Nelson Textbook of Pediatrics, 18th edition
By Robert M. Kliegman, MD, Professor and Chair, Department of Pediatrics, Medical
College of Wisconsin, Milwaukee, WI; Richard E. Behrman, MD, Executive Chair, Pediatric
Education Steering Committee, Federation of Pediatric Organizations, Menlo Park, CA;
Clinical Professor of Pediatrics, Stanford University and the University of California, San
Francisco, CA and George Washington University, Washington, DC; Hal B. Jenson, MD,
Chair, Department of Pediatrics, Director, Center for Pediatric Research, and Senior VicePresident for Academic Affairs, Eastern Virginia Medical School and Children's Hospital of the
King's Daughters, Norfolk, VA; and Bonita F. Stanton, MD, Schotanus Professor and Chair,
Department of Pediatrics, Wayne State University, Detroit, MI, USA
ISBN 1416024506 / 9781416024507 · Hardback · 3200 Pages · 1800 Illustrations
Saunders · Published August 2007
Price: £ 87.99
For nearly three quarters of a century, Nelson Textbook of Pediatrics has been the
world’s most trusted resource for best approaches to pediatric care. Now in full color for
easier referencing, this New Edition continues the tradition, incorporating a wealth of
exciting updates and changes—ensuring you have access to today’s authoritative
knowledge to best diagnose and treat every pediatric patient you see. Whether you’re
treating patients in the office or in the hospital, or preparing for the boards, Nelson
Textbook of Pediatrics, 18th Edition is your comprehensive guide to providing the best
possible care.
Features
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Get an enhanced focus on general pediatrics with editorial contributions from
new editor Dr. Bonita F. Stanton.
Treat your inpatient and ambulatory patients more effectively with the absolute
latest on new topics such as quality improvement and patient care safety •
school violence and bullying • preventive measures • vitamin deficiencies •
adolescent rape • effect of war on children • and more.
Improve your therapeutic skills with the newest knowledge on the principles of
antibiotic therapy • antiviral therapy • antiparasitic therapy • antimycobacterial
therapy • and others.
Understand the principles of therapy and which drugs and dosages to prescribe
for every disease.
Locate key content more easily and identify clinical conditions quicker thanks to
a new full-color design and full-color photographs.
Contents
Part I: The Field of Pediatrics
1 Overview of Pediatrics 2 Quality and Safety in Healthcare for Children 3 Ethics in Pediatric
Care 4 Cultural Issues in Pediatric Care 5 Maximizing Children’s Health: Screening,
Anticipatory Guidance, and Counseling
Part II: Growth, Development, and Behavior
6 Overview and Assessment of Variability 6.1 Assessment of Fetal Growth and Development
7 The Newborn 8 The First Year 9 The Second Year 10 Preschool Years 11 Middle
Childhood 12 Adolescence 13 Sexual Behavior 13.1 The Development of Sexual Behavior
13.2 Gender Identity Disorder (GID) 13.3 Adolescent Homosexuality 14 Assessment of
Growth 15 Developmental Screening and Surveillance 16 Child Care: How Pediatricians Can
Support Children and Famillies 17 Separation, Loss, and Bereavement 18 Sleep Medicine
Part III: Child and Adolescent Psychology
19 Assessment and Interviewing 20 Psychological Treatment of Children and Adolescents
20.1 Illness and Death 20.2 Psychopharmacology 20.3 Psychotherapy 20.4 Psychiatric
Hospitalization 21 Psychosomatic Illness 22 Vegetative Disorders 22.1 Rumination Disorders
22.2 Pica 22.3 Enuresis (Bedwetting) 22.4 Encopresis 23 Habit and Tic Disorders 24 Anxiety
Disorders 25 Mood Disorders 25.1 Major Depression 25.2 Dysthymic Disorder 25.3 Bipolar
Disorder 26 Suicide and Attempted Suicide 27 Anorexia Nervosa, Bulimia, and Binge Eating
28 Disruptive Behavioral Disorders 29 Pervasive Developmental Disorders and Childhood
Psychosis 29.1 Autistic Disorder 29.2 Asperger’s Disorder 29.3 Childhood Disintegrative
Disorder 29.4 Rett's Disorder 29.5 Childhood Schizophrenia 29.6 Acute Phobic Hallucinations
30 Patterns of Development and Function in the School-Aged Child 31 Attention Deficit
Hyperactivity Disorder 32 Specific Reading Disability 32.1 Language Development and
Communication Disorders
PART IV: Children with Special Needs
33 Adoption 34 Foster Care 35 Impact of Violence on Children 35.1 School Violence and
Bullying 35.2 Effect of War on Children 36 Abuse and Neglect of Children 36.1 Sexual Abuse
36.2 Munchausen Syndrome by Proxy 36.3 Parental Styles 37 Failure to Thrive 38
Developmental Disabilities and Chronic Illness 38.1 Mental Retardation (Intellectual Disability)
39 Organ Transplantation 40 Pediatric Palliative Care: The Care of Children with Life-Limiting
Illness 40.1 Grief and Bereavement
Part V: Nutrition
41 Nutritional Requirements 42 The Feeding of Infants and Children 43 Food Insecurity,
Hunger, and Undernutrition 44 Overweight and Obesity 45 Vitamin A Deficiency and Excess
46 Vitamin B Complex Deficiency and Excess 46.1 Thiamin (Vitamin B-1) 46.2 Riboflavin
(Vitamin B-2) 46.3 Niacin 46.4 Vitamin B-6 (Pyridoxine) 46.5 Biotin 46.6 Folate 46.7 Vitamin
B-12 (Cobalamin) 47 Vitamin C Deficiency (Ascorbic Acid) 48 Rickets and Hypervitaminosis D
49 Vitamin E Deficiency 50 Vitamin K Deficiency 51 Micronutrient Deficiency
Part VI: The Pathophysiology of Body Fluids and Fluid Therapy
52 Electrolyte and Acid-Base Disorders 52.1 Composition of Body Fluids 52.2 Regulation of
Osmolality and Volume 52.3 Sodium 52.4 Potassium 52.5 Magnesium 52.6 Phosphorus 52.7
Acid-Base Balance 53 Maintenance and Replacement Therapy 54 Deficit Therapy 55 Fluids
and Electrolyte Treatment of Specific Disorders 55.1 Acute Diarrhea and Oral Rehydration
55.2 Diarrhea in Chronically Malnourished Children 55.3 Pyloric Stenosis 55.4 Perioperative
Fluids
Part VII: Pediatric Drug Therapy
56 Pharmacogenetics, Pharmacogenomics, and Pharmacoproteins 57 Principles of Drug
Therapy 58 Poisonings 59 Herbal Medicines
Part VIII: Critical Care Medicine
60 Evaluation of the Sick Child in the Office and Clinic 61 Injury Control 62 Emergency
Medical Services for Children 63 Interfacility Transfer of the Critically Ill Infant and Child 64
Monitoring Techniques for the Critically Ill Infant and Child 65 Scoring Systems and Predictors
of Mortality 66 Pediatric Emergencies and Resuscitation 67 Neurologic Emergencies and
Stabilization 67.1 Brain Death 68 Shock 69 Respiratory Distress and Failure 70 Mechanical
Ventilation 70.1 Chronic Mechanical Ventilation 71 Acute Care of the Multiple Trauma Victim
72 Nutritional Stabilization 73 Drowning 74 Burn injuries 75 Cold Injuries 76 Anesthesia and
Perioperative Care 77 Pediatric Pain Management
Part IX: Human Genetics
78 The Genetic Approach in Pediatric Medicine 79 The Human Genome 80 Patterns of
Genetic Transmission 81 Cytogenetics 82 Genetics of Common Disorders: Diagnosis and
Management 83 Integration of Genetics into Pediatric Practice
Part X: Metabolic Diseases
84 An Approach to Inborn Errors of Metabolism 85 Defects in Metabolism of Amino Acids
85.1 Phenylalanine 85.2 Tyrosine 85.3 Methionine 85.4 Cysteine/Cystine 85.5 Tryptophan
85.6 Valine, Leucine, Isoleucine, and Related Organic Acidemias 85.7 Glycine 85.8 Serine
85.9 Proline 85.1-0 Glutamic Acid 85.11 Urea Cycle and Hyperammonemia (Arginine,
Citrulline, Ornithine) 85.12 Histidine 85.13 Lysine 85.14 Aspartic Acid (Canavan Disease) 86
Defects in Metabolism of Lipids 86.1 Disorders of Mitochondrial Fatty Acid β-Oxidation 86.2
Disorders of Very Long Chain Fatty Acids 86.3 Disorders of Lipoprotein Metabolism and
Transport: Epidemiology of Blood Lipids and Cardiovascular Disease 86.4 Lipidoses 86.5
Mucolipidoses 87 Defects in Metabolism of Carbohydrates 87.1 Glycogen Storage Diseases
87.2 Defects in Galactose Metabolism 87.3 Defects in Fructose Metabolism 87.4 Defects in
