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Partha’s Management Algorithms in PEDIATRIC AND ADOLESCENT PRACTICE
Partha’s Management Algorithms in PEDIATRIC AND ADOLESCENT PRACTICE Editor-in-Chief A Parthasarathy MD DCH DSc (Hon) FIAP Senior Consultant Pediatrician AP Child Care Former Distinguished Professor Department of Pediatrics The Tamil Nadu Dr MGR Medical University Retired Senior Clinical Professor Department of Pediatrics Madras Medical College Deputy Superintendent Institute of Child Health and Hospital for Children Chennai, Tamil Nadu, India Associate Editors Alok Gupta MD (Ped) FIAP Senior Consultant and Counselor Pediatric Specialties Clinic Former Assistant Professor Department of Pediatrics Mahatma Gandhi Medical College and Hospital Jaipur, Rajasthan, India
Anupama S Borker MD DCH Consultant Pediatrician, Hematologist and Oncologist Asian Cancer Institute Mumbai, Maharashtra, India
Dhanya Dharmapalan MD PG Dip in PID (Oxford) Consultant in Pediatrics and Pediatric Infectious Diseases Apollo Hospitals Mumbai, Maharashtra, India
Remesh Kumar MD FIAP Dip in Ped Env Health Medical Superintendent and Chief Pediatrician NSS Medical Mission Superspecialty Hospital Pandalam, Kerala, India
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© 2018, Jaypee Brothers Medical Publishers The views and opinions expressed in this book are solely those of the original contributor(s)/author(s) and do not necessarily represent those of editor(s) of the book. All rights reserved. No part of this publication may be reproduced, stored or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission in writing of the publishers. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book. Medical knowledge and practice change constantly. This book is designed to provide accurate, authoritative information about the subject matter in question. However, readers are advised to check the most current information available on procedures included and check information from the manufacturer of each product to be administered, to verify the recommended dose, formula, method and duration of administration, adverse effects and contraindications. It is the responsibility of the practitioner to take all appropriate safety precautions. Neither the publisher nor the author(s)/editor(s) assume any liability for any injury and/or damage to persons or property arising from or related to use of material in this book. This book is sold on the understanding that the publisher is not engaged in providing professional medical services. If such advice or services are required, the services of a competent medical professional should be sought. Every effort has been made where necessary to contact holders of copyright to obtain permission to reproduce copyright material. If any have been inadvertently overlooked, the publisher will be pleased to make the necessary arrangements at the first opportunity.
Inquiries for bulk sales may be solicited at: [email protected] Partha’s Management Algorithms in Pediatric and Adolescent Practice First Edition: 2018 ISBN: 978-93-5270-383-8
Dedicated to The Practicing Pediatricians of India whose enthusiasm and zeal to update their knowledge through Continuing Medical Education has encouraged us to publish a series of books in Pediatric and Adolescent Practice
Contributors A Balachandran
Pediatric Pulmonologist Dr Mehta Children’s Hospital Chennai, Tamil Nadu, India
Anand K Shandilya
Consultant Pediatrician Dr Anand’s Hospital for Children Mumbai, Maharashtra, India
Anupama S Borker
Consultant Pediatric Hematologist and Oncologist Asian Cancer Institute Mumbai, Maharashtra, India
Professor Department of Pediatrics Ruxmaniben Deepchand Gardi Medical College Ujjain, Madhya Pradesh, India
Head Department of Pediatric Hematology and Oncology Bai Jerbai Wadia Hospital for Children Mumbai, Maharashtra, India
Assistant Professor and Consultant Pediatric Endocrinologist Incharge, Division of Pediatric Endocrinology Department of Pediatrics Sri Ramachandra Medical College and Research Institute Chennai, Tamil Nadu, India
Junior Resident Department of ENT, Head and Neck Surgery Sri Ramachandra Medical College and Research Institute Chennai, Tamil Nadu, India
Professor and Head Department of Dermatology Sree Balaji Medical College and Hospital Chennai, Tamil Nadu, India
Pediatric Neurologist KP Hospitals Former Additional Professor Department of Pediatric Neurology Madras Medical College Chennai, Tamil Nadu, India
Assistant Professor Department of Developmental Pediatrics Child Development Center Government Medical College Thiruvananthapuram, Kerala, India
Assistant Professor Department of Pediatrics Sri Ramachandra Medical College and Research Institute Chennai, Tamil Nadu, India
Consultant Pediatric Surgeon, Urologist and Laparoscopist Tara Neo-Surg Hospital Consultant Jaslok, Nanavati, Seven Hills and Hiranandani Hospitals Mumbai, Maharashtra, India
Chairman Lakeside Center for Health Promotion/Lakeside Education Trust Bengaluru Karnataka, India
Senior Pediatrician and Pulmonologist Dr Mehta’s Children’s Hospital Chennai, Tamil Nadu, India
Hemchand K Prasad
Consultant and Head Department of Pediatric Endocrinology and Diabetes Dr Mehta’s Children’s Hospital Chennai, Tamil Nadu, India
Professor Sree Mookambika Institute of Medical Sciences Kanyakumari, Tamil Nadu, India
Professor and Head Department of Pediatrics Dr BR Ambedkar Medical College and Hospital Bengaluru, Karnataka, India
viii Partha’s Management Algorithms in Pediatric and Adolescent Practice Major K Nagaraju
Director VN Allergy and Asthma Research Centre Visiting Pediatric Allergist Apollo Children’s Hospital Professor and Head Department of Allergy and Clinical Immunology Saveetha Medical College and Hospital Chennai, Tamil Nadu, India
Consultant Pediatric Gastroenterologist Sundaram Medical Foundation and Kanchi Kamakoti CHILDS Trust Hospital Chennai, Tamil Nadu, India
Vice Chancellor Kerala University of Health Sciences Thrissur, Kerala Emeritus Professor Department of Developmental, Behavioral and Adolescent Pediatrics Founder Director Child Development Centre Medical College Thiruvananthapuram, Kerala, India
Consultant Pediatrician, Pediatric Hematologist and Oncologist Lilavati Hospital and Research Centre Shushrusha Citizens’ Co-operative Hospital Mumbai, Maharashtra, India
Professor and Head Department of Pediatrics Sri Ramachandra Medical College and Research Institute Chennai, Tamil Nadu, India
Consultant Pediatric Nephrologist PD Hinduja Hospital Mumbai, Maharashtra, India
Professor Department of Dermatology Government Royapettah Hospital Chennai, Tamil Nadu, India
Specialist (Pediatrician) and Nodal Officer General Hospital Sanjay Gandhi Postgraduate Institute of Medical Sciences Lucknow, Uttar Pradesh, India
Assistant Professor SAT Hospital Govt Medical College Thiruvananthapuram, Kerala, India
Associate Professor and Senior Consultant Department of Pediatrics Sri Ramachandra Medical College and Research Institute Chennai, Tamil Nadu, India
Senior Consultant Department of Pediatric Ophthalmology and Strabismus Sankara Nethralaya Chennai, Tamil Nadu, India
Associate Consultant Department of Pediatric Ophthalmology Sankara Nethralaya Chennai, Tamil Nadu, India
Rakesh Kumar Pilania
Senior Consultant Department of Neonatology Kerala Institute of Medical Sciences Thiruvananthapuram, Kerala, India
DM Fellow Pediatric Clinical Immunology and Rheumatology Postgraduate Institute of Medical Education and Research Chandigarh, India
Director Deep Children Hospital and Research Centre Kutch, Gujarat, India
Head, Pediatrics Clinical Operations and Quality Dr Mehta’s Hospital Chennai, Tamil Nadu, India
Consultant Neonatologist Director Neonatal Intensive Care Unit Neo Clinic and Hospital Aurangabad, Maharashtra, India
Institute of Child Health and Hospital for Children Chennai, Tamil Nadu, India
Contributors S Gnanasambandam
Professor Department of Pediatric Cardiology Madras Medical College Head Department of Pediatric Cardiology Institute of Child Health and Hospital for Children Chennai, Tamil Nadu, India
Pediatric Consultant and Director Department of Pediatrics Mehta Multispecialty Hospital Former Reader Department of Pediatrics Institute of Child Health and Hospital for Children Madras Medical College Chennai, Tamil Nadu, India
DM Fellow Pediatric Clinical Immunology and Rheumatolgy Postgraduate Institute of Medical Education and Research Chandigarh, India
Senthil Ganesh Kamaraj
Visiting Consultant Pediatric Urologist Kanchi Kamakoti CHILDS Trust and Apollo Hospitals Chennai, Tamil Nadu, India
Registrar Mehta Multispecialty Hospital Chennai, Tamil Nadu, India
Consultant in Developmental Pediatrics Kanchi Kamakoti CHILDS Trust Hospital Consultant Pediatrician Sabari Child Care Clinic and Centre for Development Formerly, Senior Assistant Professor Department of Pediatrics Institute of Child Health and Hospital for Children Madras Medical College Chennai, Tamil Nadu, India
Asian Cancer Institute Mumbai, Maharashtra, India
Senior Resident Department of Pediatrics Dr BR Ambedkar Medical College and Hospital Bengaluru, Karnataka, India
Senior Assistant Professor Department of Pediatric Gastroenterology Institute of Child Health and Hospital for Children Madras Medical College Chennai, Tamil Nadu, India
Professor Department of ENT, Head and Neck Surgery Sri Ramachandra Medical College and Research Institute Chennai, Tamil Nadu, India
Medical Research Foundation Chennai, Tamil Nadu, India
Head Department of Pediatrics and Pediatric Allergy Immunology Unit Postgraduate Institute of Medical Education and Research Chandigarh, India
Consultant Pediatrician Dr Anand’s Hospital for Children Mumbai, Maharashtra, India
V Suhas Das
Institute of Child Health and Hospital for Children Chennai, Tamil Nadu, India
Vaman V Khadilkar
Pediatric and Adolescent Endocrinologist Jehangir Hospital and Bharati Vidyapeeth Medical College Pune, Maharashtra, India
Vignesh Kumaran CR
Registrar Department of Pediatrics Mehta Multispecialty Hospital Chennai, Tamil Nadu, India
Foreword MAPAP: Masterpiece on Algorithms for Practitioners & Academicians by Partha —Pramod Jog Clinical scenarios presenting in our day-to-day practice require careful analysis and one must have a logical approach for their proper management. Going through a textbook may be timeconsuming and googling may be mind-boggling. Partha’s MAPAP (Management Algorithms in Pediatric and Adolescent Practice) provides ‘at a glance’ format which is approach-based, algorithmic and can help on-the-spot decision-making. I am sure that the book will act as a support system for medical teachers. Protocolized thinking would get ingrained in the minds right from the days of pediatric upbringing and would help the undergraduates to update grey cells, postgraduates to polish grey cells and practitioners to protect grey cells! The book gives instant guidelines for treatment, bringing uniformity in the management. With each protocol, the book provides concise, precise and up-to-date information. MA-PAP, like C-PAP, will give Positive Academic Pressure and provide oxygen of knowledge to remove hypoxia of ignorance. Reading the algorithms from North to South (top to bottom) will break the monotony of reading text from West to East (left to right) helping the grey cells keep firing in all directions. In the spirit of this book, the readers will develop the algorithm of learning: Reading ↓ Understanding ↓ Applying ↓ Excelling While turning the pages of this book, the readers will cherish the memories of the days of their residency. It would remind them of their former roommates with whom they had academic discussions; the grand rounds; the lovable teachers at those rounds demonstrating an enthusiasm for teaching and for delving into medical history; mentors, who set the example that excellent medical care has to be combined with compassionate doctor–patient communication and; of course, the lessons from patients and families, who taught the most about the patient care. Even though the Internet and notebooks become popular, bias for real books will continue despite the prediction that books will be obsolete in the future. E-books can neither compete with the underlines, highlights and words written in the columns of a printed book nor with the parched tree leaves interspersed in between the pages taking us on a journey down the memory lane. It is the feeling of the paper against one’s fingers, that familiar smell of pulp and glue, a momentary stimulation to the brain when one turns each page… sensations which regulate and focus the brain and make it work better. In every publication, there is a special person who quietly stands out, septuagenarian Partha is that person. For him, working on a new project is fun and he enjoys it. Production of this wonderful source of information for pediatricians must have been a matter of pride and pleasure for him and his colleagues.
xii Partha’s Management Algorithms in Pediatric and Adolescent Practice In the words of Meryl Streep, ‘they have taken their heart to work’. The smile that I see on the face of an audience member who has clearly understood my lecture, is what motivates me to deliver even more lectures. I am sure that the authors of this book will get a similar boost through the millions of these unseen expressions of happiness of their readers. With lyrics (chapters) contributed by eminent pediatricians and music directed by Partha, the algorithms will rightly set the academic rhythm of the readers. Pramod Jog MD (Ped) DNB (Ped) FIAP
Professor Department of Pediatrics DY Patil Medical College Pune, Maharashtra, India President, Indian Academy of Pediatrics, 2016
Preface Partha’s Management Algorithms in Pediatric and Adolescent Practice is really a challenging task for practicing pediatricians. The innumerable doubts and clarifications asked by the parents and adolescent patients are an educating experience by itself. What the pediatricians have not learnt in medical colleges during their undergraduate and postgraduate days, learn new information and gain their own experience through interaction with their parent clients. Attending several continuing medical education (CME) programs, seminars and conferences, no doubt, enriches the practicing pediatricians’ learning skills. At the same time, after attending the CME lectures and discussions, the doctors develop further curiosity to gain more knowledge through interaction with experts. It is with this objective in mind, I conceived the idea of coming out with a series of three books, viz. “Case Scenarios”, “Frequently Asked Questions”, and “Management Algorithms in Pediatric and Adolescent Practice”. The third title is now in your hands. A formidable team of national and international experts joined hands with me with the sole objective of educating the upcoming country’s practicing pediatricians by sharing the rich experience they gained from their own archives. Being luminaries in their own fields, they share their practical knowledge to benefit the readers purely out of academic interest without expecting any remuneration whatsoever. The book is presented with 23 chapters covering, newborn to adolescent care, including several subspecialty disciplines in about 400 pages. The algorithms which have come out of the experts’ own rich experience over decades, I am sure, will be of great use to the practicing pediatricians in managing common and uncommon selected cases in their day-to-day practice. I am confident that this book will serve as a desk-top reference volume. We will strive our best to improve the standard of the title in its subsequent editions. Your suggestions are most welcome. A Parthasarathy Editor-in-Chief
Acknowledgments I acknowledge with gratitude the painstaking efforts of all the contributors for so meticulously presenting the algorithms from their own archives. My grateful thanks go to all of them who have toiled to send their respective files by burning midnight oil. My special gratitude and thanks go to Associate Editors Alok Gupta, Anupama S Borker, Dhanya Dharmapalan and Remesh Kumar, who rendered devoted and dedicated hard work in reviewing, editing and preparing the ‘Print-ready Files’, despite their busy academic and professional commitments. This publication would not have seen the light of the day on schedule without their timely help. Chapter reviewers of this book are Alok Gupta: Chapters 1–5, 12, Anupama S Borker: Chapters 6–11, Dhanya Dharmapalan: Chapters 13–17, and Remesh Kumar: Chapters 18–23. For secretarial assistance, I am grateful to Mrs Nirmala Parthasarathy (PRO), AP Child Care; Dr (Mrs) Prathiba Janardhnan; Mrs Kavitha Balaji; Ms Shruthi Pavana; Ms Swathi Pavana; Ms Kavya Balaji; Ms Mahiya Balaji; Mr R Janardhanan; Mr P Balaji; Mr A Sriramulu (Chennai); Dr Sweta Gupta; Dr Khushbu Jain and Dr Anant Gupta (Jaipur). As in the past, we offer gratitude to Shri Jitendar P Vij (Group Chairman), Mr Ankit Vij (Group President), Ms Ritu Sharma (Director–Content Strategy), Ms Chetna Malhotra Vohra (Associate Director–Content Strategy), and Ms Sunita Katla (PA to Group Chairman and Publishing Manager), of M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India. I would also like to thank Ms Payal Bharti (Senior Manager–Professional Publishing), Mr Ashutosh Srivastava (Asstt. Editor), Mr Amit Mathur (Graphics Designer), and Mr Rakesh Kumar (Typesetter), all of them had taken personal interest and painstaking efforts to produce this state-of-the-art book to whom we are extremely indebted. A Parthasarathy Editor-in-Chief
Contents Chapter 1: Newborn
Rhishikesh Thakre, Naveen Jain
1.1 1.2 1.3 1.4
Thermal Care 2 Best Practices in Breast Milk Feeding 5 Developmental Supportive Care 8 Screening Protocols for High-risk Newborns at Discharge 9
Chapter 2: Growth and Development
MKC Nair, Deepa Bhaskaran
2.1 2.2 2.3 2.4
Approach to a Child with Precocious Puberty 14 Evaluation of a Toddler with Speech Delay 16 Evaluation of an Obese Adolescent 19 Evaluation of an Adolescent with Poor School Performance 23
Chapter 3: Nutrition
KE Elizabeth, Priya Sreenivasan
3.1 3.2 3.3 3.4
Severe Acute Malnutrition 34 Nutritional Rickets 36 Nutritional Anemia 37 Feeding in Low-birth-weight Babies 39
Chapter 4: Immunization
Ashish Pathak, Naveen Thacker
4.1 4.2 4.3 4.4
Cold Chain Failure 42 Management of Adverse Effects Following Immunization 45 Missed Opportunity for Immunization and its Management 51 Immunization in High-risk Groups 54
Chapter 5: Infectious Diseases
Anand K Shandilya, Surpreet Nagi
5.1 5.2 5.3 5.4
Pertussis 58 Rickettsial Disease 60 H1N1 Infection 63 Tuberculosis 66
Chapter 6: Neurology
K Pandian, Shivan Kesavan
6.1 6.2 6.3 6.4
An Approach to Headache 70 Acute Encephalopathy 71 Seizure Disorder 72 Approach to Status Epilepticus in Children 73
Chapter 7: Cardiology
S Gnanasambandam, V Suhas Das, Somasundaram A, S Chidambaram
7.1 7.2 7.3 7.4
Approach to Cyanotic Heart Disease 78 Approach to Congenital Heart Disease 81 Management Algorithm of Kawasaki Disease 84 Management Algorithm in Pediatric Viral Myocarditis 86
xviii Partha’s Management Algorithms in Pediatric and Adolescent Practice Chapter 8: Pulmonology
NC Gowrishankar, L Subramanyam, A Balachandran
8.1 8.2 8.3 8.4
Arterial Blood Gas Analysis—Simplified 92 Management of Cough 96 Nonresolving Pneumonia—Approach to Management 101 Pulmonary Function Test—Spirometry 105
Chapter 9: Gastroenterology and Hepatology
Malathi Sathiyasekaran, Sumathi Bavanandam
9.1 9.2 9.3 9.4
Neonatal Cholestasis Syndrome 110 Constipation in Children 114 Upper Gastrointestinal Bleeding in Adolescents 116 Isolated Hepatomegaly in Adolescents 119
Chapter 10: Nephrology
Pankaj Deshpande, G Sangeetha
10.1 10.2 10.3 10.4
Renal Tubular Acidosis 124 Infection-related Glomerulonephritis 126 Approach to Hypertension in Children and Adolescents 129 Approach to Recurrent Urinary Tract Infection in Children 134
Chapter 11: Urology
Senthil Ganesh Kamaraj
11.1 Phimosis 140 11.2 Congenital Hernias and Hydrocele 142 11.3 Torsion of Testes and Other Causes of Acute Scrotum in Children 145
Chapter 12: Hematology
MR Lokeshwar, Bharat Agarwal
12.1 12.2 12.3 12.4
Iron-deficiency Anemia 150 Acute Lymphoblastic Leukemia 154 Hemorrhagic Disease of the Newborn 158 Hemophilia 160
Chapter 13: Pediatric Oncology
Anupama S Borker, Sujata Mushrif
13.1 13.2 13.3 13.4
Acute Leukemia 168 Lymphoma 171 Abdominal Tumors 176 Brain Tumors 180
Chapter 14: Allergy
H Paramesh, Major K Nagaraju
14.1 14.2 14.3 14.4 14.5 14.6
Allergic Rhinitis 186 Asthma 189 Anaphylaxis 192 Food Allergy 193 Insect Sting Allergy 198 Urticaria and Angioedema 203
Chapter 15: Rheumatology
Sandesh Guleria, Rakesh Kumar Pilania, Surjit Singh
15.1 Pediatric Rheumatology 210
Chapter 16: Endocrinology
J Dhivyalakshmi, Vaman V Khadilkar, Hemchand K Prasad
16.1 16.2 16.3 16.4
Short Stature 218 Precocious Puberty 228 Childhood Hypothyroidism 237 Delayed Puberty in Children 241
Chapter 17: Poisoning
P Ramachandran, PS Rajakumar
17.1 17.2 17.3 17.4
Iron Poisoning 248 Kerosene (Hydrocarbon) Poisoning 250 Paracetamol Poisoning 252 Organophosphate Poisoning 254
Chapter 18: Intensive Care
Piyali Bhattacharya, S Thangavelu, Vignesh Kumaran CR
18.1 Assessment and Treatment of Children and Adolescents with Substance Abuse Disorders 260 18.2 Intensive Care in Adolescent Pregnancy 265 18.3 Approach to Sodium Disturbances 269
Chapter 19: Dermatology
Jayakar Thomas, Parimalam Kumar
19.1 19.2 19.3 19.4
Acne Vulgaris 276 Alopecia Areata 278 Atopic Dermatitis 279 Impetigo 280
Chapter 20: Ophthalmology
Meenakshi S, R Srikanth, Supraja Kasturirangan
20.1 20.2 20.3 20.4
Retinopathy of Prematurity 284 Assessment of Vision 287 Strabismus 289 Leukocoria 292
Chapter 21: Otorhinolaryngology
Somu Lakshmanan, J Shivapriya
21.1 21.2 21.3 21.4
Congenital Lesions of Larynx: Laryngomalacia 296 Laryngeal Web 296 Obstructive Sleep Apnea in Children 297 Pediatric Rhinosinusitis 298
Chapter 22: Pediatric Surgery
22.1 Empyema Thoracis 300 22.2 Abdominal Pain in Children 302 22.3 Chronic Constipation in Children 310
Chapter 23: World Health Organization Standard Algorithms
M Govindaraj, Suman MG
23.1 23.2 23.3 23.4
Integrated Management of Childhood Illnesses 316 Early Infant Diagnosis 326 Malaria Management 328 Dengue Fluid Management 331
Newborn Contributors: Rhishikesh Thakre, Naveen Jain Reviewer: Alok Gupta
¯¯ Thermal Care ¯¯ Best Practices in Breast Milk Feeding ¯¯ Developmental Supportive Care ¯¯ Screening Protocols for High-risk Newborns at Discharge
Partha’s Management Algorithms in Pediatric and Adolescent Practice
1.1 Thermal Care Rhishikesh Thakre
INTRODUCTION The purpose of thermal care is a series of measures to be taken to ensure that the newborn: •• Maintains a normal body temperature (36.5–37.5°C) •• Does not become too cold (37.5°C = hyperthermia) These measures must be initiated from the time of birth, during hospital stay and at home (Boxes 1.1.1 and 1.1.2). Neonatal temperature may start falling by 0.5–1°C every minute if not supported after birth. All newborns must be assessed for thermal wellbeing at every opportunity. No single sign is pathognomonic of thermal instability (Table 1.1.1). The recommended method Box 1.1.1: Best practices for thermal care in the delivery room. • • • • • • •
Warm delivery room: 26–28°C Prewarming surface Drying the newborn immediately, especially the head Wrapping in prewarmed dry blankets that cover the head Eliminating drafts Promoting early skin-to-skin contact with mother Use of plastic wraps, especially in very-low-birth-weight (VLBW) babies • Delay bathing and weighing • Promote early breastfeeding.
Box 1.1.2: Innovative methods for thermal wellbeing in community. • Direct contact with skin of the mother—kangaroo mother care • Application of oil or liquid paraffin • Use of thermocol box without lid • Using solar-based apparatus • Overhead lamps/electric bulbs.
TABLE 1.1.1: Assessment of thermal wellbeing. Signs of hypothermia
Signs of hyperthermia
•• •• •• •• ••
•• •• •• •• •• •• ••
Cool skin temperature Pallor Mottling of extremities Decreased pulses Prolonged capillary refill time •• Apnea •• Heart rate changes •• Decreased activity
Increased heart rate Increased respiratory rate Flushed appearance Extended posture Skin warm to touch Brisk capillary refill Apnea
of screening for thermal wellbeing is measuring axillary temperature by thermometer.
CHOICE OF HEATING EQUIPMENT: WARMER OR INCUBATOR The decision of using incubator or radiant warmer in hospital care is based upon: 1. Familiarity and ease of use. 2. Experience of the staff. 3. Quality of infection control measures. Each equipment has its advantages and disadvantages. Following babies may be candidates for incubator care: •• Care of extremely low-birth-weight (LBW) babies for humidification. •• For isolating an infected baby to achieve barrier nursing. •• For use at low ambient temperature or when there is a lot of convective current where a radiant warmer fails to work. •• For transporting babies.
MODE OF THERMAL CONTROL—SKIN OR AIR MODE Skin Mode The heater output is controlled by the baby’s skin temper ature which is set at a desired level. The heater cycles to keep the skin temperature at that constant.
Advantages 1. Useful in rewarming of hypothermic babies in a graded manner. 2. A set skin temperature of 36°C would suffice for all LBW babies. 3. Reduced need for close monitoring of infant temperature.
Disadvantages 1. Marked fluctuations in air temperature. 2. If the skin probe gets partially dislodged or displaced, overheating occurs. 3. Inappropriately low ambient temperature if the infant is febrile. 4. Fever is likely to be missed unless infant temperature is checked frequently. 5. Masking of hypo- or hyperthermia in baby.
Air Mode The air temperature is set to a desired level and a thermostat in the air flow maintains this temperature.
Advantages 1. Proportionate heat control. 2. Fluctuations in air temperature minimal.
Disadvantages 1. If the air probe is placed away from the body, it will cause variable heating. 2. If the air probe is covered, it will cause variable temperature changes.
SITE OF TEMPERATURE PROBE •• The thermal sensor in skin mode needs to be fixed over abdomen firmly if the baby is in supine position. •• In prone position, the flank may be used. •• Areas with high metabolic rate as the right hypo chondrium (due to underlying liver) need to be avoided. •• Probe should not be applied over bruised or broken skin.
SETTING TEMPERATURE During the first week after birth, LBW babies should be provided with a carefully regulated thermal environment that is near the thermoneutral point. This can be achieved by adjusting temperature to maintain an anterior abdominal skin temperature of at least 36.5°C, using either servocontrol or frequent manual adjustment of air temperature.
MANAGING A COLD BABY HYPOTHERMIA (TABLE 1.1.2) •• Active intervention must be done if the axillary temperature is less than 36.5°C.
•• The method, or combination of methods selected will depend on the severity of the hypothermia and the availability of staff and equipment. •• Measure the baby’s temperature every hour for 3 hours. Once the baby’s temperature is normal, measure the baby’s temperature every 3 hours for 12 hours. •• If the baby’s temperature is increasing at least 0.5°C per hour over the last 3 hours, rewarming is successful. •• Ensure feeding and euglycemia if baby is active or start IV fluids and check sugar. •• Every hypothermic newborn should be assessed for infection.
MANAGING HYPERTHERMIA (>37.5°C) Hyperthermia, because of overheating or underlying infection, can be ruled out by following bedside clues (Table 1.1.3). •• Put off the heat source. •• Do not give antipyretic drugs to reduce the baby’s temperature. •• If hyperthermia is due to overheating, reduce the temperature setting on the warming device: –– Undress the baby partially or fully for 10 minutes and then clothe the baby. –– Observe for signs of sepsis (e.g. poor feeding, vomiting, and breathing difficulty) and repeat when the baby’s temperature is within the normal range. –– Measure the baby’s temperature every hour until it is within the normal range. –– Review nursing care practices to ensure that the problem does not happen again. •• If the hyperthermia is due to exposure to a high ambient temperature or sun exposure: –– Place the baby in a normal temperature environment (25°–28°C). –– Undress the baby partially or fully for 10 minutes, then dress and cover the baby.
TABLE 1.1.2: Management of hypothermia. Mild hypothermia (36–36.5°C)
Moderate hypothermia (32–36°C)
Severe hypothermia (34 weeks), by expressed milk in (32–34 weeks, watispoon), by tube feeding ( 34 weeks)
On demand in well babies. Every 2–3 hourly in sick babies (as indicated)
Position, attachment, weight, frequency and color of urine/stool, maternal concerns and wellbeing
Exclusive till 6 months
A subset of < 32 weeks ( 4 mL) •• Penis TS > II •• Labs: Luteinizing hormone (LH), follicle-stimulating hormone (FSH), and testosterone levels are pubertal. Bone age (BA) is advanced.
A thorough history includes details of pregnancy, birth, gestational age, neonatal course, central nervous system (CNS) insults or infections, medication or radiation exposure, potential exogenous exposure to sex steroids, recent onset visual problems, etc. A pedigree chart including details of extended family regarding the occurrence of precocious puberty and parental pubertal milestones should be drawn. A detailed physical examination including assessment of growth velocity and BA are the initial screening tools. A detailed neurologic assessment including fundus examination is necessary, along with a thorough dermatologic examination to detect skin lesions such as neurofibromas, hyperpigmented patches, axillary freckling, acanthosis nigricans, and acne. Also rule out persistent headache, seizures, focal deficits, adverse affective emotional states, loss of temporal vision, polyuria, polydipsia, and galactorrhea. The pubertal stage can be determined by the Tanner method. Pubic hair, apocrine odor, and axillary hair in both boys and girls are typically related to adrenal androgen
Flowchart 2.1.1: Evaluation of precocious puberty in a child.
Abbreviations: CAH, congenital adrenal hyperplasia; PP, precocious puberty; GnRH, gonadotropin-releasing hormone; β-hCG, beta-human chorionic gonadotropin, 17-OHP, 17-hydroxy progesterone; MRI, magnetic resonance imaging; α-FP, alpha fetoprotein; CPP, central precocious puberty
Growth and Development production (adrenarche). Not only the age of onset but the rate of progression of the pubertal changes is also important. The abdomen, thyroid, and testis should be examined for the presence of a mass or enlargement. The laboratory and radiologic studies are chosen based on the patient’s age, gender, growth velocity, and whether the patient has both breast and pubic hair development or just one secondary sexual characteristic. Investigations are done: •• In a boy who is younger than age 9 years with the onset of any secondary sexual characteristics (like testes size ≥ 4 mL, appearance of pubic/axillary hair). •• In a girl younger than 8 years old with breast stage 2 and/ or pubic hair development. The basic investigation is BA. BA indicates skeletal maturation and is an important marker of hypothalamic maturation. If the BA is advanced as compared to chronological age (CA), especially if by more than two SD, then a thorough evaluation must ensue to determine the etiology. If BA is not advanced or significantly delayed, then no further evaluation is needed. The affected child must be frequently followed up for accelerated growth and pubertal signs, dermatologic changes, and neurologic deficits. Radiological investigations include MRI of the brain to rule out CNS pathologies. Other investigations include pelvic ultrasound and abdominal ultrasound, computed tomography scan or MRI of abdomen and pelvis to assess the size and condition of the reproductive organs and to exclude an adrenal mass. Flowchart 2.1.2: Evaluation of precocious puberty in a girl.
The basic hormonal studies include basal serum levels of LH, FSH, and the sex steroids (estradiol or testosterone). Gonadotropin-releasing hormone (GnRH) analog stimulation test is done in cases of suspected central precocious puberty (CPP). Other necessary hormonal studies include thyroid studies, androstenedione, and human chorionic gonadotropin levels. If signs of androgen excess are evident, electrolytes, dehydroepiandrosterone sulfate (DHEA-S), plasma 17-hydroxy progesterone and urinary ketosteroids are of importance. An adrenocorticotropic hormone (ACTH) stimulation test may be required if congenital adrenal hyperplasia (CAH) is suspected.
