultimate solution; it is merely a tem• Identify patients at risk for suicide. porary fix. Addressing the real cause and –– Conduct a risk assessment to identify implementing the real solution will prevent patients at risk for suicide. the same mistake for reoccurring.

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• Near miss: If the potential harm from a medi- Haig KM, Sutton S, Whittington J. National patient safety goals. SBAR: a shared mental model for cal error does not reach the patient. improving communication between clinicians. • Sentinel event: Medical error leading to a Jt Comm J Qual Patient Saf. 2006;32(3):167–75. patient’s unexpected death or serious physiInstitute of Medicine (US) Committee on Quality cal or psychological injury. of Health Care in America. Crossing the quality chasm: a new health system for the 21st century. Suggested Reading Washington, DC: National Academies Press (US); 2001. https://www.ncbi.nlm.nih.gov/ Bartman T, McClead RE.  Principles of quality books/NBK222274/. Accessed 20 Dec 2018. improvement and patient safety. Pediatr Rev. McClead RE, Brady M.  Sentinel events/patient 2016;37(10):407–17. safety events. Pediatr Rev. 2016;37(10):448–50. Crowley E, Williams R, Cousins D.  Medication Pereira-Argenziano L, Levy FH. Patient safety and errors in children: a descriptive summary quality improvement: terminology. Pediatr Rev. of medication error reports submitted to the 2015;36(9):403–11. United States Pharmacopeia. Curr Ther Res. 2001;62(9):627–40.

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GENERAL PEDIATRIC PHARMACOLOGY Absorption (Table 31.1) • Absorption is the process by which a drug enters the bloodstream or another body compartment from the site of administration. • Drugs administered via the intravenous (IV) route are 100% bioavailable, meaning that the entire drug dose has reached the circulation. • Due to diminished intestinal motility and delayed gastric emptying in neonates and infants, it takes longer for a drug to reach a similar plasma concentration as in older children after oral administration.

Bioavailability • Bioavailability is defined as the rate and extent to which the active drug is absorbed and becomes available at the site of drug action to produce a pharmacologic response

Table 31.1  Absorption of drugs Drug Acetaminophen Tetracycline, doxycycline, minocycline Antihistamines, e.g., cetirizine Iron

Levothyroxine Griseofulvin NSAIDs, e.g., ibuprofen

Drug-food interaction Food may delay absorption Milk and dairy products affect the absorption Food may delay the absorption Acidic juices may enhance the iron absorption Antacids interfere with iron absorption Grapefruit, soybeans, and soy milk may interfere with absorption Should be taken with whole milk or other food containing fat for optimum bioavailability Food or milk may prevent stomach irritation

NSAID Nonsteroidal anti-inflammatory drugs

Hepatic Metabolism • The cytochrome P450 (CYP) isoenzymes in the liver, primarily 3A4, 2D6, 1A2, 2C9, and 2C19, are responsible for the oxidation of 75% of all drugs (Table 31.2)

O. I. Naga (*) Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 O. I. Naga (ed.), Pediatric Board Study Guide, https://doi.org/10.1007/978-3-030-21267-4_31

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Table 31.2  Cytochrome P450 (CYP) enzyme inhibitors and inducers and potential interactions Cytochrome P450 (CYP) enzyme inhibitors Amiodarone, cimetidine, fluconazole, fluoxetine, metronidazole, trimethoprim/ sulfamethoxazole, diphenhydramine, clarithromycin, itraconazole, erythromycin Cytochrome P450 (CYP) enzyme inducers Carbamazepine, phenobarbital, rifampin, glucocorticoids

Potential interactions May increase the risk of toxicity when combined with certain drugs, e.g., methotrexate, tacrolimus Potential interactions Decrease the activity of certain drugs, e.g., warfarin, digoxin, and oral contraceptives

Drug-Drug Interactions

• Patients with lower muscle mass (e.g., neonates, females, malnourished patients). • Patients with vomiting and diarrhea, or dehydrated, and at the same time using diuretics may develop increased serum drug concentrations with routine dosing and potential attainment of a toxic level. • The dosing interval of renally eliminated drugs, e.g., gentamicin or vancomycin, may need to be prolonged when coadministered with ibuprofen or indomethacin or in neonates with a history of birth hypoxia/asphyxia or cyanotic congenital heart disease. • A postoperative cardiac patient who has poor renal perfusion and who requires antibiotics for concerns of sepsis may require a less frequent dosing interval for vancomycin, an antibiotic that is cleared renally.

• Pediatricians should screen routinely for potential drug-­drug, drug-herbal product, or drug-food interactions before prescribing a Methods of dose adjustment clinically new medication, e.g.: • Trough level (Fig. 31.1) –– Beta-blocker prescribed for hypertension –– The lowest concentration reached by a or migraines inhibits the activity of a beta drug before the next dose is administered. agonist given to treat an asthma –– Trough routinely used for certain drug monexacerbation itoring, e.g., gentamicin, or vancomycin. –– The effect of a broad-spectrum antibiotic –– Trough concentrations are obtained at the on warfarin by eradicating the gut flora end of a dosing interval just before the next needed to metabolize the anticoagulant dose is administered to verify that the –– Increased potential toxicity of phenytoin drug’s concentration is still in the therawhen combined with sulfa drugs, e.g., tripeutic range and to prevent drug toxicity. methoprim/sulfamethoxazole –– If the first trough level obtained is acceptable, repeat the trough levels at 4–6  days into therapy to ensure nontoxic level. Renal Elimination –– If the trough level is higher than acceptable, the drug should not be given, and Examples of drugs predominantly eliminated another level checked 6 h later. This should by the kidneys: be repeated as needed until a safe level is • NSAIDs, angiotensin-converting enzyme obtained. inhibitors, angiotensin II receptor blockers, • Peak level (see Fig. 31.1) aminoglycosides, sulfamethoxazole/trime–– The highest concentration reached by a thoprim, vancomycin, ciprofloxacin, antividrug after the dose is administered rals, amphotericin B –– Peak level should be obtained 30 min after the infusion of gentamicin or vancomycin Dose adjustment of renally eliminated medica–– Peak levels are not necessary for cases tions, e.g.: being treated with a course of antibiotics • Patients with primary pathologic kidney without a positive culture disease.

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Fig. 31.1  Trough, peak, and steady-state levels of a drug curve

Peak after the dose is administered Steady state level

Trough before the next dose

Drug dosing intervals

–– If a blood culture is positive and an organ- Table 31.3  Examples of t½ and drug dosing intervals ism and sensitivities are identified, both Drug Half-life (t½) Drug-dosing intervals  2 months) with meningitis in combination ically absorbed) with third-generation cephalosporin • S. aureus infections, including MRSA • MRSA infection • Prophylaxis before prosthetic device implanSummary of antibiotics and adverse reactions tation requiring major surgery (Table 31.4). • Enterally for C. difficile • Acute infectious endocarditis if S. aureus is the likely cause Table 31.4  Antibiotics and common adverse effects Commonly used antibiotics Penicillins, e.g., amoxicillin, amoxicillin/clavulanic acid (Augmentin; GSK), piperacillin/ tazobactam (Zosyn; Pfizer)

Common adverse effects Non-IgE-mediated: vomiting, diarrhea, headache, or a non-urticarial, nonpruritic rash IgE-mediated type1 hypersensitivity: urticarial rash, angioedema, anaphylaxis (0.3–3%) Nausea, vomiting, diarrhea, rash, AST and ALT elevation, allergic reaction.

Cephalosporins, e.g., cephalexin, cefazolin, cefuroxime, cefdinir, cefixime, ceftriaxone Aminoglycosides, e.g., Nephrotoxicity/ototoxicity gentamicin, tobramycin Glycopeptides, e.g., vancomycin Red man syndrome; pruritic erythematous rash, and flushing Nausea, vomiting, diarrhea, anorexia, Antituberculosis drugs, e.g., hemolytic anemia, liver toxicity, rifampin, isoniazid, peripheral neuropathy pyrazinamide, ethambutol Orange body fluids (rifampin) Macrolides, e.g., erythromycin, Nausea, vomiting, abdominal pain, azithromycin, clarithromycin diarrhea, anorexia, taste changes (clarithromycin) Nausea/vomiting, diarrhea, anorexia, Sulfonamides, e.g., trimethoprim-­sulfamethoxazole, abdominal pain, rash, photosensitivity, headache, dizziness sulfadiazine (Bactrim; Roche) Tetracyclines, e.g., tetracycline, Nausea, vomiting, diarrhea, anorexia, doxycycline, minocycline abdominal pain, photosensitivity, tooth discoloration in children   14  mm) with thickened

Fig. 32.22

965

hypoechoic muscular layer (solid line, >  3  mm). Note the fluid within the gastric antrum (arrow) and lack of fluid in the duodenal bulb (arrowhead). No gastric contents were seen traversing the pylorus during the ultrasound exam. Diagnosis:  Hypertrophic pyloric stenosis.

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Case 23 14-year-old male presenting with nausea, vomiting, and periumbilical pain that migrated to the right lower quadrant, with laboratory results revealing a white blood cell count of 18,000 Imaging findings (Fig.  32.23): Focused right lower quadrant ultrasound image demonstrates a dilated (> 6 mm), tubular, and thickened appendix

Fig. 32.23

W. G. Kim and M. George

(arrows). An echogenic shadowing structure is seen within the lumen of the appendix (∗), most in keeping with an appendicolith. Note the fluid and echogenic fat surrounding the appendix, denoting periappendiceal edema. Diagnosis:  Acute appendicitis.

32  Radiology Review

Case 24 4-day-old male presenting with bilious emesis, abdominal distension, and feeding intolerance Imaging findings (Fig. 32.24): Upper GI fluoroscopic image of the abdomen during administration of water-­soluble enteric contrast demonstrates an abnormal corkscrew appearance of the proximal duodenum (arrow), which does not cross midline.

Fig. 32.24

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Diagnosis:  Malrotation with volvulus. The second and fourth segments of the duodenum should course retroperitoneally, and the duodenojejunal junction should be located at the same level of the duodenal bulb at approximately the L1 vertebral body on the left.

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Case 25 2-year-old male with recent history of viral upper respiratory tract infection presenting with intermittent bouts of severe abdominal pain and lethargy, with a palpable tubular mass in the upper abdomen on examination Imaging findings (Fig. 32.25): Frontal abdominal radiograph (a) demonstrates a rounded soft tis-

a

sue filling defect within the mid transverse colon (arrow). Right lower quadrant ultrasound images (b, c) reveal a targetoid appearance of multiple alternating layers of bowel wall (arrows) and interposed mesenteric fat (∗), lymph nodes (∗), and fluid (∗). Diagnosis:  Ileocolic intussusception.

b

c

Fig. 32.25

32  Radiology Review

Case 26 Newborn infant with trisomy 21 and history of polyhydramnios, presenting with bilious emesis Imaging findings (Fig.  32.26): Radiograph of the abdomen demonstrates a “double-bubble”

Fig. 32.26

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appearance of gaseous distension of the stomach (arrow) and duodenal bulb (arrowhead) with absence of gas distally. Diagnosis:  Duodenal atresia.

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Case 27 12-day-old infant with a diagnosis of 2q22.1 chromosomal deletion with multiple congenital abnormalities, presenting with abdominal distension Imaging findings (Fig.  32.27): Abdominal radiograph (a) demonstrates gas distension of the distal colon in the left hemiabdomen (arrow). Fluoroscopic contrast enema (b) demonstrates a

a

Fig. 32.27

b

small caliber rectum with a transition point (arrow) at the rectosigmoid junction and dilatation of the colon proximally. Normally, the rectum should be larger in caliber than the sigmoid colon (rectosigmoid ratio > 1). Diagnosis:  Hirschsprung disease.

32  Radiology Review

Case 28 9-month-old male with a non-reducible left-sided scrotal mass Imaging findings (Fig.  32.28): Ultrasound image of the left scrotum shows a structure extending from the inguinal canal adjacent to the sper-

Fig. 32.28

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matic cord. This structure demonstrates walls with alternating hypoechoic and hyperechoic layers (arrow), indicative of gut signature. Note the small adjacent hydrocele (arrowhead). Diagnosis:  Incarcerated inguinal hernia.

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Case 29 2-week-old infant presenting with jaundice and conjugated hyperbilirubinemia Imaging findings (Fig.  32.29): Hepatobiliary iminodiacetic acid scintigraphy (HIDA scan) shows uptake of radiotracer into the liver but no

excretion into the small bowel. This was confirmed on 24-h delayed imaging. No gallbladder was identified. Diagnosis:  Biliary atresia.

Study name: Hepatobiliary Anterior %

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Fig. 32.29

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Case 30 10-year-old male presenting with lower gastrointestinal bleeding Imaging findings (Fig.  32.30): Dynamic abdominal scintigraphy with technetium-99m-labeled sodium pertechnetate demonstrates an

intense focus of persistent abnormal tracer activity in the right lower quadrant (arrow). There is normal uptake of tracer by the stomach. Diagnosis:  Functioning ectopic gastric mucosa (Meckel’s diverticulum).

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Case 31 2-year-old child presenting with throat pain Imaging findings (Fig. 32.31): Frontal (a) and lateral (b) radiographs of the chest show a discoid metallic foreign body projecting over the upper esophagus at the level of the aortic arch. The double rim and slight step off along the edges of the

a

Fig. 32.31

W. G. Kim and M. George

object distinguish this from a simple coin. Ingested foreign bodies in the esophagus are most commonly seen at the levels of the thoracic inlet, the aortic arch, and gastroesophageal junction. Diagnosis:  Button battery ingestion.

b

32  Radiology Review

Case 32 2-year-old with history of gastroschisis, presenting with abdominal pain and distention Imaging findings (Fig. 32.32): Frontal and lateral decubitus radiographs (a, b) of the abdomen demonstrate markedly dilated loops of small bowel a

Fig. 32.32

975

with multiple air-fluid levels (arrows). There is also a paucity of gas in the sigmoid colon and rectum (∗). Diagnosis:  Small bowel obstruction.

b

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Case 33 12-year-old male with several weeks of intermittent abdominal pain and diarrhea Imaging findings (Fig. 32.33): MR enterography was performed. Gadolinium contrast-enhanced coronal (a) and axial (b) T1-weighted, fat-saturated MR images show enhancement and thickena

Fig. 32.33

W. G. Kim and M. George

ing of a long segment of the terminal ileum in the right lower quadrant (arrows). Note also the engorgement of the mesenteric vessels (arrowheads). Diagnosis:  Crohn’s disease with terminal ileitis.

b

32  Radiology Review

Case 34 3-year-old male presenting with abdominal pain and failure to thrive, with a palpable left upper quadrant mass on examination and an abnormal abdominal ultrasound which prompted further evaluation with CT

Fig. 32.34

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Imaging findings (Fig.  32.34): Coronal contrast-enhanced CT of the abdomen demonstrates a large, round, and heterogeneously enhancing left suprarenal mass (∗). The left kidney is displaced inferiorly (arrow). Diagnosis:  Neuroblastoma.

978

GENITOURINARY RADIOLOGY Case 35 1-month-old infant presenting with discharge from the umbilicus Imaging findings (Fig.  32.35): Sagittal ultrasound of the midline lower abdomen demonstrates a fluid-filled tract (arrows) extending from the dome of the bladder to the umbilicus. Thickening of the soft tissues surrounding the umbilicus suggests inflammation. Diagnosis:  Infected patent urachus.

Fig. 32.35

W. G. Kim and M. George

Case 36 Newborn male infant with prenatal diagnosis of hydronephrosis Imaging findings (Fig.  32.36): Fluoroscopic cyclic voiding cystourethrogram was performed. An oblique fluoroscopic image of the pelvis during voiding demonstrates abnormal dilatation and elongation of the posterior urethra (arrow) proximal to a thin radiolucent band of tissue (arrowhead). Note also the trabecular appearance of the bladder (∗) due to muscular hypertrophy. Diagnosis:  Posterior urethral valve causing bladder outlet obstruction.

Fig. 32.36

32  Radiology Review

Case 37 16-month-old male with febrile urinary tract infection Imaging finding (Fig.  32.37): Fluoroscopic voiding cystourethrography demonstrates abnormal reflux of contrast into dilated renal collecting systems bilaterally. There is mild distension and

Fig. 32.37

979

blunting of the renal calyces in the right kidney and moderate distention of the renal pelvis and calyces in the left kidney. The left ureter is also dilated and tortuous. Diagnosis:  Left grade IV and right grade III vesicoureteral reflux.

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Case 38 1-week-old infant with history of oligohydramnios, presenting with hypertension and bulging flanks on examination Imaging findings (Fig.  32.38): Ultrasound images of the left and right kidneys (a) demonstrate markedly enlarged, echogenic kidneys with diffuse, innumerable small cystic lesions, and loss

a

Fig. 32.38

b

of corticomedullary differentiation. Coronal T2-weighted, fat-saturated MR image (b) shows the extent to which the kidneys occupy a majority of the abdomen. Diagnosis:  Autosomal recessive polycystic kidney disease.

32  Radiology Review

Case 39 3-year-old male with hemihypertrophy presenting with a palpable abdominal mass on examination and an abnormal abdominal ultrasound which prompted further evaluation with contrastenhanced CT Imaging findings (Fig.  32.39): Coronal contrast-enhanced CT of the abdomen demonstrates a large, heterogeneous, rounded mass centered at the superior pole of the left kidney (∗). Note the thin rim of renal parenchyma inferiorly (arrows) stretching around the mass.

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Case 40 Newborn infant with prenatal diagnosis of a left adrenal mass Imaging findings (Fig.  32.40): Ultrasound image (a) demonstrates a left suprarenal mass with complex cystic features and multiple septations (arrows). Multiple follow-up ultrasounds were obtained, which showed sequential decrease in size. Follow-up imaging at 9  months of age (b) demonstrates complete resolution of the mass. Diagnosis:  Adrenal hemorrhage.

Diagnosis:  Wilms tumor.

a

b

Fig. 32.39

Fig. 32.40

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Case 41 15-year-old premenarchal female presenting with recurrent pelvic pain Imaging findings (Fig.  32.41): Sagittal ultrasound image of the pelvis demonstrates a markedly distended endometrial (∗) and cervical (arrowhead) canal with homogeneous echogenic material.

W. G. Kim and M. George

Case 42 15-year-old female presenting with acute-onset right-­sided pelvic pain and vomiting Imaging findings (Fig.  32.42): Transverse ultrasound image of the pelvis (a) demonstrates an asymmetrically enlarged and heterogeneously hypoechoic right ovary (arrow). Sagittal image of the right ovary (b) shows peripheralized follicles (arrows) and lack of color Doppler flow.

Diagnosis:  Hematometrocolpos, in this case secondary to an imperforate hymen. Diagnosis:  Right ovarian torsion.

a

b

Fig. 32.41

Fig. 32.42

32  Radiology Review

Case 43 12-year-old male presenting with right-sided testicular pain for 4 h Imaging findings (Fig.  32.43): Transverse ultrasound image of the scrotum (a) demonstrates asymmetrically diminished Doppler flow to the right testis (∗). Sagittal ultrasound image of the right scrotum (b) demonstrates an abnormal contour of the spermatic cord and epididymal head (arrow), indicative of a bell clapper deformity. There is a small associated hydrocele.

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Case 44 19-month-old male presenting with hematuria Imaging findings (Fig.  32.44): Transverse ultrasound image of the bladder (a) demonstrates an intraluminal, exophytic, lobular mass near the base of the bladder. There is color flow within this mass on Doppler ultrasound (b), indicative of vascularity. Diagnosis:  Rhabdomyosarcoma of the bladder.

Diagnosis:  Acute right testicular torsion. a

a

b

b

Fig. 32.43

Fig. 32.44

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Case 45 16-year-old sexually active female presenting with pelvic pain, fever, and cervical motion tenderness Imaging findings (Fig.  32.45): Ultrasound image of the pelvis (a) demonstrates a complex cystic mass in the left adnexal region (arrow). The surrounding mesenteric fat is echogenic, suggestive of adjacent inflammation. Note the normal right ovary (∗). Post-gadolinium enhanced axial a

Fig. 32.45

b

T1-weighted MRI of the pelvis (b) demonstrates a complex, rim enhancing tubular structure (arrow) in the left hemipelvis, with extensive adjacent enhancement. Diagnosis:  Tubo-ovarian abscess.

32  Radiology Review

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oblique lucency through the tibial metaphysis (arrows). On the lateral view, there is clear widening of the physis anteriorly (arrowhead). No epiphyseal component is appreciated.

Case 46 12-year-old male with ankle pain after a basketDiagnosis:  Salter Harris II fracture. ball injury Imaging findings (Fig. 32.46): Frontal and lateral radiographs of the ankle demonstrate an

Fig. 32.46

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Case 47 15-month-old female presenting to the emergency room with fussiness, with multiple bruises noted on physical examination Imaging findings (Fig.  32.47): Frontal radiograph (a) of the right wrist demonstrates triangular bone fragments along the lateral aspect of the distal radial metaphysis and medial aspect of the distal ulnar metaphysis (arrows), in keeping with corner fractures. Lateral radiograph (b) shows the

a

Fig. 32.47

b

radial fracture extending to both the anterior and posterior corners of the distal radius (arrowheads), which has a ­ “bucket-­ handle” appearance. This patient had similar appearing fractures in the tibia as well. Towne view of the skull (c) in the same patient shows a left occipital fracture. Diagnosis:  Non-accidental trauma with classic metaphyseal lesions and skull fracture.

c

32  Radiology Review

Case 48 4-year-old female with a history of multiple fractures Imaging findings (Fig.  32.48): Frontal radiograph of the hips and lower extremities demonstrates numerous healed fractures of the right iliac

Fig. 32.48

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wing, femoral shafts, and distal tibial diaphysis bilaterally (arrows). The bones are diffusely demineralized with thinning of the cortex. Numerous growth arrest lines are present (∗). Diagnosis:  Osteogenesis imperfecta.

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Case 49 17-month-old female presenting to the emergency room with fevers and right knee warmth Imaging findings (Fig.  32.49): Frontal radiograph of the right knee (a) demonstrates a subtle lucency in the medial distal femoral metaphysis (arrow). T1 weighted coronal MRI (b) of the right

a

Fig. 32.49

b

knee demonstrates loss of normal fatty signal in the same region (arrow). This is accompanied by high signal on the T2-weighted MRI (c), indicative of marrow edema (arrow). Additional edema is noted in the overlying soft tissues and medial epiphysis (arrowheads). Diagnosis:  Acute osteomyelitis.

c

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ping, and expansion of the distal radial and ulnar Case 50 2-year-old male with history of chronic renal dis- metaphyses bilaterally (arrows). ease presenting with weakening grip Imaging findings (Fig.  32.50): Frontal radio- Diagnosis:  Rickets. graphs of the wrists demonstrates fraying, cup-

Fig. 32.50

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Case 51 7-year-old male with worsening, gradual right thigh pain Imaging findings (Fig.  32.51): Frontal radiograph of the right thigh (a) demonstrates a permeative, moth-eaten diaphyseal bone lesion (arrow) with poorly defined margins, aggressive appearing periosteal reaction along the lateral cortex

a

Fig. 32.51

b

­(arrowhead) and large soft tissue component (∗). No internal matrix is appreciable. Axial, contrastenhanced MRI (b) demonstrates a large surrounding soft tissue mass with robust enhancement (arrows). Diagnosis:  Ewing sarcoma.

32  Radiology Review

Case 52 8-year-old male with worsening right knee pain and no reported trauma Imaging findings (Fig. 32.52): Frontal and lateral radiographs of the distal left femur demonstrate an aggressive, permeative lesion with a wide zone of transition between normal and abnormal marrow. There is an aggressive, hair-­on-­end peri-

Fig. 32.52

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osteal reaction along the posteromedial metaphysis of the distal femur (arrow), and the periosteum has been lifted from the bone (Codman’s triangle, arrowhead). Dense, amorphous calcific matrix is present (∗) in the lesion. Diagnosis:  Osteosarcoma.

992

Case 53 11-year-old female with painless, firm, palpable lump of the left lower thigh Imaging findings (Fig.  32.53): Frontal radiograph of the left knee demonstrates a protuberant bone lesion arising from the medial metaphysis of

Fig. 32.53

W. G. Kim and M. George

the distal femur. The lesion is oriented away from the joint space and is in continuity with the bone. There is no soft tissue component or periosteal reaction. Diagnosis:  Osteochondroma.

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Case 54 17-year-old male with worsening left ankle pain and no antecedent trauma Imaging findings (Fig.  32.54): Frontal radiograph (a) of the left ankle demonstrates an expansile, lucent lesion of the distal fibula (∗). There is a narrow zone of transition proximally (arrowhead).

a

Fig. 32.54

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No soft tissue component or periosteal reaction is present. Sagittal, fluid sensitive MRI (b) of the distal left fibula demonstrates numerous fluid–fluid levels within the lesion (arrow). Diagnosis:  Aneurysmal bone cyst.

b

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Case 55 4-year-old female with short stature Imaging findings (Fig.  32.55): Frontal radiograph of the abdomen and pelvis (a) demonstrates narrowing rather than widening of the interpeduncular distances of the lower lumbar spine (arrowheads). There iliac wings are broad (arrow) and

a

Fig. 32.55

b

the pelvis narrows sharply (∗). Frontal radiograph of the hand (b) demonstrates short, broad metacarpals with irregular metaphyses (arrow) and splaying of the digits. Diagnosis:  Achondroplasia.

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Case 56 8-year-old male with fall on an outstretched hand Imaging findings (Fig.  32.56): Lateral radiograph of the elbow demonstrates linear lucency through the anterior cortex of the distal humerus (arrow). The posterior fat pad is displaced from the olecranon fossa (arrowhead), indicative of joint effusion.

995

Case 57 11-day-old infant with torticollis to the left Imaging findings (Fig. 32.57): High frequency gray-scale ultrasound of the right and left neck demonstrates asymmetric enlargement of the right sternocleidomastoid muscle (∗). Diagnosis:  Fibromatosis colli.

Diagnosis:  Supracondylar fracture.

Fig. 32.56

Fig. 32.57

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Case 58 9-year-old male with a fall on an outstretched hand Imaging findings (Fig.  32.58): Frontal radiograph of the elbow demonstrates abnormal medial and inferior positioning of the medial epicondyle (arrow). There is subcutaneous edema over the medial aspect of the elbow (∗). The lateral epicondyle (arrowhead) is also abnormally laterally positioned.

W. G. Kim and M. George

Case 59 16-year-old male who complains of right hip pain after sustaining an injury while playing hockey and is unable to flex the right hip on examination Imaging findings (Fig.  32.59): Frontal radiograph of the pelvis demonstrates a small fragment adjacent to the right anterior superior iliac spine (arrow).

Diagnosis:  Apophyseal avulsion fracture of the right anterior superior iliac spine, at the attachDiagnosis:  Avulsion of the medial and lateral epi- ment of the sartorius tendon. condyles of the elbow.

Fig. 32.59 Fig. 32.58

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NEURORADIOLOGY

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(∗) predominantly within the right lateral ventricle. There is also abnormal echogenicity of the right frontal periventricular white matter (arrow), Case 60 1-day-old infant born at 25 weeks gestational age indicative of parenchymal hemorrhage with evolvpresenting with seizures and drop in hematocrit ing leukomalacia. Notice also the diffusely echogenic ependymal lining, suggestive of chemical level Imaging findings (Fig. 32.60): Anterior fonta- ventriculitis. nel approach coronal gray-scale ultrasound image IV intraventricular of the brain demonstrates enlarged lateral and third Diagnosis:  Grade hemorrhage. ventricles, with large volume echogenic material

Fig. 32.60

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Case 61 Newborn infant with a sacral dimple on exam Imaging findings (Fig.  32.61): Sagittal ultrasound image of the spine (a) demonstrates a lowlying conus medullaris terminating at the sacral level with thickened and echogenic appearance of the filum terminale (arrow). Sagittal T2-weighted

a

MRI of the spine (b) better demonstrates a soft tissue mass with fat signal (arrow) between the lowlying conus and the terminus of the thecal sac. Diagnosis:  Tethered cord with a terminal lipoma.

b

Spine SAG

Fig. 32.61

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Case 62 5-year-old male presenting with neck pain, stiffness, fever, and difficulty swallowing Imaging findings (Fig.  32.62): Lateral radiograph of the neck (a) demonstrates abnormal thickening of the retropharyngeal soft tissues (∗), larger than the width of a cervical vertebral body. There is also adenoid enlargement causing nar-

a

Fig. 32.62

b

rowing of the nasopharynx (arrow). This prompted a contrast-­enhanced CT of the neck (b), which shows a peripherally enhancing, low density collection extending along the retropharyngeal soft tissues (∗). Diagnosis:  Retropharyngeal abscess.

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Case 63 5-year-old male with multiple cutaneous neurofibromas and café-au-lait spots presenting with arm weakness and palpable left neck mass Imaging findings (Fig. 32.63): Coronal MRI of the cervical spine demonstrates multiple lobular hyperintense lesions extending from the neural

Fig. 32.63

W. G. Kim and M. George

foramina along the nerve roots bilaterally, larger on the left side. Diagnosis:  Bilateral cervical and brachial plexus plexiform neurofibromas in the setting of neurofibromatosis type 1.

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1001

Case 64 9-month-old male with facial port wine stain and intractable seizures Imaging findings (Fig. 32.64): Axial CT of the brain (a) shows cortical and subcortical mineralization in the bilateral posterior parietal and occipital lobes (arrows). Axial T2-weighted MRI (b)

a

Fig. 32.64

b

demonstrates associated parenchymal volume loss. Axial susceptibility weighted MRI (c) better shows the distribution of predominantly subcortical mineralization. Diagnosis:  Sturge–Weber syndrome.

c

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Case 65 5-day-old infant with prenatal history of multiple cardiac and brain lesions Imaging findings (Fig.  32.65): Axial T1-weighted inversion recovery MRI of the brain shows multiple hyperintense subependymal nod-

Fig. 32.65

W. G. Kim and M. George

ules along the bilateral lateral ventricles (arrows). There are also multiple areas of cortical and subcortical thickening and hyperintensity, indicative of cortical tubers (∗). Diagnosis:  Tuberous sclerosis complex.

32  Radiology Review

Case 66 Newborn infant with congenital hydrocephalus and macrocephaly Imaging findings (Fig. 32.66): Axial and sagittal MRI of the brain (a, b) demonstrates severe hydrocephalus with marked dilatation of the lateral and third ventricles (∗). The cerebral aqueduct is not patent (arrow). Note is also made of dysgen-

a

Fig. 32.66

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esis of the corpus callosum, a Z-shaped configuration of the brainstem, and inferior vermian hypoplasia. Diagnosis:  Non-communicated hydrocephalus due to aqueductal stenosis, in the setting of Walker– Warburg syndrome.

b

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W. G. Kim and M. George

Case 67 15-year-old male who sustained an asphyxiation/ strangulation event and subsequent cardiac arrest Imaging findings (Fig. 32.67): Axial and sagittal CT of the brain (a, b) demonstrates profound cerebral and cerebellar swelling with uncal, transtentorial, and tonsillar herniation. Note the loss of gray white matter differentiation, loss of extraaxial spaces, and also the more pronounced focal

a

b

Fig. 32.67

c

low-attenuation in the deep gray nuclei (arrows). Axial diffusion-­ weighted MRI of the brain (c) demonstrates symmetrically decreased diffusivity involving the cortex, caudate, basal ganglia, and thalami, indicating diffuse ischemic change. Diagnosis:  Global hypoxic ischemic injury.

32  Radiology Review

Case 68 5-year-old child who fell off his bike and hit his head Imaging findings (Fig. 32.68): Axial CT of the brain in bone (a) and soft tissue (b) windows demonstrate a non-­displaced right parietal bone fracture (arrow) with an overlying scalp hematoma.

a

Fig. 32.68

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There is a subjacent biconvex hyperdense collection (∗) causing mild mass effect on the brain with slight midline shift toward the left. Diagnosis:  Skull fracture with an epidural hematoma.

b

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Case 69 4-year-old female presenting with several weeks of headaches and gait instability Imaging findings (Fig. 32.69): Axial CT of the brain (a) demonstrates a large mass lesion centered within the left cerebellar hemisphere and crossing the midline (arrows). There is resultant acute

a

Fig. 32.69

obstructive supratentorial hydrocephalus with transependymal edema (∗). Post-contrast axial MRI (b) shows the cystic and solid components of the tumor in the posterior fossa. Diagnosis:  Juvenile pilocytic astrocytoma.

b

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Case 70 17-year-old male presenting with headache and bitemporal visual field defect Imaging findings (Fig.  32.70): Sagittal contrast-enhanced MRI of the brain demonstrates a mildly enhancing, solid, sellar mass (∗) expanding the sella (arrow) and extending into the suprasellar cistern, exerting mass effect upon the optic chiasm.

1007

Case 71 1-day-old infant born via spontaneous vaginal delivery presenting with apneic episodes Imaging findings (Fig.  32.71): Axial diffusionweighted MRI of the brain shows geographic diffusion restriction of the entire left middle cerebral artery (MCA) territory, including the thalamus, lentiform nucleus, and internal capsule. Diagnosis:  Acute left MCA territory infarction.

Diagnosis:  Pituitary adenoma.

Fig. 32.70 Fig. 32.71

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Case 72 Newborn infant presenting with high output congestive heart failure and cranial bruit Imaging findings (Fig.  32.72): Sagittal ultrasound images (a) and post-contrast sagittal MRI (b) of the brain demonstrate aneurysmal dilatation of the median prosencephalic vein (arrows), which drains into enlarged dural venous sinuses (∗). a

Fig. 32.72

b

There are multiple feeding vessels from the pericallosal and anterior choroidal arteries (arrowhead). Note the yin-yang appearance of turbulent vascular flow on color Doppler ultrasound imaging. Diagnosis:  Vein of Galen malformation.

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Case 73 14-year-old female presenting with headache and 2 months of lower extremity paresthesia and acuteonset bilateral upper extremity paresthesia and facial numbness Imaging findings (Fig. 32.73): Axial T2 fluidsuppressed MRI of the brain demonstrates numerous ovoid T2 hyperintense lesions within the juxtacortical and periventricular white matter of the supratentorial brain, particularly along the callosal-septal junction. Additional lesions were present in the posterior fossa, upper cervical cord, and upper thoracic cord.

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Case 74 2-month-old female with a history of birth trauma presenting with a hard palpable lump on the head Imaging findings (Fig.  32.74): Radiograph of the skull demonstrates fusiform elevation of the left parietal subperiosteum with a thin rim of calcification (arrow). Diagnosis:  Left cephalohematoma.

Diagnosis:  Multiple sclerosis.

Fig. 32.74 Fig. 32.73

parietal

calcified

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Case 75 15-year-old male presenting with progressive right eye pain, swelling, and fever Imaging findings (Fig.  32.75): Axial (a) and coronal (b) contrast-enhanced CT of the orbits demonstrates diffuse sinonasal opacification on the right with a medial right orbital subperiosteal abscess (∗), intraconal fat stranding (gray arrow),

a Fig. 32.75

b

and asymmetric thickening of the medial rectus and superior oblique muscles (arrowheads). There is proptosis of the right orbit (white arrow). Diagnosis:  Postseptal orbital cellulitis complicated by a subperiosteal abscess and myositis of the medial rectus and superior oblique muscles.

32  Radiology Review

Case 76 7-year-old male with recurrent ear infections, presenting with left ear pain, posterior auricular swelling, and fluctuance Imaging findings (Fig. 32.76): Axial CT of the temporal bones (a) demonstrates opacification and coalescence of the left mastoid air cells. There is

a

Fig. 32.76

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osseous erosion of the lateral mastoid cortex (white arrow). Axial post-contrast MRI (b) demonstrates a subperiosteal abscess in the left postauricular region, with intracranial extension (gray arrow). Diagnosis:  Coalescent mastoiditis complicated by intracranial and subperiosteal abscesses.

b

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Case 77 12-year-old patient with a midline anterior neck mass that moves with swallowing and tongue protrusion, with an ultrasound initially performed, prompting further evaluation with CT Imaging findings (Fig.  32.77): Axial (a) and sagittal (b) contrast-enhanced CT of the neck demonstrates a cystic structure along the anterior midline infrahyoid neck (arrows), embedded within the strap muscles. There is a thin enhancing wall and no adjacent fat stranding to indicate active inflammation.

W. G. Kim and M. George

Case 78 9-year-old male presenting with growth arrest and worsening vision Imaging findings (Fig.  32.78): Sagittal postcontrast MRI of the brain demonstrates a heterogeneously enhancing, suprasellar mass (∗) centered within the pituitary fossa and that extends into the suprasellar cistern. Punctate areas of hypointensity within the mass are indicative of calcifications. Diagnosis:  Craniopharyngioma.

Diagnosis:  Thyroglossal duct cyst. a

b

Fig. 32.78

Fig. 32.77

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ture closure of the sagittal suture (arrow) with Case 79 10-month-old male with abnormal head shape, associated dolichocephaly. worsening over several months Imaging findings (Fig.  32.79): Surface ren- Diagnosis:  Isolated sagittal craniosynostosis. dered CT images of the skull demonstrate prema-

Fig. 32.79

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Case 80 2-month-old male presenting after fall and change in mental status Imaging findings (Fig. 32.80): Coronal CT of the brain (a) demonstrates multiple thin hyperdense crescentic collections along the cerebral

a

W. G. Kim and M. George

convexities and within the interhemispheric fissure (arrows). Surface-rendered CT of the skull (b) demonstrates a depressed right parietal skull fracture (∗). Diagnosis:  Depressed skull fracture with traumatic subdural hemorrhages.

b

Fig. 32.80

Suggested Reading

Donnelly L.  Fundamentals of pediatric imaging. 2nd ed. Philadelphia: Elsevier; 2016. American College of Radiology. ACR Walters M, Robertson R. Pediatric radiology: The requisites. 4th ed. Philadelphia: Elsevier; 2017. Appropriateness Criteria®. n.d. Available at https://acsearch.acr.org/list. Accessed 11 Nov 2018.

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Last-Minute Review Osama I. Naga

Last-Minute Review (LMR) Sections 1 General Pediatrics 2 Neonatology 3 Adolescent Medicine 4 Genetic Disorders 5 Metabolic Disorders 6 Mental and Behavioral Health 7 Emergency Medicine 8 Critical Care 9 Infectious Diseases 10 Hematology/Oncology 11 Allergy and Immunology 12 Endocrinology 13 Orthopedics 14 Sports Medicine 15 Rheumatology 16 Neurology 17 Ophthalmology 18 Ear, Nose, and Throat 19 Cardiology 20 Pulmonology 21 Nutrition 22 Gastroenterology 23 Nephrology 24 Fluids and Electrolytes 25 Urology 26 Dermatology 27 Psychosocial Issues and Child Abuse 28 Ethics 29 Research and Statistics 30 Patient Safety and Quality Improvement 31 Pharmacology and Pain Management

LMR Section Page Number 1016 1022 1027 1032 1036 1039 1044 1049 1052 1060 1066 1070 1078 1083 1085 1087 1092 1095 1099 1104 1108 1110 1115 1119 1120 1122 1125 1128 1131 1132 1134

Main Chapter Page Number 1 35 81 103 143 167 197 247 267 345 391 417 465 507 525 551 585 611 643 691 731 757 799 825 841 855 887 903 913 921 927

O. I. Naga (*) Department of Pediatrics, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 O. I. Naga (ed.), Pediatric Board Study Guide, https://doi.org/10.1007/978-3-030-21267-4_33

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GENERAL PEDIATRICS Osama I. Naga

Last-Minute Review—General Pediatrics Which growth charts should be used for children between 0 and 2 years? Which growth charts should be used for children > 2 years? Birth weight of newborn usually regained at what age? Birth weight doubles at what age? Birth weight triples at what age? Normal weight gain after 2 years of age per year Body mass index (BMI) should be used starting at what age? How is BMI calculated? What is the most common cause of failure to thrive? How much is birth length increased by 1 year of age? Birth length doubles at what age? What is the average growth length (growth velocity) per year after 2 years of age (later childhood)? What is the approximate range of pubertal peak growth velocities in boys and girls? How much does the head circumference increase per month in the 1st year? When does the head grow the fastest? Head circumference should be measured routinely in each well visit until what age? What is the risk for a premature infant with an enlarged head circumference? What is the study of choice for an infant who presents with macrocephaly? What is the study of choice for an infant who presents with absolute microcephaly? Child with enlarged head > 98th percentile, similar to the father, no symptoms and normal cognitive function, head imaging study showed prominent subarachnoid space especially in the frontal region Anterior displacement of the occiput on one side and the frontal region on the ipsilateral side and the ear is more anterior on the side of occipital flattening (parallelogram) Anterior displacement of the occiput on one side and frontal bossing on the contralateral side and the ear is displaced more posteriorly (trapezoid)

Most Likely Answer World Health Organization (WHO) chart CDC growth charts 10–14 days 5–6 months 1 year 2–3 kg/year approximately 2 years Weight (kg)/[height (m)]2 Inadequate caloric intake 50% 3–4 years 5 cm/year approximately 7–12 cm/year in boys 6–10.5 cm/year in girls 1 cm/month First 60 days of life (0.5 cm/week) 2 years Hydrocephalus Head ultrasound (US) Brain MRI Benign familial macrocephaly

Positional plagiocephaly

Posterior plagiocephaly (craniosynostosis)

33  Last-Minute Review

Last-Minute Review—General Pediatrics The most common type of craniosynostosis

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Most Likely Answer Long narrow head (scaphocephaly), which is an early closure of the sagittal sutures A 6-month-old with progressive head enlargement Scaphocephaly (craniosynostosis) and crossing percentiles from 25th percentile at 2 months to 98th percentile at 6 months well visits. The head is elongated in the anterior–posterior diameter and shortened in the biparietal diameter. Ridging of the sagittal suture is palpable What is the next best step in a child with suspected Refer to a pediatric neurosurgeon (imaging craniosynostosis? studies are not required to make the diagnosis in typical cases) What is the only vaccine that can be given at birth? Hepatitis B Infant born to HBsAg positive mom what should Hepatitis B and HepB immunoglobulin in the be given? first 12 h What is the maximum age you can give DTaP? DTaP is only for children younger than 7 years old Encephalopathy within 7 days of administration is DTaP an absolute contraindication in which vaccine? When can Tdap or Td be given? 7 years and older Rotavirus, measles, mumps, rubella (MMR), oral Live attenuated virus vaccines poliovirus vaccine (OPV), and varicella are How are MMR, varicella, and inactivated polio Subcutaneously (IPV can be given either IM or (IPV) given? SC) Can you give MMR vaccine and perform a purified Yes protein derivative (PPD) test at the same time? If you give only MMR vaccine, how long should 4–6 weeks you wait to do PPD test? Which vaccines are contraindicated to be given to Live vaccines, e.g., MMR, varicella, and immunocompromised children? rotavirus Anaphylaxis reaction to neomycin or gelatin is an MMR absolute contraindication to which vaccine? The child who received MMR vaccine 2 weeks ago Rubella is now having pain in the hip joints. Which component of the vaccine is responsible for this reaction? When does Haemophilus influenzae type b 5 years or older vaccination not need to be given to healthy children? When does pneumococcal (PCV13) vaccination 5 years or older not need to be given to healthy children? For which conditions can you give Haemophilus One dose for unimmunized persons with influenzae type b vaccination at 5 years of age or functional or anatomical asplenia and HIV older? infection. 3 doses after hematopoietic stem cell transplant (HSCT) regardless of the history of HIB immunization

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Last-Minute Review—General Pediatrics Which pneumococcal vaccines should be given to high-risk children 2 years and older, e.g., HIV, sickle cell disease, asplenia, cochlear implant? An 11-year-old female is here for HPV vaccination; how many doses are recommended? A 15-year-old female is here for HPV vaccination; how many doses are recommended? Children younger than 9 years of age, never been vaccinated for influenza before; how many doses should they receive during the first instance of influenza vaccination? Child has a severe egg allergy (anaphylaxis). Can he or she receive the MMR vaccine? Child is allergic to eggs (only hives). Can he or she receive the influenza vaccine today? Child has a severe egg allergy (anaphylaxis). Can he or she receive the influenza vaccine? Child with a previous severe allergic reaction (anaphylaxis) to the flu vaccine. Can he or she receive the influenza vaccine? A 4-year-old boy has 104 °F fever and ear infection; can he be vaccinated today? An unimmunized 4-month-old child came for catch-up vaccination. Can he or she receive the rotavirus vaccine? A 9-month-old boy came for catch-up vaccination, and he had only one dose of rotavirus vaccine at 2 months well visit. Can he receive the rotavirus vaccine? If a household member is immunocompromised, e.g., HIV, leukemia, or severe combined immunodeficiency (SCID), can you give the 4-month-old who is living in the same house oral poliovirus vaccine (OPV) A 2-month-old child with complement component deficiency. What are the vaccines that should be given at the 2-months well visit? A mother declined vaccination of her child. What is the next best step? Visual acuity for a newborn is At what age is a social smile seen? An infant is able to track an object to 180° Moro reflex disappears by what age? An infant is able to roll from front to back and has no head lag

O. I. Naga

Most Likely Answer PCV13, and PPSV23 (PPSV23 is given at least 8 weeks after any prior PCV13) 2 doses 6–12 months apart 3 doses 0, 1–2 months, and 6 months are recommended 2 doses 1 month apart

Yes Yes Yes. Under the supervision of a health care provider who can recognize and manage severe allergic conditions No Yes No No

No

Routine 2 months vaccines plus meningococcal vaccine Explore any misconceptions about the safety and efficacy of vaccines 20/400 1–2 months 2 months 3–6 months 4 months

33  Last-Minute Review

Last-Minute Review—General Pediatrics An infant is able to roll from back to front, supports weight with legs and bounces, able to transfer objects from hand to hand, knows familiar faces, knows own name, and begins to have separation anxiety An infant should be able to sit without support An infant pulls to stand, says “mama/dada” nonspecifically, and imitates sounds An infant is able to stand and take steps, says “mama/dada” specifically, has a mature pincer grasp Child is able to say 3–5 words, turns pages of a book, builds a tower of 3 blocks Child is able to say 10–25 words, throws a ball overhand, builds 4 blocks towers, uses a spoon, and knows 3 body parts At what age are most toddlers able to use a cup well? Child says 50 words, 2-word sentences, throws overhand, goes upstairs both feet on each step, engages in parallel play Child copies a circle, three-word sentences, alternates feet on stairs, knows 2 colors, 75% intelligible speech Child copies a square, 5-word sentences, identifies gender, knows 5 colors, 100% intelligible speech Child copies a triangle, uses scissors, and can count to ten At what age are most children able to copy a diamond? Inability to hold the head steady is a red flag for abnormal motor development at what age? Inability to sit alone and lack of rolling are a red flag for abnormal motor development at what age? Inability to walk independently is a red flag for abnormal motor development at what age? A 1-year-old boy is unable to stand or crawl, unable to point and is not speaking a single word An 18-month-old with only 3 words, lack of joint attention, not sharing the interest in an object

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Most Likely Answer 6 months

7 months 9 months 12 months 15 months 18 months 15–18 months 24 months 36 months (3 years) 48 months (4 years) 60 months (5 years) 6–7 years 4 months 9 months 18 months Motor, cognitive, and language delay Screen for autism spectrum disorder and developmental delay with 2 separate tools, e.g., M-CHAT and ASQ Hearing test

A 2-year-old uses only 5 words. What is the best test to order? The American Academy of Pediatrics (AAP) All infants with significant congenital hearing recommends that universal hearing screening of all loss should be identified by 3 months of age, and infants occur by what age? necessary intervention initiated by 6 months of age

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Last-Minute Review—General Pediatrics AAP recommends universal screening for anemia with determination of Hb concentration at what age? AAP recommends lead screening at what age? What is the non-hematologic consequence of iron deficiency anemia? AAP recommends autism screening at what age? AAP recommends discussing tobacco, alcohol, or drug use with children at what age? The US Preventive Services Task Force (USPSTF) recommended age to begin screening all adolescents for HIV Screening for depression should be done annually starting at what age? Screening for dyslipidemia should be done at what ages?

