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Part I The Field of Pediatrics 1 Overview of Pediatrics 2 Ethics in Pediatric Care 3 Cultural Issues in Pediatric Care 4 Child Health in the Developing World 5 Preventive Pediatrics 6 The Well Child Part II Growth and Development 7 Overview and Assessment of Variability 8 Fetal Growth and Development 9 The Newborn 10 The First Year 11 The Second Year 12 Preschool Years 13 Middle Childhood 14 Adolescence 15 Assessment of Growth 16 Developmental Assessment Part III Psychologic Disorders 17 Assessment and Interviewing 17.1 The Clinical Interview 17.2 Psychosocial Problems 18 Psychiatric Considerations of Central Nervous System Injury 19 Psychosomatic Illness 20 Vegetative Disorders 20.1 Rumination Disorder 20.2 Pica 20.3 Enuresis 20.4 Encopresis 20.5 Sleep Disorders 21 Habit Disorders 22 Anxiety Disorders 23 Mood Disorders 23.1 Major Depression 23.2 Dysthymic Disorder 23.3 Bipolar Disorder 24 Suicide and Attempted Suicide 25 Disruptive Behavioral Disorders 26 Sexual Behavior and Its Variations 26.1 Gender Identity Disorder 26.2 Homosexuality 27 Pervasive Developmental Disorders and Childhood Psychosis 27.1 Autistic Disorder 27.2 Asperger Disorder 27.3 Childhood Disintegrative Disorder 27.4 Rett Disorder

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27.5 Childhood Schizophrenia 28 Psychologic Treatment of Children and Adolescents 28.1 Illness and Death 28.2 Psychopharmacology 28.3 Psychotherapy 28.4 Psychiatric Hospitalization 29 Neurodevelopmental Dysfunction in the SchoolAged Child 29.1 Patterns of Development and Function 29.2 Attention-Deficit/Hyperactivity Disorder 29.3 Specific Reading Disability Part IV Social Issues 30 Adoption 31 Foster Care 32 Child Care 33 Separation, Loss, and Bereavement 33.1 Separation & Loss 33.2 Grief and Bereavement 34 Impact of Violence on Children 35 Abuse and Neglect of Children 35.1 Sexual Abuse 35.2 Nonorganic Failure to Thrive 35.3 Munchausen Syndrome by Proxy Part V Children with Special Health Needs 36 Failure to Thrive 37 Developmental Disabilities and Chronic Illness 37.1 Chronic Illness in Childhood 37.2 Mental Retardation 38 Pediatric Palliative Care: The Care of Children with Life-Limiting Illness 39 Children at Special Risk Part VI Nutrition 40 Nutritional Requirements 41 The Feeding of Infants and Children 42 Food Insecurity, Hunger, and Undernutrition 43 Obesity 44 Vitamin Deficiencies and Excesses Part VII Pathophysiology of Body Fluids and Fluid Therapy 45 Electrolyte and Acid-Base Disorders 45.1 Composition of Body Fluids 45.2 Regulation of Osmolality and Volume 45.3 Sodium 45.4 Potassium 45.5 Calcium 45.6 Magnesium 45.7 Phosphorus 45.8 Acid-Base Balance 46 Maintenance and Replacement Therapy 47 Deficit Therapy

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48 Fluid and Electrolyte Treatment of Specific Disorders 48.1 Acute Diarrhea and Oral Rehydration 48.2 Diarrhea in Chronically Malnourished Children 48.3 Pyloric Stenosis 48.4 Perioperative Fluids Part VIII The Acutely Ill Child 49 Evaluation of the Sick Child in the Office and Clinic 50 Injury Control 51 Emergency Medical Services for Children 52 Pediatric Critical Care: An Overview 53 Interfacility Transfer of the Critically Ill Infant and Child 54 Effective Communication with Families in the PICU 55 Monitoring Techniques for the Critically Ill Infant and Child 56 Scoring Systems and Predictors of Mortality 57 Stabilization of the Critically Ill Child 57.1 Pediatric Emergencies and Resuscitation 57.2 Shock 57.3 Respiratory Distress and Failure 57.4 Mechanical Ventilation 57.5 Renal Stabilization 57.6 Nutritional Stabilization 57.7 Neurologic Stabilization 57.8 Acute Care of the Multiple Trauma Victim 58 Acute 59 Continuous Hemofiltration 60 Transplantation Issues in the PICU 61 Drowning and Near-Drowning 62 Burn Injuries 63 Cold Injuries 64 Withdrawal or Withholding of Life Support, Brain Death, and Organ Procurement 65 Anesthesia and Perioperative Care 66 Pediatric Pain Management Part IX Human Genetics 67 The Molecular Basis of Genetic Disorders 68 Molecular Diagnosis of Genetic Diseases 69 Patterns of Inheritance 70 Chromosomal Clinical Abnormalities 71 Gene Therapy 72 Genetic Counseling Part X Metabolic Diseases 73 An Approach to Inborn Errors of Metabolism 74 Defects in Metabolism of Amino Acids 74.1 Phenylalanine 74.2 Tyrosine

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74.3 Methionine 74.4 Cysteine/Cystine 74.5 Tryptophan 74.6 Valine, Leucine, Isoleucine, and Related Organic Acidemias 74.7 Glycine 74.8 Serine 74.9 Proline and Hydroxyproline 74.10 Glutamic Acid 74.11 Urea Cycle and Hyperammonemia 74.12 Histidine 74.13 Lysine 74.14 Aspartic Acid 75 Defects in Metabolism of Lipids 75.1 Disorders of Mitochondrial Fatty Acid Oxidation 75.2 Disorders of Very Long Chain Fatty Acids 75.3 Disorders of Lipoprotein Metabolism and Transport 75.4 Lipidoses 75.5 Mucolipidoses 76 Defects in Metabolism of Carbohydrates 76.1 Glycogen Storage Diseases 76.2 Defects in Galactose Metabolism 76.3 Defects in Fructose Metabolism 76.4 Defects in Intermediary Carbohydrate Metabolism Associated with Lactic Acidosis 76.5 Deficiency of Xylulose Dehydrogenase 76.6 Disorders of Glycoprotein Degradation and Structure 77 Mucopolysaccharidoses 78 Disorders of Purine and Pyrimidine Metabolism 79 Progeria 80 The Porphyrias 81 Hypoglycemia Part XI The Fetus and the Neonatal Infant 1 Noninfectious Disorders 82 Overview of Mortality and Morbidity 83 The Newborn Infant 83.1 History in Neonatal Pediatrics 83.2 Physical Examination of the Newborn Infant 83.3 Routine Delivery Room Care 83.4 Nursery Care 83.5 Parent-Infant Bonding 84 High-Risk Pregnancies 85 The Fetus 85.1 Fetal Growth and Maturity 85.2 Fetal Distress 85.3 Maternal Disease and the Fetus 85.4 Maternal Medication and Toxin Exposure and the Fetus

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85.5 Teratogens 85.6 Radiation 85.7 Intrauterine Diagnosis of Fetal Disease 85.8 Treatment and Prevention of Fetal Disease 86 The High-Risk Infant 86.1 Multiple Pregnancies 86.2 Prematurity and Intrauterine Growth Retardation 86.3 Post-Term Infants 86.4 Large for Gestational Age 86.5 Infant Transport 87 Clinical Manifestations of Diseases in the Newborn Period 88 Nervous System Disorders 88.1 The Cranium 88.2 Intracranial-Intraventricular Hemorrhage and Periventricular Leukomalacia 88.3 Spine and Spinal Cord 88.4 Peripheral Nerve Injuries 88.5 Hypoxia-Ischemia 88.6 Intrauterine Infection 89 Delivery Room Emergencies 90 Respiratory Tract Disorders 90.1 Transition to Pulmonary Respiration 90.2 Apnea 90.3 Hyaline Membrane Disease 90.4 Transient Tachypnea of the Newborn 90.5 Aspiration of Foreign Material 90.6 Meconium Aspiration 90.7 Persistent Pulmonary Hypertension of the Newborn 90.8 Extrapulmonary Extravasation of Air 90.9 Pulmonary Hemorrhage 91 Digestive System Disorders 91.1 Meconium Ileus in Cystic Fibrosis 91.2 Neonatal Necrotizing Enterocolitis 91.3 Jaundice and Hyperbilirubinemia in the Newborn 91.4 Kernicterus 92 Blood Disorders 92.1 Anemia in the Newborn Infant 92.2 Hemolytic Disease of the Newborn 92.3 Plethora in the Newborn Infant 92.4 Hemorrhage in the Newborn Infant 93 Genitourinary System 94 The Umbilicus 95 Metabolic Disturbances 96 The Endocrine System 96.1 Infants of Diabetic Mothers 96.2 Hypoglycemia 97 Dysmorphology

