Nelson Textbook of Pediatrics

Part I: The Field of Pediatrics 1Overview of Pediatrics 2Quality and Safety in Healthcare for Children 3Ethics in Pediatric Care 4Cultural Issues in Pediatric Care 5Maximizing Children's Health: Screening, Anticipatory Guidance, and Counseling Part II: Growth, Development, and Behavior 6Overview and Assessment of Variability 6.1Assessment of Fetal Growth and Development 7The Newborn 8The First Year 9The Second Year 10Preschool Years 11Middle Childhood 12Adolescence 13Sexual Behavior 13.1The Development of Sexual Behavior 13.2Gender Identity Disorder (GID) 13.3Adolescent Homosexuality 14Assessment of Growth 15Developmental Screening and Surveillance 16Child Care: How Pediatricians Can Support Children and Famillies 17Separation, Loss, and Bereavement 18Sleep Medicine Part III: Child and Adolescent Psychology 19Assessment and Interviewing 20Psychological Treatment of Children and Adolescents 20.1Illness and Death 20.2Psychopharmacology 20.3Psychotherapy 20.4Psychiatric Hospitalization 21Psychosomatic Illness 22Vegetative Disorders 22.1Rumination Disorders 22.2Pica 22.3Enuresis (Bedwetting) 22.4Encopresis 23Habit and Tic Disorders 24Anxiety Disorders 25Mood Disorders 25.1Major Depression 25.2Dysthymic Disorder 25.3Bipolar Disorder 26Suicide and Attempted Suicide 27Anorexia Nervosa, Bulimia, and Binge Eating 28Disruptive Behavioral Disorders 29Pervasive Developmental Disorders and Childhood Psychosis 29.1Autistic Disorder 29.2Asperger's Disorder 29.3Childhood Disintegrative Disorder 29.4Rett's Disorder 29.5Childhood Schizophrenia 29.6Acute Phobic Hallucinations 30Patterns of Development and Function in the School-Aged Child 31Attention Deficit Hyperactivity Disorder 32Specific Reading Disability 32.1Language Development and Communication Disorders PART IV: Children with Special Needs 33Adoption 34Foster Care 35Impact of Violence on Children 35.1School Violence and Bullying 35.2Effect of War on Children 36Abuse and Neglect of Children 36.1Sexual Abuse 36.2Munchausen Syndrome by Proxy 36.3Parental Styles 37Failure to Thrive 38Developmental Disabilities and Chronic Illness 38.1Mental Retardation (Intellectual Disability) 39Organ Transplantation 40Pediatric Palliative Care: The Care of Children with Life-Limiting Illness 40.1Grief and Bereavement Part V: Nutrition 41Nutritional Requirements 42The Feeding of Infants and Children 43Food Insecurity, Hunger, and Undernutrition 44Overweight and Obesity 45Vitamin A Deficiency and Excess 46Vitamin B Complex Deficiency and Excess 46.1Thiamin (Vitamin B-1) 46.2Riboflavin (Vitamin B-2) 46.3Niacin 46.4Vitamin B-6 (Pyridoxine) 46.5Biotin 46.6Folate 46.7Vitamin B-12 (Cobalamin) 47Vitamin C Deficiency (Ascorbic Acid) 48Rickets and Hypervitaminosis D 49Vitamin E Deficiency 50Vitamin K Deficiency 51Micronutrient Deficiency Part VI: The Pathophysiology of Body Fluids and Fluid Therapy 52Electrolyte and Acid-Base Disorders 52.1Composition of Body Fluids 52.2Regulation of Osmolality and Volume 52.3Sodium 52.4Potassium 52.5Magnesium 52.6Phosphorus 52.7Acid-Base Balance 53Maintenance and Replacement Therapy 54Deficit Therapy 55Fluids and Electrolyte Treatment of Specific Disorders 55.1Acute Diarrhea and Oral Rehydration 55.2Diarrhea in Chronically Malnourished Children 55.3Pyloric Stenosis 55.4Perioperative Fluids Part VII: Pediatric Drug Therapy 56Pharmacogenetics, Pharmacogenomics, and Pharmacoproteins 57Principles of Drug Therapy 58Poisonings 59Herbal Medicines Part VIII: Critical Care Medicine 60Evaluation of the Sick Child in the Office and Clinic 61Injury Control 62Emergency Medical Services for Children 63Interfacility Transfer of the Critically Ill Infant and Child 64Monitoring Techniques for the Critically Ill Infant and Child 65Scoring Systems and Predictors of Mortality 66Pediatric Emergencies and Resuscitation 67Neurologic Emergencies and Stabilization 67.1Brain Death 68Shock 69Respiratory Distress and Failure 70Mechanical Ventilation 70.1Chronic Mechanical Ventilation 71Acute Care of the Multiple Trauma Victim 72Nutritional Stabilization 73Drowning 74Burn injuries 75Cold Injuries 76Anesthesia and Perioperative Care 77Pediatric Pain Management Part IX: Human Genetics 78The Genetic Approach in Pediatric Medicine 79The Human Genome 80Patterns of Genetic Transmission 81Cytogenetics 82Genetics of Common Disorders: Diagnosis and Management 83 Integration of Genetics into Pediatric Practice Part X: Metabolic Diseases 84An Approach to Inborn Errors of Metabolism 85Defects in Metabolism of Amino Acids 85.1Phenylalanine 85.2Tyrosine 85.3Methionine 85.4Cysteine/Cystine 85.5Tryptophan 85.6Valine, Leucine, Isoleucine, and Related Organic Acidemias 85.7Glycine 85.8Serine 85.9Proline 85.1-0Glutamic Acid 85.11Urea Cycle and Hyperammonemia (Arginine, Citrulline, Ornithine) 85.12Histidine 85.13Lysine 85.14Aspartic Acid (Canavan Disease) 86Defects in Metabolism of Lipids 86.1Disorders of Mitochondrial Fatty Acid beta-Oxidation 86.2Disorders of Very Long Chain Fatty Acids 86.3Disorders of Lipoprotein Metabolism and Transport: Epidemiology of Blood Lipids and Cardiovascular Disease 86.4Lipidoses 86.5Mucolipidoses 87Defects in Metabolism of Carbohydrates 87.1Glycogen Storage Diseases 87.2Defects in Galactose Metabolism 87.3Defects in Fructose Metabolism 87.4Defects in Intermediary Carbohydrate Metabolism Associated with Lactic Acidosis 87.5Defects in Pentose Metabolism 87.6Disorders of Glycoprotein Degradation and Structure 88Mucopolysaccharidoses 89 Disorders of Purine and Pyrimidine Metabolism 90Progeria 91The Porphyrias 92Hypoglycemia Part XI: Perinatal and Neonatal Medicine 93Overview of Mortality and Morbidity 94The Newborn Infant 94.1History in Neonatal Pediatrics 94.2Physical Examination of the Newborn Infant 94.3Routine Delivery Room Care 94.4Nursery Care 94.5Parent-Infant Bonding 95High-Risk Pregnancies 96The Fetus 96.1Fetal Growth and Maturity 96.2Fetal Distress 96.3Maternal Disease and the Fetus 96.4Maternal Medication and Toxin Exposure and the Fetus 96.5Teratogens 96.6Radiation 96.7Intrauterine Diagnosis of Fetal Disease 96.8Treatment and Prevention of Fetal Disease 97The High-Risk Infant 97.1Multiple Gestation Pregnancies 97.2Prematurity and Intrauterine Growth Retardation 97.3Post-Term Infants 97.4Large for Gestational Age 97.5Infant Transport 98Clinical Manifestations of Diseases in the Newborn Period 99Nervous System Disorders 99.1The Cranium 99.2Traumatic, Epidural, Subdural, and Subarachnoid Hemorrhage 99.3Intracranial-Intraventricular Hemorrhage and Periventricular Leukomalacia 99.4Brain Injury from Inflammation, Infection, and Medications 99.5Hypoxia-Ischemia 99.6Spine and Spinal Cord 99.7Peripheral Nerve Injuries 100Delivery Room Emergencies 101Respiratory Tract Disorders 101.1Transition to Pulmonary Respiration 