O. I. Naga

Most Likely Answer 1 year of age All Medicaid-eligible children and those whose families receive any governmental assistance must be screened at ages 1 and 2 years Neurocognitive changes 18 months and 24 months 11 years Once between 15 and 18 years (younger if increased risk, e.g., male-to-male sexual contact) Starting at 12 years

Between 9 and 11 years and again between 17 and 21 years, or if there are risk factors (obesity, diabetes, etc.) A 7-year-old boy has a parent with total cholesterol Order fasting lipid profile, then repeat after of 300 mg/dL. What is the next best step? 2 weeks to 3 months Infant is exclusively breastfed. At what age should First few days of life you recommend daily vitamin D supplementation (400 IU)? Infant is feeding more than 1 liter of formula per No (1 liter of formula has 406 IU of vitamin D) day in addition to breastfeeding; does he or she need vitamin D supplementation? Infant is exclusively breastfed. At what age should 4 months you recommend daily iron (1 mg/kg/day)? At what age should you recommend starting solid 4–6 months foods in infants? What is the reason for starting solid foods between May decrease the risk of allergy to that specific 4 and 6 months of age? food A 4-week-old infant has been exclusively Continue breastfeeding breastfeeding, and the mother is scheduled for MRI with contrast A 4-week-old infant has been exclusively Continue breastfeeding (can continue on the breastfeeding, and the mother has mastitis other side if breast abscess or cellulitis with direct contact with infant’s mouth) A 4-week-old infant has been exclusively Discontinue breastfeeding until effective breastfeeding, and the mother recently diagnosed maternal treatment for the initial 2 weeks or the with active tuberculosis. No treatment yet infant is receiving isoniazid) Newborn with a mother who is HIV positive Breastfeeding is contraindicated (except in resource-limited settings) What is the age when infants can drink cow’s 12 months milk?

33  Last-Minute Review

Last-Minute Review—General Pediatrics At what age can a child be given low-fat milk? AAP recommends the initial visit to the dentist at what age? What usually are the first teeth to erupt? What is the latest age for first tooth eruption? Child with white lines and spots at the bases of several teeth A 5-year-old boy hit his head and knocked out his 2 primary central incisors 5 min ago. What is the next best step? A 10-year-old boy hit his head and knocked out his 2 permanent central incisors 5 min ago. What is the next best step? If a tooth has been knocked out, it should be placed back into its socket until the child can see the dentist, should that prove impossible, what is the best solution in which to keep it? When should the knocked-out tooth be implanted? At what temperature should you set the water heater at home? Child with a capillary lead level of > 5 μg/ dL. What is the next best step? What is the safe blood lead level (BLL)?

1021

Most Likely Answer > 2 years 12 months of age Lower anterior incisors 18 months (after that, dental consult) Referral to a dentist (sign of dental caries) Reassurance (re-implantation of the primary tooth may damage the developing permanent tooth) Re-implant the teeth immediately (best prognosis if avulsed tooth re-implanted within 15–30 min) Cold milk

Immediate treatment is essential 120 °F or less Obtain venous sample

There is no safe BLL, and any detectable lead level must be managed Children should be secured with a rear-facing seat? Birth to 2–4 years, as long as possible, or until they reach the highest weight or height allowed Children who have outgrown their rear-facing seat Forward-facing car seat until they reach the should use? highest weight or height allowed or at least 5 years of age Children who have outgrown their forward seating Booster seat and are less than 57 in. (4 ft 9 in.) tall should use? Children who have outgrown their booster (57 in., Seatbelt; if the seat belts fit properly when the or 4 ft 9 in. tall) seat should use? lap belt lays across the upper thighs (not the stomach), and the shoulder belt lays across the chest (not the neck) What is the recommended preventative measure to Enclosing the pool with a 4-ft fence help prevent drowning? What is the recommended measure to prevent Gun and bullets stored in locked and separate accidental gun injuries? locations AAP does not recommend the use of repellents for Children younger than 2 months children at what age? Treatment of a large local reaction after a mosquito Antihistamine, ice, and topical hydrocortisone bite cream What is the significant risk of sunburns? Increases risk of melanoma at all ages What are the long-term complications of artificial Cataracts, skin aging, and cancer ultraviolet rays (e.g., skin tanning)

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NEONATOLOGY Mamta Fuloria

Last-Minute Review—Neonatology Birth weight less than the 10th percentile Birth weight more than the 90th percentile Birth weight less than 2500 g Birth weight less than 1500 g Birth weight less than 1000 g Gestational age of screening for group B Streptococcus (GBS) What is the drug of choice for GBS prophylaxis? Mother currently GBS negative, but the previous infant had GBS disease. Is GBS prophylaxis recommended? Which group has the highest infant mortality rate in the USA? Most common cause of infant deaths in the USA What is the clinical significance of a single umbilical artery? The third trimester presents with Hemolysis, Elevated Liver enzymes, Low Platelet count What is the definitive treatment for preeclampsia/ HELLP syndrome? The best course of action if fetal scalp pH  160 beats/min Fetal heart rate  50 mmHg)

5 mm uncuffed 4–4.5 mm cuffed C-A-B-D: Circulation—Airway—Breathing—Defibrillate Vasogenic

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Last-Minute Review—Critical Care Most Likely Answer A patient with persistent intracranial hypertension Uncal herniation now has unilateral third nerve palsy and unilateral fixed dilated pupils deviating downward and laterally. What is the type of herniation? In the above scenario, what would be the likely Midbrain location of this constellation of symptoms in the brain?

INFECTIOUS DISEASES Matthew B. Laurens

Last-Minute Review—Infectious Diseases Diarrhea and turtle at home Childcare center, fever, vomiting, bloody diarrhea, new-onset seizure, leukocytosis, bandemia, and rectal prolapse Diarrhea, high BUN/creatinine, thrombocytopenia, and hemolytic anemia Child with his family to the Bahamas on a cruise ship, all of them have diarrhea, and a large number of people on the ship have the same Child had rice in a restaurant, presents with vomiting and diarrhea Child ate potato salad 3 h ago, presents with sudden onset of nausea, vomiting, severe abdominal cramps and diarrhea Adolescent recently had grilled rare pork meat presents with acute right lower quadrant (RLQ) abdominal pain, normal appendix on abdominal US Child living in a farm and has been drinking unpasteurized cow milk, presenting with fever, bloody diarrhea, and vomiting. What is the most likely cause? Campylobacter is associated with which of the following neurological conditions? A 6-month-old infant presents with constipation, and poor feeding (mother tried honey for the first time) What is the best test to confirm the diagnosis in the previous case with suspected botulism? Community outbreak of diarrhea, news reports that the drinking water has been contaminated with acid-fast protozoa

Most Likely Answer Nontyphoidal Salmonella Shigella Hemolytic uremic syndrome E. coli O157: H7 Norovirus outbreak Bacillus cereus Staphylococcus aureus (preformed enterotoxin) Yersinia enterocolitica Campylobacter jejuni

Guillain–Barré syndrome Botulism Detection of botulism toxins or spores in stool Cryptosporidium

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Last-Minute Review—Infectious Diseases What are the common pathogens causing recreational water-­associated outbreaks of acute gastroenteritis Traveled to Mexico; foul smelling diarrhea, with burping and flatulence Traveled to Mexico; bloody diarrhea, tenesmus, and without fever Patient with bloody diarrhea with mucus, fever, abdominal pain, liver abscess and recent travel to Mexico What is the best diagnostic test in cases with suspected invasive amebiasis? Unimmunized and buccal cellulitis Adolescent presents with, pneumonia, diarrhea, headache, and confusion Adolescent presents with, cough, low-grade fever, headache, wheezing, and negative cold agglutinins A 3-day-old newborn, copious purulent eye discharge, and eyelid edema Erythromycin ointment is considered the best regimen for prophylaxis against neonatal conjunctivitis because of its efficacy against A 6-week-old, staccato cough, and eye discharge A 3-month-old presents with a staccato cough, no fever, and chest radiograph positive for pneumonia A 16-year-old with fever, recurrent non-productive cough, and malaise; patient was exposed to exotic birds in South America Breeds turkey, high fever, pneumonia, muscle pain, and splenomegaly Fever of unknown origin with elevated liver enzymes, lives on a farm, the most likely cause Tick bite, fever, rash, myalgia, headache, pancytopenia, elevated liver enzymes, and hyponatremia Tick bite, fever, rash on palms and soles, headache, joint pain, low platelet, and hyponatremia A 4-year-old with RMSF. What is the drug of choice? Connecticut, target skin lesion (erythema migrans), next step Child was camping in a park in New York, developed Bell’s palsy, no rash, no other symptoms A mother found a tick attached to her child’s thigh

1053

Most Likely Answer Cryptosporidium, Shigella, Giardia, norovirus, and E. coli O157: H7 Giardiasis Amebiasis (Entamoeba histolytica) Entamoeba histolytica Treatment: metronidazole plus paromomycin Serum antibodies to Entamoeba histolytica Haemophilus influenzae type b (Hib) Legionella pneumophila Chlamydia pneumoniae Gonococcal conjunctivitis Gonococcal, and nongonococcal nonchlamydial pathogens (does not prevent Chlamydia trachomatis transmission from mother to infant) Chlamydia trachomatis Chlamydia trachomatis Chlamydia psittaci Chlamydia psittaci Brucella, blood culture is the best test and treat with doxycycline + rifampin Ehrlichiosis Rocky Mountain spotted fever (RMSF) Rickettsia rickettsii Doxycycline Treat (Lyme disease); do not order serology Order Lyme serology and treat if positive Ticks should be removed by using forceps or tweezers without twisting or crushing

1054

Last-Minute Review—Infectious Diseases Child visited Oklahoma with family, they hunted and skinned rabbits, the child presented with a large lymph node in the groin, and fever Neonate, peripherally inserted central catheter (PICC) line is positive for Candida albicans Most common electrolyte disturbances associated with amphotericin B therapy Infant presents with 3 days of high fever, febrile seizure, develops a rash when fever resolves Fever, headache, runny nose, rash on the cheeks (looks like slapped), lacy rash on both arms Very high fever, cough, coryza, conjunctivitis, bluish-gray specks on the buccal mucosa, the maculopapular rash spreading from the head down, splenomegaly, and lymphadenopathy Child with mumps. For how long should children with mumps be excluded from school? During school outbreak of mumps. For how long unimmunized children should be excluded from school? Posterior auricular and suboccipital lymphadenopathy, headache, eye pain, sore throat, maculopapular rash, low-grade fever, and chills Newborn with microcephaly, chorioretinitis, periventricular calcification and a major cause of sensorineural hearing loss Newborn with microcephaly, subcortical intracranial calcifications, eye anomalies, and hyperreflexia. Mother immigrated from Brazil 2 months before giving birth in the USA. She recalls having a fever in the first trimester A fully immunized 6-year-old presented with malaise, low-grade fever, and a mild vesicular rash that resembles “dew drops on a rose petal” Child is born to a mother who is diagnosed with varicella. When should the varicella zoster immunoglobulin (VZIG) be given? A 5-year-old male with sudden onset of high fever during the month of March; he has body aches, chills, sore throat, and generalized fatigue A 20-month-old boy with sudden onset of high fever 105 °F during the month of January. He has a runny nose, cough, and malaise. O/E: he has nasal flaring and retractions, bilateral rhonchi. Rapid influenza and respiratory syncytial virus (RSV) tests are negative. What is the next best step?

O. I. Naga

Most Likely Answer Tularemia (Francisella tularensis) Remove the catheter and start IV antifungal Hypokalemia Hypomagnesemia Human herpesvirus 6 infection (roseola infantum) Erythema infectiosum (parvovirus B19) Measles

5 days from onset of parotid gland swelling At least 26 days after the onset of parotitis in the last person with mumps in the affected school German measles (rubella) Cytomegalovirus (CMV) Congenital Zika syndrome

Varicella zoster—may have mild episode even if vaccinated If the mother is diagnosed from 5 days before birth to 2 days after birth Influenza Start oseltamivir and order influenza molecular assays (no need to wait for the result in highrisk children before starting the medicine)

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Last-Minute Review—Infectious Diseases Who is at risk of hospitalization and development of complications caused by influenza infection? What is the sensitivity of rapid diagnostic influenza assays test? What is the sensitivity of influenza molecular assay? A 3-year-old male never vaccinated against influenza should receive how many doses? What is the most common cause of croup in children? A 4-month-old unvaccinated child with profuse foul-smelling diarrhea, dehydration, and electrolyte abnormalities An 8-year-old female with recurrent cold sore on her lower lip A 16-year-old sexually active male is complaining of painful vesicles noted on the penis A 14-year-old with a history of severe eczema presents with diffuse clusters and vesicles noted on the affected area A 16-year-old sexually active female presents with cauliflower-­like lesions in the genital region Which strains of HPV are more commonly associated with cervical cancer? Adolescent male present with mumps (parents are asking about the possible complications) Chickenpox rash is infectious for how long? Limping, after stepping on a nail with a shoe on Kitten at home, large axillary and cervical lymph nodes What is the best laboratory test to establish the diagnosis of cat scratch disease Dog bite, 12 h later presents with swelling of the hand, tenderness, and erythema Dog bite, 5 days later presents with swelling of the hand, tenderness, and erythema Dog, cat, and human bite drug of choice Dog bite with severe complications, the patient is hospitalized Dog bite and allergic to penicillin Bitten by a fox Dead bat found in the same room as the patient Bitten by a domestic dog during aggressive play

1055

Most Likely Answer Children younger than 2 years or who have underlying medical conditions Ranges from 10% to 70% (negative test result does not rule out influenza) Ranges from 86% to 100% From 6 months to 8 years, if never received a previous influenza vaccine, 2 doses separated by 1 month are needed, then annually afterward Parainfluenza virus Rotavirus Herpes labialis (HSV-1) HSV-1 or HSV-2 Eczema herpeticum Treatment: acyclovir HPV—strains 6 and 11 are commonly associated with anogenital warts HPV—strains 16 and 18 Epididymo orchitis, arthritis, encephalitis 1–2 days before the rash, and until all lesions are crusted over Pseudomonas aeruginosa Bartonella henselae (cat scratch disease) Serologic test (indirect immunofluorescent assay) Pasteurella species S. aureus Amoxicillin/clavulanate Ampicillin/sulbactam IV Clindamycin and TMP-SMX Give rabies vaccine and immunoglobulin Give rabies vaccine and immunoglobulin Give amoxicillin/clavulanate

1056

Last-Minute Review—Infectious Diseases The most common organism that causes infection in cat bite Cochlear implants are associated with an increased risk of which bacterial infection? Child with perianal painful rash and rectal pain for 3 days. O.E: bright red, sharply demarcated rash around the anal area What is the best empiric treatment in cases of perianal bacterial dermatitis? A 5-year-old, fever, headache, pharyngeal erythema, palatal petechiae, abdominal pain, nausea A 5-year-old presents with fever, headache, pharyngeal exudates, and diffuse sandpaper-like rash. Gram-positive beta-hemolytic streptococci isolated from throat culture A 2-year-old child presents with low-grade fever, thick nasal discharge, and anterior cervical lymphadenopathy A 3-year-old, fever, runny nose, hoarse voice, cough, and pharyngeal exudates A 12-year-old, throat pain with exudates, fever, headache, large cervical lymph node, and splenomegaly Best screening test for suspected EBV infection in ≥ 5-year-old A football player is diagnosed with EBV without splenomegaly. How soon can he return to contact sports? Abrupt onset of pharyngitis, palpebral conjunctivitis, fever, a moderate degree of illness, and preauricular lymphadenopathy, mild cough, and nasal congestion, rhinorrhea, abdominal pain Prodromal high fever and irritability, followed by painful vesicles that ulcerate on the anterior palate, tongue, and buccal mucosa, with intensely inflamed gingivae A 6-year-old child with fever, headache, throat pain and abdominal pain. O/E: pharyngeal erythema, palatal petechiae, and positive cervical lymphadenopathy. What is the next best step?

O. I. Naga

Most Likely Answer Pasteurella multocida Streptococcus pneumoniae Perianal bacterial dermatitis (caused by Streptococcus pyogenes or S. aureus), Oral cephalexin or another antistaphylococcal antibiotic depends on the local pattern of antibiotic resistance and sensitivity S. pyogenes (group A streptococcus) Scarlet fever

Streptococcosis (Streptococcus) Viral pharyngitis Epstein–Barr virus (EBV) infectious mononucleosis Monospot test No contact sports for at least 4–6 weeks— increased risk of splenic rupture Adenovirus infection (pharyngoconjunctival fever) Primary herpetic gingivostomatitis

Rapid antigen Streptococcus testing (RAST). If RAST is negative, obtain a throat culture. Prescribing antibiotics for presumed group A Streptococcus (GAS) without testing is not appropriate

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Last-Minute Review—Infectious Diseases Poor feeding, drooling, tiny vesicles, and erythematous ulcers occur on the posterior pharynx, involving the soft palate, uvula, and tonsillar pillars, and resolve spontaneously within 1 week. With or without fever A painful 1- to 3-mm vesicles on an erythematous base involving the buccal mucosa, palate, tongue, uvula, and anterior tonsillar pillars. A gray-white vesicle surrounded by erythema primarily on the palms and soles, also on the buttocks and distal extremities Throat pain, fever, grayish-white membrane on the pharynx; the child is not immunized and looks toxic Child with a persistent tooth abscess developed multiple sinuses drainage on the cheeks with sulfur granules seen in the exudates A 12-year-old boy with a history of swimming in freshwater lagoons, developed headaches, myalgia, and fever; 7 days later he became jaundiced, with elevated creatinine level, high bilirubin level, mild elevation of AST and ALT Unimmunized, dirty wound, and fracture of the femur Immunizations up to date, last tetanus vaccine was 3 years ago, dirty wounds, and multiple compound fractures in a car accident A 12-year-old boy stepped on a dirty rusty nail, the last DTaP immunization was 8 years ago (received 5 doses of DTaP by the age 4 years of age) A 12-year-old boy stepped on a clean object at home, presents with a minor, clean wound (received 5 doses of DTaP by the age 4 years of age) Young adolescent works on an animal farm developed skin papule on the arm that eventually ulcerates and forms black eschar with non-pitting, painless induration and swelling Unimmunized, presents with fever, muscle weakness and paralysis involving the proximal muscles first. History of foreign travel A 2-month-old developed bronchiolitis and negative RSV

1057

Most Likely Answer Herpangina (enteroviral stomatitis)

Hand-foot-mouth disease (enteroviral stomatitis with exanthem)

Diphtheria Actinomycosis Leptospirosis

Tetanus vaccine and tetanus immunoglobulin (TIG) No tetanus vaccine nor TIG Tdap immunization Needs booster dose for pertussis component at 11–12 years (Tdap). Tetanus booster required every 10 years Anthrax

Poliomyelitis Human metapneumovirus

1058

Last-Minute Review—Infectious Diseases Child sustained significant burn a few days ago, starts having a fever, tachypnea, tachycardia and new discoloration of wound edges. What is the next best step? Central line, methicillin-resistant S. aureus (MRSA) infection. What is the drug of choice? IV vancomycin, suddenly develop a rash, itchiness, flushing, and tachycardia Recently traveled to Africa, seizure, decreased level of consciousness, retinal hemorrhage, and hypoglycemia. What is the most likely cause? For travel to Africa, the prophylactic antimicrobial therapy of choice for malaria is A 3-year-old developed osteomyelitis, culture is negative, not responding to vancomycin. What is the most likely cause? Neonate presents with fever, and blood culture grows Citrobacter. What is the most common complication? The best study for neonates presenting with fever and Citrobacter bacteremia Late-onset (7 days to 3 months of life) group B streptococcal infection presents with Stiff neck, fever, CSF WBC  13 and high total iron-binding capacity (TIBC) Low hemoglobin, low iron, low/normal TIBC, normal/high ferritin level Mild anemia, low MCV, normal iron, normal TIBC, normal ferritin, normal RDW, Mentzer index  16 oz/day) and microcytic anemia What are the best initial laboratory tests in cases with suspected iron deficiency anemia? What is the best indicator of response to iron therapy? How long should iron therapy continue in cases of iron deficiency anemia?

Most Likely Answer Iron deficiency anemia

Anemia of chronic disease Thalassemia trait Alpha thalassemia trait Beta thalassemia major. (Alpha thalassemia major leads to severe anemia and hydrops fetalis in utero, typically incompatible with life without treatment) Normal electrophoresis Beta thalassemia minor Beta thalassemia major Anemia of prematurity ~2 g/dL Iron deficiency anemia CBC and reticulocyte count An increase in hemoglobin, reticulocyte count, and MCV within 1–4 weeks At least 1–2 months after anemia has been corrected to replete iron stores

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Last-Minute Review—Hematology/Oncology What is the classic dose of iron in cases of iron deficiency anemia? A 2-year-old infant with a hemoglobin of 4 g/dL, normal MCV, low reticulocyte count, normal ADA (adenosine deaminase activity), negative direct Coombs test and no signs of hemolysis A 7-year-old child presents with pancytopenia, on exam also noted to have hypoplastic thumb and radius, hyperpigmentation, and abnormal facies A 4-month-old infant with severe anemia, high MCV (macrocytic), elevated ADA, and exam shows triphalangeal thumb Macrocytic anemia, neutropenia, thrombocytopenia, exocrine pancreatic insufficiency, ring sideroblasts in the bone marrow Short stature, imperforate anus, hypoplastic teeth, frequent infections, macrocytic anemia, neutropenia, thrombocytopenia, and exocrine pancreatic insufficiency Child who consumes goat’s milk and has macrocytic anemia Child whose family is strictly vegan and has macrocytic anemia Child with macrocytic anemia, glossitis, abdominal pain, gait instability with positive anti-IF antibodies Child with pallor, increased jaundice, splenomegaly, reticulocytosis, and normocytic hemolytic anemia. Peripheral smear shows RBCs without central pallor An African-American boy recently started on Bactrim for UTI with sudden onset of dark urine, jaundice, and pallor. Splenomegaly on the exam. Labs are notable for anemia, reticulocytosis, indirect hyperbilirubinemia, low haptoglobin, and normal G6PD enzyme activity (during the episode). Peripheral smear is positive for Heinz bodies Fava beans, primaquine, sulfa drugs, and nitrofurantoin are known to exacerbate which condition? Sickle cell anemia, swollen hands and feet, severe pain in hands and feet The most common cause of sepsis in patients with sickle cell disease Child with sickle cell disease presents with severe anemia, reticulocytosis, thrombocytopenia, and rapidly enlarging spleen

1061

Most Likely Answer 3–6 mg/kg/day of “elemental iron” Transient erythroblastopenia of childhood

Fanconi anemia Diamond–Blackfan anemia Pearson marrow-pancreas syndrome Shwachman–Diamond syndrome

Folic acid deficiency Suspect B12 deficiency. Supplement with B12 Pernicious anemia (B12 deficiency due to IF antibodies) Hereditary spherocytosis

G6PD deficiency. Enzyme activity test is usually normal (false negative) during active hemolysis due to the destruction of older erythrocytes (that are G6PD deficient) and presence of younger erythrocytes and reticulocytes (that have normal/near-normal enzyme activity). The test should be repeated during remission, not during active hemolysis G6PD deficiency Dactylitis Streptococcus pneumoniae Splenic sequestration Next best step → transfusion of packed RBCs (monitor hemoglobin, expect additional rise in Hgb from auto-transfusion from spleen)

1062

Last-Minute Review—Hematology/Oncology Child with sickle cell disease, fever, malaise, rash, severe anemia, and reticulocytopenia Which virus is the most common cause of aplastic crisis? Common causes of morbidity and mortality in children with sickle cell disease Child with sickle cell anemia presents with fever, chest pain, tachypnea, shortness of breath, and new pulmonary infiltrate on imaging. Management? Sickle cell patients are at higher risk of which type of organisms? What is the most common reason for hospitalization in the child with sickle cell anemia? Management? Adolescent male with a painful erection that has lasted for several hours. Management?

O. I. Naga

Most Likely Answer Aplastic crisis → treatment packed RBCs transfusion as needed Parvovirus B19 Infection, acute chest syndrome, stroke Acute chest syndrome—start ceftriaxone + macrolide (to cover for atypical organisms). Avoid overhydration Encapsulated organisms—due to functional asplenia. Make sure vaccines are up to date Vaso-occlusive pain crisis Treatment: IV hydration, NSAIDs, and opioids

Prolonged priapism—needs emergent evaluation and treatment. Ask patient to come to the ER, aspiration +/− irrigation, phenylephrine, pain control, possible surgical management What is the most common cause of osteomyelitis in Salmonella a child with sickle cell disease? What is the next best step in a child with sickle cell Imaging studies (MRI), blood culture, disease and suspected osteomyelitis? antibiotics (cover Salmonella and other Gram-­ negative bacilli, as well as S. aureus), consider biopsy for culture Adolescent with sudden onset of fatigue, pallor, Autoimmune hemolytic anemia (AIHA) scleral icterus, and tachycardia, high reticulocyte count, positive direct antibody test. What is the most likely diagnosis? What is the next best step in the previous lifeStart steroids. Supportive care may include threatening case of autoimmune hemolytic anemia transfusion of the least incompatible packed (AIHA)? RBC unit(s) Fever and absolute neutrophil count (ANC)  100,000. Peripheral smear shows Auer rods in blasts Chronic myelogenous leukemia is associated with which chromosome translocation? A 1-year-old with very large spleen, moderate leukocytosis (increased monocytes), xanthoma, eczema, and café-au-lait spots Child with an abdominal mass presents with abdominal pain, weakness, lethargy, oliguria, edema, elevated lactate dehydrogenase (LDH) and uric acid, hyperkalemia, elevated phosphate, and low calcium What is the next best step in the previous case of tumor lysis syndrome?

Microscopic picture of Hodgkin lymphoma The most common type of lymphoma in children Most common malignant tumor of the kidney in children Child with macroglossia and Wilms tumor Most common soft-tissue tumor in children Long-term complications of radiotherapy Complication of doxorubicin therapy Complication of vincristine therapy This antineoplastic drug can cause renal impairment and ototoxicity Complication of methotrexate therapy Complication of cyclophosphamide therapy

1063

Most Likely Answer Referral to a pediatric oncologist (must be biopsied or investigated) Neuroblastoma Acute lymphocytic leukemia Astrocytoma Infantile hemangioendothelioma (most commonly occurs in the first 6 months of life, rarely seen in children > 3 years of age) Neuroblastoma

Acute myelogenous leukemia Philadelphia chromosome t(9:22) Juvenile myelomonocytic leukemia (JMML). JMML has an association with NF1 and Noonan syndrome Burkitt lymphoma (tumor lysis syndrome)

Transfer immediately to oncology unit or PICU for supportive care, including hydration, correction of electrolytes—hyperkalemia, hyperphosphatemia, hyperuricemia, renal dysfunction. May even require hemodialysis Reed-Sternberg cell Non-Hodgkin lymphoma Wilms tumor Beckwith–Wiedemann syndrome Rhabdomyosarcoma Growth retardation, hypothyroidism, early onset coronary artery disease, pulmonary fibrosis, secondary malignancy Cardiomyopathy Neuropathy Cisplatin Renal and liver toxicity Hemorrhagic cystitis

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Last-Minute Review—Hematology/Oncology Common electrolyte abnormalities in tumor lysis syndrome? A 12-year-old boy with pain and swelling above the knee, pain is worse at night; radiograph shows a bone lesion with Codman’s triangle and sunburst pattern A 16-year-old girl with back pain and limp, fever, weight loss, radiograph shows a mass on the iliac bone with a lytic lesion, onion-skin appearance The translocation is commonly seen in patients with Ewing sarcoma Child with a painless, bony mass on the knee, radiograph shows broad base projection Child with persistent pain in the lower part of the right femur, radiograph shows metaphyseal lucency surrounded by sclerotic bone, NSAIDs relieve the pain Hemangioblastoma, pheochromocytoma, renal cell carcinoma, pancreatic cyst, and café-a- lait spots Impaired upward gaze, mid-dilated pupil, nystagmus, and lid retraction New-onset head tilt or torticollis, early morning vomiting, headache, and gait disturbance Most common malignant CNS tumor in children Most common CNS tumor Brain tumor with the best survival rate in children Child with recurrent headaches, growth failure, polydipsia, double vision A 6-month-old child with strabismus, absent red reflex, and leukocoria The liver tumor that is associated with prematurity The tumor that is associated with cryptorchidism An infant with intracranial hemorrhage, prothrombin time (PT), partial thromboplastin time (PTT), and platelet count, fibrinogen and vWD panel is within normal limits Child with normal PT, very prolonged PTT, has no history of excessive bleeding even after injuries A 5-year-old with upper respiratory tract infection 2 weeks ago, presents with a bloody nose, petechial rash all over the body and oral mucosa. CBC is normal except platelet count is 12,000, peripheral smear shows very few large platelets What is the treatment in the previous case of ITP?

O. I. Naga

Most Likely Answer Hyperkalemia, hyperuricemia, hyperphosphatemia, and hypocalcemia Osteosarcoma

Ewing sarcoma t (11;22) Osteochondroma Osteoid osteoma

Von–Hippel–Lindau disease Parinaud syndrome Posterior fossa brain tumor, e.g., medulloblastoma Medulloblastoma Low-grade glioma (pilocytic astrocytoma is the most common LGG) Pilocytic astrocytoma Craniopharyngioma Retinoblastoma Hepatoblastoma Gonadoblastoma Factor XIII deficiency

Factor XII deficiency Idiopathic thrombocytopenic purpura (ITP)

Observation if no signs of bleeding; if signs of bleeding, then treat with IVIG or steroids

33  Last-Minute Review

Last-Minute Review—Hematology/Oncology A 2-year-old boy with recurrent infections, eczema, severe thrombocytopenia, and small platelets Newborn with severe thrombocytopenia, maternal history of ITP or other autoimmune disorder Newborn with severe thrombocytopenia, maternal history of prior children with neonatal thrombocytopenia and no maternal history of autoimmunity Unusual bleeding since birth, recurrent bruising, recurrent mucosal bleeding, low to normal platelet count, normal PT and PTT, normal fibrinogen, normal von Willebrand antigen and activity A 10-year-old with recurrent epistaxis, easy bruising, gingival bleeding, normal count and morphology of platelets, platelets agglutinate to ristocetin, poor platelet aggregation with adenosine diphosphate (ADP), epinephrine, and collagen A 10-year-old with a suspected bleeding disorder, workup shows mild thrombocytopenia, with large abnormal platelets, platelets do not agglutinate to ristocetin but agglutinate to ADP, epinephrine, and collagen Most appropriate management for life-threatening bleeding in a child with a known or suspected platelet function disorder A 48-h-old newborn presents with prolonged bleeding after circumcision, CBC shows severe thrombocytopenia. On exam no radii in both forearms but with normal thumbs Male newborn with prolonged bleeding after circumcision, and prolonged PTT A 15-year-old girl with excessive menstrual bleeding every month since menarche, normal PT and PTT, decrease in biological activity of ristocetin cofactor assay (rCoF) A 4-year-old child with a recent history of vomiting and bloody diarrhea found to have thrombocytopenia, elevated BUN and creatinine, schistocytes on peripheral smear Child currently hospitalized in the PICU with prolonged PT, PTT, elevated D-dimer, thrombocytopenia, and decreased fibrinogen

1065

Most Likely Answer Wiskott–Aldrich syndrome Neonatal ITP → give IVIG Neonatal alloimmune thrombocytopenia → transfuse maternal platelets (gold standard); however, difficult to obtain. The alternative option is donor platelets +/− IVIG, steroids Platelet function disorders, e.g., Bernard– Soulier syndrome, Glanzmann thrombasthenia Glanzmann thrombasthenia (normal platelet count and size)

Bernard–Soulier syndrome (large platelets, can have low platelet count)

Infusion of platelets with normal function Thrombocytopenia with absent radii (TAR syndrome) Factor VIII or IX deficiency, or hemophilia A or B Von Willebrand disease

Hemolytic uremic syndrome—occurs in infants and children after prodromal diarrhea, associated with bacteria particularly E. coli O157: H7, and Shigella dysenteriae Disseminated intravascular coagulation (DIC) Treatment: treat the underlying cause (e.g., antibiotics for sepsis). Supportive care with blood products—FFP +/− cryoprecipitate A 17-year-old Caucasian boy with recurrent Factor V Leiden mutation—resistance to episodes of DVT along with a strong family history activated protein C of DVT

1066

Last-Minute Review—Hematology/Oncology A 2-year-old boy with oral ulcers, cradle cap-like rash, and gingivitis, with radiograph showing lytic lesions in the skull A 1-year-old sick-appearing child with fever, hepatosplenomegaly, pancytopenia, hypertriglyceridemia, and very elevated ferritin; presence of hemophagocytosis in bone marrow

O. I. Naga

Most Likely Answer Langerhans cell histiocytosis—Birbeck granules on electron microscopy Hemophagocytic lymphohistiocytosis (HLH)

ALLERGY AND IMMUNOLOGY Maria I. Garcia Lloret and Caroline Y. Kuo

Last-Minute Review—Allergy and Immunology Antibody that has a major role in allergic conditions, e.g., anaphylaxis, atopy, asthma, allergic rhinitis, food allergies Antibody that mediates type I hypersensitivity reaction First antibody produced in an infection An antibody found in body mucosal secretions What is the prevalence of atopic disorders in children with one affected parent? What is the prevalence of atopic disorders in children with 2 affected parents? What are atopic disorders? What are the indications of allergy testing? An infant with severe eczema and/or severe egg allergy

Most Likely Answer IgE IgE IgM IgA Up to 60% Up to 80% (family history is critical in all atopic disorders) Atopic dermatitis, asthma, allergic rhinitis, and food allergies Significant allergies, e.g., asthma, anaphylaxis, food or drug allergies, difficult to treat allergies or requirement for specific treatment Evaluate for peanut reactivity with either skinprick testing and/or serum IgE levels, and if necessary, oral food challenge Radioallergosorbent test (RAST) (allergenspecific IgE antibody)

Which allergy test is preferred in cases of dermatographism, generalized dermatitis, or a clinical history of severe anaphylactic reactions to a given food? Which allergy test is associated with a high false Both skin-prick testing and serum IgE levels positive rate? Child currently on diphenhydramine for allergies is At least 5 days prior to testing scheduled for skin allergy testing. When should diphenhydramine be stopped? Child currently on cetirizine for allergies is At least 7 days prior to testing scheduled for skin allergy testing. When should cetirizine be stopped?

33  Last-Minute Review

Last-Minute Review—Allergy and Immunology Child currently on amitriptyline for migraine headache prophylaxis is scheduled for skin allergy testing. When should amitriptyline be stopped? What is the first step that should be taken in the management of allergic rhinitis? The first-line pharmacologic treatment of allergic rhinitis Common complications of untreated allergic rhinitis Complications of prolonged use of nasal adrenergic drops An 8-year-old male presents with congestion, itchy nose, and watery eyes. Symptoms are exacerbated when playing with the pet cat. He loves his cat. What is the most effective treatment? Child with a history of pollen allergy develops rapid onset of itching, swelling of the lips, mouth, and throat when eating raw fruits and vegetables. What is the most likely cause? What is the best treatment for OAS? How long do the allergies to peanuts, tree nuts, seafood, and fish last? How long do the allergies to milk, eggs, and soy last? A 2-month-old exclusively breastfed infant is seen for bloody stools; weight gain is appropriate, and physical exam is normal Induration reaction to TB testing after 72 h is an example of Allergy to contrast media is an example of Child with a history of severe allergic reaction to radiographic contrast media is going for CT scan with IV contrast Child with a history of severe allergic reaction to seafood is going for an abdominal CT scan with oral and IV contrast. Does he or she need a pretreatment with prednisone and diphenhydramine? A 16-year-old male with a new watch notices an area of erythema located on the wrist where the watch was worn. No other lesions What is the best treatment in cases of contact dermatitis? Child received penicillin 10 days ago for the first time, presents with fever, nausea, vomiting, pruritic skin rash (urticaria), angioedema, joint pain, lymphadenopathy, myalgia, and proteinuria

1067

Most Likely Answer At least 2 weeks prior to testing Avoidance or reduction of allergen exposures Intranasal steroids and/or second-generation oral antihistamines Recurrent acute otitis media, sinusitis, chronic cough, and asthma Rhinitis medicamentosa Avoid the trigger (e.g., by keeping the cat at least outside the bedroom or the house all the time; HEPA filters can help) Oral allergy syndrome (OAS) (cross-reactivity with pollen) Avoid offending foods Lifelong allergies Most children outgrow by 5 years of age Reassurance (counsel mother to avoid dairy, soy, eggs for 2 weeks, then re-evaluate) Type IV: cell-mediated hypersensitivity Non-IgE mediated Administer prednisone and diphenhydramine before injection or choose other alternative imaging tests No (iodine allergy is not a risk factor for allergic-type contrast reactions)

Type 4—contact dermatitis, a delayed hypersensitivity reaction Avoid offending agents Serum sickness

1068

Last-Minute Review—Allergy and Immunology A common trigger of allergic reactions in a patient with spina bifida or congenital urogenital problems What is the most common specific autoimmune association with chronic urticaria? Sudden onset of lip swelling, abdominal pain, swelling of both feet, non-pruritic erythematous skin rash; one family member has the same condition What is the cause of hereditary angioedema (HAE)? Initial screening test for a patient with suspected hereditary angioedema The test that can differentiate between various types of hereditary angioedema A 6-year-old male with yellow-tan macules located on the upper extremities. Parents notice localized erythema following scratching of the lesions and after taking a hot shower Common diagnostic lab for mastocytosis A 15-year-old male presents with several erythematous, pruritic circumscribed lesions that occur with exercise Child presents a few minutes after eating peanut butter with urticaria, skin flushing, pruritus, angioedema, rhinorrhea, wheezing, shortness of breath, abdominal pain, vomiting, diarrhea, lightheadedness. What is the next best step? Child with a history of life-threatening reaction to a bee sting is coming to your office for a follow-up after he was discharged from the ER with EpiPen prescription. What is the next best step? A 4-year-old male scheduled for a well child check; he was recently treated with a 5-day course of oral steroids for asthma exacerbation. Which vaccines can be given? A 4-year-old male scheduled for a well child check; he has been treated with high dose steroids for 4 weeks. Should the MMR and varicella vaccines be given? Child is being treated with intranasal steroids for allergic rhinitis. Should the MMR and varicella vaccines be given? What is the best initial test for any child with suspected immunodeficiency?