Intermediary Carbohydrate Metabolism Associated with Lactic Acidosis 87.5 Defects in
Pentose Metabolism 87.6 Disorders of Glycoprotein Degradation and Structure 88
Mucopolysaccharidoses 89 Disorders of Purine and Pyrimidine Metabolism 90 Progeria 91
The Porphyrias 92 Hypoglycemia
Part XI: Perinatal and Neonatal Medicine
93 Overview of Mortality and Morbidity 94 The Newborn Infant 94.1 History in Neonatal
Pediatrics 94.2 Physical Examination of the Newborn Infant 94.3 Routine Delivery Room Care
94.4 Nursery Care 94.5 Parent-Infant Bonding 95 High-Risk Pregnancies 96 The Fetus 96.1
Fetal Growth and Maturity 96.2 Fetal Distress 96.3 Maternal Disease and the Fetus 96.4
Maternal Medication and Toxin Exposure and the Fetus 96.5 Teratogens 96.6 Radiation 96.7
Intrauterine Diagnosis of Fetal Disease 96.8 Treatment and Prevention of Fetal Disease 97
The High-Risk Infant 97.1 Multiple Gestation Pregnancies 97.2 Prematurity and Intrauterine
Growth Retardation 97.3 Post-Term Infants 97.4 Large for Gestational Age 97.5 Infant
Transport 98 Clinical Manifestations of Diseases in the Newborn Period 99 Nervous System
Disorders 99.1 The Cranium 99.2 Traumatic, Epidural, Subdural, and Subarachnoid
Hemorrhage 99.3 Intracranial-Intraventricular Hemorrhage and Periventricular Leukomalacia
99.4 Brain Injury from Inflammation, Infection, and Medications 99.5 Hypoxia-Ischemia 99.6
Spine and Spinal Cord 99.7 Peripheral Nerve Injuries 100 Delivery Room Emergencies 101
Respiratory Tract Disorders 101.1 Transition to Pulmonary Respiration 101.2 Apnea 101.3
Congenital Central Hypoventilation Syndrome 101.4 Respiratory Distress Syndrome (Hyaline
Membrane Disease) 101.5 Transient Tachypnea of the Newborn 101.6 Aspiration of Foreign
Material (Fetal Aspiration Syndrome, Aspiration Pneumonia) 101.7 Meconium Aspiration
101.8 Persistent Pulmonary Hypertension of the Newborn (Persistent Fetal Circulation) 101.9
Diaphragmatic Hernia 101.10 Foramen of Morgagni Hernia 101.11 Paraesophageal Hernia
101.12 Eventration 101.13 Extrapulmonary Extravasation of Air (Pneumothorax,
Pneumomediastinum, Pulmonary Interstitial Emphysema) 101.14 Pulmonary Hemorrhage
102 Digestive System Disorders 102.1 Meconium Ileus in Cystic Fibrosis 102.2 Neonatal
Necrotizing Enterocolitis (NEC) 102.3 Jaundice and Hyperbilirubinemia in the Newborn 102.4
Kernicterus 103 Blood Disorders 103.1 Anemia in the Newborn Infant 103.2 Hemolytic
Disease of the Newborn (Erythroblastosis Fetalis) 103.3 Plethora in the Newborn Infant
(Polycythemia) 103.4 Hemorrhage in the Newborn Infant 104 Genitourinary System 105 The
Umbilicus 106 Metabolic Disturbances 106.1 Maternal Selective Serotonin Reuptake
Inhibitors and Neonatal Behavioral Syndromes 106.2 Fetal Alcohol Syndrome 107 The
Endocrine System 107.1 Infants of Diabetic Mothers 107.2 Hypoglycemia 108
Dysmorphology 109 Infections of the Neonatal Infant 109.1 Pathogenesis and Epidemiology
109.2 Modes of Transmission and Pathogenesis 109.3 Immunity 109.4 Etiology of Fetal and
Neonatal Infection 109.5 Epidemiology of Early- and Late-Onset Neonatal Infections 109.6
Clinical Manifestations of Transplacental Intrauterine Infections 109.7 Diagnosis 109.8
Treatment 109.9 Complications and Prognosis 109.1'0 Prevention
Part XII: Adolescent Medicine
110 The Epidemiology of Adolescent Health Problems 111 Delivery of Health Care to
Adolescents 111.1 Legal Issues 111.2 Screening Procedures 111.3 Health Enhancement 112
Violent Behavior 113 Substance Abuse 113.1 Alcohol 113.2 Tobacco 113.3 Marijuana 113.4
Volatile Inhalants 113.5 Hallucinogens 113.6 Cocaine 113.7 Amphetamines 113.8 Opiates
113.9 Anabolic Steroids 114 The Breast 115 Menstrual Problems 115.1 Amenorrhea 115.2
Abnormal Uterine Bleeding 115.3 Dysmenorrhea 115.4 Premenstrual Syndrome 116
Contraception 116.1 Barrier Methods 116.2 Spermicides 116.3 Combination Methods 116.4
Hormonal Methods 116.5 Emergency Contraception 116.6 Intrauterine Devices 117
Adolescent Pregnancy 118 Adolescent Rape 119 Sexually Transmitted Infections 120
Chronic Fatigue Syndrome
Part XIII: Immunology
Section 1: Evaluation of the Immune Systems 121 Evaluation of Suspected Immunodeficiency
Section 2: The T-, B-, and NK-Cell Systems 122 T Lymphocytes, B Lymphocytes, and Natural
Killer Cells 123 Primary Defects of Antibody Production 123.1 Treatment of B-Cell Defects
124 Primary Defects of Cellular Immunity 125 Primary Combined Antibody and Cellular
Immunodeficiencies 125.1 Severe Combined Immunodeficiency (SCID) 125.2 Combined
Immunodeficiency (CID) 125.3 Defects of Innate Immunity 125.4 Treatment of Cellular or
Combined Immunodeficiency 125.5 Immune Dysregulation with Autoimmunity or
Lymphoproliferation Section 3: The Phagocytic System 126 Neutrophils 127 Monocytes and
Macrophages and Dendritic Cells 128 Eosinophils 129 Disorders of Phagocyte Function 130
Leukopenia 131 Leukocytosis Section 4: The Complement System 132 The Complement
System 133 Disorders of the Complement System 133.1 Evaluation of the Complement
System 133.2 Genetic Deficiencies of Complement Components 133.3 Deficiencies of
Plasma, Membrane, or Serosal Complement Control Proteins 133.4 Secondary Disorders of
Complement 133.5 Treatment of Complement Disorders Section 5: 134 Principles and
Clinical Indications 135 HSCT from Alternative Sources and Donors 136 Graft vs Host
Disease (GVHD) and Rejection 137 Infectious Complications of HSCT 138 Late Effects of
HSCT
Part XIV: Allergy
139 Allergy and the Immunologic Basis of Atopic Disease 140 Diagnosis of Allergic Disease
141 Principles of Treatment of Allergic Disease 142 Allergic Rhinitis 143 Childhood Asthma
144 Atopic Dermatitis (Atopic Eczema) 145 Insect Allergy 146 Ocular Allergies 147 Urticaria
and Angioedema (Hives) 148 Anaphylaxis 149 Serum Sickness 150 Adverse Reactions to
Foods 151 Adverse Reactions to Drugs
Part XV: Rheumatology
152 Evaluation of Suspected Rheumatic Disease 153 Treatment of Rheumatic Diseases 154
Juvenile Rheumatoid Arthritis 155 Ankylosing Spondylitis and Other Spondyloarthropathies
156 Reactive Arthritis 157 Systemic Lupus Erythematosus 157.1 Neonatal Lupus 158
Juvenile Dermatomyositis 159 Scleroderma and Raynaud Syndrome 160 BehVet’s Disease
161 Sjögren’s Syndrome 162 Hereditary Periodic Fever Syndromes 163 Amyloidosis 164
Sarcoidosis 165 Kawasaki Disease 166 Vasculitis Syndromes 166.1 Henoch-Schönlein
Purpura 166.2 Takayasu Arteritis 166.3 Polyarteritis Nodosa 166.4 Wegener Granulomatosis
166.5 Other Vasculitic Syndromes 167 Musculoskeletal Pain Syndromes 167.1 Fibromyalgia
167.2 Complex Regional Pain Syndrome (Reflex Sympathetic Dystrophy 167.3
Erythromelalgia 168 Miscellaneous Conditions Associated With Arthritis
Part XVI: Infectious Diseases
Section 1. General Considerations 169 Diagnostic Microbiology Section 2. Preventive
Measures (NEW) 170 (NEW) Immunization Practices 170.1 (NEW) International
Immunization Practices 171 Infection Control and Prophylaxis 172 Childcare and
Communicable Diseases 173 Health Advice for Children Traveling Internationally 174 Fever
175 Fever Without a Focus 176 Sepsis, Septic Shock, and System Inflammatory Response
Syndrome (SIRS) 177 Infections in Immunocompromised Persons 178 Infections Associated
with Medical Devices Section 3. Antibiotic Therapy (NEW) 179 Principles of Antibacterial
Therapy Section 4. Gram-Positive Bacterial Infections 180 Staphylococcus 180.1
Staphylococcus aureus 180.2 Toxic Shock Syndrome 180.3 Coagulase-Negative
Staphylococci 181 Streptococcus pneumoniae (Pneumococcus) 182 Group A Streptococcus
182.1 Rheumatic Fever 183 Group B Streptococcus 184 Non–Group A or B Streptococcus
184 Enterococcus 186 Diphtheria (Corynebacterium diphtheriae) 187 Listeria monocytogenes
188 Actinomyces 189 Nocardia Section 5. Gram Negative Bacterial Infections 190 Neisseria
meningitidis (Meningococcus) 191 Neisseria gonorrhoeae (Gonococcus) 192 Haemophilus
influenzae 193 Chancroid (Haemophilus ducreyi) 194 Pertussis (Bordetella pertussis and B.