101.2Apnea 101.3Congenital Central Hypoventilation Syndrome 101.4Respiratory Distress Syndrome (Hyaline Membrane Disease) 101.5 Transient Tachypnea of the Newborn 101.6Aspiration of Foreign Material (Fetal Aspiration Syndrome, Aspiration Pneumonia) 101.7Meconium Aspiration 101.8Persistent Pulmonary Hypertension of the Newborn (Persistent Fetal Circulation) 101.9Diaphragmatic Hernia 101.10Foramen of Morgagni Hernia 101.11Paraesophageal Hernia 101.12Eventration 101.13Extrapulmonary Extravasation of Air (Pneumothorax, Pneumomediastinum, Pulmonary Interstitial Emphysema) 101.14Pulmonary Hemorrhage 102 Digestive System Disorders 102.1Meconium Ileus in Cystic Fibrosis 102.2Neonatal Necrotizing Enterocolitis (NEC) 102.3Jaundice and Hyperbilirubinemia in the Newborn 102.4Kernicterus 103Blood Disorders 103.1Anemia in the Newborn Infant 103.2Hemolytic Disease of the Newborn (Erythroblastosis Fetalis) 103.3Plethora in the Newborn Infant (Polycythemia) 103.4Hemorrhage in the Newborn Infant 104Genitourinary System 105The Umbilicus 106Metabolic Disturbances 106.1Maternal Selective Serotonin Reuptake Inhibitors and Neonatal Behavioral Syndromes 106.2Fetal Alcohol Syndrome 107The Endocrine System 107.1Infants of Diabetic Mothers 107.2Hypoglycemia 108Dysmorphology 109Infections of the Neonatal Infant 109.1Pathogenesis and Epidemiology 109.2Modes of Transmission and Pathogenesis 109.3Immunity 109.4Etiology of Fetal and Neonatal Infection 109.5Epidemiology of Early- and Late-Onset Neonatal Infections 109.6Clinical Manifestations of Transplacental Intrauterine Infections 109.7Diagnosis 109.8Treatment 109.9 Complications and Prognosis 109.1'0Prevention Part XII: Adolescent Medicine 110The Epidemiology of Adolescent Health Problems 111Delivery of Health Care to Adolescents 111.1Legal Issues 111.2Screening Procedures 111.3Health Enhancement 112Violent Behavior 113Substance Abuse 113.1Alcohol 113.2Tobacco 113.3Marijuana 113.4Volatile Inhalants 113.5Hallucinogens 113.6Cocaine 113.7Amphetamines 113.8Opiates 113.9Anabolic Steroids 114The Breast 115Menstrual Problems 115.1Amenorrhea 115.2Abnormal Uterine Bleeding 115.3Dysmenorrhea 115.4Premenstrual Syndrome 116Contraception 116.1Barrier Methods 116.2Spermicides 116.3Combination Methods 116.4Hormonal Methods 116.5Emergency Contraception 116.6Intrauterine Devices 117Adolescent Pregnancy 118Adolescent Rape 119Sexually Transmitted Infections 120Chronic Fatigue Syndrome Part XIII: Immunology Section 1: Evaluation of the Immune Systems 121Evaluation of Suspected Immunodeficiency Section 2: The T-, B-, and NK-Cell Systems 122T Lymphocytes, B Lymphocytes, and Natural Killer Cells 123Primary Defects of Antibody Production 123.1Treatment of B-Cell Defects 124Primary Defects of Cellular Immunity 125Primary Combined Antibody and Cellular Immunodeficiencies 125.1Severe Combined Immunodeficiency (SCID) 125.2Combined Immunodeficiency (CID) 125.3Defects of Innate Immunity 125.4Treatment of Cellular or Combined Immunodeficiency 125.5Immune Dysregulation with Autoimmunity or Lymphoproliferation Section 3: The Phagocytic System 126Neutrophils 127Monocytes and Macrophages and Dendritic Cells 128Eosinophils 129Disorders of Phagocyte Function 130Leukopenia 131Leukocytosis Section 4: The Complement System 132The Complement System 133Disorders of the Complement System 133.1Evaluation of the Complement System 133.2Genetic Deficiencies of Complement Components 133.3Deficiencies of Plasma, Membrane, or Serosal Complement Control Proteins 133.4Secondary Disorders of Complement 133.5Treatment of Complement Disorders Section 5: 134Principles and Clinical Indications 135HSCT from Alternative Sources and Donors 136Graft vs Host Disease (GVHD) and Rejection 137Infectious Complications of HSCT 138Late Effects of HSCT Part XIV: Allergy 139Allergy and the Immunologic Basis of Atopic Disease 140Diagnosis of Allergic Disease 141Principles of Treatment of Allergic Disease 142Allergic Rhinitis 143Childhood Asthma 144Atopic Dermatitis (Atopic Eczema) 145Insect Allergy 146Ocular Allergies 147Urticaria and Angioedema (Hives) 148Anaphylaxis 149Serum Sickness 150Adverse Reactions to Foods 151Adverse Reactions to Drugs Part XV: Rheumatology 152Evaluation of Suspected Rheumatic Disease 153Treatment of Rheumatic Diseases 154Juvenile Rheumatoid Arthritis 155Ankylosing Spondylitis and Other Spondyloarthropathies 156Reactive Arthritis 157Systemic Lupus Erythematosus 157.1Neonatal Lupus 158Juvenile Dermatomyositis 159Scleroderma and Raynaud Syndrome 160BehVet's Disease 161Sjogren's Syndrome 162Hereditary Periodic Fever Syndromes 163Amyloidosis 164Sarcoidosis 165Kawasaki Disease 166Vasculitis Syndromes 166.1Henoch-Schonlein Purpura 166.2Takayasu Arteritis 166.3Polyarteritis Nodosa 166.4Wegener Granulomatosis 166.5Other Vasculitic Syndromes 167Musculoskeletal Pain Syndromes 167.1Fibromyalgia 167.2Complex Regional Pain Syndrome (Reflex Sympathetic Dystrophy 167.3Erythromelalgia 168Miscellaneous Conditions Associated With Arthritis Part XVI: Infectious Diseases Section 1. General Considerations 169Diagnostic Microbiology Section 2. Preventive Measures (NEW) 170(NEW) Immunization Practices 170.1(NEW) International Immunization Practices 171Infection Control and Prophylaxis 172Childcare and Communicable Diseases 173Health Advice for Children Traveling Internationally 174Fever 175Fever Without a Focus 176Sepsis, Septic Shock, and System Inflammatory Response Syndrome (SIRS) 177Infections in Immunocompromised Persons 178Infections Associated with Medical Devices Section 3. Antibiotic Therapy (NEW) 179 Principles of Antibacterial Therapy Section 4. Gram-Positive Bacterial Infections 180Staphylococcus 180.1Staphylococcus aureus 180.2Toxic Shock Syndrome 180.3Coagulase-Negative Staphylococci 181Streptococcus pneumoniae (Pneumococcus) 182Group A Streptococcus 182.1Rheumatic Fever 183Group B Streptococcus 184Non-Group A or B Streptococcus 184Enterococcus 186Diphtheria (Corynebacterium diphtheriae) 187Listeria monocytogenes 188Actinomyces 189Nocardia Section 5. Gram Negative Bacterial Infections 190Neisseria meningitidis (Meningococcus) 191Neisseria gonorrhoeae (Gonococcus) 192Haemophilus influenzae 193Chancroid (Haemophilus ducreyi) 194Pertussis (Bordetella pertussis and B. parapertussis) 195Salmonella 195.1Nontyphoidal Salmonellosis 195.2Enteric Fever (Typhoid Fever) 196Shigella 197Escherichia coli 198Cholera (Vibrio cholerae) 199Campylobacter 200Yersinia 200.1Yersinia enterocolitica 200.2Yersinia pseudotuberculosis 200.3Plague (Yersinia pestis) 201Aeromonas and Plesiomonas 201.1Aeromonas 201.2Plesiomonas shigelloides 202Pseudomonas, Burkholderia, and Stenotrophomonas 202.1Pseudomonas aeruginosa 202.2Burkholderia 202.3Stenotrophomonas 203Tularemia (Francisella tularensis) 204Brucella 205Legionella 206Bartonella 206.1Bartonellosis (Bartonella bacilliformis) 206.2Cat-Scratch Disease (Bartonella henselae) 206.3Trench Fever (Bartonella quintana) 206.4Bacillary Angiomatosis and Bacillary Peliosis Hepatis (Bartonellahenselae and Bartonella quintana) Section 6: Anaerobic Bacterial Infections 207Botulism (Clostridium botulinum) 208Tetanus (Clostridium tetani) 209Pseudomembranous Colitis (Clostridium difficile) 210Other Anaerobic Infections Section 7: Mycobacterial Infections 211 (NEW) Principles of Antimycobacterial Therapy 212Tuberculosis (Mycobacterium tuberculosis) 213Hansen Disease (Mycobacterium leprae) 214Nontuberculous Mycobacteria Section 8. Spirochectal Infections 215Syphilis (Treponema pallidum) 216Nonvenereal Treponemal Infections 216.1Yaws (Treponema pertenue) 216.2Bejel Endemic Syphilis (Treponema pallidum subsp. endemicum) 216.3Pinta (Treponema carateum) 217Leptospira 218Relapsing Fever (Borrelia) 219Lyme Disease (Borrelia burgdorferi) Section 9. Mycroplasmal Infections 220Mycoplasma pneumoniae 221Genital Mycoplasmas (Mycoplasma hominis, Mycoplasma Genitalium, and Ureaplasma urealyticum) Section 10. Chlamydial Infections 222Chlamydiophila pneumoniae 223Chlamydia trachomatis 223.1Trachoma 223.2Genital Tract Infections 223.3Conjunctivitis and Pneumonia in Newborns 223.4Lymphogranuloma Venereum (LGV) 224Psittacosis (Chlamydia psittaci) Section 11. Rickettsial Infections 225Spotted Fever Group Rickettsioses 225.1Rocky Mountain Spotted Fever (Rickettsia rickettsii) 225.2Mediterranean Spotted Fever or Boutonneuse Fever (Rickettsia conorii) 225.3Rickettsialpox (Rickettsia akari) 226Scrub Typhus (Orientia tsutsugamushi) 227Typhus Group Rickettsioses 227.1Murine Typhus (Rickettsia typhi) 227.2Epidemic Typhus (Rickettsia prowazekii) 228Ehrlichioses and Anaplasmosis 229Q Fever (Coxiella burnetii) Section 12. Fungal Infections 230 Principles of Antifungal Therapy 231Candida 231.1Neonatal Infections 231.2Infections in Immunocompetent Children and Adolescents 231.3Infections in Immunocompromised Children and Adolescents 231.4Chronic Mucocutaneous Candidiasis 232Cryptococcus neoformans 233Malassezia 234Aspergillus 234.1Hypersensitivity Syndromes 234.2Saprophytic (Noninvasive) Syndromes 234.3Invasive Disease 235Histoplasmosis (Histoplasma capsulatum) 236Blastomycosis (Blastomyces dermatitidis) 237Coccidioidomycosis (Coccidioides immitis) 238Paracoccidioides brasiliensis 239Sporotrichosis (Sporothrix schenckii) 240Zygomycosis (Mucormycosis) 241Pneumocystis carinii (P. jiveci) Section 13. Viral Infections 242Principles of Antiviral Therapy 243 Measles 244Rubella 245Mumps 246Polioviruses 247Nonpolio Enteroviruses 248Parvovirus B19 249Herpes Simplex Virus 250Varicella-Zoster Virus 251Epstein-Barr Virus 252Cytomegalovirus 253Roseola (Human Herpesviruses 6, 7) 254Human Herpesvirus 8 255Influenza Viruses 256Parainfluenza Viruses 257Respiratory Syncytial Virus 258Human Metapneumovirus 259 Adenoviruses 260 Rhinoviruses 261Coronavirus 261.1Severe Acute Respiratory Syndrome (SARS)-associated corona virus 262Rotaviruses, Calicivirus, and Astrovirus (new title) 263Human Papillomaviruses 264Arboviral Encephalitis in North America 265Arboviral Encephalitis Outside North America 265.1Venezuelan Equine Encephalitis 265.2Japanese Encephalitis 265.3Tick-Borne Encephalitis 266Dengue Fever and Dengue Hemorrhagic Fever 267Yellow Fever 268Other Viral Hemorrhagic Fevers 269Lymphocytic Choriomeningitis Virus (LCMV) 270Hantavirus Pulmonary Syndrome 271Rabies 272Polyomaviruses (JC virus and BK Virus) 273Acquired Immunodeficiency Syndrome (Human Immunodeficiency Virus) 274Human T-Cell Lymphotropic Viruses Types I and II 275Transmissible Spongiform Encephalopathies Section 14. Antiparasitic Therapy (NEW)276Principles of Antiparasitic Therapy Section 15. Protozoan Diseases 277Primary Amebic Meningoencephalitis 278Amebiasis 279Giardiasis and Balantidiasis 279.1Giardia lamblia 279.2Balantidiasis 280Cryptosporidium, Isospora, Cyclospora, and Microsporidia 281Trichomoniasis (Trichomonas vaginalis) 282Leishmaniasis (Leishmania) 283African Trypanosomiasis (Sleeping Sickness; Trypanosoma brucei complex) 284American Trypanosomiasis (Chagas Disease; Trypanosoma cruzi) 285Malaria (Plasmodium) 286Babesiosis (Babesia) 287Toxoplasmosis (Toxoplasma gondii) Section 16. Helminthic Diseases 288Ascariasis (Ascaris lumbricoides) 289Hookworms (Necator americanus and Ancylostoma) 289.1Cutaneous Larva Migrans 290Trichuriasis (Trichuris trichuria) 291Enterobiasis (Enterobius vermicularis) 292Strongyloidiasis (Strongyloides stercoralis) 293Lymphatic Filariasis (Brugia malayi, B. timori, and Wuchereria bancrofti) 294Other Tissue Nematodes 295Toxocariasis (Visceral and Ocular Larva Migrans) 296Trichinosis (Trichinella spiralis) 297Schistosomiasis (Schistosoma) 298Flukes (Liver, Lung, and Intestinal) 299Adult Tapeworm Infections 300Cysticercosis 301Echinococcosis (Echinococcus granulosus and E. multilocularis) Part XVII: The Digestive System Section 1. Clinical Manifestations of Gastrointestinal Disease 302Normal Digestive Tract Phenomena 303Major Symptoms and Signs of Digestive Tract Disorders Section 2. The Oral Cavity 304Development and Developmental Anomalies of the teeth 305Disorders of the Oral Cavity Associated with Other Conditions 306Malocclusion 307Cleft Lip and Palate 308Syndromes with Oral Manifestations 309Dental Caries 310Periodontal Diseases 311Dental Trauma 312Common Lesions of the Oral Soft Tissues 313Diseases of the Salivary Glands and Jaws 314Diagnostic Radiology in Dental Assessment Section 3. The Esophagus 315Embryology, Anatomy, and Function of the Esophagus 315.1Common Clinical Manifestations and Diagnostic Aids 316Congenital Anomalies: Esophageal Atresia and Tracheoesophageal Fistula 316.1Congenital Anomalies: Laryngotracheoesophageal Clefts 317Obstructing and Motility Disorders of the Esophagus 318Dysmotility 319Hiatal Hernia 320Gastroesophageal Reflux Disease (GERD) 320.1Complications of GERD 321Non-GERD Esophagitis 322Esophageal Perforation 323Esophageal Varices 324Ingestions 324.1Foreign Bodies in the Esophagus 324.2Caustic Ingestions Section 4. Stomach and Intestines 325Normal Development, Structure, and Function 326Pyloric Stenosis and Other Congenital Anomalies of the Stomach 326.1Hypertropic Pyloric Stenosis 326.2Congenital Gastric Outlet Obstruction 326.3Gastric Duplication 326.4Gastric Volvulus 326.5Hypertrophic Gastropathy 327Intestinal Atresia, Stenosis, and Malrotation 327.1Duodenal Obstruction 327.2Jejunal and Ileal Atresia and Obstruction 327.3Malrotation 328Intestinal Duplications, Meckel Diverticulum, and Other Remnants of the Omphalomesenteric Duct 328.1Intestinal Duplication 328.2Meckel Diverticulum and Other Remnants of the Omphalomesenteric Duct 329Motility Disorders and Hirschsprung Disease 329.1Chronic Intestinal