O. I. Naga

Most Likely Answer Latex Autoimmune thyroid disease (laboratory evaluation should include thyroid-stimulating hormone (TSH) level and thyroid antibodies) Hereditary angioedema

Low levels or decreased function of plasma protein C1 inhibitor (C1-INH). (Autosomal dominant) C4 level most reliable and cost-effective screening test for HAE C1-INH functional assay Mastocytosis—Darier sign: urticaria after stroking lesions Elevated tryptase levels Exercise-induced urticaria IM epinephrine to administer as quickly as possible

Referral to an allergist for immunotherapy

All vaccines including MMR and varicella

No—patients receiving high steroids for greater than 2 weeks should be off steroids for at least 1 month Yes Complete blood count (CBC)

33  Last-Minute Review

Last-Minute Review—Allergy and Immunology Patient with recurrent meningococcal meningitis Initial screening test for a patient with suspected complement deficiency, e.g., recurrent (Neisseria meningitidis) meningitis Complement deficiency that increases the risk of systemic lupus erythematosus What is the best screening test for cell-mediated immunity associated with T-cell defects? What is the best initial test for an infant with suspected humoral immune deficiency? An 8-week-old boy presents with diarrhea, pneumonia, persistent oral thrush, eczematous-like skin lesions, sepsis, lymphopenia, and failure to thrive The enzyme deficiency that is found in SCID? A 9-month-old boy, previously healthy, presents with recurrent otitis media, 2 episodes of pneumonia in the last 2 months, persistent giardiasis. O/E: the lymph nodes, the tonsils are absent Adolescent presents with recurrent sinus and pulmonary infections due to encapsulated bacteria, malabsorption, hepatosplenomegaly, and low level of immunoglobulins (IgG, IgM, and IgA) The best treatment for a child with asymptomatic transient hypogammaglobulinemia of infancy An 8-year-old boy presents with eczema, recurrent Staphylococcus aureus skin infections without inflammatory response “cold abscess,” pneumatoceles, coarse facial features, eosinophilia, and IgE level is 80,000 IU A 5-month-old presents with Pneumocystis jiroveci pneumonia, mouth ulcers, severe neutropenia, recurrent sinusitis, otitis media, chronic diarrhea, failure to thrive, and negative HIV A 4-year-old boy with recurrent skin abscesses, spleen and liver abscesses, and osteomyelitis Test of choice in a patient with a suspected chronic granulomatous disease Severe progressive infectious mononucleosis and Epstein–Barr virus (EBV) fulminant hepatitis Highly elevated WBC in a 10-week-­old infant who still has an umbilical cord Test of choice in a patient with suspected leukocyte adhesion defect

1069

Most Likely Answer The defect in terminal complement C5–C9 deficiency (CH50) test C2 deficiency T-cell phenotyping (CD4/CD8, memory vs. naïve T cells) and T-cell proliferative responses Immunoglobulin levels Severe combined immunodeficiency (SCID)

Adenosine deaminase deficiency X-linked agammaglobulinemia (usually starts after first 6 months of life)

Common variable immunodeficiency

Observation (no treatment is necessary) Job syndrome (autosomal dominant hyper-IgE syndrome)

X-linked hyper IgM syndrome

Chronic granulomatous disease (X-linked) DHR oxidation is preferred, NBT reduction can be used X-linked lymphoproliferative syndrome (Duncan syndrome) Leukocyte adhesion defect type I Flow cytometry beta 2 integrin CD11b/CD18 on leukocytes

1070

Last-Minute Review—Allergy and Immunology Newborn with hypocalcemia, tetralogy of Fallot, interrupted aortic arch, and abnormal facial features Recurrent ear infections, eczema, profuse bleeding during a circumcision procedure, thrombocytopenia, and small platelets Persistent thrush, nail dystrophy, and endocrinopathies Short stature, fine hair, and severe varicella infection Oculocutaneous albinism, recurrent infections, and easy bruising Candidiasis with raw egg ingestion A 4-year-old with short stature, micrognathia, telangiectasia, immunodeficiency, learning disability, deficiency of DNA ligase I An 8-year-old boy presents with recurrent ear and sinus infections, ataxia, oculocutaneous telangiectasia, and elevated α1-fetoprotein

O. I. Naga

Most Likely Answer DiGeorge anomaly (deletion of chromosome 22q11.2) Wiskott–Aldrich syndrome Chronic mucocutaneous candidiasis Cartilage-hair hypoplasia with short-limbed dwarfism Chédiak–Higashi syndrome Biotin-dependent carboxylases deficiency Bloom syndrome Ataxia–telangiectasia (autosomal recessive)

ENDOCRINOLOGY Amr Morsi

Last-Minute Review—Endocrinology What is the first sign of puberty in a boy? What is the first sign of puberty in a girl? The height acceleration peaks in girls is at which sexual maturation rating (SMR) stage? The height acceleration peaks in boys is at which SMR stage? How many years after breast development does menarche start? A 5-year-old female, pubic hair, adult odor, no breast development, bone age is equal to chronological age, slightly increased dehydroepiandrosterone (DHEA) level, normal growth pattern for age A 2-year-old female with bilateral breast buds, unchanged for 1 year, no growth acceleration A 4-year-old female with new-onset bilateral breast enlargement, advanced bone age, and elevated luteinizing hormone (LH) and folliclestimulating hormone (FSH)

Most Likely Answer Testicular enlargement Breast budding Between stage 2 and 3 SMR Between stage 4 and 5 SMR 2.5 years (approximately) Premature adrenarche

Benign premature thelarche Central precocious puberty is very likely

33  Last-Minute Review

Last-Minute Review—Endocrinology A 4-year-old boy presents with an adult-size phallus, pubic and axillary hair, acne, well-defined muscle tone, prepubertal size testicles A 4-year-old boy presents with an adult-size phallus, pubic and axillary hair, acne, well-defined muscle tone, pubertal size testicles, advanced bone age A 4-year old boy with new-onset adult body odor, recent growth acceleration, pubic and axillary hair, thinning of the scrotum, enlargement of both testicles. Elevated LH and FSH. What is the best study to establish the diagnosis? A 5-year-old girl with pubic hair, mild hyperpigmentation of skin folds, slightly enlarged clitoris Second newborn screen positive for high 17-hydroxyprogesterone. What is the next best step? Newborn with proximal hypospadias (e.g., penoscrotal) and cryptorchidism What is the best treatment of congenital adrenal hyperplasia? What is the treatment for a patient with congenital adrenal hyperplasia who presents with vomiting and low blood pressure? A 2-week-old male with failure to thrive, persistent vomiting, dehydration, acidosis Ambiguous genitalia, nephropathy, Wilms tumor, renal failure by 3 years of age Female phenotype at birth with undifferentiated streak gonads, presence of vagina/fallopian tubes, at puberty no breast development/menstruation, development of gonadoblastoma is the highest risk Newborn with a small penis, bifid scrotum, urogenital sinus, blind vaginal pouch, testes are in the inguinal canal, raised as a female, virilization occurs at the time of puberty, enlargement of penis and scrotum, sperm formation, and normal adult height Infant phenotypically female at birth, raised as female, vagina ends in a blind pouch, no uterus, no fallopian tubes, intra-abdominal testes, normal breast development, no menses, normal male adult height, testosterone level is normal

1071

Most Likely Answer Peripheral precocious puberty Central precocious puberty

Brain MRI

Simple virilizing CAH-21 OH deficiency Repeat 17-hydroxyprogesterone test Ultrasonography for internal genitalia, karyotype, and serum electrolytes to screen for congenital adrenal hyperplasia Hydrocortisone and fludrocortisone IV hydrocortisone and IV fluid hydration CAH 21-OH deficiency (pyloric stenosis is associated with metabolic alkalosis) Denys–Drash syndrome Swyer syndrome (XY pure gonadal dysgenesis)

5-alpha reductase deficiency (autosomal recessive)

Androgen insensitivity syndrome; 46, XY (X-linked recessive disorder)

1072

Last-Minute Review—Endocrinology XY normal male phenotype, inguinal hernia, undescended testis, Müllerian structures found incidentally (uterus and fallopian tubes) A 4-year-old with precocious puberty, large caféau-lait spots, skeletal fibrous dysplasia, and vaginal bleeding are associated with A slow growth rate in the first 2 years of life ( 97th percentile, hypertension, acanthosis nigricans; her blood glucose level is 350 mg/dL, A1C is 10%. What is the best initial treatment? What is the A1C goal recommended by the American Diabetes Association for all pediatric age-groups with type 1 diabetes mellitus? A 3-day-old infant, 10 lbs at birth, jittery

O. I. Naga

Most Likely Answer Nephrogenic diabetes insipidus Cushing syndrome Adrenal insufficiency

Addison disease Addison disease IV hydration: 10 mL/kg of intravenous normal saline over 1 h

Diabetic ketoacidosis (DKA) Insulin omission Cerebral edema Fasting blood glucose level Metformin

Insulin Blood glucose level ≥ 250 mg/dL, A1C ≥ 8.5% insulin is the best initial treatment A1C target  2 years of age, unilateral, progressive after “genu varum” 1 year, thigh leg angle > 20°, suspected rickets or associated deformities A 3-year-old African-American girl with obesity Blount disease has severe progressive genu varum; plain radiograph shows proximal metaphyseal beaking

33  Last-Minute Review

Last-Minute Review—Orthopedics Basketball player presents with left knee pain, recurrent effusion, quadriceps atrophy, and pain with range of motion; plain radiograph shows subchondral fragment with a lucent line separating it from the condyle A 13-year-old female with right knee pain; she feels that her knee cap is unstable, parapatellar tenderness, plain radiograph sunrise view shows lateral tilt of patella A 5-year-old has cystic mass in the back of the left knee for 3 months, it is painless, with no tenderness, normal range of motion The best management of Baker cyst Knee pain with prolonged sitting, activity, and climbing or descending stairs, feeling of knee instability. Tenderness over the medial patellar facet, pain with patellar compression, and mild swelling The best management of patellofemoral pain syndrome The most common cause of intoeing in children > 3 years A 7-year-old girl, patellae are looking inward (kissing patellae), running like an egg-beater, always sitting in W position, internal rotation of the hip is more than external rotation Management of femoral anteversion Are shoe wedges, twister cables, night splint, or discouraging W-sitting effective in cases of femoral anteversion? The most common cause of intoeing in children between 18 months and 3 years A 2-year-old with both feet pointing medially, especially when running, patellae in both legs are pointing anteriorly. The child trips frequently Management of internal tibial torsion

1079

Most Likely Answer Osteochondritis dissecans

Recurrent patellar subluxation and dislocation

Popliteal cyst (Baker cyst) Observation for 12 months Patellofemoral pain syndrome (PFPS)

Ice, rest, NSAID, quadriceps and hamstring strengthening Femoral torsion Femoral anteversion

Reassurance (spontaneous resolution in more than 80% of the cases) Showed to be ineffective Tibial torsion Internal tibial torsion Reassurance (almost all cases resolve spontaneously) Metatarsus adductus

A 4-month-old with a curved foot; by drawing an imaginary line bisecting the foot, it passes laterally to the fourth toe The best management of metatarsus adductus Observation (if persists beyond 6 months and deformity is rigid, a referral is necessary) Newborn with a deformed foot; the foot can be Postural or positional (calcaneovalgus foot) this everted and dorsiflexed (the foot touches the is not a clubfoot anterior tibia)

1080

Last-Minute Review—Orthopedics The best management of calcaneovalgus foot

O. I. Naga

Most Likely Answer Observation—condition due to the intrauterine position Newborn male infant with turned inward right foot. Clubfoot or congenital talipes equinovarus The right foot can be passively stretched almost to (TEV) the midline. The ankle is in equinus (downward), the foot is supinated (varus) and adducted, dorsiflexion beyond 90° is not possible Best management of clubfoot Serial casting (requires an immediate referral) The most common neurological conditions Myelomeningocele and cerebral palsy associated with clubfoot The most common condition associated with cavus Charcot–Marie–Tooth syndrome foot A mother is concerned that her 6-month-old has a Reassurance (medial arch of the foot does not flat foot develop until 4 years of age and reaches adult value by 8 years) A 3-year-old child with tiptoe walking, normal Physical therapy for 6 months for Achilles neurological examination, the best course of action tendon stretching; if no improvement, orthopedic referral A 15-year-old presents with progressive back Scheuermann kyphosis deformity, plain radiographs of the thoracic spine shows 3 adjacent wedged vertebral bodies of at least 5° A 12-year-old female has spinal scoliosis detected Adolescent idiopathic scoliosis (AIS) by school nurse; the scoliometer measures 7° Cases with AIS should be referred to orthopedic if Scoliometer 7° or more, Cobb angle > 20° Management of female adolescent with AIS and Bracing (if skeletal growth remaining) Cobb angle > 25° Management of female adolescent with AIS and Usually, surgery is required Cobb angle > 50° The indication for MRI in cases with scoliosis Pain, left thoracic curve, abnormal neurological exam, infantile and juvenile types Adolescent with low-back pain for a few months. Mechanical low-back pain The pain is worse after physical activity or prolonged sitting. O/E: pain is exaggerated with lumbar flexion and bilateral rotation. Tenderness to palpation along the lumbar paraspinal muscles; tightness of the hamstring and calf muscles. Normal neurologic examination. No other symptoms. Normal spine radiograph What is the best management in the previous case? Physical therapy (lumbar/core strength and stability exercises) A 10-year-old female does gymnastics; presents Spondylolysis with low-back pain that increases with the extension of the spine, plain radiograph shows defect in pars interarticularis, oblique view shows Scotty dog collar sign

33  Last-Minute Review

Last-Minute Review—Orthopedics A 10-year-old female does gymnastics; presents with low-back pain that increases with the extension of the spine, plain radiograph shows forward slippage in L5 over S1 Best initial management of spondylolysis Management of spondylolisthesis A 15-year-old boxer complaining of dull pain in radial aspect of the right wrist that is exacerbated by clenching, and tenderness in the anatomic snuffbox; plain radiograph on the right wrist is negative The best management of scaphoid fracture The motor manifestation of posterior interosseous nerve injury The motor manifestation of radial nerve injury The motor manifestation of ulnar nerve injury The motor manifestation of median nerve injury The most common sports injury in the knee, e.g., female playing soccer A 14-year-old complains of right shoulder pain after a fall, arm held in abduction, and externally rotated, the shoulder is boxlike. Patient resists adduction and internal rotation, plain radiograph shows a subcoracoid position of the humeral head in the AP view and humeral head lies anterior to the “Y” in an axillary view A 14-year-old complains of right shoulder pain after an electric shock, the arm is held in adduction and internal rotation, patient resists external rotation and abduction. Plain AP radiograph shows a humeral head that resembles an ice cream cone. The scapular “Y” view reveals the humeral head behind the glenoid (the center of the “Y”) Child with anterior shoulder dislocation loses the pinprick sensation in the deltoid Right shoulder pain after a fall during basketball practice, prominent clavicle with loss of the normal contour of the shoulder, shoulder radiographs show separation between the clavicle and acromion Right shoulder pain after a fall during basketball practice directly onto the lateral aspect of the right shoulder, pain when adducting the arm across the chest, there is mild swelling and tenderness at the distal end of the clavicle, shoulder radiographs are normal

1081

Most Likely Answer Spondylolisthesis

NSAID and rest Referral to orthopedics Possible scaphoid fracture. (Radiograph is usually negative in the first 2 weeks). Treat if highly suspected Thumb spica splint and repeat radiograph in 2 weeks Finger drop (inability to extend the fingers at the metacarpophalangeal joint) Wrist drop and finger drop Partial claw hand Inability to flex the index finger Anterior cruciate ligament (ACL) injury Anterior shoulder dislocation

Posterior shoulder dislocation

Axillary nerve injury (check axillary nerve sensation before and after reduction) Acromioclavicular joint disruption

Acromioclavicular joint sprain

1082

Last-Minute Review—Orthopedics The most common ligaments affected in ankle sprain

O. I. Naga

Most Likely Answer Lateral ligaments of the ankle (anterior talofibular most common, calcaneofibular, and posterior talofibular ligaments) When can a patient with an ankle sprain go back to If no pain and painless range of motion sports? The best way to differentiate between an ankle Bony tenderness is usually a fracture sprain and fracture A 2-year-old boy fell 2 h ago; now he is refusing to Apply a cast on the left lower extremity and walk. He appears to have tenderness over the distal repeat radiography in 2 weeks (possible toddler third of the left tibia. Radiographs of lower fracture) extremities are normal. What is the next best step? A 12-year-old boy had a fracture of right tibia, Compartment syndrome. Presence of pain fixed with an above-knee cast. He continues to despite fracture immobilization and pain have pain afterward, the pain keeps getting worse medication is a red flag for compartment despite the maximum dose of prescribed pain syndrome medicine, any movement of the toes causes him excruciating pain, also he has numbness between the first 2 toes What is the next best step in the previous case of Report immediately to the nearest ER compartment syndrome? (immediate removal of cast and orthopedic consultation) The most common orthopedic complication of Compartment syndrome snake bite in the extremities A 12-year-old with right knee trauma. Knee Fibrous cortical defect (non-ossifying fibromas), radiograph showed no fracture but incidentally Most cases are accidentally discovered in found a small, well-defined radiolucent cortical radiographs taken for other reasons. No lesion with a surrounding rim of sclerosis in the treatment is required in most cases upper tibia. The longitudinal axis of the lesion is parallel to the axis of the tibia A 12-year-old with severe pain in the upper part of Osteoid osteoma the right tibia at night improved dramatically with ibuprofen; radiograph showed 1.5 cm sharp round lesion (nidus) surrounded by a rim of radiodensity A 2-year-old child suddenly stops moving his right Nursemaid elbow arm after his brother forcibly pulled his hand Short, webbed neck decreased the range of motion Klippel–Feil syndrome in the cervical spine, low hairline. Fusion of cervical vertebrae on radiograph Common associations with Klippel–Feil syndrome Sprengel’s deformity (elevation of the scapula), thoracolumbar anomalies, renal and cardiac anomalies

33  Last-Minute Review

1083

SPORTS MEDICINE Daniel Murphy

Last-Minute Review—Sports Medicine Adolescent plays football, presents with pain in the right knee, swollen tender tibial tubercle; plain radiograph shows ossification of the tibial tubercle with fragmentation What is the best treatment of Osgood–Schlatter disease?

Most Likely Answer Osgood–Schlatter disease

Icing, muscle stretching, and strengthening decrease activities triggering the pain, NSAIDs, knee brace Sever disease

An 8-year-old boy recently starts playing basketball for 2 h every day, he is complaining of pain in both heels, the pain is worse when jumping and with exercise. The exam is positive for tenderness at both heels and and with dorsiflexion of both feet What is the best treatment of Sever disease? Icing, stretching of Achilles tendon, NSAIDs, heel cups, avoid activities triggering the pain, e.g., jumping Adolescent girl is complaining of right hip pain Anterior inferior iliac spine (AIIS) apophyseal that developed during track practice. She felt a pop avulsion injury (due to strong muscle contraction in the front of her right hip and had immediate and skeletal immaturity and relatively weak onset of pain. There is tenderness over anterior apophysis) inferior iliac spine, pain and weakness with resisted hip flexion, and pain with passive extension of the hip. What is the most likely diagnosis? Healthcare providers should educate athletes at Strengthening, balance, and plyometric (jump) high risk for ACL tear about neuromuscular training, proper knee position and technique training programs that reduce the risk of injury. What are the components of neuromuscular training? What are the highest rates of dental injuries among Basketball, baseball, wrestling, and soccer high school sports in boys? What are the highest rates of dental injuries among Field hockey, softball, basketball, and lacrosse high school sports in girls? What is the best protective gear during sports that Mouthguard decreases oral injuries, including tooth avulsions, tooth fractures, and lacerations? Adolescent with a history of 3 concussions is Neuropsychological testing suffering from forgetfulness and difficulty concentrating since last concussion, which was 2 months ago. What is the best diagnostic test to identify impaired cognitive function?

1084

Last-Minute Review—Sports Medicine Adolescent with a history of scoliosis is in your office for sports clearance. Physical examination is significant for pectus excavatum, hypermobility of the wrist and finger joints, long and thin fingers, arm span–to–height ratio that is greater than 1.05 What is the next best step? What is the best treatment of apophyseal avulsion injuries? Adolescent boy presents to your office for preparticipation evaluation before football season. He has a history of 2 episodes of syncope. What is the most important finding that warrants a through cardiology evaluation? What is the best drink for sports activities lasting 1 h or less? What is the best drink for sports activities lasting longer than 1 h?

O. I. Naga

Most Likely Answer Echocardiography (Marfan syndrome is a high risk of aortic root rupture and aortic dissection)

Rest and protected weight-­bearing; surgery is rarely indicated Syncope during exercise

Water is enough for hydration

Sports drinks (contain electrolyte and sugar; may encourage for hydration with longer activities) A father is asking about energy drinks for his Not recommended for young children and children during sports adolescents (due to high caffeine level in energy drinks) An athlete has been doing strenuous exercise for a Heat exhaustion prolonged period in hot weather, excessively sweating, dizziness, thirst, weakness, headache, malaise, tachycardia, and the temperature is 103 °F An athlete has been doing strenuous exercise for a Heat stroke prolonged period in hot weather, presents with dry and hot skin, feeling dizzy, weak, altered mental status, low blood pressure, and tachycardia, and the temperature is 105 °F What is the next best step in an athlete with Rapid and immediate cooling (ice water bath) at suspected heat stroke? the scene then transfer to the ED An athlete is complaining of headaches, dizziness, Remove from sports. Neurocognitive rest poor concentration after a head concussion while playing volleyball. What is the next best step? Adolescent boy is presenting with a large swelling Soft-tissue hematoma in the left thigh after hitting another player during the game, radiograph on the left femur is normal. What is the most likely diagnosis? A 17-year-old boy is interested in building Anabolic steroids intake muscles, the mother is concerned about his acne, aggressive behavior. Gonads are small for age. What is the most likely underlying cause?

33  Last-Minute Review

1085

RHEUMATOLOGY Dawn M. Wahezi

Last-Minute Review—Rheumatology A 7-year-old with morning stiffness, knee and ankle swelling for 2 months, ESR is normal, antinuclear antibody (ANA) 1:160 A 2-year-old female with recently diagnosed oligo JIA and positive ANA requires frequent screening for this comorbidity Fatigue, weight loss, arthritis in multiple joints for > 6 weeks, positive RF, anti-cyclic citrullinated peptide antibodies present and ANA is negative Fatigue, weight loss, no fever, arthritis in multiple joints, negative RF, ANA is positive A 5-year-old girl recently diagnosed with JIA, her ANA is positive; how frequently does she need screening for uveitis? A 9-year-old girl recently diagnosed with JIA, her ANA is positive, how frequently does she need screening for uveitis? An 8-year-old with knee pain for 6 weeks, noted to have pain, swelling, decreased range of motion, difficulty bearing weight, synovial fluid shows decreased viscosity and WBC 15,000 Fever, salmon-colored rash with fever and hot showers, arthritis in major joints, hepatosplenomegaly, leukocytosis, thrombocytosis, anemia of chronic disease, elevated ESR, negative RF, and negative ANA Side effects include immunosuppression, adrenal suppression, weight gain, cushingoid facies, diabetes and acne Child with systemic JIA presents with elevated liver enzymes, prolonged PTT, positive D-dimer, thrombocytopenia, hyperferritinemia, and low ESR Malar rash, arthritis, proteinuria, leucopenia, thrombocytopenia, positive ANA, and anti-dsDNA An African-American girl with pericarditis, pleurisy, recurrent oral ulcers, hemolytic anemia, and RBC casts in urine This test is very sensitive but not specific for SLE Autoantibodies associated with arterial/venous thrombosis or recurrent miscarriage (in patients with or without SLE)

Most Likely Answer Oligoarticular juvenile idiopathic arthritis (JIA) Chronic anterior uveitis Polyarticular JIA Polyarticular JIA with an increased risk of uveitis Every 3–4 months (JIA,  7 years and positive ANA) Inflammatory arthritis

Systemic JIA

Corticosteroids Macrophage activation syndrome Systemic lupus erythematosus (SLE) SLE Antinuclear antibody (ANA) Antiphospholipid antibodies

1086

Last-Minute Review—Rheumatology The most severe type of lupus nephritis resulting in hematuria, proteinuria, elevated blood pressure and can lead to end-stage renal disease Neonate born with heart block, annular erythematous plaques, anemia, thrombocytopenia, and elevated liver enzymes, positive SSA (Ro) and SSB (La) antibodies Recurrent parotitis, xerophthalmia, conjunctivitis, xerostomia, positive ANA, RF, and anti-Ro A 7-year-old female, with proximal muscle weakness in both sides, arthralgia, heliotrope rash, elevated creatine phosphokinase (CPK) and LDH A 6-year-old female with difficulty climbing stairs and voice change, Gottron papules and abnormal nailfolds, and telangiectasias A 15-year-old had diarrhea positive for Yersinia 2 weeks ago, now is having conjunctivitis, urethritis, arthritis of the hip and knee Adolescent with inflammatory bowel disease (IBD) has arthritis An 8-year-old, pain in the sacroiliac joint, tenderness, stiffness and joint pain in the morning that improves with activity, and positive HLA-B27 Child with nail pitting, psoriasis, arthritis, positive ANA Adolescent with recurrent oral and genital ulcers, positive pathergy test Adolescent girl with chronic left foot pain, minimal touch aggravates the pain, foot is swollen, warm to touch, and mottled skin A 7-year-old boy with pain in both legs, worse in the evening, sometimes awakens him from sleep, no fever, no limping, joints are normal on exam, pain responds to ibuprofen and heat massage Adolescent, 1 year with fatigue, multiple areas of pain, tenderness, no signs of inflammation, and labs are normal A 5-year-old boy with acute onset palpable purpura on lower extremities, joint swelling and abdominal pain, labs reveal normal CBC, PT and PTT, positive proteinuria An 18-month-old with fever for 6 days, rash, conjunctivitis, strawberry tongue, and erythema of the palms and soles The primary treatment for Kawasaki disease, ideally given between day 5 and 10 of illness

O. I. Naga

Most Likely Answer Diffuse lupus nephritis (membranoproliferative, class IV) Neonatal lupus

Sjögren syndrome Juvenile dermatomyositis Juvenile dermatomyositis Reactive arthritis Arthritis-related to IBD Enthesitis-related arthropathies Juvenile psoriatic arthritis Behçet disease Complex regional pain syndrome or reflex sympathetic dystrophy (RSD) Growing pain

Fibromyalgia Henoch-Schönlein purpura (HSP)

Kawasaki disease IVIG, aspirin

33  Last-Minute Review

Last-Minute Review—Rheumatology A 15-year-old female with fatigue, weight loss, recurrent sinusitis, and joint pain. Labs reveal hematuria, proteinuria, and positive cANCA Pain, numbness, and discoloration of fingers (white then blue then red) that is triggered by cold weather A 12-year-old female with a linear hyperpigmented lesion on her leg that appears white and sclerotic in the center and has an erythematous border A 14-year-old female with severe Raynaud disease with ulceration on her fingertip, tightening of her skin, sclerodactyly, positive ANA, positive anti-Scl-70 A chest radiograph with pulmonary infiltrates and hilar adenopathy, biopsy with non-caseating granulomas, elevated ACE Recurrent knee arthritis in an otherwise well child with a prior history of camping in the woods Newborn infant presents a few hours after birth with hypotonia, facial muscle weakness, ptosis, weak cry, respiratory distress, the mother has a history of muscle weakness Adolescent with muscle weakness, worsens with repetitive movement and at the end of the day, difficulty breathing, abnormal ocular movement A 3-year-old child from the Middle East, recurrent fever and abdominal pain, during the episode the ESR and CRP are elevated, WBC 25,000 Child with fever for 3–5 days every month associated with mouth ulcers, throat pain, cervical lymphadenitis. The patient is well in- between episodes

1087

Most Likely Answer Granulomatous polyangiitis (GPA, formerly Wegener granulomatosis) Raynaud’s disease Localized scleroderma Systemic sclerosis

Sarcoidosis Lyme arthritis Neonatal myasthenia gravis

Juvenile myasthenia gravis Familial Mediterranean fever PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis)

NEUROLOGY Ivet HartonianJong W. Yoo, and Jason T. Lerner

Last-Minute Review—Neurology Most Likely Answer An 18-month-old turns cyanotic associated with a Reassurance (cyanotic breath-holding spell) brief loss of consciousness that occurred during a temper tantrum An 18-month-old turns pale with limp jerking and Reassurance (pallid breath-holding spell) brief loss of consciousness that occurred after the child was scared by his sibling

1088

Last-Minute Review—Neurology A 4-month-old infant is having episodes of tonic neck extension and dystonic posturing of trunk associated only with feedings. Has a normal neurologic exam A premature infant has brief jerking of the right upper extremity that cannot be suppressed A previously healthy 16-month-old boy has 60 s generalized seizure in the setting of febrile illness (not involving the CNS) and is now acting normal A previously healthy 16-month-old boy has had 2 febrile seizures in the last 24 h, and the infant is now acting normal What is the recurrence risk after the first simple febrile seizure? What is the risk of developing epilepsy in children with simple febrile seizures? An 8-year-old boy is having multiple brief daily episodes of behavioral arrest and eye fluttering with an EEG showing 3 Hz/s spike-and-wave discharges The first-line treatment for absence seizures Antiseizure medication that should be avoided in women of child-bearing age due to teratogenicity A 6-month-old infant having episodes of tonic flexion of trunk, head, and extremities, occurring in clusters Triad of infantile spasms, hypsarrhythmia on EEG, developmental regression A 3-year-old boy with a prior history of infantile spasms who now has intellectual disability, multiple seizure types, EEG showing slow spikewave activity An 16-year-old girl who is an excellent student has a generalized tonic-clonic seizure after a sleepover party with her friends. She also reports having jerking movements of her arms in the mornings A 5-year-old with nighttime seizures involving the face and focal centrotemporal spikes in sleep A 3-year-old with language regression and continuous spike-wave discharges in slow wave sleep A 9-year-old previously healthy girl with intractable focal seizures as well as hemiparesis and cognitive decline. MRI of the brain shows atrophy of one hemisphere

O. I. Naga

Most Likely Answer Sandifer syndrome

Neonatal seizure Simple febrile seizure Complex febrile seizures Approximately 30% Approximately 2% Absence seizure (petit mal seizure)

Ethosuximide Valproic acid Infantile spasms West syndrome Lennox–Gastaut syndrome

Juvenile myoclonic epilepsy

Rolandic epilepsy with centrotemporal spikes Landau–Kleffner syndrome Rasmussen encephalitis

33  Last-Minute Review

Last-Minute Review—Neurology An infant with rapid head growth, full fontanel, irritability, vomiting The most common cause of macrocephaly An infant with failure to thrive, developmental delay, intractable seizures with an MRI showing a “smooth brain” Elevated maternal alpha-fetoprotein, an infant born with a large cranial defect, abnormalities of the face and eyes, without a cortex but an intact brainstem Global intellectual disability, brain MRI showing bilateral clefts within the cerebral hemisphere An infant with a sacral tuft of hair and normal neurologic exam MRI showing downward displacement of the cerebellar tonsils through the foramen magnum Cystic expansion of the fourth ventricle in the posterior fossa, associated with hydrocephalus, cerebellar ataxia, and associated with corpus callosum agenesis Newborn with a skull defect, a sac-like protrusion containing brain material Infant born with a short neck, very low hairline in the back of the head, and limited range of motion in the neck Child with a stroke-like event has MRI with the appearance of a “puff of smoke” Adolescent girl presents with ptosis and double vision and is also complaining that she feels weaker by the end of the day Preschool age boy has a history of toe walking, frequent falls, and enlarged calves. O/E: he has significant proximal muscle weakness, positive Gower sign, and laboratory evaluation shows elevated CPK (creatine phosphokinase) A 10-year-old boy presents frequent falls, and weakness for 3 months. O/E: he has mild proximal muscle weakness, and calf pseudohypertrophy and his echocardiogram shows cardiomyopathy, laboratory shows elevated serum CPK Newborn infant presents with hypotonia, respiratory distress, and facial muscle weakness. The genetic test is positive for CTG trinucleotide repeat

1089

Most Likely Answer Hydrocephalus Benign familial macrocephaly Lissencephaly Anencephaly

Schizencephaly Spina bifida occulta Arnold–Chiari malformation Dandy–Walker syndrome

Encephalocele Klippel–Feil syndrome Moyamoya disease Juvenile myasthenia gravis Duchenne muscular dystrophy

Becker muscular dystrophy—older onset and milder form

Congenital myotonic dystrophy

1090

Last-Minute Review—Neurology Group of disorders characterized by decreased deep tendon reflexes, decreased proprioception, and a vibratory sense that is caused by defective peripheral nerve demyelination A 12-year-old girl previously healthy over few months presents with progressive clumsiness, gait and limb ataxia, recurrent falls, rapid, jerky movements of both eyes, areflexia, lower extremity weakness, dysarthria, and dysphagia. Positive frataxin gene sequencing, associated with cardiomyopathy and increased risk of diabetes mellitus A 3-year-old girl presents with recurrent respiratory infections, clumsiness, gait and limb ataxia, recurrent falls, oculomotor apraxia, choreoathetosis, dysarthria, and ocular telangiectasias, and serum α-fetoprotein is elevated Adolescent with a history of herpes zoster presents with peripheral nerve paralysis, facial pain, and deafness History of diarrhea followed by progressing ascending weakness and loss of deep tendon reflexes with CSF showing elevated protein A 4-month-old infant with severe hypotonia and feeding difficulty. O/E: the infant is in frog-leg position and has tongue fasciculations Progressive weakness in legs with focal back pain, bowel and bladder dysfunction and sensory level on the exam. Eventually develops into spastic diplegia A 6-month-old consistently reaches for toys with the right hand. What is the most likely underlying cause? An 18-month-old boy with a history of prematurity, including bilateral intraventricular hemorrhages, who is brought in for evaluation because he is not walking and has increased tone in his legs. Scissoring of the legs is noted when he is held in a vertical position Child with dyskinetic cerebral palsy and a history of elevated bilirubin A school-age child develops abnormal limb movements a few weeks after a group A betahemolytic strep infection

O. I. Naga

Most Likely Answer Charcot–Marie–Tooth syndrome

Friedreich ataxia—GAA trinucleotide repeat (autosomal recessive and presents in older children)

Ataxia–telangiectasia (autosomal recessive and presents in young children)—mutation of the ATM gene Ramsay Hunt syndrome Guillain–Barré syndrome Spinal muscular atrophy Transverse myelitis

Central or peripheral neurologic abnormality of the opposite side, including hemiparesis Spastic diplegic cerebral palsy

Kernicterus Sydenham chorea

33  Last-Minute Review

Last-Minute Review—Neurology An 18-month-old girl with acquired microcephaly, language regression, repetitive hand-wringing movements, loss of purposeful hand use. Genetic testing reveals a mutation in the MECP2 gene Adolescent girl is complaining of right frontal pulsating headache with photophobia and nausea. She reports that during the headache episodes, she prefers to be in a dark, quiet room. Her father and paternal grandmother also get headaches Adolescent complaining of a mild headache described as “band-like” around the head. Headache is responsive to over-the-counter analgesics Adolescent girl with a BMI more than 98th % presenting with headaches, nausea, vomiting, double vision, papilledema, and inability to abduct her left eye. What is the most likely underlying cause? A 1-year-old girl presents with an increasing number of café-au-lait spots all over her body. She has more than 6 macules, each macule measuring more than 5 mm. The rest of the exam is normal. No family history of neurofibromatosis. What is the next best step? The child in the previous example is positive for Lisch nodules. What are the recommendations? Multiple café-au-lait spots, Lisch nodules on ophthalmology exam, and presence of multiple neurofibromas Presents with ringing in the ears; imaging shows bilateral vestibular schwannomas A 10-month-old infant presents with infantile spasms and is noted to have multiple hypomelanotic macules (ash leaf spots). MRI brain shows cortical tubers History of port-wine stain, seizures, and glaucoma Child presenting with chronic back pain, lower extremity weakness, leg length discrepancy, foot deformities, scoliosis, neurogenic bladder, and recurrent urinary tract infections. There is no acral hair or skin abnormalities in the lower back Toddler refusing to walk or stand, with back tenderness and elevated ESR

1091

Most Likely Answer Rett syndrome

Migraine headache

Tension headache

Idiopathic intracranial hypertension (pseudotumor cerebri)

Referral to a pediatric ophthalmologist to check for Lisch nodules annually

Genetic consultation, annual pediatric ophthalmology evaluation, regular developmental assessment, and regular blood pressure monitoring Neurofibromatosis type 1 Autosomal dominant Neurofibromatosis type 2 Autosomal dominant Tuberous sclerosis complex

Sturge–Weber syndrome Tethered cord syndrome

Diskitis

1092

Last-Minute Review—Neurology The child with a ventriculoperitoneal (V/P) shunt with a triad of high fever, focal neurologic deficits, and headache Child with recurrent throat clearing, facial grimacing, and grunting, but otherwise acting normal What is the first-line therapy for tic disorder with no other complications? An infant with nystagmus, titubation, and torticollis A teenager with excessive daytime sleepiness, sudden episodes of loss of muscle tone and hallucinations when going to sleep A 9-year-old has a sudden onset of severe abdominal pain, becomes pale, feels dizzy, and her vision slowly goes black followed by loss of consciousness for a few seconds and quick return to baseline The reflex that appears around 8–9 months in preparation for a child to stand and walk

O. I. Naga

Most Likely Answer Shunt infection—possible brain abscess Tic disorder (Tourette syndrome if symptoms > 12 months) Behavioral modification Spasmus nutans Narcolepsy Vasovagal syncope

Parachute reflex

OPHTHALMOLOGY Violeta Radenovich

Last-Minute Review—Ophthalmology A 5-day-old infant with severe bilateral purulent conjunctivitis and severe conjunctival chemosis. What is the most likely organism? A 5-day-old newborn presents with severe bilateral purulent conjunctivitis, severe conjunctival chemosis. What is the best treatment? A 10-day-old infant with mild to moderate purulent discharge also associated with a cough and congestion. What is the most likely organism? A 14-day-old infant presents with mucoid discharge from both eyes and eyelid swelling. What is the best treatment? Excessive tearing, photophobia, frequent spasms of the eyelid, corneal clouding and enlargement of the eye A newborn is being evaluated in the office for leukocoria. The reflexes are absent in both eyes. What is the next best step?

Most Likely Answer Neisseria gonorrhoeae conjunctivitis IM or IV 3rd generation cephalosporin, topical erythromycin, ophthalmology consultation Chlamydia conjunctivitis Oral erythromycin. Erythromycin ophthalmic ointment 4 times a day for 1 week Congenital glaucoma (immediate referral to pediatric ophthalmology) Immediate referral to ophthalmology—concern for cataract or retinoblastoma

33  Last-Minute Review

Last-Minute Review—Ophthalmology An 8-week-old male infant with right eye more watery than the left. There is a golden-colored crust on his eyelashes, more prevalent in the morning. No redness What is the best initial treatment of nasolacrimal duct obstruction? Most of the cases of nasolacrimal duct obstruction spontaneously resolve at what age? A 2-month-old baby boy presents with alternating deviations in both eyes, no other symptoms A 2-month-old infant presents with left eye deviated inward with no other symptoms The infant in the previous example continued to have left eye deviation at 4 months well visit How long can a newborn be monitored for poor tracking, lack of fixation, head tilt, nystagmus, or squinting? A 9-month-old boy with crossed eyes. O/E: corneal light reflex is centered in both pupils equally; cover test shows no ocular deviation A 9-month-old boy with crossed eyes. O/E: corneal light reflex is asymmetric; the cover test shows ocular deviation Red reflex is asymmetric, absent, dull, or opaque; dark spots in the red reflex; or leukocoria (white reflex). What is the next step? What is the major consequence of delaying the treatment of strabismus or cataract in pediatric patients? A 6-month-old infant presents with nystagmus, head nodding, and torticollis. The nystagmus is disconjugate, high frequency, small amplitude, pendular, and intermittent Child presents with swelling in the eyelid, hyperemia, normal vision, no pain with eye movement, no decrease in eye movement. What is the most likely diagnosis? Child with a fever, malaise, proptosis, decreased vision, pain with eye movement, orbital pain and tenderness, decreased eye movement, dark red discoloration of the eyelids, chemosis, hyperemia of the conjunctiva. What is the most likely diagnosis? Child is presenting with a painful, warm, swollen, red lump on the eyelid. What is the best treatment?

1093

Most Likely Answer Nasolacrimal duct obstruction (topical antibiotic if suspected bacterial infection) Lacrimal sac massage 2–3 times daily 6 months to 1 year with no need for probing or surgery Strabismus—if both eyes are alternating, monitor till 3 months of age (refer if persists) Strabismus—if only one eye is deviating, refer to ophthalmology to exclude underlying pathology Referral to a pediatric ophthalmologist If persist beyond 3 months of age must be referred to a pediatric ophthalmologist Reassurance (pseudostrabismus) Referral to a pediatric ophthalmologist Referral to ophthalmologist Amblyopia (lazy eye) Spasmus nutans (often disappears after a few years)—brain MRI on spasmus nutans patients to rule out optic nerve glioma that can present exactly like spasmus nutans Periorbital cellulitis (may be treated with an oral antibiotic as an outpatient) Orbital cellulitis (admit for IV antibiotics and ophthalmology consultation)

Warm compresses and massages, topical antibiotic if the lesion is draining

1094

Last-Minute Review—Ophthalmology Child is presenting with a painless nodule on the left upper eyelid for 5 months not responding to conservative measures (warm compresses and lid hygiene). What is the next best step? A 5-year-old boy presents with eye pain, foreign body sensation, and tearing after self-inflicted eye injury with a sharp pencil. What is the next best step? Management of corneal abrasion

O. I. Naga

Most Likely Answer Referral to a pediatric ophthalmologist

Examine the eye with fluorescein stain (corneal abrasion)

Topical antibiotic, an oral analgesic, refer to an ophthalmologist if no improvement in 24 h Child is presenting with sudden onset of right eye Ophthalmology consult to rule out corneal discomfort and blurring of vision after exposure to laceration and intraocular foreign bodies flying debris of broken glass. What is the next best step? A 7-year-old is noted to have blood in the anterior Hyphema—emergent ophthalmology consult Sickle cell screening if African-American chamber of the eye after blunt trauma and pain with extra-ocular movements Management of hyphema Ophthalmology consult, 45° bed elevation, bed rest, eye shield, analgesia, sedation, topical cycloplegic, and topical steroids Child is complaining of significant pain, bruising, Orbital floor fractures (due to inferior rectus and swelling in the periorbital area after eye muscle entrapment) trauma; “sunken” appearance to the eye on the affected side; decreased sensation to the cheek, upper lip, and upper gingiva on the affected side; and limitation of upward gaze on the affected side Adolescent girl with obesity is complaining of Papilledema (untreated pseudotumor cerebri can pounding headache, double vision, nausea, and result in permanent vision loss) vomiting; the headache is worse when she is leaning forward. Her vital signs are normal, but she is unable to abduct her right eye. What is the most likely finding in her eye exam? Child with pink eye, fever, cloudy rhinorrhea, Reassurance (pharyngoconjunctival fever cough, headache, pharyngeal redness with scant commonly caused by adenovirus) exudates, a palpable right preauricular lymph node, Treatment: cold compresses to the eyes, profuse tearing, and edematous nasal mucosa. The analgesics, rest, and fluids right eye conjunctiva is hyperemic, and tiny follicles are present on the inner lower lid. What is the best treatment? A 7-year-old girl is noted to have a large bloody Reassurance (viral subconjunctival hemorrhage blotch under the conjunctiva, no history of trauma; [enterovirus, or adenovirus infection]) she has a runny nose and congestion A 7-year-old is noted to have a small area of Reassurance (subconjunctival hemorrhage) unilateral eye redness in the sclera. The redness was noticed after a forceful sneeze

33  Last-Minute Review

Last-Minute Review—Ophthalmology Child with watery, itchy eyes bilaterally, mild eyelid edema, along with conjunctival erythema. No mucoid or purulent discharge Child is being treated for allergic conjunctivitis for 2 weeks with oral and topical antihistamine eye drops with no improvement. What is the next best step? A 3-year-old boy presents with different stage skin bruises; fundus examination shows bilateral multilayered flame shaped retinal hemorrhages. What is the most likely cause? Night blindness, flashes of light, visual loss. O/E: optic nerve waxy pallor, mid-peripheral retinal hyperpigmentation, retinal arteriolar attenuation Pigmentary retinopathy, polydactyly, truncal obesity, kidney dysfunction, short stature Syndromes associated with retinitis pigmentosa and hearing loss A 9-year-old girl with a history of short stature, vision 20/40, her eye exam is significant for optic nerve atrophy. What is the next best step? Risk factors commonly associated with retinopathy of prematurity (ROP) Who should screen preterm infants at risk for ROP? Do preterm infants at risk of ROP should be followed by an ROP experienced ophthalmologist after discharge from the NICU

1095

Most Likely Answer Allergic conjunctivitis Referral to an ophthalmologist (topical ophthalmic steroids require monitoring of eye pressure) Child abuse

Retinitis pigmentosa Bardet–Biedl syndrome Alport syndrome, Waardenburg syndrome, Refsum disease, Usher syndrome Brain MRI (optic nerve atrophy can be associated with a brain tumor) Birth before 30 weeks gestation, or low birth weight  4 year, and adolescents? What is the hallmark sign of otitis externa? Child with persistent purulent otorrhea for more than 2 weeks despite treatment with oral and topical antibiotics Child with persistent otorrhea for more than 6 weeks and not responding to oral and topical antibiotics. What is the most frequent cause? Child presents with persistent ear discharge more than 3 months despite the treatment with multiple courses of topical and systemic antibiotics. What is the most common bacteria associated with chronic suppurative otitis media (CSOM)? Adolescent is complaining of nasal obstruction, pain, and rhinorrhea after nasal trauma. O/E: intranasal cavity reveals a tense red mass on each side of the nasal septum. What is the next best step? Adolescent wrestler with blue swelling and redness in the right ear pinna, occurred during a school match A 7-year-old child had tympanostomy tubes placed 4 years ago because of acute otitis media with effusion and conductive hearing loss. O/E: you clearly visualize a white tympanostomy tube in the right tympanic membrane. What is the next best step? How long is a tympanostomy tube expected to remain in place? Child has otitis media with effusion (OME) less than 3 months Child has OME lasting 3 months with conductive hearing loss

O. I. Naga

Most Likely Answer Aminoglycosides

Cochlear implant Visual reinforcement/behavioral audiometry Play audiometry Conventional audiometry: pure-tone, speech Tenderness of the tragus or pinna Referral to otolaryngologist Cholesteatoma (collection of squamous epithelial cells and keratin within the middle ear) Methicillin-resistant Staphylococcus aureus (MRSA) is most common isolate, Pseudomonas is also a common cause Prompt drainage of nasal septal hematoma to prevent nasal cartilage ischemia, necrosis, and deformity Auricular (ear pinna) hematoma—urgent aspiration of the hematoma and pressure dressing Referral to ENT for surgical removal (tympanostomy tubes that remain in place for longer than 3 years should be surgically removed) 12–18 months Tympanostomy tube insertion is not indicated Tympanostomy tube insertion is indicated

33  Last-Minute Review

Last-Minute Review—Ear, Nose, and Throat Child has recurrent acute otitis media with OME at the time of presentation Child with bilateral or unilateral OME lasting at least 3 months together with risk factors for speech, language, or learning problems (e.g., neurodevelopmental disabilities, craniofacial anomalies) A 5-year-old with nasal discharge, congestion, and cough for 10 days without improvement A 5-year-old with temperature 101.3 °F with purulent rhinorrhea for 3 days A 5-year-old with worsening of nasal congestion or rhinorrhea, cough, and fever after a 3- to 4-day period of improved symptoms Child presents with a 1-week history of fever of 102 °F, cough, bilateral purulent nasal discharge, and frontal sinus tenderness Child presents with nasal congestion, post nasal drip, altered sense of smell, as well as facial pressure for over 3 months Child with an isolated fracture in the paranasal sinus. What is the best treatment?

1097

Most Likely Answer Tympanostomy tube insertion is indicated Tympanostomy tube insertion is indicated

Acute bacterial sinusitis (duration of symptoms) Acute bacterial sinusitis (severity of symptoms) Acute bacterial sinusitis (worsening of symptoms) Acute sinusitis First-line treatment: amoxicillin clavulanate for 10–14 days Chronic sinusitis 1-week course of oral antibiotics and oral analgesics, referral in 1 week to ENT or a surgeon specialized in facial trauma To avoid swimming, blowing the nose, playing wind instruments, and use of drinking straws 10-day course of oral penicillin V

What are the sinus precautions in cases of paranasal sinus fractures? A 6-year-old boy presents with throat pain, fever, headache, and abdominal pain. Rapid antigen detection test for group A Streptococcus is positive. What is the best treatment? A 6-year-old boy presents with the third episode of Clindamycin for 10 days streptococcal pharyngitis in the last 3 months. Rapid antigen detection test for group A Streptococcus is positive. He was treated previously with penicillin V and amoxicillin. What is the next best treatment? A 6-year-old boy presents with throat pain, fever, Clindamycin for 10 days headache, and abdominal pain. Rapid antigen detection test for group A Streptococcus is positive. He had an anaphylactic reaction to penicillin. What is the best treatment? A 6-year-old boy presents with throat pain, fever, Oral cephalosporin for 10 days headache, and abdominal pain. Rapid antigen detection test for group A Streptococcus is positive. He had a non-anaphylactic reaction to penicillin. What is the best treatment?

1098

Last-Minute Review—Ear, Nose, and Throat A 5-year-old boy presents with the insidious onset of fever, sore throat, neck stiffness, tachypnea, drooling, and stridor. Lateral neck radiograph shows thickened prevertebral soft tissues What is the next best step in the previous case with retropharyngeal abscess? Adolescent female presents with fever, sore throat, difficulty opening her mouth, muffled voice, and dysphagia. She has tender anterior cervical lymphadenopathy. Her right tonsil is erythematous and enlarged, pushing her uvula to the left What is the next best step in the previous case with peritonsillar abscess? A 9-month-old presents with fever, ear tugging, and runny nose. Both tympanic membranes are bulging; this is the first ear infection. What is the first-line treatment? The same child presents to the office 3 weeks later with the same symptoms. Which antibiotic should be used? A 2-year-old child presents with fever and purulent conjunctivitis. Physical exam shows bulging of the tympanic membrane. Which antibiotic should be used? Child presents with fever, tenderness, and edema in the postauricular region. Child was diagnosed with otitis media recently, but the parent was not compliant with therapy Newborn child with severe cyanosis that improves with crying. Nurse attempts to pass a 6 French catheter and is not successful Child with chronic nasal congestion, mucoid rhinorrhea, and noisy breathing for several months. No history of recurrent serious bacterial infections and normal weight for age. On examining the nasal passages, you note glistening, bluish-gray, grapelike masses bilaterally. What is the next best test? Children with nasal polyps should be screened for The most common cause of epistaxis in a child A 2-year-old with unilateral rhinorrhea and foul smell from the left nostril. The child is otherwise acting normal? Child presents with runny nose, congestion, itchy eyes, sneezing, and darkened skin around the eyes What is the most conservative method to control allergic rhinitis symptoms?

O. I. Naga

Most Likely Answer Retropharyngeal abscess

Airway management, IV fluids, IV antibiotics, emergent ENT consultation Peritonsillar abscesses

Needle aspiration of her right tonsil (diagnostic and therapeutic) Amoxicillin 90 mg/kg/day for 10 days

Amoxicillin/clavulanate—due to treatment failure within the last 30 days Amoxicillin/clavulanate—likely due to nontypeable Haemophilus influenzae with concurrent bacterial conjunctivitis Mastoiditis—needs ENT consult and IV antibiotics Bilateral choanal atresia—requires immediate airway, can also be unilateral (less severe) and associated with CHARGE Sweat chloride test

Cystic fibrosis Trauma secondary to digital manipulation Foreign body in the nose Allergic rhinitis (under eye circles are called allergic shiners) To avoid allergic triggers such as pets

33  Last-Minute Review

Last-Minute Review—Ear, Nose, and Throat Child has a painless blue mass noted on the right lower lip. The child is otherwise healthy, but the mother is concerned A 4-year-old girl presents with unilateral cervical lymphadenopathy, mild fevers; she was scratched by her new kitten several weeks ago What is the most common complication of an adenoidectomy?