PREMATURE THELARCHE (TABLE 2.1.1) This is referred to the appearance of palpable glandular tissue in girls aged between birth and 6 months of age (maternal hormonal effect). The problem can also occur up to 2 years without progressive growth—the etiology is unclear, but may be caused by small ovarian cysts that produce small, transient amounts of estrogen. Hormonal testing and pelvic ultrasound with additional intervention TABLE 2.1.1: Evaluation of precocious puberty. Evaluation premature thelarche History
Onset of breast development: Birth—2 years
•• Only breast development •• Normal height, no accelerated growth
•• Estradiol: Prepubertal, might be slightly elevated •• Bone age: Chronological age Evaluation of premature adrenarche
Abbreviations: GnRH, gonadotropin-releasing hormone; LH, luteinizing hormone; FSH, follicle-stimulating hormone; PP, precocious puberty; hCG, human chorionic gonadotropin; MRI, magnetic resonance imaging
Onset of age: 4–8 years
•• Pubic hair ±, axillary hair development •• No breast development or testicular enlargement •• Normal height, growth velocity can be slightly increased but normal for pubic hair, Tanner stage on the growth velocity charts
•• Luteinizing hormone (LH)/ follicle-stimulating hormone (FSH): Prepubertal •• Bone age slightly advanced but equal to height age
Partha’s Management Algorithms in Pediatric and Adolescent Practice should be done only in cases where breast size increases over a 4–6-month period or is accompanied by rapid growth. Maintaining a proper growth chart and close follow-up to look for any new sign of puberty appearing is very important in the management of such children. In premature thelarche, palpate the breast and rule out whether it is breast tissue which is firm to palpation or adipose tissue (lipomastia) which is soft to palpation. Lipomastia can occur as a manifestation of obesity in children.
Treatment If there is a definitive etiology, then treatment can be focused on that. If BA is significantly more advanced than the CA and the predicted adult height falls below the 5th percentile and predicted adult height is more than 5 cm below the midparental height, then pubertal suppression should be considered. Idiopathic CPP is currently treated with GnRH agonists to suppress the hypothalamo-pituitary-gonadal or adrenal axis. One of the most widely used drugs to treat CPP is intramuscular or subcutaneous leuprolide. GnRH agonists are effective at improving final adult height, especially in younger children with precocious puberty and may improve psychosocial functioning. Following treatment, regression of pubertal development occurs within the first 6 months. One of the benign variants of early pubertal development is premature adrenarche (PA), which is associated with an increase in the secretion of DHEA and DHEA-S. This can result in the appearance of pubic hair, axillary hair, body odor, and mild acne. If these signs are seen in association with normal growth rates, and there is no evidence of genital enlargement, the diagnosis is generally PA. In such cases, DHEA-S levels are typically increased for age, usually in the range of 30–150 μg/dL. Since there is no activation
of the hypothalamic-pituitary-gonadal (HPG) axis, FSH, LH, and estradiol or testosterone concentrations will be at prepubertal levels. Indications for suppression of precocious puberty are: •• Age less than 7 years for girls and less than 8 years for boys. •• Bone age more than two SD beyond CA. •• Predicted adult height more than two SD (or 10 cm) below genetic potential (less than 150 cm). •• Rapid progression of growth or pubertal signs and severe psychosocial disturbances. Height, weight and pubertal staging should be monitored every 3 months for the untreated children. Treated children should be monitored every few months. Their height, weight, and pubertal staging and interval pelvic ultrasounds, GnRH stimulation tests, hormonal studies and BA assessments should be done. Adjuvant treatments of precocious puberty include growth hormone and cyproterone acetate. Before the introduction of gonadotropic analogs, cyproterone acetate was used orally for its effect on pituitary gonadotropin secretions for the treatment of CPP. Acknowledgments: Dr Riaz I, Assistant Professor, Department of Pediatrics, SAT Hospital, Government Medical College, Thiruvananthapuram.
BIBLIOGRAPHY 1. Nield LS, Cakan N, Kamat D. A practical approach to precocious puberty. Clin Pediatr (Phila). 2007;46(4):299-306. 2. Zimlich R. (2016). AAP offers guidance on evaluating early puberty. (Online). Available from http://www. modernmedicine.com/taxonomy/term/4973 [Accessed September, 2017].
2.2 Evaluation of a Toddler with Speech Delay MKC Nair, Deepa Bhaskaran
INTRODUCTION Speech is the motor act of communicating by articulating verbal expression, whereas language is the knowledge of a symbol system used for interpersonal communication. In general, a child is considered to have speech delay if the child’s speech development is significantly below the normal for children of the same age. The speech-delayed
child’s skills are acquired in a normal sequence, but at a slower-than-normal rate. The prerequisites for normal development of speech and language are normal hearing, ability to perceive sounds of speech, normal fine motor development of larynx, lips, tongue, jaw, and palate. Moreover, they should have a mental representation of objects and activities which are important to them. Thus, in the process of language development, the domains
Growth and Development of cognition, social adaptation, and perceptual motor functions interact with each other, influencing and enhancing the growth. Delayed onset of speech may be accompanied by a number of developmental problems (Flowchart 2.2.1). Speech delay has a significant impact on personal, social, academic, and later vocational life. Early identification and appropriate intervention may reduce the emotional, social and cognitive deficits of this disability and may improve the outcome.
PROCESS OF EVALUATION Developmental History A detailed developmental history should be elicited to rule out primarily a disorder affecting language and speech or a global delay. A detailed feeding history provides early indications of neuromotor deficits which can lead on to developmental verbal apraxia. History regarding previous otitis media or central nervous system (CNS) infections should be elicited. Family history of delayed speech is often present in children with developmental language disorders (DLD). Since children with speech delay have difficulty in communicating, they have a high frequency of behavioral problems. A history of behavioral problems should also be sought.
Warning Signs of Speech Delay In each well-baby visit, look for warning signs of speech and language impairment. •• No words at 18 months. •• Not putting two words together at 2 years Flowchart 2.2.1: Classification of speech and language disorders.
•• Not making sentences at 3 years •• Unintelligible speech at 3 years •• Absence of imitative and symbolic play at 2 years.
Physical Examination Physical examination should include anthropometric measurements, such as weight, height and head circumference. A complete general and systemic examination should be done. Look for cutaneous stigmata of tuberous sclerosis like ash leaf macule and café au lait spots. External ear anomalies may indicate underlying hearing impairment. A neurological examination should be done including assessment of movements of tongue, lips, and soft palate. The child should be asked to imitate movements of the lips, and tongue. Rule out the presence of soft neurological signs such as left–right discrimination, fine motor clumsiness and oculomotor disorders.
Development Assessment Development screening should be done using Trivandrum Developmental Screening Chart (TDSC: 0–6 years) or Denver Developmental Screening Chart (DDST-II). The speech and language should be screened using Language Evaluation Screening Test (LEST: 0–6 years). Confirmation of the speech delay and categorization into expressive or combined delay can be done using Receptive Expressive Emergent Language Scale (REELS). IQ can be assessed in children above 3 years of age so that isolated DLD can be differentiated from the delay in speech and communication that occurs as part of developmental delay.
Investigations Hearing assessment should be done in all children with speech delay even if they appear to have intact hearing. In a male child with autism or intellectual developmental disability, fragile X syndrome should be ruled out. In children with isolated language disorder or acquired aphasia, an electroencephalogram (EEG) should be done to exclude subclinical seizures. If the child has associated neurological deficits, seizures, micro- or macrocephaly, they may need evaluation with CT scan or MRI.
Disorders of Speech Voice disorders may be due to congenital malformations of larynx like stenosis, web or cleft, or due to abnormal neuromuscular control of larynx. Neuromotor dysfunction is the most common cause of speech abnormalities in children. These include dysarthria associated with cerebral palsy, dysfluency disorders, e.g. stuttering. The dysarthria in
Partha’s Management Algorithms in Pediatric and Adolescent Practice a child with cerebral palsy is often associated with drooling and swallowing difficulties, besides other sensory and motor deficits which can coexist in such children. Stuttering is a disorder of speech rhythm. A transient dysfluency is common between 3–4 years of age. Persistent and worsening stuttering beyond age of 4 years and presence of grimacing with blocking of speech are to be taken seriously. Even though the child can formulate language, he is unable to say it because of involuntary, repetitive prolongation or cessation of a sound. The exact cause is unknown, but a genetic predisposition has been observed.
Developmental Language Disorders Developmental language disorder is a very common developmental disorder in children and affects approximately 5% children. DLD is defined as failure to acquire language in absence of hearing loss, brain injury or other medical conditions, and in presence of verbal skills two standard deviations below nonverbal skills. Failure to develop normal expressive language at 3 years should be considered pathological. DLD can be classified into three broad types: (1) expressive only, (2) mixed receptive-expressive, and (3) higher-order processing type. Expressive and mixed receptive-expressive disorders involve phonology and phonology-syntax aspects of language, respectively. Higher-order processing disorders mainly affect semantic and pragmatic aspects of language. Combined receptive–expressive DLD are the most common type of DLD in children.
Secondary Language Disorders •• Hearing impairment: Approximately one child per 1,000 is thought to suffer from sensorineural hearing loss with consequent impairment of language development. Children can have intermittent hearing loss as in conductive deafness due to glue ear. In them there maybe delay in language development but not a language disorder. Infants with cleft lip and palate, even those with submucosal cleft, have greater susceptibility to otitis media. If hearing loss is acquired after development of language, the effect on verbal skills is less. There is difficulty in understanding spoken language in these children. In contrast, prelingual deafness has profound effect on development of language, even if diagnosed early and is characterized by limited vocabulary and sentence structure. •• Autism spectrum disorder: In children with autism spectrum disorder (ASD), language acquisition is
delayed or, in some cases, there is regression of early language development. The diagnosis of autism is based on two criteria as per Diagnostic and Statistical Manual V (DSM-V): (1) persistent deficits in social communication and social interaction across contexts not accounted for by general developmental delay; (2) restricted, repetitive patterns of behavior, interests, or activities. In children with ASD, verbal and nonverbal communication skills are affected. All children with autism have language deficit, however, the extent of this deficit and handicap is variable. The term “semantic pragmatic disorder” is reserved for children with social and communication problems but without any restricted, repetitive behavior (RRB). •• Acquired epileptic aphasia: Acquired epileptic aphasia (Landau–Kleffner syndrome) is an acquired aphasia. This is seen in association with an abnormal EEG characterized by spikes, sharp waves or spike and wave discharge, which is usually bilateral and occurring predominantly over the temporal and parietal region. There may not be clinical seizures in up to a quarter of the patients. The language development is normal prior to the onset of seizure or the onset of aphasia. The peak age of symptoms is between 5 years and 7 years. These children tend to have severe receptive and expressive deficit amounting to verbal auditory agnosia. The prognosis of seizure control and normalization of EEG is good but recovery of language is variable. Corticosteroids and sodium valproate have been shown to be somewhat effective.
REMEDIAL THERAPY Remediation is done after assessment by the psychologist and speech and language pathologist. The psychologist should assess the intelligence, memory, attention as well as learning style. The family members of children with DLD are taught language stimulation techniques so that they can perform intervention at home. If there is impairment in receptive skills, visual symbols can be taught to the child for communication. Sign language or use of gestures may be taught to facilitate communication.
PROGNOSIS A good response to speech therapy is seen in pure articulation disorders. Correction of conductive hearing loss may result in normal language development if done early. If a child suffers recurrent ear disease during the first 3 years, their speech and language skills may show a lag in the long
Growth and Development term. In patients with sensorineural hearing loss, prognosis is variable. Most patients with DLD, except for severe verbal dyspraxia and verbal auditory agnosia, will learn to speak reasonably well by school age. Important prognostic factors are IQ and associated deficits. There is a causal relationship between DLDs and reading disabilities. Acknowledgments: Ms Indulekha G, Speech Pathologist and Audiologist, Child Development Centre, Medical College, Thiruvananthapuram.
BIBLIOGRAPHY 1. Aneja S. Evaluation of a child with communication disorder. Indian Pediatr. 1999;36(9):891-900. 2. Bzoch KR, League R. Receptive–expressive Emergent Language Scale, 1st edition. Gainesville: The Tree of Life Press; 1971.
3. Frankenburg WK, Dodds J, Archer P, et al. Denver II: a major revision and restandardization of the Denver developmental screening test. Pediatrics. 1992;89(1):91-7. 4. Hall NE. Developmental language disorders. Semin Pediatr Neurol. 1997;4(2):77-85. 5. Leung AK, Kao CP. Evaluation and management of the child with speech delay. Am Fam Physician. 1999;59(11):3121-8, 3135. 6. Nair MKC, Harikumaran GS, George B, et al. Language Evaluation Scale Trivandrum (LEST 3-6 years) Development and Validation. Indian Pediatr. 2016;53(3):257-8. 7. Nair MKC, Harikumaran Nair GH, Mini AO, et al. Development and validation of Language Evaluation Scale Trivandrum for children aged 0–3 years-LEST (0–3). Indian Pediatr. 2013;50:463-7. 8. Nair MKC, Nair GS, George B, et al. Development and validation of Trivandrum Development Screening Chart for children aged 0-6 years [TDSC (0-6)]. Indian J Pediatr. 2013;80 Suppl 2:S248-55.
2.3 Evaluation of an Obese Adolescent MKC Nair, Deepa Bhaskaran
INTRODUCTION Assessment of a child or adolescent with overweight or obesity has been shown in Flowchart 2.3.1 as well as described in the following six points. 1. Diagnosis: “Obesity” is defined as body mass index (BMI) at or above the 95th percentile and “overweight” is defined as BMI between 85th and 94th percentile for gender and age. BMI above the 99th percentile is the cutoff point for “severe obesity”. 2. Detailed history: A detailed clinical history should be taken to assess the adolescent’s health status. Lifestyle factors should be analyzed so as to understand the energy need and energy expenditure. The food intake history should be taken in detail including snacking habits. The level of physical activity including both organized sports and daily activities such as walking to school, helping with chores, and playing should be assessed. The screen time spent by a child watching television and using computers or smartphones should be regarded as an independent risk factor for childhood obesity and a detailed inventory should be taken. A psychosocial history taking and assessment are essential to rule out psychological disturbances like depression, body dissatisfaction, loss-of-control eating,
unhealthy and extreme weight control behaviors, impaired social relationships, and decreased healthrelated quality of life. 3. A thorough physical examination should be done with special focus on findings suggestive of endocrine, developmental or genetic causes. Blood pressure should be recorded. Hypertension is diagnosed if systolic or diastolic blood pressure falls over 95th percentile for age, gender, and height after recording on three different occasions. General examination should rule out goiter which may indicate an underlying hypothyroidism, acanthosis nigricans which may indicate insulin resistance, hirsutism, excessive acne which are suggestive of polycystic ovary syndrome, violaceous striae, moon face which may indicate Cushing syndrome. Tanner staging should be done to assess reproductive maturity. Premature puberty (age < 8 years in girls, and age < 9 years in boys), apparent micropenis (stretched penile length may be normal), undescended testis/ micropenis (Prader–Willi syndrome), etc. must be evaluated. If the adolescent has headache, optic disks should be examined for optic edema due to pseudotumor cerebri. Respiratory system should be examined to rule out respiratory problems like asthma
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 2.3.1: Assessment of a child or adolescent with overweight or obesity.
Abbreviations: AST, aspartate aminotransferase; ALT, alanine aminotransferase; BMI, body mass index; SBP, systolic blood pressure; DBP, diastolic blood pressure
and sleep apnea. Hepatomegaly should be looked for, to rule out nonalcoholic fatty liver. Musculoskeletal problems, such as slipped capital femoral epiphysis and Blount disease should be ruled out. In spite of all this, the cause of obesity is idiopathic in 95% of cases. Anthropometric measurements form the most important part of investigation in a case of obesity. Baseline values should be recorded in the first visit and it should be repeated in follow-up visits to evaluate the effectiveness of the treatment. BMI (weight/height2) is the preferred index for routine clinical measurement of overweight and obesity in children and adolescents over 2 years of age since it correlates closely with total body adiposity. Other measurement devices such as bioelectrical bioimpedance analysis could be used for detection of obesity instead of BMI. Central fat distribution is associated with increased cardiometabolic risk in children as in adults. Central adiposity is measured by the waist circumference to height ratio. A ratio of more than 0.5 is linked with cardiovascular and metabolic risk in normal and overweight or obese school-aged children.
Skinfold thickness measurements can also be used to measure body fat. They should be measured accurately. The measurement should be compared with age and gender-specific norms. A single anthropometric measurement index may not produce accurate classification in obese and overweight children. Hence multiple indices may have to be measured. 4. Lab testing: Laboratory tests are done only in children above 2 years with obesity and that too depending on BMI and other risk factors. According to the 2007 US Expert Committee’s recommendation, overweight children (BMI 85–94th percentile) should have a fasting lipid-screening test. If risk factors coexist, fasting glucose, alanine aminotransferase (ALT), and aspartate aminotransferase (AST) should be tested every 2 years. High levels of ALT and AST are indicative of nonalcoholic fatty liver disease. The risk factors include hypertension, dyslipidemia, family history of diabetes and history of coronary artery disease (CAD)/angiogram/angioplasty/ coronary artery bypass graft surgery (CABG) in firstdegree relatives of the child.
Growth and Development In children who are obese (BMI ≥ 95th percentile) serum levels of fasting lipids, glucose, ALT, and AST should be measured every 2 years, regardless of risk factors. However, Institute for Clinical Systems Improvement (ICSI) guideline in 2013, based on Krebs et al. study, reported that children 2–6 years of age without risk factors do not need laboratory tests. Interpretation: If the fasting glucose screen test is more than 126 mg/dL, counseling and repeating test are indicated. Lipid profile may be abnormal in obese children. If total cholesterol level is on the borderline (170–200 mg/ dL), the screen test should be repeated after 1 year and if it is elevated (≥ 200 mg/dL), cardiac referral may be needed. Measurement of blood urea nitrogen and creatinine levels for those who are obese (BMI ≥ 95th percentile) is recommended by some expert committees so as to detect renal dysfunction. Hypothyroidism may be associated with pediatric obesity. Routine screening for thyroid hormones is not recommended for obese or overweight children. Recent studies suggest that obesity can be one of the risk factors for vitamin D deficiency and an increase in risk for type 2 diabetes is associated with decreased 25-OH-vitamin D serum level in obese children. 5. Targets for obesity treatment: For overweight adolescents in the age group of 12–18 years, weight maintenance is adequate. For obese adolescents in the same group, a moderate weight loss not more than 1 kg/week may be attempted. A multidisciplinary approach is needed for the treatment of overweight and obesity in children and adolescents. The team should include a pediatrician, dietician, physical instructor, behavioral therapist and a social worker in addition to a motivated team of parents, caretakers, teachers, and policy makers. The first and foremost goal is to bring down the rate of weight gain. The next step is weight maintenance and finally weight reduction to improve BMI. The long-term goal is to improve quality of life and bring down morbidity as well as mortality associated with overweight and obesity. 6. Components and phases of obesity treatment: The different components of overweight and obesity treatment are dietary management, physical activity enhancement and restriction of sedentary behavior, pharmacotherapy and bariatric surgery.
DIETARY MANAGEMENT The aim is weight maintenance or weight loss without compromising appropriate calorie intake and normal
nutrition. Healthy eating patterns should be emphasized and maintained. The recommended fat intake is 25–35% in children in the age group of 4–18 years; carbohydrate intake of 45–65% kcal in all children and adults; and protein intake of 10–30% kcal in children in the age group of 4–18 years. As per the Dietary Intervention Study in Children (DISC), intervention diet can be introduced in obese children who are 8 years or older, without compromising growth, development and pubertal maturity. In this diet, 58% of total calorie intake is provided by carbohydrates, 28% by fats and 14% by protein. Of the 28% calories from fats, 11% should be from monounsaturates, 9% from polyunsaturates and less than 8% from saturates. Cholesterol intake must be less than 75 mg/1000 kcal. It should not exceed 150 mg/ day. Encourage age-appropriate serving sizes including five or more servings of fruit and vegetables, three or more servings of low fat milk or dairy products and six or more servings of whole-grain and grain products per day. Make sure to provide adequate amounts of dietary fiber (age in year + 5 g/d). Reduction of eat-outs should be emphasized. Healthy snacks, balanced diet, adequate intake of fruits and vegetables, adequate fiber content of diet and avoidance of high calorie/high fat foods should be insisted upon. Sugar-sweetened soft drinks and fruit juices have been identified as one of the dietary contributors to overweight and obesity (Joint WHO/FAO Expert Consultation 2003). This report stated that each can of soft drink consumed per day increases the risk of being obese by 60%. Other public health organizations have acknowledged the link between the consumption of sugar-sweetened beverages and obesity and have advised a reduction in intake of such beverages to help to prevent weight gain (Joint WHO/FAO Expert Consultation 2003; Committee on Prevention of Obesity in Children and Youth 2004; Murray et al. 2004; Dietary Guidelines Advisory Committee 2005). Other sugary beverages such as cordials, fruit drinks, fruit juices, energy drinks, and sports drinks can also contribute to an energy imbalance.
PHYSICAL ACTIVITY ENHANCEMENT For preventing overweight and obesity as well as for the treatment, moderately intense regular physical activity is essential. Children and adolescents should engage in not less than 60 minutes of moderate to vigorous physical activity per day to achieve optimum cardiovascular health. For children and young people, physical activity includes play, games, sports, transportation, chores, recreation, physical education, or planned exercise, in the context of family, school, and community activities. The recommendations to improve cardiorespiratory and muscular fitness, bone health, and cardiovascular and metabolic health, the biomarkers are:
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Children and youth aged 5–17 years should engage in at least 60 minutes of moderate to vigorous intensity physical activity daily. •• Amounts of physical activity greater than 60 minutes provide additional health benefits. •• Most of the daily physical activity should be aerobic. Vigorous intensity activities should be incorporated, including those that strengthen muscle and bone, at least three times per week. Higher target levels should be set for overweight and obese children to achieve similar results. In order to burn body fat, longer periods of moderate intensity exercises like brisk walking, are more efficient. Children should get involved in physical activity that is safe, developmentally appropriate, interesting, practical, and preferably with a social element. Other members of the family should also be involved in the exercise program. The activities should be supervised on a regular basis as this will improve compliance. Exercise training also brings about beneficial changes in fat and lean body mass, cardiovascular fitness, muscular strength, endothelial function and glucose metabolism, all of which significantly reduce the morbidity associated with excess weight.
RESTRICTION OF SEDENTARY BEHAVIOR Sedentary activities indulged by children and adolescents include watching TV, nonacademic use of computers and smartphones and playing video games. Every hour of sedentary activity not only increases the chance of obesity but also contributes to failure of many weight reduction attempts in adolescents and children. Screen time should be restricted to less than 2 hours per day in adolescents. Because of excessive TV viewing, children tend to consume larger amounts of energy, fat, sweet, and salty snacks and carbonated beverages. This, in turn, leads to reduced consumption of fruits and vegetables. Hence restricting sedentary behavior will complement dietary management.
PHARMACOLOGICAL TREATMENT There is very limited data supporting the use of pharmacological therapy for pediatric obesity. The drugs sibutramine, orlistat and metformin are currently in use among obese children and adolescents with varying results. Sibutramine is a serotonin non-adrenaline reuptake inhibitor. It enhances satiety and has been shown to be the most effective drug in treating adolescent obesity. The usual side effects are increase in heart rate and blood pressure. Hence, it cannot be used in obese adolescents with high blood pressure.
Orlistat is a pancreatic lipase inhibitor. It reduces fat digestion and acts by increasing fecal fat loss. The side effects are flatulence, diarrhea, gallbladder diseases, and malabsorptive stools. Since fat absorption is affected, fat-soluble vitamin supplementation and monitoring are important. Orlistat is less effective in diets which are low in fats as is the case of many Indian diets. Metformin is of great use in treating obese adolescents with severe insulin resistance, impaired glucose tolerance or polycystic ovarian syndrome. First-line treatment is always lifestyle interventions. If these fail, pharmacotherapy should be initiated as a second line of management. Some anti-obesity drugs may not be as effective as behavioral interventions in reducing BMI: a meta-analysis of behavioral interventions in obese adolescents reported an effect of 3.04 kg/m 2 [95% confidence interval (CI) 3.14–2.94] at 6 months, which was maintained at 12 months follow-up. However, since behavioral interventions require a great investment from both the medical staff and the family, it may not be practical in daily life. Drugs should not be prescribed instead of other treatments but should be, in most cases, a step to discuss before performing bariatric surgery.
SURGICAL TREATMENT Surgical treatment may be needed in some cases of severe adolescent obesity. Bariatric surgery is indicated in adolescents who are severely obese (defined by BMI of > 40), have attained a majority of skeletal maturity (generally > 13 years of age for girls and > 15 years of age for boys), and have comorbidities related to obesity that might be remedied with durable weight loss. If the BMI is more than 50 kg/m2, surgery may be indicated even if there are no associated comorbidities. The preferred bariatric procedures in adolescents are Roux-en-Y gastric bypass and adjustable gastric banding. The usual late complications are smallbowel obstruction, incisional hernias, weight regain, as well as vitamin and micronutrient deficiencies. Meticulous, lifelong medical supervision is of prime importance in patients undergoing surgery.
BIBLIOGRAPHY 1. Alizadeh Z, Khosravi S, Borna S. Obese and overweight children and adolescents: an algorithmic clinical approach. Iran J Pediatr. 2013;23(6):621-31. 2. Barlow SE. Expert committee recommendations regarding the prevention, assessment, and treatment of child and adolescent overweight and obesity: summary report. Pediatrics. 2007;120(Suppl 4):S164-92.
Growth and Development 3. Garanty-Bogacka B, Syrenicz M, Goral J, et al. Serum 25-hydroxyvitamin D (25-OH-D) in obese adolescents. Endokrynol Pol. 2011;62(6):506-11. 4. Krebs NF, Himes JH, Jacobson D, et al. Assessment of child and adolescent overweight and obesity. Pediatrics. 2007;120(Suppl 4):S193-228. 5. Olson ML, Maalouf NM, Oden JD, et al. Vitamin D deficiency in obese children and its relationship to glucose homeostasis. J Clin Endocrinol Metab. 2012;97(1):279-85. 6. Oude Luttikhuis H, Baur L, Jansen H, et al. Interventions for treating obesity in children. Cochrane Database Syst Rev. 2009:CD001872.
7. Raj M, Kumar RK. Obesity in children and adolescents. Indian J Med Res. 2010;132:598-607. 8. Sweeting HN. Measurement and definitions of obesity in childhood and adolescence: a field guide for the uninitiated. Nutr J. 2007;6:32. 9. World Health Organization. (2011). Information sheet: global recommendations on physical activity for health 5-17 years old. [online] Available from http://www.who.int/ dietphysicalactivity/factsheet_young_people/en/ [Accessed September, 2017].
2.4 Evaluation of an Adolescent with Poor School Performance MKC Nair, Deepa Bhaskaran
INTRODUCTION Approach to an adolescent with poor school performance has been described in Flowchart 2.4.1.
DEFINITION Adolescents are said to be performing poorly in academics, if they fail in one or more subject or class or if their marks are below 10th percentile in a particular class or subject or Flowchart 2.4.1: Approach to an adolescent with poor school performance.
if they are identified by the parent or teacher as “difficult” to teach.
CAUSES The predictor variables identified for poor scholastic performance are: (1) not studying daily lessons, (2) poor concentration in studies, (3) lower education status of father, and (4) unhappy family. The causes can be divided into extrinsic (environmental) and intrinsic (individual) factors. The three main factors responsible are: (i) family factors, (ii) school factors, (iii) and adolescent factors.
Family Factors •• •• •• •• •• •• •• •• •• •• ••
Low socioeconomic status Low maternal education Illiteracy Marital discord Divorce Single parent Authoritarian parenting Punitive parenting High parental expectations Violence Abuse.
Abbreviations: PSP, poor school performance; SLD, speech and language delay; ADHD, attention-deficit hyperactivity disorder
•• •• •• ••
Change of school Change in medium of instruction Crowded classrooms Poor teaching methodology
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• •• •• •• ••
High student–teacher ratio Overexpectations of teachers Abusive teachers Bullying Disruptive peer group.
Adolescent Factors •• •• •• •• •• •• •• •• •• •• •• •• •• •• •• ••
Poor study skills Learning disability Low intellectual ability Autism spectrum disorder Attention-deficit hyperactivity disorder (ADHD) Oppositional defiant disorder Adolescent depression Conduct disorder Poor motivation Speech/language impairment Anemia Hearing impairment Visual impairment Mental health disorders Chronic diseases Head injury.
HISTORY TAKING Birth History A detailed birth history, including prematurity, low birth weight, birth asphyxia, hypoglycemia, septicemia, hyperbilirubinemia, and other neonatal complications should be elicited. Over 33% of premature babies born between 32 weeks and 35 weeks of pregnancy and over 25% of newborn babies weighing less than 2,000 g are believed to develop school problems in the future.
Developmental History Detailed developmental history, including speech and language development should be elicited.
Presenting Problems Psychosomatic symptoms like headache, body ache, etc. or change in school performance and behavior should be elicited from the parents and the adolescent. The onset and duration of learning difficulty should be recorded. The nature of the problem, namely incomplete notes, concentration, spelling mistakes, memory lapses, number of failures in a class or a subject should be noted. The present status of the disease, the extent of severity, compliance to treatment should be assessed. Rule out symptoms of obstructive sleep apnea like habitual snoring (often with intermittent pauses, snorts, or gasps), disturbed sleep, and daytime sleepiness, neurobehavioral problem, etc. Rule out chronic diseases like hypothyroidism, diabetes mellitus, chronic asthma, and determine the factors leading to anemia—overt or covert.
Psychosocial History One of the practical methods of eliciting a psychosocial history is by using the HEEADSSS tool. HEEADSSS is an acronym that stands for home, education, eating habits, activities, depression, substance use, sexuality, and safety. HEEADSSS is an excellent tool to delineate strengths of the adolescents like nonacademic achievements that can be used in strength-based counseling. It also identifies the weaknesses of the adolescents and the various adolescent, family and school factors contributing to poor academic performance.
CLINICAL EXAMINATION Physical, systemic, and mental status examinations are essential. Rule out signs of undernutrition, nutrient deficiency, anemia, goiter, visual, and hearing impairment. In a male child, rule out the stigmata of fragile X syndrome (large testes) and Klinefelter syndrome (small testes).
Rule out any past history of meningitis or head injury.
Standard IQ tests (e.g. Stanford–Binet and Wechsler series) assess global intellectual functioning by testing performance on multiple tasks. Intellectual disability can be labeled according to the IQ score into: mild (50–70), moderate (35–50), severe (20–35), and profound (below 20). Mild intellectual disability (slow learners) are the most commonly seen and correspond to 65–75% of all cases.
Family History Family history of speech and, language delay (SLD), ADHD, autism and mental disorder are also to be taken as these are heritable.
Growth and Development Diagnostic and statistical manual 5 (DSM 5): Criteria and Conners scale should be done to rule out ADHD in suspected cases. In suspected cases of depression, Beck’s inventory for depression should be done.
Visual Disorders Rule out visual disorders (convergence insufficiency and failure of accommodation, refractive errors).
Hearing Problems Rule out hearing problems (repeated otitis media, suspected hearing impairment, etc.).
Anemia Rule out anemia. Normal hemoglobin value in 10–18 years old girls is more than 12 g/dL. In boys, between 10 years and 14 years, the normal value is more than 12 g/dL and between 14 years and 18 years above 13 g/dL. The severity of anemia is graded as mild (>10 g/dL but below age-related cutoff for defining anemia), moderate (7–9.9 g/dL) and severe (8 mm)
Abbreviations: IVIG, intravenous immunoglobulin; LMWH, low-molecular-weight heparin
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Age is less than or equal to 12 months •• Male sex.
TREATMENT The medical management of Kawasaki disease is shown in Table 7.3.2.
CORONARY ARTERY OCCLUSION Treadmill test to detect and prognosticate coronary ischemia. Fibrinolysis, percutaneous transluminal coronary angioplasty with stent implantation or coronary artery bypass grafting.
BIBLIOGRAPHY 1. Burns JC, Mason WH, Glode MP, et al. Clinical and epidemiologic characteristics of patients referred for
evaluation of possible Kawasaki disease. United States Multicenter Kawasaki Disease Study Group. J Pediatr. 1991;118(5):680-6. 2. Dengler LD, Capparelli EV, Bastian JF, et al. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J. 1998;17(6):478-81. 3. Newburger JW, Burns JC, Beiser AS, et al. Altered lipid profile after Kawasaki syndrome. Circulation. 1991;84(2):625-31. 4. Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 2004;110(17):2747-71. 5. Rowley AH, Gonzalez-Crussi F, Gidding SS, et al. Incomplete Kawasaki disease with coronary artery involvement. J Pediatr. 1987;110(3):409-13. 6. Ting EC, Capparelli EV, Billman GF, et al. Elevated gammaglutamyltransferase concentrations in patients with acute Kawasaki disease. Pediatr Infect Dis J. 1998;17(5):431-2.
7.4 Management Algorithm in Pediatric Viral Myocarditis Somasundaram A
INTRODUCTION Acute myocarditis is one of the most challenging diseases to diagnose and treat in cardiology. It continues to be a significant cause of morbidity and mortality among children and young adults. The true incidence of the disease is unknown. It is thought that subclinical cases (“silent” myocarditis) occur much more often than severe cases do. Many cases are unrecognized because of the wide range of signs and symptoms and, in some patients, the complete lack of clinical findings. Myocarditis has been defined by the World Health Organization/International Society and Federation of Cardiology as an inflammatory disease of the heart muscle diagnosed by established histological, immunologic, and immunohistological criteria.