Pseudo-Obstruction 329.2Functional Constipation 329.3Congenital Aganglionic Megacolon (Hirschsprung Disease) 329.4Intestinal Neuronal Dysplasia 329.5Superior Mesenteric Artery Syndrome, Wilkie Syndrome, Cast Syndrome, Arteriomesenteric Duodenal Compression Syndrome 330Ileus, Adhesions, Intussusception, and Closed-Loop Obstructions 330.1Ileus 330.2Adhesions 330.3Intussusception 330.4Closed-Loop Obstructions 331Foreign Bodies and Bezoars 331.1Foreign Bodies in the Stomach and Intestine 331.2Bezoars 332Peptic Ulcer Disease in Children 332.1Zollinger-Ellison Syndrome 333Inflammatory Bowel Disease 333.1Chronic Ulcerative Colitis 333.2Crohn Disease (Regional Enteritis, Regional lleitis, Granulomatous Colitis) 333.3Behcet Syndrome 334 Food Allergy (Food Hypersensitivity) 334.1Eosinophilic Gastroenteritis 335Disorders of Malabsorption 335.1Evaluation of Children with Suspected Intestinal Malabsorption 335.2Gluten-Sensitive Enteropathy (Celiac Disease) 335.3Other Malabsorptive Sydromes 335.4Intestinal Infections Associated with Malabsorption 335.5Immunodeficiency Disorders 335.6Immunoproliferative Small Intestinal Disease 335.7Short Bowel Syndrome 335.8Chronic Malnutrition 335.9Enzyme Deficiencies 335.1-0Liver and Biliary Disorders Causing Malabsorption 335.11Defects of Absorption or Transport 336Intestinal Transplantation in Children 337Acute Gastroenteritis in Children (new) 337.1Probiotics in Gastrointestinal Disorders (new) 338Chronic Diarrhea 338.1Diarrhea from Hormone-Secreting Tumors 339Recurrent Abdominal Pain of Childhood 340Acute Appendicitis 341Surgical Conditions of the Anus, Rectum, and Colon 341.1Anorectal Malformations 341.2Anal Fissure 341.3Perianal Abscess and Fistula 341.4Hemorrhoids 341.5Rectal Prolapse 341.6Pilonidal Sinus and Abscess 342Tumors of the Digestive Tract 343Inguinal Hernias Section 5. Exocrine Pancreas 344Embryology, Anatomy, and Physiology 344.1Anatomic Abnormalities 344.2Physiology 345Pancreatic Function Tests 346Disorders of the Exocrine Pancreas 347Treatment of Pancreatic Insufficiency 348Pancreatitis 348.1Acute Pancreatitis 348.2Chronic Pancreatitis 349Pseudocyst of the Pancreas 350Pancreatic Tumors Section 6. The Liver and Biliary System 351Morphogenesis of the Liver and Biliary System 352Manifestations of Liver Disease 352.1Evaluation of Patients with Possible Liver Dysfunction 353Cholestasis 353.1Neonatal Cholestasis 353.2Cholestasis in the Older Child 354Metabolic Diseases of the Liver 354.1Inherited Deficient Conjugation of Bilirubin (Familial Nonhemolytic Unconjugated Hyperbilirubinemia) 354.2Wilson Disease 354.3Indian Childhood Cirrhosis 354.4Neonatal Iron Storage Disease (NISD) 354.5Miscellaneous Metabolic Diseases of the Liver 355Viral Hepatitis 356Liver Abscess 357Liver Disease Associated with Systemic Disorders 358Mitochondrial Hepatopathies 359Autoimmune and Chronic Hepatitis 360Drug- and Toxin-Induced Liver Injury 361Fulminant Hepatic Failure 362Cystic Diseases of the Biliary Tract and Liver 363Diseases of the Gallbladder 364Portal Hypertension and Varices 365Liver Transplantation Section 7. Peritoneum 366Malformations 367Ascites 367.1Chylous Ascites 368Peritonitis 368.1Acute Primary Peritonitis 368.2Acute Secondary Peritonitis 368.3Acute Secondary Localized Peritonitis (Peritoneal Abscess) 369Epigastric Hernia 369.1Incisional Hernia Part XVIII: Respiratory System Section 1. Development and Function 370Respiratory Pathophysiology and Regulation 370.1Lung Volumes and Capacities in Health and Disease 370.2Chest Wall 370.3Pulmonary Mechanics and Work of Breathing in Health and Disease 370.4Airway Dynamics in Health and Disease 370.5Interpretation of Clinical Signs to Localize the Site of Pathology 370.6Ventilation-Perfusion (V/Q) Relationship in Health and Disease 370.7Gas Exchange in Health and Disease 370.8Interpretation of Blood Gases 370.9Pulmonary Vasculature in Health and Disease 370.1-0Immune Response of the Lung to Injury 370.11Regulation of Respiration 371Diagnostic Approach to Respiratory Disease 372Sudden Infant Death Syndrome Section 2. Disorders of the Respiratory Tract 373Congenital Disorders of the Nose 374Acquired Disorders of the Nose 374.1Foreign Body 374.2Epistaxis 375Nasal Polyps 376The Common Cold 377Sinusitis 378Acute Pharyngitis 379Retropharyngeal Abscess, Lateral Pharyngeal (Parapharyngeal) Abscess, and Peritonsillar Cellulitis/Abscess 380Tonsils and Adenoids 381Chronic or Recurrent Respiratory Symptoms 382Acute Inflammatory Upper Airway Obstruction (Croup, Epiglottitis, Laryngitis, and Bacterial Tracheitis) 382.1Infectious Upper Airway Obstruction 382.2Bacterial Tracheitis 383Congenital Anomalies of the Larynx, Trachea, and Bronchi 383.1 Laryngomalacia 383.2Congenital Subglottic Stenosis 383.3Vocal Cord Paralysis 383.4Congenital Laryngeal Webs and Atresia 383.5Congenital Subglottic Hemangioma 383.6Laryngoceles and Saccular Cysts 383.7Posterior Laryngeal Cleft (PLC) and Laryngotracheoesophageal Cleft (LTEC) 383.8Vascular and Cardiac Anomalies 383.9Tracheal Stenoses, Webs, and Atresia 383.1-0Foregut Cysts 383.11Tracheomalacia and Bronchomalacia 384Foreign Bodies of the Airway 384.1Laryngeal Foreign Bodies 384.2Tracheal Foreign Bodies 384.3Bronchial Foreign Bodies 385Laryngotracheal Stenosis, Subglottic Stenosis 385.1Congenital Subglottic Stenosis 385.2Acquired Laryngotracheal Stenosis 386Bronchomalacia and Tracheomalacia 387Neoplasms of the Larynx, Trachea, and Bronchi 387.1Vocal Nodules 387.2Recurrent Respiratory Papillomatosis (RRP) 387.3Congenital Subglottic Hemangioma 387.4Vascular Anomalies 387.5Other Laryngeal Neoplasms 387.6Tracheal Neoplasms 387.7Bronchial Tumors 388Wheezing, Bronchiolitis, and Bronchitis 388.1Wheezing in Infants and Bronchiolotis 388.2Bronchitis 389Emphysema and Overinflation 390alpha1-Antitrypsin Deficiency and Emphysema 391Other Distal Airway Diseases 391.1Bronciolitis Obliterans 391.2Follicular Bronchitis 391.3Pulmonary Alveolar Microlithiasis 392 Congenital Disorders of the Lung 392.1Pulmonary Agenesis and Aplasia 392.2Pulmonary Hypoplasia 392.3Cystic Adenomatoid Malformation 392.4 Pulmonary Sequestration 392.5Bronchogenic Cysts 392.6Congenital Pulmonary Lymphangiectasia 392.7 Lung Hernia 392.8Other Congenital Malformations of the Lung 393Pulmonary Edema 394Aspiration Syndromes 395Chronic Recurrent Aspiration 396 Parenchymal Disease with Prominent Hypersensitivity, Eosinophilic Infiltration, or Toxin-Mediated Injury 396.1Hypersensitivity to Inhaled Materials 396.2Silo Filler Disease 396.3Paraquat Lung 396.4Eosinophilic Lung Disease (Formerly Loffler Syndrome) 397Pneumonia 398Bronchiectasis 399Pulmonary Abscess 400Cystic Fibrosis 401 Primary Ciliary Dyskinesia (Immotile Cilia Syndrome) 402Interstitial Lung Diseases 403Pulmonary Alveolar Proteinosis 404Inherited Disorders of Surfactant Metabolism 