1099

Most Likely Answer Reassurance unless bothersome, then can consider excision (mucocele) Cat scratch fever—Bartonella henselae Hypernasal speech secondary undiagnosed predisposition to velopharyngeal insufficiency (such as submucosal cleft palate)

CARDIOLOGY Grace KungAllison Hill, and Jennifer Su

Last-Minute Review—Cardiology Newborn with cyanosis, pulse oximetry changed from 60% to 64% only on 100% oxygen Newborn with cyanosis, pulse oximetry changed from 60% to 88% on 100% O2 What is the reason that left to right shunt lesions may not present until 1 month of age? A 1-day-old infant with a history of maternal diabetes, cyanosis, and tachypnea, poor response to supplemental oxygen, loud single second heart sound, no murmur, chest radiograph shows narrow mediastinum with small heart tipped on side, increased pulmonary vascularity What is the next best step in a newborn with suspected transposition of the great vessels?

Most Likely Answer Cardiac (most likely) Pulmonary (most likely) The pulmonary vascular resistance drops to normal levels at that time Transposition of great vessels

Prostaglandin E1 to keep the patent ductus arteriosus (PDA) open, followed by +/− balloon atrial septostomy and surgery The most common cause of cyanotic heart disease Transposition of the great vessels presenting a few days after birth Newborn presents with cyanosis in the lower Persistent pulmonary hypertension (R→L extremities, tachycardia, respiratory distress, and shunting across the PDA) loud single S2 sound A 1-day-old newborn presents with cyanosis, Tricuspid atresia with pulmonary atresia single first and second heart sounds, chest radiograph, shows decreased lung markings, and electrocardiogram shows left axis deviation Newborn presents with cyanosis (mother was on a Ebstein anomaly medicine for severe bipolar disorder), chest radiograph shows cardiomegaly and right atrial enlargement

1100

Last-Minute Review—Cardiology Newborn presents with severe cyanosis, systolic ejection murmur, and a single second heart sound, chest radiograph shows decreased pulmonary vascular markings Newborn presents with intense cyanosis and respiratory distress, chest radiograph shows a “snowman” shaped heart An 8-week-old boy presents with feeding difficulties, poor weight gain, episodes of bluish discoloration of the skin while feeding and crying, a harsh systolic ejection murmur (SEM) is heard over the pulmonic area and left sternal border; chest radiograph shows diminished vascularity in the lungs and diminished prominence of the pulmonary arteries, a boot-shaped heart (cœur en sabot) A 2-year-old with a history of tetralogy of Fallot has progressive agitation, increasing cyanosis, and increased fussiness During the first 48 h of life, a newborn rapidly develops cyanosis, tachypnea, respiratory distress, pallor, lethargy, metabolic acidosis, oliguria, weak pulses in all extremities, hepatosplenomegaly, and no murmur What is the next best step for the newborn in the previous case with suspected hypoplastic left heart? A 2-week-old boy develops congestive heart failure, severe metabolic acidosis, and poor perfusion of the lower extremities Newborn presents with shock; the echocardiogram shows coarctation of the aorta. What is the drug of choice? A 12-year-old presents with hypertension, occasional headache, leg cramps, weak and delayed femoral pulse, and blood pressure in the upper limb is higher than the lower limb, chest radiograph shows rib notching and scalloping on the undersurface of posterior ribs A girl with Turner syndrome presents with hypertension, weak and delayed femoral pulse Newborn infant presents with a soft, harsh systolic ejection murmur, best heard at the axillae, and precordium and no symptoms The most common cardiac lesion associated with trisomy 21 (Down syndrome)

O. I. Naga

Most Likely Answer Severe pulmonary stenosis

Supracardiac total anomalous pulmonary venous return Tetralogy of Fallot

Hypercyanotic spell (Tet spell)—next step is the knee-chest position Hypoplastic left heart (as PDA closes)

Prostaglandin E1 Coarctation of the aorta Prostaglandin E1 Coarctation of the aorta

Coarctation of the aorta Peripheral pulmonary stenosis (PPS) Endocardial cushion defect

33  Last-Minute Review

Last-Minute Review—Cardiology The most common cardiac lesion associated with trisomy 18 The most common cardiac lesion associated with Turner syndrome The most common cardiac lesion associated with Williams syndrome The most common cardiac lesion associated with Alagille syndrome The most common cardiac lesion associated with Noonan syndrome The most common cardiac lesion associated with DiGeorge syndrome The most common cardiac lesion associated with cri du chat syndrome The most common cardiac lesion associated with Holt–Oram syndrome The most common cardiac lesion in fetal alcohol syndrome The most common cardiac lesion associated with lithium teratogen The most common cardiac lesion associated with supraventricular tachycardia The most common cardiac lesion associated with the infant of a diabetic mother The most common cardiac lesion associated with tuberous sclerosis The most common valvular lesion associated with acute rheumatic fever The most common cardiac lesion associated with Marfan syndrome The most common congenital cardiac lesion overall The syndrome that is associated with true interrupted aortic arch Adolescent routine physical exam, apical midsystolic non-ejection click, and late systolic murmur; the murmur is louder when goes from a supine to a standing position, and the murmur becomes softer when squatting Child routine physical exam, systolic murmur with a vibratory character, best heard in the lower sternal border, varies with changes in respiration and position A 6-year-old with a continuous murmur, lowpitched sound, best heard in the infraclavicular region, disappears when supine and with gentle pressure on the jugular vein

1101

Most Likely Answer Ventricular septal defect (VSD) Bicuspid aortic valve Supravalvar aortic stenosis Branch pulmonary stenosis Pulmonary stenosis Tetralogy of Fallot VSD Atrial septal defect (ASD) VSD, ASD Ebstein anomaly Ebstein anomaly Ventricular hypertrophy Cardiac rhabdomyoma Mitral regurgitation Aortic root dilation (risk for dissection) VSD DiGeorge syndrome Mitral valve prolapse

Still’s murmur

Venous hum

1102

Last-Minute Review—Cardiology While having her hair brushed, a 15-year-old girl develops cold sweats, pallor, and palpitations and loses consciousness for 10 s While running, a 15-year-old girl lost consciousness A 15-year-old girl faints while running and has a positive family history of deafness and sudden death The most common cause of sudden cardiac death in an athlete Newborn fails hearing screen; EKG shows a very prolonged QT interval A 5-year-old, heart rate is 230 beats/min, chest discomfort; the heart rate decreases to 80 beats/ min after ice is applied to the face What is the definitive treatment for SVT? Child presents with a history of intermittent tachycardia; EKG shows a short PR interval, slurred and slow rise of the initial upstroke of QRS (delta wave), widened QRS complex Child presents with chest pain, fever, friction rub; EKG shows diffuse ST-segment elevation, had upper respiratory infection 10 days before Adolescent diagnosed with influenza presents with fever, tachycardia, edema, and gallop; chest radiograph shows pulmonary edema, cardiomegaly, low-voltage EKG An athlete presents with dyspnea while playing; systolic ejection crescendo-­decrescendo murmur best heard at the apex and left sternal border, and radiates to the suprasternal notch; the murmur is louder while standing and with Valsalva maneuver A football player presents with chest pain with exertion and several near syncope episodes during his football game. Next best step? EKG in a 12-day-old shows negative T wave in V6 A 15-year-old boy with a history of recurrent chest pain during exercise faints and dies while playing basketball; hypertrophic cardiomyopathy ruled out as a cause of death. What is the next likely cause? What is the most common organism responsible for infective endocarditis in pediatric patients with or without congenital heart disease? History of repaired VSD with a small residual VSD next to the VSD patch, going in for dental work. Is subacute bacterial endocarditis (SBE) prophylaxis indicated?

O. I. Naga

Most Likely Answer Vasovagal syncope Thorough cardiac evaluation and referral to a cardiologist Long QT syndrome Hypertrophic cardiomyopathy Jervell and Lange-­Nielsen syndrome Supraventricular tachycardia (SVT) Radiofrequency ablation Wolff–Parkinson–White syndrome (WPW)

Pericarditis Myocarditis

Hypertrophic cardiomyopathy

Restrict from sports then EKG and echocardiogram Left ventricular hypertrophy Anomalous left coronary artery is most likely

Staphylococcus aureus Antibiotic prophylaxis

33  Last-Minute Review

Last-Minute Review—Cardiology Child with prosthetic mitral valve going for surgery; is SBE prophylaxis indicated? Child with mitral regurgitation and VSD, going in for dental work. Is SBE prophylaxis indicated? A mildly desaturated child with tetralogy of Fallot going in for dental work; is SBE prophylaxis indicated? Child with a previous history of endocarditis; is SBE prophylaxis indicated? Tall, peaked T waves in precordial leads indicates An infant of diabetic mother presents a few hours after birth with jitteriness, hypoglycemia, cyanosis; EKG shows prolonged QT interval EKG shows sinus tachycardia, widened QRS complex with an interval greater than 100 ms, in a child who presents with altered mental status after accidentally ingesting grandmother’s medication EKG shows progressive prolongation of PR interval followed by a drop in QRS EKG shows normal PR intervals and periodic drop in QRS An asymptomatic adolescent with blood pressure 137/87, all labs normal, renal US and chest radiograph normal. What is the next best step? A late complication of an untreated ASD or VSD that results in desaturation A 6-month-old infant with failure to thrive, diaphoresis, and hepatomegaly. Echocardiogram shows a large VSD. Next best step? A 4-year-old boy with physical examination significant for widely split and fixed S2 and crescendo-decrescendo systolic ejection murmur heard in the second intercostal space at the upper left sternal border. EKG shows a RSR1 pattern in V1. What is the most likely diagnosis? A premature infant with a continuous machine-like murmur and bounding pulses Which medication is used to close a PDA in a premature infant? What are some common side effects of indomethacin?

1103

Most Likely Answer Antibiotic prophylaxis No antibiotic prophylaxis Antibiotic prophylaxis Antibiotic prophylaxis Hyperkalemia Hypocalcemia Tricyclic antidepressant (TCA) toxicity

Type I second degree AV block (Mobitz I or Wenckebach) Type II second degree AV block (Mobitz II) Salt restriction in diet Eisenmenger syndrome—shunt becomes a right to left shunt Surgical correction Atrial septal defect

PDA Indomethacin

Thrombocytopenia GI bleeding Necrotizing enterocolitis Renal failure Systolic murmur most commonly heard at the right Aortic stenosis upper sternal border radiates to the neck and is associated with an ejection click

1104

Last-Minute Review—Cardiology Most common valve abnormality associated with aortic stenosis What is the most likely etiology of an early highpitched diastolic murmur associated with bounding pulses in a patient with Marfan syndrome? High-pitched holosystolic blowing murmur heard loudest at the apex and radiates to the axilla Late crescendo systolic murmur associated with a mid-systolic click, may be seen in adolescents Late diastolic rumbling murmur with an opening snap heard at the apex What is the most feared complication of Kawasaki disease? An 8-year-old presents with sharp stabbing nonspecific chest pain at rest that resolves shortly. There are no other symptoms and no past medical history A 10-year-old male presents with sharp chest pain; the pain is reproducible on physical exam At what ages is lipid screening universally recommended in the pediatric population? What is the initial management for an obese adolescent with elevated cholesterol levels?

O. I. Naga

Most Likely Answer Bicuspid aortic valve Aortic regurgitation Mitral valve regurgitation Mitral valve prolapse Mitral valve stenosis Coronary artery aneurysm Reassurance (precordial catch syndrome)

Costochondritis Once between 9 and 11 and again between 17 and 21 Diet and lifestyle modifications, and if cholesterol is still elevated after 6 months, then start statin

PULMONOLOGY Osama I. Naga

Last-Minute Review—Pulmonology Newborn with inspiratory stridor and noisy breathing; stridor improves in the prone position with head elevated and worsens in the supine position What is the treatment for laryngomalacia?

Most Likely Answer Laryngomalacia (usually benign, self-limiting and improves as the child reaches age 1–2 years) Reassurance (careful observation and growth monitoring) Unilateral vocal cord paralysis

Newborn with a hoarse voice, weak cry, and biphasic stridor that is louder when awake. Improves when positioned to be lying down on one side An infant with bulging anterior fontanelle, highBilateral vocal cord paralysis pitched biphasic stridor, respiratory distress, and recurrent pneumonia

33  Last-Minute Review

Last-Minute Review—Pulmonology Adolescent male present with shortness of breath, choking sensation within a few minutes after starting track training; there is a voice change during exercise. He was treated for exercise-­ induced asthma with no improvement in his symptoms. What is the next best step? Child with a history of chin hemangioma, worsening inspiratory stridor Newborn with intermittent cyanosis that disappears when crying but prominent during feeding; nasogastric tube unable to pass through the nostrils An infant with cyanosis, the mother is mixing the formula with well water; normal cardiac and pulmonary examination, normal pulse oximetry. Chocolate-colored blood noticed when collecting the blood for testing Boy with unilateral persistent offensive smelling nasal discharge Recurrent pneumonia and nasal polyps Failure to thrive, rectal prolapse, persistent cough Sinusitis, bronchiectasis, situs inversus, reduced male fertility A 4-year-old boy is suffering from recurrent sinusitis, chronic otitis media; during the neonatal period he had respiratory distress, daily nasal congestion, and wet cough. What is the most likely diagnosis? Child with no known health problem woke up suddenly coughing blood. What is the most likely cause? Child with a 1-day history of low-grade fever, malaise, congestion, and very thick, very green nasal discharge Child with 2 weeks of clear nasal discharge and a cough that is worse at night and while lying down supine. Not responding to nasal allergy medications A 7-year-old with fever, runny nose, throat pain; the pharynx is erythematous and shows white exudate A 7-year-old with abrupt onset of fever, headache, stomach pain, mild throat pain; the pharynx is erythematous, with petechiae, no white exudates A 15-month-old boy presents with poor feeding, high fever, thick, purulent profuse nasal discharge, crust and irritation around the nostrils

1105

Most Likely Answer Vocal cord evaluation. Most likely diagnosis is paradoxical vocal cord dysfunction

Subglottic hemangioma Choanal atresia Methemoglobinemia

Nasal foreign body Cystic fibrosis Cystic fibrosis Kartagener syndrome Primary ciliary dyskinesia

Epistaxis Viral upper respiratory tract infection Acute bacterial sinusitis

Viral pharyngitis Strep throat (Streptococcus pyogenes) Streptococcal fever or streptococcosis

1106

Last-Minute Review—Pulmonology When can a child with streptococcal infection go back to school after taking an antibiotic (become noninfectious)? Toddler with a barking cough, fever, inspiratory stridor, suprasternal retractions, and neck radiograph is normal What is the mainstay treatment of croup? A toddler presents with high fever, looks toxic, brassy cough, and stridor. He was sent home on oral antibiotics and ibuprofen, a few hours later he died A 5-year-old, unimmunized, presents with sudden onset of fever, stridor, drooling and throat pain, leaning forward and crying Preschool child has been having recurrent attacks of barking cough and croup over the last few nights, and no symptoms of cough in-between the attacks A 3-month-old with fever, cough, runny nose, tachypnea and retractions. O/E: wheezing, and crackles in both lung fields; pulse oximetry is 92% What is the first-line treatment for bronchiolitis? A preterm boy with chronic lung disease is receiving palivizumab prophylaxis, recovered from RSV bronchiolitis a few days ago. Can he take his next due dose of palivizumab? A 1-month-old infant who was born at 35 weeks during winter has been having nasal congestion for the last 2 days; stopped breathing for a few seconds and turned blue, positive RSV A 3-week-old with pneumonia; chest radiograph shows bilateral infiltrates Adolescent with fever, cough, chest pain, shortness of breath, tachypnea, and pleural friction rub Adolescent had influenza A infection, now is having a very high fever, looks toxic; tachypnea, respiratory distress, and tachycardia; chest radiograph is positive for infiltration, cavities, and pleural effusion A 7-year-old boy has a cough, on and off fever, and headache for 2 weeks, on the exam he has diffuse expiratory wheezing and crackles in both lung fields with no retractions or tachypnea. Pulse oximetry is normal. The wheezing is not fully responsive to nebulized albuterol treatment. What is the most likely cause?

O. I. Naga

Most Likely Answer Next day if improved (typically 24 h after the antibiotic) Croup Dexamethasone and racemic epinephrine can be used in moderate/severe cases Bacterial tracheitis

Epiglottitis Spasmodic croup or due to GI reflux

Acute bronchiolitis Nasal suctioning and supportive care No (discontinue palivizumab for the season)

Apnea secondary to RSV viral infection

Chlamydia trachomatis Streptococcus pneumoniae Staphylococcus aureus pneumonia

Mycoplasma pneumoniae

33  Last-Minute Review

Last-Minute Review—Pulmonology What is the most helpful diagnostic test in cases of atypical pneumonia possibly due to Mycoplasma pneumoniae What is the antibiotic of choice for older children with atypical pneumonia who are ill enough to require hospitalization? Mississippi and Ohio river valleys, chickens, caves, low-grade fever, cough, hilar lymphadenopathy Adolescent living in Arkansas presents with flu-like symptoms; fever, chills, headache, myalgia, and cough; 2 weeks later developed hemoptysis, chest pain, shortness of breath, weight loss, extreme fatigue, and skin lesions. Chest radiograph shows a focal mass with well-defined margins about 6 cm in size in the right upper lobe and few other focal segmental opacities Child visited California’s San Joaquin Valley 3 weeks ago, now has a fever, chills, cough, weight loss, chest pain, and erythema nodosum. Chest radiograph is normal What is the most sensitive serologic test in cases with suspected coccidioidomycosis? History of asthma, recurrent attacks of fever, fatigue, coughing mucus plugs, hemoptysis, eosinophilia, high IgE A sore throat with hoarseness; 3 weeks later develops pneumonia Toddler with a history of choking 2 weeks ago; he has had a cough since then, wheezing, diminished breath sounds on the right; normal chest radiograph Chest radiograph views that may help in the diagnosis of cases with suspected foreign body aspiration Child with progressive dyspnea, fatigue, recurrent cough, new-onset hemoptysis; sputum shows hemosiderosis-laden alveolar macrophages, and CBC is consistent with iron deficiency anemia African-American with shortness of breath, blurring of vision, erythema nodosum, hypercalcemia, elevated ACE level. Chest radiograph shows bilateral hilar lymphadenopathy Child with recurrent episodes of left lower lobe pneumonia; chest radiographs demonstrated focal consolidation in the same location in all events; between episodes the child is well, active, and playful

1107

Most Likely Answer Polymerase chain reaction (PCR) from nasal washing for Mycoplasma antigen Macrolide antibiotic to shorten the course of illness Histoplasmosis Blastomycosis

Coccidioidomycosis

Coccidioides immunoglobulin M (IgM) Allergic bronchopulmonary aspergillosis Chlamydophila pneumoniae Foreign body aspiration

Inspiratory and expiratory or bilateral decubitus views to see asymmetric hyperinflation on the side with foreign body due to ball-valve effect Pulmonary hemosiderosis

Sarcoidosis

Pulmonary sequestration

1108

Last-Minute Review—Pulmonology Child with fever, chest pain, and productive cough; chest radiograph shows cyst-like lesion close to the mediastinum An infant has difficulty with feeding, stridor, recurrent wheezing, history of recurrent pneumonia. Barium esophagography showed posterior compressions. What is the most likely diagnosis? Asthma > 1 night/week, throughout the day, extreme limitation of activity, FEV1:  80% Asthma ≤ 2 days/week, 0 nights/month, no limitation of activity, FEV1 > 80% Step 1 management of intermittent asthma Step 2 management of mild persistent asthma Step 3 management of moderate persistent asthma Step 4 management of severe persistent asthma What is the most effective primary controller treatment for asthma of any severity? A 10-year-old boy has shortness of breath and cough every time he runs or exercises; positive family history of asthma; on physical exam the lung is clear, the pulmonary function test is normal. What is the next best step? Child with nighttime snoring, enlarged tonsils, and difficulty concentrating in school

O. I. Naga

Most Likely Answer Bronchogenic cyst Vascular ring

Severe persistent Moderate persistent Mild persistent Intermittent Short-acting beta agonists (SABA) as needed Low-dose inhaled corticosteroids (ICS) Medium-dose ICS, and consider short course oral corticosteroids (OCS) Medium-dose ICS + LABA and consider a short course of OCS ICS Exercise-induced asthma—use albuterol (bronchodilator) inhaler 15 min before exercise. Warm up prior to strenuous activity Obstructive sleep apnea

NUTRITION Susan S. Baker

Last-Minute Review—Nutrition Xerophthalmia, corneal opacity, bitot spots, night blindness, growth failure, and recurrent infection Gingival bleeding, anemia, corkscrew-coiled hairs, anorexia, and irritability Breastfed children should be supplemented with which vitamin from birth?

Most Likely Answer Vitamin A deficiency Vitamin C deficiency Vitamin D

33  Last-Minute Review

Last-Minute Review—Nutrition Child with a history of cystic fibrosis presents with ataxic gait, diminished deep tendon reflexes in the lower extremities, as well as generalized weakness in the lower extremities Vitamin that affects prothrombin, factor VII, factor IX, factor X Child is receiving a prophylactic antibiotic for the last few months. Prothrombin time (PT) and partial thromboplastin time (PTT) are mildly prolonged Foot and wrist drop, ataxia, ophthalmoplegia, confusion, abnormal sensation, heart failure, dyspnea, and edema Redness and fissuring of lips (cheilitis), soreness of tongue, anemia, fatigue Diarrhea, dementia, dermatitis, and death in severe cases Which vitamin is needed to supplement a child on a strict vegan diet? The site of vitamin B12 absorption Infant drinks goat’s milk, looks pale, CBC shows macrocytic anemia Acute illness with diarrhea, fever, and vomiting for 2 days, followed by persistent diarrhea for 3 weeks, 6–9 episodes of liquid stools without visible blood or mucus, associated with generalized abdominal pain, distended and flatulent since the acute illness. A positive reducing substance in the stool. What is the best management? Taste and smell impairment, night blindness, and depressed immunity Deficiency associated with recurrent diarrhea, alopecia, and rash (acrodermatitis enteropathica) Child previously healthy is living with stepfather, generalized loss of muscle mass, and no subcutaneous fat Child lives in a shelter, poor nutrition, failure to thrive, weakness, edema, moon facies, a swollen abdomen (potbelly), dark, dry skin, with pale areas between the cracks, depigmentation of hair, and fatty liver

1109

Most Likely Answer Vitamin E deficiency

Vitamin K Vitamin K deficiency Vitamin B1 deficiency (thiamine) Vitamin B2 deficiency (riboflavin) Vitamin B3 deficiency (niacin) Vitamin B12 (other supplements to consider: iron, zinc, and calcium) Ileum Folic acid deficiency Lactose-free diet and lactase supplement (lactose intolerance)

Zinc deficiency Zinc deficiency Marasmus and possible calorie deprivation Kwashiorkor (protein-energy malnutrition)

1110

O. I. Naga

GASTROENTEROLOGY Robert D. Baker

Last-Minute Review—Gastroenterology An exclusively breastfed infant has not stooled for 5 days with no other symptoms. The stool is soft with no rectal bleeding. The infant is gaining weight A 1-week-old child with frequent spit-ups, otherwise doing well What are the upper GI series useful for? A 3-week-old first newborn boy presents with nonbilious projectile vomiting, hypochloremic, hypokalemic metabolic alkalosis, and dehydration What is the next best step in cases with suspected pyloric stenosis? Weight loss, abdominal pain, nausea, effortless postprandial regurgitation after at least 1 meal daily for 1 month, regurgitated food occasionally reswallowed, rechewed, or spit out Child with no known health problem woke up suddenly vomiting blood. The child is stable and acting normal. What is the most likely cause? Nausea and vomiting every 1–2 months, each episode lasts for few hours, otherwise healthy, no symptoms in-between episodes, positive family history of migraine A 7-year-old healthy child, with periumbilical abdominal pain worse in the morning prior to school, improves during weekends with normal growth parameters High achieving adolescent complains of crampy abdominal pain, diarrhea, and at other times, constipation; pain is relieved with stooling Adolescent presents with recurrent episodes of abdominal pain, diarrhea, and sometimes constipation in the previous 3 months. No weight loss and all labs are normal. What is the best treatment? A mother brought her toddler with a diaper full of undigested food, the child is holding a large bottle of apple juice The best management of toddler’s diarrhea Child with a low-grade fever, 6 episodes of diarrhea, otherwise reassuring medical exam. What is the treatment of choice?

Most Likely Answer Reassurance (breastfed infants may go several days or even a week between bowel movements) Reassurance (newborn reflux is normal) To rule out anatomic or motility problems. Does not diagnose reflux Pyloric stenosis Abdominal US (pylorus) Rumination syndrome

Epistaxis (nose bleeding is the most common source in healthy children) Cyclic vomiting syndrome

Reassurance (functional abdominal pain)

Irritable bowel syndrome Peppermint oil, diet modifications, cognitive behavioral therapy

Toddler diarrhea Juice restriction and allow normal dietary fat Oral rehydration therapy (avoid anti-diarrheal agents)

33  Last-Minute Review

Last-Minute Review—Gastroenterology What is the major concern of using antimotility drugs such as Loperamide? An infant presents with bright red blood stool, poor weight gain, diarrhea, and fussiness; the infant is breastfeeding, supplemented with standard infant formula; stool guaiac test is positive Child with dysphagia, recurrent food impaction; biopsy shows an increased eosinophil? Adolescent with recurrent headaches takes ibuprofen as needed, presents with dysphagia and chest discomfort (does not like to drink water with medicine) Child accidentally swallowed caustic liquid 6 h ago, presents with dysphagia, oral pain, chest pain, nausea, and vomiting Swallowed a coin, no symptoms, and radiograph showed the coin still in the esophagus Swallowed a coin, no symptoms, and radiograph showed the coin in the stomach 4 weeks passed and the coin still in the stomach with no symptoms Swallowed a coin, excessive drooling, and chest pain, and radiograph showed the coin still in the esophagus Swallowed a button battery (BB), and passed to the stomach with symptoms Swallowed a BB, and passed to the stomach without symptoms Swallowed a BB that got stuck in the esophagus Swallowed small pieces of magnet metals; the abdominal radiograph showed the pieces in the stomach An older child with bloating, constant burping, sharp epigastric pain that awakens the child from sleep The most common cause of chronic gastritis in pediatrics The best and most definitive test for peptic ulcer disease What is the treatment of H. pylori infection?

1111

Most Likely Answer May induce ileus Cow milk protein intolerance

Eosinophilic esophagitis Pill-induced esophagitis

Endoscopy in 12–24 h after ingestion Observe for 12–24 h, removal of the coin if it does not pass to the stomach or if the patient became symptomatic Checking the stool for passage for 4 weeks, with weekly radiographs, if indicated If the coin does not pass through the stomach by 4 weeks or if the patient is symptomatic, removal by endoscopy should be considered Immediate removal Immediate removal Urgent removal (if age  5 juvenile polyps What is the next step in children with ≥ 5 juvenile polyps or any number of adenomatous intestinal polyps? 100 or more adenomatous polyps in the large and/ or small intestines Intestinal polyps, osteoma of the mandible, papillary carcinoma of thyroid, and hepatoblastoma Intestinal polyps and brain tumor

O. I. Naga

Most Likely Answer Gilbert syndrome Crigler–Najjar syndrome type I (exchange transfusion is warranted) Dubin–Johnson syndrome Biliary atresia Percutaneous liver biopsy Intraoperative cholangiography Wilson disease Copper (excess) Ceruloplasmin  250 ug/g dry weight. Urine copper > 100 ug/24 h. Presence of Kayser–Fleischer rings Alagille syndrome Hepatoblastoma Progressive familial intrahepatic cholestasis (PFIC) type 1

Lethal during childhood unless treated early Bannayan–Riley–Ruvalcaba syndrome Juvenile polyposis Genetic testing Familial adenomatous polyposis Gardner syndrome Turcot syndrome

33  Last-Minute Review

Last-Minute Review—Gastroenterology Intestinal polyps, hematochezia, mucocutaneous freckling, and a family history of polyposis Hamartomas involving many areas of the body, e.g., skin, oral mucosa, thyroid, breast, and colon Associated risks of Cowden syndrome Hemihypertrophy, very large extremities, epidermal nevus, hamartomatous polyps, intellectual disability Potential risks of Proteus syndrome The best diagnostic test for lactose intolerance

1115

Most Likely Answer Peutz–Jeghers syndrome (increases the risk of cancer) Cowden syndrome Cancer, e.g., thyroid cancer Proteus syndrome Deep vein thrombosis (DVT) and thromboembolism Hydrogen breath test

NEPHROLOGY Beatrice Goilav

Last-Minute Review—Nephrology A 5-year-old hospitalized and receiving penicillin IV for 10 days, developed rash, eosinophilia, eosinophiluria, pyuria (sterile), hematuria, moderate proteinuria (usually  400 mOsm/L, urine Na  20, large muddy brown granular cast A male infant with posterior urethral valves, born prematurely and is found to have high BUN/Cr, elevated FeNa, normal urine osmolality, elevated urine Na Status post-cardiac arrest, BUN and creatinine are elevated, hyperkalemia, hyponatremia, hyperphosphatemia, hypocalcemia, urine shows muddy brown, and granular casts Adolescent with severe muscle cramps, numbness, low blood pressure, fatigue, metabolic alkalosis, hypochloremia, hypokalemia, hyponatremia, hypomagnesemia, polyuria, low urine calcium, and high urinary chloride 70 mEq/L. High aldosterone and renin levels An infant with failure to thrive, dehydration, low blood pressure, metabolic alkalosis, hypochloremia, hypokalemia, hyponatremia, normal serum magnesium level, polyuria, normal urine calcium, and high urinary chloride 70 mEq/L. High aldosterone and renin levels. High urinary prostaglandin level

1117

Most Likely Answer Nephrotoxins and rhabdomyolysis Renal tubular acidosis (RTA) type 1 Fanconi syndrome

RTA type 2 RTA type 4 Goodpasture syndrome Prerenal acute kidney injury Acute tubular necrosis (intrarenal acute kidney injury) Postrenal acute kidney injury

Acute tubular necrosis secondary to ischemia

Gitelman syndrome (metabolic alkalosis and high urine chloride, low serum Mg, and low urine Ca)

Bartter syndrome (metabolic alkalosis and high urine chloride, normal serum Mg, and normal or high urine Ca)

1118

Last-Minute Review—Nephrology Child with a positive family history of hypertension (parents) presenting with headache, hypertension, hypokalemia, metabolic alkalosis, and high urinary chloride Adolescent with mild glomerulonephritis, history of allergies/asthma with elevated eosinophil levels on CBC An older child with a prolonged history of fever, weight loss, hematuria, and hemoptysis. Radiograph shows necrotizing granuloma and C-ANCA positive A young child with palpable kidneys bilaterally, hypertension, and associated with a history of oligohydramnios The predominant type of polycystic kidney disease seen in adults How is hypertension defined in children?

How is elevated blood pressure defined in children? Adolescent with severe muscle weakness after exercise, hypophosphatemia, hypokalemia and elevated CPK with myoglobinuria Child with nausea, severe flank pain, and hematuria The most common type of stones in children Type of kidney stone associated with staghorn calculi and Proteus? Type of stone associated with an autosomal recessive pattern First-line therapy for children with primary monosymptomatic nocturnal enuresis Recommended lifestyle changes in all cases of monosymptomatic nocturnal enuresis Diurnal enuresis after continence

O. I. Naga

Most Likely Answer Liddle syndrome (autosomal dominant)

Churg Strauss syndrome Granulomatosis with polyangiitis (formerly Wegner granulomatosis) Autosomal recessive polycystic kidney disease Autosomal dominant polycystic kidney disease BP > 95th percentile for age, height, and gender on 3 different occasions or greater than 130/90 mmHg in children aged 13 years and older BP ≥ 90th (≥ 13 years BP > 120/80 mmHg matching new adult guideline) Rhabdomyolysis—heme positive urine but no RBC Renal stone Calcium oxalate Struvite stones Cystine stones Bedwetting alarm and desmopressin (both) Adequate hydration during the day Limit fluids before bed (≤ 200 mL) Void before bed Regular sleep and wake schedule Requires prompt evaluation

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1119

FLUIDS AND ELECTROLYTES Beatrice Goilav

Last-Minute Review—Fluids and Electrolytes Low urinary fractional excretion of sodium, high urine osmolality, high serum osmolality High urinary fractional excretion of sodium, low serum sodium, low serum osmolality, inappropriately high urine osmolality Low urine osmolality, high serum sodium, and serum osmolality (urine osmolality  10 mm AP diameter of the renal pelvis. What is the next best step?

Most likely Answer Renal US after 48 h of life

33  Last-Minute Review

Last-Minute Review—Urology What is the best test to confirm the diagnosis of urinary tract infection (UTI) A one-year-old child presents with the first febrile UTI. What is the appropriate imaging study? What is the gold standard test for the diagnosis of anatomical details of the renal system and degree of reflux? What are the indications of VCUG?

1121

Most likely Answer Urine culture Renal-bladder US (RBUS) Voiding cystourethrography (VCUG). It should not be routinely performed in children after a first febrile UTI Findings on RBUS that suggest the presence of high-grade vesicoureteral reflux or the recurrence of febrile UTIs Presence of fever and urine infection is highly suggestive of pyelonephritis Constipation Enterococcal UTI

What is the main difference between cystitis and acute pyelonephritis? Prevalent cause of recurrent UTIs in children Child with an indwelling catheter and a urine dipstick analysis negative for nitrites Empiric antibiotics in patients suspected of having Combination of ampicillin or amoxicillin and, an enterococcal UTI third-generation cephalosporin or aminoglycoside A 4-year-old boy with weak urine stream, failure VCUG for the possibility of a posterior urethral to thrive, recurrent UTIs, enuresis. The renal US is valve positive for bilateral hydronephrosis. What is the best test to establish the diagnosis? A 4-year-old female with a history of chronic Female urethral prolapse constipation presenting with vaginal bleeding and urethral mass A 6-year-old female with persistently damp Ectopic ureter underwear (day and night) A 7-year-old uncircumcised boy with penile pain, Emergent reduction of the foreskin to its swollen foreskin O/E: head of the penis is enlarged anatomical position. May apply compression, and congested with a collar of edematous foreskin. sugar, pin-pricks to decrease the edema A constricting band of retracted foreskin is noted past the head of the penis. What is the best treatment? What are the most common anatomical Hypospadias with incomplete foreskin, contraindications of circumcision? epispadias, ambiguous genitalia An uncircumcised 3-year-old boy presents with a Reassurance (normal smegma) nonpainful, white, mobile mass just distal to the corona of the boy’s penis Full-term male newborn presents with right Referral to a surgeon if the testis remains undescended testis. The remainder of the physical undescended at 6 months of age examination is unremarkable. What is the next best step? A 15-year-old boy presents with a painless, solid, Testicular cancer until proven otherwise firm, irregular mass in the left testicle A 12-year-old boy presents with sudden onset of Testicular torsion testicular pain, vomiting, the right testicle is swollen, tender, absent cremasteric reflex

1122

Last-Minute Review—Urology Patient presents with testicular torsion A 12-year-old boy presents with pain, tenderness, and swelling in the upper pole of the right testicle. O/E: bluish dot is visible through the scrotum A 12-year-old boy presents with soft non-tender fullness within the left hemiscrotum, homogenous glow without internal shadows on transillumination; testes are palpable posteriorly An 18-year-old male presents with a left-sided mass that feels like a bag of worms; the left testicle is smaller than normal; the mass increases in size with Valsalva maneuver Adolescent male is complaining about a mobile nodule noted above the testis; the mass does not increase with Valsalva A sexually active adolescent male presents with testicular pain that improves with testicular elevation; he has dysuria and fever. The cremasteric reflex is intact A 10-year-old boy with a sudden onset of right flank colicky abdominal pain, nausea, vomiting, frequent urination. Physical examination is normal. The urine test is positive for 20 RBCs. The renal US shows 3 mm stone in the right ureter A 10-year-old boy presents with blood in urine and difficulty voiding after blunt trauma to the genital area. O/E: there is a drop of blood on the meatus

O. I. Naga

Most likely Answer Immediate urology consult (the testicular US should not delay the consultation) Torsion of the testicular appendage Hydrocele

Varicocele

Spermatocele Epididymitis

Pain medicine, aggressive hydration (most stones smaller than 5 mm pass spontaneously in children) Urethral injury (best study is retrograde urethrography)

DERMATOLOGY Megan Craddock and Jennifer Ruth

Last-Minute Review—Dermatology Pustules of erythema toxicum contain Pustules of transient neonatal pustular melanosis contain Newborn with white papules on the hard palate Newborn with pinhead white papules on the face Newborn with white papules on the upper gums An infant with small papules and pustules on the forehead, nose, and cheeks and an absence of comedones.

Most Likely Answer Eosinophils (reassurance) Neutrophils (reassurance) Epstein pearls (reassurance) Milia (reassurance) Bohn nodules (reassurance) Neonatal acne (resolves without treatment)

33  Last-Minute Review

Last-Minute Review—Dermatology Newborn with blistering and erosions of the skin. May have mucous membrane and nail involvement. Infection has been ruled out What is the best diagnostic test for epidermolysis bullosa? Well-defined erythematous plaques on the knees and elbows, covered with silvery scales, bleed when removed, pitting of the nails Child with ulcerative colitis presents with bright red, tender nodules on the anterior leg Erythematous, scaly plaques in periorificial and acral areas, alopecia, and diarrhea A 2-year-old boy with fever, fragile blisters, and denuded skin, predominantly affecting the flexures and perioral skin Adolescent boy with a history of cold sores presents with targetoid papules, distributed acrally, and hemorrhagic crusting of the lips Child is taking penicillin for a dental abscess, developed macules, papules, vesicles, bullae, and ulcerations on 8% of the body surface area. Sloughing, blistering, and ulceration around the lips, eyes, and genitalia are also present Most common virus that triggers erythema multiforme Poorly defined, hypopigmented rough macules and patches on the cheeks Adolescent with oval scaly papules and plaques on the trunk that run parallel to skin cleavage lines Adolescent boy with hypopigmented scaly lesions on the neck, chest, and back that worsen with sun exposure What is the cause of tinea versicolor? Child with an itchy rash affecting both feet. Exam shows scaling, fissuring, and maceration in the interdigital spaces. What is the best treatment? Child with one bald spot on the scalp with scale and “black-dot” hairs (the remnants of broken hairs within follicles). What is the best treatment? Child with one bald spot on the scalp with no associated scale or redness. What is the best treatment? A young child with diffuse thinning of the hair on the scalp after undergoing major surgery 3 months prior

1123

Most Likely Answer Epidermolysis bullosa Skin biopsy of an induced blister or genetic testing Psoriasis Erythema nodosum Acrodermatitis enteropathica (zinc deficiency) Staph scalded skin syndrome (SSSS) Erythema multiforme Stevens–Johnson syndrome

Herpes simplex virus (HSV)  Pityriasis alba Pityriasis rosea Tinea versicolor Malassezia furfur Topical antifungal cream, e.g., terbinafine cream Oral antifungal Reassurance or topical steroids (alopecia areata) Telogen effluvium

1124

Last-Minute Review—Dermatology Condition with pegged-shaped teeth, prominent ears, small chin, frontal bossing, absence of sweating with associated overheating Female neonate with blistering and/or hyperpigmentation of the skin in a blaschkoid distribution. May be associated with eye and neurologic abnormalities Fish-like scaling of the body, sparing flexural areas, with corneal opacities and history of cryptorchidism Extremities are covered with fine, irregular, polygonal scales, hyperlinear palms; worse with dry weather and during winter, family history of “dry skin” Erythematous, scaly, itchy plaques in the antecubital and popliteal fossae, older brother with asthma A 3-year-old boy was exposed to poison ivy while playing in the garden. He developed itchy linear streaks of vesicles on both arms. What is the best treatment? What is the most important recommendation for all cases of contact dermatitis? Folliculocentric papules with central keratinous debris on the upper arms and thighs Unilateral, irregular brown to blue-gray pigmentation of the neck, shoulder, supraclavicular, deltoid, and/or upper arm skin. May darken at puberty Unilateral, irregular, blue-gray discoloration in the periorbital area and sclera (Ocular) A nevus of Ota involving the sclera requires monitoring by ophthalmology due to increased risk of Large congenital nevi likely carry an increased risk of what type of cancer? Child is going to the beach for swimming and parents are concerned about sunburn. How would you counsel the family about sunscreen use? Large facial port-wine stain involving V1, seizures, and glaucoma Posterior fossa malformations (Dandy–Walker), hemangiomas, arterial anomalies, cardiac defects (e.g., coarctation of the aorta), and eye abnormalities

O. I. Naga

Most Likely Answer Hypohidrotic ectodermal dysplasia Incontinentia pigmenti (X-linked dominant)

X-linked recessive ichthyosis Ichthyosis vulgaris

Atopic dermatitis Topical steroids (Rhus dermatitis)

Avoidance of triggering agents Keratosis pilaris Nevus of Ito

Nevus of Ota Glaucoma > ocular melanoma Melanoma Apply SPF 30 or greater sunscreen 15–30 min before sun exposure, reapply every 2 h Sturge–Weber syndrome PHACE syndrome

33  Last-Minute Review

Last-Minute Review—Dermatology An infant with a large hemangioma on the upper eyelid is at risk for What is the best treatment for a hemangioma on the upper eyelid that obstructs the visual axis? Annular plaque without scale not responding to topical antifungals An infant with orange to brown macules and papules that become red and swollen when stroked An infant with a 4-month history of seborrheic dermatitis-like rash on the scalp, behind the ears, and in the diaper area not responsive to topical antifungals or steroid cream A 12-year-old boy with learning disabilities and seizure disorder presents with persistent papules on the face despite acne treatment. He is also found to have hypopigmented skin lesions on the exam A 10-month-old with light brown macules and small patches on the body. How many café-au-lait macules should raise concern for neurofibromatosis? What is the treatment for head lice? What is the treatment for scabies?

1125

Most Likely Answer Amblyopia Oral propranolol Granuloma annulare Urticaria pigmentosa (cutaneous mastocytosis) Langerhans cell histiocytosis

Tuberous sclerosis

Six

Permethrin 1% liquid; 2 treatments spaced 1 week apart Permethrin 5% cream; 2 treatments spaced 1 week apart

PSYCHOSOCIAL ISSUES AND CHILD ABUSE Mohamed Zebda

Last-Minute Review—Psychosocial Issues and Child Abuse Factors that determine the understanding of death and expression of grief What does exposure to high levels of parental conflict lead to? What are the most helpful measures for children in cases of divorce? Consequences of divorce on a child’s emotional adjustment Consequences of adoption on children from institutional or orphanage care Child is being placed in foster care; how soon should this child be evaluated by a provider?

Most Likely Answer Chronologic age and levels of cognitive development Predictive of poor emotional adjustment by the child regardless of the parents’ marital status Regular schedule with flexibility Consistency with structure and routine Cooperative co-parenting May affect his/her subsequent intimate relationships A higher risk of medical and developmental problems than their counterparts who have resided in foster care Initial visit should take place within 72 h after placement in the foster care system

1126

Last-Minute Review—Psychosocial Issues and Child Abuse TV watching is not recommended at what age? How long should children 2 years and older be allowed to watch TV? Increased aggressive behavior and acceptance of violence, obscuring of the distinction between fantasy and reality, trivialization of sexuality are effects of One of the most common causes of school refusal in children A set of clinical features in which unfounded parental anxiety about the health of a child results in disturbances of the parent–child interaction At what age should discussions start regarding transition of care for adolescents and young adults with chronic medical conditions? A child tends to have asynchronous developmental patterns, very advanced in one domain area compared to the rest Maternal depression, substance use/abuse, and physical injuries A frequent risk factor for child abuse Consequences of children who are exposed to corporal punishment and intimate partner violence What is the most common form of child abuse? What is the most common presentation in an abused child?

O. I. Naga

Most Likely Answer Children younger than 2 years of age 2 h/day or less for all children, including other forms of screen (solitary TV watching is highly discouraged) Excessive media time

Separation anxiety disorder Vulnerable child syndrome Around 12 years of age Gifted child May indicate intimate partner violence

Intimate partner violence More likely to exhibit aggressive/violent behaviors than other children Neglect Asymptomatic—just because there are no physical signs does not mean that the child has not been abused What is the most common cause of head injury in a Child abuse child less than 1 year of age? A 3-week-old girl presents to ER with lethargy, Abusive head trauma (shaken baby syndrome) poor sucking, and retinal hemorrhages; mother stated the child rolled over and fell from the bed. What is the most likely cause? What are the consequences of child abuse during Physiologic and anatomic changes in the brain, early development? increased risk of physical and behavioral problems Parents or guardians who failed to provide Medical neglect adequate medical care for a child, e.g., refused to administer medications for a serious medical condition Parents or guardian fail to provide basic needs Physical neglect (nutrition, shelter, clothes) or who abandon the child

33  Last-Minute Review

Last-Minute Review—Psychosocial Issues and Child Abuse Parents or guardian fail to enroll the child in school or provide homeschooling, allow frequent absenteeism, or ignore special education needs Parents or guardian isolate the child, withhold emotional support, expose the child to interpersonal violence or substance abuse, e.g., fight or engage in sex in front of the child Parents or guardian leave the child alone or improperly supervised, fail to keep the child from safety hazards, e.g., leaving strong chemicals open and not safely secured in the house Posterior rib fracture, bucket handle fracture, femur fracture in child less than 1 year old, distal humeral physeal fracture, and humeral shaft fracture in child less than 3 years old are high suspicion of Bucket handle fracture (also known as a classic metaphyseal fracture or metaphyseal corner fracture) in infants What is the next best step with any suspicion of child abuse in a child less than 2 years of age? Difference between bucket handle fractures and buckle fractures A 7-month-old healthy infant with a few bruises on arms and legs A 7-year-old healthy child with multiple bruises on both tibial shins and elbows Entire foot is burned, with a well-­demarcated line of injury around the leg and absence of splash marks (stocking distribution) Entire hand is burned, with a well-­demarcated line of injury around the forearm and absence of splash marks (glove distribution) What is the most common physical examination finding in a child with sexual abuse? Physical contact between the victim and the perpetrator, with or without oral, anal, or vaginal penetration Which gender is less likely to disclose sexual abuse and might be victimized more often than the reported ratio A 16-year-old adolescent presents to the ER due to a sexual assault that occurred 1 day ago, what should be done?

