Nelson TEXTBOOK OF
Transcription
Nelson TEXTBOOK OF
Nelson T E X T B O O K OF PEDIATRICS Richard E. Behrman, MD Executive Chair Federation of Pediatric Organizations, Inc. Pediatric Education Steering Committee Palo Alto, California and Clinical Professor of Pediatrics Stanford University School of Medicine University of California, San Francisco, School of Medicine Robert M. Kliegman, MD Professor and Chair Department of Pediatrics Medical College of Wisconsin Pediatrician in Chief Pam and Les Muma Chair in Pediatrics Children's Hospital of Wisconsin Milwaukee, Wisconsin Hal B. Jenson, MD Professor and Chair, Department of Pediatrics Director, Center for Pediatric Research Eastern Virginia Medical School and Children's Hospital of The King's Daughters Senior Vice President for Academic Affairs Children's Hospital of The King's Daughters Norfolk, Virginia 17th Edition SAUNDERS An Imprint of Elsevier Science Contents «Part I The Field of Pediatrics Chapter 18 Psychiatric Considerations of Central Nervous System Injury 71 1 Chapter 1 Overview of Pediatrics Jorge H. Daruna, Marc A. Forman, and Neil W. Boris Chapter 19 Psychosomatic Illness 1 Chapter 2 Ethics in Pediatric Care Chapter 20 Vegetative Disorders 6 Robert M. Nelson and Norman Fast 20.1 Chapter 3 Cultural Issues in Pediatric Care JillE.Korbin Chapter 10 4 Child Health in the Developing World 14 20.5 20 Habit Disorders 80 Neil W. Boris and Richard Dalton Part II Growth and Development Chapter 22 Anxiety Disorders 23 Chapter 23 Mood Disorders Chapter 7 Overview and Assessment of Variability 23 Chapter 8 Fetal Growth and Development Chapter 9 The Newborn 30 Chapter 10 The First Year 31 Chapter 11 TheSecondYear 27 86 Neil W. Boris and Richard Dalton Chapter 25 Disruptive Behavioral Disorders Chapter 26 Sexual Behavior and Its Variations 26.1 26.2 53 58 Chapter 16 Developmental Assessment Part-Ill Psychologic Disorders 90 Richard Dalton 51 Chapter 15 Assessment of Growth 88 Neil W. Boris and Richard Dalton 44 Chapter 13 Middle Childhood 84 Neil W. Boris, Richard Dalton, and Marc A. Forman 23.1 Major Depression 84 23.2 Dysthymic Disorder 85 23.3 Bipolar Disorder 85 Chapter 24 Suicide and Attempted Suicide 38 Chapter 12 PreschoolYears 81 Brian Stafford, Neil W. Boris, and Richard Dalton Robert D. Needlman 62 67 Chapter 17 Assessment and Interviewing 17.2 73 Rumination Disorder 73 Neil W. Boris and Richard Dalton Pica 73 Neil W. Boris and Richard Dalton Enuresis (Bed-wetting) 74 Neil W. Boris and Richard Dalton Encopresis 75 Neil W. Boris and Richard Dalton Sleep Disorders 75 Judith A. Owens Chapter 21 Paul L. McCarthy 17.1 20.3 20.4 Chapter 5 Preventive Pediatrics Theodore C. Sectish Chapter 14 Adolescence 20.2 12 Kristine Torjesen and Karen Olness Chapter 6 The Well Child 72 Alessandra N. Kazura, Neil W. Boris, and Richard Dalton Richard E. Behrman 67 The Clinical Interview (History) 67 Neil W.'Boris, Marc A. Forman, and Jorge H. Daruna Psychosocial Problems 70 Richard Dalton and Marc A. Forman Gender Identity Disorder Homosexuality 91 91 Chapter 27 Pervasive Developmental Disorders and Childhood Psychosis 93 Richard Dalton, Marc A. Forman, and Neil W. Boris 27.1 Autistic Disorder 93 27.2 Asperger Disorder 94 27.3 Childhood Disintegrative Disorder 94 27.4 Rett Disorder 94 27.5 Childhood Schizophrenia 94 Chapter 28 Psychologic Treatment of Children and Adolescents 95 Richard Dalton xxiii XXIV • 28.1 28.2 28.3 28.4 Contents Illness and Death 95 Psychopharmacology 96 