405Pulmonary Hemosiderosis 406Pulmonary Hemorrhage, Embolism, and Infarction 406.1Pulmonary Embolus and Infarction 406.2Pulmonary Hemorrhage and Hemoptysis 407Atelectasis 408Pulmonary Tumors 409Pleurisy 409.1Dry or Plastic Pleurisy 409.2Serofibrinous Pleurisy 409.3Purulent Pleurisy or Empyema 410 Pneumothorax 411Pneumomediastinum 412Hydrothorax 413Hemothorax 414 Chylothorax 415 Bronchopulmonary Dysplasia 416Skeletal Diseases Influencing Pulmonary Function 416.1Pectus Excavatum (Funnel Chest) 416.2Pectus Carinatum and Sternal Clefts 416.3Asphyxiating Thoracic Dystrophy (Thoracic-Pelvic-Phalangeal Dystrophy) 416.4Achondroplasia 416.5Kyphoscoliosis: Adolescent Idiopathic Scoliosis and Congential Scoliosis 416.6Congenital Rib Anomalies 417Neuromuscular Diseases with Pulmonary Consequences 418Extrapulmonary Diseases with Pulmonary Manifestations 419 Chronic Severe Respiratory Insufficiency Part XIX: The Cardiovascular System Section 1. Developmental Biology of the Cardiovascular System 420Cardiac Development 420.1Early Cardiac Morphogenesis 420.2Cardiac Looping 420.3Cardiac Septation 420.4Aortic Arch Development 420.5Cardiac Differentiation 420.6Developmental Changes in Cardiac Function 421The Fetal to Neonatal Circulatory Transition 421.1The Fetal Circulation 421.2The Transitional Circulation 421.3The Neonatal Circulation 421.4Persistent Pulmonary Hypertension of the Neonate (Persistence of Fetal Circulatory Pathways) 421.5The Influence of Fetal Environmental Factors on Adult Cardiovascular and Metabolic Disease Section 2. Evaluation of the Cardiovascular System 422History and Physical Examination 423Laboratory Evaluation 423.1Radiologic Assessment 423.2Electrocardiography 423.3Hematologic Data 423.4Echocardiography 423.5Exercise Testing 423.6MRI, MRA, CT, and Radionuclide Studies 423.7Diagnostic and Interventional Cardiac Catheterization Section 3. Congenital Heart Diseases 424Epidemiology of Congenital Heart Disease 425Evaluation of the Infant or Child with Congenital Heart Disease 426Acyanotic Congenital Heart Disease: The Left-to-Right Shunt Lesions 426.1Atrial Septal Defect 426.2Ostium Secundum Defect 426.3Sinus Venosus Atrial Septal Defect 426.4Partial Anomalous Pulmonary Venous Return 426.5Atrioventricular Septal Defects (Ostium Primum and Atrioventricular Canal or Endocardial Cushion Defects) 426.6Ventricular Septal Defect 426.7Supracristal Ventricular Septal Defect with Aortic Insufficiency 426.8Patent Ductus Arteriosus 426.9Aorticopulmonary Window Defect 426.1-0Coronary-Arteriovenous Fistula (Coronary-Cameral Fistula) 426.11Ruptured Sinus of Valsalva Aneurysm 427Acyanotic Congenital Heart Disease: The Obstructive Lesions 427.1Pulmonary Valve Stenosis with Intact Ventricular Septum 427.2Infundibular Pulmonary Stenosis and Double-Chamber Right Ventricle 427.3Pulmonary Stenosis in Combination with an Intracardiac Shunt 427.4Peripheral Pulmonary Stenosis 427.5Aortic Stenosis 427.6Coarctation of the Aorta 427.7Coarctation with Ventricular Septal Defect 427.8Coarctation with Other Cardiac Anomalies and Interrupted Aortic Arch 427.9Congenital Mitral Stenosis 427.1-0Pulmonary Venous Hypertension 428Acyanotic Congenital Heart Disease: Regurgitant Lesions 428.1Pulmonary Valvular Insufficiency and Congenital Absence of the Pulmonary Valve 428.2Congenital Mitral Insufficiency 428.3Mitral Valve Prolapse 428.4Tricuspid Regurgitation 429Cyanotic Congenital Heart Disease: Evaluation of the Critically Ill Neonate with Cyanosis and Respiratory Distress 430Cyanotic Congenital Heart Lesions: Lesions Associated with Decreased Pulmonary Blood Flow 430.1Tetralogy of Fallot 430.2Tetralogy of Fallot with Pulmonary Atresia 430.3Pulmonary Atresia with Intact Ventricular Septum 430.4Tricuspid Atresia 430.5Double-Outlet Right Ventricle with Pulmonary Stenosis 430.6Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis 430.7Ebstein Anomaly of the Tricuspid Valve 431Cyanotic Congenital Heart Disease: Lesions Associated with Increased Pulmonary Blood Flow 431.1d-Transposition of the Great Arteries 431.2d-Transposition of the Great Arteries with Intact Ventricular Septum 431.3Transposition of the Great Arteries with Ventricular Septal Defect 431.4l-Transposition of the Great Arteries (Corrected Transposition) 431.5Double-Outlet Right Ventricle Without Pulmonary Stenosis 431.6Double-Outlet Right Ventricle with Transposition of the Great Arteries (Taussig-Bing Anomaly) 431.7Total Anomalous Pulmonary Venous Return 431.8Truncus Arteriosus 431.9Single Ventricle (Double-Inlet Ventricle, Univentricular Heart) 431.10Hypoplastic Left Heart Syndrome 431.11Abnormal Positions of the Heart and the Heterotaxy Syndromes (Asplenia, Polysplenia) 432Other Congenital Heart and Vascular Malformations 432.1Anomalies of the Aortic Arch 432.2Anomalous Origin of the Coronary Arteries 432.3Pulmonary Arteriovenous Fistula 432.4Ectopia Cordis 432.5Diverticulum of the Left Ventricle 433Pulmonary Hypertension 433.1Primary Pulmonary Hypertension 433.2Pulmonary Vascular Disease (Eisenmenger Syndrome) 434 General Principles of Treatment of Congenital Heart Disease 434.1The Adult with Congenital Heart Disease Section 4. Cardiac Arrhythmias 435Disturbances of Rate and Rhythm of the Heart 435.1Principles of Antiarrhythmic Therapy 435.2Sinus Arrhythmias and Extrasystoles 435.3Supraventricular Tachycardia 435.4Ventricular Tachyarrhythmias 435.5Long Q-T Syndrome 435.6Sinus Node Dysfunction 435.7AV Block 436 Sudden Death Section 5. Acquired Heart Disease 437Infective Endocarditis 438Rheumatic Heart Disease Section 6. Diseases of the Myocardium and Pericardium 439Diseases of the Myocardium 439.1Dilated Cardiomyopathy 439.2Hypertrophic Cardiomyopathy 439.3Restrictive Cardiomyopathies 439.4Myocarditis 439.5Nonviral Causes of Myocarditis 439.6Endocardial Fibroelastosis 440Diseases of the Pericardium 440.1Acute Pericarditis 440.2Constrictive Pericarditis 441Tumors of the Heart Section 7. Cardiac Therapeutics (Heart Failure and End-Stage Cardiac Disease) 442Heart Failure 442.1Cardiogenic Shock 443Pediatric Heart and Heart-Lung Transplantation 443.1Pediatric Heart Transplantation 443.2Heart-Lung and Lung Transplantation Section 8. Diseases of the Peripheral Vascular System 444Disease of the Blood Vessels (Aneurysms and Fistulas) 444.1Kawasaki Disease (see also Ch 165) 444.2Arteriovenous Fistulas 445Systemic Hypertension Part XX: Disease of the Blood Section 1. The Hematopoietic System 446Development of the Hematopoietic System 447The Anemias Section 2. Anemias of Inadequate Production 448Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia ) 449Pearson Marrow-Pancreas Syndrome 450Acquired Pure Red Blood Cell Anemias 451Anemia of Chronic Disease (ACD) and Renal Disease 451.1Anemia of Chronic Disease 451.2Anemia of Renal Disease 452Congenital Dyserythropoietic