1127

Most Likely Answer Educational neglect Emotional neglect

Supervision neglect

Child abuse

High specificity for child abuse. It may occur with shaking, vigorous pulling or twisting of an infant’s extremity Skeletal survey Fracture of the distal radius (buckle fracture) is common and is not associated with child abuse Must be evaluated for child abuse and reported to child protective services Reassurance Child abuse (possible forced immersion into hot water) Child abuse (possible forced immersion into hot water) Normal examination Child sexual abuse Boys SANE exam—should be done by a specialized nurse. The cutoff for exam 72–120 h (within 5 days)

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Last-Minute Review—Psychosocial Issues and Child Abuse What is the SANE exam? A healthy child with multiple recurrent ER visits; the mother is a healthcare professional who demands tests and imaging with each visit; before discharge patient’s blood glucose is 40 mg/ dL. Further evaluation shows high serum insulin level and absence of serum C-peptide level

O. I. Naga

Most Likely Answer Exam performed by a Sexual Assault Nurse Examiner who examines the victim of sexual assault and collects all forensic evidence Caregiver-fabricated illness

ETHICS Mohamed Zebda

Last-Minute Review—Ethics The ethical principle of referring a child to an expert in the field or providing the best treatment available, considering treatment efficacy and potential to lessen disability The ethical principle of not ordering a head CT scan for a well-appearing child with trivial head injury or referring a child with obstructive sleep apnea secondary to enlarged tonsils and adenoids to ENT (weighing the risks and benefits in each case and avoiding possible complications) The ethical principle of informing an adolescent about their diagnosis The ethical principle of maintaining the confidentiality of emancipated minor with STD The ethical principle of ideal distribution of risks and benefits, and resolving potential conflict The ethical principle of ensuring access to medical care with federally sponsored child health insurance The ethical principle of autonomy of thought, intention, and action when making decisions regarding health care procedures or treatment Newborn with ambiguous genitalia. What is the best strategy in helping the parents consider the gender in which to rear the child?

Most Likely Answer Beneficence (doing good for others or the best interest of the patient) Nonmaleficence (do no harm)

Veracity (truthfulness or truth-telling) Fidelity, e.g., maintaining confidentiality or faithfulness Justice, e.g., clinical research trials in children Justice Autonomy A multidisciplinary team should give parents as much information as possible about their child’s diagnosis and prognosis

33  Last-Minute Review

Last-Minute Review—Ethics Family desires to continue invasive therapy for child with irreversible and devastating neurological damage. The critical care physician and the neurologist strongly believe that there will be neither benefit nor quality of life for the child in continuing life support What is the best approach to the family in the previous example?

1129

Most Likely Answer Physicians are not obligated to provide futile care

Physicians must provide families with relevant risks and benefits of available options and to provide specific recommendations Declaration of brain death requires Two independent examinations, including a physical examination and apnea testing Following the agreement of parents or guardian for 7 years and older (unless cognitively impaired) a child to participate in clinical research, at what age is consent of the child also required? A physician is receiving financial incentives for This practice is prohibited because of the recruiting children to participate in a clinical drug potential element of undue influence and trial coercion What are the most critical criteria required to Consent of guardians, child ≥ 7 years, the best conduct a clinical drug trial in children? interests of the child, minimizing harm, safety committees, monitoring, meaningful and measurable outcomes How should the consent document for the clinical Easy to understand (sixth to eighth-grade trial or research be written? reading level) What is the age of a minor to give consent for Laws related to minor consent vary by state screening of sexually transmitted infections, consent for contraceptive services, and consent for general medical care? At what age are contraceptive pills (progestin-only 17 years of age and older emergency contraceptive) available over the counter (OTC) to adolescents in all states in the USA? What is a minor called if the minor is married, in Emancipated minor (has the right to provide the military, a parent, self-supporting while not informed consent for medical care). The criteria living with parents, or a high school graduate? vary by jurisdiction A 4-year-old boy involved in a car accident Transfuse the child (informed consent is not requires a lifesaving blood transfusion. His adult required to treat a child with a life-threatening parents refuse based on religious reasons. What condition) should be done? A 19-year-old involved in a car accident requires a Respect the patient’s wishes and do not transfuse lifesaving blood transfusion. Based on religious (patient is an adult) reasons he is refusing the blood transfusion. What should be done? The process in which the risks and benefits along Informed consent—must be sought from the with potential alternatives are discussed with the parents unless there is a life-threatening parents before performing any procedure is known emergency and unable to contact them as

1130

Last-Minute Review—Ethics A 16-year-old teenager requests confidential STD testing and pregnancy test. The mother comes without her child to demand the results of the pregnancy/STD testing. What should you do? A 16-year-old teenager positive for gonorrhea is in the office with her 17-year-old boyfriend, who is waiting outside. She begs you not to tell him about her diagnosis. What should you do? In what situation is a breach in confidentiality allowed, and parents or guardian must be notified about an adolescent’s medical condition? The mother of a 15-year-old female is here to obtain lab results that were ordered for obesity. The patient is unable to be present. What should you do? You are seeing a 15-year-old female who says she has had consensual sexual activity with her boyfriend who is 21 years old. What should be done in this situation? At what age is a minor considered developmentally mature and able to understand the consequences of his or her medical decision, and thus should be involved in making decisions about their medical care? Parents have asked the pediatrician not to reveal a diagnosis to their adolescent because of the psychological impact. What is the best approach?

O. I. Naga

Most Likely Answer Do not disclose the results and protect patient confidentiality Do not tell the boyfriend, report her diagnosis to the CDC. Encourage her to inform him herself Risk of harming self or others Disclose the results to the mother (guardian)

A report should be filed with child protective services—the child is a minor (age may vary by state) and the boyfriend is an adult The beginning of 12–13 years of age (mature minor doctrine)

Arrange a meeting with parents and the adolescent to discuss the diagnosis and prognosis and provide psychological support if needed A 10-year-old girl recently diagnosed with a brain Discuss the diagnosis and treatment with parents tumor; parents are asking you not to tell the child and the child (the child must know) about her condition. What should be done in this situation? What are the ethical principles in the previous 2 Veracity (truthfulness or being honest) and examples? fidelity (faithfulness) A 15-year-old boy with terminal cancer has been Medical team meeting with the child and family hospitalized for 3 months because of life-­ to elicit his preferences, inform him and his threatening complications and recurrent relapses. family about end-of-life decisions, improve He decides to discontinue his medical care, and the communication, and increase agreement among family desires to continue treatment regardless of all involved parties regarding end-of-life care outcome. What is the best approach to the end-of life-care? A family with a well-known adult-onset genetic No testing until the children reach adulthood disorder (in the fifth to sixth decade) desires (after 18 years of age) when able to make an genetic testing for their children to know their independent and informed decision future risks

33  Last-Minute Review

1131

Last-Minute Review—Ethics Parents are new to your practice and refuse vaccinations to their children

Most Likely Answer Listen to parents and address all their concerns about vaccines Explain all risks and benefits of the vaccines in question Parents continue to reject the vaccination to their Continue their care. Discuss risks and benefits in children each subsequent visit If parents continue to refuse vaccinations for their No. Pediatrician should continue care unless a child, is the pediatrician obligated to dismiss the strong sense of distrust develops that impacts a child from his or her practice? child’s overall care Parents brought their unimmunized 7-year-old boy Report the family to state welfare agency who is bleeding from a raccoon bite. Parents refuse (medical neglect) rabies vaccine

RESEARCH AND STATISTICS Mohamed Zebda

Last-Minute Review—Research and Statistics Study best suited to evaluate the risks and benefits of a new treatment, establishes a direct causal relationship between treatment and outcomes Study in which 2 groups are followed prospectively over time to see which exposures/ risks cause disease and to provide information about prognosis Retrospective study in which people with a disease are compared to those without the disease to evaluate risk factors, useful for rare outcomes Type of study used to compare a new diagnostic test to the current gold standard diagnostic test in a given population Retrospective statistical analysis of several studies on the same topic What is the best way to eliminate confounding variables? Type of bias that occurs when an association between an exposure and an outcome is distorted by another variable The percent of people with the disease in a given population being studied The number of new cases of a given disease in a specific period of time The probability of correctly identifying those who truly have the disease

Most Likely Answer Randomized control trial Cohort study (type of observational study)

Case-control study (type of observational study) Cross-sectional study Meta-analysis Randomization of study subjects Confounding bias Prevalence Incidence Sensitivity = TP/(TP+FN) SnOUT sensitivity → rules out disease when they’re negative

1132

O. I. Naga

Last-Minute Review—Research and Statistics Most Likely Answer The probability of correctly identifying those who Specificity = TN/(TN+FP) truly do not have the disease SPIN specificity → rules in disease when they’re positive The probability of correctly identifying those who Positive predictive value = TP/(TP+FP) truly have the disease among those whose tests are positive The probability of correctly identifying those not Negative predictive value = TN/(TN+FN) having a disease among those whose tests are negative The hypothesis of no difference, i.e., daily Null hypothesis exercising does not reduce the risk of heart disease The hypothesis of difference, i.e., daily exercising Alternative hypothesis does reduce the risk of heart disease Rejecting the null hypothesis when in fact it is Type 1 error true—a difference that was seen when one does not exist Failure to reject the null hypothesis when the null Type 2 error hypothesis is false—a difference that was not seen when one does exist The probability that a study can detect a treatment Power of a study effect A range of values with a specified probability that Confidence interval a given parameter falls in that range What is the interpretation of a confidence interval There is no statistical significance that includes 0 or 1 The total number needed to treat to prevent one The number needed to treat NNT = 1/absolute risk reduction bad outcome or adverse events The total number of patients receiving intervention The number needed to harm NNH = 1/attributable risk for each patient that is harmed

 ATIENT SAFETY AND QUALITY P IMPROVEMENT Mohamed Zebda

Last-Minute Review—Patient Safety and Quality Improvement Wrong plan to achieve a desired aim An error reaches the patient but does not result in harm Recognized and corrected errors before it reaches the patient An unexpected occurrence of death or serious physical or psychological injuries

Most Likely Answer Medical error Non-intercepted near miss error Intercepted near miss error Sentinel event

33  Last-Minute Review

Last-Minute Review—Patient Safety and Quality Improvement A 4-year-old child with leukemia who requires a transfusion receives the wrong type of blood, resulting in a serious transfusion reaction When a sentinel event has been identified, an investigation is undertaken immediately to determine the root causes that have led to the event. An action plan is then developed and implemented to monitor the system in order to minimize the risk that such an event will recur in the future A patient who is allergic to penicillin was prescribed amoxicillin and developed a skin rash after drug administration A patient with no history of allergic reaction to penicillin developed a severe allergic reaction to amoxicillin Suspected child physical or sexual abuse is an example of Serious reportable hospital events that should not have occurred, resulting in death or significant disability Mistakenly, a surgery was performed on the healthy left knee instead of the right knee with torn ligaments Frequent nonclinically relevant alarm alerts result in desensitization to the alarms, and caregiver may miss some signals that should necessitate an intervention What are some ways to prevent dosing and medication errors? What are some ways to prevent medication administration errors? Child in your clinic received the wrong immunization; what should be done in this situation? What are the components of a successful quality improvement project?

1133

Most Likely Answer Sentinel event Root cause analysis

Preventable medical error Non-preventable medical error Mandatory reporting Never events Surgical never event Alarm fatigue (adjust alarm thresholds to reduce nonclinically relevant noises); patients’ variability should be considered Avoid trailing zeros such as 20.0 mg Use leading zeros such as 0.1 mg Avoid abbreviations such as BID Write out unit Syringes are the preferred dosing device, measuring cups and spoons calibrated and marked in milliliters are acceptable alternatives Provide apology to parents The error should be disclosed to the parents in a clear manner, and the steps that need to be taken to prevent further errors should be discussed Plan—what will be changed/what intervention Do—try the change on a small scale Study—analyze results of the change/ intervention Act—implement changes on a larger scale

1134

O. I. Naga

PHARMACOLOGY AND PAIN MANAGEMENT Mohamed Zebda

Last-Minute Review—Pharmacology and Pain Management The time it takes the plasma concentration of a drug to decrease by half The lowest concentration reached by a drug before the next dose is administered The highest concentration reached by a drug after the dose is administered Child is taking griseofulvin for tinea capitis. What is the best recommendation to maximize absorption? Child is taking oral penicillin for streptococcal infection developed non-pruritic rash, nausea, and vomiting. What is the type of this reaction? Child is taking oral penicillin for streptococcal infection developed pruritic rash, swollen lips and difficulty breathing. What is the type of this reaction? What is the most common side effects of drugs in general? Anxiolysis with the maintenance of consciousness Controlled depressed consciousness, airway reflexes, and airway patency are maintained; the patient responds appropriately to age-appropriate commands and touch Controlled depressed consciousness, airway reflexes and airway patency may not be maintained, ventilatory function may be impaired, patient not easily aroused but responds purposefully following repeated or painful stimulation Loss of consciousness occurs; impaired airway reflexes, airway patency, and ventilatory function; not arousable; not responsive to painful stimulation In order to decrease the requirements of morphine post-operatively in neonates, infants, and children, what is the best recommendation? Complication of gentamicin therapy, especially in newborns? Which complication is associated with erythromycin in children less than 1 month? Complication of tetracyclines in children less than 8 years old

Most Likely Answer Half-life (t½) Trough levels Peak level To be taken with whole milk or fatty meal Non-IgE-mediated IgE mediated “anaphylaxis” to penicillin

Nausea Minimal sedation Moderate sedation (conscious sedation)

Deep sedation

General anesthesia Give an appropriate dose of acetaminophen (oral or rectal) for mild to moderate pain even after major surgeries Ototoxicity Pyloric stenosis Teeth staining

33  Last-Minute Review

Last-Minute Review—Pharmacology and Pain Management Mother is asking if she can give her 5-month-old benzocaine gel for teething What are the common side effects of ADHD medications? In young children with aspirin use, and concurrent viral infection such as varicella, may cause

1135

Most Likely Answer Not recommended; increases the risk of methemoglobinemia Weight loss, difficulty sleeping, palpitations Reye syndrome

Index

A Abdominal masses, 50 Abdominal migraine, 759, 760 Abdominal pain acalculous cholecystitis, 765 acute abdominal pain, 757 acute appendicitis, 761, 762 acute pancreatitis, 763 cholangitis, 765 cholecystitis, 764–765 choledocholithiasis, 765 cholelithiasis, 764 chronic, recurrent pain, 760 cyclic vomiting, 759, 760 functional abdominal pain, 757–759 intussusception, 763 malrotation, 762 recurrent pancreatitis, 763–764 referred pain, 760, 761 volvulus, 762, 763 Abdominal trauma, 210 Abdominal wall defects, 50–52 Abnormal alpha-fetoprotein (AFP), 37 ABO incompatibility classification markers, 348 clinical approach to child with anemia, 348 clinical presentation, 347 diagnosis, 348 prevalence, 347 treatment, 348 Acalculous cholecystitis, 765 Acanthosis nigricans, 463, 884 Accommodative esotropia, 604 Acetaminophen ingestion, 223–225 Achondroplasia, 131–132, 994 ACID-BASE metabolic acidosis, 836–837 metabolic alkalosis, 837, 838 regulation, 836 Acne vulgaris, 860–862 Acquired bleeding disorders disseminated intravascular coagulopathy, 373 hemorrhagic disease of newborn, 373 vitamin K deficiency, 373 Acquired nystagmus, 606 Acrocyanosis, 43 Acrodermatitis enteropathica, 860 Acromegaly, 428 Acromioclavicular (AC) dislocation, 515–516 Active tuberculosis, 958 Acute abdomen age-dependent presentation, 201 initial resuscitation, 202 non-surgical causes, 202–204

pain, 757, 758 surgical abdominal emergencies, 201–204 Acute Addison disease, 450 Acute appendicitis, 761, 762, 966 Acute bronchiolitis, 707, 708 Acute cerebellar ataxia, 575 Acute chest syndrome clinical presentation, 362 etiology, 362 treatment, 362 Acute hematogenous osteomyelitis, 469, 470 Acute intermittent porphyria (AIP), 161–162 Acute interstitial nephritis (AIN), 820, 821 Acute kidney injury (AKI), 819–820 Acute laryngitis, 638 Acute left MCA territory infarction, 1007 Acute leukemia, 375 Acute life-threatening event (ALTE), 725, 726 Acute lymphoblastic leukemia (ALL) ataxia telangiectasia, 376 Bloom syndrome, 376 clinical presentation, 375 diagnostic evaluation, 375, 376 epidemiology, 375 Fanconi anemia, 377 peripheral blood abnormalities, 375 treatment, 375 Trisomy 21, 376 Acute mastoiditis, 616 Acute myeloid leukemia (AML) associated syndromes/risk factors, 377 clinical presentation, 377 diagnostic evaluation, 377 epidemiology, 377 peripheral blood abnormalities, 377 treatment, 377 Acute osteomyelitis, 988 Acute otitis media (AOM) antimicrobial therapy, 613 clinical presentation, 613 common pathogens, 613 complications, 613 diagnosis, 613 management, 613 myringotomy and tympanostomy tubes, 615 risk factors, 613 signs of, 613 Acute pancreatitis, 763 Acute pericarditis, 679, 680 Acute postinfectious glomerulonephritis, 808, 809 Acute respiratory distress syndrome (ARDS), 960 Acute rheumatic fever (ARF), 675 Acute rhinosinusitis, 624–625 Acute right testicular torsion, 983

© Springer Nature Switzerland AG 2020 O. I. Naga (ed.), Pediatric Board Study Guide, https://doi.org/10.1007/978-3-030-21267-4

1137

1138 Acute stress disorder, 182 Acute tubular necrosis (ATN), 821 Acyanotic shunt lesions, 661 Acyclovir, 935 Adamantanes, 279 Addison disease, 450 Adenotonsillectomy, 629 Adenovirus, 276 clinical presentation, 277, 278 incubation period, 277 laboratory test, 278 mode of transmission, 277 respiratory viruses, 278 treatment, 278 Adenylate deaminase deficiency, 155 Adjustment disorders, 183 Adolescence, development and physiological changes boys, puberty sequence peak height velocity, 81 penile growth, 81 pubarche, 81 testicular volume increase, 81 emancipation and health care decisions, 83 girls, puberty sequence menarche, 81 peak height velocity, 81 pubarche, 81 thelarche, 81 routine health visit anticipatory guidance, 84, 85 immunization, 84 interview, 83 laboratory, 84 physical examination, 84 sexuality, 83 skeletal growth, 82 tanner staging, 82 cognitive development, 82 common problems, 82 family influence, 83 hematological changes, 82 hospitalizations, 82 identity development, 82 management of poor self-image, 82 psychological separation, 83 risks and conditions, 82 Adolescent idiopathic scoliosis (AIS), 475–476 Adolescent medicine, 1027–1032 Adolescents to young adulthood, vulnerable populations, 893 Adrenal disorders Addison disease, 450 adrenal insufficiency, 449, 450 Cushing syndrome, 448, 449 hyperaldosteronism, 449 PCOS, 451, 452 pheochromocytoma, 451 Adrenal hemorrhage, 981 Adrenal insufficiency, 450 Aedes mosquitoes, 288 Aggression associated conditions, 179 childhood aggression, 178 clinical presentation, 178, 179 etiology, 178 management, 179 screening and rating scales, 179 Alagille syndrome, 124–125, 671, 792 Albinism, 876 Albright hereditary osteodystrophy (AHO), 437

Index Alternative hypothesis, 918 Alkaptonuria, 149 Allergic rhinitis (AR) allergens, 391, 392 clinical presentation, 392 comorbidities, 391 diagnosis, 393 intermittent disease, 392 persistent disease, 392 treatment, 393, 394 Allergy adverse drug reactions cross-reactivity, 400 desensitization, 400 immune response, 400 specific drug reaction, 400 timing of reaction, 400 anaphylaxis (see Anaphylaxis) AR (see Allergic rhinitis (AR)) causes, 391 food (see Food allergy) hypersensitivity types, 391, 392 and immunology, 1066–1070 insects anaphylactic reactions, 401 diagnosis, 402 large local reactions, 401 systemic cutaneous reactions, 401 treatment, 402 mastocytosis (see Mastocytosis) serum sickness causes, 401 clinical presentation, 401 treatment, 401 skin testing ELISA, 403 first-generation antihistamine, 403 immunotherapy, 403, 404 indications, 402 medications, 402, 403 method of testing, 403 second-generation antihistamine, 403 specific triggers avoidance, 403 steroid, 403 urticaria (see Urticaria) Alopecia areata, 880–881 5-alpha-reductase deficiency, 455 Alpha thalassemia, 351 Alport syndrome, 619, 810 Altered mental status (AMS) coma, 219 consciousness, 219 CSF shunt malfunction, 221 definition, 219 delirium (agitation), 219 etiology of, 219, 220 Glasgow Coma Scale score, 220 infection, 221 lethargy, 219 management, 221 metabolic anomalies, 221 neoplasm, 221 obtunded, 219 toxic ingestions, 221 trauma, 221 Alveolar rhabdomyosarcoma, 387 Ambiguous genitalia, 52–53 Amblyopia, 605 Amenorrhea causes, 91

Index clinical approach, 91, 92 contraception, 95 dysfunctional uterine bleeding, 93 dysmenorrhea, 92 gynecomastia, 94 imperforate hymen, 93 labial adhesions, 93 ovarian cyst, 94 ovarian torsion, 93 PCOS, 92 primary amenorrhea, 91 scrotal masses, 94 secondary amenorrhea, 91 American Academy of Pediatrics (AAP), 1019 Amino acid metabolism disorder, 150 alkaptonuria, 149 glycine encephalopathy, 150 homocystinuria, 149 non-classic phenylketonuria, 149 phenylketonuria, 148 tyrosinemia type I, 150 tyrosinemia type II, 150–151 191-amino acid (191-AA) single chain polypeptide, 422 Aminoglycosides, 930 Amniocentesis, 112 Amniotic band disruption sequence, 139–140 Amoxicillin-clavulanate, 931 Ampicillin, 930–931 Amplified musculoskeletal pain syndrome (AMPS), 542, 543 Anaerobes Clostridium botulinum, 302 Clostridium difficile clinical presentation, 304 diagnosis, 304 prevention, 304 risk factor, 303 treatment, 304 Clostridium perfringens, 302, 303 Clostridium tetani common sources, 303 generalized tetanus, 303 neonatal tetanus, 303 prevention, 303 treatment, 303 foodborne botulism, 302 Anagen effluvium, 881 Anaphylactic shock, 257 Anaphylaxis biphasic presentation, 205 causes exercise, 398 food, 397 immunotherapy, 398 latex, 398 medications, 397 radiographic contrast, 397 stinging insects, 398 vaccination, 398 clinical presentation, 398 definition, 204 diagnosis, 205 differential diagnosis, 205, 398 home care, 206 pathophysiology, 204 patient disposition, 206 signs and symptoms, 397 symptoms, 205 treatment, 205, 206, 398, 399 ANCA-associated vasculitis (AAV), 543

1139 Ancylostoma duodenale, 332 Androgen insensitivity syndrome (AIS), 455 Anemia in newborn, 345, 346 in older children, 349 prevalence, 345, 346 Aneurysmal bone cyst (ABC), 502, 503, 993 Angelman syndrome, 126–127 Angular deformities blount disease (Tibia Vara), 485–486 genu valgum (knock-knee), 485 genu varum (bowleg), 484–485 Anisocoria, 593 Ankle fractures, 499–501 Ankle sprain, 517 Ankyloglossia/tongue-tie, 47, 631 Anorexia nervosa, 88 complications, 89 laboratory, 89 management, 90 subtypes, 89 Anterior cruciate ligament (ACL) injury, 482 Anterior fontanelle, 44 Anterior plagiocephaly, 130 Anterior shoulder (glenohumeral) dislocation, 515 Anterior uveitis, 590 Antibody deficiency syndromes CVID (see Common variable immune deficiency (CVID)) sIgAD (see Selective IgA deficiency (sIgAD)) THI (see Transient hypogammaglobulinemia of infancy (THI)) X-linked agammaglobulinemia BTK defect, 408 clinical presentation, 408 diagnosis, 408 treatment, 408 Anticholinergics ingestion, 226 Antidotes, 223 Antiemetics, 941 Anti-epileptic drugs (AED), 558 Antifreeze, 229 Anti-glomerular basement disease, 812, 813 Antihypertensive medications, 939–940 Anti-IL-1 agents, 531 Anti-IL-6 agent, 531 Anti-Müllerian hormone (AMH), 452 Antiparasitics, 936–937 Antiphospholipid antibody syndrome (APS), 534 Anti-pseudomonal penicillins, 931 Antisocial behaviors and delinquency associated conditions, 178 clinical presentation, 177 etiology, 177 FISTS, 178 management, 178 prevention, 178 rating scales, 178 risk factors, 177 Anti-TNF alpha agents, 531 Anti-ulcers, 940–941 Antivirals acyclovir, 935 foscarnet, 935 ganciclovir (IV), 935 nonnucleoside reverse transcriptase inhibitors, 936 nucleoside reverse transcriptase inhibitors, 935–936 other agents, 935 protease inhibitors, 936 valacyclovir, 935 valganciclovir, 935

1140 Anxiety disorder associated conditions, 181 developmental fears, 180 generalized anxiety disorders, 180 genetics, 180 management, 181 obsessive compulsive disorders, 181 panic disorder, 180 parenting styles, 180 prognosis, 181 screening and rating scales, 181 selective mutism, 180 separation anxiety disorder, 180 social phobia, 180 Aortic stenosis, 671, 672 Apert syndrome, 130 Apgar Score, 43 Aphthous ulcers, 630 Aplasia cutis congenita, 43 Aplastic anemia, 370 Aplastic crisis clinical presentation, 363 etiology, 363 laboratory test, 363 treatment, 363 Apnea, 699, 700 Apophyseal avulsion fracture, 996 Arbovirus dengue fever clinical presentation, 289 epidemiology, 288 laboratory test, 289 treatment, 289 West Nile virus clinical presentation, 288 diagnosis, 288 epidemiology, 288 treatment, 288 Arcanobacterium haemolyticum, 301, 302 Arnold–Chiari malformation type I, 564 Arnold–Chiari malformation type II, 564, 565 Arterial blood gases, 693 Arteriovenous malformations (AVMS), 568, 870 Arthritis associated with rheumatic fever, 469 differential diagnosis, 526 DIL, 536 IBD, 532 JIA complications of, 530 definitions, 525 ERA, 527 imaging, 530 laboratory abnormalities, 529, 530 MAS, 529 oligoarticular, 526, 527 polyarticular, 527 psoriatic, 528 systemic-onset, 528 treatment of, 530, 531 uveitis, 529 jSLE APS, 534 cardiac manifestations, 534 clinical presentation, 532 cutaneous manifestations, 532, 533 endocrine manifestations, 534 gastrointestinal manifestations, 534 general manifestations, 532 hematologic involvement, 534

Index laboratory evaluation, 534, 535 management, 535 musculoskeletal manifestations, 533 neuropsychiatric manifestation, 533 pulmonary manifestations, 534 renal manifestations, 533 localized scleroderma, 536, 537 MCTD, 537 NLE, 535 PSRA, 531 reactive arthritis, 532 Sjögren syndrome, 537 systemic scleroderma, 536 Arthrogryposis, 466 Ascaris lumbricoides clinical presentation, 332 diagnosis, 332 mode of transmission, 332 treatment, 332 Aseptic meningitis, 273, 338 Aspergillus species causes, 327 clinical presentation, 327 diagnosis, 327 mode of transmission, 327 treatment, 327 Asphyxial arrest, 251 Asthma acute asthma exacerbations, 714 assessment, 710, 711 beta-2 agonists, 712, 713 classification, 711 differential diagnosis for, 710 EIA, 711, 712 inhaled corticosteroids, 713 initial controller therapy, 713 leukotriene receptor antagonists, 714 management of, 712 pathophysiology, 710 symptoms and natural course, 709, 710 Asthma predictive index, 709 Astrocytoma, 383 Ataxia, 575 Ataxia telangiectasia, 376, 575, 576 clinical presentation, 413 diagnosis, 413 risk of malignancy, 413 treatment, 413 Atlantoaxial instability, 570, 571 Atlantoaxial subluxation, 472 Atopic (seasonal) allergic conjunctivitis, 589–590 Atopic dermatitis, 857–859 Atrial fibrillation (AF), 647, 653, 654 Atrial septal defect (ASD), 662, 663 Atrioventricular block (AVB), 658 Attention-deficit/hyperactivity disorders (ADHD) administration of medications to children, 176 associated conditions, 172 diagnosis, 172 differential diagnoses, 172, 173 management, 173–176 risk factors, 172 screening and rating scales, 172 Auditory brainstem response (ABR), 620 Autism screening, 25 Autism spectrum disorders diagnostic criteria, 168 differential diagnosis, 168 management, 169 prognosis, 169

Index screening and testing, 168 Autoimmune hemolytic anemia (AIHA) cold AIHA, 367 etiology, 366, 367 warm AIHA clinical presentation, 367 laboratory test, 367 treatment, 367 Autoimmune hepatitis (AIH), 796 Autoimmune thyroiditis, 430, 431 Autoinflammatory diseases CRMO, 540 FMF, 539, 540 periodic fever syndromes, 539 sarcoidosis, 540, 541 Automated external defibrillator (AED), 264 Autosomal aneuploidies, chromosome disorders down syndrome, 113–116 trisomy 13/Patau syndrome, 116–118 trisomy 18/Edwards syndrome, 116 Autosomal dominant (AD) inheritance autosomal dominant inheritance, 104 examples, 105 genetic transmission, 104 Autosomal dominant polycystic kidney disease (ADPKD), 818 Autosomal recessive (AD) inheritance examples, 106 genetic transmission, 105 new mutation, 106 uniparental disomy, 105 Autosomal recessive polycystic kidney disease (ARPKD), 817, 980 AV nodal tachycardia (AVNRT), 654 Avian influenza H5N1 clinical presentation, 279 epidemiology, 279 mode of transmission, 279 prevention, 279 Azithromycin, 302, 305–307, 309–313, 315–317, 325 B Bacillus anthracis, 301 Bacillus Calmette-Guérin (BCG) vaccine, 323 Bacillus cereus, 301 Back disorders adolescent idiopathic scoliosis, 475–476 back pain, 472–473 Scheuermann kyphosis, 476, 477 scoliosis, 474–475 spondylolisthesis, 474 spondylolysis, 473–474 Back pain, 472–473 Bacterial conjunctivitis, 587 causes, 586 clinical presentation, 587 management, 587 Bacterial infections cellulitis, 875 impetigo, 874–875 intertrigo, 875–876 necrotizing fasciitis, 876 staphylococcal scalded skin syndrome, 875 Bacterial meningitis, 309 Bacterial overgrowth syndrome, 782, 783 Bacterial tracheitis, 703, 704 Balanoposthitis, 847 Baloxavir, 279 Bannayan-Riley-Ruvalcaba syndrome (BRRS), 789 Bartonella henselae Citrobacter, 314

1141 clinical presentation, 313 diagnosis, 313 Klebsiella, 314 source, 313 surgical treatment, 314 treatment, 314 Bartter syndrome, 815 Basilar skull fracture, 207 Bat bites, 212 Becker muscular dystrophy, 572 Beckwith–Wiedemann syndrome (BWS), 136–137 Bedbugs, 866 Bedtime refusal/frequent awakening, 190–191 Behavioral healthcare adherence to medical regimens, 85 developmentally-appropriate psychosocial history, 85 risk taking behaviors, 85 stress, 85 violence, 85 Behavioral observation audiometry (BOA), 620 Behçet disease, 547, 548 Bell’s palsy, 574 Benign epilepsy with centrotemporal spikes (BECTS), 553, 554 Benign familial macrocephaly, 2 Benign familial neutropenia, 368 Benign joint hypermobility syndrome (BJHS), 542 Benign paroxysmal positional vertigo (BPPV), 617 Benign sleep myoclonus of infancy, 191 Berger disease, 809, 810 Bernard Soulier syndrome, 373 Beta blockers ingestion, 226 Beta lactam antibiotics, 930 Beta-2 agonists, 713 Beta-Thalassemia beta-thalassemia intermedia, 352 beta-thalassemia major, 352, 353 beta-thalassemia minor, 352 clinical manifestations, 351 Bezoars, 769 Bias confounding bias, 913 definition, 913 Hawthorne effect, 913 procedure bias, 913 pygmalion effect, 913 recall, 913 reduction, 913 selection, 913 Bifid uvula, 630–631 Bifidobacterium sp., 268 Bilateral cervical and brachial plexus plexiform neurofibromas, 1000 Biliary atresia, 790–791, 972 Bilirubin-induced neurologic dysfunction (BIND), 60 Biophysical profile test, 37 Biotin, 736 Biotinidase deficiency, 147 Bipolar disorder associated condition, 186 cyclothymic disorders, 186 diagnostic criteria, 186 early onset bipolar disorder, 186 lifetime prevalence, 186 management of, 186 prevention, 187 prognosis, 187 screening and rating scales, 186 type I, 186 type II, 186

1142 Birth defect amniotic band disruption sequence, 139–140 Goldenhar syndrome/hemifacial microsomia, 138 Pierre Robin sequence, 139 Poland sequence, 138–139 VACTERL association, 140 Black widow spider bite, 214 Blacklegged tick (Ixodes scapularis), 319 Bladder Exstrophy, 846 Blastomyces, 328 Blood gas analysis, 693 Blood pressure screening, 24 Bloom syndrome, 376 Blount Disease (Tibia Vara), 485–486 Blue sclera, 46 Body fluid composition, 825 Body odors, 164–165 Bohn nodules, 47 Bone-anchored hearing aid, 622 Bone and joint infection/inflammation acute hematogenous osteomyelitis, 469, 470 diskitis, 470 septic arthritis, 468–469 transient synovitis, 468 vertebral body osteomyelitis, 470 Bone and mineral disorders albright hereditary osteodystrophy, 437 familial hypocalciuric hypercalcemia, 437 hyperparathyroidism, 437, 438 hypocalcemia (see Hypocalcemia) hypoparathyroidism (see Hypoparathyroidism) pseudohypoparathyroidism, 436, 437 Bone marrow biopsy, 376 Bone marrow infiltration, 369 Bone tumors/tumors-like conditions aneurysmal bone cyst, 502, 503 Ewing sarcoma, 504, 505 osteochondroma, 503 osteoid osteoma, 503 osteosarcoma, 504 unicameral cyst, 502 Bordetella pertussis, 268 catarrhal phase, 311 convalescent phase, 311 diagnosis, 312 immunization, 312 incubation period, 311 paroxysmal phase, 311 pertussis complications, 311 prophylaxis, 312 thoracic pressure-related complications, 312 treatment, 312 Borrelia burgdorferi diagnosis, 320 early disseminated disease stage II, 320 early localized disease stage I, 320 incubation period, 320 late disseminated disease stage III, 320 treatment, 320 Botulism, 571 Botulism immune globulin (BIG), 302, 571 Bowel obstruction, 248 Bowel perforation with pneumoperitoneum, 963 Brachial plexus injuries (BPI) associated injuries, 48 classification, 48 clinical presentation, 48 diagnosis, 48 Duchenne-Erb palsy, 48

Index Klumpke palsy, 48 management, 49 Brachycephaly, 130 Bradycardia, 50 Brain abscess antimicrobial therapy, 341 causes, 340 cerebellar abscesses, 340 clinical presentation, 340 frontal lobe abscesses, 340 laboratory diagnosis, 340 neuroimaging, 340 prognosis, 341 surgical intervention, 341 Brain death, 252, 253 Brain malformations Arnold–Chiari malformation type I, 564 Arnold–Chiari malformation type II, 564, 565 corpus callosum, agenesis of, 565 Dandy-Walker malformation, 565, 566 lissencephaly, 565 polymicrogyria, 565 Brain tumors associated syndrome, 384 astrocytoma, 383 clinical presentation, 383 craniopharyngioma, 384 ependymoma, 384 hepatoblastoma associated syndromes/risk factors, 385 clinical presentation, 385 diagnosis, 385 incidence, 383, 384 treatment, 385 medulloblastoma, 383, 384 pathologic diagnosis, 383 pineoblastoma, 384 Branchial cleft cyst, 637–638 Branchio-oto-renal syndrome, 619 Breast milk, 268 and formula, 744 Breastfeeding, 269 colostrum, 741, 742 mature breast milk, 742–744 transition milk, 742 Brief resolved unexplained event (BRUE), 725, 726 Bronchiectasis, 716, 717 Bronchogenic cyst, 704 Bronchopulmonary dysplasia (BPD), 708, 709, 836 Brown recluse spider bite, 214–215 Brown-Vialetto-Van Laere syndrome, 737 Brucellosis causes, 312 diagnosis, 312 risks, 312 treatment, 313 Brugada syndrome, 686 Bruises, 896 Bruton tyrosine kinase (BTK), 408 Bruxism, 183 Buccal infections, 310 Bulging fontanelle, 44 Bullous impetigo, 297 Bullying, 179, 180 Burkitt lymphoma, 380, 381 Burns, 270, 754, 896 deep partial thickness burn, 215 electrical burns, 216 extensive burns treatment, 217

Index full thickness burn, 215 inhalation injury, 216 Palmar method, 216 partial thickness burn, 215 Rule of Nines, 216 superficial burn, 215 supportive home therapy, 216, 217 Button battery ingestion, 974 C Café-au-Lait Macules (CALM), 878 Calcaneovalgus foot, 489–490 Calcium, 732 Campylobacter species clinical presentation, 304 diagnosis, 305 epidemiology, 304 treatment, 305 Candida albicans, 325 Candida species candidal diaper dermatitis clinical presentation, 326 treatment, 326 etiology, 325 neonates, 327 oral thrush clinical presentation, 326 incidence, 325 infection sources, 326 recurrence, 326 risk of infection, 326 treatment, 326 vulvovaginitis clinical presentation, 327 risk factors, 326 treatment, 327 Candidal diaper dermatitis, 326 Capillary blood gases (CBG), 693 Capillary malformations, 869, 870 Caput succedaneum, 45 Carbamazepine ingestion, 226–227 Carbapenems, 932 Carbohydrate metabolism disorder, 156 adenylate deaminase deficiency, 155 fructokinase deficiency, 155 fructose 1,6-diphosphatase deficiency, 155 galactokinase deficiency, 153 galactosemia, 153 glycogen storage diseases, 153 Pompe disease, 154 Von Gierke disease, 154 Carbohydrates, 741 Carbon monoxide (CO) poisoning, 228 Carbuncle, 290 Cardiac arrhythmias AF, 653, 654 atrial rates, 653 AVB, 658 ECGs, 652 history for, 646 LQTS, 656, 657 PACs, 652, 653 prolonged QT interval, 656 PVC, 656 rhythm disturbance, 646 sinus arrhythmia, 652 SSS, 655 SVT, 654, 655

1143 VF, 657 VT, 657 WPW syndrome, 655 Cardiac glycosides ingestion, 227 Cardiac tamponade, 680, 681 Cardiogenic shock, 255 Cardiology, 1099–1104 Cardiopulmonary resuscitation (CPR) AED, 264 chest compression depth, 263, 264 chest compression rate, 263 chest compression techniques, 264 chest compression to breath ratio, 264 Cardiothoracic radiology active tuberculosis, 958 acute respiratory distress syndrome, 960 congenital diaphragmatic hernia, 949 congenital lobar emphysema, 951 congenital pulmonary airway malformation, type II, 950 croup (laryngotracheobronchitis), 954 cystic fibrosis, 960 Ebstein’s anomaly, 953 foreign body aspiration, 955 long gap esophageal atresia with tracheoesophageal fistula, 952 lymphoma, 961 meconium aspiration syndrome, 949 pulmonary interstitial emphysema, 948 pulmonary thromboembolism, 959 round pneumonia, 956 surfactant deficiency disease, 947 tetralogy of Fallot, 953 Cardiovascular disorders arrhythmias AF, 653, 654 atrial rates, 653 AVB, 658 ECGs, 652 history for, 646 LQTS, 656, 657 prolonged QT interval, 656 PVC, 656 rhythm disturbance, 646 sinus arrhythmia, 652 SSS, 655 SVT, 654, 655 VF, 657 VT, 657 WPW syndrome, 655 chest pain arrhythmias, 643 cardiac abnormalities, 643 clinical approach to, 644–645 coronary artery diseases, 643 gastrointestinal causes of, 644 infections/autoimmune disorders, 643 noncardiac causes of, 644 noncardiac chest pain, 643 psychogenic causes of, 644 red flags, 643 respiratory causes of, 644 congenital heart defects, 661, 662 dilated cardiomyopathy, 684 dyslipidemia, 686, 687 fetal cardiac output, 658 genetics, 674, 675 HCM, 685 heart failure causes, 682, 683 clinical presentation, 683

1144 Cardiovascular disorders (cont.) diagnostic evaluation, 683 management, 683, 684 infection/vasculitis acute pericarditis, 679, 680 ARF, 675 cardiac tamponade, 680, 681 IE, 678, 679 KD, 675–678 myocarditis, 681, 682 left-to-right shunts ASD, 662, 663 atrioventricular canal defects, 664, 665 PDA, 665, 666 VSD, 663, 664 murmur, 660, 661 obstructive lesions aortic stenosis, 671, 672 coarctation of aorta, 673, 674 HLHS, 674 PPS, 671 pulmonary valve stenosis, 670, 671 restrictive cardiomyopathy, 684, 685 right-to-left shunt lesions Ebstein anomaly, 668, 669 TAPVR, 669, 670 tetralogy of fallot, 666, 667 transposition of great arteries, 667, 668 tricuspid atresia, 667 truncus arteriosus, 669 sudden cardiac death, 686 superior vena cava syndrome, 686 syncope, 645 Caregiver-fabricated illness, 900 Carpenter syndrome, 130 Cartilage hair hypoplasia, 369 Castleman’s disease, 276, 636 Cat bites, 212 Cat scratch disease, 313–314, 635 Cataracts, 594, 595 Caustic esophageal injuries, 768 Caustic ingestion, 230 Cavus foot, 490 CD40 ligand (CD40L), 414 Cefotaxime, 295 Ceftriaxone plus vancomycin, 295 Celiac disease, 779, 780 Cellular immunity CMC clinical presentation, 410 diagnosis, 410 feature, 410 treatment, 410 DiGeorge syndrome CATCH 22, 409 clinical presentation, 410 diagnosis, 410 prognosis, 410 treatment, 410 types, 410 HIES (see Hyper-IgE syndrome (HIES)) XLP syndrome (see X-linked lymphoproliferative (XLP) syndrome) Cellulitis, 875 Central line-associated bloodstream infections (CLABSI), 270, 327 Central nervous system (CNS) infections brain abscess (see Brain abscess) encephalitis causes, 336 clinical presentation, 336 definition, 336

Index diagnosis, 336, 337 epidemiology, 336 initial evaluation, 336 treatment, 337 meningitis (see Meningitis) Cephalexin, 290, 298 Cephalohematoma, 45 Cephalosporins (penicillinase-resistant), 931–932 Cerebral edema asphyxial arrest, 251 causes, 233 cytotoxic edema, 251 initial resuscitation, 233 interstitial edema, 252 osmotic cerebral edema, 252 pathophysiology, 233 risk factors, 233 signs, 233 symptoms, 252 treatment, 233 vasogenic edema, 251 Cerebral palsy, 510, 578, 579 Cerebral salt wasting, 427, 428 Cerebral venous thrombosis, 569 Cerebrospinal fluid analysis, 339 Cervical intraepithelial neoplasia (CIN), 289 Cervical lymphadenitis, 634–635 Cervical spine injury, 209–210 Cestodes (platyhelminthes), 333 Chalazion, 591–592 Chediak–Higashi syndrome, 369 CHS1/LYST gene, 407 clinical presentation, 407 diagnosis, 407 risk of malignancy, 407 treatment, 407 Chemical conjunctivitis, 589 Chemoprophylaxis, 269, 340 Chest breast hypertrophy and galactorrhea, 49 clavicle fracture, 49 crepitation, 49 pain, 508 pectus excavatum, 49 supernumerary nipple, 49 Turner syndrome, 49 Chiari malformation type I and type II, 564 Chickenpox, 274, 275 Child abuse clinical background, 895 clinical features, 897 clinical presentation, 896, 897 diagnosis, 897 management, 897 report, 897 risk factors, 895 Child-care centers, 267 Childhood absence epilepsy, 552, 553 Childhood schizophrenia clinical presentation, 188 differential diagnosis, 188 management, 188 prognosis, 188 risk factors, 188 screening, 188 Child neglect, 898 Chlamydia pneumoniae, 308, 395 clinical presentation, 305 diagnosis, 305 epidemiology, 305

Index treatment, 305 Chlamydia trachomatis, 97 conjunctivitis due to clinical presentation, 305 epidemiology, 305 treatment, 305 pneumonia due to clinical presentation, 306 diagnosis, 306 etiology, 306 treatment, 306 Chlamydophila psittaci, 305 Chloride (Cl), 732, 835 Choanal atresia, 46, 623, 695 Cholangitis, 765 Cholecystitis, 764–765 Choledocholithiasis, 765 Cholelithiasis, 764 Cholesteatoma, 616 Chorioamnionitis clinical history, 41 clinical presentation, 41 management, 41 Chorionic villus sampling (CVS), 112, 113 Chromium, 732 Chronic abdominal pain, 760 Chronic benign neutropenia, 368 Chronic bilirubin encephalopathy, 61 Chronic diarrhea, 784–785 Chronic granulomatous disease (CGD) clinical presentation, 406 diagnosis, 406 treatment, 407 Chronic hypoxia, 700 Chronic illness, 893, 894 Chronic keratoconjunctivitis, 306 Chronic liver disease, 753 Chronic lymphocytic leukemia (CLL), 378 Chronic mucocutaneous candidiasis (CMC) clinical presentation, 410 diagnosis, 410 feature, 410 treatment, 410 Chronic myelogenous leukemia (CML), 378 Chronic nonprogressive headache, 562 Chronic pain syndrome, 894 Chronic progressive headache, 562 Chronic recurrent multifocal osteomyelitis (CRMO), 540 Chronic sinusitis, 625 Chronic stridor differential diagnosis choanal atresia, 695 laryngeal web, 696 laryngomalacia, 695 laryngotracheoesophageal cleft, 697 macroglossia, 697 SGS, 696 subglottic hemangioma, 697 VCP, 695, 696 Chronic suppurative otitis media (CSOM), 615 Chronic urticaria causes, 395 diagnosis, 395 differential diagnosis, 395 treatment, 395 Circadian rhythm disorder, 191 Circumcision, 847, 848 Citrobacter, 314 Clavicular fracture, 494