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2 Infections of the Neonatal Infant 98 Pathogenesis and Epidemiology 98.1 Modes of Transmission and Pathogenesis 98.2 Immunity 98.3 Etiology of Fetal and Neonatal Infection 98.4 Epidemiology of Early- and Late-Onset Neonatal Infections 98.5 Clinical Manifestations of Transplacental Intrauterine Infections 98.6 Diagnosis 98.7 Treatment 98.8 Complications and Prognosis 98.9 Prevention Part XII Special Health Problems During Adolescence 99 The Epidemiology of Adolescent Health Problems 100 Delivery of Health Care to Adolescents 100.1 Legal Issues 100.2 Screening Procedures 100.3 Health Enhancement 101 Depression 102 Suicide 103 Violent Behavior 104 Anorexia Nervosa and Bulimia 105 Substance Abuse 105.1 Alcohol 105.2 Marijuana 105.3 Tobacco 105.4 Volatile Substances 105.5 Hallucinogens 105.6 Cocaine 105.7 Amphetamines 105.8 Opiates 105.9 Anabolic Steroids 106 The Breast 107 Menstrual Problems 107.1 Amenorrhea 107.2 Abnormal Uterine Bleeding 107.3 Dysmenorrhea 107.4 Premenstrual Syndrome 108 Contraception 108.1 Barrier Methods 108.2 Spermicides 108.3 Combination Methods 108.4 Hormonal Methods 108.5 Emergency Contraception 108.6 Intrauterine Devices 109 Pregnancy

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110 Sexually Transmitted Diseases 111 Chronic Fatigue Syndrome Part XIII The Immunologic System and Disorders 1 Evaluation of the Immune System 112 Evaluation of Suspected Immunodeficiency 2 The T-, B-, and NK-Cell Systems 113 T Lymphocytes, B Lymphocytes, and Natural Killer Cells 114 Primary Defects of Antibody Production 114.1 Treatment of B-Cell Defects 115 Primary Defects of Cellular Immunity 116 Primary Combined Antibody and Cellular Immunodeficiencies 116.1 Severe Combined Immunodeficiency 116.2 Combined Immunodeficiency 116.3 Treatment of Cellular or Combined Immunodeficiency 3 The Phagocytic System 117 Neutrophils 118 Monocytes and Macrophages 119 Eosinophils 120 Disorders of Phagocyte Function 121 Leukopenia 122 Leukocytosis 4 The Complement System 123 The Complement System 124 Disorders of the Complement System 124.1 Evaluation 124.2 Primary Deficiencies of Complement Components 124.3 Deficiencies of Plasma, Membrane, or Serosal Complement Control Proteins 124.4 Secondary Disorders of Complement 124.5 Treatment of Complement Disorders 5 Hematopoietic Stem Cell Transplantation 125 Clinical Indications 126 Matching and Rejection 127 Graft Versus Host Disease 128 Principles of Immunosuppression 129 Late Effects of Stem Cell Transplantation Part XIV Allergic Disorders 130 Allergy and the Immunologic Basis of Atopic Disease 131 Diagnosis of Allergic Disease 132 Principles of Treatment of Allergic Disease 133 Allergic Rhinitis 134 Childhood Asthma 135 Atopic Dermatitis 136 Urticaria and Angioedema 137 Anaphylaxis 138 Serum Sickness 139 Adverse Reactions to Drugs 140 Insect Allergy

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141 Ocular Allergies 142 Adverse Reactions to Foods Part XV Rheumatic Diseases of Childhood 143 Evaluation of Suspected Rheumatic Disease 144 Treatment of Rheumatic Diseases 145 Juvenile Rheumatoid Arthritis 146 Ankylosing Spondylitis and Other Spondyloarthropathies 147 Postinfectious Arthritis and Related Conditions 148 Systemic Lupus Erythematosus 148.1 Neonatal Lupus 149 Juvenile Dermatomyositis 150 Scleroderma 151 Behet Disease 152 Sjgren Syndrome 153 Familial Mediterranean Fever 154 Amyloidosis 155 Sarcoidosis 156 Kawasaki Disease 157 Vasculitis Syndromes 157.1 Henoch-Schnlein Purpura 157.2 Takayasu Arteritis 157.3 Polyarteritis Nodosa 157.4 Wegener Granulomatosis 157.5 Other Vasculitic Syndromes 158 Musculoskeletal Pain Syndromes 158.1 Reflex Sympathetic Dystrophy 158.2 Erythromelalgia 159 Miscellaneous Conditions Associated with Arthritis Part XVI Infectious Diseases 1 General Considerations 160 Diagnostic Microbiology 161 Fever 162 Fever Without a Focus 163 Sepsis and Shock 164 Infections in Immunocompromised Persons 165 Infection Associated with Medical Devices 2 Gram-Positive Bacterial Infections 166 Staphylococcus 166.1 Staphylococcus aureus 166.2 Toxic Shock Syndrome 166.3 Coagulase-Negative Staphylococci 167 Streptococcus pneumoniae 168 Group A Streptococcus 168.1 Rheumatic Fever 169 Group B Streptococcus 170 Non-Group A or B Streptococci

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171 Enterococcus 172 Diphtheria 173 Listeria monocytogenes 174 Actinomyces 175 Nocardia 3 Gram-Negative Bacterial Infections 176 Neisseria meningitidis 177 Neisseria gonorrhoeae 178 Haemophilus influenzae 179 Chancroid 180 Pertussis 181 Salmonella 181.1 Nontyphoidal Salmonellosis 181.2 Enteric Fever 182 Shigella 183 Escherichia coli 184 Cholera 185 Campylobacter 186 Yersinia 186.1 Yersinia enterocolitica 186.2 Yersinia pseudotuberculosis 186.3 Plague 187 Aeromonas and Plesiomonas 187.1 Aeromonas 187.2 Plesiomonas shigelloides 188 Pseudomonas, Burkholderia, and Stenotrophomonas 188.1 Pseudomonas aeruginosa 188.2 Burkholderia 188.3 Stenotrophomonas 189 Tularemia 190 Brucella 191 Legionella 192 Bartonella 192.1 Bartonellosis 192.2 Cat-Scratch Disease 192.3 Trench Fever 192.4 Bacillary Angiomatosis and Bacillary Peliosis Hepatis 4 Anaerobic Bacterial Infections 193 Botulism 194 Tetanus 195 Clostridium difficile-Associated Diarrhea 196 Other Anaerobic Infections 5 Mycobacterial Infections 197 Tuberculosis 198 Hansen Disease 199 Nontuberculous Mycobacteria