ETIOPATHOGENESIS A broad range of insults—infectious, autoimmune, toxic, drug-induced, hypersensitive, and vasculitic have been implicated as causes. The viral infectious causes have evolved over time and are summarized in Figure 7.4.1. The principal mechanism of myocardial damage is given in Flowchart 7.4.1.
Fig. 7.4.1: Evolution of viral causes of myocarditis over time.
Abbreviations: CVA, coxsackie virus A; CVB, coxsackie virus B; EBV, Epstein-Barr virus; HCV, hepatitis C virus
DIAGNOSTIC EVALUATION The diagnostic approach for a child with suspected myocarditis includes strategies to both—aid in establishing the diagnosis and rule out disease processes—that may mimic myocarditis (e.g. a structural cardiac defect or pericardial effusion). In addition, many of these interventions provide an estimate of myocardial function and can help in establishing clinical interventions. Table 7.4.1 summarizes the findings of the various tests done.
Cardiology Flowchart 7.4.1: Principal mechanism of viral-induced myocardial damage.
Abbreviations: TNF, tumor necrosis factor; INF, interferon
TABLE 7.4.1: Findings in myocarditis. Investigation
•• Tachycardia: Sinus or supraventricular •• Atrioventricular block •• Low-voltage QRS complexes •• ST-T wave abnormalities (elevation or depression) •• Prolonged QT interval •• Ventricular ectopy
•• Impaired ventricular function (left > right) •• Ventricular dilatation •• Atrioventricular valve regurgitation •• Left ventricular thrombi
•• Increased levels of creatine kinase and its MB isoenzyme •• Increased level of cardiac troponin C •• Elevated sedimentation rate •• Increased level of C reactive protein •• Increased level of white blood cells, with lymphocytes predominating viral titers •• Increased level of immunoglobulin G Contd...
Cultures: Blood, stool, cerebrospinal fluid, and nasopharyngeal secretions
Presence of virus
Myocyte destruction, fibrosis, inflammatory cells, and lymphocytic infiltrates
Cardiac magnetic resonance imaging (cMRI)
Assess left ventricular ejection, chamber size, wall thickness, localized tissue injury, including edema, hyperemia, and fibrosis
Rule out other congenital, rheumatic, or ischemic heart disease and to determine need for inotropic or mechanical support based on hemodynamic parameters
The criteria used to classify patients as possible, probable, and confirmed myocarditis are given in Table 7.4.2. The Lake Louise Cardiac MRI Diagnostic Criteria for suspected myocarditis is summarized in Box 7.4.1.
TREATMENT The cornerstones of treatment for myocarditis in children remain supportive, and many patients will recover without long-term cardiac sequelae. However, some will suffer continued cardiac compromise including the development of dilated cardiomyopathy and even sudden cardiac death. In
Partha’s Management Algorithms in Pediatric and Adolescent Practice TABLE 7.4.2: Diagnostic classification for patients with myocarditis.
Box 7.4.1: Lake Louise cardiac magnetic resonance imaging
Cardiac MRI findings are consistent with myocardial inflammation, if at least two of the following criteria are present. 1. Regional or global myocardial signal intensity increase in T2-weighted images. 2. Increased global myocardial early enhancement ratio between myocardium and skeletal muscle in gadolinium-enhanced T1-weighted images. 3. There is at least one focal lesion with nonischemic regional distribution in inversion-recovery prepared gadoliniumenhanced T1-weighted images (delayed enhancement). Cardiac MRI study is consistent with myocyte injury or scar caused by myocardial inflammation, if the third criterion is present. A repeat cardiac MRI study between first and second week after the initial cardiac MRI study is recommended, if • None of the criteria are present but onset of symptoms is very recent and there is strong clinical evidence for myocardial inflammation. • One of the criteria is present. The presence of left ventricular dysfunction or pericardial effusion provides additional supportive evidence for myocarditis.
Electrocardiogram or imaging
Possible subclinical acute myocarditis In the clinical context of Absent possible myocardial injury without cardiovascular symptoms but with at least one of the following: •• Biomarkers of cardiac injury raised •• Electrocardiogram findings suggestive of cardiac injury •• Abnormal cardiac function on echocardiogram or cardiac MRI
Probable acute myocarditis In the clinical context Absent of possible myocardial injury with cardiovascular symptoms and at least one of the following: •• Biomarkers of cardiac injury raised •• Electrocardiogram findings suggestive of cardiac injury •• Abnormal cardiac function on echocardiogram or cardiac MRI
Definite myocarditis Histological or immunohistological
the acute phase of the disease, current practice is to support the patient to the degree needed with inotropes, afterload reduction, mechanical ventilation, or extracorporeal membrane oxygenation (ECMO) as the condition warrants.
diagnostic criteria for suspected myocarditis.
•• Afterload reducing agents may be considered, if volume overload exists with preserved cardiac output. •• Antiarrhythmic agents in patients with significant arrhythmias •• Mechanical ventilation in patients with respiratory failure secondary to myocardial failure. •• Mechanical support with left ventricular-assist devices (LAD) or ECMO in patients with rapidly progressive severe heart failure and used as a bridge to transplantation. •• Anticoagulation in patients with low-molecular-weight heparin (LMWH) in patients with severe myocardial depression/dilatation. •• Implantable cardioverter/defibrillator for patients with risk for sudden cardiac death due to conduction system diseases.
Immunosuppression and antiviral therapy: •• High-dose immune globulin during the acute phase has been associated with improved recovery and a tendency for better survival. •• Steroids, azathioprine, cyclosporine, and other immunosuppressive agents, have all been proposed as effective agents, although with insufficient evidence. •• Antiviral therapy does not have significant role in myocarditis management. •• Use of interferon is being widely studied.
•• Inotropic support with milrinone, dopamine, or dobutamine in patients with evidence of low cardiac output. •• Diuretics
The management algorithm is summarized in Flowchart 7.4.2. The summary of the pathogenesis, diagnostic workup, and therapy during various stages of the disease is given in Flowchart 7.4.3.
Initial Management •• Stabilization of airway, breathing and circulation (ABC) •• Bed rest and limitation of activity.
Cardiology Flowchart 7.4.2: Management algorithm of clinically suspected acute myocarditis.
Abbreviations: LV: left ventricle; MRI: magnetic resonance imaging; VAD: ventricular assist device
Flowchart 7.4.3: The summary of the pathogenesis, diagnostic workup, and therapy during various stages of the disease.
Abbreviations: PCR, polymerase chain reaction; TLR, toll-like receptors; CMR, cardiac magnetic resonance; MMP, matrix metalloproteinases; ACEi, angiotensin-converting enzyme inhibitors; ARB, angiotensin receptor blocker
Partha’s Management Algorithms in Pediatric and Adolescent Practice
PROGNOSIS Prognosis is often dictated by clinical presentation and underlying etiology. Mortality is high during the newborn period. Transplantation is, sometimes, the only option for children with significant cardiac failure or dilated cardiomyopathy due to myocarditis. Preferably, children are not put on a list for transplantation until they have progressed to the chronic phase of the disease, because recovery is possible in even the most severe cases.
BIBLIOGRAPHY 1. Canter CE, Simpson KE. Diagnosis and treatment of myocarditis in children in the current era. Circulation. 2014;129(1):115-7.4.
2. Foerster SR, Canter CE. Contemporary etiology, outcomes, and therapy in pediatric myocarditis. Prog Pediatr Cardiol. 2011;31(2):123-8. 3. Kühl U, Schultheiss HP. Myocarditis in children. Heart Fail Clin. 2010;6(4):483-96. 4. Levine MC, Klugman D, Teach SJ. Update on myocarditis in children. Curr Opin Pediatr. 2010;22(3):278-83. 5. Shauer A, Gotsman I, Keren A, et al. Acute viral myocarditis: current concepts in diagnosis and treatment. Isr Med Assoc J. 2013;15(3):180-5.
Pulmonology Contributors: NC Gowrishankar, L Subramanyam, A Balachandran Reviewer: Anupama S Borker
¯¯ Arterial Blood Gas Analysis—Simplified ¯¯ Management of Cough ¯¯ Nonresolving Pneumonia—Approach to Management ¯¯ Pulmonary Function Test—Spirometry
Partha’s Management Algorithms in Pediatric and Adolescent Practice
8.1 Arterial Blood Gas Analysis—Simplified NC Gowrishankar, L Subramanyam, A Balachandran
INTRODUCTION Acid–base status is usually assessed by measuring the components of the bicarbonate–carbon dioxide buffer system in blood. When blood gas analysis is carried out, the partial pressure of CO2 (pCO2) and the pH are each measured using specific electrodes, and the serum bicarbonate (HCO3) concentration is calculated with the Henderson–Hasselbalch equation. When the HCO3 is measured in venous blood, it is usually measured directly as “total CO2” with an ion-selective electrode. The directly measured venous HCO3– is generally about 2 mEq/L greater than the simultaneously calculated arterial HCO3. For an arterial sample, the normal range for pH is 7.36–7.44; bicarbonate (HCO3) concentration is 22–26 mEq/L; and for PCO2 is 36–44 mm Hg. Each simple acid–base disorder is normally associated with a compensatory respiratory or renal response that reduces the change in the HCO3/pCO2 ratio and therefore in pH. The presence of a single disturbance of acid–base status, with the appropriate respiratory or renal compensation for that disturbance, is called a simple acid–base disorder. The simultaneous presence of more than one acid– base disturbance is called a mixed acid–base disorder. Mixed acid–base disorders can be suspected from the patient’s history, from a lesser- or greater-than-expected compensatory response, and from analysis of the Δanion gap (ΔAG) and ΔHCO3. There are two methods for interpretation of arterial blood gas (ABG) analysis: 1. First method is the conventional approach: This method helps to understand the relationship between the measurements but is not very useful for mixed disorders. 2. Second method is simple and quick and can easily handle multiple concurrent acid–base disorders.
pH. Respiratory alkalosis is characterized by low-arterial PCO2 and an elevated arterial pH. Step 2: Assess the degree of compensation if the compensation is inadequate or excessive, this is indicative of a mixed acid–base disorder. Step 3: Determine if the anion gap is elevated. If it is, then analyze the ratio of the increase in anion gap to the decrease in the HCO3 concentration. This is the Δanion gap/ΔHCO3 ratio. When an anion gap acidosis exists, these changes should be quantitatively similar to one another; that is, the Δanion gap should be of similar magnitude as the ΔHCO3 (Box 8.1.1).
METHOD 2 Simplified Acid–Base Analysis The information required to determine exactly what the acid–base status is: •• Arterial blood gas: pH and pCO2 •• Anion gap = Na+ − (HCO3− + Cl−). There are four major steps to this method. Table 8.1.1 gives the steps and the explanation of each step is given below. Step 1: It has to be noted that the body does not fully compensate for primary acid–base disorders and, therefore, the pH narrows down what the primary disturbance is Box 8.1.1: Analysis of ΔAG and ΔHCO3 • For each unit rise in AG (above normal) HCO3 should fall by one unit (below normal) • Normal values: AG = 12, HCO3 = 24. AG
HCO3 is ↓ less than predicted, So HCO3 is too high; Hence, HAGMA and metabolic alkalosis
HCO3 is ↓ more than predicted, So HCO3 is too low; Hence, HAGMA and NAGMA.
The following is a three-step approach for the evaluation of most patients with acid–base disorders: Step 1: Establish the primary diagnosis. Metabolic acidosis is characterized by a low-serum HCO3 and a low-arterial pH; the serum anion gap may be increased or normal. Metabolic alkalosis is characterized by an elevated serum HCO3 and an elevated arterial pH. Respiratory acidosis is characterized by an elevated arterial PCO2 and a low-arterial
Abbreviation: HAGMA, high anion gap metabolic acidosis; NAGMA, normal anion gap metabolic acidosis
Pulmonology Step 2b: Calculation of the change in anion gap (∆AG) = (Calculated AG) – 12 (or) [Na+ − (HCO3− + Cl−)] – 12.
TABLE 8.1.1: Evaluating acid–base disorders. Step
How to determine answer
Determine serum pH
Look at ABG results
2. Anion gap
a. What is the anion gap (AG)?
Na − (HCO3 + Cl )
b. What is the change in AG (measured – normal)
Measured AG-12 [Delta anion gap (ΔAG)]
a. What is expected HCO3?
24-Delta AG (ΔAG)
b. Compare expected HCO3− to actual HCO3− 4. pCO2
a. What is expected pCO2?
15 + measured HCO3−
b. Compare expected pCO2 to actual pCO2. Abbreviation: ABG, arterial blood gas
(assuming no treatment). If the patient has an acidemia, the primary disturbance is metabolic or respiratory acidosis. If the patient has alkalemia, the primary disturbance is a metabolic or respiratory alkalosis: •• Serum pH less than 7.35 defines acidemia •• Serum pH greater than 7.45 defines alkalemia. Steps 2 and 3 are to be evaluated for a metabolic component. Physiologic explanation for steps 2 and 3: The body has complex buffering systems for acidosis (intracellular and extracellular systems). The main extracellular buffer is bicarbonate and its main job is to neutralize the acid to keep the blood pH stable. For every increase of one unit in an acidic anion in the blood, the bicarbonate level should reduce by 1 (because of the neutralization). (In general, the ratio of anions to bicarbonate is 1:1). So as the anions go up, bicarbonate goes down proportionally. If there are no extra anions, then the bicarbonate should be normal. Metabolic acidosis occurs when there is a net gain of H+ – or net loss of HCO3 and can be due to: •• ↑ acid generation: Exogenous source (e.g. salicylate ingestion) or endogenously (e.g. lactic acidosis). •• ↓ in HCO3: Gastrointestinal (GI) loss (e.g. diarrhea) or renal cause [proximal renal tubular acidosis (RTA)], and •• ↓ acid excretion by kidney: Reduced GFR (e.g. renal failure) or impaired ability to acidify urine (e.g. distal RTA). Step 2a: Calculation of the anion gap = Na+ − (HCO3− + Cl−). Normal anion gap is 12. If the AG is more than 12, high anion gap metabolic acidosis (HAGMA) is present and this is the first potential acid–base diagnosis.
Step 3a: Calculate the expected bicarbonate level. If the AG is elevated, the expected bicarbonate = [24 − (∆AG)]. If AG is not elevated, the expected bicarbonate = 24. Essentially what one is doing in this step is reducing the bicarbonate by 1 for every unit increase in acidic anion that the bicarbonate neutralizes. Step 3b: It is comparing the expected bicarbonate from Step 3a to the actual bicarbonate. If the measured bicarbonate is less than what is expected, a nonanion gap metabolic acidosis (NAGMA) is present. If the measured bicarbonate is more than what is expected, a metabolic alkalosis is present. Nonanion gap metabolic acidosis and the metabolic alkalosis can coexist with a HAGMA. A standard deviation of 3 exists on each of these calculations; so if the calculated and observed values are within the 3 numbers, call them “close enough”. Step 4: Evaluate the patient for a respiratory component. Step 4a: Calculate the expected pCO2. The expected pCO2 = 15 + actual HCO3 from the blood biochemistry. Step 4b: Compare the expected pCO2 to the actual pCO2 from the blood gas. If more CO2 is present on the blood gas than you expect, a respiratory acidosis is present. If less CO2 is present on the blood gas than you expect, a respiratory alkalosis is present. These respiratory disorders can coexist with any of the metabolic disorders. A standard deviation of 3 exists on each of these calculations; so if calculated and observed values are within the 3 numbers, call them “close enough”.
EXERCISE 1 Blood gas: pH-7.50/pCO2-20 Na = 140, Cl = 103, bicarbonate = 15. Step 1: First look at pH. It is 7.50, which tells our primary disorder is either a respiratory or metabolic alkalosis. Step 2a: Calculate the anion gap. 140 – (103 + 15) = 22. Because AG is more than 12, HAGMA is present. Step 2b: Calculate the ∆AG: AG – normal AG = 22 – 12 = 10 Step 3: Now look for metabolic disorders. Calculate the difference between expected and normal HCO3. Expected HCO3 = 24 − ∆AG = 24 – 10 = 14. Measured HCO3 is 15. This is close enough. No additional metabolic disorder is present.
Partha’s Management Algorithms in Pediatric and Adolescent Practice Step 4: Now look for respiratory disorders. Calculate the expected pCO2 and compare it to the actual. Expected = 15 + 15 = 30. Actual pCO2 is 20. There is less CO2 than you expect there to be, so a respiratory alkalosis is present. This is the primary disorder because Step 1 defines the primary disorder as an alkalemia. So the patient has a primary respiratory alkalosis + HAGMA. This scenario is seen with salicylate poisoning. Salicylates initially increase the respiratory drive causing respiratory alkalosis and then metabolic acidosis develops.
EXERCISE 2 Blood gas: pH-7.30/pCO2-40 Na = 145, Cl = 100, bicarbonate = 24 Step 1: First look at the pH. It is 7.30, which tells us our primary disorder is either a respiratory or metabolic acidosis. Step 2a: Calculate the anion gap. 145 – (100 + 24) = 21. Because AG is more than 12, HAGMA is present. Step 2b: Calculate the ∆AG: AG – normal AG = 21 – 12 = 9. Step 3: Now look for metabolic disorders. Calculate the difference between expected and normal HCO3. Expected HCO3 = 24 – 9 = 15. Measured HCO3 is 24. There is more bicarbonate than expected, so an additional metabolic alkalosis is present. Step 4: Now look for respiratory disorders. Calculate the expected pCO2 and compare it to the actual. Expected = 15 + 24 = 39. Actual pCO2 is 40. This is close enough. There are no additional respiratory disorders. Because the patient is acidemic, the patient has a primary HAGMA + metabolic alkalosis.
An approach to the most common metabolic disorder (metabolic acidosis) in clinical practice is given below. Infants and children with metabolic acidosis can present with varying degrees of acute and chronic illness that range from a seemingly healthy child to the one, a very sick child, needing pediatric intensive care unit (PICU) care. Though there are no distinguishing clinical features of pediatric metabolic acidosis, the symptoms are related to the underlying condition that produces it. In acute metabolic acidosis, the features are due to respiratory compensation—an increase in respiratory rate and in older children—increased depth of respiration (e.g. Kussmaul respirations), while in chronic metabolic acidosis, it is growth failure. The clues in history and physical examination are given in Box 8.1.2. An algorithmic approach to metabolic acidosis is given in Flowcharts 8.1.1 to 8.1.3. Box 8.1.2: Clues in history and physical examination. In history • Diarrhea: Bicarbonate loss/increased lactate due to hypovolemia • Diabetic ketoacidosis: Polyuria ± weight loss, abdominal pain, and vomiting • Chronic kidney disease (CKD)/renal tubular acidosis (RTA)—failure to thrive • Inborn errors of metabolism (IEM) (mitochondrial disorders and organic acidurias)—severe hypotonia and seizures • History of sudden and unexpected onset of symptoms— toxin ingestion. In physical examination • Growth impairment: RTA/CKD • Hypertension: Acute kidney injury (AKI)/CKD • Tachycardia, cool extremities, and low blood pressure— hypoperfusion leading to lactic acidosis.
Flowchart 8.1.1: Approach to metabolic acidosis.
* Anion gap (AG) = [serum (Na) – serum (Cl + HCO3)]. Normal AG = 12 mEq/L.
Pulmonology Flowchart 8.1.2: Approach to high-anion metabolic acidosis.
*Serum osmolal gap: Difference between the measured and calculated serum osmolality. Calculated serum osmolal gap = [2 × Na mmol/L] + [glucose (mg/dL)]/18 + [blood urea nitrogen (mg/dL)]/2.8. Normal osmolal gap is usually less than 10. (IEM, inborn errors of metabolism)
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 8.1.3: Approach to normal anion metabolic acidosis.
* Urine anion gap = urine (Na+ + K+ – Cl). A positive urine gap is less than 10. (RTA, renal tubular acidosis)
BIBLIOGRAPHY 1. Emmett M. The delta anion gap/delta HCO3 ratio in patients with a high anion gap metabolic acidosis. In: Sterns RH (Ed). Topic 2292 Version 11.0. UpToDate. [online] Available from https://www.uptodate.com/contents/the-delta-aniongap-delta-hco3-ratio-in-patients-with-a-high-anion-gapmetabolic-acidosis?source=search_result&search=the%20 delta%20anion%20gap%2Fdelta%20HCO3%20ratio%20 in&selectedTitle=1~150 [Accessed 23rd Sept., 2017]. 2. Kher K, Sharron M. Approach to the child with metabolic acidosis. In: Mattoo TK (Ed). Topic 6100 Version 24.0. UpToDate. [online] Available from https://www.uptodate. com/contents/approach-to-the-child-with-metabolicacidosis?source=search_result&search=approach%20 to%20child%20with%20metabolic%20acidosis&selected Title=1~150 [Accessed 23rd Sept., 2017]. 3. Shapiro BA, Harrison RA, Cane RD, et al. Clinical approach to interpretation of blood gases. In: Shapiro B (Ed). Clinical Application of Blood Gases, 4th edition. Chicago: Year Book Medical Publishers; 1989. pp. 77-8. 4. Subramanyam L, Vijayasekeran D, Balachandran A. Arterial blood gas analysis in clinical practice. Indian Pediatr. 2001;38(10):1116-28. 5. Subramanyam L, Vijayasekeran D, Somu N. Interpretation of blood gas analysis. In: Somu N, Subramanyam L (Eds). Essentials of Pediatric Pulmonology, 2nd edition. Chennai: Siva & Co.; 1996. pp. 169-79.
8.2 Management of Cough NC Gowrishankar, L Subramanyam, A Balachandran
INTRODUCTION There is no consensus as to the length of time in the definition of chronic cough in children. The American College of Chest Physicians (ACCP), Thoracic Society of Australia and New Zealand (TSANZ), and many studies have defined chronic cough as one that lasts more than 4 weeks, because most acute respiratory infections in children resolve within this interval. In comparison, guidelines from the British Thoracic Society (BTS) define chronic cough as the one that lasts more than 8 weeks. However, these guidelines also describe a “prolonged acute cough” as one that lasts at least 3 weeks and is “relentlessly progressive.”
EVALUATION OF COUGH (FLOWCHART 8.2.1) Age and Circumstances at Onset Onset of symptoms in early infancy and recurrent cough since birth suggests a congenital anomaly. Cough in a young infant, particularly under 6 months of age, is unusual and should be evaluated regardless of duration. While most of these infants will have an upper respiratory viral-induced cough and require no further work-up, a small number will have more serious problems such as cystic fibrosis (CF), gastroesophageal reflux with aspiration, cricopharyngeal
Pulmonology Flowchart 8.2.1: Evaluation of cough.
Abbreviations: UACS, upper airway cough syndrome; PNDS, postnasal discharge; PBB, protracted bacterial bronchitis; CSLD, chronic suppurative lung disease; HRAD, hyper-reactive airway disease; GERD, gastroesophageal reflux disease; URTI, upper respiratory tract infections; LRTI, lower respiratory tract infections; ILD, interstitial lung disease; FB, foreign body
incoordination, etc. In sudden onset of cough with respiratory distress or acute life-threatening event (ALTE) aspiration secondary to inhalation or ingestion has to be ruled out.
Nature of the Cough Chronic dry paroxysmal cough triggered by exercise, cold air, sleep or allergens is often seen in patients with asthma and typically worsens during sleep. In contrast, a chronic productive or “wet” cough suggests a suppurative process and may require further investigation to exclude bronchiectasis, CF, or immune deficiency. Staccato cough in infants can be the result of infection with Chlamydia trachomatis.
Timing and Triggers Cough due to postnasal drip is typically worse at night, immediately on lying down in bed, while cough due to bronchiectasis is worse and most productive early in the
day. Cough soon after eating, or which is worse while supine, may suggest gastroesophageal reflux. Psychogenic cough is typically worst and most disruptive during the day but disappears at night.
ASSOCIATED SYMPTOMS Family history of atopy suggests a diagnosis of either allergic rhinitis or asthma in a child with recurrent or chronic cough. Response to prior therapy may yield some diagnostic clues regarding the cause of cough. The less common disorders like recurrent aspiration, immunodeficiency, bronchiectasis (cystic fibrosis and ciliary dysfunction), cavitary lung disease (tuberculosis), congestive heart failure, and vascular lesions must be excluded if the cough is unusually severe and frequent or when there is exertional dyspnea, chest pain, and hemoptysis along with evidence of failure to thrive like growth retardation, clubbing, and hypoxemia. History of progressive dyspnea, tachypnea, exercise intolerance, and failure to thrive may suggest interstitial lung disease (ILD).
Partha’s Management Algorithms in Pediatric and Adolescent Practice If the history and examination provides clues suggesting a specific cause of cough (i.e. caused by an underlying disease), further evaluation is focused on that diagnostic possibility.
beyond 7–10 days, a bacterial etiology becomes more likely. Clinically differentiating these in the first week is difficult.
It is, sometimes, called persistent bacterial bronchitis and is increasingly recognized as a cause of chronic wet cough, particularly in young children (60 mEq/L) by quantitative pilocarpine iontophoresis method. •• Ciliary dyskinesia can be identified by saccharin clearance, a simple test to assess the ciliary beat frequency. The confirmatory test is by studying either the nasal brush biopsy or the scrapping of nasal mucosa under electron microscope. •• Echocardiography helps to rule out congenital heart disease. •• Hypersensitivity pneumonitis can be diagnosed by doing lung biopsy. These patients may show gradual improvement in hospital atmosphere and relapse on
returning to home when exposed to the offending antigens in the environment. The absence of the α-1 globulin band on serum protein electrophoresis will reveal probable α-1 antitrypsin deficiency. Further, genetic analysis for α-1 antitrypsin can be performed. •• Bronchoalveolar lavage or lung biopsy is performed when a child presents with anemia and recurrent pneumonia, which may show alveolar macrophages filled with hemosiderin pigments which is suggestive of pulmonary hemosiderosis.
DIFFUSE LUNG DISEASE Diffuse lung disease (DLD), traditionally known as interstitial lung disease (ILD), consists of a diverse group of disorders that involve the pulmonary parenchyma and interfere with gas exchange. Because many of these disorders are associated with extensive alteration of alveolar and airway architecture, in addition to changes in the interstitial compartment, the term DLD is more preferred than ILD. Lung biopsy is typically considered if above studies are nondiagnostic and if there is sufficient clinical urgency (e.g. severe or progressive disease and/or consideration for lung transplantation). Video-assisted thoracoscopy (VATS) is the procedure of choice for performing lung biopsy for this purpose.
BIBLIOGRAPHY 1. Balachandran A, Shivbalan So. Evaluation of recurrent/ persistent pneumonia in children. Indian J Pract Pediatr. 2002;4(1):30-7. 2. Barson WJ. (2017). Pneumonia in children: inpatient treatment. UpToDate. Topic 6054 Version 48.0. [online] Available from https://www.uptodate.com/contents/ pneumonia-in-children-inpatient-treatment?source=search_ result&search=slowly%20resolving%20pneumonia& selectedTitle=3~150. [Accessed Sept., 2017]. 3. Kelly MS, Sandora TJ. Community-acquired pneumonia. In: Kleigman RM, Stanton BF, St Geme JW, Schor NF, Behrman RE (Eds). Nelson Textbook of Pediatrics. First South Asia Edition. Gurugram, Haryana: Reed Elsevier India (P) Ltd; 2016. pp. 2088-94. 4. Ost D, Fein A, Feinsilver SH. (2017). Nonresolving pneumonia. UpToDate. Topic 6992 Version 14.0. [online] Available from https://www.uptodate.com/contents/nonresolvingpneumonia?source=search_result& search=persistent%20 pneumonia&selectedTitle=1~150. [Accessed Sept., 2017]. 5. Recurrent/persistent pneumonia in children. In: Subramanyam L, Shivbalan SO, Gowrishankar NC, Vijayasekeran D, Balachandran A (Eds). Essentials of Pediatric Pulmonology, 3rd Edition. Chennai: Pediatric Pulmonology Foundation of India; 2008. pp. 177-82.
8.4 Pulmonary Function Test—Spirometry NC Gowrishankar, L Subramanyam, A Balachandran
INTRODUCTION Pulmonary function tests (PFTs) can measure obstructive, restrictive, and diffusion defects and respiratory muscle function. Measurements of flow and volume are most useful in the office setting. In the office setting, spirometry is typically used to detect, confirm, and monitor obstructive airway disease: asthma. The major types of PFTs include spirometry, spirometry before and after bronchodilator, lung volumes and diffusing capacity of the lung for carbon monoxide. Other PFTs include measurements of maximal respiratory pressures and bronchoprovocation challenge, which are useful in specific clinical circumstances.
SPIROMETRY Spirometry is used to measure forced expiratory flow rates and volumes. Spirometry measures the volume of air exhaled at specific time points during a forceful and complete exhalation after a maximal inhalation. The total exhaled volume, known as the forced vital capacity (FVC), the volume exhaled in the first second, known as the forced expiratory volume in first second (FEV1), and their ratio (FEV1/FVC) is the most important variable reported. The test takes 10–15 minutes to perform. Spirometry is a key diagnostic test for asthma (when performed before and after bronchodilator) and is useful for evaluation of other causes of airflow obstruction and chronic cough. It is also used to monitor a broad spectrum of respiratory diseases not only asthma but also interstitial lung disease and neuromuscular diseases affecting respiratory muscles. The way to interpret spirometry values and to diagnose other respiratory diseases is given in Flowcharts 8.4.1 and 8.4.2. Forced expiratory volume in 1 second and FVC are the primary measurements obtained by spirometry. Their ratio (FEV1/FVC) is important for distinguishing obstructive airways disease and restrictive disease. A reduced ratio suggests obstructive airway disease. A reduced FVC in combination with a normal or increased ratio suggests restrictive disease, if accompanied by reduced lung volumes. The other important parameter derived from spirometry includes indices of flow, including a flow between 25%
and 75% of the vital capacity forced expiratory flow (FEF 25–75%), also known as the maximal midexpiratory flow rate (MEF 25-75) and peak expiratory flow rate (PEFR). Measurement of lung volumes complements spirometry. Common measurements include total lung capacity (TLC), functional residual capacity (FRC), and residual volume (RV). Decreased lung volumes if accompanied by a normal or increased FEV1/FVC ratio, suggest restrictive disease. Increased lung volumes suggest static hyperinflation due to obstructive airways disease if accompanied by decreased FEV1/FVC ratio. Coexisting restriction and obstruction can be identified when the TLC and FEV1/FVC ratio is reduced.
FLOW-VOLUME LOOPS The flow-volume loop is a plot of inspiratory and expiratory flow (on the Y-axis) against volume (on the X-axis) during the performance of maximal forced inspiratory and expiratory maneuvers. The contour of the flow-volume loop provides additional information about the location of airway obstruction, beyond that provided by the numeric values for FEV1 and FVC. Flow-volume loops with maximal inspiratory and expiratory data can identify upper airway obstruction, which can be undetectable with standard expiratory measurements. A characteristic limitation of flow (i.e. a plateau) during forced inhalation suggests variable extrathoracic obstruction, while limitation of flow during forced exhalation suggests variable intrathoracic obstruction. Fixed upper airway obstruction causes flow limitation during both forced inhalation and forced exhalation.
POST-BRONCHODILATOR Performance of spirometry before and after bronchodilator is used to determine the degree of reversibility of airflow limitation. Administration of salbutamol by nebulization is indicated if baseline spirometry demonstrates airway obstruction or if one suspects asthma. Spirometry should be repeated 10–15 minutes after administration of a bronchodilator. In a patient with airway obstruction, an increase in the FEV1 of more than 12% and/or greater than 0.2 L suggests acute bronchodilator responsiveness. In patients with asthma, bronchodilator administration often results in improvement.
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 8.4.1: Spirometry interpretation.
Abbreviations: FVC, forced vital capacity; FEV1, forced expiratory volume in 1 second; VC, vital capacity; ILD, interstitial lung disease; DLCO, diffusing capacity of the lung for carbon monoxide
DIFFUSING CAPACITY FOR CARBON MONOXIDE
Flowchart 8.4.2: Spirometry normal evaluation.
Diffusing capacity of the lung for carbon monoxide (DLCO) is decreased by anything that interrupts gas-blood O2 exchange. Decrease in DLCO implies a loss of effective capillary-alveolus interface. It is usually due to loss of alveolar-capillary units. Measurement of DLCO assesses gas exchange. A decreased DLCO accompanied by restrictive disease suggests intrinsic lung disease, whereas a normal DLCO accompanied by restrictive disease suggests a nonpulmonary cause of restriction. A markedly decreased DLCO accompanied by obstructive airways disease suggests emphysema. DLCO is useful for differentiating emphysema from asthma in a patient with spirometric evidence of airflow obstruction.
MAXIMAL RESPIRATORY PRESSURES Maximal inspiratory and expiratory pressures are easily measured using a simple mechanical pressure gauge
connected to a mouthpiece. Maximal inspiratory pressure (MIP) measures the ability of the diaphragm and the other respiratory muscles to generate inspiratory force, reflected by a negative airway pressure.