1145 Cleft lip and palate, 632–633 Clindamycin, 291, 293–296, 298–300, 303, 304, 311, 331, 932 Clonidine ingestion, 227 Clonorchis sinensis, 333 Clostridium botulinum causes, 302 clinical presentation, 302 diagnosis, 302 infant botulism, 302 treatment, 302 Clostridium difficile, 268 clinical presentation, 304 diagnosis, 304 prevention, 304 risk factor, 303 treatment, 304 Clostridium perfringens, 302, 303 Clostridium tetani common sources, 303 generalized tetanus, 303 neonatal tetanus, 303 prevention, 303 treatment, 303 Clover-leaf skull or Kleeblattschädel anomaly, 130 Clubfoot (talipes equinovarus), 489 Coagulation disorders acquired bleeding disorders disseminated intravascular coagulopathy, 373 hemorrhagic disease of newborn, 373 vitamin K deficiency, 373 bleeding disorder, 373 erythrocytosis, 374 inherited bleeding disorders hemophilia A and B, 374 Von Willebrand disease, 374 polycythemia vera, 374, 375 Coalescent mastoiditis, 1011 Cobalamin, 354 Coccidioidomycosis clinical presentation, 328 diagnosis, 328 mode of transmission, 328 treatment, 328 Cochlear implants, 622 Cognitive and neurodevelopmental disorders autism spectrum disorders diagnostic criteria, 168 management, 169 prognosis, 169 screening and testing, 168 intellectual disability causes, 167 classification, 167 clinical presentation, 167 differential diagnosis, 168 management, 168 prevalence, 167 sensory over-responsivity, 169 Cognitive behavioral therapy (CBT), 760 Cold agglutinin disease, 367 Cold autoimmune hemolytic anemia, 367 Cold panniculitis, 631 Coloboma, 46, 593–594 Colonic atresias, 774 Colostrum and mature breast milk, 741, 742 Combined antibody and cellular immunodeficiency ataxia-telangiectasia (see Ataxia-Telangiectasia) SCID (see Severe combined immunodeficiency (SCID)) Wiskott–Aldrich syndrome (see Wiskott–Aldrich syndrome) XHIM (see X-linked hyper-IgM syndrome (XHIM))

1146 Common variable immune deficiency (CVID) clinical presentation, 409 definition, 409 diagnosis, 409 risk of malignancy, 409 treatment, 409 Communication disorder language disorders, 169 phonological speech disorders, 169 social communication disorder, 169–170 Compartment syndrome, 500 Complement defect clinical presentation, 414 diagnosis, 414 initial defect, 414 terminal defect, 414 treatment, 415 Complementary and alternative medicine (CAM), 910, 911 Complete AV canal defect (CAVC), 662 Concussion definition, 234 epidemiology, 235 management, 235, 236 risk factors, 235 signs and symptoms, 235 Conduct disorder associated conditions, 177 management, 177 prevention, 177 prognosis, 177 screening and rating scales, 177 Conductive hearing loss (CHL), 618 Condyloma acuminatum, 874 Condylomata acuminata, 289 Confidence interval, 919 Confounding bias, 913 Congenital adrenal hyperplasia (CAH) causes, 453 clinical presentation in females, 453 in males, 454 diagnosis, 454 treatment, 454 Congenital chloride, 785 Congenital diaphragmatic hernia (CDH), 949 clinical presentation, 74 delivery room management, 74 imaging studies, 74 laboratory tests, 74 management, 75 Congenital esotropia, 603 Congenital hearing loss, 618–619 Congenital heart block (CHB), 535 Congenital heart defects, 661, 662 Congenital hypopituitarism clinical presentation, 424 diagnosis, 424 Hall–Pallister syndrome, 423 septo-optic dysplasia, 424 treatment, 424 Congenital hypoplastic anemia, see Diamond–blackfan anemia Congenital hypothyroidism causes, 429 clinical presentation, 429 laboratory test, 429 prognosis, 429 treatment, 429 Congenital lobar emphysema, 705, 706, 951 Congenital long Qt syndrome (LQTS), 656, 657 Congenital melanocytic nevi (CMN), 879

Index Congenital muscular torticollis, 471 Congenital myotonic dystrophy, 572 Congenital nephrotic syndrome, 807 Congenital neutropenia, 368 Congenital pulmonary adenomatoid malformation, 705 Congenital pulmonary airway malformation, 950 Congenital rubella syndrome, 287 Congenital sensory nystagmus, 606 Congenital syphilis, 320, 321 clinical presentation, 71 diagnosis, 71 infection, 71 management, 71 transmission rate, 71 treponema-specific tests, 71 Congenital toxoplasmosis clinical presentation, 70 infection in first trimester, 70 infection in third trimester, 70 transmission risk, 70 treatment, 70 Conjugated hyperbilirubinemia, 60 Connective tissue dysplasias Ehlers–Danlos Syndrome (EDS), 134–135 Marfan syndrome, 132–134 osteogenesis imperfecta, 135–136 Constitutional delay of growth and puberty (CDGP), 419 Contact dermatitis, 859 Contact lens-related problems, 606 Contraction stress test, 37 Copper, 732, 733 Corneal abrasion, 602 Corpus callosum, agenesis of, 565 Corynebacterium diphtheriae, 300 Cough, 697, 698 Cowden syndrome (CS), 384, 789 Craniofacial defects craniosynostosis syndromes, 130–131 Treacher Collins syndrome, 129–130 Waardenburg syndrome, 129 Craniopharyngioma, 384, 425, 426, 1012 Craniosynostosis syndromes, 3–4, 567 abnormalities, 131 causes, 130 health supervision and preventive care, 131 prevalence, 130 types, 130 Cri-du-chat syndrome, 122, 674 Crigler-Najjar syndrome, 791 type 1, 60, 792 type 2, 60, 792 Critical care, 1049–1052 Critical congenital heart diseases screening, 39 Crohn’s disease, 778, 976 Croup (laryngotracheobronchitis), 954 Crouzon syndrome, 130 Cryptorchidism, 52, 849, 850 Cryptosporidiosis clinical presentation, 330 mode of transmission, 329 treatment, 330 Cryptosporidium, 269, 329 Culex mosquitoes, 288 Cultural issues in medical care, 891, 892 Cushing syndrome, 448, 449 Cutaneous lupus erythematosus (CLE), 871–872 Cutis marmorata, 44 Cyanide ingestion, 228–229 Cyanosis, 700 Cyanotic lesions, 661–662

Index Cyclic neutropenia clinical presentation, 368 laboratory test, 368 treatment, 368 Cyclic vomiting, 759, 760 Cyclothymic disorders, 186 Cystic fibrosis (CF), 753, 780, 781, 960 clinical presentation, 717, 718 complications, 718–720 diagnosis, 719 dysfunction/absence, 717 management, 719, 720 therapies for treatment, 720 Cystic kidney diseases ADPKD, 818 ARPKD, 817 Laurence-Moon-Bardet-Biedl syndrome, 818, 819 multicystic dysplastic kidney disease, 819 NPH, 818 Cystinosis, 815, 816 Cystosarcoma phyllode, 91 Cytochrome P450 (CYP) enzyme inhibitors, 928 Cytotoxic edema, 251 D Dacryostenosis, 592 Dandy–Walker malformation, 565, 566 De Grouchy syndrome, 123 Death, 887, 888 Deep hematoma of thigh, 516–517 Deep partial thickness burn, 215 Deformational plagiocephaly, 3, 4 Dehydration, 832, 833 Delayed dental eruption, 633 Delayed puberty, 446 Delayed separation of umbilical cord, 51 Delivery room care initial management, 38 resuscitation indications, 38 newborn resuscitation, 38 process steps, 38 withholding resuscitation, 38 temperature control, 38 Dengue fever clinical presentation, 289 epidemiology, 289 laboratory test, 289 treatment, 289 Dense deposit disease, 811 Dental trauma, 634 Denys–Drash syndrome, 454, 455, 809 Depressed anterior fontanelle, 44 Depressed skull fracture with traumatic subdural hemorrhages, 1014 Depression associated conditions, 185 CBT strategies, 185 diagnostic criteria, 184 differential diagnosis, 184 interpersonal therapy, 185 lifestyle modification, 185 male–female ratio, 184 management, 185 medications, 185 prognosis, 185, 186 risk factors, 184 screening and rating scales, 27, 185 Dermal melanocytosis, 878

1147 Dermal tumors dermoid cyst, 880 granuloma annulare, 879–880 mastocytosis, 880 Dermatology, 1122–1125 acanthosis nigricans, 884 acne, 860–862 dermal tumors dermoid cyst, 880 granuloma annulare, 879–880 mastocytosis, 880 disorder of pigmentation albinism, 876 Café-au-Lait macules, 878 dermal melanocytosis, 878 nevus of Ito, 878 nevus of Ota, 878 pityriasis alba, 876–877 post inflammatory hypo/ hyper-pigmentation, 877 vitiligo, 877 eczematous dermatitis acrodermatitis enteropathica, 860 atopic dermatitis, 857–859 contact dermatitis, 859 keratosis pilaris, 859 nickel dermatitis, 860 rhus dermatitis (Poison Ivy), 859 seborrheic dermatitis, 860 factitious disorders, 884 fungal infections onychomycosis, 865 tinea capitis, 862, 863 tinea corporis, 863 tinea cruris, 864 tinea pedis, 864 tinea versicolor, 864 genodermatoses ectodermal dysplasia, 882–883 epidermolysis bullosa, 881–882 ichthyosis vulgaris, 883 incontinentia pigmenti, 882 hair disorders alopecia areata, 880–881 anagen effluvium, 881 telogen effluvium, 881 traction alopecia, 881 trichotillomania, 881 hypersensitivity reactions cutaneous lupus erythematosus, 871–872 erythema multiforme, 872 Stevens–Johnson syndrome, 870, 872–873 toxic epidermal necrolysis, 872–873 urticaria, 871 infestations bedbugs, 866 papular urticaria, 866 pediculosis, 865 scabies, 865 Langerhans cell histiocytosis, 883–884 lichenoid disorders, 867–868 melanocytic tumors, 878, 879 papulosquamous disorders pityriasis rosea, 867 psoriasis, 866–867 pearls and pitfalls, 884–885 skin bacterial infections, 874–876 viral infections, 873–874 skin disorders of newborn diaper dermatitis, 857

1148 Dermatology (cont.) erythema toxicum, 855, 856 miliaria, 855–856 nevus Sebaceus (of Jadassohn), 856–857 transient neonatal pustular melanosis, 855 vascular malformations, 869, 870 vascular tumors infantile hemangiomas, 868–869 Kasabach–Merritt phenomenon, 869 viral infections of the skin, 873–874 Dermoid cyst, 880 Descriptive statistics absolute risk, 918 attributable risk, 918 odds ratio, 917 relative risk, 918 reliability (precision), 917 sample size, 917 statistical hypothesis, 918–919 validity (accuracy), 917 Developmental disorders cerebral palsy, 579 dyskinetic cerebral palsy, 580 Rett syndrome, 581 spastic diplegia, 580 spastic hemiplegia, 579, 580 spastic quadriplegia, 580 Developmental dysplasia of hip (DDH), 53, 476–478, 480 Developmental screening, 25 developmental red flags, 14 fine motor 1 month, 5 2 month, 5 3 month, 5 4 month, 6 6 month, 6 7 month, 7 9 month, 7 12 month, 8 14 month, 9 15 month, 9 18 month, 9 24 month, 10 3 year, 10 4 year, 10 5 year, 11 6 year, 11 block play, 12, 13 catching objects, 13 climbing stairs, 14 drawing, 12 social skills, 12 walking and running, 13 following objects, 11 gross motor 1 month, 5 2 month, 5 3 month, 5 4 month, 6 6 month, 6 7 month, 7 9 month, 7 12 month, 8 14 month, 8 15 month, 9 18 month, 9 24 month, 9 3 years, 10 4 years, 10

Index 5 years, 10 6 years, 11 newborn, 4 language expressive, 11 1 month, 5 2 month, 5 3 month, 5 4 month, 6 6 month, 7 7 month, 7 9 month, 8 12 month, 8 14 month, 9 15 month, 9 18 month, 9 24 month, 10 3 year, 10 4 year, 10 5 year, 11 6 year, 11 receptive, 11 primitive reflexes, 11 social/communication/problem solving 1 month, 5 2 month, 5 3 month, 5 4 month, 6 6 month, 6 7 month, 7 9 month, 7 12 month, 8 14 month, 9 15 month, 9 18 month, 9 24 month, 10 3 year, 10 4 year, 10 5 year, 11 6 year, 11 newborn, 5 speech intelligibility, 11 tools, 4 visual and reflex, newborn, 5 Diabetes insipidus (DI), 428 central DI causes, 426 clinical presentation, 426 diagnosis, 426 treatment, 426 definition, 426 nephrogenic DI causes, 426 clinical presentation, 426 diagnosis, 426 treatment, 426 Diabetes mellitus (DM), 509 DKA (see Type 2 Diabetes Mellitus) medical evaluation, 461 MODY, 461 obesity causes, 462 definition, 462 obesity-related conditions, 462, 463 Pediatric Metabolic Syndrome, 462 treatment, 463 outpatient management, 461 type 1 (see Type 1 Diabetes Mellitus) type 2 (see Type 2 Diabetes Mellitus)

Index Diabetic ketoacidosis (DKA) acidosis resolution, 460 cerebral edema, 233–234 classification, 460 degrees of severity, 232 diagnosis, 232, 460 insulin, 460 monitoring, 461 life threatening complications, 234 pathophysiology, 232, 233 in pediatric patients, 232–233 precipitating factors, 460 treatment, 460 Diagnostic errors, 922 Diamond–blackfan anemia clinical presentation, 356 etiology, 356 laboratory test, 357 prognosis, 357 treatment, 357 Diaper dermatitis, 857 Diarrhea acute diarrhea, 783 antibiotics and drug of, 784 chronic diarrhea, 784–785 clinical presentation, 783 disaccharide intolerance, 785–786 extraintestinal manifestations, 784 management of, 783, 784 Diastasis recti, 50 Diffuse ST elevation, 650 DiGeorge syndrome, 125–126, 673, 674 CATCH 22, 409 clinical presentation, 410 diagnosis, 410 prognosis, 410 treatment, 410 types, 410 Digitalis, 227 Dilated cardiomyopathy, 684 Diphtheria and tetanus toxoids and acellular pertussis vaccine (DtaP), 16 Diphyllobothrium latum, 355 Direct antiglobulin test (DAT) Coombs test, 347 Disaccharide intolerance, 785–786 Disciplinary approaches, 888 Discoloration of scrotum, 52 Disease-modifying antirheumatic drugs (DMARDs), 531 Disinhibited social engagement disorder, 182–183 Diskitis, 470 Disorder of pigmentation albinism, 876 Café-au-Lait macules, 878 dermal melanocytosis, 878 nevus of Ito, 878 nevus of Ota, 878 pityriasis alba, 876–877 post inflammatory hypo/hyper-pigmentation, 877 vitiligo, 877 Disorders of sexual development (DSD) 5-alpha-reductase deficiency, 455 AIS, 455 ambiguous genitalia, 453 CAH (see Congenital adrenal hyperplasia (CAH)) causes, 452, 456 Denys–Drash syndrome, 454, 455 sex chromosome, 453 Swyer syndrome, 455

1149 46 XX females, 452, 453 46 XY males, 452 Disseminated gonococcal infection (DGI), 96, 307 Disseminated intravascular coagulopathy, 373 Distal intestinal obstruction syndrome (DIOS), 781 Distal RTA type I, 814 Distributive shock, 255–257 Diuretics, 938–939 Divorce, 888 Dizygotic twins, 55 Dog bites, 212 Down syndrome (DS), 114, 510 abnormalities, 114 audiology referral, 116 car safety seat evaluation, 116 clinical findings, 113 clinical investigations, 115 counseling, 115 early childhood intervention, 115 ears-nose-throat/otolaryngology referral, 116 endocrine referral, 116 eye evaluation, 116 hematology referral, 116 immunizations, 116 microarray analysis, 113 minor anomalies, 115 parental recurrence risk, 113 physical examination, 115 physical therapy and exercise, 116 prevalence, 113 pulmonologist referral, 116 risk of recurrence, 113 speech and occupational therapy, 115 Doxycycline, 291, 305, 306, 313, 314, 319–321, 331, 341 Drowning air-filled swimming aids, 238 classification, 236 definition, 236 disposition, 237 epidemiology, 236 management, 236, 237 mechanism of injury, 236 personal flotation devices/life jackets, 238 prevention and guidance, 237, 238 swim lessons, 238 Drug-induced lupus (DIL), 536 Duchenne muscular dystrophy, 572 Duchenne-Erb palsy, 48 Duodenal atresia, 773, 969 Duodenitis, 772 Dwarfism, 131–132 Dyscalculia, 170 Dysfunctional uterine bleeding, 93 Dysgraphia, 170 Dyskinetic cerebral palsy, 580 Dyslipidemia, 27, 459, 686, 687 Dysmenorrhea, 92 Dysmorphology, 103 Dyssomnias, 191 Dystonic reactions, 578 E Eagle–Barrett Syndrome, 51, 845–846 Ear lacerations, 211, 212 Ear, nose, and throat, 1095–1099 Early childhood caries, 633–634 Early onset breastfeeding jaundice, 58–59 Early term birth, 35

1150 Ears acute mastoiditis, 616 AOM, 613–614 BPPV, 617 cholesteatoma, 616 CSOM, 615 external ear (Pinna) foreign body, 612 hematoma, 612–613 labyrinthitis, 616–617 malformed ears, 46 Meniere’s disease, 618 otalgia, 611 otitis externa, 612 otitis media with effusion, 614–615 preauricular pits/sinus, 611 recurrent acute otitis media, 614 vertigo, 617 Eating disorders, 511 anorexia nervosa clinical presentation, 89 complications, 89 laboratory, 89 management, 90 subtypes, 89 bulimia clinical presentation, 90 diagnostic criteria, 90 management, 90 non-purging subtype, 90 purging subtype, 90 indication of hospitalization, 88 morbidity and mortality, 88 suspicious behaviors, 88 Ebstein’s anomaly, 668, 669, 953 Ecchymoses, 44 Eclampsia, 42 Ectodermal dysplasia, 882–883 Eczema herpeticum, 273 Eczematous dermatitis acrodermatitis enteropathica, 860 atopic dermatitis, 857–859 contact dermatitis, 859 keratosis pilaris, 859 nickel dermatitis, 860 rhus dermatitis (Poison Ivy), 859 seborrheic dermatitis, 860 Educational neglect, 898 Egg allergy, 279, 341 Ehlers–Danlos Syndrome (EDS), 135 abnormalities, 134, 135 classical/hypermobile EDS, 134 diagnosis, 134 health supervision and preventive care, 135 prevalence, 134 vascular EDS, 134 Ehrlichiosis clinical presentation, 319 epidemiology, 319 laboratory findings, 319 treatment, 319 Electrical burns, 216 Emancipated minors, 83 Embden–Meyerhof–Parnas pathway, 364 Emergency child support flail chest, 258 hemothorax, 258 PARDS (see Pediatric acute respiratory distress syndrome (PARDS))

Index shock (see Shock) Emergency life support, see Ventilatory support Emergency medicine, 1044–1049 Emotional neglect, 898 Encephalitis, 273 causes, 336 clinical presentation, 336 definition, 336 diagnosis, 337 epidemiology, 336 initial evaluation, 336 treatment, 337 Endocrinology, 1070–1078 adrenal disorders (see Adrenal disorders) bone and mineral disorders (see Bone and mineral disorders) DM (see Diabetes Mellitus (DM)) DSD (see Disorders of sexual development (DSD)) growth disorder (see Growth disorder) pituitary gland (see Pituitary gland disorder) puberty (see Puberty disorder) rickets (see Rickets) thyroid disorder (see Thyroid disorders) Enteroaggregative E. coli (EAEC), 317 Enterobius vermicularis clinical presentation, 332 diagnosis, 332 mode of transmission, 331 treatment, 332 Enterococcal infections, 301 Enterococcus faecalis, 301 Enterococcus faecium, 301 Enterohemorrhagic E. coli (EHEC), 317 Enteroinvasive E. coli (EIEC), 317 Enteropathogenic E. coli (EPEC), 317 Enterotoxigenic E. coli (ETEC), 317 Enterovirus non-polio virus (see Non-polio virus) poliomyelitis (see Poliomyelitis) Enthesitis-related arthritis (ERA), 527 Environmental safety counseling bicycle injuries prevention, 30 breathing threats prevention, 30 drowning prevention, 29 falls prevention, 30 fire and burns prevention, 29 gun accidents prevention, 30 mosquito bites prevention, 31, 32 motor vehicle injuries prevention, 29 poisoning prevention, 30 sunburn injuries prevention, 31 Enzyme-linked immunosorbent assay (ELISA), 403 Eosinophilic esophagitis (EE), 768 Ependymoma, 384 Epidemiological research methods false negative, 916 false positive, 915 incidence, 915 likelihood ratio, 916–917 negative predictive value, 916 positive predictive value, 916 prevalence, 915 sensitivity, 916 specificity, 916 Epidermolysis bullosa (EB), 881–882 Epididymitis (gonococcus), 307 Epidural hematoma, 208 Epiglottitis, 309, 310, 703 Epilepsy mimics, 558, 559 Epilepsy syndromes, 557

Index Epinephrine, 397 Epispadias, 52 Epistaxis, 623 Erysipelas GAS, 298 Erythema infectiosum, 276, 277 Erythema multiforme (EM), 394, 872 Erythema toxicum, 855, 856 Erythroblastosis, 345–348 Erythrocytosis clinical presentation, 374 definition, 374 primary, 374 Erythropoietic protoporphyria, 162–163 Escherichia coli acute bacterial meningitis, 316 causes, 316 enteric infections, 317 intra-abdominal infections, 317 pneumonia, 317 Esophageal atresia, 771 Esophageal stenosis, 771 Esophagus bezoars, 769 caustic esophageal injuries, 768 eosinophilic esophagitis, 768 esophageal atresia, 771 esophageal stenosis, 771 foreign body, 769–771 gastroesophageal reflux, 767, 768 reflux esophagitis, 771 rumination, 769 Esotropia, 603, 604 Ethics, 1128–1131 battery, 903 in cochlear implants, 908 complementary and alternative medicine, 910, 911 consents, 905–907 death and dying children, 909 financial conflict of interest, 911 in genetics, 908 good Samaritan Laws, 903 imperiled newborn infants, 908–909 medical decision-making, 904, 905 medical testimony, 911 negligence and malpractice, 903 organ donation/transplantation, 909 principles, 903, 904 research involving children, 909, 910 Ethnic neutropenia, 368 Ethylene glycol ingestion, 229 Ewing sarcoma, 504, 505, 990 Exanthem Subitum, 276 Exercise-induced asthma (EIA), 711, 712 Exercise-induced bronchoconstriction, 509 Exfoliative toxins, 292 Exotoxin, 292 Exotropia, 603, 604 Extreme low birth weight (ELBW), 35 Eye foreign body, 603 Eye prophylaxis, 39 Eyes Blue sclera, 46 coloboma, 46 congenital glaucoma, 46 congenital myasthenia gravis, 46 Horner syndrome, 46 red reflex examination, 46 brushfield spots, 46 strabismus, 46

1151 subconjunctival hemorrhage, 46 trauma clinical evaluation, 600 closed-globe injuries, 601 corneal abrasion, 602 eye foreign body, 603 history, 600 hyphema, 603 open-globe injuries, 601 orbital fracture, 602 retinal detachment, 601 traumatic iritis, 601 treatment, 601 F Fabry disease, 159 Facial angiofibromas, 560 Factitious disorders, 884 Failure to pass meconium in 48h of life, 72 Failure to thrive (FTT), 752 Familial adenomatous polyposis (FAP), 789 Familial benign hypercalcemia, see Familial hypocalciuric hypercalcemia Familial hypercholesterolemia, 163 Familial hypocalciuric hypercalcemia, 437 Familial mediterranean fever (FMF), 539, 540 Familial short stature (FSS), 419–421 Familial thin basement membrane nephropathy, 813 Family and environmental issues cultural issues in medical care, 891, 892 death, 887, 888 discipline, 888 divorce, 888 foster care, 890 immigrants and internationally adopted children, 890–891 media, 889–890 school refusal, 889 separation anxiety disorder, 889 sibling rivalry and jealousy, 889 Family and societal violence, 895 Family chip system, 889 Fanconi anemia, 369, 377 clinical presentation, 357 complications, 358 genetics, 357 laboratory findings, 358 treatment, 358 Fats, 740, 741 Fat-soluble vitamins, 736 Fatty acid oxidation defects (FAOD) glutaric acidemia type II, 152 MCAD, 152 FDA Adverse Event Reporting System (FAERS), 930 Febrile neonate, 334 Female athlete triad, 520–521 Female breast masses cystosarcoma phyllode, 91 fibroadenoma, 91 indication for surgical intervention, 91 intraductal papilloma, 91 solitary cyst, 91 Female urethral prolapse, 845 Femoral anteversion, 486–487 Fetal alcohol syndrome, 77, 78 Fetal cardiac output, 658 Fetal distress, 37 Fetal macrosomia, 55

1152 Fever, 508 febrile neonate, 334 fever of unknown origin definition, 335 differential diagnosis, 335 laboratory test, 335 physical examination, 335 treatment, 336 in 1–3 month infants, 334 in 3–36 months, 334, 335 Fever of unknown origin (FUO), 335, 336 Fibroadenoma, 91 Fibromatosis colli, 995 Fifth disease, see Erythema infectiosum Fitz–Hugh–Curtis syndrome, 306 Flail chest, 258 Flat foot (pes planus), 490–491 Flattened nasal bridge, 46 Fluid compartment changes, 826 Fluid expansion as supportive therapy study (FEAST), 257 Fluids and electrolytes, 1119, 1120 body fluid composition, 825 fluid compartments, 825–826 hyperosmotic, 826 hypoosmotic, 826 isosmotic, 826 Fluorescent in situ hybridization (FISH) test, 109 Fluorescent treponemal antibody absorption (FTA-ABS) test, 321 Fluoride, 733 Fluoroquinolones, 933 Folate/folic acid, 736 Folic acid deficiency clinical presentation, 354 etiology congenital abnormalities, 354 decreased folic acid absorption, 354 drug-induced abnormal metabolism, 354 inadequate intake, 354 importance, 354 laboratory test, 354 treatment, 354 Folliculitis, 290, 291 Fontanelles, 44 Food allergy clinical presentation, 399, 400 diagnosis, 400 mechanism, 399 prognosis, 400 treatment, 400 Foodborne botulism clinical presentation, 302 incubation period, 302 treatment, 302 Foot disorders calcaneovalgus foot, 489–490 cavus foot, 490 clubfoot (talipes equinovarus), 489 flat foot (pes planus), 490–491 ingrowing toenail, 493 tarsal coalition, 491–492 tip-toe walking, 492 Foreign body, 770 Foreign body (FB) aspiration, 706, 955 lower airway, 231 management, 231 upper airway, 231 Foreign body ingestion esophagus, 231 management, 232

Index stomach and lower gastrointestinal tract, 232 symptoms, 231 Formula feeding, 744, 745 Foscarnet, 935 Foster care, 890 Four square model, 915–916 Fractures ankle fractures, 499–501 clavicular fracture, 494 compartment syndrome, 500 humerus fracture, 495 lateral condyle fracture, 496–497 medial epicondyle fracture, 497, 498 proximal humeral fracture, 494–495 radial head subluxation, 501–502 Salter–Harris Injuries, 493–494 scaphoid fracture, 497–499 supracondylar fracture of humerus, 495–496 tibial shaft fracture, 499 toddler fracture, 499, 500 Fragile X syndrome health supervision and preventive care, 128 major abnormalities, 128 minor anomalies, 128 Francisella tularensis clinical presentation, 318 diagnosis, 318 prevalence, 318 prevention, 318 treatment, 318 Frasier syndrome, 808 Friedreich ataxia, 576 Frontal sinus trauma, 626 Fructokinase deficiency, 155 Fructose 1,6-diphosphatase deficiency, 155 Full term birth, 35 Full thickness burn, 215 Functional abdominal pain, 757–759 Functional constipation abdominal radiograph, 774 anal fissure, 776 clinical presentation, 774 definition, 774 encopresis, 775, 776 Hirschsprung disease, 776 non-functional causes of, 775 rectal prolapse, 777 treatment, 775 and withholding, 774 Functioning ectopic gastric mucosa (Meckel’s diverticulum), 973 Fungal infections Aspergillus species causes, 327 clinical presentation, 327 diagnosis, 327 mode of transmission, 327 treatment, 327 Blastomyces, 328 Candida (see Candida species) coccidioidomycosis clinical presentation, 328 diagnosis, 328 mode of transmission, 328 treatment, 328 histoplasmosis clinical presentation, 328 mode of transmission, 327 treatment, 328

Index Malassezia furfur, 327 onychomycosis, 865 Sporothrix schenckii, 328 tinea capitis, 862, 863 tinea corporis, 863 tinea cruris, 864 tinea pedis, 864 tinea versicolor, 864 Furuncles, 290 G GABHS rapid antigen test, 626 Galactosemia clinical presentation, 153 complications, 153 diagnosis, 153 treatment, 153 Ganciclovir (IV), 935 Gastric lavage, 222 Gastroenteritis, 838 Gastroenterology, 1110–1115 Gastroesophageal reflux (GER), 698 Gastrointestinal bleeding esophageal varices, 786–787 lower bleeding, 787 Meckel diverticulum, 787–788 upper bleeding, 786 Gastrointestinal radiology acute appendicitis, 966 biliary atresia, 972 bowel perforation with pneumoperitoneum, 963 button battery ingestion, 974 Crohn’s disease with terminal ileitis, 976 duodenal atresia, 969 functioning ectopic gastric mucosa (Meckel’s diverticulum), 973 Hirschsprung disease, 970 hypertrophic pyloric stenosis, 965 ileocolic intussusception, 968 incarcerated inguinal hernia, 971 malrotation with volvulus, 967 meconium peritonitis, 964 neuroblastoma, 977 pneumatosis intestinalis, 962 portal venous gas, 962 small bowel obstruction, 975 Gastroschisis, 51 Gaucher disease, 157–158 Gender identity disorder (GID), 192 General pediatrics, 1016–1022 Generalized anxiety disorders, 180 Generalized cyanosis, 43 Genitalia ambiguous genitalia, 52–53 anus, 53 female infants, 52 males penis, 52 testis, 52 Genetic anticipation, 108 Genetic disorders, 1032–1036 autosomal dominant inheritance, 104–105 autosomal recessive inheritance, 105–106 chromosomal inheritance, 103, 104 genetic anticipation, 108 genomic imprinting, 108 major anomalies, 103 minor anomalies, 103 mitochondrial inheritance, 108

1153 multifactorial determination, 107 X-Linked Inheritance, 106–107 Genetic nonsyndromic hearing loss, 619–620 Genetic screening maternal blood screening, 112 noninvasive pregnancy testing, 112 preconception screening, 112 preimplantation genetic diagnosis, 112 Genetic syndromic hearing loss, 619 Genetic testing amniocentesis, 112 chorionic villus sampling, 112, 113 chromosome analysis/karyotyping, 109 clinical applications, 110–112 DNA testing, 109 FISH test, 109 gene panel sequencing, 110 genetic screening, 112 genome sequencing, 110 metabolic testing, 109 phenotypic testing, 109 postnatal genetic testing, 110 presymptomatic genetic testing, 110 single gene sequencing, 110 Genital herpes, 273 Genitourinary radiology acute right testicular torsion, 983 adrenal hemorrhage, 981 autosomal recessive polycystic kidney disease, 980 hematometrocolpos, 982 infected patent urachus, 978 left grade IV and right grade III vesicoureteral reflux, 979 posterior urethral valve, 978 rhabdomyosarcoma of the bladder, 983 right ovarian torsion, 982 tubo-ovarian abscess, 984 Wilms tumor, 981 Genodermatoses ectodermal dysplasia, 882–883 epidermolysis bullosa, 881–882 ichthyosis vulgaris, 883 incontinentia pigmenti, 882 Genomic imprinting, 108 Genu valgum (knock-knee), 485 Genu varum (Bowleg), 484–485 Germ cell tumors clinical presentation, 385 diagnosis, 385 laboratory test, 385 treatment, 385 types, 385 Gestational age (GA), 35, 53, 54 Gestational diabetes mellitus (GDM) definition, 42 management, 42 risk of, 42 Gianotti-Crosti syndrome, 793 Giardia lamblia, 329 Giardiasis clinical presentation, 329 diagnosis, 329 mode of transmission, 329 treatment, 329 Gifted child, 891–892 Gigantism, 136–138, 428 Gilbert syndrome, 60, 791, 792 Gingivostomatitis, 273 Gitelman syndrome, 815 Glanzmann thrombasthenia, 373

1154 Glasgow Coma Scale (GCS) score, 220 Glaucoma, 594 Global hypoxic ischemic injury, 1004 Glomerular hematuria, 800, 801 Glucose-6-phosphate dehydrogenase (G6PD) deficiency clinical presentation, 365 genetics, 365 incidence, 365 laboratory test, 366 pathophysiology, 365 treatment, 366 Glutaric aciduria type I/glutaric acidemia, 147–148 Gluten-sensitive enteropathy, 779, 780 Glycine encephalopathy, 150 Goldenhar syndrome/hemifacial microsomia, 138 Gonococcal conjunctivitis, 586 Gonococcal pharyngitis, 307 Gonococcal proctitis, 307 Gonococcal urethritis, 307 Good Samaritan laws, 903 Goodpasture syndrome, 812 Grade IV intraventricular hemorrhage, 997 Gram-negative bacteria Bartonella henselae clinical presentation, 313 diagnosis, 313 source, 313 surgical treatment, 314 treatment, 313, 314 Bordetella pertussis catarrhal phase, 311 convalescent phase, 311 diagnosis, 312 immunization, 312 incubation period, 311 paroxysmal phase, 311 pertussis complications, 311 prophylaxis, 312 thoracic pressure-related complications, 312 treatment, 312 Borrelia burgdorferi diagnosis, 320 early disseminated disease stage II, 320 early localized disease stage I, 320 incubation period, 320 late disseminated disease stage III, 320 treatment, 320 brucellosis causes, 312 diagnosis, 312 risks, 312 treatment, 313 Campylobacter species clinical presentation, 304 diagnosis, 305 epidemiology, 304 treatment, 305 chlamydia genitourinary tract infection, 306 Chlamydia pneumoniae clinical presentation, 305 diagnosis, 305 epidemiology, 305 treatment, 305 Chlamydia trachomatis conjunctivitis due to, 305 pneumonia due to, 306 Chlamydophila psittaci, 305 Citrobacter, 314

Index clinical manifestations, 304 E. coli O157 clinical presentation, 317 incidence, 317 treatment, 317 urinary tract infections, 317 ehrlichiosis clinical presentation, 319 epidemiology, 319 laboratory findings, 319 treatment, 319 Escherichia coli acute bacterial meningitis, 316 causes, 316 enteric infections, 317 intra-abdominal infections, 317 pneumonia, 317 Francisella tularensis clinical presentation, 318 diagnosis, 318 prevalence, 318 prevention, 318 treatment, 318 Haemophilus influenzae bacterial meningitis, 309 buccal infections, 310 causes, 309 epiglottitis, 309, 310 occult bacteremia, 310 periorbital cellulitis, 310 pneumonia, 310 pyogenic arthritis, 310 rifampin antibiotic prophylaxis, 310 treatment, 310 Klebsiella, 314 mycobacterium avium-intracellulare, 325 Mycoplasma pneumonia clinical presentation, 310 incubation period, 310 testing for mycoplasma, 311 treatment, 311 N. meningitidis diagnosis, 308 prevention and indications, 309 symptoms, 308 transmission rate, 308 treatment, 309 Neisseria gonorrhoeae disseminated gonococcal infection, 307 epididymitis (gonococcus), 307 gonococcal pharyngitis, 307 gonococcal proctitis, 307 gonococcal urethritis, 307 incubation period, 307 neonatal conjunctivitis, 307 screening, 308 nontyphoidal salmonellosis antibiotic therapy, 315 clinical presentation, 315 diagnosis, 315 incubation period, 314 mode of transmission, 315 Pasteurella multocida clinical presentation, 311 risk, 311 treatment, 311 Pseudomonas species antimicrobial therapy, 314 causes, 314

Index clinical presentation, 314 risk factors, 314 RMSF (see Rocky mountain spotted fever (RMSF)) Shigella child care center, 316 clinical presentation, 316 complications, 316 diagnosis, 316 incubation period, 316 mode of transmission, 316 treatment, 316 TB (see Mycobacterium tuberculosis) trachoma, 306, 307 Treponema pallidum, 320, 321 typhoid fever clinical presentation, 315 diagnosis, 315 mode of transmission, 315 treatment, 315 Yersinia enterocolitica, 318 Gram-positive bacteria Arcanobacterium haemolyticum, 301, 302 Bacillus anthracis, 301 Bacillus cereus clinical presentation, 301 diagnosis, 301 GI tract, 301 treatment, 301 Clostridium botulinum causes, 302 clinical presentation, 302 diagnosis, 302 infant botulism, 302 treatment, 302 Clostridium difficile clinical presentation, 303 diagnosis, 304 prevention, 304 risk factor, 303 treatment, 304 Clostridium perfringens, 302, 303 Clostridium tetani common sources, 303 generalized tetanus, 303 neonatal tetanus, 303 prevention, 303 treatment, 303 Corynebacterium diphtheriae, 300 enterococcal infections, 301 epidemiology, 289, 290 erysipelas GAS, 298 folliculitis/furunculosis/carbunculosis, 290 antibiotics, 290 oral anti-staphylococcal antibiotics, 290 recurrent staphylococcal skin infections, 291 foodborne botulism clinical presentation, 302 incubation period, 302 treatment, 302 GAS pharyngitis clinical presentation, 295 diagnosis, 296 epidemiology, 295 treatment, 296 impetigo clinical presentation, 297 etiology, 297 treatment, 298 Listeria monocytogenes, 300

1155 methicillin-resistant S. aureus, 293 methicillin-sensitive S. aureus, 293 necrotizing fasciitis aerobic and anaerobic flora, 299 clinical presentation, 300 diagnosis, 300 laboratory findings, 300 treatment, 300 PANDAS, 299 perianal streptococcal dermatitis clinical presentation, 298 diagnosis, 298 incidence, 298 treatment, 298 S. aureus food poisoning clinical presentation, 292 incubation period, 292 treatment, 292 S. pneumoniae clinical manifestations, 294 diagnosis, 294 epidemiology, 294 risks, 294 treatment, 294, 295 scarlet fever causes, 296 clinical presentation, 296, 297 diagnosis, 297 streptococcosis, 297 treatment, 297 SSSS clinical presentation, 292 diagnosis, 292 treatment, 292 staphylococcal infections, 290 staphylococcal, coagulase-negative, 293 Staphylococci in blood culture, 290 streptococcal toxic shock syndrome, 299 clinical presentation, 299 diagnosis, 299 risk factors, 299 treatment, 299 TSS clinical presentation, 291 production, 291 risk factors, 291 treatment, 291, 292 Granuloma annulare (GA), 879–880 Granulomatosis infantisepticum, 300 Granulomatosis with polyangiitis (GPA), 543, 544 Graves disease, 431, 432 Grisel syndrome, 472 Group A beta-hemolytic Streptococcus (GAS) pharyngitis clinical presentation, 295 diagnosis, 296 epidemiology, 295 treatment, 296 Group B streptococcal (GBS) infection clinical presentation, 69 diagnosis, 69 early GBS, 68 intrapartum prophylaxis, 69 late GBS, 68 prevention guidelines, 69, 70 screen timing, 69 secondary prevention, 69 treatment, 69 Growing pains, 541, 542

1156 Growth abnormal growth diagnosis, 1 genetic growth, 1 growth charts, 1 growth curve reading, 2 head circumference, 2 height, 1 length or supine height measurement, 1 weight, 1 Growth disorder constitutional delay of growth and puberty, 419, 420 familial short stature, 419–421 idiopathic short stature, 420 normal growth rate, 417, 418 psychosocial dwarfism, 421 short stature bone age radiograph, 419 history, 418 laboratory evaluation, 419 normal variant, 417 pathological, 417 physical examination, 418 Guillain–Barré syndrome (GBS), 337, 546, 572, 573 Gynecomastia, 94, 447 H Habit disorder general management, 184 hair-pulling disorder, 183 nail biting, 184 teeth grinding, 183 thumb sucking, 183, 184 trichotillomania, 183 Haemophilus influenzae bacterial meningitis, 309 buccal infections, 310 causes, 309 epiglottitis, 309 occult bacteremia, 310 periorbital cellulitis, 310 pneumonia, 310 pyogenic arthritis, 310 rifampin antibiotic prophylaxis, 310 treatment, 310 Haemophilus Influenzae Type B (Hib) conjugate, 17 Hair disorders alopecia areata, 880–881 anagen effluvium, 881 telogen effluvium, 881 traction alopecia, 881 trichotillomania, 881 Hair-pulling disorder, 183 Hall–Pallister syndrome, 423 Hand, foot, and mouth disease, 282, 283 Handicapping conditions, 893, 894 Harlequin ichthyosis, 44 Hashimoto thyroiditis, 430 Hawthorne effect, 913 Head caput succedaneum, 45 cephalohematoma, 45 fontanelles, 44 head shape, 44 skull fractures, 45 subgaleal hemorrhage, 45 traumatic epidural, subdural and subarachnoid hemorrhage, 45 Head growth disorders

Index craniosynostosis, 567 hydrocephalus, 566, 567 macrocephaly, 566 microcephaly, 566 Head trauma basilar skull fracture, 207 CT, 208 epidural hematoma, 208 management, 208–209 mechanisms of injury, 206 patients at higher risk, 207 retinal hemorrhage, 208 subarachnoid bleed, 208 subdural hematoma, 207–208 temporal skull fracture, 207 Headache chronic nonprogressive headache, 562 chronic progressive headache, 562 epidemiology, 561 idiopathic intracranial hypertension, 563, 564 migraine headache, 561 post-traumatic/post-concussion headaches, 563 primary headache syndromes diagnosis, 562 treatment of, 562, 563 red flags, 562 tension headache, 561 types of, 561 Hearing aids, 622 Hearing loss audiogram, 621 bone-anchored hearing aid, 622 cochlear implants, 622 congenital hearing loss, 618–619 genetic nonsyndromic hearing loss, 619–620 genetic syndromic hearing loss, 619 hearing aids, 622 hereditary hearing loss, 622 middle ear effusion, 621 ototoxicity, 621 pediatric audiometric testing, 620–621 sound amplification devices, 622 tympanic membrane perforation, 621 types, 618 Hearing screening, 25 Heart blood pressure, 50 bradycardia, 50 murmur, 50 point of maximal cardiac impulse, 49 tachycardia, 50 failure, 753, 754 causes, 682, 683 clinical presentation, 683 diagnostic evaluation, 683 management, 683, 684 murmur, 509 Heat illness, 511 Helicobacter pylori, 395 Helminthic organism Ascaris lumbricoides clinical presentation, 332 diagnosis, 332 mode of transmission, 332 treatment, 332 cestodes (platyhelminthes), 333 Enterobius vermicularis clinical presentation, 332

Index diagnosis, 332 mode of transmission, 331 treatment, 332 Necator americanus clinical presentation, 332 mode of transmission, 332 treatment, 332 Strongyloides stercoralis, 333 toxocariasis, 333 trematodes (platyhelminthes), 333 Trichinella spiralis, 333 trichuriasis, 332 Hemangioma, 606–607 Hematology/oncology, 1060–1066 Hematoma, 612–613 Hematometrocolpos, 982 Hematuria, 800 Hemihypertrophy, 53 Hemolytic anemia AIHA (see Autoimmune hemolytic anemia (AIHA)) G6PD deficiency (see Glucose-6-phosphate dehydrogenase (G6PD) deficiency) hereditary elliptocytosis, 360 hereditary spherocytosis (see Hereditary spherocytosis) PK deficiency (see Pyruvate kinase deficiency) PNH (see Paroxysmal nocturnal hemoglobinuria (PNH)) SCD (see Sickle cell disease) Hemolytic uremic syndrome (HUS), 372, 800, 821, 822 Hemophagocytic lymphohistiocytosis (HLH), 276 Hemoptysis, 701, 702 Hemorrhagic conjunctivitis, 587 Hemorrhagic disease classic stage, 62 clinical presentation, 62 diagnosis, 62 early stage, 62 late stage, 62 of newborn, 373 prevention, 62 treatment, 62 Hemothorax, 258 Henoch-Schönlein purpura (HSP), 545–547 Hepatic dysfunction, 249 Hepatic metabolism, 927–928 Hepatitis A (HepA) vaccine, 20–21 Hepatitis A virus (HAV), 792, 793 Hepatitis B prophylaxis, 39 Hepatitis B Vaccine (HepB) vaccination, 15–16 Hepatitis B virus (HBV), 793–795 Hepatitis C virus (HCV), 795–796 Hepatoblastoma associated syndromes/risk factors, 385 clinical presentation, 385 diagnosis, 385 incidence, 384 treatment, 385 Hepatomegaly, 796, 797 Hepatorenal tyrosinemia, 150 Hereditary angioedema (HAE) clinical presentation, 397 diagnosis, 397 treatment, 397 types, 396 Hereditary elliptocytosis, 360 Hereditary hearing loss, 622 Hereditary motor sensory neuropathy (HMSN), 573 Hereditary spherocytosis clinical presentation, 359