parapertussis) 195 Salmonella 195.1 Nontyphoidal Salmonellosis 195.2 Enteric Fever
(Typhoid Fever) 196 Shigella 197 Escherichia coli 198 Cholera (Vibrio cholerae) 199
Campylobacter 200 Yersinia 200.1 Yersinia enterocolitica 200.2 Yersinia pseudotuberculosis
200.3 Plague (Yersinia pestis) 201 Aeromonas and Plesiomonas 201.1 Aeromonas 201.2
Plesiomonas shigelloides 202 Pseudomonas, Burkholderia, and Stenotrophomonas 202.1
Pseudomonas aeruginosa 202.2 Burkholderia 202.3 Stenotrophomonas 203 Tularemia
(Francisella tularensis) 204 Brucella 205 Legionella 206 Bartonella 206.1 Bartonellosis
(Bartonella bacilliformis) 206.2 Cat-Scratch Disease (Bartonella henselae) 206.3 Trench
Fever (Bartonella quintana) 206.4 Bacillary Angiomatosis and Bacillary Peliosis Hepatis
(Bartonellahenselae and Bartonella quintana) Section 6: Anaerobic Bacterial Infections 207
Botulism (Clostridium botulinum) 208 Tetanus (Clostridium tetani) 209 Pseudomembranous
Colitis (Clostridium difficile) 210 Other Anaerobic Infections Section 7: Mycobacterial
Infections 211 (NEW) Principles of Antimycobacterial Therapy 212 Tuberculosis
(Mycobacterium tuberculosis) 213 Hansen Disease (Mycobacterium leprae) 214
Nontuberculous Mycobacteria Section 8. Spirochectal Infections 215 Syphilis (Treponema
pallidum) 216 Nonvenereal Treponemal Infections 216.1 Yaws (Treponema pertenue) 216.2
Bejel Endemic Syphilis (Treponema pallidum subsp. endemicum) 216.3 Pinta (Treponema
carateum) 217 Leptospira 218 Relapsing Fever (Borrelia) 219 Lyme Disease (Borrelia
burgdorferi) Section 9. Mycroplasmal Infections 220 Mycoplasma pneumoniae 221 Genital
Mycoplasmas (Mycoplasma hominis, Mycoplasma Genitalium, and Ureaplasma urealyticum)
Section 10. Chlamydial Infections 222 Chlamydiophila pneumoniae 223 Chlamydia
trachomatis 223.1 Trachoma 223.2 Genital Tract Infections 223.3 Conjunctivitis and
Pneumonia in Newborns 223.4 Lymphogranuloma Venereum (LGV) 224 Psittacosis
(Chlamydia psittaci) Section 11. Rickettsial Infections 225 Spotted Fever Group Rickettsioses
225.1 Rocky Mountain Spotted Fever (Rickettsia rickettsii) 225.2 Mediterranean Spotted
Fever or Boutonneuse Fever (Rickettsia conorii) 225.3 Rickettsialpox (Rickettsia akari) 226
Scrub Typhus (Orientia tsutsugamushi) 227 Typhus Group Rickettsioses 227.1 Murine
Typhus (Rickettsia typhi) 227.2 Epidemic Typhus (Rickettsia prowazekii) 228 Ehrlichioses and
Anaplasmosis 229 Q Fever (Coxiella burnetii) Section 12. Fungal Infections 230 Principles of
Antifungal Therapy 231 Candida 231.1 Neonatal Infections 231.2 Infections in
Immunocompetent Children and Adolescents 231.3 Infections in Immunocompromised
Children and Adolescents 231.4 Chronic Mucocutaneous Candidiasis 232 Cryptococcus
neoformans 233 Malassezia 234 Aspergillus 234.1 Hypersensitivity Syndromes 234.2
Saprophytic (Noninvasive) Syndromes 234.3 Invasive Disease 235 Histoplasmosis
(Histoplasma capsulatum) 236 Blastomycosis (Blastomyces dermatitidis) 237
Coccidioidomycosis (Coccidioides immitis) 238 Paracoccidioides brasiliensis 239
Sporotrichosis (Sporothrix schenckii) 240 Zygomycosis (Mucormycosis) 241 Pneumocystis
carinii (P. jiveci) Section 13. Viral Infections 242 Principles of Antiviral Therapy 243 Measles
244 Rubella 245 Mumps 246 Polioviruses 247 Nonpolio Enteroviruses 248 Parvovirus B19
249 Herpes Simplex Virus 250 Varicella-Zoster Virus 251 Epstein-Barr Virus 252
Cytomegalovirus 253 Roseola (Human Herpesviruses 6, 7) 254 Human Herpesvirus 8 255
Influenza Viruses 256 Parainfluenza Viruses 257 Respiratory Syncytial Virus 258 Human
Metapneumovirus 259 Adenoviruses 260 Rhinoviruses 261 Coronavirus 261.1 Severe Acute
Respiratory Syndrome (SARS)-associated corona virus 262 Rotaviruses, Calicivirus, and
Astrovirus (new title) 263 Human Papillomaviruses 264 Arboviral Encephalitis in North
America 265 Arboviral Encephalitis Outside North America 265.1 Venezuelan Equine
Encephalitis 265.2 Japanese Encephalitis 265.3 Tick-Borne Encephalitis 266 Dengue Fever
and Dengue Hemorrhagic Fever 267 Yellow Fever 268 Other Viral Hemorrhagic Fevers 269
Lymphocytic Choriomeningitis Virus (LCMV) 270 Hantavirus Pulmonary Syndrome 271
Rabies 272 Polyomaviruses (JC virus and BK Virus) 273 Acquired Immunodeficiency
Syndrome (Human Immunodeficiency Virus) 274 Human T-Cell Lymphotropic Viruses Types I
and II 275 Transmissible Spongiform Encephalopathies Section 14. Antiparasitic Therapy
(NEW)276 Principles of Antiparasitic Therapy Section 15. Protozoan Diseases 277 Primary
Amebic Meningoencephalitis 278 Amebiasis 279 Giardiasis and Balantidiasis 279.1 Giardia
lamblia 279.2 Balantidiasis 280 Cryptosporidium, Isospora, Cyclospora, and Microsporidia
281 Trichomoniasis (Trichomonas vaginalis) 282 Leishmaniasis (Leishmania) 283 African
Trypanosomiasis (Sleeping Sickness; Trypanosoma brucei complex) 284 American
Trypanosomiasis (Chagas Disease; Trypanosoma cruzi) 285 Malaria (Plasmodium) 286
Babesiosis (Babesia) 287 Toxoplasmosis (Toxoplasma gondii) Section 16. Helminthic
Diseases 288 Ascariasis (Ascaris lumbricoides) 289 Hookworms (Necator americanus and
Ancylostoma) 289.1 Cutaneous Larva Migrans 290 Trichuriasis (Trichuris trichuria) 291
Enterobiasis (Enterobius vermicularis) 292 Strongyloidiasis (Strongyloides stercoralis) 293
Lymphatic Filariasis (Brugia malayi, B. timori, and Wuchereria bancrofti) 294 Other Tissue
Nematodes 295 Toxocariasis (Visceral and Ocular Larva Migrans) 296 Trichinosis (Trichinella
spiralis) 297 Schistosomiasis (Schistosoma) 298 Flukes (Liver, Lung, and Intestinal) 299
Adult Tapeworm Infections 300 Cysticercosis 301 Echinococcosis (Echinococcus granulosus
and E. multilocularis)
Part XVII: The Digestive System
Section 1. Clinical Manifestations of Gastrointestinal Disease 302 Normal Digestive Tract
Phenomena 303 Major Symptoms and Signs of Digestive Tract Disorders Section 2. The Oral
Cavity 304 Development and Developmental Anomalies of the teeth 305 Disorders of the Oral
Cavity Associated with Other Conditions 306 Malocclusion 307 Cleft Lip and Palate 308
Syndromes with Oral Manifestations 309 Dental Caries 310 Periodontal Diseases 311 Dental
Trauma 312 Common Lesions of the Oral Soft Tissues 313 Diseases of the Salivary Glands
and Jaws 314 Diagnostic Radiology in Dental Assessment Section 3. The Esophagus 315
Embryology, Anatomy, and Function of the Esophagus 315.1 Common Clinical Manifestations
and Diagnostic Aids 316 Congenital Anomalies: Esophageal Atresia and Tracheoesophageal
Fistula 316.1 Congenital Anomalies: Laryngotracheoesophageal Clefts 317 Obstructing and
Motility Disorders of the Esophagus 318 Dysmotility 319 Hiatal Hernia 320 Gastroesophageal
Reflux Disease (GERD) 320.1 Complications of GERD 321 Non-GERD Esophagitis 322
Esophageal Perforation 323 Esophageal Varices 324 Ingestions 324.1 Foreign Bodies in the
Esophagus 324.2 Caustic Ingestions Section 4. Stomach and Intestines 325 Normal
Development, Structure, and Function 326 Pyloric Stenosis and Other Congenital Anomalies
