PG Textbook of PEDIATRICS
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PG Textbook of PEDIATRICS Jaypee Brothers PG Textbook of PEDIATRICS Volume 2 INFECTIONS AND SYSTEMIC DISORDERS Piyush Gupta MD FAMS FIAP Professor Department of Pediatrics University College of Medical Sciences and Guru Teg Bahadur Hospital New Delhi, India PSN Menon MD FIAP Siddarth Ramji MD FNNF Consultant and Head Director-Professor Department of Pediatrics Department of Neonatology Jaber Al-Ahmed Armed Forces Hospital Maulana Azad Medical College Kuwait and Associated Lok Nayak Hospital New Delhi, India Rakesh Lodha MD Additional Professor JaypeeDepartment ofBrothers Pediatrics All India Institute of Medical Sciences New Delhi, India The Health Sciences Publisher New Delhi | London | Philadelphia | Panama Jaypee Brothers Medical Publishers (P) Ltd. Headquarters Jaypee Brothers Medical Publishers (P) Ltd. 4838/24, Ansari Road, Daryaganj New Delhi 110 002, India Phone: +91-11-43574357 Fax: +91-11-43574314 E-mail: [email protected] Overseas Offices J.P. Medical Ltd. Jaypee-Highlights Medical Publishers Inc. Jaypee Medical Inc. 83, Victoria Street, London City of Knowledge, Bld. 237, Clayton The Bourse SW1H 0HW (UK) Panama City, Panama 111, South Independence Mall East Phone: +44-20 3170 8910 Phone: +1 507-301-0496 Suite 835, Philadelphia, PA 19106, USA Fax: +44(0) 20 3008 6180 Fax: +1 507-301-0499 Phone: +1 267-519-9789 E-mail: [email protected] E-mail: [email protected] E-mail: [email protected] Jaypee Brothers Medical Publishers (P) Ltd. Jaypee Brothers Medical Publishers (P) Ltd. 17/1-B, Babar Road, Block-B, Shaymali Bhotahity, Kathmandu, Nepal Mohammadpur, Dhaka-1207 Phone: +977-9741283608 Bangladesh E-mail: [email protected] Mobile: +08801912003485 E-mail: [email protected] Website: www.jaypeebrothers.com Website: www.jaypeedigital.com © 2015, Piyush Gupta 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, photo copying, 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 practiceJaypee change constantly. This book is Brothersdesigned 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 contra indications. 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Inquiries for bulk sales may be solicited at: [email protected] PG Textbook of Pediatrics (3 Volumes) First Edition: 2015 ISBN: 978-93-5152-955-2 Printed at Contents Volume 1: General Pediatrics and Neonatology 3.3 Urea Cycle Disorders 73 PART I: Pediatrics—Yesterday, Today, Tomorrow Sunita Bijarnia-Mahay Section 1: INTRODUCTION TO PEDIATRICS 3.4 Organic Acidemias 80 Neerja Gupta Section Editors Piyush Gupta, PSN Menon 3.5 Fatty Acid Oxidation Defects 86 1.1 Evolution of Pediatrics 1 Manisha Goyal, Seema Kapoor KN Agarwal 3.6 Mitochondrial Disorders 90 1.2 20 Milestones in the History of Pediatrics 5 Sheffali Gulati, Biswaroop Chakrabarty Santosh K Bhargava 3.7 Peroxisomal Disorders 95 1.3 Status of Child Health 7 Madhulika Kabra, Neerja Gupta RN Srivastava 3.8 Lysosomal Storage Disorders 99 1.4 Millennium Development Goals for Child Health 10 IC Verma Raju C Shah, Pratima Shah, Kunal Ahya 3.9 GM1 and GM2 Gangliosidosis 106 1.5 Evidence-based Care in Pediatrics 14 Parag M Tamhankar Joseph L Mathew 3.10 Gaucher Disease 109 Mamta Muranjan PART II: Basic Sciences as Applied to Pediatrics 3.11 Niemann-Pick Disease 118 Section 2: GENETICS AND GENETIC DISORDERS Ashwin Dalal Section Editor Shubha R Phadke 3.12 Fabry Disease 122 Ratna Dua Puri 2.1 Gene, Genome and Genetic Basis of Diseases 19 Girisha KM 3.13 Defects of Carbohydrate Metabolism 126 Kausik Mandal 2.2 Patterns of Inheritance 24 Prajnya Ranganath, Shubha R Phadke 3.14 Glycogen Storage Diseases 132 Priya S Kishnani, Stephanie Austin 2.3 Clinical Dysmorphology: An Approach to Bedside Diagnosis 31 3.15 Pompe Disease 136 Ratna Dua Puri Mari Mori, Priya S Kishnani 2.4 Chromosomal Disorders 36 3.16 Mucopolysaccharidoses 139 Jaypee BrothersGirisha KM Shagun Aggarwal, Ashwin Dalal 2.5 Management of Genetic Disorders 3.17 Approach to Hypoglycemia 143 and Genetic Counseling 46 Meenakshi