1157 etiology, 359 laboratory test, 360 treatment, 360 Herpes gladiatorum, 273, 274 Herpes simplex virus (HSV) conjunctivitis, 588–589 Herpes zoster (shingles), 275 Herpetic whitlow, 273 Heterophile antibody test, 272 High-efficiency particulate air (HEPA) filter, 268 Hip disorders developmental dysplasia of the hip, 476–478 Legg–Calve–Perthes Disease, 478–479 slipped capital femoral epiphysis, 479, 480 Hirschsprung disease (HD), 776, 970 Histoplasmosis, 327, 328 History and physical examination, 247, 248 HIV infection, 509 HIV screening, 26–27 Hodgkin lymphoma clinical presentation, 380 diagnosis, 380 incidence, 379 prognosis, 380 treatment, 380 Holt-Oram syndrome, 662, 674 Home-prepared baby foods, 746 Homocystinuria clinical presentation, 149 diagnosis, 149 and Marfan syndrome, 149 prognosis, 149 treatment, 149 Homosexuality, 192 Hookworm, 332 Hordeolum (Stye), 591–592 Horner syndrome, 46 Hot tub folliculitis, 290 Human bites, 212 Human herpesvirus type 6 (HHV-6) clinical presentation, 276 treatment, 276 Human herpesvirus-7 (HHV-7), 276 Human herpesvirus-7 (HHV-8), 276 Human immunodeficiency virus (HIV) clinical presentation, 284, 285 diagnosis adult and adolescent infection, 285 children, 285 perinatal and postnatal infection, 285 incubation period, 284 mode of transmission, 284 prevention, 285, 286 Human metapneumovirus clinical presentation, 280 epidemiology, 280 treatment, 280 Human papillomavirus (HPV) clinical features, 289 epidemiology, 289 immunization, 289 vaccines, 22, 23 Humerus fracture, 495 Hunter syndrome, 156 Hurler syndrome, 156 Hydrocarbon ingestion, 230 Hydroceles, 850 Hydrocephalus, 2, 566, 567 Hyperaldosteronism, 449

1158 Hyperammonemia, 144 Hyperbilirubinemia, 56, 790 ABO and Rh incompatibility, 61 chronic bilirubin encephalopathy, 61 conjugated hyperbilirubinemia, 60 early onset breastfeeding jaundice, 58–59 kernicterus, 60–61 late onset breast milk jaundice, 59 management exchange transfusion, 62 feeding, 61 intravenous immunoglobulin, 62 phototherapy, 61, 62 pathophysiology, 57 physiologic jaundice causes, 58 clinical presentation, 58 diagnosis, 57 risk factors, 58 TSB concentrations, 57, 61 unconjugated hyperbilirubinemia, 60 Hyper-IgE syndrome (HIES) clinical presentation, 411 diagnosis, 411 DOCK8, 411 treatment, 411 Hyperkalemia, 834, 835 Hypernatremia, 827–829 Hyperosmolar nonketotic coma, 838–839 Hyperosmolar therapy, 251 Hyperparathyroidism, 437, 438 Hyperpituitarism acromegaly, 428 gigantism, 428 prolactinoma, 428 Hypersensitivity reactions cutaneous lupus erythematosus, 871–872 erythema multiforme, 872 Stevens–Johnson syndrome, 870, 872–873 toxic epidermal necrolysis, 872–873 urticaria, 871 Hypertension, 802–804 Hypertensive crisis BP measurement, 238 emergency care, 239, 240 hypertensive emergency, 238 hypertensive urgency, 238 primary hypertension, 238 secondary causes, 238, 239 secondary hypertension, 238 Hypertensive urgency, 238 Hyperthermia, 1026 Hypertrophic cardiomyopathy (HCM), 651, 685 Hypertrophic pulmonary osteodystrophy, 701 Hypertrophic pyloric stenosis, 965 Hypertropia, 603 Hyperventilation, 251 Hyphema, 603 Hypocalcemia causes, 434 clinical presentation, 435 definition, 434 diagnosis, 435 treatment, 435, 436 Hypochloremia, 835 Hypochromic/microcytic anemia, 349, 350 Hypoglycemia, management of, 56–57 Hypokalemia, 835

Index Hyponatremia, 829–831 Hyponatremic dehydration, 832 Hypoparathyroidism causes, 436 clinical presentation, 436 diagnosis, 436 treatment, 436 Hypophosphatemic rickets, 441 Hypoplastic left heart syndrome (HLHS), 674 Hypospadias, 52, 846 Hypothermia, 251 Hypotropia, 603 Hypovolemic shock, 254–255 Hypoxanthine-guanine phosphoribosyltransferase deficiency, 163 Hypoxic-ischemic encephalopathy (HIE) clinical and laboratory evidence, 75 clinical presentation, 75, 76 diagnosis, 75 imaging studies, 76 laboratory studies, 76 management, 76 pathogenesis, 75 I Ibuprofen ingestion, 225 Ichthyosis vulgaris, 883 Idiopathic hypercalciuria, 801, 802 Idiopathic intracranial hypertension, 563, 564 Idiopathic short stature (ISS), 420 IgA nephropathy, 809, 810 Ileocolic intussusception, 968 Imerslund-Gräsbeck syndrome, 355 Immersion burns, 896 Immigrants, 890–891 Immune system adaptive immune system, 404 B-cells, 404, 405 innate immune system, 404 T-lymphocytes, 404 Immune thrombocytopenic purpura (ITP), 372 Immunization common adverse reaction, 22 contraindications, 23 DtaP vaccine, 16 Hib conjugate, 17 HepA vaccine, 20–21 HepB, 15–16 HPV vaccines, 22, 23 IPV, 18 influenza vaccines, 19 meningococcal conjugate vaccines, 21–22 MMR vaccine, 19–20 pneumococcal vaccines, 17–18 refusal by parents, 15 risks and benefits, 15 rotavirus vaccine, 16 schedule, 15 Tdap vaccine, 16, 17 VAR vaccine, 20 VariZIG, 20, 21 Imperforate hymen, 93 Imperiled newborn infants, 908–909 Impetigo, 297, 874–875 clinical presentation, 297 etiology, 297 treatment, 298 Inactivated poliovirus vaccine (IPV), 18

Index Incarcerated inguinal hernia, 971 Incontinentia pigmenti, 882 Individualized Education Plans (IEPs), 894 Infantile colic, 32 Infantile hemangiomas (IH), 868–869 Infantile hypertrophic pyloric stenosis (HPS), 773 Infantile nephrotic syndrome, 807, 808 Infantile nystagmus syndrome, 606 Infantile spasms, 552 Infants of diabetic mother (IDM) complications cardiovascular anomalies, 56 congenital malformations, 56 fetal macrosomia, 55 hyperbilirubinemia, 56 hypocalcemia/hypomagnesemia, 56 impaired fetal growth, 55 iron deficiency, 56 metabolic and electrolyte abnormalities, 56 polycythemia, 56 pulmonary disease, 56 thrombocytopenia, 56 congenital malformations, 55 fetal hyperglycemia, 55 fetal hyperinsulinemia, 55 preconceptional glycemic control, 55 Infected patent urachus, 978 Infectious diseases, 1052–1060 bacterial pathogens (see Gram-negative bacteria) (see Grampositive bacteria) CNS (see Central nervous system (CNS) infections) fever (see Fever) fungal infection (see Fungal infections) helminthic organism (see Helminthic organism) prevention (see Prevention of infectious diseases) protozoa infection (see Protozoa) viral infections (see Viral infections) Infectious mononucleosis, 635 Infective endocarditis (IE), 678, 679 Infestations bedbugs, 866 papular urticaria, 866 pediculosis, 865 scabies, 865 Inflammatory bowel disease (IBD), 532, 777–779 Inflammatory myopathies JDM, 537, 538 polymyositis, 538 Influenza vaccines, 19 Influenza virus AAP immunization guidelines, 279 clinical presentation, 278 complications, 278, 279 diagnosis, 279 epidemiology, 278 treatment, 279 Ingrowing toenail, 493 Inguinal hernia, 851 Inherited bleeding disorders hemophilia A and B, 374 Von Willebrand disease, 374 Inpatient pneumonia, 295 Insect sting allergy anaphylactic reactions, 401 diagnosis, 402 large local reactions, 401–402 systemic cutaneous reactions, 401 treatment, 402

1159 Intellectual disability (ID) causes, 167 classification, 167 clinical presentation, 167 differential diagnosis, 168 management, 168 prevalence, 167 Intention-to-treat analysis, 919 Intermittent exotropia, 604 Internal tibial torsion, 487–488 International Diabetes Federation Criteria, 462 Internationally adopted children, 890–891 Interstitial edema, 252 Intertrigo, 875–876 Intestinal polyposis syndromes, 788–789 Intoeing femoral anteversion, 486–487 internal tibial torsion, 487–488 metatarsus adductus, 488 Intracranial pressure (ICP), 563 cerebral edema, 251, 252 clinical presentation, 249 dexamethasone, 251 hyperosmolar therapy, 251 hyperventilation, 251 hypothermia, 251 management of, 250 measures for intubated patients, 250 symptoms, 249 Intraductal papilloma, 91 Intrauterine growth retardation (IUGR) asymmetrical IUGR, 54 definition, 54 diagnosis, 55 fetal factors, 54 maternal factors, 54 placental factors, 54 symmetrical IUGR, 54 Intravenous rehydration, 828, 831 Intraventricular hemorrhage (IVH) classification, 76 clinical presentation, 76 complication, 77 diagnosis, 76 prevention, 77 prognosis, 77 risk factors, 76 Intussusception, 763 Iodine, 733 Ipecac, 222 Iron, 733, 734 Iron deficiency anemia screening, 26 Iron ingestion, 229 Isolated (benign) premature thelarche, 442 Isolated beta-methylcrotonyl-CoA carboxylase deficiency, 147 Isolated sagittal craniosynostosis, 1013 Isonatremic dehydration, 834 Isovaleric acidemia, 145 J Janeway lesion, 678 Jaundice, 44 Jealousy, 889 Jejunal atresia, 774 Jervell Lange–Nielsen syndrome, 619 Jeune syndrome, 782

1160 Johanson-Blizzard syndrome, 781 Juvenile dermatomyositis (JDM), 537, 538 Juvenile idiopathic arthritis (JIA), 526, 590 complications of, 530 definitions, 525 ERA, 527 imaging, 530 laboratory abnormalities, 529, 530 MAS, 529 oligoarticular, 526, 527 polyarticular, 527 psoriatic, 528 systemic-onset, 528 treatment of, 530, 531 uveitis, 529 Juvenile myoclonic epilepsy, 554 Juvenile pilocytic astrocytoma (JPA), 383, 1006 Juvenile polyposis syndrome (JPS), 788 Juvenile systemic lupus erythematosus (jSLE), 533 APS, 534 cardiac manifestations, 534 clinical presentation, 532 cutaneous manifestations, 532, 533 endocrine manifestations, 534 gastrointestinal manifestations, 534 general manifestations, 532 hematologic involvement, 534 laboratory evaluation, 534, 535 management, 535 musculoskeletal manifestations, 533 neuropsychiatric manifestation, 533 pulmonary manifestations, 534 renal manifestations, 533 K Kaposi sarcoma, 276 Kasabach-Merritt phenomenon, 869 Kawasaki disease (KD), 643, 675–678 Keratosis pilaris, 859 Kernicterus BIND, 60 causes, 60 clinical features, 60 phase 1, 60 phase 2, 61 phase 3, 61 Kernig and Brudzinski signs, 338 Kidney stones, 851, 852 Kikuchi, 636 Kinky hair disease, 164 Klebsiella, 314 Kleihauer–Betke (KB) test, 345 Klinefelter syndrome, 119, 447 health supervision and preventive care, 119 neurobehavioral abnormalities, 118 physical and medical abnormalities, 118, 119 routine karyotype or microarray analysis, 118 Klippel–Feil syndrome, 472 Klippel–Trenaunay syndrome, 782 Klumpke palsy, 48 Knee pain and injuries anterior cruciate ligament injury, 482 medial collateral ligament injury, 482 meniscal injury of the knee, 480–482 osteochondritis dissecans, 483 recurrent patellar dislocation/subluxation, 483, 484 Krabbe disease, 158

Index L Labyrinthitis, 616–617 Laceration anesthetic and anxiolytic options, 211 animal and human bites antibiotic therapy, 212, 213 bat bites, 212 black widow spider bite, 214 brown recluse spider bite, 214–215 cat bites, 212 dog bites, 212 general evaluation, 212 imaging studies, 213 laboratory, 213 management, 212 rabies prophylaxis indications, 213 scorpion stings, 215 snake bites (see Snake bites) tetanus vaccine, 212 types of human bite injuries, 212 wound care, 213 common locations, 210 definition, 210 ear lacerations, 211, 212 lip lacerations, 211 nail bed lacerations, 211 prophylactic antibiotics, 211 suture and staples, 211 suture removal timing, 212 wound aftercare, 211 wound preparation, 211 Lack of systemic arterial supply, 950 Landau–Kleffner syndrome, 554, 555 Langerhans cell histiocytosis (LCH), 883–884 clinical presentation, 381 diagnosis, 381 differential diagnosis, 381 incidence, 381 prognosis, 382 treatment, 381 Language disorders, 169 Large fontanelles, 44 Large for gestational age (LGA), 35 Laryngeal web, 696 Laryngomalacia, 640–641, 695 Laryngotracheoesophageal cleft, 697 Late onset breast milk jaundice, 59 Late term birth, 35 Latent tuberculosis infection (LTBI), 321, 323–325 Lateral condyle fracture, 496–497 Laurence–Moon–Bardet–Biedl syndrome, 818, 819 Lead poisoning, 353 Lead screening, 25–26 Learning disorders aptitude-achievement discrepancy, 170 associated conditions, 170 ADHD (see Attention-deficit/hyperactivity disorders (ADHD)) earlier signs, 170 etiology, 170 management, 170, 171 mathematics (dyscalculia), 170 poor school performance, 171 reading disorders, 170 screening, 170 written expression (dysgraphia), 170 Left grade IV and right grade III vesicoureteral reflux, 979 Left parietal calcified cephalohematoma, 1009 Legg–Calve–Perthes disease (LCPD), 478–480

Index Leg-length discrepancy, 466 Lennox–Gastaut syndrome, 554, 555 Leri-Weill dyschondrosteosis short stature syndrome, 120 Lesch–Nyhan disease, 163 Leukocyte adhesion defect clinical presentation, 407 diagnosis, 407 treatment, 407 Leukocytosis, 367, 375, 376, 388 Lichen nitidus, 867 Lichen sclerosus, 867–868 Lichenoid disorders, 867–868 Li–Fraumeni, 384 Likelihood Ratio (LR), 916–917 Limping child, 465, 466 Lip lacerations, 211 Lipidoses, 160 Fabry disease, 159 Gaucher disease, 157–158 Krabbe disease, 158 metachromatic leukodystrophy, 159–160 Niemann–Pick disease, 158 Tay–Sachs disease, 158–159 Wolman syndrome, 159 Lissencephaly, 565 Listeria monocytogenes, 300 Little Leaguer’s elbow, 516 Little Leaguer’s shoulder, 516 Live birth, 35 Liver, 50 AIH, 796 Alagille syndrome, 792 biliary atresia, 790–791 Gilbert syndrome, 791, 792 HAV, 792, 793 HBV, 793–795 HCV, 795, 796 hepatomegaly, 796, 797 jaundice, 789 neonatal cholestasis, 790 portal hypertension, 797 Wilson disease, 791 Lobar pneumonia, 957 Localized scleroderma, 536, 537 Lonestar tick (Amblyomma americanum), 319 Long gap esophageal atresia with tracheoesophageal fistula, 952 Low birth weight (LBW), 35 Lower airway infection, 199 Lower airway obstruction, 199 Lower motor neuron facial nerve palsy, 574 Lower motor neuron lesions (LMNL), 571 Lung cough, 49 respiratory distress, 49 unilateral movement of chest, 49 Lupus nephritis (LN), 812 Lyme disease, 319, 320 Lymphadenitis, 636 Lymphocytic thyroiditis, 430 Lymphogranuloma venereum (LGV), 306 Lymphoma, 636–637, 961 Lysosomal storage disorders lipidoses, 157–160 MPS type I, 156 type II, 156 type III, 156–157 type IV, 157

1161 M Macrocephaly, 137, 566 benign familial macrocephaly, 2 causes, 2 definition, 2 hydrocephalus, 2 Macrocytic anemia diamond–blackfan anemia clinical findings, 356, 357 clinical presentation, 356 etiology, 356 laboratory test, 357 prognosis, 357 treatment, 357 fanconi anemia clinical findings, 356 clinical presentation, 357 complications, 358 genetics, 357 laboratory findings, 357 treatment, 358 folic acid deficiency clinical presentation, 354 etiology, 354 importance, 354 laboratory test, 354 treatment, 354 Pearson marrow–pancreas syndrome, 356 Shwachman-Diamond syndrome clinical presentation, 358 complications, 358 diagnosis, 358 genetics, 358 laboratory findings, 358 treatment, 358 vitamin B12 deficiency clinical presentation, 355 etiology, 355 laboratory test, 355, 356 sources, 354 treatment, 356 Macroglossia, 47, 697 Macrolides, 932–933 Macronutrients calories/energy, 740 carbohydrates, 741 fats, 740, 741 protein, 741 Macrophage-activation syndrome (MAS), 528, 529 Magnesium, 734 Malaria clinical presentation, 331 complications, 331 diagnosis, 331 prevention, 331 treatment, 331 Malassezia furfur, 327 Malformed ears, 46 Malignant hyperthermia (MH), 248 Malnutrition allergies, 755 burns, 754 chronic liver disease, 753 cystic fibrosis, 753 family and cultural practices, 755 heart failure, 753, 754 malignancies, 754 neurological impairment, 755 renal disease, 754 viral gastroenteritis, 753

1162 Malrotation, 762 Malrotation with volvulus, 967 Maple syrup urine disease (MSUD), 146 Marfan syndrome, 133, 149, 674 abnormalities, 132, 134 clinical diagnosis, 132 health supervision and preventive care, 134 prevalence, 132 Marrow suppression, 372 Mastocytosis, 880 clinical presentation, 396 cutaneous, 395 diagnosis, 396 prognosis, 396 systemic, 395 treatment, 396 Masturbation, 191–192 Maternal blood screening, 112 Mature breast milk, 742–744 Maturity-onset diabetes of youth (MODY), 461 McCune–Albright syndrome, 445, 446 McMurray test, 481 Measles virus clinical presentation, 286 control and prevention, 286 diagnosis, 286 mode of transmission, 286 Measles, mumps, and rubella (MMR) vaccine, 19–20 Meckel diverticulum, 787, 788 Meconium aspiration syndrome (MAS), 949 clinical presentation, 65 diagnosis, 66 etiology, 65 management, 66 prevention, 66 risk factors, 65 Meconium ileus cause, 72 clinical presentation, 72 diagnosis, 72 management, 73 Meconium peritonitis, 964 Meconium plug syndrome clinical presentation, 72 common associated conditions, 72 etiology, 72 management, 72 Media, 889–890 Medial collateral ligament injury (MCL), 482 Medial epicondyle fracture, 497, 498 Medical error, 921 Medical neglect, 898 Medical testimony, 911 Medium-Chain Acyl-CoA dehydrogenase deficiency (MCAD), 152 Medulloblastoma, 383, 384 Melanoma, 879 Membranoproliferative glomerulonephritis (MPGN), 811 Membranous nephropathy (MN), 806, 807 Menactra, 21, 22 Mendellian syndromes achondroplasia, 131–132 Beckwith–Wiedemann Syndrome, 136–137 connective tissue dysplasias (see Connective tissue dysplasias) craniosynostosis syndromes, 130–131 fragile X syndrome, 127–128 Noonan syndrome, 128–129 Sotos syndrome, 137–138 Treacher Collins syndrome, 129–130 Waardenburg syndrome, 129 Ménétrier disease, 751

Index Meniere’s disease, 618 Meningitis child care, 340 clinical manifestations, 338 corticosteroids in bacterial meningitis, 340 diagnosis, 338, 339 drugs, 339 N. meningitidis, 338 neonatal gram-negative meningitis, 337 neonatal herpes simplex infection, 337 neonatal meningitis due to listeria, 337 neonatal streptococcal meningitis, 337 physical examination, 338 prognosis, 340 S. pneumoniae, 338 treatment, 339 Meningocele, 569 Meningococcal conjugate vaccines (MCV), 21–22, 270 administration, 22 catch-up vaccination, 21 Menactra, 21, 22 Menveo, 21, 22 routine vaccination, 21 Serogroup B meningococcal vaccines, 22 Meningococcal infections, see Neisseria meningitidis Meniscal injury of the knee, 480–482 Menkes disease, 164 Mental and behavioral health, 1039–1044 Menveo, 21, 22 Metabolic acidosis, 836–837 Metabolic alkalosis, 837, 838 Metabolic disorders, 1036–1039 amino acid metabolism disorder, 148–151 body odors, 164–165 carbohydrate metabolism disorder (see Carbohydrate metabolism disorder) classifications, 143–145 familial hypercholesterolemia, 163 FAOD glutaric acidemia type II, 152 MCAD, 152 lysosomal storage disorders (see Lysosomal storage disorders) Menkes disease, 164 newborn, clinical approach general rules, 144–145 hyperammonemia, 144 initial laboratory evaluation, 143 management, 144 pyridoxine (B6), 144 secondary tests, 144 newborn metabolic screening, 165 organic acidemia (see Organic acidemia) peroxisomal disorders, 160–161 porphyrias, 161–162 pyrimidine metabolism, 163 Smith–Lemli–Opitz syndrome, 163–164 urea cycle disorders, 151 Metachromatic leukodystrophy, 159–160 Metatarsus adductus, 488 Methanol ingestion, 229 Methemoglobinemia, 353, 701 Methicillin-resistant Staphylococcus aureus (MRSA), 293 Methicillin-sensitive S. aureus (MSSA), 293 Methylmalonic acidemia, 146 Microcephaly, 2, 566 Microcytic anemia chronic disease, 350 etiology, 350 laboratory test, 351 treatment, 351

Index iron deficiency anemia blood transfusion, 350 clinical presentation, 348, 349 dietary change, 349 etiology, 348 laboratory test, 349 oral iron therapy, 350 parenteral iron therapy, 350 lead poisoning, 353 methemoglobinemia, 353 sideroblastic anemia, 353 thalassemias (see Alpha Thalassemia) Microdeletion, chromosome disorders Alagille syndrome, 124–125 Angelman syndrome, 126–127 DiGeorge/Velocardiofacial syndrome, 125–126 Prader–Willi Syndrome (PWS), 126 WAGR syndrome, 124 Williams syndrome, 123–124 22q11.2 Microdeletion syndrome, see DiGeorge syndrome Micropenis, 848 Microscopic polyangiitis (MPA), 544 Middle ear effusion, 621 Migraine headache, 561 Mild traumatic brain injury, 513–514 Miliaria, 855–856 Minerals calcium, 732 chloride, 732 chromium, 732 copper, 732, 733 fluoride, 733 iodine, 733 iron, 733, 734 magnesium, 734 potassium, 734 selenium, 734 sodium, 734 zinc, 734, 735 Miserable malalignment syndrome, 519 Mispositioned meatus, 52 Mixed connective tissue disease (MCTD), 537 Mixed hearing loss, 618, 619 Molluscum contagiosum, 510, 873–874 Molluscum contagiosum conjunctivitis, 588 Monobactam, 932 Monozygotic twins, 55 Morquio syndrome, 157 Mouth and oral cavity ankyloglossia/tongue-tie, 47, 631 aphthous ulcers, 630 bifid uvula, 630–631 Bohn nodules, 47 cleft lip and palate, 47, 632–633 cold panniculitis, 631 dental trauma and avulsions, 634 early childhood caries, 633–634 Epstein pearls, 46, 47 high arched palate, 47 macroglossia, 47 mucocele, 631 natal teeth, 47 odontogenic infection, 633 parotitis, 631–632 Pierre Robin sequence, 47 Ranula, 47 Robin sequence, 633 Movement disorders acute cerebellar ataxia, 575 ataxia telangiectasia, 575, 576

1163 dystonic reactions, 578 Friedreich ataxia, 576 Sydenham chorea, 578 tics, 577 Tourette syndrome, 577, 578 Mucocele, 631 Mucopolysaccharidosis (MPS) type I, 156 type II, 156 type III, 156–157 type IV, 157 Multicystic dysplastic kidney disease, 819 Multiple births complications, 55 definition, 55 dizygotic twins, 55 monozygotic twins, 55 Multiple sclerosis, 1009 Mumps clinical presentation, 286 complications, 286 diagnosis, 286 mode of transmission, 286 treatment, 286 Munchausen syndrome by proxy, 900 Murmurs, 50, 660, 661 Musculoskeletal radiology achondroplasia, 994 acute osteomyelitis, 988 aneurysmal bone cyst, 993 apophyseal avulsion fracture, 996 Ewing sarcoma, 990 fibromatosis colli, 995 medial and lateral epicondyles, 996 non-accidental trauma with classic metaphyseal lesions and skull fracture, 986 osteochondroma, 992 osteogenesis imperfecta, 987 osteosarcoma, 991 rickets, 989 Salter Harris II fracture, 985 supracondylar fracture, 995 Mushrooms ingestion, 229–230 Myasthenia gravis, 571 Mycobacterium avium-intracellulare causes, 325 clinical presentation, 325 treatment, 325 Mycobacterium tuberculosis, 268 antituberculous medications side effects, 324 BCG vaccine, 323 challenges, 324, 325 chest radiographs, 323, 324 CNS disease, 322 etiology, 321 false-negative TST result, 323 false-positive TST result, 323 follow-up, 325 LTBI, 324 miliary tuberculosis, 322 pleural TB, 322 pulmonary disease, 321 risk factors, 321 skeletal tuberculosis, 322 superficial lymphadenopathy, 322 TB exposure, 324 treatment, 324 whole blood interferon-gamma release assays, 323

1164 Mycoplasma pneumonia clinical presentation, 310, 311 incubation period, 310 testing for mycoplasma, 311 treatment, 311 Myelomeningocele, 569, 570 Myocarditis, 681, 682 N Nail bed lacerations, 211 Nail biting, 184 Nail-patella syndrome, 810, 811 Narcolepsy, 191 Nasal stuffiness, 46 Nasal trauma, 624 Nasolacrimal duct obstruction, 592 Necator americanus, 332 Neck acute laryngitis, 638 atlantoaxial subluxation, 472 branchial cleft cyst, 637–638 Castleman’s disease, 636 cat scratch disease, 635 cervical lymphadenitis, 634–635 chronic laryngitis/hoarseness, 639 infectious mononucleosis, 635 Kikuchi, 636 Klippel–Feil syndrome, 472 laryngomalacia, 640–641 lymphadenitis, 636 lymphoma, 636–637 Sprengel deformity, 472 stridor, 639–640 thyroglossal cyst, 637 thyroid carcinoma, 638 torticollis, 470–471 tularemia, 636 vocal fold paralysis, 639 Neck/cervical spine trauma, 209–210 Necrotizing enterocolitis (NEC) causes, 73 clinical presentation, 73 diagnosis, 73 indication for surgery, 74 laboratory studies, 73 management, 73 mortality rates, 73 Necrotizing fasciitis, 876 aerobic and anaerobic flora, 300 clinical presentation, 300 diagnosis, 300 laboratory findings, 300 treatment, 300 Negative predictive value (NPV), 916 Neisseria gonorrhoeae, 586 clinical presentation in males, 95 clinical presentation in females, 95 DGI, 96, 307 diagnosis, 96 epididymitis, 307 gonococcal pharyngitis, 307 gonococcal proctitis, 307 gonococcal urethritis, 307 incubation period, 307 neonatal conjunctivitis, 307 screening, 308 treatment, 96 Neisseria meningitidis, 268

Index diagnosis, 308 prevention and indications, 309 symptoms, 308 transmission rate, 308 treatment, 309 Neonatal alloimmune thrombocytopenia (NAIT), 372 Neonatal and infantile acne, 861–862 Neonatal gram-negative meningitis, 337 Neonatal herpes simplex (HSV) infection, 337 Neonatal hypoglycemia causes, 424 diagnosis, 424, 425 glucose value, 424 Neonatal lupus erythematosus (NLE), 535 Neonatal meningitis due to Listeria, 337 Neonatal seizures, 552 Neonatal sepsis clinical investigations, 67, 68 clinical manifestation, 67 clinical presentations, 67, 68 early onset sepsis, 67 late onset sepsis, 67 management, 68 medications, 68 risk factors, 67 Neonatal streptococcal meningitis, 337 Neonatal testicular torsion, 849 Neonatal thyrotoxicosis, 432, 433 Neonatology, 1022–1027 Neoplastic disorders acute lymphoblastic leukemia ataxia telangiectasia, 376 Bloom syndrome, 376 clinical presentation, 375 diagnostic evaluation, 375 epidemiology, 375 Fanconi anemia, 377 peripheral blood abnormalities, 375 treatment, 375 trisomy 21, 376 acute myeloid leukemia associated syndromes/risk factors, 377 clinical presentation, 377 diagnostic evaluation, 377 epidemiology, 377 peripheral blood abnormalities, 377 treatment, 377 brain tumors (see Brain tumors) chronic lymphocytic leukemia, 378 chronic myelogenous leukemia, 378 germ cell tumors (see Germ cell tumors) Hodgkin lymphoma clinical presentation, 380 diagnosis, 380 incidence, 379 prognosis, 380 treatment, 380 Langerhans cell histiocytosis chemotherapeutic agents and side effects, 382 clinical presentation, 381 diagnosis, 381 differential diagnosis, 381 incidence, 381 prognosis, 382 treatment, 381 lymphadenopathy biopsy criteria, 379 causes, 378

Index clinical approach, 378, 379 mediastinal mass, 382 nephroblastoma (see Wilms tumor) neuroblastoma (see Neuroblastoma) non-Hodgkin lymphoma biopsy, 380, 381 clinical presentation, 380 diagnosis, 380 incidence, 380 prognosis, 381 treatment, 380 rhabdomyosarcoma clinical presentation, 387 epidemiology, 387 treatment, 388 spinal cord compression, 382 superior vena cava syndrome, 382 tumor lysis syndrome, 382 complications, 382 laboratory test, 382 treatment, 382 Nephroblastoma, see Wilms tumor Nephrogenic diabetes insipidus (NDI), 816–817 Nephrology, 1115–1119 Nephronophthisis (NPH), 818 Nephrotic syndrome, 804–806 Nerve gas agents, 230 Neuroblastoma, 386, 977 clinical presentation, 385, 386 diagnosis, 386 epidemiology, 385 treatment, 386 Neurocutaneous disorders neurofibromatosis type I, 559 neurofibromatosis type II, 559 Sturge–Weber syndrome, 560, 561 tuberous sclerosis, 560 Neurofibromatosis type I, 384, 559 Neurofibromatosis type II, 559, 560 Neurogenic shock, 257 Neurology, 1087–1092 Neuromuscular junction disorders, 571 Neuropathies Bell’s palsy, 574 GBS, 572, 573 HMSN, 573 Ramsay hunt syndrome, 574 Riley–day syndrome, 574 SMA, 574 tick paralysis, 575 Neuroradiology acute left MCA territory infarction, 1007 bilateral cervical and brachial plexus plexiform neurofibromas, 1000 coalescent mastoiditis, 1011 craniopharyngioma, 1012 depressed skull fracture with traumatic subdural hemorrhages, 1014 global hypoxic ischemic injury, 1004 grade IV intraventricular hemorrhage, 997 isolated sagittal craniosynostosis, 1013 juvenile pilocytic astrocytoma, 1006 left parietal calcified cephalohematoma, 1009 multiple sclerosis, 1009 non-communicated hydrocephalus, 1003 pituitary adenoma, 1007 postseptal orbital cellulitis, 1010 retropharyngeal abscess, 999 skull fracture with an epidural hematoma, 1005

1165 Sturge–Weber syndrome, 1001 tethered cord with a terminal lipoma, 998 thyroglossal duct cyst, 1012 tuberous sclerosis complex, 1002 vein of Galen malformation, 1008 Neutropenia acute, 368 benign familial neutropenia, 368 congenital, 368 (see Congenital neutropenia) cyclic clinical presentation, 368 laboratory test, 368 treatment, 368 ethnic, 368 Nevus of Ito, 878 Nevus of Ota, 878 Nevus Sebaceus (of Jadassohn), 856–857 Newborn bilirubin screening, 24 Newborn examination Apgar Score, 43 newborn crying, 43 temperature, 43 Newborn metabolic screening, 165 Newborn nursery care critical congenital heart diseases screening, 39 24 hours discharge criteria, 40 eye prophylaxis, 39 feeding, 39 hearing loss, 39 hepatitis B prophylaxis, 39 hyperbilirubinemia, 40 hypoglycemia, 39 newborn screening, 39 umbilical cord care, 39 vitamin K 1 mg intramuscular injection, 39 well-child visit, 40 Niacin, 737 Nickel dermatitis, 860 Niemann–Pick disease (NPD), 158 Night terrors, 190 Nightmares, 190 Nocturnal enuresis, 822, 823 Nonaccidental burn injury, 896 Non-accidental trauma with classic metaphyseal lesions and skull fracture, 986 Non-classic phenylketonuria, 149 Non-communicated hydrocephalus, 1003 Non-gomerular hematuria, 801 Non-Hodgkin lymphoma biopsy, 380, 381 clinical presentation, 380 diagnosis, 380 incidence, 380 prognosis, 381 treatment, 380 Noninvasive pregnancy testing (NIPT), 112 Nonketotic hyperglycinemia, 150 Nonnucleoside reverse transcriptase inhibitors (NNRTI), 936 Non-polio virus clinical presentation acute hemorrhagic conjunctivitis, 282 congenital and neonatal infection, 282 hand, foot and mouth disease, 282, 283 herpangina, 282 meningitis/encephalitis, 282 myocarditis/pericarditis, 282 diagnosis, 283 epidemiology, 282 treatment, 284

1166 Nonsteroidal anti-inflammatory drugs (NSAIDs), 530 Nonstress test, 37 Nontyphoidal Salmonellosis antibiotic therapy, 315 clinical presentation, 315 diagnosis, 315 incubation period, 314 mode of transmission, 315 Noonan syndrome, 671, 675, 782 DNA testing, 128 health supervision and preventive care, 128 major abnormalities, 128 minor anomalies, 128 prevalence, 128 Normal bone marrow biopsy, 370, 371 Normal renal function, 799 Normocytic anemia approach to, 359 transient erythroblastopenia of childhood (see Transient erythroblastopenia of childhood) Norovirus, 281 Nose choanal atresia, 623 epistaxis, 623 nasal trauma, 624 Nucleoside reverse transcriptase inhibitors (NRTI), 935–936 Null hypothesis, 918 Nursemaid elbow (pulled elbow), 501–502 Nutrition support, 1108, 1109 enteral tube feeds, 749 nutrition assessment, 748 PN, 749, 750 supplements, 748, 749 Nutritional disorders FTT, 752 milk protein allergy, 751, 752 protein-losing enteropathy, 751 refeeding syndrome, 752, 753 undernutrition, 750 vegetarian diets, 751 Nystagmus acquired nystagmus, 606 clinical presentation, 606 congenital sensory nystagmus, 606 management, 606 spasmus nutans, 606 O Obesity, 511 Obsessive compulsive disorders (OCD), 181 Obstructive lesions, 662 Obstructive shock, 257 Obstructive sleep apnea (OSA) syndrome, 724, 725 clinical presentation, 629 definition, 629 diagnosis, 629, 630 etiology, 629 treatment, 630 Occult bacteremia, 310 Oculocutaneous tyrosinemia, 150–151 Odds ratio (OR), 917 Odontogenic infection, 633 Odor of sweaty feet, see Isovaleric acidemia Otitis media with effusion (OME), 615 Oliguria/acute renal failure, 838 Omphalocele, 50 Omphalomesenteric duct remnants, 51 Onychomycosis, 865

Index Ophthalmia neonatorum due to chlamydia, 586 due to herpes simplex, 586 due to n. Gonorrhoeae, 585–586 Ophthalmology, 1092–1095 Opiates ingestion, 227 Oppositional defiant disorder (ODD), 176 Optic neuritis, 597 Oral health screening, 27–28 Oral thrush, 326 clinical presentation, 326 incidence, 325 infection sources, 326 recurrence, 326 risk of infection, 326 treatment, 326 Orbital cellulitis, 591 Orbital fracture, 602 Orbital rhabdomyosarcoma, 607 Organic acidemia, 148 biotinidase deficiency, 147 glutaric aciduria type I/glutaric acidemia, 147–148 isolated beta-methylcrotonyl-CoA carboxylase deficiency, 147 Isovaleric acidemia, 145 MSUD, 146 methylmalonic acidemia, 146 propionic acidemia, 146–147 Organophosphate and carbamate insecticides, 230 Ornithine transcarbamylase (OTC) deficiency, 151 Orthopedics, 1078–1083 angular deformities blount disease (Tibia Vara), 485–486 genu valgum (knock-knee), 485 genu varum (bowleg), 484–485 arthrogryposis, 466 back disorders adolescent idiopathic scoliosis, 475–476 back pain, 472–473 Scheuermann kyphosis, 476, 477 spondylolisthesis, 474 spondylolysis, 473–474 bone and joint infection/inflammation acute hematogenous osteomyelitis, 469, 470 diskitis, 470 septic arthritis, 468–469 transient synovitis, 468 vertebral body osteomyelitis, 470 bone tumors/tumors-like conditions aneurysmal bone cyst, 502, 503 Ewing sarcoma, 504, 505 osteochondroma, 503 osteoid osteoma, 503 osteosarcoma, 504 unicameral cyst, 502 foot disorders calcaneovalgus foot, 489–490 cavus foot, 490 clubfoot (talipes equinovarus), 489 flat foot (pes planus), 490–491 ingrowing toenail, 493 tarsal coalition, 491–492 tip-toe walking, 492 fractures ankle fractures, 499–501 clavicular fracture, 494 compartment syndrome, 500 humerus fracture, 495 lateral condyle fracture, 496–497 medial epicondyle fracture, 497, 498

Index proximal humeral fracture, 494–495 radial head subluxation, 501–502 Salter–Harris Injuries, 493–494 scaphoid fracture, 497–499 supracondylar fracture of humerus, 495–496 tibial shaft fracture, 499 toddler fracture, 499, 500 hip disorders developmental dysplasia of the hip, 477, 478 Legg–Calve–Perthes Disease, 478–479 slipped capital femoral epiphysis, 479, 480 intoeing femoral anteversion, 486–487 internal tibial torsion, 487–488 metatarsus adductus, 488 knee pain and injuries anterior cruciate ligament injury, 482 medial collateral ligament injury, 482 meniscal injury of the knee, 480–482 osteochondritis dissecans, 483 recurrent patellar dislocation/subluxation, 483, 484 leg-length discrepancy, 466 limping child, 465, 466 neck disorders atlantoaxial subluxation, 472 Klippel–Feil syndrome, 472 Sprengel deformity, 472 torticollis, 470–471 polydactyly of the hand, 467–468 viral myositis, 468 Oseltamivir, 279 Osgood–Schlatter syndrome, 517, 518 Osmolality, 825 Osmotic cerebral edema, 252 Osmotic equilibrium, 825 Osseo-integrated auditory implant, 622 Osteochondritis dissecans, 483 Osteochondroma, 503, 992 Osteogenesis imperfecta, 136, 987 abnormalities, 136 health supervision and preventive care, 136 prevalence, 135 type I, 135 type II, 135 type III, 136 type IV, 136 Osteoid osteoma, 503 Osteosarcoma, 504, 991 Otalgia, 611 Otitis externa, 612 Otitis media with effusion (OME), 614–615 Ototoxicity, 621 Outpatient pneumonia, 295 Ovarian cyst, 94 Ovarian torsion, 93 Over-the-counter medications, 939 P Pain management in neonates, 943 in older children, 943–944 Pain syndromes AMPS, 542, 543 BJHS, 542 growing pains, 541, 542 Painful erythematous eye, 585 Palivizumab, 280 Pallor, 43

1167 Pancreatic insufficiency, 781, 782 Pancytopenia differential diagnosis, 369 signs and symptoms, 370 treatment, 370 Panic disorder, 180 Papilledema, 595–597 Papular urticaria, 866 Papulosquamous disorders pityriasis rosea, 867 psoriasis, 866–867 Parainfluenza virus (PV) clinical manifestation, 280 epidemiology, 280 treatment, 280 Paraphimosis, 847 Parasitic conjunctivitis, 589 Parasomnias, 190 Parenteral nutrition (PN), 749, 750 Parinaud oculoglandular syndrome, 587 Parotitis, 631–632 Paroxysmal cold hemoglobinuria, 367, 368 Paroxysmal nocturnal hemoglobinuria (PNH) clinical presentation, 361 etiology, 360, 361 laboratory tests, 361 treatment, 361 Partial aneuploidies, chromosome disorders Cri-du-Chat syndrome, 122 De Grouchy syndrome, 123 Wolf–Hirschhorn syndrome, 121–122 Partial thickness burn, 215 Parvovirus B19, 276 incubation period, 276 mode of transmission, 276 Pasteurella multocida clinical presentation, 311 risk, 311 treatment, 311 Patellofemoral pain, 518–519 Patent ductus arteriosus (PDA), 665, 666 Patient safety and quality improvement, 924, 925, 1132–1134 Pearson marrow–pancreas syndrome, 356, 782 Pectus carinatum, 724 Pectus excavatum, 49, 723–724 Pediatric acute respiratory distress syndrome (PARDS) anti-fungal and anti-viral therapy, 260 cardiovascular complications, 259 causes, 258 clinical investigations, 258 clinical presentation, 258 definition, 258 gastrointestinal complications, 260 management, 259 pericardial tamponade, 260 positive pressure ventilation, 259 renal complications, 260 Pediatric Advanced Life Support (PALS), 624 Pediatric audiometric testing ABR, 620 BOA, 620 conventional audiometry, 620 evoked otoacoustic emission, 620 hearing loss classification, 620 play audiometry, 620 tympanometry, 620, 621 VRA, 620 Pediatric autoimmune neuropsychiatric disorders associated with Streptococcus infections (PANDAS), 299

1168 Pediatric metabolic syndrome, 462 Pediatric pharmacology absorption, 927 antibiotics adverse reactions, 934 aminoglycosides, 930 amoxicillin-clavulanate, 931 ampicillin, 930–931 anti-pseudomonal penicillins, 931 beta lactam antibiotics, 930 carbapenems, 932 cephalosporins (penicillinase-resistant), 931–932 clindamycin, 932 fluoroquinolones, 933 macrolides, 932–933 monobactam, 932 penicillinase-resistant penicillins, 930, 931 rifampin, 933 tetracycline, 933 trimethoprim/sulfamethoxazole, 933 vancomycin, 934–935 antiemetics, 941 antifungals, 937–938 antiparasitics, 936–937 anti-ulcers, 940–941 antivirals acyclovir, 935 antihypertensive medications, 939–940 foscarnet, 935 ganciclovir (IV), 935 nonnucleoside reverse transcriptase inhibitors, 936 nucleoside reverse transcriptase Inhibitors, 935–936 other agents, 935 protease inhibitors, 936 valacyclovir, 935 valganciclovir, 935 bioavailability, 927 diuretics, 938–939 drug reactions, 930 drug-drug interactions, 928 FDA adverse event reporting system, 930 half-life (t½), 929 hepatic metabolism, 927–928 over-the-counter medications, 939 pain management in neonates, 943 in older children, 943–944 prescribing medications, 929–930 renal elimination, 928, 929 sedation deep, 941 general, 941–942 minimal, 941 moderate, 941 preprocedural evaluation, 943 procedural, 942 protocol, 942 reversal agents, 942 Pediculosis, 99, 865 Pelvic inflammatory disease (PID) causes, 97 clinical diagnostic criteria, 97 clinical presentation, 97 diagnosis, 97 genital tract infection, 97 treatment, 98 ultrasonography, 98 Pendred syndrome, 619 Penicillinase-resistant penicillins, 930, 931

Index Peptic ulcer diseases duodenitis, 772 gastritis and peptic ulcers, 771–772 ZES, 772 Performance-enhancing drugs, 521 Perianal bacterial dermatitis, 298 clinical presentation, 298 diagnosis, 298 incidence, 298 treatment, 298 Perianal streptococcal dermatitis, see Perianal bacterial dermatitis Pericardial tamponade causes, 260 complications, 260 definition, 260 diagnosis, 260 management, 260 signs and symptoms, 260 Periodic fever syndromes, 539 Perioral irritant contact dermatitis, 859 Periorbital cellulitis, 310 Periorbital tumor, 606–607 Periorificial dermatitis, 862 Peripheral pulmonary stenosis (PPS), 671 Peritoneal irritation, 248 Peritonsillar abscess (PTA), 627 Permissive hypercapnia, 263 Peroxisomal disorders, 161 X-ALD, 160–161 Zellweger syndrome, 160 Persistent hematuria/proteinuria acute postinfectious glomerulonephritis, 808, 809 alport syndrome, 810 anti-glomerular basement disease, 812, 813 congenital nephrotic syndrome, 807 dense deposit disease, 811 familial thin basement membrane nephropathy, 813 Frasier syndrome, 808 good pasture syndrome, 813 IgA nephropathy, 809, 810 infantile nephrotic syndrome, 807, 808 lupus nephritis, 812 MN, 806, 807 MPGN, 811 nail-patella syndrome, 810, 811 nephrotic syndrome, 804–806 Persistent pulmonary hypertension of newborn (PPHN) antidepressants, 64 clinical presentation, 65 definition, 64 diagnosis, 65 etiology, 64 genetic factors, 64 management, 65 Petechiae, 44 Peutz–Jeghers syndrome (P-JS), 788–789 Pfeiffer syndrome, 130 PHACE syndrome, 673 Phagocyte function Chediak–Higashi syndrome CHS1/LYST gene, 407 clinical presentation, 407 diagnosis, 407 risk of malignancy, 407 treatment, 407 chronic granulomatous disease clinical presentation, 406 diagnosis, 406 treatment, 407