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6 Spirochetal Infections 200 Syphilis 201 Nonvenereal Treponemal Infections 201.1 Yaws 201.2 Bejel 201.3 Pinta 202 Leptospira 203 Relapsing Fever 204 Lyme Disease 7 Mycoplasmal Infections 205 Mycoplasma pneumoniae 206 Genital Mycoplasmas 8 Chlamydial Infections 207 Chlamydia pneumoniae 208 Chlamydia trachomatis 208.1 Trachoma 208.2 Genital Tract Infections 208.3 Conjunctivitis and Pneumonia in Newborns 208.4 Lymphogranuloma Venereum 209 Psittacosis 9 Rickettsial Infections 210 Spotted Fever Group Rickettsioses 210.1 Rocky Mountain Spotted Fever 210.2 Mediterranean Spotted Fever or Boutonneuse Fever 210.3 Rickettsialpox 211 Scrub Typhus 212 Typhus Group Rickettsioses 212.1 Murine Typhus 212.2 Epidemic Typhus 213 Ehrlichiosis and Anaplasmosis 214 Q Fever 10 Mycotic Infections 215 Candida 215.1 Neonatal Infections 215.2 Infections in Immunocompetent Children and Adolescents 215.3 Infections in Immunocompromised Children and Adolescents 215.4 Chronic Mucocutaneous Candidiasis 216 Cryptococcus neoformans 217 Malassezia 218 Aspergillus 218.1 Hypersensitivity Syndromes 218.2 Saprophytic Syndromes 218.3 Invasive Disease 219 Histoplasmosis 220 Blastomycosis 221 Coccidioidomycosis 222 Paracoccidioides brasiliensis 223 Sporotrichosis

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224 Zygomycosis 11 Viral Infections 225 Measles 225.1 Subacute Sclerosing Panencephalitis 226 Rubella 226.1 Progressive Rubella Panencephalitis 227 Mumps 228 Polioviruses 229 Nonpolio Enteroviruses 230 Parvovirus B19 231 Herpes Simplex Virus 232 Varicella-Zoster Virus 233 Epstein-Barr Virus 234 Cytomegalovirus 235 Roseola 236 Human Herpesvirus 8 237 Influenza Viruses 238 Parainfluenza Viruses 239 Respiratory Syncytial Virus 240 Adenoviruses 241 Rhinoviruses 242 Rotavirus and Other Agents of Viral Gastroenteritis 243 Human Papillomaviruses 244 Arboviral Encephalitis in North America 245 Arboviral Encephalitis Outside North America 245.1 Venezuelan Equine Encephalitis 245.2 Japanese Encephalitis 245.3 Tick-Borne Encephalitis 246 Dengue Fever and Dengue Hemorrhagic Fever 247 Yellow Fever 248 Other Viral Hemorrhagic Fevers 249 Lymphocytic Choriomeningitis Virus 250 Hantaviruses 251 Rabies 252 Polyomaviruses 253 Transmissible Spongiform Encephalopathies 254 Acquired Immunodeficiency Syndrome 255 Human T-Cell Lymphotropic Viruses Types I and II 12 Protozoan Diseases 256 Primary Amebic Meningoencephalitis 12 Protozoan Diseases 257 Amebiasis 258 Giardiasis and Balantidiasis 258.1 Giardia lamblia 258.2 Balantidiasis 259 Spore-Forming Intestinal Protozoa

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260 Trichomoniasis 261 Leishmaniasis 262 African Trypanosomiasis 263 American Trypanosomiasis 264 Malaria 265 Babesiosis 266 Toxoplasmosis 267 Pneumocystis carinii 13 Helminthic Diseases 268 Ascariasis 269 Hookworms 269.1 Cutaneous Larva Migrans 270 Trichuriasis 271 Enterobiasis 272 Strongyloidiasis 273 Lymphatic Filariasis 274 Other Tissue Nematodes 275 Toxocariasis 276 Trichinosis 277 Schistosomiasis 278 Flukes 279 Adult Tapeworm Infections 280 Cysticercosis 281 Echinococcosis 14 Preventive Measures 282 Immunization Practices 283 Infection Control and Prophylaxis 284 Child Care and Communicable Diseases 285 Health Advice for Children Traveling Internationally Part XVII The Digestive System 1 Clinical Manifestations of Gastrointestinal Disease 286 Normal Digestive Tract Phenomena 287 Major Symptoms and Signs of Digestive Tract Disorders 2 The Oral Cavity 288 Development and Developmental Anomalies of the Teeth 289 Disorders of the Oral Cavity Associated with Other Conditions 290 Malocclusion 291 Cleft Lip and Palate 292 Syndromes with Oral Manifestations 293 Dental Caries 294 Periodontal Diseases 295 Dental Trauma 296 Common Lesions of the Oral Soft Tissues 297 Diseases of the Salivary Glands and Jaws 298 Diagnostic Radiology in Dental Assessment 3 The Esophagus

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299 Embryology, Anatomy, and Function of the Esophagus 299.1 Common Clinical Manifestations and Diagnostic Aids 300 Congenital Anomalies: Esophageal Atresia and Tracheoesophageal Fistula 300.1 Congenital Anomalies: Laryngotracheoesophageal Clefts 301 Obstructing and Motility Disorders of the Esophagus 302 Dysmotility 303 Hiatal Hernia 304 Gastroesophageal Reflux Disease 304.1 Complications of GERD 305 Non-GERD Esophagitis 306 Esophageal Perforation 307 Esophageal Varices 308 Ingestions 308.1 Foreign Bodies in the Esophagus 308.2 Caustic Ingestions 4 Stomach and Intestines 309 Normal Development, Structure, and Function 310 Pyloric Stenosis and Congenital Anomalies of the Stomach 310.1 Hypertrophic Pyloric Stenosis 310.2 Congenital Gastric Outlet Obstruction 310.3 Gastric Duplication 310.4 Gastric Volvulus 310.5 Hypertrophic Gastropathy 311 Intestinal Atresia, Stenosis, and Malrotation 311.1 Duodenal Obstruction 311.2 Jejunal and Ileal Atresia and Obstruction 311.3 Malrotation 312 Intestinal Duplications, Meckel Diverticulum, and Other Remnants of the Omphalomesenteric Duct 312.1 Intestinal Duplication 312.2 Meckel Diverticulum and Other Remnants of the Omphalomesenteric Duct 313 Motility Disorders and Hirschsprung Disease 313.1 Chronic Intestinal Pseudo-Obstruction 313.2 Functional Constipation 313.3 Congenital Aganglionic Megacolon 313.4 Superior Mesenteric Artery Syndrome 314 Ileus, Adhesions, Intussusception, and ClosedLoop Obstructions 314.1 Ileus 314.2 Adhesions 314.3 Intussusception 314.4 Closed-Loop Obstructions 315 Foreign Bodies and Bezoars 315.1 Foreign Bodies in the Stomach and Intestine 315.2 Bezoars 316 Peptic Ulcer Disease 316.1 Secondary or Stress Ulcer Disease

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316.2 Zollinger-Ellison Syndrome 317 Inflammatory Bowel Disease 317.1 Chronic Ulcerative Colitis 317.2 Crohn Disease 317.3 Behet Syndrome 318 Food Allergy 319 Eosinophilic Gastroenteritis 320 Malabsorptive Disorders 320.1 Evaluation of Children with Suspected Intestinal Malabsorption 320.2 Chronic Malnutrition 320.3 Liver and Biliary Disorders 320.4 Intestinal Infections 320.5 Immunodeficiency 320.6 Stagnant Loop Syndrome 320.7 Short Bowel Syndrome 320.8 Gluten-Sensitive Enteropathy 320.9 Immunoproliferative Small Intestinal Disease 320.10 Other Malabsorptive Syndromes 320.11 Enzyme Deficiencies 320.12 Defects of Absorption or Transport 321 Gastroenteritis 322 Chronic Diarrhea 323 Recurrent Abdominal Pain of Childhood 324 Acute Appendicitis 325 Surgical Conditions of the Anus, Rectum, and Colon 325.1 Anorectal Malformations 325.2 Anal Fissure 325.3 Perianal Abscess and Fistula 325.4 Hemorrhoids 325.5 Rectal Prolapse 325.6 Pilonidal Sinus and Abscess 326 Tumors of the Digestive Tract 326.1 Diarrhea from Hormone-Secreting Tumors 327 Inguinal Hernias 5 Exocrine Pancreas 328 Embryology, Anatomy, and Physiology 328.1 Anatomic Abnormalities 328.2 Physiology 329 Pancreatic Function Tests 330 Disorders of the Exocrine Pancreas 331 Treatment of Pancreatic Insufficiency 332 Pancreatitis 332.1 Acute Pancreatitis 332.2 Chronic Pancreatitis 333 Pseudocyst of the Pancreas 334 Pancreatic Tumors