of the Stomach 326.1 Hypertropic Pyloric Stenosis 326.2 Congenital Gastric Outlet
Obstruction 326.3 Gastric Duplication 326.4 Gastric Volvulus 326.5 Hypertrophic Gastropathy
327 Intestinal Atresia, Stenosis, and Malrotation 327.1 Duodenal Obstruction 327.2 Jejunal
and Ileal Atresia and Obstruction 327.3 Malrotation 328 Intestinal Duplications, Meckel
Diverticulum, and Other Remnants of the Omphalomesenteric Duct 328.1 Intestinal
Duplication 328.2 Meckel Diverticulum and Other Remnants of the Omphalomesenteric Duct
329 Motility Disorders and Hirschsprung Disease 329.1 Chronic Intestinal Pseudo-Obstruction
329.2 Functional Constipation 329.3 Congenital Aganglionic Megacolon (Hirschsprung
Disease) 329.4 Intestinal Neuronal Dysplasia 329.5 Superior Mesenteric Artery Syndrome,
Wilkie Syndrome, Cast Syndrome, Arteriomesenteric Duodenal Compression Syndrome 330
Ileus, Adhesions, Intussusception, and Closed-Loop Obstructions 330.1 Ileus 330.2
Adhesions 330.3 Intussusception 330.4 Closed-Loop Obstructions 331 Foreign Bodies and
Bezoars 331.1 Foreign Bodies in the Stomach and Intestine 331.2 Bezoars 332 Peptic Ulcer
Disease in Children 332.1 Zollinger-Ellison Syndrome 333 Inflammatory Bowel Disease 333.1
Chronic Ulcerative Colitis 333.2 Crohn Disease (Regional Enteritis, Regional lleitis,
Granulomatous Colitis) 333.3 Behçet Syndrome 334 Food Allergy (Food Hypersensitivity)
334.1 Eosinophilic Gastroenteritis 335 Disorders of Malabsorption 335.1 Evaluation of
Children with Suspected Intestinal Malabsorption 335.2 Gluten-Sensitive Enteropathy (Celiac
Disease) 335.3 Other Malabsorptive Sydromes 335.4 Intestinal Infections Associated with
Malabsorption 335.5 Immunodeficiency Disorders 335.6 Immunoproliferative Small Intestinal
Disease 335.7 Short Bowel Syndrome 335.8 Chronic Malnutrition 335.9 Enzyme Deficiencies
335.1-0 Liver and Biliary Disorders Causing Malabsorption 335.11 Defects of Absorption or
Transport 336 Intestinal Transplantation in Children 337 Acute Gastroenteritis in Children
(new) 337.1 Probiotics in Gastrointestinal Disorders (new) 338 Chronic Diarrhea 338.1
Diarrhea from Hormone-Secreting Tumors 339 Recurrent Abdominal Pain of Childhood 340
Acute Appendicitis 341 Surgical Conditions of the Anus, Rectum, and Colon 341.1 Anorectal
Malformations 341.2 Anal Fissure 341.3 Perianal Abscess and Fistula 341.4 Hemorrhoids
341.5 Rectal Prolapse 341.6 Pilonidal Sinus and Abscess 342 Tumors of the Digestive Tract
343 Inguinal Hernias Section 5. Exocrine Pancreas 344 Embryology, Anatomy, and
Physiology 344.1 Anatomic Abnormalities 344.2 Physiology 345 Pancreatic Function Tests
346 Disorders of the Exocrine Pancreas 347 Treatment of Pancreatic Insufficiency 348
Pancreatitis 348.1 Acute Pancreatitis 348.2 Chronic Pancreatitis 349 Pseudocyst of the
Pancreas 350 Pancreatic Tumors Section 6. The Liver and Biliary System 351
Morphogenesis of the Liver and Biliary System 352 Manifestations of Liver Disease 352.1
Evaluation of Patients with Possible Liver Dysfunction 353 Cholestasis 353.1 Neonatal
Cholestasis 353.2 Cholestasis in the Older Child 354 Metabolic Diseases of the Liver 354.1
Inherited Deficient Conjugation of Bilirubin (Familial Nonhemolytic Unconjugated
Hyperbilirubinemia) 354.2 Wilson Disease 354.3 Indian Childhood Cirrhosis 354.4 Neonatal
Iron Storage Disease (NISD) 354.5 Miscellaneous Metabolic Diseases of the Liver 355 Viral
Hepatitis 356 Liver Abscess 357 Liver Disease Associated with Systemic Disorders 358
Mitochondrial Hepatopathies 359 Autoimmune and Chronic Hepatitis 360 Drug- and ToxinInduced Liver Injury 361 Fulminant Hepatic Failure 362 Cystic Diseases of the Biliary Tract
and Liver 363 Diseases of the Gallbladder 364 Portal Hypertension and Varices 365 Liver
Transplantation Section 7. Peritoneum 366 Malformations 367 Ascites 367.1 Chylous Ascites
368 Peritonitis 368.1 Acute Primary Peritonitis 368.2 Acute Secondary Peritonitis 368.3 Acute
Secondary Localized Peritonitis (Peritoneal Abscess) 369 Epigastric Hernia 369.1 Incisional
Hernia
Part XVIII: Respiratory System
Section 1. Development and Function 370 Respiratory Pathophysiology and Regulation 370.1
Lung Volumes and Capacities in Health and Disease 370.2 Chest Wall 370.3 Pulmonary
Mechanics and Work of Breathing in Health and Disease 370.4 Airway Dynamics in Health
and Disease 370.5 Interpretation of Clinical Signs to Localize the Site of Pathology 370.6
Ventilation-Perfusion (V/Q) Relationship in Health and Disease 370.7 Gas Exchange in
Health and Disease 370.8 Interpretation of Blood Gases 370.9 Pulmonary Vasculature in
Health and Disease 370.1-0 Immune Response of the Lung to Injury 370.11 Regulation of
Respiration 371 Diagnostic Approach to Respiratory Disease 372 Sudden Infant Death
Syndrome Section 2. Disorders of the Respiratory Tract 373 Congenital Disorders of the Nose
374 Acquired Disorders of the Nose 374.1 Foreign Body 374.2 Epistaxis 375 Nasal Polyps
376 The Common Cold 377 Sinusitis 378 Acute Pharyngitis 379 Retropharyngeal Abscess,
Lateral Pharyngeal (Parapharyngeal) Abscess, and Peritonsillar Cellulitis/Abscess 380
Tonsils and Adenoids 381 Chronic or Recurrent Respiratory Symptoms 382 Acute
Inflammatory Upper Airway Obstruction (Croup, Epiglottitis, Laryngitis, and Bacterial
Tracheitis) 382.1 Infectious Upper Airway Obstruction 382.2 Bacterial Tracheitis 383
Congenital Anomalies of the Larynx, Trachea, and Bronchi 383.1 Laryngomalacia 383.2
Congenital Subglottic Stenosis 383.3 Vocal Cord Paralysis 383.4 Congenital Laryngeal Webs
and Atresia 383.5 Congenital Subglottic Hemangioma 383.6 Laryngoceles and Saccular
Cysts 383.7 Posterior Laryngeal Cleft (PLC) and Laryngotracheoesophageal Cleft (LTEC)
383.8 Vascular and Cardiac Anomalies 383.9 Tracheal Stenoses, Webs, and Atresia 383.1-0
Foregut Cysts 383.11 Tracheomalacia and Bronchomalacia 384 Foreign Bodies of the Airway
384.1 Laryngeal Foreign Bodies 384.2 Tracheal Foreign Bodies 384.3 Bronchial Foreign
Bodies 385 Laryngotracheal Stenosis, Subglottic Stenosis 385.1 Congenital Subglottic
Stenosis 385.2 Acquired Laryngotracheal Stenosis 386 Bronchomalacia and Tracheomalacia
387 Neoplasms of the Larynx, Trachea, and Bronchi 387.1 Vocal Nodules 387.2 Recurrent
Respiratory Papillomatosis (RRP) 387.3 Congenital Subglottic Hemangioma 387.4 Vascular
Anomalies 387.5 Other Laryngeal Neoplasms 387.6 Tracheal Neoplasms 387.7 Bronchial
Tumors 388 Wheezing, Bronchiolitis, and Bronchitis 388.1 Wheezing in Infants and
Bronchiolotis 388.2 Bronchitis 389 Emphysema and Overinflation 390 α1-Antitrypsin
Deficiency and Emphysema 391 Other Distal Airway Diseases 391.1 Bronciolitis Obliterans
391.2 Follicular Bronchitis 391.3 Pulmonary Alveolar Microlithiasis 392 Congenital Disorders
of the Lung 392.1 Pulmonary Agenesis and Aplasia 392.2 Pulmonary Hypoplasia 392.3
Cystic Adenomatoid Malformation 392.4 Pulmonary Sequestration 392.5 Bronchogenic Cysts
392.6 Congenital Pulmonary Lymphangiectasia 392.7 Lung Hernia 392.8 Other Congenital
