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 Vice-President 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
 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 Toxin-
Induced 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 d-Transposition 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 3β-Hydroxysteriod
Dehydrogenase Deficiency 577.4 Congenital Adrenal Hyperplasia due to 17-
Hydroxylase 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 Stroke-Like 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 Limb-Girdle 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