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6 The Liver and Biliary System 335 Development and Function of the Liver and Biliary System Michael D. Bates and 336 Manifestations of Liver Disease 336.1 Evaluation of Patients with Possible Liver Dysfunction 337 Cholestasis 337.1 Neonatal Cholestasis 337.2 Cholestasis in the Older Child 338 Metabolic Diseases of the Liver 338.1 Inherited Deficient Conjugation of Bilirubin 338.2 Wilson Disease 338.3 Hepatic Copper Overload Syndrome 338.4 Indian Childhood Cirrhosis 338.5 Neonatal Iron Storage Disease 338.6 Miscellaneous Metabolic Diseases of the Liver 339 Viral Hepatitis 339.1 Hepatitis in Neonates 340 Liver Abscess 341 Liver Disease Associated with Systemic Disorders 342 Reye Syndrome and the Mitochondrial Hepatopathies 343 Autoimmune Hepatitis 344 Drug- and Toxin-Induced Liver Injury 345 Fulminant Hepatic Failure 346 Cystic Diseases of the Biliary Tract and Liver 347 Diseases of the Gallbladder 348 Portal Hypertension and Varices 349 Liver Transplantation 7 Peritoneum 350 Malformations 351 Ascites 351.1 Chylous Ascites 352 Peritonitis 352.1 Acute Primary Peritonitis 352.2 Acute Secondary Peritonitis 352.3 Acute Secondary Localized Peritonitis 353 Diaphragmatic Hernia 353.1 Foramen of Morgagni Hernia 353.2 Paraesophageal Hernia 353.3 Eventration 354 Epigastric Hernia 354.1 Incisional Hernia Part XVIII The Respiratory System 1 Development and Function 355 Development of the Respiratory System 356 Regulation of Respiration 357 Respiratory Pathophysiology 357.1 Respiratory Failure 357.2 Congenital Central Hypoventilation Syndrome 358 Defense Mechanisms and Metabolic Functions of the Lung

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358.1 Defense Mechanisms 358.2 Metabolic Functions 359 Diagnostic Approach to Respiratory Disease 360 Sudden Infant Death Syndrome 2 Upper Respiratory Tract 361 Congenital Disorders of the Nose 362 Acquired Disorders of the Nose 362.1 Foreign Body 362.2 Epistaxis 363 Nasal Polyps 364 The Common Cold 365 Sinusitis 366 Acute Pharyngitis 367 Retropharyngeal Abscess, Lateral Pharyngeal Abscess, and Peritonsillar Cellulitis/Abscess 368 Tonsils and Adenoids 369 Obstructive Sleep Apnea and Hypoventilation 3 Disorders of the Lungs and Lower Airways 370 Chronic or Recurrent Respiratory Symptoms 371 Acute Inflammatory Upper Airway Obstruction 371.1 Infectious Upper Airway Obstruction 371.2 Bacterial Tracheitis 372 Congenital Anomalies of the Larynx 372.1 Laryngomalacia 372.2 Congenital Subglottic Stenosis 372.3 Vocal Cord Paralysis 372.4 Congenital Laryngeal Webs and Atresia 372.5 Congenital Subglottic Hemangioma 372.6 Laryngoceles and Saccular Cysts 372.7 Posterior Laryngeal Cleft and Laryngotracheoesophageal Cleft 373 Foreign Bodies of the Airway 373.1 Laryngeal Foreign Bodies 373.2 Tracheal Foreign Bodies 373.3 Bronchial Foreign Bodies 374 Laryngotracheal Stenosis, Subglottic Stenosis 374.1 Congenital Subglottic Stenosis 374.2 Acquired Laryngotracheal Stenosis 375 Congenital Anomalies of the Trachea and Bronchi 375.1 Vascular and Cardiac Anomalies 375.2 Tracheal Stenoses, Webs, and Atresia 375.3 Foregut Cysts 376 Bronchomalacia and Tracheomalacia 377 Neoplasms of the Larynx, Trachea, and Bronchi 377.1 Vocal Nodules 377.2 Recurrent Respiratory Papillomatosis 377.3 Congenital Subglottic Hemangioma 377.4 Vascular Anomalies 377.5 Other Laryngeal Neoplasms

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377.6 Tracheal Neoplasms 377.7 Bronchial Tumors 378 Inflammatory Disorders of the Small Airways 378.1 Bronchitis 378.2 Bronchiolitis 379 Wheezing in Infants 380 Emphysema and Overinflation 381 1-Antitrypsin Deficiency and Emphysema 382 Other Distal Airway Diseases 382.1 Bronchiolitis Obliterans 382.2 Follicular Bronchitis 382.3 Pulmonary Alveolar Microlithiasis 383 Congenital Disorders of the Lung 383.1 Pulmonary Agenesis and Aplasia 383.2 Pulmonary Hypoplasia 383.3 Cystic Adenomatoid Malformation 383.4 Pulmonary Sequestration 383.5 Bronchogenic Cysts 383.6 Congenital Pulmonary Lymphangiectasia 383.7 Lung Hernia 383.8 Other Congenital Malformations of the Lung 384 Pulmonary Edema 385 Aspiration Syndromes 386 Chronic Recurrent Aspiration 387 Gastroesophageal Reflux and Respiratory Disorders 388 Parenchymal Disease with Prominent Hypersensitivity, Eosinophilic Infiltration, or ToxinMediated Injury 388.1 Hypersensitivity to Inhaled Materials 388.2 Silo Filler Disease 388.3 Paraquat Lung 388.4 Eosinophilic Lung Disease 389 Pneumonia 390 Slowly Resolving Pneumonia 391 Bronchiectasis 392 Pulmonary Abscess 393 Cystic Fibrosis 394 Primary Ciliary Dyskinesia 395 Interstitial Lung Diseases 396 Pulmonary Alveolar Proteinosis 397 Inherited Disorders of Surfactant Protein Metabolism 398 Pulmonary Hemosiderosis 399 Pulmonary Hemorrhage, Embolism, and Infarction 399.1 Pulmonary Embolism and Infarction 399.2 Hemoptysis 400 Atelectasis

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401 Pulmonary Tumors 402 Pleurisy 402.1 Dry or Plastic Pleurisy 402.2 Serofibrinous Pleurisy 402.3 Purulent Pleurisy 403 Pneumothorax 404 Pneumomediastinum 405 Hydrothorax 406 Hemothorax 407 Chylothorax 408 Bronchopulmonary Dysplasia 409 Skeletal Diseases Influencing Pulmonary Function 409.1 Pectus Excavatum 409.2 Pectus Carinatum and Fissuri Sterni 409.3 Asphyxiating Thoracic Dystrophy 409.4 Achondroplasia 409.5 Kyphoscoliosis: Adolescent Idiopathic Scoliosis and Congenital Scoliosis 409.6 Congenital Rib Anomalies 410 Neuromuscular Diseases with Pulmonary Consequences 411 Extrapulmonary Diseases with Pulmonary Manifestations 412 Chronic Severe Respiratory Insufficiency Part XIX The Cardiovascular System 1 Developmental Biology of the Cardiovascular System 413 Cardiac Development 413.1 Early Cardiac Morphogenesis 413.2 Cardiac Looping 413.3 Cardiac Septation 413.4 Aortic Arch Development 413.5 Cardiac Differentiation 413.6 Developmental Changes in Cardiac Function 414 The Fetal-to-Neonatal Circulatory Transition 414.1 The Fetal Circulation 414.2 The Transitional Circulation 414.3 The Neonatal Circulation 414.4 Persistent Pulmonary Hypertension of the Neonate 2 Evaluation of the Cardiovascular System 415 History and Physical Examination 416 Laboratory Evaluation 416.1 Radiologic Assessment 416.2 Electrocardiography 416.3 Hematologic Data 416.4 Echocardiography 416.5 Exercise Testing 416.6 MRI, Electron Beam CT, and Radionuclide Studies