Malformations of the Lung 393 Pulmonary Edema 394 Aspiration Syndromes 395 Chronic
Recurrent Aspiration 396 Parenchymal Disease with Prominent Hypersensitivity, Eosinophilic
Infiltration, or Toxin-Mediated Injury 396.1 Hypersensitivity to Inhaled Materials 396.2 Silo
Filler Disease 396.3 Paraquat Lung 396.4 Eosinophilic Lung Disease (Formerly Löffler
Syndrome) 397 Pneumonia 398 Bronchiectasis 399 Pulmonary Abscess 400 Cystic Fibrosis
401 Primary Ciliary Dyskinesia (Immotile Cilia Syndrome) 402 Interstitial Lung Diseases 403
Pulmonary Alveolar Proteinosis 404 Inherited Disorders of Surfactant Metabolism 405
Pulmonary Hemosiderosis 406 Pulmonary Hemorrhage, Embolism, and Infarction 406.1
Pulmonary Embolus and Infarction 406.2 Pulmonary Hemorrhage and Hemoptysis 407
Atelectasis 408 Pulmonary Tumors 409 Pleurisy 409.1 Dry or Plastic Pleurisy 409.2
Serofibrinous Pleurisy 409.3 Purulent Pleurisy or Empyema 410 Pneumothorax 411
Pneumomediastinum 412 Hydrothorax 413 Hemothorax 414 Chylothorax 415
Bronchopulmonary Dysplasia 416 Skeletal Diseases Influencing Pulmonary Function 416.1
Pectus Excavatum (Funnel Chest) 416.2 Pectus Carinatum and Sternal Clefts 416.3
Asphyxiating Thoracic Dystrophy (Thoracic-Pelvic-Phalangeal Dystrophy) 416.4
Achondroplasia 416.5 Kyphoscoliosis: Adolescent Idiopathic Scoliosis and Congential
Scoliosis 416.6 Congenital Rib Anomalies 417 Neuromuscular Diseases with Pulmonary
Consequences 418 Extrapulmonary Diseases with Pulmonary Manifestations 419 Chronic
Severe Respiratory Insufficiency
Part XIX: The Cardiovascular System
Section 1. Developmental Biology of the Cardiovascular System 420 Cardiac Development
420.1 Early Cardiac Morphogenesis 420.2 Cardiac Looping 420.3 Cardiac Septation 420.4
Aortic Arch Development 420.5 Cardiac Differentiation 420.6 Developmental Changes in
Cardiac Function 421 The Fetal to Neonatal Circulatory Transition 421.1 The Fetal Circulation
421.2 The Transitional Circulation 421.3 The Neonatal Circulation 421.4 Persistent
Pulmonary Hypertension of the Neonate (Persistence of Fetal Circulatory Pathways) 421.5
The Influence of Fetal Environmental Factors on Adult Cardiovascular and Metabolic Disease
Section 2. Evaluation of the Cardiovascular System 422 History and Physical Examination
423 Laboratory Evaluation 423.1 Radiologic Assessment 423.2 Electrocardiography 423.3
Hematologic Data 423.4 Echocardiography 423.5 Exercise Testing 423.6 MRI, MRA, CT, and
Radionuclide Studies 423.7 Diagnostic and Interventional Cardiac Catheterization Section 3.
Congenital Heart Diseases 424 Epidemiology of Congenital Heart Disease 425 Evaluation of
the Infant or Child with Congenital Heart Disease 426 Acyanotic Congenital Heart Disease:
The Left-to-Right Shunt Lesions 426.1 Atrial Septal Defect 426.2 Ostium Secundum Defect
426.3 Sinus Venosus Atrial Septal Defect 426.4 Partial Anomalous Pulmonary Venous Return
426.5 Atrioventricular Septal Defects (Ostium Primum and Atrioventricular Canal or
Endocardial Cushion Defects) 426.6 Ventricular Septal Defect 426.7 Supracristal Ventricular
Septal Defect with Aortic Insufficiency 426.8 Patent Ductus Arteriosus 426.9
Aorticopulmonary Window Defect 426.1-0 Coronary-Arteriovenous Fistula (Coronary-Cameral
Fistula) 426.11 Ruptured Sinus of Valsalva Aneurysm 427 Acyanotic Congenital Heart
Disease: The Obstructive Lesions 427.1 Pulmonary Valve Stenosis with Intact Ventricular
Septum 427.2 Infundibular Pulmonary Stenosis and Double-Chamber Right Ventricle 427.3
Pulmonary Stenosis in Combination with an Intracardiac Shunt 427.4 Peripheral Pulmonary
Stenosis 427.5 Aortic Stenosis 427.6 Coarctation of the Aorta 427.7 Coarctation with
Ventricular Septal Defect 427.8 Coarctation with Other Cardiac Anomalies and Interrupted
Aortic Arch 427.9 Congenital Mitral Stenosis 427.1-0 Pulmonary Venous Hypertension 428
Acyanotic Congenital Heart Disease: Regurgitant Lesions 428.1 Pulmonary Valvular
Insufficiency and Congenital Absence of the Pulmonary Valve 428.2 Congenital Mitral
Insufficiency 428.3 Mitral Valve Prolapse 428.4 Tricuspid Regurgitation 429 Cyanotic
Congenital Heart Disease: Evaluation of the Critically Ill Neonate with Cyanosis and
Respiratory Distress 430 Cyanotic Congenital Heart Lesions: Lesions Associated with
Decreased Pulmonary Blood Flow 430.1 Tetralogy of Fallot 430.2 Tetralogy of Fallot with
Pulmonary Atresia 430.3 Pulmonary Atresia with Intact Ventricular Septum 430.4 Tricuspid
Atresia 430.5 Double-Outlet Right Ventricle with Pulmonary Stenosis 430.6 Transposition of
the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis 430.7 Ebstein
Anomaly of the Tricuspid Valve 431 Cyanotic Congenital Heart Disease: Lesions Associated
with Increased Pulmonary Blood Flow 431.1 d-Transposition of the Great Arteries 431.2 dTransposition of the Great Arteries with Intact Ventricular Septum 431.3 Transposition of the
Great Arteries with Ventricular Septal Defect 431.4 l-Transposition of the Great Arteries
(Corrected Transposition) 431.5 Double-Outlet Right Ventricle Without Pulmonary Stenosis
431.6 Double-Outlet Right Ventricle with Transposition of the Great Arteries (Taussig-Bing
Anomaly) 431.7 Total Anomalous Pulmonary Venous Return 431.8 Truncus Arteriosus 431.9
Single Ventricle (Double-Inlet Ventricle, Univentricular Heart) 431.10 Hypoplastic Left Heart
Syndrome 431.11 Abnormal Positions of the Heart and the Heterotaxy Syndromes (Asplenia,
Polysplenia) 432 Other Congenital Heart and Vascular Malformations 432.1 Anomalies of the
Aortic Arch 432.2 Anomalous Origin of the Coronary Arteries 432.3 Pulmonary Arteriovenous
Fistula 432.4 Ectopia Cordis 432.5 Diverticulum of the Left Ventricle 433 Pulmonary
Hypertension 433.1 Primary Pulmonary Hypertension 433.2 Pulmonary Vascular Disease
(Eisenmenger Syndrome) 434 General Principles of Treatment of Congenital Heart Disease
434.1 The Adult with Congenital Heart Disease Section 4. Cardiac Arrhythmias 435
Disturbances of Rate and Rhythm of the Heart 435.1 Principles of Antiarrhythmic Therapy
435.2 Sinus Arrhythmias and Extrasystoles 435.3 Supraventricular Tachycardia 435.4
Ventricular Tachyarrhythmias 435.5 Long Q-T Syndrome 435.6 Sinus Node Dysfunction
435.7 AV Block 436 Sudden Death Section 5. Acquired Heart Disease 437 Infective
Endocarditis 438 Rheumatic Heart Disease Section 6. Diseases of the Myocardium and
Pericardium 439 Diseases of the Myocardium 439.1 Dilated Cardiomyopathy 439.2
Hypertrophic Cardiomyopathy 439.3 Restrictive Cardiomyopathies 439.4 Myocarditis 439.5
Nonviral Causes of Myocarditis 439.6 Endocardial Fibroelastosis 440 Diseases of the
Pericardium 440.1 Acute Pericarditis 440.2 Constrictive Pericarditis 441 Tumors of the Heart
Section 7. Cardiac Therapeutics (Heart Failure and End-Stage Cardiac Disease) 442 Heart
Failure 442.1 Cardiogenic Shock 443 Pediatric Heart and Heart-Lung Transplantation 443.1
Pediatric Heart Transplantation 443.2 Heart-Lung and Lung Transplantation Section 8.