Pulmonology Measurement of maximal inspiratory and expiratory pressures is indicated whenever there is an unexplained decrease in vital capacity or when respiratory muscle weakness is suspected clinically. Maximal inspiratory pressure, measured at RV is the maximal pressure that can be produced by the patient trying to inhale through a blocked mouthpiece after a full exhalation. Maximal expiratory pressure (MEP) is the maximal pressure measured during forced expiration (with cheeks bulging) through a blocked mouthpiece after a full inhalation (TLC). Repeated measurements of MIP and MEP are useful in following the course of patients with neuromuscular disorders.
BRONCHOPROVOCATION CHALLENGE Spirometry is used to assess the airway hyperresponsiveness to a variety of bronchoprovocation challenges, such as methacholine, histamine, and mannitol and isocapnic hyperpnea. Nonspecific bronchoprovocation testing is indicated when the diagnosis of asthma is in question (e.g. symptoms are atypical and baseline spirometry is normal). Methacholine inhalation challenge is the most common type of pharmacologic challenge.
CONCLUSION Spirometry is the commonly performed PFT in children above the age of 5 years and is often used to establish a diagnosis of asthma which requires a history of episodic symptoms of airflow obstruction or bronchial
hyperresponsiveness and demonstration that airflow obstruction is reversible and exclusion of alternate diagnoses.
BIBLIOGRAPHY 1. Aboussouan LS, Stoller JK. (2017). Flow-volume loops. UpToDate. [online] Available from: https://www.uptodate. com/contents/flow-volume-loops?source=search_result& search=flow%20volume%20loops&selectedTitle=1~48. [Accessed Sept., 2017]. 2. Haddad GG, Green TP. Diagnostic approach to respiratory diseases. In: Kleigman RM, Stanton BF, St. Geme JW, Schor NF, Behrman RE (Eds). Nelson Textbook of Pediatrics, First South Asia Edition. Gurgaon: Reed Elsevier India (P) Ltd; 2016. pp. 1993-8. 3. McCormack MC. (2017). Overview of pulmonary function testing in adults. UpToDate: Topic 6969 Version 17.0. [online] Available from: https://www.uptodate.com/ contents/overview-of-pulmonary-function-testingin-adults?source=search_result&search=pft&selected title=1~150. [Accessed Sept., 2017]. 4. Rosen DM, Colin AA. (2017). Overview of pulmonary function testing in children. In: Redding G (Ed). UpToDate. Topic 5735 Version 9.0. [online] Available from https://www. uptodate.com/contents/overview-of-pulmonary-functiontesting-in-children?source=search_result&search=PFT&se lectedTitle=2~150. [Accessed Sept., 2017]. 5. Shivbalan SO, Gowrishankar NC, Balachandran A. Spirometry. In: Subramanyam L, Shivbalan So, Gowrishankar NC, Vijayasekeran D, Balachandran A (Eds). Essentials of Pediatric Pulmonology, 3rd Edition. Chennai: Pediatric Pulmonology Foundation of India; 2008. pp. 387-93.
Gastroenterology and Hepatology Contributors: Malathi Sathiyasekaran, Sumathi Bavanandam Reviewer: Anupama S Borker
¯¯ Neonatal Cholestasis Syndrome ¯¯ Constipation in Children ¯¯ Upper Gastrointestinal Bleeding in Adolescents ¯¯ Isolated Hepatomegaly in Adolescents
Partha’s Management Algorithms in Pediatric and Adolescent Practice
9.1 Neonatal Cholestasis Syndrome Malathi Sathiyasekaran, Sumathi Bavanandam
INTRODUCTION Neonatal cholestasis is the impaired formation or flow of bile resulting in the retention within the liver of biliary substances normally excreted into the intestine through bile and occurring in neonates and young infants. Neonatal cholestasis syndrome (NCS) is a heterogeneous group of hepatobiliary disorders presenting usually 2 weeks after birth with jaundice, high-colored urine and pale yellow stools with direct bilirubin more than 1 mg/dL or more than 20% of total. The two main subsets of NCS are—(1) Hepatocellular (53%) which includes infections (neonatal hepatitis), metabolic diseases, chromosomal, endocrine, and genetic bile salt transport defects, and (2) Obstructive (38%) in which biliary atresia (BA) and choledochal cyst are the two main entities. Early detection and referral by the pediatrician to the pediatric gastroenterologist and surgeon is imperative for definitive diagnosis and effective therapy of all conditions in this assorted group of disorders which share common clinical and biochemical features. The management of NCS includes both specific and supportive therapy. A well-structured and informative algorithm
dividing NCS into three clinical groups will facilitate early diagnosis and effective therapeutic interventions (Flowchart 9.1.1).
SPECIFIC THERAPY Group A: Neonatal Cholestasis Syndrome: Sick with Pigmented Stool (Fig. 9.1.1) Some of the important causes of this group are galactosemia, tyrosinemia, neonatal hemochromatosis (NH), hemophagolymphohistiocytosis (HLH), mitochondrial hepatopathy (MH), and severe cytomegalovirus infection (CMV). An algorithmic approach for this group will help in diagnosis and assist in definitive therapy (Flowchart 9.1.2). •• Galactosemia –– Galactosemia is an autosomal recessive disorder of galactose metabolism due to the absence of enzyme galactose-1-phosphate uridyl transferase. –– Lactose present in breast milk, animal milk, and all formula feeds is converted into glucose and galactose in the intestine and, therefore, should
Flowchart 9.1.1: Algorithm for approach to neonatal cholestasis syndrome.
Abbreviations: GGTP, gamma-glutamyl transpeptidase; ALGS, Alagille syndrome; HLH, hemophago-lymphohistiocytosis; GB, gallbladder; US, ultrasound; CBC, complete blood count; LFT, liver function test; PT, prothrombin time
Gastroenterology and Hepatology be avoided. Nonlactose soy-based formulae are recommended for these infants. –– Life-long restriction of milk and milk products is essential. Restriction of galactose-containing fruits and vegetables, at present, is not recommended. –– Long-term prognosis is good on a lactose-restricted diet; however, 25–50% may have speech apraxia, language impairment, and ovarian failure due to endogenous production of galactose. •• Congenital Cytomegalovirus (CCMV) Infection –– No treatment is recommended for asymptomatic congenital cytomegalovirus (CCMV) defined as positive CMV in any secretion with normal clinical, biochemical, and imaging evaluation.
Fig. 9.1.1: Pigmented yellow stool .
–– Neonates with life-threatening infections, central nervous system involvement (clinical or imaging), disseminated infection CCMV infection [hepatosplenomegaly, hepatitis, conjugated hyperbilirubinemia, anemia, and asymmetric intrauterine growth restriction (IUGR)] with high viral load, thrombocytopenia, or petechiae need to be treated with intravenous (IV) ganciclovir 6 mg/ kg twice a day or oral valganciclovir 15 mg/kg twice a day for 6 weeks. •• Neonatal Hemochromatosis –– Neonatal hemochromatosis is a condition where there is severe liver dysfunction accompanied by extrahepatic siderosis due to gestation alloimmune liver disease. –– Intravenous immunoglobulin G (IVIG) with exchange transfusion is recommended for NH. –– Liver transplantation is the only option where medical treatment has failed; however, it is a big challenge in view of multiorgan failure. –– Prevention of NH in subsequent pregnancy can be achieved by treating the mother with IVIG 1 g/kg (max 60 g) from 14th week initially once in 2 weeks then weekly from 18th week till end of gestation. •• Tyrosinemia –– Tyrosinemia is an inborn error of tyrosine metabo lism due to defect in the enzyme fumarylacetoacetate hydrolase. –– Treatment is by restricting tyrosine and phenyl alanine in the diet. Nitisinone (NTBC) 1–2 mg/kg/ day prevents tyrosine degradation. Survival is 85%, if the treatment is started early.
Flowchart 9.1.2: Algorithm for approach to sick infant with pigmented stools.
Abbreviations: GAL-1-PUT, galactose-1-phosphate uridyl transferase; ALF, acute liver failure; NCS, neonatal cholestasis syndrome; CCMV, congenital cytomegalovirus
Partha’s Management Algorithms in Pediatric and Adolescent Practice –– Liver transplantation in failed NTBC therapy. –– There are two more conditions in this group A of a sick child with NCS. •• Hemophagolymphohistiocytosis –– Hemophago-lymphohistiocytosis is a paradigm for failed communication between innate and adaptive immunity with overstimulation of T and natural killer (NK) lymphocytes and inability of the immune system to control and terminate the immune response. –– Treatment is with intravenous steroids, etoposide, cyclosporine, and immunoglobulins. –– Liver transplantation is not rewarding. Hematopoietic stem cell transplant (HSCT) may be the definitive treatment for familial HLH. •• Mitochondrial Hepatopathy –– Mitochondrial hepatopathy occurs due to mutations in various mitochondrial deoxyribonucleic acid (DNA) results in enzyme deficiencies that fail to generate energy. –– Liver, brain, and heart are the three most important organs affected. –– Treatment of MH is supportive, liver transplantation is not recommended in MH.
•• A simple algorithm for approach to this group is shown in Flowchart 9.1.3.
Group C: Neonatal Cholestasis Syndrome: Not Sick (Fig. 9.1.3) Acholic (White Stool) (Fig. 9.1.4) The most important cause of NCS in this group is BA (Flowchart 9.1.1).
Biliary Atresia •• Biliary atresia is a progressive, obliterative, neonatal disorder of intra- and extrahepatic bile ducts leading to hepatic fibrosis and end-stage liver disease. •• Age is a modifiable and improvable predicting factor for outcome of Kasai (Flowchart 9.1.4). •• All infants with failed Kasai require liver transplant. •• Native liver survival for more than 10 years has been documented in 50% of those with successful Kasai.
Group B: Neonatal Cholestasis Syndrome: Not Sick, Pigmented Stool or Pale Yellow (Fig. 9.1.2) •• Some important conditions in this group are progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (ALGS), where a predominant symptom is itching. •• Gamma-glutamyl transpeptidase (GGTP), a cholestatic enzyme, usually increases in parallel with alkaline phosphatase.
Fig. 9.1.2: Pale yellow stool.
Flowchart 9.1.3: Algorithm for an infant who is not sick but with pale or pigmented stools.
Abbreviation: PFIC, progressive familial intrahepatic cholestasis.
Gastroenterology and Hepatology
Fig. 9.1.3: Well child with neonatal cholestasis syndrome (NCS): Biliary atresia.
Fig. 9.1.4: Chalky white stool seen in biliary atresia.
Flowchart 9.1.4: Algorithm showing a relation between outcome and day of surgery in biliary atresia (BA).
Flowchart 9.1.5: Algorithm for supportive treatment of neonatal cholestasis syndrome.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
SUPPORTIVE THERAPY The supportive treatment comprising adequate nutritional support (diet, vitamins, and minerals) to ensure proper
growth and development, and therapy for pruritus, should be offered to all infants with NCS as shown in Flowchart 9.1.5.
9.2 Constipation in Children Malathi Sathiyasekaran, Sumathi Bavanandam
INTRODUCTION Constipation is a common problem (3–5%) in children with functional constipation (FC) being most frequent (85%). Constipation is defined as “delay ( 20,000 IU/L), chronic HBeAg negative hepatitis (HBV DNA > 2000 IU/L), and inactive HBsAg carrier. •• The treatment recommended for adolescents with CHB is PEG interferon with antivirals entecavir or tenofovir. •• Entecavir 0.5 mg once a day and tenofovir 300 mg once a day. •• Treatment is for life-long or till clearance of HBsAg and seroconversion to anti-HBs positivity.
Chronic Hepatitis B and Hepatitis C Chronic hepatitis B (CHB) and chronic hepatitis C (CHC) may be unusual causes of isolated firm hepatomegaly in adolescents. Both CHB and CHC can progress to cirrhosis and hepatocellular carcinoma.
Chronic Hepatitis C •• It is preferable to treat all adolescents with chronic HCV infection. •• The new direct acting antivirals (DAA) are very safe and effective in adolescents. •• The regimens are noninterferon based using ribavirin + DAAs (sofosbuvir and daclatasvir or ledipasvir). •• The sustained viral response at 12 weeks (SVR12) reaches 95%. •• The duration of treatment in naive noncirrhotic patients is 12 weeks. •• Chronic hepatitis C can be cured with this therapy.
Space-occupying Lesions •• Space-occupying lesion is a rare cause of isolated firm or lobulated hepatomegaly in adolescents.
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Space-occupying lesion may include benign vascular lesions (hemangioma and hemangioendothelioma), liver abscess, adenoma, focal nodular hyperplasia, nodular regenerative hyperplasia, simple liver cysts, biliary cyst, hamartomas, hydatid cyst, and malignant lesions. •• Imaging like ultrasonography (USG) abdomen with Doppler, magnetic resonance (MR) imaging will help in diagnosis. •• Management: Asymptomatic benign simple hepatic cysts lesions need regular follow-up. •• For hydatid cyst, drugs like albendazole, thiabendazole, saline aspiration, and rarely surgery, are some of the treatment modalities available. •• Antibiotics are indicated for abscess.
BIBLIOGRAPHY 1. Bhaskar Raju B, Sumathi B. Constipation in children. Indian J Pract Pediatr. 2008;10(3):201-7. 2. Bhatia V, Bavdekar A, Matthai J, et al. Management of neonatal cholestasis: consensus statement of the Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics. Indian Pediatr. 2014;51(3):203-10.
3. Fawaz R, Baumann U, Ekong U, et al. Guideline for the evaluation of cholestatic jaundice in infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2017;64(1):154-68. 4. Khanna V, Poddar U, Yachha SK. Constipation in Indian children: need for knowledge not the knife. Indian Pediatr. 2010;47:1025-30. 5. Mieli-Vergani G, Heller S, Jara P, et al. Autoimmune hepatitis. J Pediatr Gastroenterol Nutr. 2009;49(2):158-64. 6. Neidich GA, Cole SR. Gastrointestinal bleeding. Pediatr Rev. 2014;35(6):243. 7. Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson Disease: an update. AASLD practice guidelines. Hepatology. 2008;47(6):2089-111. 8. Sokal EM, Paganelli M, Wirth S, et al. Management of chronic hepatitis B in childhood: ESPGHAN clinical practice guidelines. Consensus of an expert panel on behalf of the ESPGHAN. J Hepatol. 2013;59(4):814-29. 9. Thyagarajan MS, Sharif K. Space-occupying lesions in the Liver. Indian J Pediatr. 2016;83(11):1291-302.
Nephrology Contributors: Pankaj Deshpande, G Sangeetha Reviewer: Anupama S Borker
¯¯ Renal Tubular Acidosis ¯¯ Infection-related Glomerulonephritis ¯¯ Approach to Hypertension in Children and Adolescents ¯¯ Approach to Recurrent Urinary Tract Infection in Children
Partha’s Management Algorithms in Pediatric and Adolescent Practice
10.1 Renal Tubular Acidosis Pankaj Deshpande
INTRODUCTION The steps for renal tubular acidosis are shown in Flowchart 10.1.1.
MANAGEMENT GUIDELINES Renal tubular acidosis (RTA) is one of the common causes of failure to thrive in children. Lack of adequate growth according to the growth centiles should prompt one to think about RTA. However, considering the number of children
in a pediatric outpatient department who show failure to thrive, it may be difficult to do tests for RTA in all of them. Certain symptoms may help in aiding to decide regarding investigations for RTA. Presence of polyuria and polydipsia is one such symptom. It is usually not a volunteered information and may be difficult for first time parents to note. A child waking at night for water, more interested in water rather than food, excessive volumes of urine, severe constipation, and an irritable child may all indicate that investigations for RTA are required.
Flowchart 10.1.1: Renal tubular acidosis.
Abbreviation: RTA, renal tubular acidosis
Nephrology Unusual presentations should also be kept in mind. Tachypnea in babies with no respiratory signs or X-ray chest changes or children who do not show improvement in rickets despite adequate dosing of vitamin D need investigations also. A blood gas along with complete blood count (CBC), electrolytes, creatinine, and urine pH and urine routine form the basic tests for diagnosis. Strictly speaking, in RTA, the excretory function of the kidneys is preserved. Hence, the serum creatinine is normal. However, in certain conditions like cystinosis, gradually the creatinine will increase as the renal function declines. However, a child presenting with an elevated creatinine and metabolic acidosis is not considered as having RTA. In that case, the acidosis is secondary to renal dysfunction. Using the modified Schwartz formula, one should determine that the serum creatinine is normal for the height of the child. While acidosis is fairly self-evident, simple tests that help us are often ignored. The serum chloride level is one such test. While every child gets serum chlorides done along with sodium and potassium, not much attention is paid to it. In fact, a high chloride may point to a diagnosis of RTA even before the blood gas is done. In children admitted for other reasons, it may be a useful tool to pick up underlying RTA by then doing further investigations. Metabolic acidosis is a constant feature of RTA. While there are children who may have only latent acidosis that becomes evident only after ammonium chloride loading test, in practical terms, let us consider metabolic acidosis to be the cornerstone for diagnosing RTA. On the blood gas, pH value less than 7.25 and bicarbonate values less than 16 would usually be seen in metabolic acidosis. The usual and common types of RTA (type I and II) usually also show a low potassium. The anion gap (difference of positive charged ions and negatively charged ions): Sodium + Potassium – (Bicarbonate + Chloride) is normal (normal range being 5–15). This differentiates it from other causes like organic acidemias, uremia, and diabetic ketoacidosis where the anion gap is high. The presence of all four features: (1) metabolic acidosis, (2) hyperchloremia, (3) hypokalemia, and (4) normal anion gap are usually seen in all patients with RTA. However, even with the presence of all these features, conditions like celiac disease should be ruled out as children presenting with celiac crisis may exhibit such features. Features like edema, deficiencies of water-soluble vitamins, and persistent diarrhea, may warrant investigations along the line of celiac disease. As alluded to earlier, children with RTA often have polydipsia, polyuria, and severe constipation. Acidosis
makes them irritable and the difference is noted by parents on treatment. Elevated levels of renin and aldosterone are found in these patients secondary to fluid depletion. Distinguishing between the types of RTA is important from the prognosis point of view. However, the treatment is more important and should be started soon in patients with RTA. In distal RTA, the loss of bicarbonate is from the distal tubule and hence only 15% of the bicarbonate is lost. Hence, the requirement of bicarbonate is also lower. They also have hypercalciuria and hence nephrocalcinosis is a common finding in such patients. In proximal RTA, the loss of bicarbonate is from the proximal tubule where 85% of the bicarbonate is reabsorbed. Hence, the requirement of bicarbonate is larger. They may also have associated loss of phosphorus, sugar, and amino acids in the urine leading to Fanconi syndrome. Partial Fanconi syndrome would indicate loss of only some elements rather than all of them (Box 10.1.1). There is always a lot of confusion regarding the urine pH and the values. The differentiating factor is urine pH at the time of severe acidosis. Hence, a urine pH done after treatment of a child is pointless. In the presence of severe acidosis also, the urine pH will be high (> 5.5) in distal RTA as the acidifying mechanisms are lost. On the contrary, in proximal RTA, in severe acidosis, once the bicarbonate threshold is reached, bicarbonate loss in the urine stops. The distal tubule then can acidify the urine and hence the urine pH becomes acidic (< 5.5). So remember the urine pH has to be done in severe acidosis to be able to help to differentiate between the two conditions. Distal RTA can be associated with deafness. So a hearing test should be done in children with distal RTA. Both types of RTA will need treatment with bicarbonate supplements as well as potassium supplements. Initially, bicarbonate can be started as sodamint tablets (in recent times, liquid formulation is also available) in the dose of at least 3–5 mEq/kg per day in 3–4 divided doses. The dose will need titration based on the blood gas results. The blood tests can be done weekly till the required dose is established. As described earlier, the requirement is lower in distal RTA and Box 10.1.1: Common causes of Fanconi syndrome. • • • • • • •
Cystinosis Galactosemia Glycogen storage disease type I Tyrosinemia Wilson’s disease Lowe’s syndrome Heavy metals
Partha’s Management Algorithms in Pediatric and Adolescent Practice often doses from 5 mEq/kg/day to 8 mEq/kg/day are seen though occasional cases may need large doses. In proximal RTA, the doses required are large and it is not uncommon to see doses required being as high as 15–20 mEq/kg/day. Potassium supplements in liquid formulation or tablets can be given. Usual medications used are syrup Kesol or syrup Potklor. The initial starting dose should be 2 mEq/ kg/day in two divided doses and then the dose is adjusted as per the blood tests. Once the doses are established, the child can be seen every 3 months with the requisite blood tests and for growth monitoring. It is imperative to explain to the parents that it is a treatment that continues for many-many years (at least till 18 years of age) and then reviewed. It is also important to let them know that they will need a normal life as long as they take their medications! Distal RTA patients may develop renal calculi and one should be alert to diagnosing such a state if it happens. Proximal RTA can be associated with Fanconi syndrome. The underlying condition will then need to be treated and the prognosis will depend on the underlying condition. For example, cystinosis carries a grave prognosis despite providing all treatment.
Apart from type I and type II RTA, type IV RTA has also been described where the features remain the same as mentioned in the earlier types but they show a normal or high potassium. This is either secondary to absent aldosterone which is rare or commonly due to dysplastic tubules showing resistance to aldosterone action. In such a scenario, the maturation of the kidneys in the first year of life may help to alleviate the acidosis as well as the electrolyte abnormalities. Hence, the child may be off all the medications by 1 year of age.
BIBLIOGRAPHY 1. Bagga A, Bajpai A, Menon S. Approach to renal tubular disorders. Indian J Pediatr. 2005;72(9):771-6. 2. Bagga A, Sinha A. Evaluation of renal tubular acidosis. Indian J Pediatr. 2007;74(7):679-86. 3. Herrin TJ. Renal tubular acidosis. In: Avner ED, Harmon WE, Niaudet P (Eds). Pediatric Nephrology, 5th edition. Baltimore: Lippincott Williams & Wilkins; 2004. pp. 757-76. 4. McSherry E. Renal tubular acidosis in childhood. Kidney Int. 1981;20(6):799-809. 5. Yaxley J, Pirrone C. Review of the diagnostic evaluation of renal tubular acidosis. Ochsner J. 2016;16(4):525-30.
10.2 Infection-related Glomerulonephritis Pankaj Deshpande
INTRODUCTION The procedure for infection-related glomerulonephritis are shown in Flowchart 10.2.1.
MANAGEMENT GUIDELINES Postinfectious glomerulonephritis is a fairly common condition and presentation can be varied. There may be mild edema or tea- or cola-colored urine. Some children may present with hypertensive encephalopathy or seizures. Once glomerulonephritis is suspected, blood pressure measurement with an appropriate-sized cuff is absolutely vital. Blood pressure (BP) greater than the 95th centile or systolic BP greater than 100 + 2 times age indicates hypertension. If there is severe hypertension (30% > 95th centile) or encephalopathy or seizures, BP should be reduced gradually with the help of sodium nitroprusside or labetalol infusion.
In other cases, diuretics may help to reduce blood pressure. If not sufficient, antihypertensive medications should be added. Calcium-channel blockers like amlodipine (to begin at 0.1 mg/kg once a day and dose increased as required) or β-adrenergic blockers like atenolol (1–2 mg/ kg/per day) can be used. Angiotensin-converting enzyme (ACE) inhibitors like enalapril should not be used, unless the blood pressure is not controlled on other agents at all. The creatinine is usually raised in glomerulonephritis and ACE inhibitors would cause a rise in creatinine further. Rarely the child may present in cardiac output failure secondary to severe hypertension and this may become evident only after the initial treatment for cardiac failure is carried out. A child with such a scenario (infection-related glomerulonephritis) should not receive intravenous fluids. The pathology is related to volume overload and adding more fluid would worsen the clinical condition.
Nephrology Flowchart 10.2.1: Algorithm for infection-related glomerulonephritis.
Abbreviations: BP, blood pressure; BUN, blood urea nitrogen; CBC, complete blood count; US, ultrasound
Checking of serum creatinine and electrolytes would help to determine the renal function. If the creatinine is very high along with a high blood urea nitrogen (BUN) and the child has severe oligoanuria which does not respond to diuretics, dialysis would be required. It is rare but can occur. Less than 2% of children will present with such severe nephritis. Use of the modified Schwartz formula given below will help to determine the degree of renal dysfunction. Estimated glomerular filtration rate (eGFR) = k × height of the child (cm)/serum creatinine (mg/dL), k = 0.55 (for Indian children 0.45 has also been advocated).
eGFR should be normally greater than 80 mL/min. In most children, some degree of hyperkalemia and raised creatinine may be noted. Use of diuretics would help in controlling the volume overload as well as reducing the potassium. Usually frusemide in the dose of 1 mg/kg/dose twice a day will help. Complete blood count (CBC) will help to rule out other conditions like hemolytic uremic syndrome. Presence of hematuria should be established on a urine routine examination. Presence of more than 5 red blood cells (RBCs)/high power field (HPF) confirms hematuria.
Partha’s Management Algorithms in Pediatric and Adolescent Practice In some cases, this may not be seen. This is usually because to detect the RBCs, the urine sample needs to be fresh and spun immediately. Delay in examination may cause the RBCs to lyse leaving a bland urine. A low C3 at presentation is indicative of postinfectious nephritis and is a must in all such patients. A normal C3 at presentation makes the diagnosis of postinfectious nephritis very unlikely and other conditions causing nephritis should be kept in mind (Boxes 10.2.1 and 10.2.2). An ultrasound scan of the kidneys should be done in all such cases to ensure that we are not missing other conditions. The belief that postinfectious nephritis occurs about 2 weeks after a respiratory infection no longer holds true. It has been shown it can occur in a few days of the infection. Box 10.2.1: Acute glomerulonephritis (AGN) with low C3. • Systemic diseases: –– Lupus nephritis –– Subacute bacterial endocarditis (SBE), shunt nephritis, and cryoglobulinemia • Renal diseases: –– Acute postinfectious nephritis (>90%) –– Membranoproliferative glomerulonephritis (MPGN)
Box 10.2.2: Acute glomerulonephritis (AGN) with normal C3. • Systemic diseases: –– Polyarteritis nodosa and hypersensitivity vasculitis –– Wegener’s granulomatosis –– Henoch–Schönlein purpura (HSP) and Goodpasture’s syndrome • Renal diseases: –– Immunoglobulin A (IgA) nephropathy –– Idiopathic rapidly progressive glomerulonephritis (RPGN) –– Anti-glomerular basement membrane (anti-GBM) disease
A wide variety of agents are considered plausible agents of postinfectious nephritis though Streptococcus remains the commonly considered organism. In postinfectious nephritis, the gross hematuria and the renal function recover to normal within 1–2 weeks. As it is a self-limiting condition, hypertension also usually resolves in 3–4 weeks. Heavy proteinuria may be seen in the first couple of weeks. In some cases, it may take 4–6 weeks to resolve. C3 level should be repeated after 12 weeks to confirm that it has returned to normal. All the parameters including serum creatinine, blood pressure, and a urine protein/ creatinine ratio should have settled to normal by then. If so, it confirms postinfectious nephritis and usually carries a good prognosis. A kidney biopsy is indicated: •• Severe presentation needing dialysis •• Proteinuria unresolved at 10–12 weeks •• C3 not returning to normal after 12 weeks.
BIBLIOGRAPHY 1. Clark G, White RH, Glasgow EF, et al. Poststreptococcal glomerulonephritis in children: clinicopathological correlations and long-term prognosis. Pediatr Nephrol. 1988;2(4):381-8. 2. Heptinstall RH. Pathology of the Kidney, 3rd edition. Boston: Little, Brown and Co.; 1983. pp. 908-31. 3. Lange K, Seligson G, Cronin W. Evidence for the in situ origin of poststreptococcal glomerulonephritis: glomerular localization of endostreptosin and the clinical significance of the subsequent antibody response. Clin Nephrol. 1983;19(1):3-10. 4. Nordstrand A, Norgren M, Holm SE. Pathogenic mechanism of acute post-streptococcal glomerulonephritis. Scand J Infect Dis. 1999;31(6):523-37. 5. Vogl W, Renke M, Mayer-Eichberger D, et al. Longterm prognosis for endocapillary glomerulonephritis of poststreptococcal type in children and adults. Nephron. 1986;44(1):58-65.
10.3 Approach to Hypertension in Children and Adolescents G Sangeetha
INTRODUCTION Hypertension in children and adolescents is becoming a significant global health problem. Though secondary hypertension is common in younger age group, primary hypertension is showing increasing trend due to an epidemic of obesity in the younger generation. Blood pressure values are given based on gender, age, and height and provided as 50th, 90th, 95th, and 99th percentile in children less than 1 year of age (Figs 10.3.1A and B). BP percentile chart for children more than 1 year has been shown in Appendix. Hypertension is defined as average systolic or diastolic blood pressure that is greater than or equal to 95th percentile for gender, age, and height on three or more separate occasions.
STAGES AND CAUSES OF HYPERTENSION Hypertension stages are explained in Table 10.3.1.
Causes of hypertension are explained in Table 10.3.2. An approach to hypertension is explained in Flowchart 10.3.1.
MANAGEMENT OF HYPERTENSION IN CHILDREN AND ADOLESCENTS Goals of Hypertension Therapy The goal of treatment is to reduce the blood pressure (BP) to less than 95th percentiles. In children with secondary hypertension, it is always desirable to keep below 90th percentile. As per Kidney Disease Improving Global Outcomes (KDIGO) guidelines, BP in children with chronic kidney disease has to be maintained less than or equal to 50th percentile unless until the target is limited by signs or symptoms of hypotension.
Figs 10.3.1A and B: (A) Age-specific percentile of blood pressure (BP) measurements in boys—birth to 12 months of age; Korotkoff phase (IV) (K4) used for diastolic BP; (B) Age-specific percentile of blood pressure (BP) measurements in girls—birth to 12 months of age; Korotkoff phase (IV) (K4) used for diastolic BP.
Partha’s Management Algorithms in Pediatric and Adolescent Practice TABLE 10.3.1: Stages of hypertension.
TABLE 10.3.2: Common causes of hypertension in children.
Normal blood pressure (BP)
BP < 90th percentile or < 120/80 mm Hg for an adolescent
BP between 90th and 95th percentile or 120/80 mm Hg to < 95th percentile for an adolescent
Stage 1: Hypertension
95th percentile + 5 mm Hg
•• Thrombosis of renal artery or vein •• Autosomal recessive polycystic kidney disease •• Coarctation of aorta •• Bronchopulmonary dysplasia
Stage 2: Hypertension
99th percentile + 5 mm Hg
Stage 2A: Hypertensive urgency
Stage 2: Hypertension without end-organ damage
•• Renal parenchymal disease (acute glomerulonephritis, hemolytic-uremic syndrome, and reflux nephropathy) •• Renovascular disease •• Coarctation of aorta •• Neuroendocrine tumors
Stage 2B: Hypertensive emergency
Stage 2: Hypertension with end-organ damage like left ventricular failure, hypertensive encephalopathy, renal failure, pulmonary edema, and hypertensive retinopathy
•• •• •• •• ••
White coat hypertension
Child with BP levels above the 95th percentile in a physician’s office who is normotensive outside the clinic
Renal parenchymal disease Renovascular disease Endocrine causes of hypertension Primary hypertension Drug-induced/substance abuse (corticosteroids, calcineurin inhibitors, erythropoietin, and cocaine)
Child with BP levels above the 95th percentile outside the physician’s office who shows normal BP measurement in the clinic.
Transient hypertension at any age group
•• •• •• ••
Increased intracranial pressure Infection-related glomerulonephritis Acute tubular necrosis Autonomic dysfunction
Asymptomatic Hypertension in Children Try to bring down the BP to less than 95th percentile for gender, age, and height with therapeutic lifestyle changes (TLC) such as: •• Salt-restricted diet (2–3 g salt/day) •• Regular meals with healthy breakfast •• Ensure intake of fresh fruits and vegetables in the diet. Avoid calorie-dense snacks, fatty dairy products, smoking, and drug abuse •• Weight reduction to maintain the BMI less than 85th percentile. With the weight reduction of 5 kg, expected fall in BP will be 4.4 mm Hg systolic and 3.6 mm Hg diastolic, respectively •• Moderate intensity dynamic activity for 30 min/day for 5–7 days/week and reduction of sedentary activity.
Indications for Pharmacotherapy in Children with Hypertension •• Hypertension with end-organ damage •• Children with symptomatic hypertension •• Hypertension due to secondary causes
•• Comorbid conditions like diabetes mellitus and kidney diseases •• Failure to respond to nonpharmacological measure.