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416.7 Cardiac Catheterization 3 Congenital Heart Disease 417 Epidemiology and Genetic Basis of Congenital Heart Disease 418 Evaluation of the Infant or Child with Congenital Heart Disease 419 Acyanotic Congenital Heart Disease: The Left-toRight Shunt Lesions 419.1 Atrial Septal Defect 419.2 Ostium Secundum Defect 419.3 Sinus Venosus Atrial Septal Defect 419.4 Partial Anomalous Pulmonary Venous Return 419.5 Atrioventricular Septal Defects 419.6 Ventricular Septal Defect 419.7 Supracristal Ventricular Septal Defect with Aortic Insufficiency 419.8 Patent Ductus Arteriosus 419.9 Aorticopulmonary Window Defect 419.10 Coronary-Arteriovenous Fistula 419.11 Ruptured Sinus of Valsalva Aneurysm 420 Acyanotic Congenital Heart Disease: The Obstructive Lesions 420.1 Pulmonary Valve Stenosis with Intact Ventricular Septum 420.2 Infundibular Pulmonary Stenosis and Double-Chamber Right Ventricle 420.3 Pulmonary Stenosis in Combination with an Intracardiac Shunt 420.4 Peri1pheral Pulmonary Stenosis 420.5 Aortic Stenosis 420.6 Coarctation of the Aorta 420.7 Coarctation with Ventricular Septal Defect 420.8 Coarctation with Other Cardiac Anomalies and Interrupted Aortic Arch 420.9 Congenital Mitral Stenosis 420.10 Pulmonary Venous Hypertension 421 Acyanotic Congenital Heart Disease: Regurgitant Lesions 421.1 Pulmonary Valvular Insufficiency and Congenital Absence of the Pulmonary Valve 421.2 Congenital Mitral Insufficiency 421.3 Mitral Valve Prolapse 421.4 Tricuspid Regurgitation 422 Cyanotic Congenital Heart Disease: Evaluation of the Critically Ill Neonate with Cyanosis and Respiratory Distress 423 Cyanotic Congenital Heart Lesions: Lesions Associated with Decreased Pulmonary Blood Flow 423.1 Tetralogy of Fallot 423.2 Pulmonary Atresia with Ventricular Septal Defect 423.3 Pulmonary Atresia with Intact Ventricular Septum 423.4 Tricuspid Atresia 423.5 Double-Outlet Right Ventricle with Pulmonary Stenosis 423.6 Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis 423.7 Ebstein Anomaly of the Tricuspid Valve

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424 Cyanotic Congenital Heart Disease: Lesions Associated with Increased Pulmonary Blood Flow 424.1 d-Transposition of the Great Arteries 424.2 d-Transposition of the Great Arteries with Intact Ventricular Septum 424.3 Transposition of the Great Arteries with Ventricular Septal Defect 424.4 l-Transposition of the Great Arteries 424.5 Double-Outlet Right Ventricle Without Pulmonary Stenosis 424.6 Double-Outlet Right Ventricle with Transposition of the Great Arteries 424.7 Total Anomalous Pulmonary Venous Return 424.8 Truncus Arteriosus 424.9 Single Ventricle 424.10 Hypoplastic Left Heart Syndrome 424.11 Abnormal Positions of the Heart and the Heterotaxy Syndromes 425 Other Congenital Heart and Vascular Malformations 425.1 Anomalies of the Aortic Arch 425.2 Anomalous Origin of the Coronary Arteries 425.3 Pulmonary Arteriovenous Fistula 425.4 Ectopia Cordis 425.5 Diverticulum of the Left Ventricle 426 Pulmonary Hypertension 426.1 Primary Pulmonary Hypertension 426.2 Pulmonary Vascular Disease 427 General Principles of Treatment of Congenital Heart Disease 4 Cardiac Arrhythmias 428 Disturbances of Rate and Rhythm of the Heart 428.1 Sinus Arrhythmias and Extrasystoles 428.2 Supraventricular Tachycardia 428.3 Ventricular Tachyarrhythmias 428.4 Long Q-T Syndrome 428.5 Bradyarrhythmias 428.6 Sick Sinus Syndrome 428.7 Sudden Death 5 Acquired Heart Disease 429 Infective Endocarditis 430 Rheumatic Heart Disease 6 Diseases of the Myocardium and Pericardium 431 Diseases of the Myocardium 431.1 Dilated Cardiomyopathy 431.2 Hypertrophic Cardiomyopathy 431.3 Restrictive Cardiomyopathy 431.4 Myocarditis 431.5 Nonviral Causes of Myocarditis 431.6 Endocardial Fibroelastosis 432 Diseases of the Pericardium 432.1 Constrictive Pericarditis 433 Tumors of the Heart 7 Cardiac Therapeutics

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434 Heart Failure 434.1 Cardiogenic Shock 435 Pediatric Heart and Heart-Lung Transplantation 435.1 Pediatric Heart Transplantation 435.2 Heart-Lung and Lung Transplantation 8 Diseases of the Peripheral Vascular System 436 Disease of the Blood Vessels 436.1 Kawasaki Disease 436.2 Arteriovenous Fistulas 437 Systemic Hypertension Part XX Diseases of the Blood 1 The Hematopoietic System 438 Development of the Hematopoietic System 439 The Anemias 2 Anemias of Inadequate Production 440 Congenital Hypoplastic Anemia 441 Pearson Marrow-Pancreas Syndrome 442 Acquired Pure Red Blood Cell Anemias 443 Anemia of Chronic Disorders and Renal Disease 444 Congenital Dyserythropoietic Anemias 445 Physiologic Anemia of Infancy 446 Megaloblastic Anemias 446.1 Folic Acid Deficiency 446.2 Vitamin B12 Deficiency 446.3 Other Rare Megaloblastic Anemias 447 Iron-Deficiency Anemia 448 Other Microcytic Anemias 3 Hemolytic Anemias 449 Definitions and Classification of Hemolytic Anemias 450 Hereditary Spherocytosis 451 Hereditary Elliptocytosis 452 Hereditary Stomatocytosis 453 Other Membrane Defects 454 Hemoglobin Disorders 454.1 Sickle Cell Disease 454.2 Sickle Cell Trait 454.3 Other Hemoglobinopathies 454.4 Unstable Hemoglobin Disorders 454.5 Abnormal Hemoglobins with Increased Oxygen Affinity 454.6 Abnormal Hemoglobins Causing Cyanosis 454.7 Hereditary Methemoglobinemia 454.8 Syndromes of Hereditary Persistence of Fetal Hemoglobin 454.9 Thalassemia Syndromes 455 Enzymatic Defects 455.1 Pyruvate Kinase Deficiency 455.2 Other Glycolytic Enzyme Deficiencies 455.3 Glucose-6-Phosphate Dehydrogenase and Related Deficiencies 456 Hemolytic Anemias Resulting from Extracellular