Anemias (CDA) 453Physiologic Anemia of Infancy 454Megaloblastic Anemias 454.1Folic Acid Deficiency 454.2Vitamin B12 (Cobalamin) Deficiency 454.3Other Rare Megaloblastic Anemias 455Iron-Deficiency Anemia 456Other Microcytic Anemias Section 3. Hemolytic Anemias 457Definitions and Classification of Hemolytic Anemias 458Hereditary Spherocytosis 459Hereditary Elliptocytosis 460Hereditary Stomatocytosis 461Other Membrane Defects 462Hemoglobinopathies 462.1Sickle Cell Disease 462.2Sickle Cell Trait (Hemoglobin AS) 462.3Other Hemoglobinopathies 462.4Unstable Hemoglobin Disorders 462.5Abnormal Hemoglobins with Increased Oxygen Affinity 462.6Abnormal Hemoglobins Causing Cyanosis 462.7Hereditary Methemoglobinemia 462.8Syndromes of Hereditary Persistence of Fetal Hemoglobin (HPFH) 462.9Thalassemia Syndromes 463Enzymatic Defects 463.1Pyruvate Kinase (PK) Deficiency 463.2Other Glycolytic Enzyme Deficiencies 463.3Glucose-6-Phosphate Dehydrogenase (G6PD) and Related Deficiencies 464Hemolytic Anemias Resulting from Extracellular Factors 465Hemolytic Anemias Secondary to Other Extracellular Factors Section 4. Polycythemia (Erythrocytosis) 466Primary Polycythemia (Polycythemia Rubra Vara) 467Secondary Polycythemia Section 5. The Pancytopenias 468The Constitutional Pancytopenias 469The Acquired Pancytopenias Section 6. Risks of Blood Component Transfusions 470Red Blood Cell Transfusions and Erythropoietin Therapy 471Platelet Transfusions 472Neutrophil (Granulocyte) Transfusions 473Fresh Frozen Plasma Transfusions 474Risks of Blood Transfusions Section 7. Hemorrhagic and Thrombotic Diseases 475Hemostasis 475.1The Clinical and Laboratory Evaluation of Hemostasis 476Hereditary Clotting Factor Deficiencies (Bleeding Disorders) 476.1Factor VIII or Factor IX Deficiency (Hemophilia A or B) 476.2 Factor IX Deficiency (Hemophilia C) 476.3Deficiencies of the Contact Factors (Non-bleeding Disorders) 476.4Factor VII Deficiency 476.5Factor X Deficiency 476.6Prothrombin (Factor II) Deficiency 476.7Factor V Deficiency 476.8Combined Deficiency of Factors V and VIII 476.9Fibrinogen Deficiency 476.10 Factor XIII Deficiency (Fibrin-Stabilizing Factor or Transglutaminase Deficiency) 476.11Antiplasmin or Plasminogen Activator Inhibitor (PAI) Deficiency 477von Willebrand Disease 478Hereditary Predisposition to Thrombosis 479Acquired Thrombotic Disorders 480Post-Neonatal Vitamin K Deficiency 481Liver Disease 482Acquired Inhibitors of Coagulation 483Disseminated Intravascular Coagulation 484Platelet and Blood Vessel Disorders 484.1Idiopathic Thrombocytopenic Purpura (ITP) 484.2Drug-Induced Thrombocytopenia 484.3Nonimmune Platelet Destruction 484.4Hemolytic-Uremic Syndrome (HUS) 484.5Thrombotic Thrombocytopenic Purpura (TTP) 484.6Kasabach-Merritt Syndrome 484.7Sequestration 484.8Congenital Thrombocytopenic Syndromes 484.9Neonatal Thrombocytopenia 484.10 Thrombocytopenia Due to Acquired Disorders Causing Decreased Production 484.11Platelet Function Disorders 484.12Acquired Disorders of Platelet Function 484.13Congenital Abnormalities of Platelet Function 484.14Disorders of the Blood Vessels Section 8. The Spleen 485Anatomy and Function of the Spleen 486Splenomegaly 487Hyposplenism, Splenic Trauma, and Splenectomy Section 9. The Lymphatic System 488Anatomy and Function of the Lymphatic System 489Abnormalities of Lymphatic vessels 490Lymphadenopathy Part XXI: Cancer and Benign Tumors 491Epidemiology of Childhood and Adolescent Cancer 492Molecular and Cellular Biology of Cancer 493Principles of Diagnosis 494Principles of Treatment 495The Leukemias 495.1Acute Lymphoblastic Leukemia 495.2Acute Myelogenous Leukemia 495.3Down Syndrome and Acute Leukemia and Myeloproliferation 495.4Chronic Myelogenous Leukemia 495.5Juvenile Chronic Myelocytic Leukemia 495.6Infant Leukemia 496Lymphoma 496.1Hodgkin's Disease 496.2Non-Hodgkin's Lymphoma (NHL) 497Brain Tumors in Childhood 498Neuroblastoma 499Neoplasms of the Kidney 500Soft Tissue Sarcomas 501Neoplasms of Bone 501.1Malignant Tumors of Bone 501.2Benign Tumors and Tumor-like Processes of Bone 502Retinoblastoma 503Gonadal and Germ Cell Neoplasms 504Neoplasms of the Liver 505Benign Vascular Tumors 505.1Hemangiomas 505.2Lymphangiomas and Cystic Hygromas 506Rare Tumors 506.1Thyroid Tumors 506.2Melanoma 506.3Nasopharyngeal Carcinoma 506.4Adenocarcinoma of the Colon and Rectum 506.5Adrenocortical Carcinoma 506.6Desmoplastic Small Round Cell Tumor 507Histiocytosis Syndromes of Childhood 507.1Class I Histiocytoses 507.2Class II Histiocytoses: Hemophagocytic Lymphohistiocytosis (HLH) 507.3Class III Histiocytoses Part XXII: Nephrology Section 1. Glomerular Disease 508Introduction to Glomerular Diseases 508.1Anatomy of the Glomerulus 508.2Glomerular Filtration 508.3Glomerular Diseases Section 2. Conditions Particularly Associated with Hematuria 509Clinical Evaluation of the Child with Hematuria 510Isolated Glomerular Disease with Recurrent Gross Hematuria 510.1IgA Nephropathy (Berger Nephropathy) 510.2Alport Syndrome 510.3Thin Glomerular Basement Membrane Disease 511Glomerulonephritis Associated with Infections 511.1Acute Poststreptococcal Glomerulonephritis 511.2Other Chronic Infections 512Membranous Glomerulopathy (Glomerulonephritis) 513Membranoproliferative (Mesangiocapillary) Glomerulonephritis 514Glomerulonephritis Associated With Systemic Lupus Erythematosus 515Henoch-Schonlein Purpura Nephritis 516Rapidly Progressive (Crescentic) Glomerulonephritis 517Goodpasture Disease 518Hemolytic-Uremic Syndrome 519Upper Urinary Tract Causes of Hematuria 519.1Interstitial Nephritis 519.2Toxic Nephropathy 519.3Cortical Necrosis 519.4Pyelonephritis 519.5Nephrocalcinosis 519.6Vascular Abnormalities 519.7Renal Vein Thrombosis 519.8Idiopathic Hypercalciuria 520Hematologic Diseases Causing Hematuria 520.1Sickle Cell Nephropathy 520.2Coagulopathies and Thrombocytopenia 521Anatomic Abnormalities Associated with Hematuria 521.1Congenital Anomalies 521.2Autosomal Recessive Polycystic Kidney Disease 521.3Autosomal Dominant Polycystic Kidney Disease 521.4Trauma 521.5Renal Tumors 522Lower Urinary Tract Causes of Hematuria 522.1Infectious Causes of Cystitis and Urethritis 522.2Hemorrhagic Cystitis 522.3Heavy Exercise 522.4Munchhausen by Proxy Section 3. Conditions Particularly Associated with Proteinuria 523Introduction to the Child with Proteinuria 524Transient Proteinuria 525Orthostatic (Postural) Proteinuria 526Fixed Proteinuria 526.1Glomerular Proteinuria 526.2Tubular Proteinuria 527Nephrotic Syndrome 527.1Idiopathic Nephrotic Syndrome 587.2Secondary Nephrotic Syndrome 527.3Congenital Nephrotic Syndrome Section 4. Tubular Disorders 528Tubular Function 529Renal Tubular Acidosis 529.1Proximal (Type II) Renal Tubular Acidosis 529.2Distal (Type I) Renal Tubular Acidosis 529.3Hyperkalemic (Type IV) Renal Tubular