Index leukocyte adhesion defect clinical presentation, 407 diagnosis, 407 treatment, 407 Pharmacology and pain management, 1134–1135 Pharyngoconjunctival fever, 587 Phenothiazines, 227–228 Phenylketonuria (PKU), 148 clinical manifestations, 148 clinical presentation, 148 diagnosis, 148 dietary treatment, 148 imaging studies, 148 pharmacologic management, 148 Pheochromocytoma, 451 Phimosis, 847 Phonological speech disorders, 169 Physical fitness, 521 Physical neglect, 898 Physiologic jaundice causes, 58 clinical presentation, 58 diagnosis, 57 risk factors, 58 TSB concentrations, 57 Pierre Robin sequence, 139 Pilocytic astrocytoma, 383 Pineoblastoma, 384 Pinworm, 331, 332 Pituitary adenoma, 1007 Pituitary gland disorder cerebral salt wasting, 427 congenital hypopituitarism (see Congenital hypopituitarism) craniopharyngioma, 425, 426 DI (see Diabetes insipidus (DI)) growth hormone adverse effects, 422 biologic effects, 422 definition, 422 discontinuing treatment, 422 IGF-1, 422 indications, 422 therapy, 422, 423 hyperpituitarism (see Hyperpituitarism) neonatal hypoglycemia (see Neonatal hypoglycemia) SIADH (see Syndrome of inappropriate ADH secretion (SIADH)) Pityriasis alba, 876–877 Pityriasis rosea, 867 Placenta accreta, 37 Placenta increta, 37 Placenta percreta, 37 Placental abruption, 37 Plagiocephaly, 3 causes, 3 craniosynostosis, 3–4 deformational plagiocephaly, 3, 4 diagnosis, 3 treatment, 3 Plasmodium, 331 Plasmodium falciparum, 331 Platelet disorders approach to, 371 thrombocytopenia, 371 (see Thrombocytopenia) Plethora, 44 Pleural effusion, 721, 722 Pneumatosis intestinalis, 962 Pneumococcal infection, 294, 295 Pneumococcal meningitis, 295 Pneumococcal vaccines, 17–18

1169 Pneumocystis jiroveci clinical presentation, 330 diagnosis, 330 mode of transmission, 330 treatment, 330 Pneumocystis pneumonia, 330 Pneumomediastinum, 722, 723 Pneumonia, 310, 714–716 Pneumothorax and pneumomediastinum, 722 chest radiograph, 66 clinical presentation, 66 management, 66 risk factors, 66 Point of maximal cardiac impulse (PMI), 49 Poisoning and toxic exposure evaluation, 222 general measures, 222 prevention, 222 Poland sequence, 138–139 Poliomyelitis clinical presentation, 284 diagnosis, 284 epidemiology, 284 treatment, 284 Polyarthropathy syndrome, 277 Polycystic ovarian syndrome (PCOS), 92, 451, 452 Polycythemia vera, 56, 374, 375 Polydactyly of the hand, 53, 467–468 Polymicrogyria, 565 Polymyositis, 538 Polythelia, 49 Pompe disease, 154 Porphyria cutanea tarda, 162 Porphyrias acute intermittent porphyria, 161–162 erythropoietic protoporphyria, 162–163 porphyria cutanea tarda, 162 Portal hypertension, 797 Portal venous gas, 962 Positive predictive value (PPV), 916 Post inflammatory hypo/ hyper-pigmentation, 877 Posterior fontanelle, 44 Posterior plagiocephaly, 130 Posterior urethral valves (PUV), 845, 978 Postnatal rubella, 287 Postseptal orbital cellulitis, 1010 Post-streptococcal reactive arthritis (PSRA), 531 Post-term birth, 35 Post-traumatic/post-concussion headaches, 563 Post-traumatic stress disorders (PTSD), 182 Potassium (K), 734 hyperkalemia, 834, 835 hypokalemia, 835 Prader–Willi syndrome (PWS) FISH/microarray analysis, 126 health supervision and preventive care, 126 high-resolution chromosome analysis, 126 major abnormalities, 126 minor anomalies, 126, 127 prevalence, 126 Preauricular pits/sinus (PPS), 611 Precocious puberty (PP), 443 clinical presentation, 445 definition, 443 diagnosis, 445 gonadotropin-dependent PP, 444, 445 gonadotropin-independent PP, 444, 445 pubertal signs, 443 treatment, 445

1170 Preconception screening, 112 Preeclampsia, 41–42 Preimplantation genetic diagnosis (PGD), 112 Premature adrenarche, 441, 442 Premature atrial contractions (PACs), 652, 653 Premature infants, 747, 748 Premature menarche, 443 Premature rupture of membranes (PROM) clinical history, 40 expectant management, 40 indications for delivery, 41 management, 40 medications, 41 risks, 40 Premature ventricular contractions (PVC), 656 Prenatal care abnormal alpha-fetoprotein, 37 C-section, indications, 37 fetal distress, 37 general neonatal risks, 36 placenta, 37 prematurity risk, 36 preterm high mortality rate, 36 routine prenatal laboratory tests, 35, 36 umbilical cord, 36 Prenatal diagnosis, 112–113 Pre-Patellar Bursitis, 519 Preseptal cellulitis, 590–591 Preterm birth, 35 Prevention of infectious diseases antimicrobial resistance, 269 breastfeeding, 268, 269 child-care centers, 267 hospital and office, 268 immunocompromised hosts asplenia, 270 brain injury, 270 central nervous system, 269 indwelling central lines, 270 malignancy, 270 malnutrition, 269 recreational water use, 269 vector-borne disease prevention, 269 Preventive medicine autism screening, 25 blood pressure screening, 24 depression screening, 27 developmental screening, 25 hearing screening, 25 HIV screening, 26–27 iron deficiency anemia screening, 26 lead screening, 25, 26 newborn bilirubin screening, 23, 24 oral health screening, 27–28 tobacco, alcohol, and substance use, 27 TB screening, 27 UA screening, 26 vision screening, 24 Primary adrenal insufficiency, 449 Primary amenorrhea, 91 Primary ciliary dyskinesia, 720, 721 Primary craniosynostosis, 3 Primary headache syndromes diagnosis, 562 treatment of, 562, 563 Primary hypersomnia, 191 Primary hypogonadism, 446 Primary immunodeficiencies, 405–406 Primary insomnia, 191

Index Primary intestinal lymphangiectasia, 782 Primary muscular diseases Becker muscular dystrophy, 572 congenital myotonic dystrophy, 572 Duchenne muscular dystrophy, 572 Primary syphilis, 320 Primary tooth avulsion, 634 Primitive neuroectodermal tumor (PNET), 384 Primitive reflexes, 581 Procedure bias, 913 Prolactinoma, 428 Prolonged QT interval, 656 Propionic acidemia, 146–147 Protease inhibitors, 936 Protein, 741 Protein-losing enteropathy, 751 Proteinuria, 799, 800 Proton pump inhibitors (PPIs), 940–941 Protozoa cryptosporidiosis clinical presentation, 329 mode of transmission, 329 treatment, 330 Entamoeba histolytica clinical presentation, 329 diagnosis, 329 mode of transmission, 329 treatment, 329 giardiasis clinical presentation, 329 diagnosis, 329 mode of transmission, 329 treatment, 329 plasmodium (see Malaria) Toxoplasma gondii clinical presentation, 330 diagnosis, 330 mode of transmission, 330 Pneumocystis jiroveci clinical presentation, 330 diagnosis, 330 mode of transmission, 330 treatment, 330 Proximal humeral fracture, 494–495 Proximal RTA type II, 814 Prune belly syndrome, 51 Pseudohypoparathyroidism (PHP), 437 Pseudomonas species antimicrobial therapy, 314 causes, 314 clinical presentation, 314 risk factors, 314 Pseudostrabismus, 604–605 Psoriasis, 866–867 Psychosocial dwarfism, 421 Psychosocial issues and child abuse, 1125–1128 Psychosomatic disorders clinical presentation, 189 differential diagnosis, 189 management, 189 screening and rating scales, 189 symptoms, 189 Ptosis acquired, 593 congenital, 593 Puberty disorder delayed puberty, 446 gynecomastia, 447 isolated premature thelarche, 442, 443

Index Klinefelter syndrome, 447 McCune–Albright syndrome, 445, 446 PP (see Precocious puberty (PP)) premature adrenarche, 441, 442 premature menarche, 443 primary hypogonadism, 446 Turner syndrome, 448 Pulmonary function testing (PFT), 691 Pulmonary hypertension, 724 Pulmonary interstitial emphysema, 948 Pulmonary sequestration, 704 Pulmonary thromboembolism, 959 Pulmonary valve stenosis (PS), 670, 671 Pulmonary vascular resistance (PVR), 658 Pulmonology, 1104–1108 Pulse oximetry, 692 Prune-Belly syndrome, 845–846 Pygmalion effect bias, 913 Pyogenic arthritis, 310, 468 Pyruvate kinase deficiency clinical presentation, 365 deficiency, 364 laboratory, 365 treatment, 365 Q Quality improvement (QI), 924 Quinsy tonsillectomy, 627 R Rabies virus clinical presentation, 288 mode of transmission, 288 passive and active immunization, 288 treatment, 288 Radioallergosorbent (RAST) testing, 403 Radionuclide cystography, 843 Ramsay hunt syndrome, 574 Ranula, 47 Rasmussen encephalitis, 555 Rathke pouch, 421 Reactive arthritis, 532 Reactive attachment disorder, 182 Reading disorders, 170 Recall bias, 913 Rectal prolapse, 777 Recurrent acute otitis media, 614 Recurrent pancreatitis, 763–764 Recurrent patellar dislocation/subluxation, 483, 484 Red blood cell (RBC) disorders ABO incompatibility classification markers, 348 clinical approach to child with anemia, 348 clinical presentation, 347 diagnosis, 348 prevalence, 347 treatment, 348 anemia in newborn, 345, 346 prevalence, 345, 346 macrocytic anemia (see Macrocytic anemia) microcytic anemia (see Microcytic anemia) RH incompatibility antenatal diagnosis, 347 clinical presentation, 347

1171 laboratory test, 347 pathogenesis, 347 Rh sensitization prevention, 347 treatment, 347 Red reflex examination, 46 Reed–Sternberg cell, 379 Refeeding syndrome, 752, 753 Referred abdominal pain, 760, 761 Reflux esophagitis, 771 Refraction disorders contact lens-related problems, 606 nystagmus, 606 referral criteria, 606 types, 605 uncorrected refractive error, 605 Regional lymphadenopathy, 313 Reiter syndrome, 306 Renal disease, 754 Renal failure AIN, 820, 821 AKI, 819–820 ATN, 821 HUS, 821, 822 Renal tubular acidosis (RTA), 813, 814 Renal vein thrombosis (RVT), 823 Renin–Angiotensin–aldosterone system, 836 Reporting medical error, 923, 924 Research and statistics, 1131–1132 Respiratory conditions acute bronchiolitis, 707, 708 ALTE and BRUE, 725, 726 asthma acute asthma exacerbations, 714 assessment, 710, 711 beta-2 agonists, 713 classification, 711 differential diagnosis for, 710 EIA, 711, 712 inhaled corticosteroids, 713 initial controller therapy, 713 leukotriene receptor antagonists, 714 management of, 712 pathophysiology, 710 symptoms and natural course, 709, 710 BPD, 708, 709 bronchiectasis, 716, 717 cystic fibrosis clinical presentation, 717, 718 complications, 718–720 diagnosis, 719 dysfunction/absence, 717 management, 719, 720 primary ciliary dyskinesia, 720, 721 therapies for treatment, 720 diagnostic testing for blood gas analysis, 693 carbon monoxide gas, 692 CBG, limitations of, 693 chest imaging, 694 lung volumes, 692 PET, 691 pulse oximetry, 692 spirometry, 691 extrapulmonary respiratory conditions pectus carinatum, 724 pleural effusion, 721, 722 pneumomediastinum, 722, 723 pneumothorax, 722 scoliosis, 724

1172 Respiratory conditions (cont.) FB aspiration, 706 lower airway and parenchyma bronchogenic cyst, 704 congenital lobar emphysema, 705, 706 congenital pulmonary adenomatoid malformation, 705 pulmonary sequestration, 704 tracheal stenosis, 705 vascular ring/sling, 705 OSA, 724, 725 pneumonia, 714–716 pulmonary hypertension, 724 SIDS, 726 signs and symptoms acute stridor, differential diagnosis of, 694, 695 apnea, 699, 700 chronic hypoxia, 700 chronic stridor, differential diagnosis of (see Chronic stridor, differential diagnosis) cough, 697, 698 cyanosis, 700 exercise intolerance, 699 hemoptysis, 701, 702 hypertrophic pulmonary osteodystrophy, 701 methemoglobinemia, 701 respiratory failure, 699 stridor/wheezing, 694 tachypnea, 698 thoracic deformities, pectus excavatum, 723–724 pulmonary hemosiderosis, 706, 707 upper airway bacterial tracheitis, 703, 704 epiglottitis, 703 viral croup, 702, 703 Respiratory distress syndrome (RDS), 49, 947 anatomy of pediatric airway, 197–200 cause, 62, 198 clinical presentation, 63 definition, 197 diagnosis, 63 differential diagnosis, 199, 200 etiology, 198 incidence and severity, 62 initial emergency care airway management, 200 breathing management, 200 oxygenation and ventilation, 200, 201 lower airway infection, 199 lower airway obstruction, 199 management, 63 mediastinal masses, 199 pericardial tamponade, 199 pleural effusion, 199 pneumothorax/tension pneumothorax, 199 prenatal steroids, 64 risk factors, 62 surfactant, 62 surfactant protein deficiency, 62 upper airway infection, 199 upper airway obstruction, 198 See also aka Hyaline Membrane Disease Respiratory failure, 197 Respiratory syncytial virus (RSV), 280 Restless leg syndrome, 191 Restrictive cardiomyopathy, 684, 685 Reticulocyte count, 348 Retinal detachment, 601 Retinal hemorrhage, 208, 597–598 Retinitis pigmentosa (RP), 597

Index Retinoblastoma (RB), 595, 596 Retinopathy of prematurity (ROP), 599, 600 causes, 598, 599 complication, 599 definition, 598 early treatment, 600 initial retinal screening, 599 management, 599 risk factors, 599 screening descriptions, 599 treatment programs, 600 Retropharyngeal abscess, 627–628, 999 Rett syndrome, 581 RH incompatibility antenatal diagnosis, 347 clinical presentation, 347 laboratory test, 347 pathogenesis, 347 Rh sensitization prevention, 347 treatment, 347 Rhabdomyosarcoma of the bladder, 983 clinical presentation, 387 epidemiology, 387 treatment, 388 Rheumatology, 1085–1087 Rhinoviruses clinical features, 281 epidemiology, 280, 281 testing, 281 Rhus dermatitis (Poison Ivy), 859 Rhythm strips, 650 Riboflavin, 737 Rickets, 989 clinical presentation, 440 hypophosphatemic rickets, 441 vitamin D deficiency (see Vitamin D deficiency rickets) Rickettsia rickettsii, 318 Rifampin, 933 Rifampin antibiotic prophylaxis, 310 Right ovarian torsion, 982 Riley-day syndrome, 574 Ritter disease, see Staphylococcal scalded skin syndrome (SSSS) RNA virus HIV (see Human Immunodeficiency virus (HIV)) measles (see Measles virus) mumps (see Mumps) non-polio virus acute hemorrhagic conjunctivitis, 282 congenital and neonatal infection, 283 diagnosis, 283 epidemiology, 282 hand, foot and mouth disease, 282, 283 herpangina, 282 meningitis/encephalitis, 282 myocarditis/pericarditis, 282 treatment, 284 poliomyelitis clinical presentation, 284 diagnosis, 284 epidemiology, 284 treatment, 284 rabies virus (see Rabies virus) rubella virus (see Rubella virus) Robin sequence (RS), 633 Rocky mountain spotted fever (RMSF) clinical presentation, 318, 319 complications, 319

Index vs. ehrlichiosis, 319 epidemiology, 318 laboratory testing, 319 treatment, 319 Root cause analysis (RCA) process, 922 Roseola infantum, 276 Rotavirus clinical presentation, 281 diagnosis, 282 epidemiology, 281 Rotavirus vaccine, 16, 1018 Round pneumonia, 956 Routine prenatal laboratory tests, 35, 36 Rubella virus clinical presentation congenital rubella syndrome, 287 postnatal rubella, 287 epidemiology, 287 Rumack-Matthew nomogram, 225 Rumination, 769 S S. aureus food poisoning, 292 S. pneumoniae, 338 epidemiology, 294 risks, 294 Sacral dimple, 53 Saethre-Chotzen syndrome, 130 Salicylic acid ingestion, 225–226 Salmonella infection, 267 Salter–Harris Injuries, 493–494, 985 Sanfilippo syndrome, 156–157 Sapovirus, 281 Sarcoidosis, 540, 541 Scabies, 99, 865 Scaphocephaly, 130 Scaphoid fracture, 497–499 Scarlet fever (scarlatina), 297 causes, 296 clinical presentation, 296, 297 diagnosis, 297 streptococcosis, 297 treatment, 297 Scheuermann kyphosis, 476, 477 Schistosoma haematobium, 333 Schistosoma japonicum, 333 Schistosoma mansoni, 333 Schistosomiasis, 333 School refusal, 889 Schwachman–Diamond syndrome (SDS), 781 clinical presentation, 358 complications, 358 diagnosis, 358 genetics, 358 laboratory findings, 358 treatment, 358 Scoliosis, 474–475, 724 Scorpion stings, 215 Scrotal masses, 94 Seborrheic dermatitis, 860 Secondary amenorrhea, 91 Secondary/central adrenal insufficiency, 450 Secondary craniosynostosis, 3 Secondary syphilis, 320 Sedation deep, 941 general, 941–942 intraprocedural monitoring, 943

1173 minimal, 941 moderate, 941 procedural, 942 protocol, 942 reversal agents, 942 Seizures and epilepsy anti-epileptic drugs, side effects of, 556 BECTS, 553, 554 childhood absence epilepsy, 552, 553 classification of, 551 epilepsy mimics, 558, 559 febrile seizures, 551, 552 infantile spasms, 552 juvenile myoclonic epilepsy, 554 Landau–Kleffner syndrome, 554, 555 Lennox–Gastaut syndrome, 554 neonatal seizures, 552 Rasmussen encephalitis, 555 SE, 556–558 type, 556 Selection bias, 913 Selective IgA deficiency (sIgAD) clinical presentation, 409 diagnosis, 409 treatment, 409 Selective mutism, 180 Selective serotonin reuptake inhibitors (SSRIs), 64 Selenium, 734 Sensorineural hearing loss (SNHL), 618 Sentinel event, 922 Separation anxiety disorder, 180, 889 Septic arthritis, 468–469 Septic shock, 240, 255–257 Septo-optic dysplasia, 424 Serogroup B meningococcal vaccines, 22 Serum sickness causes, 401 clinical presentation, 401 treatment, 401 Sever’s disease, 520 Severe combined immunodeficiency (SCID) causes, 411 clinical presentation, 412 complications, 412 diagnosis, 412 treatment, 412 Sex chromosome aneuploidies, chromosome disorders Klinefelter syndrome, 118–119 Turner syndrome, 119–121 XYY syndrome, 119, 121 Sexual abuse clinical investigations, 899 clinical presentation, 898 examination, 899 incidence, 898 legal issues, 899 management, 900 medical history-taking, 898 Sexual behaviors gender identity disorder, 192 homosexuality, 192 masturbation, 191–192 sexual abuse, 191 sexual identity development, 192 Sexual identity development, 192 Sexual maturity ratings (SMRs) in boys, 443, 444 in girls, 443, 444

1174 Sexually transmitted disease (STDs) in adolescents, 192 Chlamydia trachomatis, 97 herpes simplex, 99 HIV, 99 HPV, 98 Neisseria gonorrhoeae, 95–97 pediculosis, 99 pelvic inflammatory disease, 97, 98 scabies, 99 trichomoniasis, 98 vaccination, 99 Shigella, 267 child care center, 316 clinical presentation, 316 complications, 316 diagnosis, 316 incubation period, 316 mode of transmission, 316 treatment, 316 Shock anaphylactic shock, 257 cardiogenic shock, 255 common features, 253 compensated stage, 253 decompensated stage, 254 definition, 253 distributive shock/septic shock, 255–257 hypovolemic shock, 254–255 management strategies, 254 neurogenic shock, 257 obstructive shock, 257 Short bowel syndrome, 782 Short stature, 420, 422 Shwachman-Diamond syndrome, 752 Sibling rivalry, 889 Sick sinus syndrome (SSS), 655 Sickle cell disease acute chest syndrome clinical presentation, 362 etiology, 362 treatment, 362 aplastic crisis clinical presentation, 363 etiology, 363 laboratory test, 363 treatment, 363 clinical presentation, 361 complications, 361 dactylitis, 363 fever, 362 hydroxyurea, 362 infection, 362 laboratory test, 361, 362 neurologic complications clinical presentation, 364 imaging, 364 incidence, 364 primary prevention of stroke, 364 secondary prevention of stroke, 364 treatment, 364 priapism, 363 renal disease, 364 splenic sequestration, 363 clinical presentation, 363 prognosis, 363 treatment, 363 and trait, 511 vaso-occlusive crisis prevention, 363 risk factors, 362

Index treatment, 363 Sickle cell nephropathy, 823 Sideroblastic anemia, 353 Sinding–Larsen–Johannsen disease, 518 Single hamartomatous polyp, 788 Single umbilical artery (SUA), 51 Sinus arrhythmia, 646, 652 Sinuses acute rhinosinusitis, 624–625 chronic sinusitis, 625 frontal sinus trauma, 626 Sjögren syndrome, 537 Skin diseases, 510 Skin disorders of the newborn diaper dermatitis, 857 erythema toxicum, 855, 856 miliaria, 855–856 nevus Sebaceus (of Jadassohn), 856–857 transient neonatal pustular melanosis, 855 Skull fractures, 45, 1005 Sleep disorders bedtime refusal/frequent awakening, 190–191 benign sleep myoclonus of infancy, 191 classifications, 189–190 dyssomnias, 191 night terrors, 190 nightmares, 190 NREM sleep, 189 parasomnias, 190 parental education, 189 REM sleep, 189 sleep needs according to age, 189 Sleep-disordered breathing (SDB), 629 Slipped capital femoral epiphysis (SCFE), 479, 480 Small bowel obstruction, 975 Small for gestational age (SGA), 35 Smith–Lemli–Opitz syndrome, 163–164 Snake bites characteristics of poisonous snakes, 213 clinical evaluation, 214 clinical presentation, 213, 214 management, 214 Snuffles (rhinorrhea), 46 Social communication disorder, 169–170 Social phobia, 180 Sodium (Na), 734 hypernatremia, 827–829 hyponatremia, 829–831 water intoxication, 831 Sodium diarrhea, 785 Solid foods adolescents, 747 children, 746 home-prepared baby foods, 746 toddlers, 746 Solitary cyst, 91 Solitary kidney, 511 Solitary thyroid nodules, 433 Sotos syndrome, 137–138 Sound amplification devices, 622 Southern tick associated rash illness (STARI), 319 Spasmus nutans, 606 Spastic diplegia, 580 Spastic hemiplegia, 579, 580 Spastic quadriplegia, 580 Spina bifida occulta, 569 Spinal cord diseases atlantoaxial instability, 570, 571 meningocele, 569 myelomeningocele, 569, 570

Index spina bifida occulta, 569 spinal cord trauma, 570 spinal epidural abscess, 570 tethered cord, 569 transverse myelitis, 570 Spinal cord trauma, 570 Spinal epidural abscess, 570 Spinal muscular atrophy (SMA), 574 Spirometry, 691 Spleen, 50 Splenic rupture, 249 Splenic sequestration clinical presentation, 363 prognosis, 363 treatment, 363 Splenomegaly, 272 Spondylolisthesis, 474 Spondylolysis, 473–474 Sporothrix schenckii, 328 Sports medicine, 1083–1085 AC dislocation, 515–516 ankle sprain, 517 anterior shoulder (glenohumeral) dislocation, 515 deep hematoma of thigh, 516–517 female athlete triad, 520–521 hydration and rehydration, 520 injury prevention, 512, 513 Little Leaguer’s elbow, 516 Little Leaguer’s shoulder, 516 mild traumatic brain injury, 513–514 Osgood–Schlatter syndrome, 517, 518 patellofemoral pain, 518–519 performance-enhancing drugs, 521 physical fitness, 521 pre-participation evaluation cardiac conditions, 509 cerebral palsy, 510 chest pain, 508 clearance, 507 clinical background, 507 diabetes mellitus, 509 Down syndrome, 510 eating disorders, 511 exercise-induced bronchoconstriction, 509 eyes, 510 fever, 508 furuncle/carbuncles/folliculitis/impetigo/cellulitis, 510 heart murmur, 509 heat illness, 511 herpes simplex, 510 HIV infection, 509 molluscum contagiosum, 510 obesity, 511 objective, 507 physical examination, 507 screening tests, 507 seizure disorder, poorly-controlled, 509 seizure disorder, well-controlled, 509 sickle cell disease and trait, 511 skin diseases, 510 solitary kidney, 511 syncope, 508 tinea corporis, 510 pre-patellar bursitis, 519 Sever’s disease, 520 Sinding–Larsen–Johannsen disease, 518 Sprengel deformity, 472 Standard error of mean (SEM), 919 Staphylococcal, coagulase-negative, 293 Staphylococcal infections, 290

1175 Staphylococcal scalded skin syndrome (SSSS), 875 clinical presentation, 292 diagnosis, 292 treatment, 292 Staphylococcus aureus, 268 Staphylococcus epidermidis, 292 Staphylococcus saprophyticus, 292 Statistical hypothesis, 918–919 alternative hypothesis, 918 confidence interval, 919 intention-to-treat analysis, 919 null hypothesis, 918 P value, 919 power of a study, 918 standard deviation, 919 type I error, 918 type II error, 918 Status epilepticus (SE), 556–558 anticipatory medication, 219 anticonvulsant medication, 218, 219 causes and risk factors, 217, 218 definitions, 217 laboratory studies, 218 management, 218 Stevens–Johnson syndrome (SJS), 870, 872–873 Stickler syndrome, 619 Strabismus, 46 amblyopia, 605 definition, 603 esotropia, 603, 604 exotropia, 603, 604 hypertropia, 603 hypotropia, 603 pseudostrabismus, 604–605 vertical misalignment, 604 Streptococcal pharyngitis, 295 Streptococcal toxic shock syndrome, 299 clinical presentation, 299 diagnosis, 299 risk factors, 299 treatment, 299 Streptococcus agalactiae, see Group B streptococcal (GBS) infection Streptococcus pneumoniae, 294 Streptococcus pyogenes, 275, 295 Stridor, 639–640 Stroke, 568 Strongyloides stercoralis, 333 Strongyloidiasis, 333 Study designs anecdotal evidence, 915 case reports, 914 case series, 914 case studies, 914 case-control studies, 914 cohort studies, 914 cross-sectional studies, 914 descriptive epidemiologic studies, 915 meta-analyses, 914 randomized controlled trials, 913 statistically significant vs. clinically significant studies, 915 systematic reviews, 914 Sturge–Weber syndrome, 560, 561, 594, 1001 Stye, 591–592 Subacute (de Quervain) thyroiditis, 431 Subarachnoid bleed, 208 Subconjunctival hemorrhage, 46 Subcutaneous fat necrosis of the newborn (SCFN), 44 Subdural hematoma, 207–208 Subgaleal hemorrhage, 45

1176 Subglottic hemangioma, 697 Subglottic stenosis (SGS), 696 Substance abuse, 193 alcohol, 87 amphetamines, 87 anabolic steroids, 88 cocaine, 87 drug/alcohol abuse, 85 drug testing, 88 hallucinogens, 87 indications, 88 inhalants, 88 marijuana, 87 opioids, 87 over-the counter medications, 88 prescription medicine, 88 red flags, 88 screening, 85, 86 tobacco, 86 Sudden infant death syndrome (SIDS), 726 Suicidal behaviors attempting suicide, risk factors, 187 completed suicide, risk factors, 187 management, 188 prevention, 188 prognosis, 188 screening and assessment, 187 Superficial burn, 215 Superior mesenteric artery syndrome, 773 Supervisory neglect, 898 Supportive home therapy, 216, 217 Supracondylar fracture of humerus, 495–496 Supraventricular tachycardia (SVT), 647, 653–655 Surfactant deficiency disease, 947 Surfactant protein (SP) deficiency, 62 Swyer syndrome, 455 Sydenham chorea, 578 Syncope, 508 Syndrome of inappropriate ADH secretion (SIADH), 428 causes, 427 clinical presentation, 427 definition, 427 diagnosis, 427 treatment, 427 Systemic inflammatory response syndrome (SIRS) criteria, 255 Systemic scleroderma, 536 T Tachycardia, 50, 646 Tachypnea, 698 Taenia solium, 333 Takayasu arteritis (TA), 544, 545 Tanner staging, 82 Tarsal coalition, 491–492 Tay–Sachs disease, 158–159 Teeth grinding, 183 Telogen effluvium, 881 Temporal skull fracture, 207 Tension headache, 561 Teratogens, 77 Teratology, 103 Tertiary syphilis, 320 Testicular appendage torsion, 849 Testicular cancer, 850 Testicular torsion, 52, 848, 849 Tetanus and diphtheria toxoids and acellular pertussis vaccine (Tdap), 16, 17 Tethered cord, 569 Tethered cord with a terminal lipoma, 998

Index Tetracycline, 933 Tetraiodothyronine (T4), 429 Tetralogy of Fallot, 953 Thalassemias alpha (see Alpha Thalassemia) beta (see Beta Thalassemia) Thiamin, 737 Throat and oropharynx adenoidectomy, 628–629 OSA syndrome (see Obstructive sleep apnea (OSA) syndrome) peritonsillar abscess, 627 retropharyngeal abscess, 627–628 tonsillitis/pharyngitis, 626–627 Throat cultures diagnostic standard, 626 Thrombocytopenia amegakaryocytic thrombocytopenia, 372 Bernard Soulier syndrome, 373 diagnosis, 371 Glanzmann thrombasthenia, 373 hemolytic uremic syndrome, 372 immune thrombocytopenic purpura, 372 Kasabach–Merritt syndrome, 371 marrow suppression, 372 neonatal thrombocytopenia, 372 thrombocytopenia absent radii syndrome, 373 thrombotic thrombocytopenic purpura, 372 Wiskott Aldrich syndrome, 373 Thrombotic thrombocytopenic purpura, 372 Thumb polydactyly, 467 Thumb sucking, 183, 184 Thyroglossal cyst, 637, 1012 Thyroid cancer, 433 Thyroid carcinoma, 638 Thyroid disorders autoimmune thyroiditis clinical presentation, 430 diagnosis, 431 prevalence, 430 treatment, 431 congenital hypothyroidism (see Congenital hypothyroidism) function, 429 Graves disease, 431, 432 location, 428 neonatal thyrotoxicosis, 432, 433 solitary thyroid nodules, 433 subacute thyroiditis, 431 thyroid-binding globulin deficiency, 429 thyroid cancer, 433 thyroid storm, 434 Thyroid storm, 434 Thyroid-binding globulin deficiency, 430 Tibial shaft fracture, 499 Tick paralysis, 575 Tics, 577 Tinea capitis, 862, 863 Tinea corporis, 510, 863 Tinea cruris, 864 Tinea pedis, 864 Tinea versicolor, 864 Tip-toe walking, 492 T-lymphocytes, 404 Toddler fracture, 499, 500 Toddlers, 746 Toilet training, 192, 193 Tonsillitis/pharyngitis diagnosis, 626 etiology, 626 symptoms, 626 treatment, 627 Torticollis, 470–471

Index Total anomalous pulmonary venous return (TAPVR), 670 Tourette syndrome, 577, 578 Toxic epidermal necrolysis (TEN), 872–873 Toxic shock syndrome (TSS) clinical presentation, 291 production, 291 risk factors, 291 treatment, 291 Toxidromes, 223 Toxocara canis, 333 Toxocara cati, 333 Toxocariasis, 333 Toxoplasma gondii clinical presentation, 330 diagnosis, 330 mode of transmission, 330 treatment, 330 Toxoplasmosis, 330 Tracheal stenosis, 705 Tracheoesophageal fistula (TEF), 771 Trachoma clinical presentation, 306 diagnosis, 306 treatment, 307 etiology, 306 Traction alopecia, 881 Transcutaneous bilirubin (TcB), 61 Transient erythroblastopenia of childhood clinical findings, 357 clinical presentation, 358 etiology, 358 laboratory test, 359 treatment, 359 Transient hypogammaglobulinemia of infancy (THI) clinical presentation, 408 diagnosis, 408 etiology, 408 treatment, 408 Transient neonatal pustular melanosis, 855 Transient synovitis, 469 Transient tachypnea of newborn (TTN) clinical presentation, 64 diagnosis, 64 risk factors, 64 treatment, 64 Transition milk, 742 Transplantation, 894 Transtentorial herniation, 248 Transverse myelitis, 570 Trauma and burns, 209 Traumatic epidural, subdural and subarachnoid hemorrhage, 45 Traumatic iritis, 601 Treacher-Collins syndrome, 129–130, 619 Treatment errors, 922–923 Trematodes (platyhelminthes), 333 Treponema pallidum clinical presentation, 320 diagnosis, 321 mode of transmission, 320 treatment, 321 Treponema-specific tests, 71 Trichinella spiralis, 333 Trichinellosis, 333 Trichomoniasis, 98 Trichotillomania, 183, 881 Trichuriasis, 332 Trichuris trichiura, 332 Tricuspid atresia, 667

1177 Tricyclic antidepressants (TCAs), 228 Trigonocephaly, 130 Triiodothyronine (T3), 429 Trimethoprim-sulfamethoxazole (TMP-SMX), 290, 291, 293, 310, 311, 315, 317, 330, 933 Triple X (XXX) syndrome, 121 Trisomy 13/Patau syndrome, 116–118 Trisomy 18/Edwards syndrome, 116, 117 Trisomy 21, 376 Truncus arteriosus, 669 Tuberculin skin test (TST), 323 Tuberculosis, see Mycobacterium tuberculosis Tuberous sclerosis, 384, 560, 1002 Tubo-ovarian abscess, 984 Tubular abnormalities Bartter syndrome, 815 cystinosis, 815, 816 distal RTA type I, 814 Gitelman syndrome, 815 proximal RTA type II, 814 RTA, 813, 814 type IV RTA, 814, 815 Tularemia, 636 Tumor lysis syndrome complications, 382 laboratory test, 382 treatment, 382 Turcot syndrome, 384 Turner syndrome, 49, 448, 672–674, 782 abnormal karyotype, 120 abnormalities, 120 distinctive phenotype, 120 health supervision and preventive care, 120 neonatal findings, 120 prevalence, 119 Turricephaly, 130 Tympanic membrane perforation, 621 Tympanometry, 620 Type 1 diabetes mellitus Addison disease, 457 celiac screening, 457 clinical presentation, 456 definition, 455 diagnosis, 456 dyslipidemia, 457 nephropathy screening, 458 retinopathy screening, 457 thyroid screening, 457 treatment, 456, 457 Type 2 diabetes mellitus causes, 458 clinical presentation, 458 depression, 460 diagnosis, 458 dyslipidemia, 459 hypertension, 459 nephropathy screening, 459 neuropathy screening, 459 non-alcoholic fatty liver disease, 459 obstructive sleep apnea, 459 PCOS, 459 retinopathy screening, 459 screening, 458 treatment, 458, 459 Type IV RTA, 814, 815 Typhoid fever clinical presentation, 315 diagnosis, 315 mode of transmission, 315 treatment, 315

1178 U U.S. Preventive Services Task Force (USPSTF), 1020 Ulcerative colitis, 778 Ulnar polydactyly, 467 Ulnar (postaxial) polydactyly, 467 Umbilical cord care, 39 Umbilical granuloma, 51 Umbilical hernia, 50 Uncal herniation, 248 Unconjugated hyperbilirubinemia causes, 60 Crigler-Najjar syndrome type 1, 60 Crigler-Najjar syndrome type 2, 60 Gilbert syndrome, 60 Unicameral cyst, 502 Unilateral parotitis, 287 Upper airway infection, 199 Upper airway obstruction, 198 Upper motor neuron facial nerve palsy, 574 Upper motor neuron lesions (UMNL), 571 Urachal remnants, 51 Urea cycle disorders (UCD), 151 Ureterocele, 844, 845 Ureteropelvic junction (UPJ) obstruction, 844 Urethral injuries, 852 Urinalysis (UA) screening, 26 Urinary incontinence, 846 Urinary tract infection (UTI) acute management, 842 clinical background, 841 clinical presentation, 842 definition, 841 imaging, 842 recurrent, 842 risk factor, 841 Urology, 1120–1122 balanoposthitis, 847 bladder exstrophy, 846 circumcision, 847, 848 cryptorchidism, 849, 850 female urethral prolapse, 845 hydroceles, 850 hypospadias, 846 inguinal hernia, 851 kidney stones, 851, 852 micropenis, 848 neonatal testicular torsion, 849 paraphimosis, 847 phimosis, 847 posterior urethral valves, 845 Prune-Belly syndrome, 845–846 testicular appendage torsion, 849 testicular cancer, 850 testicular torsion, 848, 849 ureterocele, 844, 845 ureteropelvic junction obstruction, 844 urethral injuries, 852 urinary incontinence, 846 urinary tract infection acute management, 842 clinical background, 841 clinical presentation, 842 definition, 841 imaging, 842 recurrent, 842 risk factor, 841 varicoceles, 850

Index vesicoureteral reflux antibiotic prophylaxis, 844 clinical background, 842 clinical presentation, 843 diagnosis, 843 indications, 844 International Classification System, 843 management, 843 Urticaria, 871 causes, 394 chronic urticaria causes, 395 diagnosis, 395 differential diagnosis, 395 treatment, 395 clinical presentation, 394 differential diagnosis Erythema multiforme, 394 Papular urticaria, 394 Urticaria pigmentosa, 394 incidence, 394 treatment, 394, 395 Urticaria pigmentosa (UP), 394, 395 Urticarial vasculitis, 395 Usher syndrome, 619 Usual and unusual grief reaction, 887 Uveitis, 529 V Valacyclovir, 935 Valganciclovir, 935 Vancomycin, 934–935 Varicella (VAR) vaccine, 20 Varicella zoster immune globulin (VariZIG), 20, 21, 275 Varicella-zoster virus (VZV) epidemiology, 274 herpes zoster (shingles), 275 prevention, 275, 276 treatment, 275 Varicoceles, 850 Vascular anomalies AVMS, 568 cerebral venous thrombosis, 569 stroke, 568 vein of Galen, 568 Vascular ring/sling, 705 Vascular tumors infantile hemangiomas, 868–869 Kasabach–Merritt phenomenon, 869 Vasculitis AAV, 543 Behçet disease, 547, 548 GPA, 543, 544 HSP, 545–547 MPA, 544 TA, 544, 545 Vasogenic edema, 251 Vaso-occlusive crisis prevention, 363 risk factors, 362 treatment, 363 Vegetarian diets, 751 Vein of Galen malformation, 1008 Velocardiofacial syndrome, 125–126, 696 Velopharyngeal insufficiency (VPI), 629 Venereal disease research laboratory (VDRL) test, 321 Venom of hymenoptera, 401

Index Ventilatory support common indications, 261 endotracheal tubes cuffed tube size, 261 intubation selection, 261 pediatric considerations, 261 uncuffed tube sizes, 261 pressure control mode, 262 respiratory failure, 261–263 ventilator parameters setting, 262, 263 VILI prevention, 263 volume control mode, 262 Ventricular fibrillation (VF), 657 Ventricular septal defect (VSD), 663, 664 Ventricular tachycardia (VT), 657 Verruca vulgaris, 874 Vertebral body osteomyelitis, 470 Vertebral, anorectal, cardiac, tracheo-esophageal, radial, renal, and limb (VACTERL) defects, 140 Vertigo, 617 Very low birth weight (VLBW), 35 Vesicoureteral reflux (VUR) antibiotic prophylaxis, 844 clinical background, 842 clinical presentation, 843 diagnosis, 843 indications, 844 International Classification System, 843 management, 843 Vesiculoulcerative lesions, 273 Viral croup, 702, 703 Viral gastroenteritis, 753 Viral infections adenovirus clinical presentation, 277, 278 incubation period, 277 laboratory test, 278 mode of transmission, 277 respiratory viruses, 278 treatment, 278 arbovirus (see Arbovirus) avian influenza H5N1 clinical presentation, 279 epidemiology, 279 mode of transmission, 279 prevention, 279 cytomegalovirus congenital CMV infection, 271 diagnosis, 271 horizontal transmission, 270, 271 transfusion and transplantation, 271 treatment, 271 vertical transmission, 270 Epstein–Barr virus clinical presentation, 271, 272 complications, 272 diagnosis, 272 management, 272 transmission mode, 271 herpes simplex virus clinical manifestations, 273 epidemiology, 272, 273 herpes gladiatorum, 274 herpetic whitlow, 273 treatment, 274 HPV (see Human papillomavirus (HPV)) human herpesvirus type 6 clinical presentation, 276

1179 treatment, 276 human herpesvirus-7, 276 human herpesvirus-8, 276 human metapneumovirus clinical presentation, 280 epidemiology, 280 treatment, 280 influenza AAP immunization guidelines, 279 clinical presentation, 278 complications, 278 diagnosis, 279 epidemiology, 278 treatment, 279 norovirus and sapovirus clinical presentation, 281 epidemiology, 281 parainfluenza virus clinical manifestation, 280 epidemiology, 280 treatment, 280 parvovirus B19 clinical presentation, 276, 277 incubation period, 276 mode of transmission, 276 respiratory syncytial virus, 280 rhinoviruses clinical features, 281 epidemiology, 280, 281 testing, 281 RNA virus (see RNA virus) rotavirus clinical presentation, 281, 282 diagnosis, 282 epidemiology, 281 varicella-zoster virus epidemiology, 274 herpes zoster (shingles), 275 prevention, 275, 276 treatment, 275 Viral infections of the skin, 873, 874 Viral myositis, 468 Viral vs. bacterial parotitis, 287 Vision screening, 24, 585–600 Visual acuity, 605 Visual development, 605 Visual response audiometry (VRA), 620 Vitamin A, 738, 739 Vitamin B6, 738 Vitamin B12 deficiency, 738 clinical presentation, 355 impaired absorption, 355 inadequate intake, 355 laboratory test, 355 sources, 355 transport protein, absence, 355 treatment, 356 Vitamin C, 739 Vitamin D deficiency, 739 causes, 438, 439 clinical presentation, 439 diagnosis, 440 differential diagnosis, 442 laboratory findings, 442 prevention, 441 radiography, 440 treatment, 440, 441 vitamin D metabolism, 438, 439

1180 Vitamin E, 739, 740 Vitamin K deficiency, 373, 740 See also Hemorrhagic disease Vitamins biotin, 736 folate/folic acid, 736 niacin, 737 riboflavin, 737 thiamin, 737 vitamin A, 738, 739 vitamin B12, 738 vitamin B6, 738 vitamin C, 739 vitamin D, 739 vitamin E, 739, 740 vitamin K, 740 Vitiligo, 877 Vocal cord paralysis (VCP), 695, 696 Vocal fold paralysis, 639 Voiding cystourethrography (VCUG), 843 Volvulus, 762, 763 Vomiting, 765–767 causes of, 766 Von Gierke disease, 154 Von Hippel–Lindau disease, 384 Von Willebrand disease, 374 Vulnerable child syndrome (VCS), 892–893 Vulvovaginitis, 326, 327 W Waardenburg syndrome, 129, 619 Warm autoimmune hemolytic anemia clinical presentation, 367 laboratory test, 367 treatment, 367 Water-bottle heart, 680 Waterhouse–Friderichsen syndrome, 308 Water intoxication, 831 Water-soluble vitamins, 735 Weber syndrome, 782 Well child visits, 28–29 West Nile virus clinical presentation, 288 diagnosis, 288 epidemiology, 288 treatment, 288 Whipworm, 332 White blood cell disorders aplastic anemia, 370 neutropenia (see Neutropenia) pancytopenia (see Pancytopenia) Whole blood interferon-gamma release assays (IGRAs), 323 Whole bowel irrigation, 222 Wilkie syndrome, 773 Williams syndrome, 123, 671, 672, 674 major abnormalities, 123 minor anomalies, 123, 124 prevalence, 123

Index Wilms tumor, 387, 981 clinical presentation, 386 diagnosis, 386 epidemiology, 386 treatment, 387 Wilms tumor-aniridia-growth delay-retardation (WAGR) syndrome, 124 Wilson disease, 791 Wiskott–Aldrich syndrome, 373 causes, 412 clinical presentation, 412 diagnosis, 413 treatment, 413 Wolff-Parkinson-White (WPW) syndrome, 648, 653, 655 Wolf–Hirschhorn syndrome, 121–122, 674 Wolman syndrome, 159 Wound botulism, 302 X X-linked adrenoleukodystrophy (X-ALD), 160–161 X-linked agammaglobulinemia clinical presentation, 408 diagnosis, 408 treatment, 408 X-linked hyper-IgM syndrome (XHIM) causes, 414 clinical presentation, 414 diagnosis, 414 laboratory findings, 414 treatment, 414 X-linked hypophosphatemic rickets, 441 X-linked Inheritance causes, 106 genetic transmission, 106 X-linked dominant diseases, 107 X-linked recessive disease, 106, 107 X-linked lymphoproliferative (XLP) syndrome clinical presentation, 411 diagnosis, 411 treatment, 411 XY pure gonadal dysgenesis, see Swyer syndrome XYY syndrome, 119 Y Yersinia enterocolitica causes, 318 clinical presentation, 318 treatment, 318 Z Zanamivir, 279 Zellweger syndrome, 160 Zinc, 734, 735 Zollinger–Ellison syndrome (ZES), 772