Diseases of the Peripheral Vascular System 444 Disease of the Blood Vessels (Aneurysms
and Fistulas) 444.1 Kawasaki Disease (see also Ch 165) 444.2 Arteriovenous Fistulas 445
Systemic Hypertension
Part XX: Disease of the Blood
Section 1. The Hematopoietic System 446 Development of the Hematopoietic System 447
The Anemias Section 2. Anemias of Inadequate Production 448 Congenital Hypoplastic
Anemia (Diamond-Blackfan Anemia ) 449 Pearson Marrow-Pancreas Syndrome 450
Acquired Pure Red Blood Cell Anemias 451 Anemia of Chronic Disease (ACD) and Renal
Disease 451.1 Anemia of Chronic Disease 451.2 Anemia of Renal Disease 452 Congenital
Dyserythropoietic Anemias (CDA) 453 Physiologic Anemia of Infancy 454 Megaloblastic
Anemias 454.1 Folic Acid Deficiency 454.2 Vitamin B12 (Cobalamin) Deficiency 454.3 Other
Rare Megaloblastic Anemias 455 Iron-Deficiency Anemia 456 Other Microcytic Anemias
Section 3. Hemolytic Anemias 457 Definitions and Classification of Hemolytic Anemias 458
Hereditary Spherocytosis 459 Hereditary Elliptocytosis 460 Hereditary Stomatocytosis 461
Other Membrane Defects 462 Hemoglobinopathies 462.1 Sickle Cell Disease 462.2 Sickle
Cell Trait (Hemoglobin AS) 462.3 Other Hemoglobinopathies 462.4 Unstable Hemoglobin
Disorders 462.5 Abnormal Hemoglobins with Increased Oxygen Affinity 462.6 Abnormal
Hemoglobins Causing Cyanosis 462.7 Hereditary Methemoglobinemia 462.8 Syndromes of
Hereditary Persistence of Fetal Hemoglobin (HPFH) 462.9 Thalassemia Syndromes 463
Enzymatic Defects 463.1 Pyruvate Kinase (PK) Deficiency 463.2 Other Glycolytic Enzyme
Deficiencies 463.3 Glucose-6-Phosphate Dehydrogenase (G6PD) and Related Deficiencies
464 Hemolytic Anemias Resulting from Extracellular Factors 465 Hemolytic Anemias
Secondary to Other Extracellular Factors Section 4. Polycythemia (Erythrocytosis) 466
Primary Polycythemia (Polycythemia Rubra Vara) 467 Secondary Polycythemia Section 5.
The Pancytopenias 468 The Constitutional Pancytopenias 469 The Acquired Pancytopenias
Section 6. Risks of Blood Component Transfusions 470 Red Blood Cell Transfusions and
Erythropoietin Therapy 471 Platelet Transfusions 472 Neutrophil (Granulocyte) Transfusions
473 Fresh Frozen Plasma Transfusions 474 Risks of Blood Transfusions Section 7.
Hemorrhagic and Thrombotic Diseases 475 Hemostasis 475.1 The Clinical and Laboratory
Evaluation of Hemostasis 476 Hereditary Clotting Factor Deficiencies (Bleeding Disorders)
476.1 Factor VIII or Factor IX Deficiency (Hemophilia A or B) 476.2 Factor IX Deficiency
(Hemophilia C) 476.3 Deficiencies of the Contact Factors (Non-bleeding Disorders) 476.4
Factor VII Deficiency 476.5 Factor X Deficiency 476.6 Prothrombin (Factor II) Deficiency
476.7 Factor V Deficiency 476.8 Combined Deficiency of Factors V and VIII 476.9 Fibrinogen
Deficiency 476.10 Factor XIII Deficiency (Fibrin-Stabilizing Factor or Transglutaminase
Deficiency) 476.11 Antiplasmin or Plasminogen Activator Inhibitor (PAI) Deficiency 477 von
Willebrand Disease 478 Hereditary Predisposition to Thrombosis 479 Acquired Thrombotic
Disorders 480 Post-Neonatal Vitamin K Deficiency 481 Liver Disease 482 Acquired Inhibitors
of Coagulation 483 Disseminated Intravascular Coagulation 484 Platelet and Blood Vessel
Disorders 484.1 Idiopathic Thrombocytopenic Purpura (ITP) 484.2 Drug-Induced
Thrombocytopenia 484.3 Nonimmune Platelet Destruction 484.4 Hemolytic-Uremic Syndrome
(HUS) 484.5 Thrombotic Thrombocytopenic Purpura (TTP) 484.6 Kasabach-Merritt Syndrome
484.7 Sequestration 484.8 Congenital Thrombocytopenic Syndromes 484.9 Neonatal
Thrombocytopenia 484.10 Thrombocytopenia Due to Acquired Disorders Causing Decreased
Production 484.11 Platelet Function Disorders 484.12 Acquired Disorders of Platelet Function
484.13 Congenital Abnormalities of Platelet Function 484.14 Disorders of the Blood Vessels
Section 8. The Spleen 485 Anatomy and Function of the Spleen 486 Splenomegaly 487
Hyposplenism, Splenic Trauma, and Splenectomy Section 9. The Lymphatic System 488
Anatomy and Function of the Lymphatic System 489 Abnormalities of Lymphatic vessels 490
Lymphadenopathy
Part XXI: Cancer and Benign Tumors
491 Epidemiology of Childhood and Adolescent Cancer 492 Molecular and Cellular Biology of
Cancer 493 Principles of Diagnosis 494 Principles of Treatment 495 The Leukemias 495.1
Acute Lymphoblastic Leukemia 495.2 Acute Myelogenous Leukemia 495.3 Down Syndrome
and Acute Leukemia and Myeloproliferation 495.4 Chronic Myelogenous Leukemia 495.5
Juvenile Chronic Myelocytic Leukemia 495.6 Infant Leukemia 496 Lymphoma 496.1
Hodgkin’s Disease 496.2 Non-Hodgkin’s Lymphoma (NHL) 497 Brain Tumors in Childhood
498 Neuroblastoma 499 Neoplasms of the Kidney 500 Soft Tissue Sarcomas 501 Neoplasms
of Bone 501.1 Malignant Tumors of Bone 501.2 Benign Tumors and Tumor-like Processes of
Bone 502 Retinoblastoma 503 Gonadal and Germ Cell Neoplasms 504 Neoplasms of the
Liver 505 Benign Vascular Tumors 505.1 Hemangiomas 505.2 Lymphangiomas and Cystic
Hygromas 506 Rare Tumors 506.1 Thyroid Tumors 506.2 Melanoma 506.3 Nasopharyngeal
Carcinoma 506.4 Adenocarcinoma of the Colon and Rectum 506.5 Adrenocortical Carcinoma
506.6 Desmoplastic Small Round Cell Tumor 507 Histiocytosis Syndromes of Childhood
507.1 Class I Histiocytoses 507.2 Class II Histiocytoses: Hemophagocytic
Lymphohistiocytosis (HLH) 507.3 Class III Histiocytoses
Part XXII: Nephrology
Section 1. Glomerular Disease 508 Introduction to Glomerular Diseases 508.1 Anatomy of the
Glomerulus 508.2 Glomerular Filtration 508.3 Glomerular Diseases Section 2. Conditions
Particularly Associated with Hematuria 509 Clinical Evaluation of the Child with Hematuria
510 Isolated Glomerular Disease with Recurrent Gross Hematuria 510.1 IgA Nephropathy
(Berger Nephropathy) 510.2 Alport Syndrome 510.3 Thin Glomerular Basement Membrane
Disease 511 Glomerulonephritis Associated with Infections 511.1 Acute Poststreptococcal
Glomerulonephritis 511.2 Other Chronic Infections 512 Membranous Glomerulopathy
(Glomerulonephritis) 513 Membranoproliferative (Mesangiocapillary) Glomerulonephritis 514
Glomerulonephritis Associated With Systemic Lupus Erythematosus 515 Henoch-Schonlein
Purpura Nephritis 516 Rapidly Progressive (Crescentic) Glomerulonephritis 517 Goodpasture
Disease 518 Hemolytic-Uremic Syndrome 519 Upper Urinary Tract Causes of Hematuria
519.1 Interstitial Nephritis 519.2 Toxic Nephropathy 519.3 Cortical Necrosis 519.4
Pyelonephritis 519.5 Nephrocalcinosis 519.6 Vascular Abnormalities 519.7 Renal Vein
Thrombosis 519.8 Idiopathic Hypercalciuria 520 Hematologic Diseases Causing Hematuria
520.1 Sickle Cell Nephropathy 520.2 Coagulopathies and Thrombocytopenia 521 Anatomic
Abnormalities Associated with Hematuria 521.1 Congenital Anomalies 521.2 Autosomal
Recessive Polycystic Kidney Disease 521.3 Autosomal Dominant Polycystic Kidney Disease
521.4 Trauma 521.5 Renal Tumors 522 Lower Urinary Tract Causes of Hematuria 522.1
Infectious Causes of Cystitis and Urethritis 522.2 Hemorrhagic Cystitis 522.3 Heavy Exercise
522.4 Munchhausen by Proxy Section 3. Conditions Particularly Associated with Proteinuria
523 Introduction to the Child with Proteinuria 524 Transient Proteinuria 525 Orthostatic