Pharmacotherapy •• It should be initiated with single drug when indicated. •• Gradually increase the dose of the drug till its maximum dose and then add the additive drug. •• Identify comorbid conditions such as kidney disorders, diabetes, heart disease, and other metabolic disorders. •• Monitor for adverse effects associated with specific antihypertensive agents. •• Improve adherence by giving drugs with longer halflives which permit reduction in dosing from twice daily to once daily. •• Make sure the gradual reduction of BP in hypertensive emergency. Reduce mean arterial pressure by 25% within the first 8 hours and then gradually normalize the BP from 90th centile to 95th centile in the next 24–48 hours so that the cerebral autoregulation will be maintained properly. Classification of hypertension is shown in Flowchart 10.3.2.
Nephrology Flowchart 10.3.1: Approach to hypertension
Abbreviations: UTI, urinary tract infection; BMI, body mass index; ECG, electrocardiogram; VUR, vesicoureteral reflux; DMSA, dimercaptosuccinic acid; VCUG, voiding cystourethrogram; CT, computed tomography; TSH, thyroid-stimulating hormone; MRI, magnetic resonance imaging; ANA, antinuclear antibodies; ANCA, antineutrophil cytoplasmic antibodies; PTH, parathyroid hormone; ACTH, adrenocorticotropic hormone; GBM, glomerular basement membrane; MIBG, metaiodobenzylguanidine
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 10.3.2: Stages of hypertension
TABLE 10.3.3: Different types of drugs and their antihypertensives and dosages. Drug
Calcium-channel blockers Amlodipine
0.06–0.2 mg/kg/day (maximum adult dose 20 mg/day)
Initial: 0.25 mg/kg/dose q6 to 8 hourly Maximum: 0.5 mg/kg/dose q6 to 8 hourly (maximum adult dose 20 mg q6 to 8 hourly)
Initial: 0.25–0.5 mg/kg/day q12 hourly (maximum adult dose 120 mg/day)
Initial: 0.7–1.4 mg/kg/day q24 hourly (maximum adult dose 100 mg/day)
•• Teratogenic •• Cough and angioedema common with captopril more than the other drugs of this group •• Check serum potassium, creatinine within 2 weeks of starting treatment and periodically once in 3 months •• Discontinue if the fall in eGFR > 30% from the baseline or eGFR < 30 mL/min/1.73 m2
Initial: 0.5–1 mg/kg/day to 2 mg/kg/day q12 to 24 hourly (maximum adult dose 100 mg/day)
Avoid in asthma, heart failure, and diabetes mellitus
Initial: 0.5–2 mg/kg/day q12 hourly up to 4 mg/ kg/day (maximum adult dose 240 mg/day)
Initial: 0.3–0.5 mg/kg/dose q8 hourly to 6 mg/kg/ day (maximum adult dose 450 mg/day)
Initial: 0.08 mg/kg/day up to 0.6 mg/kg/day q12 to 24 hourly (maximum adult dose 40 mg/day)
Angiotensin receptor blocker Losartan
α- and β-blocker Labetalol
Initial: 1–3 mg/kg/day q12 hourly to 40 mg/kg/ day (maximum adult dose 1200 mg/day) Intravenous (IV) dose: •• Bolus: 0.2–1 mg/kg/dose to 40 mg/dose •• Infusion: 0.25–3 mg/kg/h
Avoid in asthma, heart failure, and diabetes mellitus
Initial: 0.15–0.5 mg/kg/day q12 hourly (maximum adult dose 50 mg/day)
Heart rate is dose limiting for all drugs in these classes and may impair athletic performance Contd...
Nephrology Contd... Drug
Initial: 1 mg/kg/day to 3 mg/kg/day q12 to 24 hourly (maximum adult dose 50 mg/day)
Check electrolytes periodically and may need potassium supplement
Initial: 0.5–4.0 mg/kg/dose q6 to 24 hourly (maximum adult dose 160 mg/day)
•• Avoid loop diuretics in children with hypercalciuria or nephrocalcinosis •• Useful as add-on therapy in children with resistant HTN in association with renal failure
Initial: 5–10 µg/kg/day to 25 µg/kg/day q8 to 12 hourly (maximum adult dose 2.4 mg/day)
Sudden cessation of therapy can lead to rebound HTN
Initial: 10–60 mg/kg/day (maximum adult dose 3 g/day)
Sedation is common
Initial: 0.05–0.1 mg/kg/day q8 to 12 hourly Maximum: 0.1 mg/kg per day (maximum adult dose 20 mg/day)
May cause hypotension and syncope
Initial: 0.75 mg/kg/day @6 hourly to 7.5 mg/kg/ day (maximum adult dose 200 mg/day)
•• Tachycardia and fluid retention are common side effects •• It can cause a lupus-like syndrome in slow acetylators •• Avoid in coronary artery disease, head trauma, and intracranial hemorrhage
Continuous IV infusion 0.25–8 µg/kg/min
•• Contraindicated in coarctation of aorta, high intracranial pressure, and intracranial hemorrhage •• Toxicity risk increases with hepatic and renal failure •• Associated with cyanide and thiocyanate toxicity. Monitor cyanide levels if used for >48 hours
Peripheral α-agonist Prazosin
Abbreviations: ACE, angiotensin-converting enzyme; eGFR, estimated glomerular filtration rate; HTN, hypertension
Different types of drugs and their dosage are explained in Table 10.3.3.
BIBLIOGRAPHY 1. Bagga A, Jain R, Vijayakumar M, et al. Evaluation and manage ment of hypertension. Indian Pediatr. 2007;44(2):103-21. 2. Ellis D, Miyashita Y. Management of the Hypertensive Child. In: Avner ED, Harmon WE, Niaudet P, Yoshikawa N, Emma F, Goldstein SL (Eds). Pediatric Nephrology, 7th edition. Berlin Heidelberg: Springer-Verlag; 2016. pp. 2025-97.
3. International Society of Nephrology. Blood pressure management in children with CKD ND. Kidney Int. Suppl. 2012;2(5):372-6. 4. National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics. 2004;114(2 Suppl 4th Report):555-76. 5. Singh D, Akingbola O, Yosypiv I, et al. Emergency management of hypertension in children. Int J Nephrol. 2012;2012:15.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
10.4 Approach to Recurrent Urinary Tract Infection in Children G Sangeetha
INTRODUCTION Urinary tract infection (UTI) is one of the common bacterial infections in children next only to acute respiratory tract infection. It is identified by presence of symptoms with significant growth of a single species in the urine culture. Recurrent UTI is defined as the recurrence of symptoms with significant bacteriuria in children who have recovered clinically following the treatment. The second episode of culture-positive UTI is considered as recurrent UTI as per the Indian Society of Pediatric Nephrology (ISPN) guidelines.
DEFINITION According to the National Institute for Health and Clinical Excellence Guidelines: •• Two or more episodes of UTI with upper UTI/acute pyelonephritis, or •• One episode of UTI with acute pyelonephritis plus one or more episodes of UTI with lower UTI, or •• Three or more episodes of UTI with lower UTI. Recurrent UTI is common in infants and young children with female predominance. Various urinary tract anomalies along with voiding dysfunction are the important risk factors. Children with recurrent acute pyelonephritis with bilateral renal parenchymal defects are at risk of renal scarring and subsequent complications such as severe hypertension, complications during pregnancy, proteinuria, and chronic kidney disease. Identified risk factors for renal scarring are young age, high-grade vesicoureteric reflux (VUR) with intrarenal reflux, posterior urethral valve, neurogenic bladder, failure to identify and treat bowel bladder dysfunction (BBD), recurrent UTI, delay in initiation of therapy, inadequate treatment, and wrong choice of antibiotics.
HISTORY •• In neonates, urinary tract infection (UTI) may present as a part of septicemia such as fever, poor feeding, vomiting, jaundice, and temperature instability. •• Low-grade fever, dysuria, frequency, urgency, hematuria, and foul-smelling urine: Simple UTI/lower UTI.
•• High-grade fever with chills and rigors, persistent vomiting, dehydration: Complicated UTI/upper UTI. •• Recurrent fever, diarrhea, abdominal pain, and failure to gain weight may be the presentation in toddlers. •• Urinary incontinence, straining during urination, poor urinary stream, previous surgery of the urinary tract, anorectal malformation, and spinal abnormality— suspect underlying structural abnormality. •• Constipation, maneuvers to postpone voiding (holding maneuvers such as Vincent’s Curtsy), voiding less than 3 or more than 8 times a day—suspect bowel bladder dysfunction.
GENERAL EXAMINATION •• Growth parameters, systemic toxicity signs, and blood pressure •• Palpable distended bladder, enlarged kidneys, suprapubic, and renal angle tenderness •• Palpable fecal mass in the abdomen •• Phimosis or vulval synechiae •• Patulous anus, spinal deformity, and neurological deficit in lower limbs.
DIAGNOSIS OF URINARY TRACT INFECTION Urine Examination •• Urine microscopy: Pyuria [>5 white blood cells (WBCs)/ high-power field (HPF)]. It may get false positive in glomerulonephritis, renal stones, and foreign body in the urinary tract. •• Urine dipstick: Rapid dipstick-based tests are useful in screening for UTI. •• Positive leukocyte esterase test is suggestive of UTI. Nitrate reduction test is highly specific, but falsenegative tests are common, as urine needs to be in the bladder for at least 4 hours to accumulate a detectable amount of nitrite. Thus, a negative nitrite test does not exclude UTI. Urine culture is the gold standard investigation to diagnose UTI. Method of urine collection and probability of UTI are shown in Table 10.4.1.
Other Investigations in Recurrent Urinary Tract Infection •• Complete blood count and blood culture in children with suspected urosepsis •• C-reactive protein •• Procalcitonin •• Peripheral smear study •• Renal function test. TABLE 10.4.1: Probability of urinary tract infection (UTI) in different methods of urine collection. Method of urine collection
Colony-forming unit/mL of urine
Probability of UTI
Even a single pathogen growth is significant
Midstream clean catch
ROLE OF IMAGING IN RECURRENT URINARY TRACT INFECTION Role of imaging in recurrent urinary tract infection is shown in Table 10.4.2. Ultrasound, dimercapto succinic acid (DMSA) scan, and micturating cystourethrography (MCU) should be done in all children with recurrent UTI. Other imaging may be needed depending upon the underlying anatomical abnormality. Normal bladder wall thickness should not exceed 3 mm for a distended bladder and 5 mm for a nondistended bladder. Postvoid residue greater than 10% of bladder capacity or greater than 20 mL in any age group is significant.
MANAGEMENT OF RECURRENT URINARY TRACT INFECTION Recurrent urinary tract infection management steps are shown in Flowchart 10.4.1.
TABLE 10.4.2: Role of imaging in recurrent urinary tract infection. Imaging modality
Timing of the study
Findings and interpretations
During the acute illness
Kidney size, shape, anatomical location, corticomedullary differentiation, pelvicalyceal and ureteric dilatation, stones, posterior urethral dilatation, bladder wall thickness, and postvoid residue
MCU (micturating cystourethrography)
2–3 weeks after the treatment of UTI
•• Bladder pressure changes like diverticulum, posterior urethral valve, vesicoureteric reflux and its grading •• Spinning top configuration of bladder in bowel bladder dysfunction •• Christmas tree appearance of bladder in neurogenic bladder
DMSA scan (dimercaptosuccinic acid scan)
Acute phase or 2–3 months later
•• Parenchymal inflammation as cold areas with decreased uptake in acute phase of UTI •• Parenchymal defect and cortical scarring on follow-up scan after 3–6 months in recurrent pyelonephritis •• Split renal function of both kidneys
Direct radionuclide cystogram
Same as MCU
More specific than MCU, can pick up even mild vesicoureteric reflux (VUR). But posterior urethra and bladder anatomy cannot be delineated. Hence, it can be used for follow-up of VUR
Magnetic resonance (MR) On follow-up urography/computed tomo graphy (CT) urography
In the evaluation of duplicated collecting systems, ectopic ureter, and primary megaureter
To know the functional status of the lower urinary tract in neurogenic and nonneurogenic bladder
Magnetic resonance imaging (MRI)—spine
In the evaluation of neurogenic bladder
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 10.4.1: Management of recurrent urinary tract infection.
Abbreviations: UTI, urinary tract infection; PUV, posterior urethral valves; VUR, vesicoureteric reflux; IM, intramuscular; IV, intravenous; BP, blood pressure; BBD, bowel bladder dysfunction
BIBLIOGRAPHY 1. Indian Society of Pediatric Nephrology. Vijayakumar M, Kanitkar M, et al. Revised statement on management of urinary tract infections. Indian Pediatr. 2011;48(9):709-17. 2. National Collaborating Centre for Women’s and Children’s Health. Urinary tract infection in children diagnosis, treatment and long-term management. London: National Institute for Health and Clinical Excellence; 2007.
3. Paterson DL, Ko WC, Von Gottberg A, et al. Antibiotic therapy for Klebsiella pneumoniae bacteremia: implications of production of extended-spectrum beta-lactamases. Clin Infect Dis. 2004;39(1):31-7. 4. Santos J, Varghese A, Williams K, et al. Recommendations for the management of bladder bowel dysfunction in children. Pediatr Therapeut. 2014;4(1):191. doi:10.4172/2161-0665.1000191. 5. Sripathi V. Voiding dysfunction—A review. J Indian Assoc Pediatr Surg. 2005;10(4):229-36.
Urology Contributor: Senthil Ganesh Kamaraj Reviewer: Anupama S Borker
¯¯ Phimosis ¯¯ Congenital Hernias and Hydrocele ¯¯ Torsion of Testes and Other Causes of Acute Scrotum in Children
Partha’s Management Algorithms in Pediatric and Adolescent Practice
11.1 Phimosis Senthil Ganesh Kamaraj
INTRODUCTION Inability to retract the foreskin over the glans penis (Fig. 11.1.1).
PHYSIOLOGICAL PHIMOSIS (PRIMARY) (FIG. 11.1.2) At birth, natural adhesions exist between the glans and the prepuce, which gradually separate from the first 6 months of life onward. By 3 years of age, 80–90% of foreskins can be
retracted. About 8% of 6–7-year-old patients have phimosis, which reduces to 1% by 17 years. In asymptomatic children, older than 4–5 years, especially those with strong parenteral preferences and with boys developing balanitis and recurrent urinary tract infection—a course of local steroid application can be tried (personal preference is 0.05% betamethasone cream, applied twice daily for 1 month) before proceeding with a circumcision. In children with a background of urinary tract anomalies (especially during infancy) such as vesicoureteral reflux, posterior urethral valves and megaureters, a strong case can be made for prophylactic circumcision.
PATHOLOGICAL PHIMOSIS (SECONDARY) (FIG. 11.1.3)
Fig. 11.1.1: Examining a normal prepuce.
Usually happens after a period of initial separation (partial/ near total). The foreskin undergoes scarring and becomes nonretractable, secondary to lichen sclerosis (balanitis xerotica obliterans), or forceful retraction of foreskin, where a cicatrix forms between the de-epithelialized glans and the skin. Though steroids can be contemplated, it might be worthwhile to circumcise when scarring is noted. It is generally difficult to distinguish between lichen sclerosis and scarred prepuce.
Fig. 11.1.2: Partially retractable and supple foreskin.
Fig. 11.1.3: Pathological phimosis showing scarred foreskin and nonretractable nature.
Urology Flowchart 11.1.1: Algorithm showing physiological and pathological phimosis.
Abbreviations: VUR, vesicoureteral reflux; PUV, posterior urethral valves; UTI, urinary tract infection
Children with untreated lichen sclerosis can result in sequelae such as long length urethral strictures in adulthood.
CIRCUMCISION Despite the benefits in specific circumstances, few topics generate very much controversy in pediatric practice such as circumcision.
Complications of Circumcision •• •• •• ••
Bleeding Wound infection Recurrent penile adhesions Removal of excessive skin with resulting cosmetic deformities
•• Scar formation •• Removal of inadequate skin •• Childhood circumcision has potential morbidity and risk and should not be recommended without medical reasons, especially routine neonatal circumcision.
Contraindications to Routine Circumcision •• •• •• ••
Hypospadias Chordee without hypospadias Buried penis Micropenis. Algorithm of physiological (primary) and pathological (secondary) phimosis has been given in Flowchart 11.1.1.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
11.2 Congenital Hernias and Hydrocele Senthil Ganesh Kamaraj
INGUINOSCROTAL SWELLING IN A CHILD
•• Testicular tumor (0.35 kU/L
0.03 mL of extract of venom in increasing concentrations (0.001–1 µg/mL) injected to raise a 3 mm bleb
>3 mm wheal larger than negative control
Total serum IgE levels
Non-specific marker for atopy
100 kU/L normal, four times taken as significant
Useful when skin prick test and serum-specific testing are negative or some discrimination
An elevated baseline tryptase level indicates higher risk for severe anaphylaxis
Immunosorbent allergen chip
Measurement of specific IgE antibodies with ImmunoCAP ISAC results in a broad spectrum allergen profile on a molecular level
Abbreviations: IgE, immunoglobulin E; ISAC, immuno solid-phase allergen chip
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Avoid visiting the known sites of bee/or any stinging insect. •• Use insect repellents.
Mild Systemic Reactions •• For urticaria or intense itching, treat with oral secondgeneration antihistamines. •• Venom immunotherapy is not indicated for mild systemic reactions.
Severe Reactions •• Children suffer mainly cutaneous reactions (60%). Systemic symptoms mainly are respiratory and hypotension and loss of consciousness are less common in children. Patients should be trained in selfadministration of injection adrenaline. •• In case of severe reaction, patient is to be considered for venom immunotherapy.
Large Local Reactions •• In severe cases, corticosteroids may be necessary along with oral second-generation antihistamines. •• In case of infection of the wound, antibiotics are required. Recommended management of sting reactions is summarized in Table 14.5.5.
TABLE 14.5.5: Types of sting reaction and recommended subsequent management. Reaction
Older adolescents and adults
Large local reaction Further workup not recommended routinely, as risk of anaphylaxis extremely low in this group
Further workup not recommended routinely, as risk of anaphylaxis low
Cutaneous systemic reaction
Further workup not recommended routinely, as risk of anaphylaxis extremely low
Diagnostic testing and immunotherapy
Diagnostic testing and immunotherapy
Diagnostic testing and immunotherapy
VENOM IMMUNOTHERAPY Venom immunotherapy is very effective in preventing sting anaphylaxis. Controlled studies demonstrate that immunotherapy with vespid, honeybee, and jack jumper ant venoms are highly effective. Venom immunotherapy involves a series of subcutaneous injections starting with the lowest dose and gradual escalation until the maintenance dose (100 µg) is reached. Once the patient reaches maintenance dose, the generalized systemic reactions are very less and quality of life will be good. Approximate duration of immunotherapy is 5 years depending upon response and negative to venom-specific IgE (either skin prick tests or serum-specific venom IgE).
SUBLINGUAL IMMUNOTHERAPY Because of ease of administration and excellent safety profile, sublingual immunotherapy (SLIT) is preferred especially in children. But increase in cost compared to subcutaneous immunotherapy (SCIT) due to high dose of antigen used in SLIT is the concern. We need further studies of SLIT efficacy in children with insect sting allergy.
When to Refer to an Allergist? •• History of systemic allergic reaction to an insect sting. •• Systemic allergic reaction suspected due to insect sting. •• For educating the patient about stinging insect avoidance and for emergency management. •• Planning for venom immunotherapy. •• Patient is having anaphylaxis with coexisting situation where epinephrine is less effective (taking b-blocker).
POINTS TO REMEMBER •• Stings from insects of the order Hymenoptera are important causes of systemic allergic reactions. •• In sensitized persons, venom that is injected by a sting binds to venom-specific IgE on mast cells, with the subsequent release of mast-cell mediators that cause allergic reactions ranging from local reactions or urticaria to anaphylaxis and even death. •• Acute systemic allergic reactions typically occur very rapidly after a Hymenoptera sting but may be delayed for several hours or be biphasic. •• The treatment of a Hymenoptera-induced anaphylactic reaction (as for anaphylaxis caused by any other trigger) is the prompt administration of intramuscular epinephrine.
Allergy •• Patients who have had a systemic reaction to an insect sting should be referred to an allergist immunologist for testing for venom-specific IgE. •• Subcutaneous immunotherapy should be considered routinely in patients who have had a systemic allergic reaction to an insect sting and who have a positive test result for venom-specific IgE.
BIBLIOGRAPHY 1. Antolín-Amérigo D, Moreno Aguilar C, Vega A, et al. Venom immunotherapy: an updated review. Curr Allergy Asthma Rep. 2014;(14):449.
2. Casale TB, Burks AW. Hymenoptera-sting hypersensitivity. N Engl J Med. 2014;370:1432-9. 3. Golden DB, Demain J, Freeman T, et al. Stinging 0 4. Golden DB, Moffitt J, Nicklas RA, et al. Stinging insect hypersensitivity: a practice parameter update. J Allergy Clin Immunol. 2011;127(4):852-4.e23. 5. Hernandez RG, Cohen BA. Insect bite-induced hypersensitivity and the SCRATCH principles: a new approach to papular urticaria. Pediatrics. 2006;118(1):e189-96. 6. Ludman SW, Boyle RJ. Stinging insect allergy: current perspectives on venom immunotherapy. J Asthma Allergy. 2015(8):75-86. 7. Singh S, Mann BK. Insect bite reactions. Indian J Dermatol Venereol Leprol. 2013;79(2):151-64.
14.6 Urticaria and Angioedema Major K Nagaraju
INTRODUCTION Urticaria has been derived from the Latin word ‘urere’, which means “to burn”. It is characterized by the development of wheals or angioedema or both.
DEFINITIONS Urticaria Elevated skin lesions surrounded by erythematous base commonly described as “hives.” Often associated with pruritus.
Angioedema Rapid swelling of the dermis, subcutaneous mucosa and subcutaneous tissues involving face (eyelids, lips, and tongue), hands and feet, and, sometimes, other areas (trunk, genitalia, and mucous membranes). The characteristics of a wheal are:2 •• Central swelling of variable size, almost invariably surrounded by a reflex erythema. •• Associated with itching or sometimes a burning sensation. •• Fleeting nature, with the skin returning to its normal appearance, usually within 1–24 hours. The characteristics of angioedema are: •• Sudden, pronounced erythematous or skin-colored swelling of the lower dermis and subcutis with frequent involvement below mucous membranes, and
•• Sometimes pain rather than itching and frequent involvement below mucous membranes. Its resolution is slower than that for wheals and can take up to 72 hours.
PREVALENCE The prevalence of urticaria has been reported to be 2.1–6.7% in children and adolescents. A study in India recorded urticaria in 2.5% of children below 14 years of age.
CLASSIFICATION OF URTICARIA Classification of urticaria on the basis of its duration, frequency, and causes is as follows: Acute urticaria is defined as episodes of hives that last for less than 6 weeks. Chronic urticaria is defined as “daily or almost daily urticarial eruptions occurring for 6 weeks or more.” The risk factors for acute urticaria include immuno globulin E (IgE)-mediated reaction [foods (milk, egg, soya, and wheat) drugs (particularly antibiotics), and stinging insect venom] or non-IgE-mediated stimulation of mast cells caused by radiocontrast agents, viral agents including hepatitis B and Epstein-Barr virus, opiates, and nonsteroidal anti-inflammatory agents. Food-induced generalized acute urticaria may often have anaphylaxis. Subtypes of chronic urticaria are presented in Table 14.6.1. Disorders such as urticaria pigmentosa (cutaneous mastocytosis), urticaria vasculitis, autoinflammatory
Partha’s Management Algorithms in Pediatric and Adolescent Practice syndromes (e.g. cryopyrin-associated periodic syndromes or Schnitzler’s syndrome), and non-mast cell mediators mediated/induced angioedema (e.g. bradykinin-mediated angioedema) are not considered to be subtypes of urticaria, as they have different pathomechanisms.
DIAGNOSIS OF URTICARIA The first step in the diagnosis of urticaria is a thorough history, taking into considerations the questions in Table 14.6.2. TABLE 14.6.1: Classification of chronic urticarial. Type
Stress, infection, drugs (e.g. NSAIDs)
Inducible urticaria: •• Aquagenic •• Cholinergic •• Cold •• Delayed pressure •• Dermographism •• Exercise •• Heat •• Solar •• Vibratory
•• Contact with hot or cold water •• Exercise and emotion •• Swimming in cold water/ cold wind •• Sitting, lying, and tight clothing •• Minor trauma •• Physical exertion •• Hot bath/shower •• Sunshine •• Use of vibrating tools
Abbreviation: NSAIDs, non-steroidal anti-inflammatory drugs.
Surgical implantations and events during surgery, for example after local anesthesia
Induction by physical agents or exercise
Use of drugs (e.g. non-steroidal anti-inflammatory drugs (NSAIDs), injections, immunizations, hormones, laxatives, suppositories, ear and eye drops, and alternative remedies)
Observed correlation to food
Relationship to the menstrual cycle
Smoking habits (especially use of perfumed tobacco products or cannabis)
Type of work
Stress (eustress and distress)
Quality of life related to urticaria and emotional impact
Previous therapy and response to therapy
Previous diagnostic procedures/results
The second step of the diagnosis is the physical examination of the patient. This should include a diagnostic provocation test including drug, food, and tests as indicated by history (Table 14.6.3) and following the diagnostic algorithm (Flowchart 14.6.1).
Algorithm for Acute Urticaria/Angioedema Detailed history about recent infection, drug intake, and food intake along with any physical triggers (Flowchart 14.6.2).
TABLE 14.6.2: Questions that can help in screening of patients with urticaria. No.
Time of onset of disease
Frequency/duration of and provoking factors for wheals
Occurrence in relation to weekends, holidays, and foreign travel
Shape, size, and distribution of wheals
Associated subjective symptoms of lesions, for example: itch and pain
Family and personal history regarding urticaria and atopy
Previous or current allergies, infections, internal diseases, or other possible causes
Psychosomatic and psychiatric diseases
Specific tests: Complete blood count (CBC), urine routine examination (R/E) and C/S, erythrocyte sedimentation rate (ESR), skin prick tests/serum-specific IgE (ImmunoCAP method, if food allergies suspected.
MANAGEMENT OF URTICARIA Acute Urticaria Acute utricaria is usually self-limited and requires little treatment other than antihistamines. Avoidance of any identified trigger (food and drugs) is essential. Secondgeneration antihistamines cetirizine, levocetirizine, fexofenadine, loratadine, and desloratadine are effective and safe.
Chronic Urticaria Contd...
•• Identification and elimination of the trigger is the ideal treatment for chronic urticaria. Elimination of the following triggers and measures may be considered:
Allergy TABLE 14.6.3: Recommended diagnostic tests. Type
Recommended diagnostic tests
Additional tests (based on history)
Acute spontaneous urticaria
Allergy tests (skin prick test or specific IgE)
Chronic spontaneous urticaria
Differential blood count. ESR or CRP Omission of suspected drugs (e.g. NSAID)
Test for (in no preferred order): (i) infectious diseases (e.g. Helicobacter pylori), (ii) type I allergy (skin prick test or specific IgE), (iii) functional autoantibodies, (iv) thyroid hormones and autoantibodies, (v) skin tests including physical tests, (vi) pseudoallergen-free diet for 3 weeks, (vii) tryptase, (viii) autologous serum skin test, and (ix) lesional skin biopsy
Autoimmune Inducible urticaria
ANA, thyroid antibodies Aquagenic
Wet cloths at body temperature applied for 20 min
Exercise and hot bath provocation
Cold provocation and threshold test (ice cube, cold water, and cold wind)
Differential blood count and ESR or CRP cryoproteins rule out other diseases, especially infections
Cutaneous provocation test. Skin None tests with immediate readings, for example prick test
Pressure test and threshold test
Elicit dermographism and threshold test (dermographometer)
Differential blood count, ESR or CRP
Heat provocation and threshold test
UV and visible light of different wavelengths and threshold test
Rule out other light-induced dermatoses
Test with, for example, vortex
Abbreviations: IgE, immunoglobulin E; NSAID, non-steroidal anti-inflammatory drug; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein; ANA, antinuclear antibody; UV, ultraviolet
–– Drugs – such as non-steroidal anti-inflammatory drugs (NSAIDs) –– Physical stimuli –– Anti-infective—for Helicobacter pylori –– Avoiding allergenic foods and pseudoallergens such as food additives •• Pharmacological treatment (Flowchart 14.6.1 and Fig. 14.6.1): –– First line of therapy for chronic urticaria is the second-generation antihistamines (such as cetirizine, levocetirizine, fexofenadine, ebastine, and loratadine). –– The dose of the antihistamines can be started from once daily to up to four times a day, with increases in the dosage every 2 weeks. –– Discourage the use of first-generation antihistamines in infants and children.
Flowchart 14.6.1: Management of urticaria.
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 14.6.2: Modified BSACI guideline for the management of chronic urticaria and angioedema.
*not covered in this chapter Abbreviations: ACE, angiotensin-converting enzyme; AID, autoinflammatory disease; AAE, acquired angioedema; HAE, hereditary angioedema
–– Combination of antihistamines may be considered. –– H 2 -antagonists have been recommended in the American guidelines, however, they are not recommended in the European and British guidelines. –– Omalizumab the anti-IgE molecule (150–300 mg/ month) has been shown to be beneficial in cases of chronic spontaneous urticaria. Omalizumab has also been effective in cholinergic urticaria, delayed pressure urticaria, cold-induced urticaria, solar urticaria, heat urticaria, and in symptomatic
dermographism. Omalizumab is to be used only in antihistamine-resistant cases. –– The FDA and the EMA have approved omalizumab for the treatment of CSU only in children aged 12 years and above refractory to H1 antihistamines. –– Cyclosporin A has also been shown to be efficacious in addition to antihistamines. –– Leukotriene antagonists such as Montelukast have been used in limited clinical trials and may be used as add-on therapy.
Fig. 14.6.1: Stepwise approach of the chronic urticaria.
Figs 14.6.2A to E: (A) Urticarial rashes; (B) Papular urticaria; (C) Skin prick test; (D) Giant urticaria; (E) Autoimmune urticaria
Partha’s Management Algorithms in Pediatric and Adolescent Practice TABLE 14.6.4: The urticaria activity score (UAS) for assessing disease activity in chronic spontaneous urticaria (CSU). Score
Mild (50 wheals/24 hours or large confluent areas of wheals)
Intense (severe pruritus, which is sufficiently troublesome to interfere with normal daily activity or sleep)
Sum of score: 0–6 for each day.
–– In addition to the above, short courses of cortico steroids (maximum 10 days) can be utilized in clinical settings. Disease activity in spontaneous urticaria should be assessed in clinical care with the urticaria activity score (UAS) (Table 14.6.4).
PROGNOSIS •• Acute urticaria has good prognosis. •• Chronic spontaneous urticaria 50% will resolve by 5 years. Girls older than 10 years may have less favorable prognosis. •• Only second-generation antihistamines to be used.
When to Refer to an Allergist/ Dermatologist? •• •• •• ••
Not responding to high-dose antihistamines Patient is having concomitant other allergies Suspected autoimmune etiology For conducting skin prick tests.
BIBLIOGRAPHY 1. Bernstein JA, Lang DM, Khan DA, et al. The diagnosis and management of acute and chronic urticaria: 2014 update. J Allergy Clin Immunol. 2014;133(5):1270-7. 2. Ghaffari J, Shahmohammadi S, Ashrafi H, et al. Omalizumab (Xolair) in Children Above 12 Years With Chronic Urticaria: A Review of Literature. J Pediatr Rev. 2015;3(1):e152. 3. Godse K, Tahiliani H, Gautam M, et al. Management of urticaria in children. Indian J Paediatr Dermatol. 2014;15(3):105-9. 4. Grattan CE, Humphreys F. British Association of Dermatologists Therapy Guidelines and Audit Subcommittee. Guidelines for evaluation and management of urticaria in adults and children. Br J Dermatol. 2007;157:1116-23. 5. K Nagaraju. Urticaria and Angioedema: Manual of Pediatric Allergy, 1st edition. New Delhi: Jaypee Brothers Medical Publishers (P) Ltd; 2013. pp 45-53. 6. Licari A, Marseglia A, Caimmi S, et al. Omalizumab in Children. Paediatr Drugs. 2014;16(6):491-502. 7. Powell RJ, Leech SC, Till S, et al. BSACI guideline for the management of chronic urticaria and angioedema. Clin Exp Allergy. 2015;45(3):547-65. 8. Zuberbier T, Aberer W, Asero R, et al. The EAACI/GA(2) LEN/EDF/WAO Guideline for the definition, classification, diagnosis, and management of urticaria: the 2013 revision and update. Allergy. 2014;69(7):868-87.