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Factors Hemolytic Anemias Secondary to Other Extracellular Factors 4 Polycythemia 458 Primary Polycythemia 459 Secondary Polycythemia 5 The Pancytopenias 460 The Constitutional Pancytopenias 461 The Acquired Pancytopenias 6 Risks of Blood Component Transfusions 462 Red Blood Cell Transfusions and Erythropoietin Therapy 463 Platelet Transfusions 464 Neutrophil Transfusions 465 Fresh Frozen Plasma Transfusions 466 Risks of Blood Transfusions 7 Hemorrhagic and Thrombotic Diseases 467 Hemostasis 467.1 Hemostatic Mechanism 467.2 The Clinical and Laboratory Evaluation 468 Hereditary Clotting Factor Deficiencies 468.1 Factor VIII or Factor IX Deficiency 468.2 Factor XI Deficiency 468.3 Deficiencies of the Contact Factors 468.4 Factor VII Deficiency 468.5 Factor X Deficiency 468.6 Prothrombin Deficiency 468.7 Factor V Deficiency 468.8 Combined Deficiency of Factors V and VIII 468.9 Fibrinogen Deficiency 468.10 Factor XIII Deficiency 468.11 Antiplasmin or Plasminogen Activator Inhibitor Deficiency 469 von Willebrand Disease 470 Hereditary Predisposition to Thrombosis 471 Acquired Thrombotic Disorders 471.1 Anticoagulant and Thrombolytic Therapy 472 Postneonatal Vitamin K Deficiency 473 Liver Disease 474 Acquired Inhibitors of Coagulation 475 Disseminated Intravascular Coagulation 476 Platelet and Blood Vessel Disorders 476.1 Idiopathic Thrombocytopenic Purpura 476.2 Drug-Induced Thrombocytopenia 476.3 Nonimmune Platelet Destruction 476.4 Hemolytic-Uremic Syndrome 476.5 Thrombotic Thrombocytopenic Purpura 476.6 Kasabach-Merritt Syndrome 476.7 Sequestration 476.8 Congenital Thrombocytopenic Syndromes

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476.9 Neonatal Thrombocytopenia 476.10 Thrombocytopenia Due to Acquired Disorders Causing Decreased Production 476.11 Platelet Function Disorders 476.12 Acquired Disorders of Platelet Function 476.13 Congenital Abnormalities of Platelet Function 476.14 Disorders of the Blood Vessels 8 The Spleen 477 Anatomy and Function of the Spleen 478 Splenomegaly 479 Hyposplenism, Splenic Trauma, and Splenectomy 9 The Lymphatic System 480 Anatomy and Function of the Lymphatic System 481 Abnormalities of Lymphatic Vessels 482 Lymphadenopathy Part XXI Cancer and Benign Tumors 483 Epidemiology of Childhood and Adolescent Cancer 484 Molecular and Cellular Biology of Cancer 485 Principles of Diagnosis 486 Principles of Treatment 487 The Leukemias 487.1 Acute Lymphoblastic Leukemia 487.2 Acute Myelogenous Leukemia 487.3 Down Syndrome and Acute Leukemia and Myeloproliferation 487.4 Chronic Myelogenous Leukemia 487.5 Juvenile Chronic Myelogenous Leukemia 487.6 Infant Leukemia 488 Lymphoma 488.1 Hodgkin Disease 488.2 Non-Hodgkin Lymphoma 489 Brain Tumors in Childhood 490 Neuroblastoma 491 Neoplasms of the Kidney 492 Soft Tissue Sarcomas 493 Neoplasms of Bone 493.1 Malignant Tumors of Bone 493.2 Benign Tumors and Tumor-like Processes of Bone 494 Retinoblastoma 495 Gonadal and Germ Cell Neoplasms 496 Neoplasms of the Liver 497 Benign Vascular Tumors 498 Rare Tumors 499 Histiocytosis Syndromes of Childhood Part XXII Nephrology 1 Glomerular Disease 500 Introduction to Glomerular Diseases 500.1 Anatomy of the Glomerulus

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500.2 Glomerular Filtration 500.3 Glomerular Diseases 2 Conditions Particularly Associated with Hematuria 501 Clinical Evaluation of the Child with Hematuria 502 Isolated Glomerular Diseases with Recurrent Gross Hematuria 502.1 IgA Nephropathy 502.2 Alport Syndrome 502.3 Thin Glomerular Basement Membrane Disease 503 Glomerulonephritis Associated with Infections 503.1 Acute Poststreptococcal Glomerulonephritis 503.2 Other Chronic Infections 504 Membranous Glomerulopathy 505 Membranoproliferative Glomerulonephritis 506 Glomerulonephritis Associated with Systemic Lupus Erythematosus 507 Henoch-Schnlein Purpura Nephritis 508 Rapidly Progressive Glomerulonephritis 509 Goodpasture Disease 510 Hemolytic-Uremic Syndrome 511 Upper Urinary Tract Causes of Hematuria 511.1 Interstitial Nephritis 511.2 Toxic Nephropathy 511.3 Cortical Necrosis 511.4 Pyelonephritis 511.5 Nephrocalcinosis 511.6 Vascular Abnormalities 511.7 Renal Vein Thrombosis 511.8 Idiopathic Hypercalciuria 512 Hematologic Diseases Causing Hematuria 512.1 Sickle Cell Nephropathy 512.2 Coagulopathies and Thrombocytopenia 513 Anatomic Abnormalities Associated with Hematuria 513.1 Congenital Anomalies 513.2 Autosomal Recessive Polycystic Kidney Disease 513.3 Autosomal Dominant Polycystic Kidney Disease 513.4 Trauma 513.5 Renal Tumors 514 Lower Urinary Tract Causes of Hematuria 514.1 Infectious Causes of Cystitis and Urethritis 514.2 Hemorrhagic Cystitis 514.3 Heavy Exercise 514.4 Munchausen Syndrome by Proxy 3 Conditions Particularly Associated with Proteinuria 515 Introduction to the Child with Proteinuria 516 Transient Proteinuria

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517 Orthostatic Proteinuria 518 Fixed Proteinuria 518.1 Glomerular Proteinuria 518.2 Tubular Proteinuria 519 Nephrotic Syndrome 519.1 Idiopathic Nephrotic Syndrome 519.2 Secondary Nephrotic Syndrome 519.3 Congenital Nephrotic Syndrome 4 Tubular Disorders 520 Tubular Function 521 Renal Tubular Acidosis 521.1 Proximal Renal Tubular Acidosis 521.2 Distal Renal Tubular Acidosis 521.3 Hyperkalemic Renal Tubular Necrosis 521.4 Rickets Associated with Renal Tubular Acidosis 522 Nephrogenic Diabetes Insipidus 523 Bartter/Gitelman Syndromes and Other Inherited Tubular Transport Abnormalities 523.1 Bartter Syndrome 523.2 Gitelman Syndrome 523.3 Other Inherited Tubular Transport Abnormalities 524 Tubulointerstitial Nephritis 5 Toxic Nephropathies-Renal Failure 525 Toxic Nephropathy 526 Cortical Necrosis 527 Renal Failure 527.1 Acute Renal Failure 527.2 Chronic Renal Failure 527.3 End-Stage Renal Disease 528 Renal Transplantation Part XXIII Urologic Disorders in Infants and Children 529 Congenital Anomalies and Dysgenesis of the Kidneys 530 Urinary Tract Infections 531 Vesicoureteral Reflux 532 Obstructions of the Urinary Tract 533 Anomalies of the Bladder 534 Neuropathic Bladder 535 Voiding Dysfunction 536 Anomalies of the Penis and Urethra 537 Disorders and Anomalies of the Scrotal Contents 538 Trauma to the Genitourinary Tract 539 Urinary Lithiasis Part XXIV Gynecologic Problems of Childhood 540 History and Physical Examination 541 Vulvovaginitis 542 Bleeding 543 Breast Disorders 544 Hirsutism and Polycystic Ovarian Syndrome