Acidosis 529.4Rickets Associated with Renal Tubular Acidosis 530Nephrogenic Diabetes Insipidus 531Bartter/Gitelman Syndromes and Other Inherited Tubular Transport Abnormalities 531.1Bartter Syndrome 531.2Gitelman Syndrome 531.3Other Inherited Tubular Transport Abnormalities 532Tubulointerstitial Nephritis Section 5. Toxic Nephropathies: Renal Failure 533Toxic Nephropathy 534Cortical Necrosis 535Renal Failure (Chap Head only; not chapter) 535.1Acute Renal Failure 535.2Chronic Kidney Disease 535.3End-Stage Renal Disease 536Renal Transplantation Part XXIII: Urologic Disorders in Infants and Children 537Congenital Anomalies and Dysgenesis of the Kidneys 538Urinary Tract Infections 539Vesicoureteral Reflux 540Obstruction of the Urinary Tract 541Anomalies of the Bladder 542Neuropathic Bladder 543Voiding Dysfunction 544Anomalies of the Penis and Urethra 545Disorders and Anomalies of the Scrotal Contents 546Trauma to the Genitourinary Tract 547Urinary Lithiasis Part XXIV: Gynecologic Problems of Childhood 548History and Physical Examination 549Vulvovaginitis 550Bleeding 551Breast Disorders 552Hirsutism 553Neoplasms 554Vulvovaginal and Mullerian Anomalies 555Special Gynecologic Needs 556Gynecologic Imaging Part XXV: The Endocrine System Section 1. Disorders of the Hypothalamus and Pituitary Gland 557Hormones of the Hypothalamus and Pituitary 558Hypopituitarism 559Diabetes Insipidus 559.1Causes of Hypernatremia 560Other Abnormalities of Arginine Vasopressin Metabolism and Action 561Hyperpituitarism 562Physiology of Puberty 563Disorders of Pubertal Development 563.1Gonadotropin-Dependent Precocious Puberty 563.2Precocious Puberty Resulting from Organic Brain Lesions 563.3Precocious Puberty Following Irradiation of the Brain 563.4Syndrome of Precocious Puberty and Hypothyroidism 563.5Gonadotropin-Secreting Tumors 563.6McCune-Albright Syndrome (Precocious Puberty with Polyostotic Fibrous Dysplasia and Abnormal Pigmentation) 563.7Familial Male Gonadotropin-Independent Precocious Puberty 563.8Incomplete (Partial) Precocious Development 563.9Medicational Precocity Section 2. Disorders of the Thyroid Gland 564Thyroid Development and Physiology 564.1Thyroid Hormone Studies 565Defects of Thyroxine-Binding Globulin 566Hypothyroidism 567Thyroiditis 568Goiter 568.1Congenital Goiter 568.2Endemic Goiter and Cretinism 568.3Acquired Goiter 568.4Intratracheal Goiter 569Hyperthyroidism 569.1Graves Disease 569.2Congenital Hyperthyroidism 570Carcinoma of the Thyroid 570.1Solitary Thyroid Nodule 570.2Medullary Carcinoma Section 3. Disorders of the Parathyroid Glands 571Hormones and Peptides of Calcium Homeostasis and Bone Metabolism 572Hypoparathyroidism 573Pseudohypoparathyroidism (Albright Hereditary Osteodystrophy 574Hyperparathyroidism Section 4 Disorders of the Adrenal Glands 575The Physiology of the Adrenal Gland 575.1Histology and Embryology 575.2Adrenal Steroid Biosynthesis 575.3Regulation of the Adrenal Cortex 575.4Adrenal Steroid Hormone Actions 575.5Adrenal Medulla 576Adrenocortical Insufficiency 576.1Primary Adrenal Insufficiency 576.2Secondary Adrenal Insufficiency 577Congenital Adrenal Hyperplasia and Related Disorders 577.1Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency 577.2Congenital Adrenal Hyperplasia due to 11beta-Hydroxylase Deficiency 577.3Congenital Adrenal Hyperplasia due to 3beta-Hydroxysteriod Dehydrogenase Deficiency 577.4Congenital Adrenal Hyperplasia due to 17-Hydroxylase Deficiency 577.5Lipoid Adrenal Hyperplasia 577.6Deficiency of P5450 oxioreductase (Antley-Bixler Syndrome) 577.7Aldosterone Synthase Deficiency 577.8Glucocorticoid-Suppressible Hyperaldosteronism 578Cushing Syndrome 579Primary Aldosteronism 580Adrenal Tumors 580.1Virilizing Adrenocortical Tumors 580.2Feminizing Adrenal Tumors 581Pheochromocytoma 582Adrenal Masses 582.1Adrenal Incidentaloma 582.2Adrenal Calcification Section 5. Disorders of the Gonads 583Development and Function of the Gonads 584Hypofunction of the Testes 584.1Hypergonadotropic Hypogonadism in the Male (Primary Hypogonadism) 584.2Hypogonadotropic Hypogonadism in the Male (Secondary Hypogonadism) 585Pseudoprecocity Resulting from Tumors of the Testes 586Gynecomastia 587Hypofunction of the Ovaries 587.1Hypergonadotropic Hypogonadism in the Female (Primary Hypogonadism) 587.2Hypogonadotropic Hypogonadism in the Female (Secondary Hypogonadism) 588Pseudoprecocity Due to Lesions of the Ovary 589Disorders of Sex Development (Intersex) 589.146,XX DSD 589.246,XY DSD 589.3Ovotesticular DSD Section 6. Diabetes Mellitus In Children 590Diabetes Mellitus 590.1Introduction and Classification 590.2Type 1 Diabetes Mellitus (Immune Mediated) 590.3Type 2 Diabetes Mellitus (T2DM) 590.4Other Specific Types of Diabetes Part XXVI: Neurology 591Neurologic Evaluation 592Congenital Anomalies of the Central Nervous System 592.1Neural Tube Defects (Dysraphism) 592.2Spina Bifida Occulta 592.3Meningocele 592.4Myelomeningocele 592.5Encephalocele 592.6Anencephaly 592.7Disorders of Neuronal Migration 592.8Agenesis of the Corpus Callosum 592.9Agenesis of the Cranial Nerves 592.10Microcephaly 592.11Hydrocephalus 592.12Craniosynostosis 593Seizures in Childhood 593.1Febrile Seizures 593.2Unprovoked Seizures 593.3Partial Seizures 593.4Generalized Seizures 593.5Mechanisms of Seizures 593.6Treatment of Epilepsy 593.7Neonatal Seizures 593.8Status Epilepticus 594Conditions that Mimic Seizures 595Headaches 595.1Migraine 595.2Organic Headaches 595.3Tension or Stress Headaches 596Neurocutaneous Syndromes 596.1Neurofibromatosis 596.2Tuberous Sclerosis 596.3Sturge-Weber Syndrome 596.4Von Hippel-Lindau Disease 596.5Linear Nevus Syndrome 596.6PHACE Syndrome 597Movement Disorders 597.1Ataxias 597.2Chorea, Athetosis, Tremor 597.3Dystonia 597.4Tics 598Encephalopathies 598.1Cerebral Palsy 598.2Mitochondrial Encephalomyopathies 598.3Other Encephalopathies 599Neurodegenerative Disorders of Childhood 599.1Sphingolipidoses 599.2Neuronal Ceroid Lipofuscinoses 599.3Adrenoleukodystrophy 599.4Sialidosis 599.5Miscellaneous Disorders 600Demyelinating Disorders of the CNS 600.1Multiple Sclerosis (MS) 600.2Acute Disseminated Encephalomyelitis 601Acute Stroke Syndromes 601.1Arterial Thrombosis/Embolism 601.2Venous Thrombosis 601.3Intracranial Hemorrhage 601.4Differential Diagnosis of Stroke-Like Events 602Central Nervous System Infections 602.1Acute Bacterial Meningitis Beyond the Neonatal Period 602.2Viral Meningoencephalitis 602.3Eosinophilic Meningitis 603Brain Abscess 604Pseudotumor Cerebri 605Spinal Cord Disorders 605.1Tethered Cord 605.2Diastematomyelia (Split Spinal Cord Malformation) 605.3Syringomyelia 605.4Spinal Cord Tumors 605.5Spinal Cord Trauma 605.6Transverse Myelitis 605.7Arteriovenous Malformation Part XXVII: Neuromuscular Disorders 606Evaluation and Investigation 607Developmental Disorders of