Rheumatology Contributors: Sandesh Guleria, Rakesh Kumar Pilania, Surjit Singh Reviewer: Dhanya Dharmapalan
¯¯ Pediatric Rheumatology
Partha’s Management Algorithms in Pediatric and Adolescent Practice
15.1 Pediatric Rheumatology Sandesh Guleria, Rakesh Kumar Pilania, Surjit Singh
ALGORITHM FOR MANAGEMENT OF IGA VASCULITIS (HENOCH–SCHÖNLEIN PURPURA) Introduction Immunoglobulin A vasculitis (IgAV) is one of the most common types of vasculitis of childhood. Hitherto, it was termed eponymously as Henoch–Schönlein purpura (HSP). It usually affects children in the age group 3–10 years. Occasionally, IgAV can affect older children, including adolescents and adults. Boys are affected more commonly than girls (1.5:1). It is a leukocytoclastic vasculitis with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles). The onset of this disease is usually preceded by an upper respiratory tract or gastrointestinal infection. While dermatological and gastrointestinal manifestations of IgAV contribute to the morbidity in acute phase, long-term morbidity (and
prognosis) depends upon severity of renal involvement. Significant renal lesions are uncommon in children in preschool age group. Other risk factors for renal involvement are male sex, persistent purpuric lesions, severe abdominal symptoms, and decreased factor XIII activity.
Discussion Immunoglobulin A vasculitis is a small vessel vasculitis that can have various clinical manifestations. Diagnosis of HSP is usually clinical. Most common clinical manifestations are cutaneous (100%), gastrointestinal (80%), arthritis (75%), and renal (Figs 15.1.1A and B). Gastrointestinal involvement can be due to a variety of reasons (e.g. appendicitis, volvulus, intussusception, and vasculitis) and may occasionally be severe. At times, bleeding arising from IgAV can be severe and life-threatening (Flowchart 15.1.1). Severe renal involvement may manifest as nephritic syndrome, nephritic-nephritic syndrome, oligoanuria, gross hematuria or azotemia (Flowchart 15.1.2). A renal
Flowchart 15.1.1: Algorithm for management of Henoch–Schönlein purpura.
Abbreviations: GI, gastrointestinal, HSP, Henoch–Schönlein purpura
Rheumatology Flowchart 15.1.2: Evaluation and management algorithm of Henoch–Schönlein purpura nephritis.
Abbreviations: RPRF, rapidly progressive renal failure; HSP, Henoch–Schönlein purpura; BP, blood pressure
Figs 15.1.1A and B: Palpable purpuric lesions involve the buttocks, heels, and ankle of a patient with Henoch–Schönlein purpura.
biopsy is recommended in such situations. For management of severe gastrointestinal involvement glucocorticoids (prednisolone 1–2 mg/kg) are recommended. In presence of severe gastrointestinal hemorrhage, pulse therapy with intravenous methyl prednisone can be considered. Treatment of renal involvement is on similar lines as IgA nephropathy and is usually guided by renal biopsy findings. For crescentic glomerulonephritis induction cyclophosphamide followed by maintenance azathioprine can be considered. Prognosis for severe nephritis resulting from IgAV remains guarded.
MANAGEMENT ALGORITHM OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS Introduction Juvenile idiopathic arthritis (JIA) is the most common rheumatological disease in childhood. Systemic juvenile idiopathic syndrome (SJIA) is an important subgroup of JIA constituting about 10–15% patients (Figs 15.1.2A and B). It can be associated with significant morbidity
Partha’s Management Algorithms in Pediatric and Adolescent Practice and occasional mortality. Mortality in SJIA can occur as a result of overwhelming infections, macrophage activation syndrome (MAS) and amyloidosis. The following algorithms are suggested for the management of SJIA (Flowcharts 15.1.3 to 15.1.5).
Discussion As is evident from the accompanying algorithms, treatment of SJIA depends upon clinical presentation and response to initial treatment. Various studies have documented better disease control with early use of biologics in SJIA, especially systemic features predominant SJIA or in children with severe disease. The American College of Rheumatology has also recommended anakinra to be the drug of choice in this subset of SJIA patients. However, due to nonavailability of some biologics like anakinra in India and nonaffordability due to high cost, biologics are less often used in India. Leflunomide and thalidomide are other effective drugs for
treatment but, are not as effective as anakinra or tocilizumab. Due to propensity for the development of MAS, inflammation should be rapidly controlled with immunosuppressive agents in these patients. Glucocorticoids have many adverse effects so corticosteroids should be tapered off with target of reduction to half the initial dose by 3 months and to stop by 6 months.
MANAGEMENT ALGORITHM OF ENTHESITIS-RELATED ARTHRITIS Introduction Arthritis is one of the most common chronic diseases of childhood with long-term morbidity. JIA is the most common rheumatological disorder of childhood, characterized by chronic joint inflammation of unknown etiology and onset before the age of 16 years. The International League of Associations for Rheumatology has
Flowchart 15.1.3: Management algorithm in a child presenting with SJIA.
Abbreviations: IVIG, intravenous immunoglobulin; MAS, macrophage activation syndrome; MP, methylprednisolone; SJIA, systemic juvenile idiopathic syndrome; ESR, erythrocyte sedimentation rate
Rheumatology Flowchart 15.1.4: Management algorithm in a child with SJIA who presents with predominant arthritis but without significant systemic features.
Abbreviations: LFN, leflunomide; MTX, methotrexate; NSAID, nonsteroidal anti-inflammatory drug
recognized seven distinct categories of JIA. These include systemic JIA (SJIA), oligoarthritis, rheumatoid factor (RF)– negative polyarthritis, RF-positive polyarthritis, juvenile psoriatic arthritis, enthesitis-related arthritis (ERA), and undifferentiated arthritis. A group of related rheumatic diseases, which begin prior to 16 years of age and are strongly associated with human leukocyte antigen (HLA)-B27, are named ERA. This is also known as juvenile spondyloarthropathies. Mean age at diagnosis has been reported to be around 10–13 years with significant male predominance. It usually presents with asymptomatic lower limb arthritis and is characterized by inflammation at the entheses (i.e. bony sites where ligaments, tendons, capsule or fascia is inserted) (Figs 15.1.3A and B). Involvement of the joints of the axial skeleton (spine or sacroiliac joint) is usually not seen in the pediatric age group, but usually can develop over next 5–10 years from onset of disease. The following algorithm is suggested for management of ERA (Flowchart 15.1.6).
Discussion Patients with ERA usually respond well to NSAIDs and intra-articular glucocorticoid treatment (IAGc). Naproxen (15–20 mg/kg/day, maximum 500 mg twice daily) is the preferred NSAID in children. In all patients, physical exercise and maintenance of normal posture are recommended. Gc as a bridging medication can be used for immediate control of inflammation as it requires about 4–6 weeks for action of sulfasalazine to set in. For Intra-
Flowchart 15.1.5: Management algorithm in a child with SJIA who has both active systemic features and significant arthritis.
Abbreviations: MP, methylprednisolone; IACI, intra-articular corticosteroid injection; MTX, methotrexate
articular glucocorticoids triamcinolone hexacetonide is recommended at a dose of 1 mg/kg (max dose in larger joints 40 mg). Uveitis that can be associated with ERA is found in less than 10% cases and is usually symptomatic unlike the chronic asymptomatic uveitis seen in young children with oligoarthritis. Oligoarthritis can mimic ERA at onset of disease; however, oligoarthritis is predominately a disease of young girls while ERA typically affects older boys and adolescents. Another differentiating feature is presence of enthesitis in ERA. Development of axial skeleton can be monitored in follow-up by modified Schober test.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
Figs 15.1.2A and B: (A) Evanescent rash on abdomen in a child with SJIA. (B) Arthritis left ankle joint in a patient with SJIA.
Flowchart 15.1.6: Enthesitis-related arthritis (ERA) algorithm.
Abbreviations: NSAIDs, nonsteroidal anti-inflammatory drugs; IAGc, intra-articular glucocorticoids. *Gc: glucocorticoid; used as bridging medication or during flare # Poor prognosis factors: involvement of axial skeleton and/or radiology showing joint erosion or joint space narrowing
MANAGEMENT ALGORITHM OF SYSTEMIC LUPUS ERYTHEMATOSUS Introduction Systemic lupus erythematosus (SLE) is an autoimmune, multi-system disease with an onset which maybe acute, episodic or insidious. It can affect any organ system but the skin (Fig. 15.1.4), kidneys, musculoskeletal and
hematological systems are commonly affected. Antinuclear antibodies are almost always present. Currently, the Systemic Lupus International Collaborating Clinics (SLICC, 2012) classification criteria are used to establish a diagnosis of SLE. The treatment approaches are based on the clinical manifestations which are either serious systemic involvement or minor symptoms. The treatment algorithms are different for both types of presentation as depicted below (Flowcharts 15.1.7 and 15.1.8).
Flowchart 15.1.7: Management of a child with mild form of lupus.
Figs 15.1.3A and B: Asymmetrical arthritis of knee and ankle joint in the patient of enthesitis-related arthritis.
Abbreviations: HCQ, hydroxychloroquine; APLA, anti-phospholipid antibody; CBC, complete blood counts
Flowchart 15.1.8: Management of a child with severe form of lupus.
Abbreviations: MP, methylprednisolone; AZA, azathioprine; MMF, mycophenolate mofetil; CYC, cyclophosphamide; IVIG, intravenous immunoglobulin; HCQ, hydroxychloroquine; CVD, cardiovascular disease
Partha’s Management Algorithms in Pediatric and Adolescent Practice
Fig. 15.1.4: Malar rash in a child with systemic lupus erythematosus (SLE).
Discussion Children with lupus as compared to adults have more severe symptoms at onset with more aggressive clinical course
and multiorgan involvement. They also have increased need for glucocorticoids. All patients with SLE should be advised for photoprotective measures like application of sunscreen lotions (SPF-30), long-sleeved clothes and use of umbrella to avoid sun exposure. Patients should be followed for drug compliance and early detection of major organ involvement. Glucocorticoids are the mainstay of treatment but major organ involvement or severe diseases (Flowchart 15.1.8) require additional immunosuppressive agents (e.g. cyclophosphamide, mycophenolate mofetil, and azathioprine). Renal involvement is the most common cause of morbidity and mortality in SLE patients. The two treatment protocols [National Institutes of Health (NIH) regimen and Euro-Lupus Nephritis Trial (ELNT) regimen] followed for proliferative lupus nephritis treatments have similar effectiveness and short- as well as long-term followup.
Endocrinology Contributors: J Dhivyalakshmi, Vaman V Khadilkar, Hemchand K Prasad Reviewer: Dhanya Dharmapalan
¯¯ Short Stature ¯¯ Precocious Puberty ¯¯ Childhood Hypothyroidism ¯¯ Delayed Puberty in Children
Partha’s Management Algorithms in Pediatric and Adolescent Practice
16.1 Short Stature J Dhivyalakshmi
Growth is defined as increase in the physical size of the body as a whole or any of its part associated with increase in cell number (hyperplasia) or cell size (hypertrophy). Growth of a child (Flowchart 16.1.1) depends on interplay of various factors starting from prenatal/fetal period to puberty (Fig. 16.1.1).
Short stature is classically defined as height below −2 SD (third percentile) for age and gender within the population, or height below −2 SD (third percentile) below the midparental target height.
Flowchart 16.1.1: Major factors for somatic growth.
Abbreviations: EGF, epidermal growth factor, TGF, transforming growth factor; ICP, infancy–childhood–puberty
Fig. 16.1.1: Infancy–childhood–puberty (ICP) model of somatic growth.
Note: Growth curve is a sigmoid-shaped curve (bold line). This is due to rapid growth occurring in infancy, followed by a slow-decelerating childhood growth and height spurt during puberty. Dotted line represents growth without added up childhood and pubertal component.
Endocrinology Other definitions: •• Abnormally slow growth velocity (less than 25th percentile on height velocity charts) or •• Height dropping across two major centile lines on the growth chart. Dwarfism refers to more severe short stature, defined as height below −3 SD for age and gender norms. Figure 16.1.2 demonstrates normal height velocity or growth rates in children. Formula to calculate height velocity: Ht (cm) at time2 − Ht (cm) at time1 × 12 No. of months between time1 and time2
CAUSES OF SHORT STATURE The causes of short stature are shown in Flowchart 16.1.2.
DIAGNOSING SHORT STATURE The diagnosis of short stature is shown in Flowchart 16.1.3.
CLINICAL EVALUATION OF A CHILD WITH SHORT STATURE The clinical evaluation of a child with short stature is shown in Flowchart 16.1.4.
Fig. 16.1.2: Normal growth rates in children: (Rules of “5”).
Note: • Growth velocity during 2nd year: 12 cm, 3rd and 4th year is 7–10 cm/year (average about 10 cm/year between 2nd year and end of 4th year). • Pubertal height spurt occurs in Tanner stage 2 (breast) for girls and Tanner stage 4 (testicular volume) for boys. Hence, height spurt occurs early in girls compared to boys. • Once puberty is completed, child completes his/her growth potential. This is due to fusion of epiphysis and metaphysis in bones occurring under the influence of sex steroids. Hence, further growth after completion of puberty is not possible (as opposed to common belief—child can grow up to 18 years).
Flowchart 16.1.2: Causes of short stature.
Abbreviations: BMI, body mass index; DM, diabetes mellitus; GH, growth hormone; IUGR, intrauterine growth restriction; SHOX, short stature homeobox
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 16.1.3: Diagnosing short stature.
Abbreviations: MPH, mid-parental height; WHO, World Health Organization; IAP, Indian Academy of Pediatrics
Endocrinology Flowchart 16.1.4: Clinical evaluation of a child with short stature.
Abbreviations: SMR, sexual maturity ratings, BMI, body mass index, SGA, small for gestational age, CDGP, constitutional delay in growth and puberty.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
INVESTIGATIONS FOR A CHILD WITH SHORT STATURE The investigations for a child with short stature are shown in Flowchart 16.1.5. Flowchart 16.1.5: Investigations for a child with short stature.
Note: *Bone age is read using Greulich–Pyle’s atlas or Tanner–Whitehouse method. *Skeletal survey may be needed for children with suspected skeletal dysplasias. Abbreviations: CBC, complete blood count; GH, growth hormone; IGFBP, insulin-like growth factor-binding protein; IGF, insulin-like growth factor
CONCEPT OF CHRONOLOGICAL AGE, HEIGHT AGE AND BONE AGE FOR INTERPRETATION OF SHORT STATURE The concept of chronological age, height age, and bone age for interpretation of short stature is shown in Flowchart 16.1.6. Flowchart 16.1.6: Concept of chronological age, height age and bone age for interpretation of short stature.
Note: • Bone age is more compromised—most likely—endocrine cause of short stature. • Significant delay in bone age is considered when there is more than 2 years delay between bone age and chronological age.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
MANAGEMENT AND FOLLOW-UP OF A CHILD WITH GROWTH HORMONE DEFICIENCY The management and follow-up of a child with growth hormone deficiency is shown in Flowchart 16.1.7.
Flowchart 16.1.7: Management and follow-up of a child with growth hormone deficiency.
Abbreviations: GH, growth hormone; IGFBP, insulin-like growth factor-binding protein; IGF, insulin-like growth factor
MANAGEMENT OF PROPORTIONATE SHORT STATURE OTHER THAN GROWTH HORMONE DEFICIENCY The management of proportionate short stature other than growth hormone deficiency is shown in Flowchart 16.1.8. Flowchart 16.1.8: Management of proportionate short stature other than growth hormone deficiency.
Abbreviations: GH, growth hormone; CDGP, constitutional delay in growth and puberty
Partha’s Management Algorithms in Pediatric and Adolescent Practice
APPROACH TO A CHILD WITH DISPROPORTIONATE SHORT STATURE An approach to a child with disproportionate short stature is shown in Flowchart 16.1.9. Flowchart 16.1.9: Approach to a child with disproportionate short stature.
TABLE 16.1.1: Dose of growth hormone for various USFDA-approved conditions. Indications
Growth hormone deficiency
Short for gestational age with poor catch-up growth
Idiopathic short stature
Chronic kidney disease
SHOX gene haploinsufficiency
Abbreviation: SHOX, short stature homeobox
MONITORING GROWTH IN CHILDREN Growth monitoring in children is shown in Flowchart 16.1.10. Flowchart 16.1.10: Growth monitoring in children.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
BIBLIOGRAPHY 1. Khadilkar V, Yadav S, Agrawal K, et al. Revised IAP growth charts for height, weight and body mass index for 5- to 18-year-old Indian children. Indian Pediatr. 2015;52(1):4755. 2. Khadilkar VV, Khadilkar AV, Choudhury P, et al. IAP Growth monitoring guidelines for children from birth to 18 years. Indian Pediatr. 2007;44(3):187-97.
3. Lifshitz F. Growth, adrenal, sexual, thyroid, calcium, and fluid balance disorders, 5th edition. New York, NY: Informa Healthcare; 2007. 4. Raine J, Donaldson M, Gregory J, et al. Practical Endocrinology and Diabetes in Children, 2nd edition. Oxford: WileyBlackwell; 2006. 5. Sperling M. Pediatric Endocrinology, 4th edition. Philadelphia, PA: Saunders/Elsevier; 2014.
16.2 Precocious Puberty J Dhivyalakshmi
INTRODUCTION Puberty is the stage of growth caused by increased secretion of gonadotropins and sex steroids leading to sexual maturity and reproductive capacity. The normal physiology of puberty is mentioned in Flowchart 16.2.1.
PUBERTAL DEVELOPMENT IN GIRLS AND BOYS The pubertal development in girls and boys is shown in Flowchart 16.2.2.
Flowchart 16.2.1: Physiology of puberty.
Note: Dotted arrows indicate negative feedback. Central nervous system (CNS) insults [e.g. hypoxic-ischemic encephalopathy (HIE), encephalitis, trauma, etc.] destruct the inhibitory neurons causing premature activation of hypothalamic–pituitary–gonadal (HPG) axis leading to precocious puberty. Abbreviations: GABA, gamma-aminobutyric acid; GPR, G-protein-coupled receptor; AVPV, anteroventral periventricular nucleus; GnRH, gonadotropinreleasing hormone; FSH, follicle-stimulating hormone; LH, luteinizing hormone
Endocrinology Flowchart 16.2.2: Normal sequence of puberty.
Note: • Pubertal development following the above-mentioned sequence occurs in central precocious puberty. Pubertal development not following this sequence is termed as discordant pubertal development and is seen in peripheral/pseudoprecocity. • For sexual maturity rating (SMR) according to Tanner staging of pubertal development (Table 16.2.1). • Height spurt is an early event in girls (occurring after Tanner 2 breast stage) compared to boys (Tanner 3–4 genital stage; see Table 16.2.1). • Average age at Tanner stage 2 breast development in girls: 10.3 years for American girls and 10.9 years for Indian girls. • Average age of Tanner stage 2 genital development in boys: 10.1 years for American boys and 11.9 years in Indian boys. • Age at menarche: American girls around 13 years, Indian girls around 12.5 years.
TABLE 16.2.1: Tanner staging of pubertal development in girls and boys. Staging
Pubic hair staging
No pigmented hair
Pigmented hair (inner aspect of labia majora)
Accelerated growth velocity
Enlargement of the breast and areola
Coarser and pigmented hair spreading over mons
Peak growth velocity, thickening of vaginal mucosa, and axillary hair
Secondary mound of areola and papilla
Adult type but to smaller extent
Menarche (stage 3 or 4), decelerating growth rate
Mature breast development
Testicular volume (using Prader’s orchidometer)
Pubic hair staging
No pigmented hair
Pigmented hair at base of penis
Early voice changes
Coarse and curly hair extending to midline above penis
Increased penile length and girth, upper lip hair growth, acne, accelerated growth velocity
Adult type but to smaller extent
Adult distribution (spread to medial aspect of thighs and up to umbilicus)
Partha’s Management Algorithms in Pediatric and Adolescent Practice
DEFINITION OF PRECOCIOUS PUBERTY Precocious puberty is defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys. Note: Lawson Wilkins Pediatric Endocrine Society (LWPES) in 1999 recommended evaluation of girls with breast development or pubic hair growth before 7 years in white girls and 6 years in African–American girls. However, pubertal development nowadays occurs earlier by at least 1 year compared to that occurred last 2 decades. Hence, there are high chances of missing any pathological causes, if the above mentioned age cut-offs are used. Hence, generalization of this definition in Indian children needs further evaluation.
ETIOLOGY AND CLASSIFICATION OF PRECOCIOUS PUBERTY The etiology and classification of precocious puberty are shown in Flowchart 16.2.3.
CLINICAL ASSESSMENT OF A CHILD WITH EARLY PUBERTAL DEVELOPMENT The clinical assessment of a child with early pubertal development is shown in Flowchart 16.2.4.
CHILD WITH PRECOCIOUS PUBERTY The evaluation of a child with precocious puberty is shown in Flowchart 16.2.5.
CHILD WITH NORMAL VARIANT OF EARLY PUBERTAL DEVELOPMENT Approach to a child with normal variant of early pubertal development is shown in Flowchart 16.2.6.
APPROACH TO PRECOCIOUS PUBERTY IN GIRLS The approach to precocious puberty in girls is shown in Flowchart 16.2.7.
APPROACH TO PRECOCIOUS PUBERTY IN BOYS The approach to precocious puberty in boys is shown in Flowchart 16.2.8.
MANAGEMENT AND FOLLOW-UP OF PRECOCIOUS PUBERTY The management and follow-up of precocious puberty are shown in Flowchart 16.2.9.
Flowchart 16.2.3: Etiology and classification of precocious puberty.
*Gelastic seizures are seen in hypothalamic hamartoma. Abbreviations: CAH, congenital adrenal hyperplasia; LH, luteinizing hormone
Endocrinology Flowchart 16.2.4: Clinical assessment of a child with early pubertal development.
Note: *Compare current height with previous height measurements. Height crossing percentiles indicates height spurt. Also height crossing more than target height range indicates height spurt. Consider, central precocious puberty, if • Progressive puberty and/or height spurt • History of CNS insults (trauma, encephalitis, cerebral palsy, etc.) or signs of raised intracranial pressure, e.g. gelastic seizures seen in hypothalamic hamartoma. Consider, peripheral precocious puberty, if • Discordant pubertal development (pubertal development not following the normal sequence) • Neurocutaneous markers (e.g. café-au-lait spots in McCune–Albright syndrome), acne (indicates adrenal lesions, especially in less than 6 years old), virilization (virilizing adrenal or gonadal tumors), goiter, myxedematous facies (hypothyroidism). Abbreviation: SMR, sexual maturity rating according to Tanner stages
Partha’s Management Algorithms in Pediatric and Adolescent Practice
Fig. 16.2.1B Figs 16.2.1A and B: Growth chart as a tool. Diagnosing precocious puberty (scenario: An 8-year-old female child presenting with breast
development). Case 1 (Fig. 16.2.1A): An 8-year-old girl with SMR–A1P1B2 (axillary hair stage 1, pubic hair stage 1, and breast stage 2). Note there is no height spurt, current height within target height and normal bone age (BA, black triangle); all indicate normal variant of early pubertal development. Case 2 (Fig. 16.2.1B): An 8-year-old girl with SMR–A1P2B3 (axillary hair stage 1, pubic hair stage 2, and breast stage 3). Note that there is pubertal progression. Height spurt, current height exceeding target height and advanced bone age (black triangle), all indicate precocious puberty (BA > HA > CA). Note: Midparental height (purple leftward arrow) and target height range (purple upward and downward arrows) are marked at 18 years of age. Abbreviations: BA, bone age; CA, chronologic age; HA, height age
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 16.2.5: Evaluation of a child with precocious puberty.
Note: *Bone age: X-ray of wrist and hand. • GnRH stimulation test: LH level is greater than 5 mIU/L, LH response more than FSH response, along with rise in estradiol or testosterone, following GnRH stimulation test indicates central precocious puberty (CPP). Since GnRH availability is limited, GnRH analogs (e.g. triptorelin and leuprolide) are also used for stimulation nowadays. • Ultrasonogram (USG abdomen and pelvis): Uterine volume is greater than 2 mL, ovarian volume is greater than 1 mL, endometrial thickness is greater than 3 mm—suggestive of puberty. • Child with precocious puberty and short stature (bone age delay+)—seen in chronic hypothyroidism. Abbreviation: DHEAS, dehydroepiandrosterone sulfate
Flowchart 16.2.6: Approach to a child with normal variant of early pubertal development.
Abbreviation: SGA, small for gestational age
Endocrinology Flowchart 16.2.7: Approach to precocious puberty in girls.
Abbreviations: ACTH, adrenocorticotropic hormone; BA, bone age; CA, chronological age; CAH, congenital adrenal hyperplasia; DHEAS, dehydroepiandrosterone sulfate; GnRH, gonadotropin-releasing hormone; 17-OHP, 17-hydroxyprogesterone; MRI, magnetic resonance imaging
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 16.2.8: Approach to precocious puberty in boys.
Abbreviations: CA, chronological age; FSH, follicle-stimulating hormone; DHEAS, dehydroepiandrosterone sulfate; LH, luteinizing hormone; 17-OHP, 17-hydroxyprogesterone; ACTH, adrenocorticotropic hormone; CAH, congenital adrenal hyperplasia; AFP, alpha-fetoprotein; FMPP, familial male-limited precocious puberty; MAS, McCune-Albright syndrome; hCG, human chorionic gonadotropin
Flowchart 16.2.9: Management and follow-up of precocious puberty.
Note: *These drugs have been tried only on research basis from various uncontrolled trials. None of these drugs has been approved for use in precocious puberty.
Endocrinology TABLE 16.2.2: Dosage, route of administration of GnRH analog therapy. Drug
Route of administration
0.3 mg/kg/month (United States)
11.25 mg, 3 monthly (European)
4-week (3.6 mg) or 12-week (10.8 mg) implants
Abbreviation: IM, intramuscular
BIBLIOGRAPHY 1. Carel JC, Eugster EA, Rogol A, et al. Consensus statement on the use of gonadotropin-releasing hormone analogs in children. Pediatrics. 2009;123(4):e752-62. 2. Carel JC, Leger J. Clinical practice. Precocious puberty. N Engl J Med. 2008;358(22):2366-77. 3. Fuqua JS. Treatment and outcomes of precocious puberty: An update. J Clin Endocrinol Metab. 2013;98(6):2198-207. 4. Khadilkar VV, Stanhope RG, Khadilkar V. Secular trends in puberty. Indian Pediatr. 2006;43(17):475-8. 5. Kletter GB, Klein KO, Wong YY. A Pediatrician’s guide to central precocious puberty. Clin Pediatr. 2015;54(5):414-24. 6. Lee PA, Houk CP. Puberty and its disorders. In: Lifshitz F (Ed). Pediatric Endocrinology. New York: Informa Health Care; 2007. pp. 273-303. 7. Sun SS, Schubert CM, Chumlea WC, et al. National estimates of the timing of sexual maturation and racial differences among US children. Pediatrics. 2002;110(5):911-9.
16.3 Childhood Hypothyroidism Vaman V Khadilkar, Hemchand K Prasad
Flowchart 16.3.1: Neonatal thyroid screening protocol.
Hypothyroidism is the most common endocrine problem encountered by a pediatrician. Hypothyroidism may be congenital or juvenile. This chapter provides the reader a simple algorithm-based approach to: •• Congenital hypothyroidism screening in a newborn baby. •• Evaluation of congenital hypothyroidism. •• Management of congenital hypothyroidism. •• Evaluation of juvenile hypothyroidism and goiter. •• Management of juvenile hypothyroidism in children and adolescents. •• General principles of dosing and therapy.
NEONATAL THYROID SCREENING PROTOCOL An approach to congenital hypothyroidism screening in a newborn baby is shown in Flowchart 16.3.1.
WORK-UP OF CONGENITAL HYPOTHYROIDISM An approach to evaluation of congenital hypothyroidism is shown in Flowchart 16.3.2.
Note: 1. If TSH is greater than 40, do not wait for venous sample report. 2. Venous sample report must be available in 24 hours. 3. Do not wait for evaluation beyond 3 weeks. Abbreviations: TSH, thyroid-stimulating hormone; FT4, free T4
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 16.3.2: Work-up of congenital hypothyroidism.
Abbreviations: TSHR, thyroid-stimulating hormone receptor; USG, ultrasonography
MANAGEMENT AND FOLLOW-UP OF CONGENITAL HYPOTHYROIDISM An approach to management and follow-up of congenital hypothyroidism is shown in Flowchart 16.3.3. Flowchart 16.3.3: Management and follow-up of congenital hypothyroidism.
Partha’s Management Algorithms in Pediatric and Adolescent Practice Flowchart 16.3.4: Approach to goiter and juvenile hypothyroidism.
Box 16.3.1: General thyroxine doses. Dose: • 100 μg/m2 or • Neonate: 10–15 μg/kg • 4 days): Resolution or progressive liver failure and death.
Supportive Therapy and Indication for Liver Transplantation The supportive therapy and indication for liver transplantation of paracetamol poisoning are shown in Box 17.3.2.
Fig. 17.3.1: Rumack–Matthew nomogram for paracetamol. Note: • If sustained release preparation is used, level should be repeated at 8 hours. • Nomogram cannot be used, if level is measured after 24 hours, for multiple supratherapeutic doses and when time of ingestion is unknown. • Use with caution in children who have co-ingested drugs which delay gastric emptying.
Partha’s Management Algorithms in Pediatric and Adolescent Practice Box 17.3.1: NAC (N-acetylcysteine) dosage regimens.
Box 17.3.2: Supportive therapy.
N-Acetylcysteine (NAC) dosage regimens Oral dose: 140 mg/kg loading dose followed by 70 mg/kg q4h for 17 additional doses (total 1,330 mg/kg over 72 hours). Intravenous regimen: • Lading dose: 150 mg/kg over 60 minutes • Dose 2: 50 mg/kg over 4 hours • Dose 3: 100 mg/kg over 16 hours. Alternate IV regimen: Loading dose 150 mg/kg over 60 minutes followed by 10 mg/kg/h over 24–72 hours. Duration of NAC infusion: Usually oral regimen is for 72 hours and IV for 24 hours. But, if there is liver dysfunction and coagulopathy, infusion can be continued till 72 hours or till INR normalizes. Mode of action of NAC: In overdose, the pathways for metabolizing paracetamol to nontoxic metabolites are saturated and excess drug is metabolized to a toxic product called NAPQI which is conjugated by glutathione to nontoxic metabolites. In overdose, glutathione stores are exhausted and NAPQI accumulates. NAC acts as substitute for glutathione and detoxifies NAPQI. It also scavenges free radicals and improves hepatic microcirculation.
Supportive therapy: • Intravenous dextrose containing fluids and monitoring to prevent hypoglycemia. • Vitamin K and FFP/cryoprecipitate infusion to correct coagulopathy. • Packed cells transfusion, if there is bleeding. • Ammonia-lowering strategies like oral lactulose, if there is hyperammonemia. • Management of coma. • If there is kidney injury, conservative management or dialysis. Indication for liver transplantation (King’s college criteria): • If all three of the following occur in a 24-hour period: – Serum creatinine > 3.4 mg/dL. – PT > 100 seconds (INR > 6.5). – Grade III/IV encephalopathy. • Arterial lactate > 3.5 mmol/L after adequate fluid resuscitation.
PREVENTION OF OVERDOSE DUE TO ERRORS Paracetamol dose and type of formulation should be accurate and legible and communicated to the parents clearly to avoid drug overdose.
COMMON TYPES OF ERRORS RESULTING IN PARACETAMOL OVERDOSE
•• Unintentionally prescribing two preparations as paracetamol is also available in some cough preparations. •• Decimal point errors, e.g. 0.6 mL of drops written as 0.6 mL, can be mistaken for 6 mL. •• Giving paracetamol through different routes, e.g. rectal as well as oral. •• Iatrogenic IV overdose: Dose in mg misinterpreted as mL of injection.
•• Giving concentrated preparation inadvertently, e.g. paracetamol drops instead of suspension, double strength instead of single strength.
17.4 Organophosphate Poisoning P Ramachandran, PS Rajakumar
INTRODUCTION Organophosphate compounds (OPCs) and carbamates are potent cholinesterase inhibitors. They cause severe cholinergic toxicity following ingestion, skin exposure, or inhalation.
MECHANISM OF ACTION Organophosphate compounds inhibit acetylcholinesterase (AChE) by phosphorylation. After a period of time, usually
about 72 hours, this bond matures or ages. This renders the enzyme irreversibly resistant to reactivation by oxime antidote. Inhibition of AChE leads to the accumulation of acetylcholine (ACh) at cholinergic synapses (muscarinic receptors at parasympathetic nerve endings, preganglionic synapses and nicotinic receptors in neuromuscular junctions and cholinergic endings in the brain). This interferes with the normal function of the autonomic, somatic, and central nervous systems resulting in a range of clinical manifestations, known as acute cholinergic crisis.