(Postural) Proteinuria 526 Fixed Proteinuria 526.1 Glomerular Proteinuria 526.2 Tubular
Proteinuria 527 Nephrotic Syndrome 527.1 Idiopathic Nephrotic Syndrome 587.2 Secondary
Nephrotic Syndrome 527.3 Congenital Nephrotic Syndrome Section 4. Tubular Disorders 528
Tubular Function 529 Renal Tubular Acidosis 529.1 Proximal (Type II) Renal Tubular Acidosis
529.2 Distal (Type I) Renal Tubular Acidosis 529.3 Hyperkalemic (Type IV) Renal Tubular
Acidosis 529.4 Rickets Associated with Renal Tubular Acidosis 530 Nephrogenic Diabetes
Insipidus 531 Bartter/Gitelman Syndromes and Other Inherited Tubular Transport
Abnormalities 531.1 Bartter Syndrome 531.2 Gitelman Syndrome 531.3 Other Inherited
Tubular Transport Abnormalities 532 Tubulointerstitial Nephritis Section 5. Toxic
Nephropathies: Renal Failure 533 Toxic Nephropathy 534 Cortical Necrosis 535 Renal
Failure (Chap Head only; not chapter) 535.1 Acute Renal Failure 535.2 Chronic Kidney
Disease 535.3 End-Stage Renal Disease 536 Renal Transplantation
Part XXIII: Urologic Disorders in Infants and Children
537 Congenital Anomalies and Dysgenesis of the Kidneys 538 Urinary Tract Infections 539
Vesicoureteral Reflux 540 Obstruction of the Urinary Tract 541 Anomalies of the Bladder 542
Neuropathic Bladder 543 Voiding Dysfunction 544 Anomalies of the Penis and Urethra 545
Disorders and Anomalies of the Scrotal Contents 546 Trauma to the Genitourinary Tract 547
Urinary Lithiasis
Part XXIV: Gynecologic Problems of Childhood
548 History and Physical Examination 549 Vulvovaginitis 550 Bleeding 551 Breast Disorders
552 Hirsutism 553 Neoplasms 554 Vulvovaginal and Müllerian Anomalies 555 Special
Gynecologic Needs 556 Gynecologic Imaging
Part XXV: The Endocrine System
Section 1. Disorders of the Hypothalamus and Pituitary Gland 557 Hormones of the
Hypothalamus and Pituitary 558 Hypopituitarism 559 Diabetes Insipidus 559.1 Causes of
Hypernatremia 560 Other Abnormalities of Arginine Vasopressin Metabolism and Action 561
Hyperpituitarism 562 Physiology of Puberty 563 Disorders of Pubertal Development 563.1
Gonadotropin-Dependent Precocious Puberty 563.2 Precocious Puberty Resulting from
Organic Brain Lesions 563.3 Precocious Puberty Following Irradiation of the Brain 563.4
Syndrome of Precocious Puberty and Hypothyroidism 563.5 Gonadotropin-Secreting Tumors
563.6 McCune-Albright Syndrome (Precocious Puberty with Polyostotic Fibrous Dysplasia
and Abnormal Pigmentation) 563.7 Familial Male Gonadotropin-Independent Precocious
Puberty 563.8 Incomplete (Partial) Precocious Development 563.9 Medicational Precocity
Section 2. Disorders of the Thyroid Gland 564 Thyroid Development and Physiology 564.1
Thyroid Hormone Studies 565 Defects of Thyroxine-Binding Globulin 566 Hypothyroidism 567
Thyroiditis 568 Goiter 568.1 Congenital Goiter 568.2 Endemic Goiter and Cretinism 568.3
Acquired Goiter 568.4 Intratracheal Goiter 569 Hyperthyroidism 569.1 Graves Disease 569.2
Congenital Hyperthyroidism 570 Carcinoma of the Thyroid 570.1 Solitary Thyroid Nodule
570.2 Medullary Carcinoma Section 3. Disorders of the Parathyroid Glands 571 Hormones
and Peptides of Calcium Homeostasis and Bone Metabolism 572 Hypoparathyroidism 573
Pseudohypoparathyroidism (Albright Hereditary Osteodystrophy 574 Hyperparathyroidism
Section 4 Disorders of the Adrenal Glands 575 The Physiology of the Adrenal Gland 575.1
Histology and Embryology 575.2 Adrenal Steroid Biosynthesis 575.3 Regulation of the
Adrenal Cortex 575.4 Adrenal Steroid Hormone Actions 575.5 Adrenal Medulla 576
Adrenocortical Insufficiency 576.1 Primary Adrenal Insufficiency 576.2 Secondary Adrenal
Insufficiency 577 Congenital Adrenal Hyperplasia and Related Disorders 577.1 Congenital
Adrenal Hyperplasia due to 21-Hydroxylase Deficiency 577.2 Congenital Adrenal Hyperplasia
due to 11β-Hydroxylase Deficiency 577.3 Congenital Adrenal Hyperplasia due to 3βHydroxysteriod Dehydrogenase Deficiency 577.4 Congenital Adrenal Hyperplasia due to 17Hydroxylase Deficiency 577.5 Lipoid Adrenal Hyperplasia 577.6 Deficiency of P5450
oxioreductase (Antley-Bixler Syndrome) 577.7 Aldosterone Synthase Deficiency 577.8
Glucocorticoid-Suppressible Hyperaldosteronism 578 Cushing Syndrome 579 Primary
Aldosteronism 580 Adrenal Tumors 580.1 Virilizing Adrenocortical Tumors 580.2 Feminizing
Adrenal Tumors 581 Pheochromocytoma 582 Adrenal Masses 582.1 Adrenal Incidentaloma
582.2 Adrenal Calcification Section 5. Disorders of the Gonads 583 Development and
Function of the Gonads 584 Hypofunction of the Testes 584.1 Hypergonadotropic
Hypogonadism in the Male (Primary Hypogonadism) 584.2 Hypogonadotropic Hypogonadism
in the Male (Secondary Hypogonadism) 585 Pseudoprecocity Resulting from Tumors of the
Testes 586 Gynecomastia 587 Hypofunction of the Ovaries 587.1 Hypergonadotropic
Hypogonadism in the Female (Primary Hypogonadism) 587.2 Hypogonadotropic
Hypogonadism in the Female (Secondary Hypogonadism) 588 Pseudoprecocity Due to
Lesions of the Ovary 589 Disorders of Sex Development (Intersex) 589.1 46,XX DSD 589.2
46,XY DSD 589.3 Ovotesticular DSD Section 6. Diabetes Mellitus In Children 590 Diabetes
Mellitus 590.1 Introduction and Classification 590.2 Type 1 Diabetes Mellitus (Immune
Mediated) 590.3 Type 2 Diabetes Mellitus (T2DM) 590.4 Other Specific Types of Diabetes
Part XXVI: Neurology
591 Neurologic Evaluation 592 Congenital Anomalies of the Central Nervous System 592.1
Neural Tube Defects (Dysraphism) 592.2 Spina Bifida Occulta 592.3 Meningocele 592.4
Myelomeningocele 592.5 Encephalocele 592.6 Anencephaly 592.7 Disorders of Neuronal
Migration 592.8 Agenesis of the Corpus Callosum 592.9 Agenesis of the Cranial Nerves
592.10 Microcephaly 592.11 Hydrocephalus 592.12 Craniosynostosis 593 Seizures in
Childhood 593.1 Febrile Seizures 593.2 Unprovoked Seizures 593.3 Partial Seizures 593.4
Generalized Seizures 593.5 Mechanisms of Seizures 593.6 Treatment of Epilepsy 593.7
Neonatal Seizures 593.8 Status Epilepticus 594 Conditions that Mimic Seizures 595
Headaches 595.1 Migraine 595.2 Organic Headaches 595.3 Tension or Stress Headaches
596 Neurocutaneous Syndromes 596.1 Neurofibromatosis 596.2 Tuberous Sclerosis 596.3
Sturge-Weber Syndrome 596.4 Von Hippel-Lindau Disease 596.5 Linear Nevus Syndrome
596.6 PHACE Syndrome 597 Movement Disorders 597.1 Ataxias 597.2 Chorea, Athetosis,
Tremor 597.3 Dystonia 597.4 Tics 598 Encephalopathies 598.1 Cerebral Palsy 598.2
Mitochondrial Encephalomyopathies 598.3 Other Encephalopathies 599 Neurodegenerative
Disorders of Childhood 599.1 Sphingolipidoses 599.2 Neuronal Ceroid Lipofuscinoses 599.3
Adrenoleukodystrophy 599.4 Sialidosis 599.5 Miscellaneous Disorders 600 Demyelinating
Disorders of the CNS 600.1 Multiple Sclerosis (MS) 600.2 Acute Disseminated
Encephalomyelitis 601 Acute Stroke Syndromes 601.1 Arterial Thrombosis/Embolism 601.2
Venous Thrombosis 601.3 Intracranial Hemorrhage 601.4 Differential Diagnosis of StrokeLike Events 602 Central Nervous System Infections 602.1 Acute Bacterial Meningitis Beyond
the Neonatal Period 602.2 Viral Meningoencephalitis 602.3 Eosinophilic Meningitis 603 Brain
Abscess 604 Pseudotumor Cerebri 605 Spinal Cord Disorders 605.1 Tethered Cord 605.2
Diastematomyelia (Split Spinal Cord Malformation) 605.3 Syringomyelia 605.4 Spinal Cord