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545 Neoplasms 546 Vulvovaginal and Mllerian Anomalies 547 Athletic Gynecologic Problems 548 Special Gynecologic Needs 549 Gynecologic Imaging Part XXV The Endocrine System 1 Disorders of the Hypothalamus and Pituitary Gland 550 Hormones of the Hypothalamus and Pituitary 551 Hypopituitarism 552 Diabetes Insipidus 552.1 Causes of Hypernatremia 553 Other Abnormalities of Arginine Vasopressin Metabolism and Action 554 Hyperpituitarism, Tall Stature, and Overgrowth Syndromes 555 Physiology of Puberty 556 Disorders of Pubertal Development 556.1 Gonadotropin-Dependent Precocious Puberty 556.2 Precocious Puberty Resulting from Organic Brain Lesions 556.3 Precocious Puberty Following Irradiation of the Brain 556.4 Syndrome of Precocious Puberty and Hypothyroidism 556.5 Gonadotropin-Secreting Tumors 556.6 McCune-Albright Syndrome 556.7 Familial Male Gonadotropin-Independent Precocious Puberty 556.8 Incomplete Precocious Development 556.9 Medicational Precocity 2 Disorders of the Thyroid Gland 557 Thyroid Development and Physiology 557.1 Thyroid Hormone Studies 558 Defects of Thyroxine-Binding Globulin 559 Hypothyroidism 560 Thyroiditis 561 Goiter 561.1 Congenital Goiter 561.2 Endemic Goiter and Cretinism 561.3 Sporadic Goiter 561.4 Intratracheal Goiter 562 Hyperthyroidism 562.1 Graves Disease 562.2 Congenital Hyperthyroidism 563 Carcinoma of the Thyroid 563.1 Solitary Thyroid Nodule 563.2 Medullary Carcinoma 3 Disorders of the Parathyroid 564 Hormones and Peptides of Calcium Homeostasis and Bone Metabolism 565 Hypoparathyroidism 566 Pseudohypoparathyroidism

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567 Hyperparathyroidism 4 Disorders of the Adrenal Glands 568 The Physiology of the Adrenal Gland 568.1Histology and Embryology 568.2 Adrenal Steroid Biosynthesis 568.3 Regulation of the Adrenal Cortex 568.4 Adrenal Steroid Hormone Actions 568.5 Adrenal Medulla 569 Adrenocortical Insufficiency 569.1 Primary Adrenal Insufficiency 569.2 Secondary Adrenal Insufficiency 570 Congenital Adrenal Hyperplasia and Related Disorders 570.1 Congenital Adrenal Hyperplasia due to 21Hydroxylase Deficiency 570.2 Congenital Adrenal Hyperplasia due to 11Hydroxylase Deficiency 570.3 Congenital Adrenal Hyperplasia due to 3Hydroxysteroid Dehydrogenase Deficiency 570.4 Congenital Adrenal Hyperplasia due to 17Hydroxylase Deficiency 570.5 Lipoid Adrenal Hyperplasia 570.6 Aldosterone Synthase Deficiency 570.7 Glucocorticoid-Suppressible Hyperaldosteronism 571 Cushing Syndrome 572 Primary Aldosteronism 573 Adrenal Tumors 573.1 Virilizing Adrenocortical Tumors 573.2 Feminizing Adrenal Tumors 574 Pheochromocytoma 575 Adrenal Masses 575.1 Adrenal Incidentaloma 575.2 Adrenal Calcification 5 Disorders of the Gonads 576 Development and Function of the Gonads 577 Hypofunction of the Testes 577.1 Hypergonadotropic Hypogonadism in the Male 577.2 Hypogonadotropic Hypogonadism in the Male 578 Pseudoprecocity Resulting from Tumors of the Testes 579 Gynecomastia 580 Hypofunction of the Ovaries 580.1 Hypergonadotropic Hypogonadism in the Female 580.2 Hypogonadotropic Hypogonadism in the Female 581 Pseudoprecocity Due to Lesions of the Ovary 582 Intersex 582.1 46,XX Intersex 582.2 46,XY Intersex 582.3 True Gonadal Intersex 6 Diabetes Mellitus in Children 583 Diabetes Mellitus 583.1 Introduction and Classification 583.2 Type 1 Diabetes Mellitus

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583.3 Type 2 Diabetes Mellitus 583.4 Other Specific Types of Diabetes Part XXVI The Nervous System 584 Neurologic Evaluation 585 Congenital Anomalies of the Central Nervous System 585.1 Neural Tube Defects 585.2 Spina Bifida Occulta 585.3 Meningocele 585.4 Myelomeningocele 585.5 Encephalocele 585.6 Anencephaly 585.7 Disorders of Neuronal Migration 585.8 Agenesis of the Corpus Callosum 585.9 Agenesis of the Cranial Nerves 585.10 Microcephaly 585.11 Hydrocephalus 585.12 Craniosynostosis 586 Seizures in Childhood 586.1 Febrile Seizures 586.2 Unprovoked Seizures 586.3 Partial Seizures 586.4 Generalized Seizures 586.5 Mechanisms of Seizures 586.6 Treatment of Epilepsy 586.7 Neonatal Seizures 586.8 Status Epilepticus 587 Conditions that Mimic Seizures 588 Headaches 588.1 Migraine 588.2 Organic Headaches 588.3 Tension or Stress Headaches 589 Neurocutaneous Syndromes 589.1 Neurofibromatosis 589.2 Tuberous Sclerosis 589.3 Sturge-Weber Disease 589.4 von Hippel-Lindau Disease 589.5 Linear Nevus Syndrome 589.6 PHACE Syndrome 590 Movement Disorders 590.1 Ataxias 590.2 Chorea, Athetosis, Tremor 590.3 Dystonia 590.4 Tics 591 Encephalopathies 591.1 Cerebral Palsy

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591.2 Mitochondrial Encephalomyopathies 591.3 Other Encephalopathies 592 Neurodegenerative Disorders of Childhood 592.1 Sphingolipidoses 592.2 Neuronal Ceroid Lipofuscinoses 592.3 Adrenoleukodystrophy 592.4 Sialidosis 592.5 Miscellaneous Disorders 593 Acute Stroke Syndromes 593.1 Arterial Thrombosis/Embolism 593.2 Venous Thrombosis 593.3 Intracranial Hemorrhage 593.4 Differential Diagnosis of Strokelike Events 594 Central Nervous System Infections 594.1 Acute Bacterial Meningitis Beyond the Neonatal Period 594.2 Viral Meningoencephalitis 594.3 Eosinophilic Meningitis 595 Brain Abscess 596 Pseudotumor Cerebri 597 Spinal Cord Disorders 597.1 Spinal Cord Tumors 597.2 Spinal Cord Trauma 597.3 Tethered Cord 597.4 Diastematomyelia 597.5 Syringomyelia 597.6 Transverse Myelitis 597.7 Arteriovenous Malformation Part XXVII Neuromuscular Disorders 598 Evaluation and Investigation 599 Developmental Disorders of Muscle 599.1 Myotubular Myopathy 599.2 Congenital Muscle Fiber-Type Disproportion 599.3 Nemaline Rod Myopathy 599.4 Central Core Disease 599.5 Brain Malformations and Muscle Development 599.6 Amyoplasia 599.7 Muscular Dysgenesis 599.8 Benign Congenital Hypotonia 599.9 Arthrogryposis 600 Muscular Dystrophies 600.1 Duchenne and Becker Muscular Dystrophies 600.2 Emery-Dreifuss Muscular Dystrophy 600.3 Myotonic Muscular Dystrophy 600.4 Limb-Girdle Muscular Dystrophy 600.5 Facioscapulohumeral Muscular Dystrophy 600.6 Congenital Muscular Dystrophy