Poisoning Flowchart 17.4.1: Organophosphate compound poisoning
Partha’s Management Algorithms in Pediatric and Adolescent Practice Contd…
TABLE 17.4.1: Clinical severity assessment in organophosphate compound poisoning. Mild
Walks and talks
Unconscious, no pupillary reflex
Headache, dizzy, nausea and vomiting, abdominal pain, sweating, salivation, rhinorrhea stable vitals
Weak voice, muscle Muscle twitching, flaccid paralysis, increased bronchial secretions, twitching (fasciculation), dyspnea, crackles/wheeze, respiratory failure. general weakness, anxiety, Convulsions restlessness, small pupils (miosis)
Serum acetylcholinesterase is 20–50% of normal
Serum acetylcholinesterase enzyme is 10–20% of normal
Carbamates cause transient and reversible inhibition of AChE. Acetylcholinesterase inhibited by carbamates does not age, allowing spontaneous reactivation and restoration of normal nervous function. The enzyme activity may be restored within 48 hours.
MANAGEMENT ALGORITHM FOR ORGANOPHOSPHATE COMPOUND POISONING The flowchart for organophosphate compound poisoning is shown in Flowchart 17.4.1.
BIBLIOGRAPHY 1. Bryant S, Singer J. Management of toxic exposure in children. Emerg Med Clin North Am. 2003;21(1):101-19. 2. Cachia EA, Fenech FF. Kerosene poisoning in children. Arch Dis Child. 1964;39:502-4.
Serum acetylcholinesterase enzyme is 104°F) associated with psychomotor agitation may require immediate neuromuscular paralysis to rapidly decrease temperature. Temperature control should be achieved within 15–20 minutes upon presentation in order to prevent multiorgan failure and death. In patients with acute oral ingestion, GI decontamination is performed by the administration of activated charcoal. Orogastric lavage may be performed when the patient presents with acute life-threatening intoxication shortly after ingestion. Foley catheter placement may be useful to monitor urine output, particularly in situations in which diuretics are administered to manage pulmonary edema. Patients often have decreased urination due to the effects on bladder sphincter muscles to prevent passing urine. Significant cardiac dysrhythmias may require cardioversion, defibrillation, and antidysrhythmics. Benzodiazepine sedation can be used to initially manage hypertension, if present.
Treatment for Patients with Cocaine Intoxication •• Check the nares for residual cocaine, and remove if present. •• Monitor for hypoglycemia, which may present as any neuropsychiatric abnormality.
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Carefully use naloxone for patients with altered mental status, if opioid use cannot be excluded. •• Administer benzodiazepines to manage seizures. The effects of cocaine are generally short-lived. Monitor patients until they are no longer tachycardic and hypertensive and also until they are calm and cooperative.
OPIOIDS Heroin, morphine, codeine, oxycodone, and fentanyl are among the most commonly abused opioids. Signs and symptoms: Signs of intoxication include sedation, psychomotor difficulties, confusion, pinpoint pupils and, in more extreme cases, respiratory depression. Symptoms of opioid withdrawal include diarrhea, abdominal cramping, generalized pain, piloerection, and rhinorrhea. Although opioid withdrawal is usually very uncomfortable, it is not life-threatening.
Treatment Aggressive airway control: Endotracheal intubation is indicated for patients who are unable to protect their airway. In patients lacking spontaneous respirations, orotracheal intubation is preferred. If advanced life support (ALS) is available, IV naloxone (Narcan) may be given to reduce respiratory depression, only an amount sufficient to return spontaneous respirations is recommended. In suspected habituated opiate users, slowly administer 0.1–0.4 mg IV aliquots every 1–2 minutes for a more controlled and partial reversal of opiate effect. Assisted bag-valve-mask breathing can be provided until the patient is ventilating adequately. Judicious application of restraints in a potentially violent patient is advisable in close quarters. Exercise caution while administering naloxone because it can transform a peacefully sleeping patient into an agitated bundle of nerves.
CLUB DRUGS These agents are popular at dance clubs, parties, and rock concerts. They may have stimulating or psychedelic properties and include methylenedioxymethamphetamine (MDMA) (ecstasy), gamma hydroxybutyrate (GHB) (liquid ecstasy), ketamine (nicknamed vitamin K), Rohypnol, and lysergic acid diethylamide (LSD) nicknamed acid. Signs and symptoms: Dreamlike states, altered perceptions and, at times, hallucinations. Intoxication can also cause symptoms varying from mild confusion to impairment of
motor functions, mood swings, increased blood pressure, and difficulty in breathing.
Treatment There are no GHB detection tests for use in emergency rooms, and as many clinicians are unfamiliar with the drug, many GHB incidents likely go undetected. Patients with a ketamine overdose are managed through supportive care for acute symptoms, with special attention to cardiac and respiratory functions. Treatment for Rohypnol follows accepted protocols for any benzodiazepine, which may consist of a 3–5 day inpatient detoxification program with 24-hour intensive medical monitoring and management of withdrawal symptoms, since withdrawal from benzodiazepines can be life-threatening.
LABORATORY TESTS Integration of clinical data and drug-screen results is the most effective way to determine whether an SUD is present. Toxicological screening is, however, limited by the time needed to obtain results, lack of correlation between drug concentration and observed results, and the risk of falsepositive and false-negative results. A positive drug screen does not prove substance abuse or dependence, but does indicate substance use. Similarly, a negative drug screen is not sufficient to rule out either substance use or SUDs. The effectiveness of drug screens may be limited by the brief presence in the body of many substances of abuse and their metabolites. Stimulants are detectable in the urine for up to 1 or 2 days. Cocaine and its major metabolite are present for up to several days whereas cannabis, which is lipid soluble, may be detected for 1 month or more when use has been chronic. Other issues, e.g. noninclusiveness of routine drug screens, difficulty in obtaining urine and blood samples for a drug screen from uncooperative patients and the premature discharge before drug-screen results are available because of overcrowding hurdles in evaluation of substance abuse cases in a busy ED.
Associated Treatment Considerations •• Psychosocial treatments: Family therapy, interpersonal or psychodynamic therapy, psychoeducation, cognitivebehavioral therapy, psychopharmacology, 12-step programs, and self-help groups. •• Pharmacotherapy for comorbid diseases like diabetes, hypertension, etc.
CONCLUSION To achieve wholesome adolescent health, we need to have a multidimensional approach covering all the adolescent health problems with special emphasis on mental health issues. Some recommendations are as follows: •• Psychological disorders can be greatly reduced by giving top priority and effective implementation of mental health services at all levels. •• Counseling should be strengthened by well-trained healthcare providers and involve parents and elders of the family in home-based counseling. Educate parents and teachers to improve the quality of relationship with children to ensure a safe, secure, and appropriate environment. •• Encourage community participation in mobilizing adolescents to build life skills and to take active part in community programs.
•• Strict enforcement of laws related to traffic rules, prohibiting sale of tobacco, alcohol, and other substance of abuse to minors.
BIBLIOGRAPHY 1. Kelley AE, Schochet T, Landry CF. Risk taking and novelty seeking in adolescence: introduction to part I. Ann NY Acad Sci. 2004;1021:27-32. 2. National Crime Records Bureau. Accidental Deaths and Suicides in India. New Delhi: Ministry of Home Affairs, Government of India; 2013. 3. NCPCR. (2013). Assessment of Pattern and Profile of Substance Use among Children in India (Tikoo V, Dhawan A, Pattanayak R). [online]. Available from http://www.ncpcr. gov.in/view_file.php?fid=17 [Accessed September 8, 2014]. 4. Patel V, Ramasundarahettige C, Vijayakumar L, et al. Suicide mortality in India: a nationally representative survey. Lancet. 2012;379(9834):2343-51.
18.2 Intensive Care in Adolescent Pregnancy Piyali Bhattacharya
INTRODUCTION The 2014 World Health Statistics indicate that: •• About 16 million girls aged 15–19 years and some 1 million girls less than 15 years give birth every year— most in low- and middle-income countries. •• Complications during pregnancy and childbirth are the second cause of death for 15–19 years old girls globally. •• Every year, some 3 million girls aged 15–19 years undergo unsafe abortions. •• Babies born to adolescent mothers face a substantially higher risk of dying than those born to women aged 20–24 years. For most women, early and regular prenatal care promotes a healthy pregnancy and delivery without complications. The teenager’s bodies, however, are not equipped to manage pregnancy. Subsequently, pregnancy in teens increases the risk for preeclampsia, gestational high blood pressure, and a variety of other complications. Such high-risk pregnancies should receive care from a special team of healthcare providers to ensure that their pregnancies are healthy and that they can carry their fetus to term.
COMPLICATIONS Potential Complications for the Teenage Mother •• •• •• •• ••
Mortality at child birth Pregnancy-induced hypertension (PIH) Preeclampsia (PE)/eclampsia Cephalopelvic disproportion and cesarean sections Premature labor, emergency delivery, and resuscitation of newborn.
Potential Complications for the Baby •• Premature and low-birth-weight (LBW) babies with high death rates in the neonatal period. •• The incidence of birth asphyxia, respiratory distress syndrome, and neonatal hyperbilirubinemia are significantly more in babies born to teenage mothers. •• Congenital defects, e.g. anencephaly, spina bifida, and meningomyelocele occur more frequently.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
Pregnancy-induced Hypertension Pregnancy-induced hypertension or toxemia is unique to pregnancy and manifested by hypertension, proteinuria, and edema. Whereas about 5% of the general population develops pregnancy-induced hypertension (PIH), the condition is the most prevalent complication associated with adolescent pregnancy. In 2015, the American College of Obstetricians and Gynecologists Committee on Obstetric Practice issued updated guidelines regarding the emergency treatment of acute-onset severe hypertension during pregnancy, including the following: •• Acute-onset severe hypertension that is accurately measured using standard techniques and is persistent for 15 minutes or longer, is considered a hypertensive emergency. •• Intravenous labetalol [0.25–1 mg/kg intravenous (IV) over 2 minutes, repeated after 5 minutes or a continuous infusion 0.4–3.0 mg/kg/hr] and hydralazine [0.1–0.5 mg/kg/dose q6 h IV/intramuscular (IM), maximum of 2 mg/kg/dose] have long been considered first-line medications for the management of acute-onset severe hypertension in pregnant women. •• Available evidence suggests that oral nifedipine (0.25– 0.5 mg/kg/dose 4–6 h orally with a maximum of 10 mg) may also be considered as a first-line therapy. •• Parenteral labetalol should be avoided in patients with asthma, heart disease, or congestive heart failure. •• When urgent treatment is needed before the establishment of IV access, the oral nifedipine algorithm can be initiated as IV access is being obtained, or a loading dose of labetalol can be administered orally. The latter can be repeated in 30 minutes if appropriate improvement is not observed. •• Magnesium sulfate is not recommended as an antihypertensive agent, but remains the drug of choice for seizure prophylaxis in severe preeclampsia and for controlling seizures in eclampsia. •• Sodium nitroprusside (0.5–0.8 mg/kg/min IV infusion, 50 mg to be dissolved in 1 L of 5% dextrose to provide concentration of 50 mg/mL) should be reserved for extreme emergencies and used for the shortest amount of time possible because of concerns about cyanide and thiocyanate toxicity in the mother and fetus or newborn, and increased intracranial pressure with potential worsening of cerebral edema in the mother.
Preeclampsia Preeclampsia is more common at the extremes of maternal age (35 years). Symptoms of preeclampsia
may include visual disturbances, typically scintillations, and scotomata, presumed to be due to cerebral vasospasm. The history of sudden rapid weight gain could be an indicator to developing preeclampsia. However, edema is no longer included among the criteria for the diagnosis of preeclampsia. Preeclampsia is rare before the third trimester, and the diagnosis of severe hypertension or preeclampsia in the first or early second trimester necessitates exclusion of gestational trophoblastic disease or molar pregnancy. New seizures in pregnancy suggest preeclampsia-eclampsia, but primary neurologic disorders must be excluded. If the blood pressure is sustained greater than 160 mm Hg systolic and/or 110 mm Hg diastolic at any time, lowering the blood pressure quickly with rapid-acting agents is indicated for maternal safety. Anticonvulsant therapy may be undertaken in the setting of severe preeclampsia (primary prophylaxis) or in the setting of eclamptic seizures (secondary prophylaxis). The most effective agent is IV magnesium sulfate indicated to prevent seizures associated with preeclampsia, and for control of seizures with eclampsia.
Treatment and Prevention of Seizures in Severe Preeclampsia or Eclampsia Adolescents and adults: 4–6 g magnesium sulfate over 15–20 minutes; followed by a 1–2 g magnesium sulfate/hour continuous infusion; or may follow with IM doses of 4–5 g magnesium sulfate in each buttock every 4 hours. Note: Initial infusion may be given over 3–4 minutes if eclampsia is severe, maximum: 40 g magnesium/24 hours.
Once the mother’s condition is stabilized following a seizure, the physician will prepare for emergency delivery of the infant either by a cesarean section or induction of labor. If the patient is already in labor, labor can be allowed to progress provided there is no evidence that the baby has become “distressed” or compromised by the seizure.
MANAGEMENT Management of Preterm Labor Preterm labor is defined as the presence of uterine contractions of sufficient frequency and intensity to effect progressive effacement and dilation of the cervix prior to term gestation (between 20 weeks and 37 weeks). Goals of management of preterm labor should include: •• Timely diagnosis of preterm labor •• Evaluating fetal well-being
PREVALENCE OF PRETERM, LBW, PERINATAL DEATHS IN ADOLESCENT PREGNANCY
•• Providing prophylactic pharmacologic therapy to prolong gestation and reduce the incidence of respiratory distress syndrome (RDS) and intra-amniotic infection (IAI), and •• Initiating tocolytic therapy when indicated.
A comparison of some studies showing prevalence of preterm, LBW, and perinatal deaths in adolescent pregnancy is given in Table 18.2.1. The main neonatal complications found were prematurity, low or very low birth weight, and perinatal mortality. The Indian study done by Mukhopadhyay et al. compared the perinatal differences between 350 adolescents (13–19 years) and 350 adults (20–29 years), both groups of primigesta, by means of medical record analysis, and demonstrated that there was a greater proportion of premature deliveries (27.7%), LBW (39.1%), and rate of stillborns (5.1%), in comparison with adult mothers. Stabilization of the preterm newborn in the delivery room with adequate respiratory and thermal management is crucial to the immediate and long-term outcome of premature infants. Principles of respiratory management are as follows: •• Recruit and maintain adequate lung volume or optimal lung volume in infants with respiratory distress: With early continuous positive airway pressure (CPAP) given nasally, by mask, or by using an endotracheal tube when ventilation and/or surfactant is administered. •• Avoid hyperoxia and hypoxia by immediately attaching a pulse oximeter and keeping the oxygen saturation (SaO2) between 86% and 93% by using an oxygen blender. •• Prevent barotrauma or volutrauma by using a ventilator that permits measurement of the expired tidal volume and by keeping it 4–7 mL/kg. •• Administer surfactant early ( 20 mEq/L: Renal Urine Na < 20 mEq/L: Nonrenal ↓ Na ↓ K ↑ Cl: RTA ↓ Na ↑ K ¯ ↓ glucose: Adrenal insufficiency
Causes •• Renal failure •• Nephrotic syndrome •• Congestive heart failure •• Protein-energy malnutrition •• Cirrhosis liver
Abbreviations: SIADH, syndrome of inappropriate antidiuretic hormone secretion
5. Common cause in this category is syndrome of inappropriate ADH secretion (SIADH). The simplest therapy in restricting water intake, i.e. two-third maintenance should be given as isotonic fluid. If the child is symptomatic with convulsions, hypertonic (3%) saline administration is the standard treatment irrespective whether it is hypovolemic, isovolemic or hypervolemic hyponatremia. If this is ineffective or if severe hyponatremia is present, combination of hypertonic saline and diuretic can be used. 6. Here ECF volume is increased, so water is restricted to a volume less than the urine output and sodium is also restricted. In a symptomatic child, hypertonic saline is used along with diuretic. Consider dialysis, in the presence of severe hyponatremia in renal failure. 7. Hyponatremia in complex situations: A. Syndrome of inappropriate ADH secretion: The causes are meningitis, head trauma, spinal or intracranial surgery, respiratory problems, such as asthma, pneumonia, and tuberculosis, drugs, such as carbamazepine, vincristine, and cyclophosphamide. Diagnostic criteria are hyponatremia, no dehydration no edema, normal renal, hepatic, adrenal, and thyroid function. Urine osmolality is greater than 100 mOsm. Usually more than serum osmolality. Serum osmolality is less than 280. Serum sodium is less than 135. Urine sodium greater than 20 mEq/L. B. For hyponatremia with seizures and shock: Ringer lactate or normal saline boluses used to treat shock will correct sodium deficit.
Example: 1 year old, weight 10 kg, severe dehydration and shock. 3 boluses (60 mL) per kg given in 1 hour, 600 mL (0.6 L) × 131 mEq/L = 79 mEq. This alone will raise the Na level by 13 mEq/L. Hence, it is better to recheck the sodium level after correction and decide further management. C. In a child with suspected adrenal insufficiency when hyperkalemia accompanies hyponatremia in the absence of renal failure, hydrocortisone is used. 8. Pitfalls in the management of hyponatremia are: (1) Failure to exclude causes of pseudohyponatremia. (2) Failure to identify and treat hyponatremia allowing the ongoing risk of cerebral edema. (3) Too rapid correction which can cause central pontine demyelination due to destruction of medullary sheaths in the center of the basilar portion of the pons. Clinical features range from coma, pseudobulbar palsy, and quadriparesis to minor behavioral changes and will need magnetic resonance imaging (MRI) to confirm the diagnosis. Serum sodium should be raised rapidly only during seizures. In an asymptomatic child, deficit correction should never be more than 0.5 mEq/hr or more than 12 mEq/L per 24 hours. 9. Sodium content of various IV fluids The sodium content of various IV fluids (Table 18.3.2): •• Simple mnemonic to remember this! •• Normal serum sodium is 130–150 mEq/L •• Normal saline contains upper limit of normal serum sodium •• Ringer lactate contains lower limit
Partha’s Management Algorithms in Pediatric and Adolescent Practice TABLE 18.3.2: Sodium content of various intravenous (IV) fluids. IV fluid
Normal (0.9%) saline
0.45% (half normal) saline
Sodium bicarbonate (8.4%)
Sodium bicarbonate (7.5%)
Flowchart 18.3.2: Steps of hypernatremia in management.
•• Ringer lactate contains approximately serum potassium: 5 mEq/L.
HYPERNATREMIA Hypernatremia is relatively less common than hyponatremia and, sometimes, iatrogenic. A sodium concentration greater than 145 mmol/L is defined as hypernatremia. Very often these children present with irritability and convulsions with subdued signs of dehydration, hence mistaken as meningoencephalitis or intracranial hemorrhage. Serum electrolyte estimation in any child with convulsion and irritability is mandatory to recognize hypernatremia, otherwise it will be missed.
Management Objective of the management of hypernatremia is to provide adequate free water to reduce the serum sodium (Flowchart 18.3.2). •• Monitor hydration, urine output, electrolytes, glucose, and calcium 6th hourly and modify the therapy. •• Correct hypocalcemia, which is a common association with hypernatremia. •• Do not correct hyperglycemia, which is another common association unless it is persistent. •• Attempt to identify the cause. •• If renal failure or multiple electrolyte disturbances exist or refractory, decide on dialysis. •• Use vasopressin, if the cause is diabetes insipidus. •• Hypernatremia occurs due to two reasons: (1) Loss of water is more than that of sodium leading to hypernatremic dehydration, where the above said plan is the treatment. (2) Salt excess where it is due to addition of salt when the child is not having dehydration. Here the strategy is different. •• In acute salt poisoning or salt excess causing hypervolemic hypernatremia, management is different. Use frusemide
TABLE 18.3.3: Time taken for the correction on initial serum Na level. Serum Na (mEq)
Time taken (in hours)
to cause diuresis and replace the volume of urine output with one-fourth or one-half normal saline in 5% dextrose monitoring serum Na 4–6 hourly. 1. Initial emergency measures: Stabilization of airway, support of ventilation if needed, correction of shock with normal saline irrespective of serum sodium level and control of seizures. (Ringer lactate is not preferred because of its relative hypotonicity). Correction of hypernatremia should be done gradually. Time taken for the correction should be based on initial serum Na level (Table 18.3.3). Unlike in patients with hyponatremia or isonatremia with dehydration, fluid should not be run at a faster rate during the first 8 hours. It is administered at a constant rate over the whole period of correction. There are many methods and complex calculations available for correction of hypernatremia. But following is the simple and practical method
Intensive Care a. If there is shock, correct with normal saline boluses. Remember that Ringer lactate is not the preferred solution for correction of shock in hypernatremia, because it is relatively hypo-osmolar compared to the hyperosmolarity of the serum due to hypernatremia. b. After correction of shock, intravenous fluid therapy (IVF) should be one-fourth or one-half normal saline in 5% dextrose. 2. Hypernatremia causes shifting water from intracellular to extracellular compartment causing cellular dehydration. Hence the typical signs of dehydration are less pronounced and plasma volume is preserved unless there is very severe dehydration. If fluid loss is more than 10%, shock develops. As a result of cellular dehydration, brain shrinkage occurs leading to cerebral hemorrhage and thrombosis. Clinical features: Usual signs of dehydration are absent because of the preservation of ECF volume; doughy feeling of skin, irritability, seizures, and altered consciousness are the usual signs. Hyperglycemia and hypocalcemia are commonly found to be present in a child with hypernatremia. Investigation: Fluid balance charting, serum electrolytes, glucose, calcium, urea, creatinine, serum, and urine osmolality. Water deprivation test and CNS imaging are valuable if diabetes insipidus is suspected. 3. Initial starting fluid should contain (when there is no shock or after correction of shock) a. 40–75 mEq of sodium (one-fourth to one-half normal saline with 5% dextrose) and 20 mEq of potassium unless contraindicated. b. Total volume required in 24 hours should be 1.25–1.5 times the maintenance per 24 hours. c. When there is hypernatremia with edema, use the lower range (1.25 times maintenance) and otherwise use the higher range (1.5 times maintenance). This is applicable for all types of hypernatremia, whether hypovolemic, isovolemic or hypervolemic. 4. Reassess serum sodium concentration every 6 hourly and adjust the IV fluid based on clinical status and serum sodium. Expected rate of fall of serum sodium should be 0.5 mEq/hr or 12 mEq in 24 hours. One of the following four possibilities can occur and based on that, fluid is titrated. a. If sodium decreases too rapidly (>0.5 mEq/h) - It is advised to increase sodium concentration of IV fluid e.g.: One-fourth glucose normal saline (GNS) to one-third GNS to one-half GNS, or - Decrease rate of IV fluid administration e.g.: 1.5 times to 1.25 times to 1.0 time.
b. If sodium decreases too slowly ( 1/4 chest on X-ray), an intercostal drainage (ICD) may be placed to ensure complete evacuation of fluid and lung expansion. In case, there is inadequate expansion and relief within 48 hours, further assessment for possibility of encysted effusion/ empyema has to be considered. In the fibrinopurulent stage: An early ICD placement may avoid the need for further surgical intervention. However, in those cases when there is inadequate expansion in 24 hours, instillation of fibrinolytics has been recommended by many. Early VATS is an alternative option, which ensures complete evacuation of the pleural space and facilitates rapid recovery. In those patients who are seen beyond 1 week from the onset of the disease process, conservative and expectant
Pediatric Surgery line of treatment is not likely to help. Additionally, the presence of persistent pus in the pleural cavity increases the morbidity as well as the parenchymal damage. These patients most likely need VATS as a therapeutic assertion. However, an ICD [under ultrasound (USG) guidance] may be placed initially to reduce the toxicity till surgical expertise for the VATS is available. VATS decortication involves visualization of the pleural cavity, release of septations and loculations (Fig. 22.1.1), debridement of the pleural surfaces (Fig. 22.1.2), release of lung adhesions, opening of fissures, and clearance of diaphragmatic surface. In selected cases, small bronchopleural fistulae may be plugged with fibrin glue or stapled. Open thoracotomy should be the last resort in most cases of empyema, especially in view of the significant long-term morbidity of an open thoracotomy in children.
Suggested Treatment Protocol •• Clinical suspicion of empyema. •• Plain X-ray of chest: Posteroanterior (PA) view to diagnose and, if necessary, lateral view for further localization. –– In case of hydropneumothorax on plain X-ray—ICD directly (Fig. 22.1.3). –– In case of pure effusion—USG chest. •• USG chest: For confirmation in doubtful cases, e.g. whiteout (Fig. 22.1.4) and better localization and assessment. –– If free fluid on USG → pleural tapping preferably under USG guidance—diagnostic and adequate drainage. -- Assess cell counts and bacteriological assessment -- Modify antibiotics accordingly.
Fig. 22.1.1: Thoracoscopic view of the septations and
Fig. 22.1.3: Right hydropneumothorax.
22.1.2: Fibrinopurulent material retrieved during video-assisted thoracoscopic surgery (VATS) decortication.
Fig. 22.1.4: Complete whiteout on left side.
honeycombing within the pleura.
Partha’s Management Algorithms in Pediatric and Adolescent Practice
Figs 22.1.5A and B: Computed tomography (CT) chest showing the loculations and the thick peel.
–– If thick fluid → consider ICD. –– If inadequate drainage either dry tap or drainage of small quantity of supernatant fluid → possibility of loculations and septae → consider VATS decortication. –– If multiple loculations and septations on USG → consider VATS decortication. •• After tapping, review clinically and with repeat USG. If refills → consider ICD. •• After ICD, reassess after 48–72 hours. –– If adequate resolution—remove ICD after no significant fresh drainage.
–– If inadequate drainage → consider fibrinolytic therapy. -- If successful, review again after 48–72 hours as above -- In case of failed fibrinolytic therapy → VATS decortication. –– If bronchopleural fistula suspected (air bubbling through ICD) → VATS decortication. •• VATS decortication should be preceded by CT chest to accurately localize effusion and loculations besides assessing the condition of the underlying parenchyma (Figs 22.1.5A and B).
22.2 Abdominal Pain in Children Ketan Parikh
INTRODUCTION The abdomen has often been referred to as a pandora’s box. This is because very often the clinical presentation and investigative conclusions in abdominal conditions are rivaled by the final diagnosis. This enigma reiterates the need for systematic evaluation of every patient of abdominal pain to avoid long-term morbidity or, at times, even mortality. The term acute abdominal pain refers to a sudden, severe pain in the abdomen of short duration—usually of 24–48 hours. In contrast, the term acute abdomen indicates a much graver clinical condition (symptom complex) with
other manifestations besides the acute abdominal pain, more particularly: vomiting, abdominal distension, fever, constipation or diarrhea. There may also be an element of general toxicity due to dehydration or septicemia. The term recurrent abdominal pain has conventionally been defined by Apley’s definition as: a child having three bouts of abdominal pain significantly affecting his activities over 3 months for more than a year. On studying the original reference of Apley, one realizes that this definition was not arrived by any prognostic relevance but was only a criterion to evaluate children from a school-based survey. Therefore, the definition has been modified in the subsequent years according to the social and cultural needs of the society.
Pediatric Surgery The term chronic abdominal pain has been variably used for either recurrent abdominal pain or a persistent low-grade abdominal pain which may temporarily disturb an activity.
PATHOPHYSIOLOGY Clinically, three categories of pain may arise from the abdomen: 1. Visceral (splanchnic) pain: Usually dull, poorly localized, and experienced in the midline,) results from stimulation of a viscus with a stimulus like stretching or ischemia. Most forms of recurrent abdominal pain or chronic abdominal pains are of the visceral variety and are, therefore, experienced in the midline. –– Visceral pain: Arising from paired structures like ureter or adnexae is usually felt in the flanks. This may be acute and of lesser intensity. 2. Parietal pain: Usually sharp, discrete, localized, and aggravates on coughing or movement, results from inflammation of the parietal peritoneum. 3. Referred pain: Characteristics of parietal pain but is felt in remote areas supplied by the same dermatome as the diseased organ. A classic example: abdominal pain experienced due to the supra-diaphragmatic irritation of pleura due to lobar pneumonia. 4. The fact that abdominal pain is so elusive is often a cause for it being mislabeled as functional or psychogenic in some cases. However, it needs to be emphasized that the labeling of this pain as psychogenic should be an opinion of exhaustive exclusion rather than a default assumption. In spite of the multitude of investigative armamentarium available, evaluation of a child with abdominal pain relies significantly on the clinical assessment and its correlation with the investigations. In doubtful situations, repeated physical examination by the same physician is often useful.
CLINICAL ASSESSMENT OF A CHILD WITH ACUTE ABDOMEN History of the Pain Acute Abdomen The chronology of the clinical presentation and the age of the child are important in the evaluation. In the acute surgical abdomen, pain generally precedes vomiting, while the reverse is true in medical conditions. Most preadolescents have a poor sense of onset or location of pain. In children who cannot verbalize, the initial symptoms of nausea or periumbilical (visceral) pain may go
unnoticed, and thus they often present at the second stage of visceral pain. A neonate or an infant with abdominal pain may present as excessive crying, transient apneic spells or even grunting. These subtle symptoms require astute supervision to raise an early alarm to avoid missing lifethreatening surgical emergencies. Pain preceding vomiting—possibly surgical abdomen. Vomiting preceding pain—more likely medical abdomen
Associated symptoms, which have a significant bearing, are: •• Vomiting: –– Nonbilious vomiting: -- Common with a routine gastroenteritis -- It can also be an early feature of a surgical abdomen (intussusception) or an inflammatory cause (like acute appendicitis). -- Persistent nonbilious vomiting along with abdominal pain at any age may also be seen in peritonitis. These patients may have fever/ abdominal distension and constipation. -- Some of the urogenital causes of acute abdomen also may have nonbilious vomiting. ◊ Pyelonephritis—hypertension, urine examination, and abdominal ultrasonography (USG). ◊ Testicular torsion—clinical examination. ◊ Ovarian torsion—USG or other imaging. ◊ Renal colics—dysuria/USG. –– Bilious vomiting, at almost any age, signifies an intestinal obstruction unless proved otherwise. •• Abdominal distension: It is usually a late sign in most cases of acute abdomen. It may result from– –– Gas (aerophagia)—predominant gaseous distension is more a feature of hypoperistalsis. –– Intestinal obstruction—usually distension is a late symptom and may present as visible/palpable loops of bowel. –– Fluid in the peritoneal cavity—in an acute abdomen, this may suggest peritonitis. –– If accompanied by acute constipation or obstipation → surgical cause. –– A neonate with a scaphoid abdomen or distension restricted to upper half could possibly either be: -- A high bowel atresia (duodenal/high jejunal). -- A midgut volvulus due to an intestinal malrotation, especially if there is a delayed onset of intestinal obstruction. Whereas either case will need a surgical exploration, a delay in surgically exploring a case of midgut volvulus could lead to extensive gangrene of the entire midgut which is
Partha’s Management Algorithms in Pediatric and Adolescent Practice incompatible with normal life without lifelong total parenteral nutrition (TPN)/intestinal transplantation. -- In those cases where the abdominal distension is more generalized, a lower intestinal obstruction may be considered. –– If accompanied with diarrhea -- Severe enterocolitis (there would also be frequent passage of flatus) or -- Peritonitis (there is usually infrequent passage of flatus and minimal fecal content) and, therefore, is a strong indication for a surgical opinion. A bogginess in the prerectal space on a per-rectal examination would almost be diagnostic of a pelvic peritonitis. -- Acute appendicitis in a preschool child may present directly as peritonitis because of the subtlety of the early symptoms and the rapid progression of the pathology at this age. •• Constipation: Unlike chronic constipation, acute constipation usually has an organic cause. –– Absolute constipation (obstipation—failure to pass feces/flatus) → strongly suggests a surgical cause of acute abdomen. –– Frequent passage of flatus in a patient with abdominal distension → possibly medical cause/ pneumonia. –– Chronic constipation preceding abdominal pain → more likely primary constipation. –– Constipation following vomiting and abdominal pain → more likely surgical. Child may pass 1 or 2 normal stools and constipation may set in later once the bowel distal to the obstruction is completely evacuated. In neonates with a congenital bowel obstruction, there may be a history of passage of some meconium but in most cases, direct questioning of the neonate’s attendant may reveal that this meconium was pale colored. In those neonates where the acute abdomen has set in a few days after the passage of normal meconium, the possibility of volvulus with malrotation becomes higher. –– In the occasional patient with a high obstruction and peritonitis, the baby may pass loose motions due to peritoneal irritation of the distal bowel but this would be predominantly mucoid and not bile containing. •• Fever: –– If the acute abdominal pain follows a febrile illness, it is likely to be secondary to gastroenteritis or gastritis resulting from the medications. –– If fever follows an acute abdominal pain episode for 1–3 days → possibility of localized or generalized
peritonitis/liver abscess or some other inflammatory pathology of the abdomen or even chest. Loose motions: Note content of loose motions: –– If feculent—more chances of enteritis/colitis. –– If mucoid—need to consider surgical causes. Bleeding per rectum: –– In infants—(typical currant jelly stools—mucoid mixed with blood), high chances of intussusception. –– If content is predominantly altered blood with mild colics: Consider bleeding due to a Meckel’s diverticulum. –– After 2 years of age—mild bleeding with feces with abdominal pain (not severe)—possibility of rectal juvenile polyps leading to localized intussusception. Dysuria: –– In case with lateralized colics—renal colics. –– If right-sided pain with dysuria, the possibility of acute appendicitis with ipsilateral ureteric/vesical irritation needs to be considered. A history of recent trauma may indicate the cause of pain.