Tumors 605.5 Spinal Cord Trauma 605.6 Transverse Myelitis 605.7 Arteriovenous
Malformation
Part XXVII: Neuromuscular Disorders
606 Evaluation and Investigation 607 Developmental Disorders of Muscle 607.1 Myotubular
Myopathy 607.2 Congenital Muscle Fiber-Type Disproportion (CMFTD) 607.3 Nemaline Rod
Myopathy 607.4 Central Core Disease and Minicore Myopathy 607.5 Myofibrillar Myopathies
607.6 Brain Malformations and Muscle Development 607.7 Amyoplasia 607.8 Muscular
Dysgenesis (Proteus Syndrome Myopathy) 607.9 Benign Congenital Hypotonia 607.1`0
Arthrogryposis 608 Muscular Dystrophies 608.1 Duchenne and Becker Muscular Dystrophies
608.2 Emery-Dreifuss Muscular Dystrophy 608.3 Myotonic Muscular Dystrophy 608.4 LimbGirdle Muscular Dystrophy 608.5 Facioscapulohumeral Muscular Dystrophy 608.6 Congenital
Muscular Dystrophy 609 Endocrine and Toxic Myopathies 610 Metabolic Myopathies 610.1
Periodic Paralyses (Potassium-Related) 610.2 Malignant Hyperthermia 610.3 Glycogenoses
610.4 Mitochondrial Myopathies 610.5 Lipid Myopathies 610.6 Vitamin E Deficiency Myopathy
611 Disorders of Neuromuscular Transmission and of Motor Neurons) 611.1 Myasthenia
Gravis 611.2 Spinal Muscular Atrophies 611.3 Other Motor Neuron Disorders 612 Hereditary
Motor-Sensory Neuropathi 612.1 Peroneal Muscular Atrophy (Charcot-Marie-Tooth Disease,
HMSN Type I) 612.2 Peroneal Muscular Atrophy (Axonal Type) 612.3 Déjerine-Sottas
Disease (HMSN Type III) 612.4 Roussy-Lévy Syndrome 612.5 Refsum Disease 612.6 Fabry
Disease 612.7 Giant Axonal Neuropathy 612.8 Congenital Hypomyelinating Neuropathy
612.9 Tomaculous Neuropathy 612.10 Leukodystrophies 613 Toxic Neuropathies 614
Autonomic Neuropathies 614.1 Familial Dysautonomia 614.2 Other Autonomic Neuropathies
615 Guillain-Barré Syndrome 616 Bell Palsy
Part XXVIII: Disorders of the Eye
617 Growth and Development 618 Examination of the Eye 619 Abnormalities of Refraction
and Accommodation 620 Disorders of Vision 621 Abnormalities of Pupil and Iris 622
Disorders of Eye Movement and Alignment 623 Abnormalities of the Lids 624 Disorders of the
Lacrimal System 625 Disorders of the Conjunctiva 626 Abnormalities of the Cornea 627
Abnormalities of the Lens 628 Disorders of the Uveal Tract 629 Disorders of the Retina and
Vitreous 630 Abnormalities of the Optic Nerve 631 Childhood Glaucoma 632 Orbital
Abnormalities 633 Orbital Infections 633.1 Orbital Cellulitis 633.2 Dacroadenitis and
Dacrocystadenitis 634 Injuries to the Eye
Part XXIX: The Ear
635 The Ear 636 Hearing Loss 637 Congenital Malformations 638 External Otitis (Otitis
Externa) 639 Otitis Media 640 The Inner Ear and Diseases of the Bony Labyrinth 641
Traumatic Injuries of the Ear and Temporal Bone 642 Tumors of the Ear and Temporal Bone
Part XXX: The Skin
643 Morphology of the Skin 644 Evaluation of the Patient 644.1 Cutaneous Manifestations of
Systemic Diseases 645 Principles of Therapy 646 Diseases of the Neonate 647 Cutaneous
Defects 648 Ectodermal Dysplasias 649 Vascular Disorders 650 Cutaneous Nevi 651
Hyperpigmented Lesions 652 Hypopigmented Lesions 653 Vesiculobullous Disorders 654
Eczematous Disorders 655 Photosensitivity 656 Diseases of the Epidermis 657 Disorders of
Keratinization 658 Diseases of the Dermis 659 Diseases of Subcutaneous Tissue 660
Disorders of the Sweat Glands 661 Disorders of Hair 662 Disorders of the Nails 663
Disorders of the Mucous Membranes 664 Cutaneous Bacterial Infections 665 Cutaneous
Fungal Infections 666 Cutaneous Viral Infections 667 Arthropod Bites and Infestations 668
Acne 669 Tumors of the Skin 670 Nutritional Dermatoses
Part XXXI: Bone and Joint Disorders
Section 1. Orthopaedic Problems 671 Growth and Development 672 Evaluation of the Child
673 The Foot and Toes 673.1 Metatarsus Adductus 673.2 Calcaneovalgus Feet 673.3
Talipes Equinovarus (Clubfoot) 673.4 Congenital Vertical Talus 673.5 Hypermobile Pes
Planus (Flexible Flatfeet) 673.6 Tarsal Coalition 673.7 Cavus Feet 673.8 Osteochondroses
673.9 Puncture Wounds of the Foot 673.10 Toe Deformities 673.11 Painful Foot 673.12
Shoes 674 Torsional and Angular Deformities 674.1 Normal Developmental of Limb 674.2
Evaluation 674.3 Torsional Deformities 674.4 Coronal Plane Deformities 674.5 Congenital
Angular Deformities of the Tibia and Fibula 675 Leg Length Discrepancy 676 The Knee 676.1
Discoid Lateral Meniscus 676.2 Popliteal Cyst 676.3 Osteochondritis Dissecans 676.4
Osgood-Schlatter Disease 676.5 Idiopathic Adolescent Anterior Knee Pain Syndrome 676.6
Patellar Subluxation and Dislocation 677 The Hip 677.1 Developmental Dysplasia of the Hip
677.2 Transient Monoarticularsynovitis (Toxic Synovitis) 677.3 Legg-Calvé-Perthes Disease
677.4 Slipped Capital Femoral Epiphysis 678 The Spine 678.1 Idiopathic Scoliosis 678.2
Congenital Scoliosis 678.3 Neuromuscular Scoliosis, Genetic Syndromes, and Compensatory
Scoliosis 678.4 Kyphosis 678.5 Back Pain in Children 678.6 Spondylolysis and
Spondylolisthesis 678.7 Disk Space Infection 678.8 Intervertebral Disk Herniation/Slipped
Vertebral Apophysis 678.9 Tumors 679 The Neck 679.1 Torticollis 679.2 Klippel-Feil
Syndrome 679.3 Cervical Anomalies and Instabilities 680 The Upper Limb 681 Arthrogryposis
682 Common Fractures 682.1 Unique Characteristics of Pediatric Fractures 682.2 Pediatric
Fracture Patterns 682.3 Upper Extremity Fractures 682.4 Fractures of Lower Extremity 682.5
Operative Treatment 682.6 Complications of Fractures in Children 682.7 Outcomes
Assessment 683 Osteomyelitis (new title) 684 Supperative Arthritis (Septic Arthritis) Section
2. Sports Medicine 685 Epidemiology and Prevention 686 Management of Musculoskeletal
Injury 686.1 Growth Plate Injuries 686.2 Shoulder Injuries 686.3 Elbow Injuries 686.4 Low
Back Injuries 686.5 Hip and Pelvis Injuries 686.6 Knee Injuries 686.7 Lower Leg Pain: Shin
Splints and Stress Fractures and Chronic Compartment Syndrome 686.8 Ankle Injuries 686.9
Foot Injuries 687 Head and Neck Injuries 688 Heat Injuries 689 Female Athletes: Menstrual
Problems and Risk for Osteopenia 690 Ergogenic Aids 691 Specific Sports and Associated
Injuries Section 3. The Skeletal Dysplasias 692 General Considerations 693 Disorders of
Involving Cartilage Matrix Proteins 694 Disorders Involving Transmembrane Receptors 695
Disorders Involving Ion Transporter 696 Disorders Involving Transcription Factors 697
Disorders Involving Defective Bone Resorption 698 Disorders for Which Defects Are Poorly
Understood or Unknown 699 Osteogenesis Imperfecta 700 Marfan Syndrome Section 4.
Metabolic Bone Disease 701 Bone Structure, Growth, and Hormonal Regulation 702 Primary
Chondrodystrophy (Metaphyseal Dysplasia) 703 Hypophosphatasia 704 Hyperphosphatasia
705 Osteoporosis
Part XXXII: Environmental Health Hazards
706 Biologic Effects of Radiation on Children 707 Chemical Pollutants 708 Heavy Metal
Poisoning 709 Lead Poisoning 710 Nonbacterial Food Poisoning 710.1 Mushroom Poisoning
710.2 Solanine Poisoning 710.3 Seafood Poisoning 711 Biologic and Chemical Terrorism 712
Animal and Human Bites 712.1 Rat-Bite Fever 712.2 Monkeypox 713 Envenomations
PART XXXIII: Laboratory Medicine
714 Laboratory Testing in Infants and Children 715 Reference Ranges for Laboratory Tests
and Procedures 716 Medications