Muscle 607.1Myotubular Myopathy 607.2Congenital Muscle Fiber-Type Disproportion (CMFTD) 607.3Nemaline Rod Myopathy 607.4Central Core Disease and Minicore Myopathy 607.5Myofibrillar Myopathies 607.6Brain Malformations and Muscle Development 607.7Amyoplasia 607.8Muscular Dysgenesis (Proteus Syndrome Myopathy) 607.9Benign Congenital Hypotonia 607.1'0Arthrogryposis 608Muscular Dystrophies 608.1Duchenne and Becker Muscular Dystrophies 608.2Emery-Dreifuss Muscular Dystrophy 608.3Myotonic Muscular Dystrophy 608.4Limb-Girdle Muscular Dystrophy 608.5Facioscapulohumeral Muscular Dystrophy 608.6Congenital Muscular Dystrophy 609Endocrine and Toxic Myopathies 610Metabolic Myopathies 610.1Periodic Paralyses (Potassium-Related) 610.2Malignant Hyperthermia 610.3Glycogenoses 610.4Mitochondrial Myopathies 610.5Lipid Myopathies 610.6Vitamin E Deficiency Myopathy 611Disorders of Neuromuscular Transmission and of Motor Neurons) 611.1Myasthenia Gravis 611.2Spinal Muscular Atrophies 611.3Other Motor Neuron Disorders 612Hereditary Motor-Sensory Neuropathi 612.1Peroneal Muscular Atrophy (Charcot-Marie-Tooth Disease, HMSN Type I) 612.2Peroneal Muscular Atrophy (Axonal Type) 612.3Dejerine-Sottas Disease (HMSN Type III) 612.4Roussy-Levy Syndrome 612.5Refsum Disease 612.6Fabry Disease 612.7Giant Axonal Neuropathy 612.8Congenital Hypomyelinating Neuropathy 612.9Tomaculous Neuropathy 612.10Leukodystrophies 613Toxic Neuropathies 614Autonomic Neuropathies 614.1Familial Dysautonomia 614.2Other Autonomic Neuropathies 615Guillain-Barre Syndrome 616Bell Palsy Part XXVIII: Disorders of the Eye 617Growth and Development 618Examination of the Eye 619Abnormalities of Refraction and Accommodation 620Disorders of Vision 621Abnormalities of Pupil and Iris 622Disorders of Eye Movement and Alignment 623Abnormalities of the Lids 624Disorders of the Lacrimal System 625Disorders of the Conjunctiva 626Abnormalities of the Cornea 627Abnormalities of the Lens 628Disorders of the Uveal Tract 629Disorders of the Retina and Vitreous 630Abnormalities of the Optic Nerve 631Childhood Glaucoma 632Orbital Abnormalities 633Orbital Infections 633.1Orbital Cellulitis 633.2Dacroadenitis and Dacrocystadenitis 634Injuries to the Eye Part XXIX: The Ear 635The Ear 636Hearing Loss 637Congenital Malformations 638External Otitis (Otitis Externa) 639Otitis Media 640The Inner Ear and Diseases of the Bony Labyrinth 641Traumatic Injuries of the Ear and Temporal Bone 642Tumors of the Ear and Temporal Bone Part XXX: The Skin 643Morphology of the Skin 644Evaluation of the Patient 644.1Cutaneous Manifestations of Systemic Diseases 645Principles of Therapy 646Diseases of the Neonate 647Cutaneous Defects 648Ectodermal Dysplasias 649Vascular Disorders 650Cutaneous Nevi 651Hyperpigmented Lesions 652Hypopigmented Lesions 653Vesiculobullous Disorders 654Eczematous Disorders 655Photosensitivity 656Diseases of the Epidermis 657Disorders of Keratinization 658Diseases of the Dermis 659Diseases of Subcutaneous Tissue 660Disorders of the Sweat Glands 661Disorders of Hair 662Disorders of the Nails 663Disorders of the Mucous Membranes 664Cutaneous Bacterial Infections 665Cutaneous Fungal Infections 666Cutaneous Viral Infections 667Arthropod Bites and Infestations 668Acne 669Tumors of the Skin 670Nutritional Dermatoses Part XXXI: Bone and Joint Disorders Section 1. Orthopaedic Problems 671Growth and Development 672Evaluation of the Child 673The Foot and Toes 673.1Metatarsus Adductus 673.2Calcaneovalgus Feet 673.3Talipes Equinovarus (Clubfoot) 673.4Congenital Vertical Talus 673.5Hypermobile Pes Planus (Flexible Flatfeet) 673.6Tarsal Coalition 673.7Cavus Feet 673.8Osteochondroses 673.9Puncture Wounds of the Foot 673.10Toe Deformities 673.11Painful Foot 673.12Shoes 674Torsional and Angular Deformities 674.1Normal Developmental of Limb 674.2Evaluation 674.3Torsional Deformities 674.4Coronal Plane Deformities 674.5Congenital Angular Deformities of the Tibia and Fibula 675Leg Length Discrepancy 676The Knee 676.1Discoid Lateral Meniscus 676.2Popliteal Cyst 676.3Osteochondritis Dissecans 676.4Osgood-Schlatter Disease 676.5Idiopathic Adolescent Anterior Knee Pain Syndrome 676.6Patellar Subluxation and Dislocation 677The Hip 677.1Developmental Dysplasia of the Hip 677.2Transient Monoarticularsynovitis (Toxic Synovitis) 677.3Legg-Calve-Perthes Disease 677.4Slipped Capital Femoral Epiphysis 678The Spine 678.1Idiopathic Scoliosis 678.2Congenital Scoliosis 678.3Neuromuscular Scoliosis, Genetic Syndromes, and Compensatory Scoliosis 678.4Kyphosis 678.5Back Pain in Children 678.6Spondylolysis and Spondylolisthesis 678.7Disk Space Infection 678.8Intervertebral Disk Herniation/Slipped Vertebral Apophysis 678.9Tumors 679The Neck 679.1Torticollis 679.2Klippel-Feil Syndrome 679.3Cervical Anomalies and Instabilities 680The Upper Limb 681Arthrogryposis 682Common Fractures 682.1Unique Characteristics of Pediatric Fractures 682.2Pediatric Fracture Patterns 682.3Upper Extremity Fractures 682.4Fractures of Lower Extremity 682.5Operative Treatment 682.6Complications of Fractures in Children 682.7Outcomes Assessment 683Osteomyelitis (new title) 684Supperative Arthritis (Septic Arthritis) Section 2. Sports Medicine 685Epidemiology and Prevention 686Management of Musculoskeletal Injury 686.1Growth Plate Injuries 686.2Shoulder Injuries 686.3Elbow Injuries 686.4Low Back Injuries 686.5Hip and Pelvis Injuries 686.6Knee Injuries 686.7Lower Leg Pain: Shin Splints and Stress Fractures and Chronic Compartment Syndrome 686.8Ankle Injuries 686.9Foot Injuries 687Head and Neck Injuries 688Heat Injuries 689Female Athletes: Menstrual Problems and Risk for Osteopenia 690Ergogenic Aids 691Specific Sports and Associated Injuries Section 3. The Skeletal Dysplasias 692General Considerations 693Disorders of Involving Cartilage Matrix Proteins 694Disorders Involving Transmembrane Receptors 695Disorders Involving Ion Transporter 696Disorders Involving Transcription Factors 697Disorders Involving Defective Bone Resorption 698Disorders for Which Defects Are Poorly Understood or Unknown 699Osteogenesis Imperfecta 700Marfan Syndrome Section 4. Metabolic Bone Disease 701Bone Structure, Growth, and Hormonal Regulation 702Primary Chondrodystrophy (Metaphyseal Dysplasia) 703Hypophosphatasia 704Hyperphosphatasia 705Osteoporosis Part XXXII: Environmental Health Hazards 706Biologic Effects of Radiation on Children 707Chemical Pollutants 708Heavy Metal Poisoning 709Lead Poisoning 710Nonbacterial Food Poisoning 710.1Mushroom Poisoning 710.2Solanine Poisoning 710.3Seafood Poisoning 711Biologic and Chemical Terrorism 712Animal and Human Bites 712.1Rat-Bite Fever 712.2Monkeypox 713Envenomations PART XXXIII: Laboratory Medicine 714Laboratory Testing in Infants and Children 715Reference Ranges for Laboratory Tests and Procedures 716Medications