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601 Endocrine Myopathies 602 Metabolic Myopathies 602.1 Periodic Paralyses 602.2 Malignant Hyperthermia 602.3 Glycogenoses 602.4 Mitochondrial Myopathies 602.5 Lipid Myopathies 602.6 Vitamin E Deficiency Myopathy 603 Disorders of Neuromuscular Transmission and of Motor Neurons 603.1 Myasthenia Gravis 603.2 Spinal Muscular Atrophies 603.3 Other Motor Neuron Diseases 604 Hereditary Motor-Sensory Neuropathies 604.1 Peroneal Muscular Atrophy 604.2 Peroneal Muscular Atrophy 604.3 Dejerine-Sottas Disease 604.4 Roussy-Lvy Syndrome 604.5 Refsum Disease 604.6 Fabry Disease 604.7 Giant Axonal Neuropathy 604.8 Congenital Hypomyelinating Neuropathy 604.9 Tomaculous Neuropathy 604.10 Leukodystrophies 605 Toxic Neuropathies 606 Autonomic Neuropathies 606.1 Familial Dysautonomia 606.2 Other Autonomic Neuropathies 607 Guillain-Barr Syndrome 608 Bell Palsy Part XXVIII Disorders of the Eye 609 Growth and Development 610 Examination of the Eye 611 Abnormalities of Refraction and Accommodation 612 Disorders of Vision 613 Abnormalities of Pupil and Iris 614 Disorders of Eye Movement and Alignment 615 Abnormalities of the Lids 616 Disorders of the Lacrimal System 617 Disorders of the Conjunctiva 618 Abnormalities of the Cornea 619 Abnormalities of the Lens 620 Disorders of the Uveal Tract 621 Disorders of the Retina and Vitreous 622 Abnormalities of the Optic Nerve 623 Childhood Glaucoma

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624 Orbital Abnormalities 625 Injuries to the Eye Part XXIX The Ear 626 Clinical Manifestations 627 Hearing Loss 628 Congenital Malformations 629 Diseases of the External Ear 630 Otitis Media 631 The Inner Ear and Diseases of the Bony Labyrinth 632 Traumatic Injuries of the Ear and Temporal Bone 633 Tumors of the Ear and Temporal Bone Part XXX The Skin 634 Morphology of the Skin 635 Evaluation of the Patient 635.1 Cutaneous Manifestations of Systemic Diseases 636 Principles of Therapy 637 Diseases of the Neonate 638 Cutaneous Defects 639 Ectodermal Dysplasias 640 Vascular Disorders 641 Cutaneous Nevi 642 Hyperpigmented Lesions 643 Hypopigmented Lesions 644 Vesiculobullous Disorders 645 Eczematous Disorders 646 Photosensitivity 647 Diseases of the Epidermis 648 Disorders of Keratinization 649 Diseases of the Dermis 650 Diseases of Subcutaneous Tissue 651 Disorders of the Sweat Glands 652 Disorders of Hair 653 Disorders of the Nails 654 Disorders of the Mucous Membranes 655 Cutaneous Bacterial Infections 656 Cutaneous Fungal Infections 657 Cutaneous Viral Infections 658 Arthropod Bites and Infestations 659 Acne 660 Tumors of the Skin 661 Nutritional Dermatoses Part XXXI Bone and Joint Disorders 1 Orthopedic Problems 662 Growth and Development 663 Evaluation of the Child 664 The Foot and Toes

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664.1 Metatarsus Adductus 664.2 Calcaneovalgus Feet 664.3 Talipes Equinovarus 664.4 Congenital Vertical Talus 664.5 Hypermobile Pes Planus 664.6 Tarsal Coalition 664.7 Cavus Feet 664.8 Osteochondroses 664.9 Puncture Wounds of the Foot 664.10 Toe Deformities 664.11 Painful Foot 664.12 Shoes 665 Torsional and Angular Deformities 665.1 Normal Developmental Alignment 665.2 Internal Femoral Torsion 665.3 Internal Tibial Torsion 665.4 External Femoral Torsion 665.5 External Tibial Torsion 665.6 Genu Varum 665.7 Genu Valgum 665.8 Congenital Angular Deformities of the Tibia and Fibula 666 Leg-Length Discrepancy 667 The Knee 667.1 Discoid Lateral Meniscus 667.2 Popliteal Cyst 667.3 Osteochondritis Dissecans 667.4 Osgood-Schlatter Disease 667.5 Idiopathic Adolescent Anterior Knee Pain Syndrome 667.6 Patella Subluxation and Dislocation 668 The Hip 668.1 Developmental Dysplasia of the Hip 668.2 Transient Monoarticular Synovitis 668.3 Legg-Calv-Perthes Disease 668.4 Slipped Capital Femoral Epiphysis 669 The Spine 669.1 Idiopathic Scoliosis 669.2 Congenital Scoliosis 669.3 Neuromuscular Scoliosis, Genetic Syndromes, and Compensatory Scoliosis 669.4 Kyphosis 669.5 Back Pain in Children 669.6 Spondylolysis and Spondylolisthesis 669.7 Disk Space Infection 669.8 Intervertebral Disk Herniation 669.9 Tumors 670 The Neck

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670.1 Torticollis 670.2 Klippel-Feil Syndrome 670.3 Atlantoaxial Instability 671 The Upper Limb 671.1 Sprengel Deformity 671.2 Shoulder Dislocation 671.3 Nursemaid's Elbow 671.4 Panner Disease 671.5 Ganglion 671.6 Radial Clubhand 671.7 Polydactyly 671.8 Syndactyly 671.9 Congenital Trigger Thumb and Finger 672 Arthrogryposis 672.1 Amyoplasia 673 Common Fractures 673.1 Pediatric Fracture Patterns 673.2 Clavicular Fractures 673.3 Proximal Humerus Fractures 673.4 Distal Humerus Fractures 673.5 Distal Radius and Ulna Fractures 673.6 Phalangeal Fractures 673.7 Toddler Fracture 673.8 Lateral Malleolar Fractures 673.9 Metatarsal Fractures 673.10 Toe Phalangeal Fractures 673.11 Operative Treatment 674 Osteomyelitis and Suppurative Arthritis 2 Sports Medicine 675 Epidemiology and Prevention of Injuries 676 Management of Musculoskeletal Injury 676.1 Growth Plate Injuries 676.2 Shoulder Injuries 676.3 Elbow Injuries 676.4 Back Injuries 676.5 Hip and Pelvis Injuries 676.6 Knee Injuries 676.7 Shin Splints and Stress Fractures of the Lower Leg 676.8 Ankle Injuries 676.9 Foot Injuries 677 Head and Neck Injuries 678 Heat Injuries 679 Female Athletes: Menstrual Problems and the Risk for Osteopenia 680 Ergogenic Aids 681 Specific Sports and Associated Injuries

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3 The Skeletal Dysplasias 682 General Considerations 683 Disorders Involving Cartilage Matrix Proteins 684 Disorders Involving Transmembrane Receptors 685 Disorders Involving Ion Transporter 686 Disorders Involving Transcription Factors 687 Disorders Involving Defective Bone Resorption 688 Disorders for Which Defects Are Poorly Understood or Unknown 689 Osteogenesis Imperfecta 690 Marfan Syndrome 4 Metabolic Bone Disease 691 Bone Structure, Growth, and Hormonal Regulation 692 Primary Chondrodystrophy 693 Idiopathic Hypercalcemia 694 Hypophosphatasia 695 Hyperphosphatasia 696 Familial Hypophosphatemia 697 Vitamin D-Dependent Rickets 698 Oncogenous Rickets 699 Osteoporosis Part XXXII Environmental Health Hazards 700 Pediatric Radiation Injuries 701 Chemical Pollutants 702 Heavy Metal Intoxication 703 Lead Poisoning Poisonings: Drugs, Chemicals, and Plants 704.1 Epidemiology and Approach to Management 704.2 Acetaminophen 704.3 Salicylates 704.4 Ibuprofen 704.5 Antidepressants 704.6 Clonidine 704.7 Calcium Channel Blockers 704.8 Iron 704.9 Caustics 704.10 Methanol and Ethylene Glycol 704.11 Hydrocarbons 704.12 Cholinesterase-Inhibiting Insecticides 704.13 Toxic Gases 704.14 Plants 705 Nonbacterial Food Poisoning 705.1 Mushroom Poisoning 705.2 Solanine Poisoning 705.3 Seafood Poisoning 706 Biologic and Chemical Terrorism 707 Animal and Human Bites

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708 Envenomations Part XXXIII Laboratory Medicine, Drug Therapy, and Reference Tables 709 Laboratory Testing in Infants and Children 710 Reference Ranges for Laboratory Tests and Procedures 711 Principles of Drug Therapy 712 Medications 713 Herbal Medicines

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