Additional History •• Cough or shortness of breath, and chest pain point to a thoracic source. •• Polyuria and polydipsia suggest diabetes mellitus. •• Joint pain, rash, and smoke-colored urine suggest Henoch–Schönlein purpura. •• High fever with toxicity: Acute hemolytic syndromes like malaria/dengue which may mimic an acute abdomen. •• Medication: A detailed drug history, especially— salicylates/NSAIDS/steroids. •• Gynecologic history: Relevant, especially in adolescent girls.
Past History All previous hospitalizations or significant illnesses, e.g. sickle cell anemia/porphyria. A history of previous surgery or peritonitis may suggest adhesive obstruction.
Family History Family history of sickle cell/or cystic fibrosis may indicate the diagnosis.
CLINICAL EXAMINATION General Examination A throat examination to identify enlarged tonsils and a chest examination to rule out a pleural/pulmonary pathology are important complementary clinical guides.
Pediatric Surgery •• Gait: Whereas a child with colic writhes with pain, children with peritonitis remain quiet, still, and resist movement. A patient with acute appendicitis typically walks with a stoop to the right. •• Vital signs: Dehydration should be assessed, fever (its severity as above). Kussmaul’s respiration indicates diabetic ketoacidosis. •• Abdominal examination: The abdominal movements should be observed and the patient should be asked to distend the abdomen and then flatten it. Restricted abdominal movements indicate a peritoneal irritation in that area. –– Visible contusions would suggest a traumatic etiology. –– A definite examination of the hernial orifices is mandatory in any case of acute abdominal pain since a strangulated hernia may present as an acute abdomen (Fig. 22.2.1). –– Mottling of the abdomen may indicate an inflammatory etiology, especially in a neonate (Fig. 22.2.2). –– Tenderness of abdomen needs to be evaluated with the palmar (flat) surface of the fingers rather than the tips of the fingers. In a crying child, it is best evaluated between breaths whereas other children may be asked to take deep breaths with an open mouth to relax the abdominal muscles. Since smaller children are not able to localize parietal pain well, guarding/rigidity is more relevant than tenderness. –– Deeper palpation is necessary to discover masses and organomegaly. –– A per-rectal examination may reveal: -- A loaded rectum leading to constipation and abdominal, pain, or -- A bogginess in the pelvis indicative of peritonitis, or -- Blood in the rectum in case of an intussusception, or -- A palpable rectal polyp.
Fig. 22.2.1: A strangulated left inguinal hernia in a small female patient.
INVESTIGATIONS These should be tailored to the patient’s symptoms and clinical findings. Hemogram: Leukocytosis, especially in the presence of a shift to the left and toxic granulations in the peripheral smear, indicates an infection. Urinalysis—pyuria, hematuria—may suggest a renal cause or a gastrointestinal (GI) cause irritating the urinary tract, e.g. appendicitis. Plain vertical abdominal X-ray is as important as abdominal USG.
Erect/lateral decubitus plain abdominal films: •• Gas under diaphragm → major GI perforation. •• Fluid levels → bowel obstruction (Fig. 22.2.3).
Fig. 22.2.2: Mottling of the abdominal wall in an infected meconium peritonitis.
Fig. 22.2.3: Multiple fluid levels (mid-intestinal obstruction).
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Ground glass appearance with or without fluid levels— peritonitis (Fig. 22.2.4). Chest radiographs: Gas under diaphragm/pneumonia/ raised diaphragm due to a liver abscess. Abdominal USG (results are operator dependent): Most useful in diagnosing gynecologic pathology such as ovarian cysts, ovarian torsion, or periappendiceal inflammation (Fig. 22.2.5). It, however, can miss acute appendicitis in as much as 20% of the cases and also various other bowel pathologies. Abdominal computed tomography (CT) involves radiation exposure and may require the use of contrast agents. CT may be necessary if excessive bowel gas precludes ultrasonographic examination.
Diagnostic laparoscopy: It is increasingly being used prior to a surgical intervention and thus may avoid a formal laparotomy in selected cases. In the doubtful cases, it may also avoid delaying a surgical intervention. Some of these situations may include acute appendicitis, Henoch– Schönlein purpura, Meckels’ diverticulitis, idiopathic omental necrosis, and diagnosing a non-viable bowel loop (Figs 22.2.6 to 22.2.8), etc.
SPECIFIC CONDITIONS CAUSING AN ACUTE ABDOMEN Causes of abdominal pain according to age have been described in Table 22.2.1.
Fig. 22.2.4: Central fluid levels surrounded by groundglass
Fig. 22.2.6: Laparoscopic view of an acutely inflamed appendix
Fig. 22.2.5: Ultrasonographic picture of
Fig. 22.2.7: Idiopathic omental torsion with
appearance of peritonitis.
an acutely inflamed appendix.
(covered with surrounding omentum).
necrosis of the omentum.
Fig. 22.2.8: Laparoscopic view of gangrenous bowel.
TABLE 22.2.1: Causes of abdominal pain according to age. Infants
Almost any age
Pelvic inflammatory disease
Urinary tract infection
Cholecystitis Hepatitis Pancreatitis Inflammatory bowel disease Trauma Mesenteric lymphadenitis Henoch–Schönlein purpura Intestinal obstruction Sickle cell crisis Meckel’s diverticulum
•• The duodenojejunal flexure is to the right of the midline. •• Cecum and the colon are to the left of the midline with bands extending from the cecum to the right lateral abdominal wall compressing the second part of the duodenum (Ladd’s bands). •• A very narrow mesenteric root which, therefore, permits a twist of the entire mid-gut (jejunum, ileum, and proximal colon) around the superior mesenteric artery. This twist is usually in the clockwise direction. In case of moderate degree of twist, the intestinal lumen gets compressed and leads to an intestinal obstruction from the duodenal level distally. In more severe twists, the superior mesenteric artery gets kinked and jeopardizes the blood supply of the entire midgut which, if left untreated, for long, can lead to gangrene of a variable portion of the gut. Spontaneous derotations are rare if complete obstruction has set in.
Clinical Features An apparently normal child who might have passed normal meconium and even normal milk stools for a few days suddenly develops: •• Bilious vomiting •• May have bleeding per rectum •• A scaphoid abdomen till gangrene and peritonitis set in •• Sudden dehydration sets in with early peritonitis and septicemia •• There may be history of one or two similar milder episodes which have settled on its own.
Investigations •• X-ray abdominal (erect) shows double bubble sign but there may be some gas shadows seen in the distal bowel. •• Ultrasonography abdomen: It will show a dilated stomach and duodenum. Doppler studies will document the reversal of axis of superior mesenteric artery and vein. It can also indicate the vascularity of the bowel. •• Computed tomography scan of abdomen: May be done in cases which are not an emergency to document the pathological anatomy. •• Gastrointestinal contrast studies: (In case CT scan is not possible). –– Barium (Ba) meal (only if not obstructed): Rightsided duodenojejunal flexure –– Barium enema: Left-sided colon.
It is the direct consequence of the most common type of malrotation of gut, in which the abnormal features are:
•• Quick rehydration and electrolyte correction. •• Emergency surgery before gangrene supervenes.
Partha’s Management Algorithms in Pediatric and Adolescent Practice –– Exploratory laparotomy –– Identifying the volvulus and derotation –– Releasing the Ladd’s bands –– Widening the root of the mesentery –– Rule out intrinsic duodenal obstruction –– Appendectomy SOS –– Closure. In a small proportion of cases the volvulus is known to recur; but, in most patients the postoperative course is uneventful. If on exploration bowel is non-viable even after derotation: •• If partly gangrenous, then resection with anastomosis, or •• Enterostomy with second stage closure but if a large part of the bowel is non-viable, then after derotation, the abdomen is closed with a second look laparotomy after 24–48 hours. In such cases the prognosis is quite poor.
Appendicitis Appendicitis is the most common surgical condition in children leading to an acute abdomen.
Epidemiology Western literature places the incidence as approximately 1 in 15 persons.
Clinical Presentations Whereas older children may show the classical pain— vomiting-fever chronology, younger children may present directly as peritonitis. Most children have the pain mainly in the periumbilical area with vomiting in a few and the pain then shifts to the right side only after localized peritonitis sets in.
situations. Laparoscopy enables a confirmation of the diagnosis in doubtful cases and also enables the surgical removal of the inflamed appendix in most cases.
Intussusception Common around 8–10 months of age—occurs due to a hypertrophied Peyer’s patch (acting as a lead point). Such a hypertrophy may occur due to a change in the bacterial flora during weaning or gastrointestinal infection or due to ingestion of infected respiratory secretions.
Clinical Features •• There may be a prior history of an upper respiratory tract infection. •• Colics—pain occurs in short bursts with intermittent periods of remission. •• Vomiting—bilious only in late cases. •• Red currant jelly stools—usually with no fecal matter after 1–2 times (a differentiating feature from dysentery). •• On abdominal palpation—intussusception may be felt as a banana-shaped mass with the concavity toward the umbilicus. Occasionally, the mass can be felt per rectally. •• In late cases—features of septicemia and peritonitis.
Investigations •• Ultrasound of the abdomen can be diagnostic. •• Alternatively, a barium enema—(Fig. 22.2.9) the coiled spring sign or the claw sign.
Investigations In spite of the various investigative modalities, acute appendicitis still remains a clinical diagnosis and investigations only have a supportive role.
Management Surgery remains the gold standard for the management of acute appendicitis, which avoids the morbidity and sequela of a missed perforation and its complications, in spite of some scattered publications experimenting with the conservative treatment of early appendicitis. In most advanced centeres, laparoscopic appendicectomy is rapidly becoming the standard of care for acute appendicitis with open surgical intervention being restricted to a few select
Fig. 22.2.9: Coil-spring shadow on barium (Ba) enema in a child with currant-jelly stools (inset).
Pediatric Surgery Pain not responding to antispasmodics, tender/palpable loop of bowel, evidence of perforation/ischemia on imaging and failure of conservative treatment for more than a few days, will be indications for surgical intervention.
Intestinal Obstruction Intestinal obstruction is uncommon in older children unless there is a previous history of abdominal surgery. Additional causes include volvulus, intussusception, incarcerated hernia, congenital or postinflammatory bands including intestinal tuberculosis. An erect abdominal X-ray would be diagnostic in most cases. A trial of conservative management under surgical supervision may be indicated in case there is no suspicion of bowel ischemia. Pain not responding to antispasmodics, tender/palpable loop of bowel, evidence of perforation/ischemia on imaging and failure of conservative treatment for more than a few days, are indications for surgical intervention. Pelvic inflammatory disease or primary peritonitis is typically seen in females and the diagnosis is mainly by laparoscopic/surgical exclusion of known causes of peritonitis.
Indications for Surgical Consultation in Children with Acute Abdominal Pain •• Severe or increasing abdominal pain with progressive signs of deterioration •• Bile-stained or feculent vomitus •• Involuntary abdominal guarding/rigidity •• Rebound abdominal tenderness •• Marked abdominal distension with diffuse tympany •• Signs of acute fluid or blood loss into the abdomen •• Significant abdominal trauma •• Suspected surgical cause for the pain •• Abdominal pain without an obvious etiology
ASSESSMENT OF A CHILD WITH CHRONIC ABDOMINAL PAIN History of the Pain A detailed story of the pain needs to be taken preferably from the patient himself or from the immediate caregiver. •• Onset of symptoms, if following an episode of conservatively treated acute abdomen, may indicate a chronic appendicitis. •• Location: Most chronic abdominal pains are experienced in the midline. In case they are experienced in the epigastrium, a suspicion of dyspeptic etiology needs to be considered. Pain arising from duodenal and
•• •• •• ••
pancreatic pathologies will also be experienced in the epigastrium. Hypogastric pain is possibly related to chronic constipation or lower urinary tract pathologies. Flank pain may indicate a renal etiology or even an ovarian/testicular etiology. Relation to food: Dyspeptic pain is often felt on empty stomach whereas pain related to chronic appendicitis/ biliary colics, etc, may be postprandial. Relief with passing feces: Most likely in cases of chronic constipation. Relation to urinary passage: Pain originating from uretero-vesico-urethral regions. Relation to certain other activities: Less pleasant activities like schooling may indicate a functional origin and pain occurring during pleasant activities like playing or watching favorite TV program could indicate a nonfunctional origin. Disturbance of sleep would be a strong indication of a nonfunctional origin (although inability to ascertain cause may force some of these pains to be labeled as functional). Heart-burn associated with pain may indicate dyspeptic origin although may also be seen in chronic appendicitis/ cholecystitis.
Associated Symptoms •• Nausea: May indicate dyspeptic origin or even chronic appendicitis/cholecystitis. •• Vomiting: Occasional vomiting with pain may indicate nonfunctional origin. •• Bleeding per rectum may be seen in rectal polyps. •• Increased frequency of stools (>4/day) would indicate irritable bowel syndrome (IBS). •• Photophobia with headache and a family history of migraine may suggest abdominal migraine. •• Dysuria: May indicate either vesical origin or vesical irritation.
Abdominal Examination •• Epigastric tenderness may indicate a pathology of dyspeptic origin. •• Epigastric fullness may indicate a chronic duodenal obstruction. •• Right hypochondriac tenderness/Murphy’s sign may indicate a biliary origin whereas a right iliac tenderness may indicate appendicular origin. •• Left iliac tenderness may be identified in cases of chronic constipation or even IBS. •• Renal angle tenderness may be seen in cases of renal origin of pain.
Partha’s Management Algorithms in Pediatric and Adolescent Practice •• Presence of inguinal hernia—may explain dull recurrent pain. •• A loaded rectum on per-rectal examination may suggest constipation as the cause of the pain.
Investigations •• Eosinophilia on a hemogram may indicate worm infestation. •• Microscopic hematuria or a significant crystalluria may indicate urological origin. •• Plain X-ray abdomen: –– Loaded colon in case of chronic constipation –– Bowel gas pattern—few fluid levels may indicate chronic obstruction as in chronic inflammations (e.g. abdominal tuberculosis) or a past history of peritonitis with adhesions. –– Calculous disease may be identified. •• Abdominal USG: Helps to diagnose cholelithiasis, renal/ ureteral/vesical diseases. May occasionally diagnose a chronically inflamed appendix. Chronic pancreatitis may be identified in some cases. Mesenteric adenitis if persistent over months, would indicate a chronic
infection like a chronic appendicitis. In older female children, the presence of ovarian cysts may give a clue to the cause of the pain. •• Computed tomography abdomen may complement the findings of abdominal USG.
Endoscopic Examination Upper GI endoscopy may be indicated in those specific situations like dysphagia, persistent dyspepsia whereas a lower GI endoscopy may be indicated in cases suspected to be having polyps.
Laparoscopy Diagnostic laparoscopy has been a significant addition in the armamentarium of investigations in the cases of persistent recurrent abdominal pain (RAP). Conditions, which are not diagnosed by imaging techniques, may get identified and can often be treated laparoscopically. Many of these conditions hitherto being treated as functional pain for many years, are now getting diagnosed to have a pathological origin.
22.3 Chronic Constipation in Children Ketan Parikh
INTRODUCTION Chronic constipation has been defined as delay or difficulty in defecation present for 2 or more weeks, which causes significant distress to the patient. It is definitely one of the most common symptoms in pediatric practice. Whereas most cases of chronic constipation (90–95%) are either functional or dietary in nature, a small percentage of children (5–10%) who have a pathological cause for their constipation will require to be identified by the primary care pediatrician and need to be attended to either medically or, at times, by the surgeon. Many of these surgical causes can be suspected clinically and early suspicion helps in early diagnosis, timely management and significant alleviation of symptoms for the patient and the distraught parents (Table 22.3.1).
DETAILED HISTORY •• Onset and progress of constipation: Most cases of pathological constipation date back to early infancy
or even neonatal age. In contrast, dietary constipation rarely starts before late infancy (usually after 6–9 months of age). A neonatal history of delayed passage of meconium (beyond 24 hours) and persistent passage of meconium (beyond 5 days) is highly suggestive of Hirschsprung’s disease (HD). Most cases of pathological constipation rarely respond adequately to oral laxatives whereas dietary constipation responds well initially, before becoming refractory. TABLE 22.3.1: Causes of chronic constipation in children. Medical
Drugs (e.g. antispasmodics/ codeine)/hypothyroidism
Spinal pathologies: Tethered cord, meningocoele, cord lipoma, etc.
Hypokalemia; hypo-/ hypercalcemia
Anal anomalies (ectopic anus, anal stenosis, vulval anus, and anteposed anus), pelvic masses, and chronic intestinal obstruction
Pediatric Surgery •• Dietary history: Persistent bottle-feeding, high percentage of dairy/maida products and low-fiber diet are important reasons for persistence of dietary constipation. •• Previous history of spinal surgery (meningomyelocoele, etc.), may be elicited. •• Encopresis: It is a feature common with dietary constipation but is rare with HD. •• A history of urinary incontinence will suggest a spinal pathology. •• Massive abdominal distension—a feature specific to HD. In most other cases, there may be mild distension. This is because, in HD, there is inability to pass flatus also whereas in most other causes of constipation, flatus is passed easily and the encopresis invariably accompanies the flatus.
EXAMINATION FINDINGS •• Abdominal distension is a strong indicator of HD. In most other varieties of constipation, fecalomas may be palpable per abdomen but in HD, the transverse colon may be palpable. •• General and associated features of primary medical disease may be evident, e.g. hypothyroidism, hypocalcemia, neurological impairment, etc. Children with HD are usually undernourished whereas children with dietary constipation are often wellfed. •• Spinal examination may reveal a suggestion of an intraspinal pathology. •• Perineal examination may reveal; –– An anteposed anus (anus is anteriorly placed with respect to the superficial sphincter), or –– A lax anal opening due to a neurological pathology. –– Spotting of undergarments would usually go against the diagnosis of HD. •• Per rectal examination, if done, will reveal: –– Lowly placed fecalomas in case of dietary constipation –– An empty rectum in case of HD. –– A forceful/explosive passage of flatus (with/without feces) on withdrawal of the finger in case of HD. –– Pelvic masses may be identified, e.g. presacral teratoma, etc.
•• Abdominal sonography or other modes of imaging the abdomen have limited relevance in chronic constipation except on suspicion of certain causes of chronic intestinal obstruction like abdominal cysts, tuberculosis, pelvic masses, etc. •• Barium enema for the diagnosis HD is a totally different investigation as compared to the barium enema performed routinely and the radiologists need to be sensitized accordingly. No bowel preparation should be done prior to the procedure, the procedure should be done without inflating a Foley’s balloon in the rectum and with minimal barium instilled under imaging—just enough to document the narrow segment and the “cone” of hypoganglionosis merging to ganglionic bowel. A 24hour film will document retention of the barium in the colon. A classical “cone” is diagnostic of HD but in the equivocal cases, a full thickness rectal biopsy becomes mandatory. •• Anorectal manometry is the only reliable investigation to diagnose ultrashort segment HD and needs to be resorted to in case this is being considered. •• Suction rectal biopsy helps to diagnose HD but full thickness rectal biopsy is the gold standard in the diagnosis of HD. •• Medical conditions like hypothyroidism, calcium metabolism disorders, etc. leading to chronic constipation need to be assessed clinically and biochemically. Causes of severe constipation by age group have been described in Table 22.3.2.
HIRSCHSPRUNG’S DISEASE The incidence of HD ranges from 1:4,000 to 1:700 newborns in various series. It is a condition characterized by aganglionosis of a variable length of the terminal bowel leading to absence of propulsive peristalsis in this segment
TABLE 22.3.2: Causes of severe constipation by age group. Age group
•• •• •• •• •• ••
•• Functional constipation •• Milk protein allergy •• Intestinal neuronal dysplasia
•• Functional constipation •• Dietary constipation •• Intestinal pseudo-obstruction
INVESTIGATIONS •• Plain X-ray of the abdomen: –– Fecal impaction in most cases of chronic constipation –– Dilated gas filled colon in a case of HD –– Dilated small bowel loops in case of chronic intestinal obstruction –– Absent rectal gas shadow in a neonate with HD.
Intestinal atresia/stenosis Meconium plug syndrome Intestinal duplication Small left colon syndrome Meconium ileus Imperforate anus
Partha’s Management Algorithms in Pediatric and Adolescent Practice
Normal peristaltic activity involves a wave of relaxation preceding a wave of contraction. Parasympathetic innervation is essential for this wave of relaxation.
•• Rarely, attacks of foul-smelling diarrhea interspersed with constipation may confuse the picture. •• In chronic cases—child fails to thrive and is malnourished. •• Abdominal distension—usually marked (Fig. 22.3.1), the transverse colon is often visible (Fig. 22.3.2). •• On rectal examination: The rectum is felt to be empty (Figs 22.3.3A and B), and on withdrawal of the examining finger, there is an explosive passage of flatus and feces.
In HD, the parasympathetic ganglia are absent from the Meissner and Auerbach’s plexuses of the affected bowel. The aganglionic bowel is normal in caliber but because of the absence of the peristaltic activity, the wave of relaxation is absent thus leading to a functional obstruction. The chronic obstruction leads to massive dilatation and hypertrophy of the normally ganglionated proximal bowel. The junctional portion of the bowel often shows a section of hypoganglionosis with partial function. The mucosa in the proximal hypertrophied portion of the bowel may show ulceration and hyperplasia due to chronic inflammation subsequent to stagnation. Depending on the zone of aganglionosis, the condition is classified as: 1. Short-segment HD: This is the most common variant whereby the aganglionosis extends up to rectum or sigmoid region. 2. Long-segment HD: When the aganglionosis extends proximal to the sigmoid region. 3. Ultrashort-segment HD: This is a condition wherein the aganglionosis is restricted to the internal sphincter and thus is rarely identifiable on a routine full thickness rectal biopsy. This condition can usually be diagnosed only by anorectal manometry.
•• A plain X-ray of the abdomen may show features of intestinal obstruction—large dilated colon loops. It may also show absence of rectal gas shadow.
and resultant intestinal obstruction. The aganglionosis starts at the anorectal junction and extends proximally for a variable length.
Fig. 22.3.1: Abdominal distension in case of Hirschsprung’s disease (HD).
Clinical Features •• The clinical profile often depends on the age of presentation. •• At birth: There is failure to pass meconium for more than 48 hours after birth and gradual onset of intestinal obstruction (95% of normal newborns pass meconium within the first 24 hours and another 5% within 48 hours). Even if the baby passes meconium with some assistance, he again may get obstructed. There may be a persistent passage of meconium even until 1 week of life. •• In case they are not diagnosed at birth and seen later— constipation dating back to early infancy is an important history. •• Intestinal obstruction—may manifest at any age which may be relieved by enemas.
Fig. 22.3.2: Visible and palpable transverse colon.
Figs 22.3.3A and B: (A) Per rectal findings in a case of habitual constipation; (B) Hirschsprung’s disease (HD).
TABLE 22.3.3: Differential diagnoses of Hirschsprung’s disease and idiopathic constipation. Hirschsprung’s disease
Usually >6 months
Radiology: •• Megacolon •• Transitional zone
•• Almost always •• Almost always
•• Common •• No
Missing rectoanal reflex
Positive rectoanal reflex
Missing ganglion cells
Normal ganglion cells
Normal activity (acetylcholinesterase staining)
•• Barium enema [essential to do with isotonic contrast, without overdistending the rectum, without a preprocedure evacuation enema and under screening or image intensifier television (IITV) control]: a coneshaped colon at the junction of the ganglionic and aganglionic segments. •• Anorectal manometry: It may strongly suggest the diagnosis of HD. •• The final confirmation is only with a full thickness biopsy of the rectal wall which will show absence of ganglion cells in the rectal muscle wall (Table 22.3.3).
TREATMENT •• Conservative treatment involving repeated enemas/ suppositories may help in overcoming an acute attack of intestinal obstruction and may help to postpone the surgery. However, many infants may not respond at all to conservative treatment and may need an emergency surgery. In such a case, an emergency colostomy may be the only option to save the child. •• Definitive surgical treatment: The principle of the surgery is the removal of the aganglionic segment of colon and ensuring that normal ganglionic bowel is anastomosed to the terminal rectum as close as possible to the anal canal. Traditionally, this surgery is done in stages but in recent years, single stage surgery is being done with variable success rates. •• Various commonly performed surgeries include the modified Duhamel’s procedure, Swenson’s procedure, and Soave’s procedure. Either of these may be performed by regular laparotomy or by laparoscopic methods.
World Health Organization Standard Algorithms Contributors: M Govindaraj, Suman MG Reviewer: Remesh Kumar
¯¯ Integrated Management of Childhood Illnesses ¯¯ Early Infant Diagnosis ¯¯ Malaria Management ¯¯ Dengue Fluid Management
Partha’s Management Algorithms in Pediatric and Adolescent Practice
23.1 Integrated Management of Childhood Illnesses M Govindaraj, Suman MG
INTRODUCTION Integrated Management of Childhood Illnesses (IMCI) clinical guidelines are based on the following principles: Examining all sick children up to 5 years of age for general danger signs and all infants for signs of very severe diseases. These signs indicate severe illness and the need for immediate referral or admission to hospital. The children and infants are then assessed for main symptoms: In older children, the main symptoms include: •• Cough or difficulty in breathing •• Diarrhea •• Fever, and •• Ear infection. In infants, the main symptoms include: •• Local bacterial infection •• Diarrhea, and •• Jaundice.
In addition, all sick children are routinely checked for: •• Nutritional and immunization status •• HIV status in high HIV settings, and A combination of individual signs leads to a child’s classification within one or more symptom groups rather than a diagnosis. The classification of illness is based on a color-coded triage system: •• “Pink” indicates urgent hospital referral admission; “Yellow” indicates initiation of specific outpatient treatment; and “Green” indicates supportive home care. Integrated Management of Childhood Illnesses management procedures use a limited number of essential drugs and encourage active participation of caregivers in the treatment of their children. An essential component of IMCI is the counseling of caregivers regarding home care: •• Appropriate feeding and fluids •• When to return to the clinic immediately, and •• When to return for follow-up.
World Health Organization protocols for assessing the sick child
World Health Organization Standard Algorithms
Partha’s Management Algorithms in Pediatric and Adolescent Practice
World Health Organization Standard Algorithms
Partha’s Management Algorithms in Pediatric and Adolescent Practice
World Health Organization Standard Algorithms
Partha’s Management Algorithms in Pediatric and Adolescent Practice
World Health Organization Standard Algorithms
Partha’s Management Algorithms in Pediatric and Adolescent Practice
World Health Organization Standard Algorithms Contd...
Partha’s Management Algorithms in Pediatric and Adolescent Practice
23.2 Early Infant Diagnosis M Govindaraj, Suman MG Early infant diagnosis is the testing of infants to determine their human immunodeficiency virus (HIV) status following possible exposure to HIV during pregnancy, delivery, and postpartum through breastfeeding (Flowchart 23.2.1). In view of high mortality in the first year of life among untreated HIV-infected infants, early HIV testing, prompt return of results, and rapid initiation of treatment are essential. Definitive confirmation of HIV in infants is by virological testing using nucleic acid testing (NAT) technologies. This is because transplacentally transmitted maternal HIV antibody may persist in a child up to 18 months of age preventing use of serological test. It is strongly recommended that all HIV-exposed infants have HIV virological testing at 4–6 weeks of age or at the earliest opportunity thereafter. The following assays (and respective specimen types) are strongly recommended for use: HIV deoxyribonucleic acid (DNA) on whole blood specimen or dry blood spot (DBS); HIV ribonucleic acid (RNA) on plasma or DBS; US p24 Ag on plasma or DBS for infant testing. In infants with an initial positive virological test result, it is strongly recommended that antiretroviral therapy (ART) be started without delay and, at the same time, a second
specimen be collected to confirm the initial positive virological test result. Immediate initiation of ART is lifesaving and should not be delayed while waiting for the results of the confirmatory test. In infants with signs or symptoms suggestive of HIV, it is strongly recommended they undergo HIV serological testing and, if positive (reactive), virological testing is to be done. Rapid diagnostic tests for HIV serology can be used at 9 months to rule out HIV infection in asymptomatic HIVexposed infants. Infants who have reactive serological assays at 9 months should have a virological test to identify HIV infection and start on ART. Children (18 months or older) with suspected HIV infection or HIV exposure should have serological testing performed according to the same standard diagnostic HIV serological testing algorithm used in adults. Cotrimoxazole prophylaxis is recommended for HIVexposed infants from 4–6 weeks of age and should be continued until HIV infection has been excluded by an age-appropriate HIV test to establish final diagnosis after complete cessation of breastfeeding.
World Health Organization Standard Algorithms Flowchart 23.2.1: WHO-testing strategy for early infant diagnosis: Consolidated guidelines on the use of antiretroviral drugs for treating and preventing HIV infection
Based on these revised Guidelines addition of NAT at birth to the existing testing algorithm can be considered. POC NAT can be used to diagnose HIV infection at birth, but positive results should be confirmed using laboratory-based NAT assays, because of limited experience with POC assays close to birth. bStart ART, without delay. At the same time, retest to confirm infection. As maternal treatment is scaled up and MTCT transmission rates decrease, false-positive results are expected to increase and re-testing after a first positive NAT is important to avoid unnecessarily treatment, particularly in settings with lower transmission rates. If the second test is negative, a third NAT should be done before interrupting ART. cFor children who were never breastfed additional testing following a negative NAT at 4-6 weeks is included in this algorithm to account for potential false-negative NAT results. dSigns and symptoms suggestive of HIV (oral thrush, recurrent or severe bacterial infections such as pneumonia or sepsis, FTT/wasting or AIDS indicator condition http://www.who.int/hiv/pub/paediatric/infants2010/en/. eIf infant presents with signs and symptoms of HIV disease (see footnote d above) but NAT is unavailable, consider starting ART, especially if an antibody test is conducted and result positive at 9 months or later. A DBS specimen must be collected prior to starting treatment for later NAT testing to confirm HIV diagnosis, because subsequent diagnostic testing while already on ART might be difficult to interpret. fIf infant presents with signs and symptoms of HIV disease (see footnote d above) consider starting ART while waiting for NAT result. However, another DBS specimen should be collected prior to starting treatment for later NAT testing to confirm HIV diagnosis. gRegular and periodic monitoring should be ensured while waiting for NAT to be available or for antibody testing to be conducted at 18 months. If infant presents with signs and symptoms of HIV disease should be managed as described previously (see footnote e). hThe risk of HIV transmission remains as long as breastfeeding continues. If the 9 months antibody testing is conducted earlier than 3 months after cessation of breastfeeding, infections acquired in the last days of breastfeeding may be missed so retesting at 18 months should be ensured for final assessment of HIV status. iIf breastfeeding beyond 18 months, final diagnosis of HIV status can only be assessed at the end of breastfeeding. If breastfeeding ends before 18 months, final diagnosis of HIV status with antibody testing can only be assessed at 18 months. Antibody testing should be undertaken at least 3 months after cessation of breastfeeding (to allow for development of HIV antibodies). For infants < 18 months of age positive antibody testing requires NAT to confirm infection. If infant is > 18 months, negative antibody testing confirms infant is uninfected; positive antibody testing confirms infant is infected a
Partha’s Management Algorithms in Pediatric and Adolescent Practice
23.3 Malaria Management M Govindaraj, Suman MG
INTRODUCTION Malaria control requires an integrated approach, including prevention (primarily vector control) and prompt treatment with effective antimalarial agents.
CLINICAL FEATURES Initial symptoms of malaria are nonspecific and similar to those of a minor systemic viral illness. They comprise headache, lassitude, fatigue, abdominal discomfort and muscle and joint pains, usually followed by fever, chills, perspiration, anorexia, vomiting, and worsening malaise. In young children, malaria may also present with lethargy, poor feeding, and cough. Severe malaria usually manifests with one or more of the following: coma (cerebral malaria), metabolic acidosis, severe anemia, hypoglycemia, acute renal failure or acute pulmonary edema.
DIAGNOSIS All cases of suspected malaria should have a parasitological test [(microscopy or rapid diagnostic test (RDT)] to confirm the diagnosis. In nearly all cases of symptomatic malaria, examination of thick and thin blood films by a competent microscopist will reveal malaria parasites. Malaria RDTs should be used if quality-assured malaria microscopy is not readily available. Uncomplicated malaria: A patient who presents with symptoms of malaria and a positive parasitological test (microscopy or RDT) but with no features of severe malaria. Severe falciparum malaria is defined as one or more of the following, occurring in the absence of an identified alternative cause and in the presence of Plasmodium falciparum asexual parasitemia. •• Impaired consciousness: A Glasgow coma score