Bothra, Vandana Jain Shubha R Phadke 3.18 Porphyrias 149 2.6 Prevention of Genetic Disorders 52 Meenal Agarwal, Shubha R Phadke Neerja Gupta, Madhulika Kabra 3.19 Newborn Screening 153 Seema Kapoor, Madhulika Kabra Section 3: METABOLIC DISORDERS Section Editors Madhulika Kabra, Neerja Gupta Section 4: IMMUNITY, IMMUNE DISORDERS 3.1 Approach to Inborn Errors of Metabolism 57 AND ALLERGY Chitra Prasad, CA Rupar Section Editors Surjit Singh, Amit Rawat 3.2 Defects of Amino Acid Metabolism 65 4.1 Basics of Immunology 157 Anil B Jalan, Ketki V Kudalkar Amit Rawat, Biman Saikia vi 4.2 Laboratory Diagnosis of Immune-mediated 7.4 Pediatric Advanced Life Support 233 Diseases 163 Banani Poddar Biman Saikia, Deepti Suri 4.3 Primary Immunodeficiency Disorders 170 Section 8: INTENSIVE CARE Manisha Madkaikar Section Editor Rakesh Lodha 4.4 Therapy for Primary Immunodeficiency 8.1 Pediatric Intensive Care Unit 244 Disorders 177 Dhiren Gupta, Neeraj Gupta, Manpreet Sethi Revathi Raj, Surjit Singh 8.2 Shock 247 4.5 Intravenous Immunoglobulin 178 Srinivas Murthy, Niranjan Kissoon Sujoy Khan, Surjit Singh 8.3 Acute Respiratory Distress Syndrome 253 4.6 Approach to a Child with Allergic Disorder 183 Venkat Shankar H Paramesh 8.4 Respiratory Failure 259 Lokesh Guglani Section 5: FLUID AND ELECTROLYTES 8.5 Mechanical Ventilation 266 Section Editor Rakesh Lodha Dinesh Raj, Rakesh Lodha 5.1 Body Fluids 186 8.6 Sedation and Analgesia 274 Raghuvamsi Chaitra, Suchitra Ranjit Venkat Shankar 5.2 Maintenance Fluid Therapy 190 Banani Poddar, Kirti M Naranje Section 9: POISONING AND ENVENOMATION 5.3 Acid-Base Equilibrium 194 Section Editor Rakesh Lodha PG Textbook of Pediatrics Textbook PG Dinesh Raj, Rakesh Lodha 9.1 Clinical Approach to a Poisoned Child 280 5.4 Abnormalities of Acid-Base Balance 198 P Ramesh Menon Dinesh Raj, Rakesh Lodha 9.2 Poisonings by Common Drugs 285 5.5 Sodium and Related Disorders 203 Indumathy Santhanam, Sharada Sathish Andrew C Argent, Mignon I McCulloch 9.3 Hydrocarbon Poisoning 292 5.6 Potassium 208 Jayashree M, Karthi N Virendra Kumar 9.4 Poisoning in the Household 294 5.7 Magnesium Balance 211 Narayanan P, Harikrishnan KN Virendra Kumar 9.5 Corrosive Poisoning 302 Urmila Jhamb Section 6: DRUGS AND THERAPEUTICS Section Editor Sandeep B Bavdekar 9.6 Snakebite 306 S Mahadevan, R Ramesh Kumar 6.1 Principles of Drug Therapy 214 9.7 Scorpion Sting 316 Sandeep B Bavdekar S Mahadevan, R Ramesh Kumar 6.2 Administration of MedicationsJaypee 218 Brothers Jeeson C Unni Section 10: CHILDHOOD INJURIES Section Editor Rakesh Lodha PART III: Intensive Care and Emergencies 10.1 Trauma: Stabilization, Triage and Transport 323 Shruti Kant, Manu Madhok Section 7: ACUTELY ILL CHILD AND 10.2 Drowning/Submersion Injuries 328 RESUSCITATION Arun Bansal Section Editor Rakesh Lodha 10.3 Animal Related Injuries 330 7.1 Assessment and Triage 223 Arun Kumar Baranwal Jayashree M, Sunit Singhi 10.4 Pediatric Burns 334 7.2 Cardiopulmonary Resuscitation 226 K Mathangi Ramakrishnan, V Jayaraman, Utpal Bhalala, Vijay Srinivasan T Mathivanan, V Srinivasan 7.3 Basic Life Support 230 10.5 Cold Injuries 342 Shalu Gupta Manu Madhok, Shruti Kant PART IV: The Newborn Infant Section 14: HIGH-RISK NEWBORN vii Section Editor Siddarth Ramji Section 11: NEONATAL PHYSIOLOGY AND 14.1 Recognition of High-risk Neonate 421 ORGANIZATION OF CARE Niranjan Thomas, Vijay Gupta Section Editor Siddarth Ramji 14.2 Birth Injuries 429 11.1 Fetal Physiology and Growth 349 Neeraj Gupta Deepak Chawla 14.3 Jaundice in the Newborn 435 11.2 Maternal Influence on the Fetus 352 Sheila S Mathai, Kannan Venkatnarayan Jayashree Mondkar, Swati Manerkar 14.4 Infant of Diabetic Mother 445 Srinivas Murki, Tejo Oleti Pratap 11.3 Transition of the Fetus to Newborn 355 Contents Siddarth Ramji 14.5 Neonatal Hypoglycemia 449 11.4 Neonatal Mortality and Morbidities: Shiv Sajan Saini, Praveen Kumar An Overview 358 14.6 Anemia and Polycythemia 455 Aparna Chandrasekaran, M Jeeva Sankar Venkataseshan Sundaram, Bikramjit Das 11.5 Organization of Neonatal