Psychotherapy 100 Psychiatric Hospitalization Part VI Nutrition 100 153 Chapter 40 Nutritional Requirements William C. Heird 153 Chapter 29 Neurodevelopmental Dysfunction in the School-Aged Child 100 Chapter 41 The Feeding of Infants and Children 157 29.1 Patterns of Development and Function 100 Desmond P. Kelly William C. Heird 29.2 Attention-Defidt/Hyperactivity Disorder 107 Chapter 42 Food Insecurity, Hunger, and Mark D. Simms Undernutrition 167 29.3 Specific Reading Disability (Dyslexia) 110 William C. Heird G. ReidLyon, Sally E. Shaywitz, and Bennett A. Shaywitz Chapter 43 Obesity Patricia A. Donohoue Part IV Social Issues Chapter 30 Adoption 113 173 Chapter 44 Vitamin Deficiencies and Excesses William C. Heird 113 177 Mark D. Simms andMadelyn Freundlich Chapter 31 Foster Care 114 Mark D. Simms and Madelyn Freundlich Chapter 32 Child Care . Part VII Pathophysiology of Body Fluids and ; Fluid Therapy 191 115 Paul H. Dworkin Larry A. Greenbaum Chapter 33 Separation, Loss, and Bereavement 116 33.1 Separation and Loss 116 Richard Dalton 33.2 Grief and Bereavement 117 Linda Sayler Gudas and Gerald P. Koocher Chapter 34 Impact of Violence on Children 120 Marilyn Augustyn and Barry Zuckerman Chapter 35 Abuse and Neglect of Children Charles F. Johnson 35.1 Sexual Abuse 126 35.2 Nonorganic Failure to Thrive 129 35.3 Munchausen Syndrome by Proxy (MSBP) 121 193 Chapter 46 Maintenance and Replacement Therapy 242 130 Part V Children with Special Health Needs 133 Chapter 36 Failure toThrive Chapter 45 Electrolyte and Acid-Base Disorders 191 45.1 Composition of Body Fluids 191 45.2 Regulation of Osmolality and Volume 45.3 Sodium 196 45.4 Potassium 202 45.5 Calcium 208 45.6 Magnesium 217 45.7 Phosphorus 219 45.8 Acid-Base Balance 223 133 Chapter 47 DeficitTherapy 245 Chapter 48 Fluids and Electrolyte Treatment of Specific Disorders 249 48.1 Acute Diarrhea and Oral Rehydration 249 48.2 Diarrhea in Chronically Malnourished Children 48.3 Pyloric Stenosis 251 48.4 Perioperative Fluids 251 250 Howard Bauchner Chapter 37 Developmental Disabilities and Chronic Illness 134 James M. Perrin 37.1 Chronic Illness in Childhood 135 James M. Perrin 37.2 Mental Retardation 138 Bruce K. Shapiro and Mark L. Batshaw Chapter 38 Pediatric Palliative Care:The Care of Children with Life-Limiting Illness 143 Stephen Liben Chapter 39 Children at Special Risk Richard E. Behrman 148 PartVili The Acutely III Child 253 Chapter 49 Evaluation of the Sick Child in the Office and Clinic 253 Paul L. McCarthy Chapter 50 Injury Control 256 Frederick P. Rivara and David Grossman Chapter 51 Emergency Medical Services for Children 263 M. Denise Dowd and Frederick P. Rivara Contents Chapter 52 Pediatric Critical Care: An Overview 268 Chapter 66 Pediatric Pain Management XXV 358 Brenda Bursch and Lonnie K. Zeltzer Lorry R. Frankel Chapter 53 InterfacilityTransfer of the Critically III Infant and Child 269 Lorry R. Frankel Chapter 54 Effective Communication with Families inthePICU 270 Lawrence H. Mathers and Lorry R. Frankel Chapter 55 MonitoringTechniques for the Critically III Infant and Child 272 Lorry R. Frankel Joseph V. DiCarlo and Lorry R. Frankel Chapter 68 Molecular Diagnosis of Genetic Diseases 371 H. Eugene Hoyme Lorry R. Frankel and Joseph V. DiCarlo Chapter 59 Continuous Hemofiltration 317 Joseph V. DiCarlo Chapter 60 Transplantation Issues in thePICU 318 Lawrence H. Mathers and Lorry R. Frankel Drowning and Near-Drowning 321 Harry J. Kallas 330 Alia Y. Antoon and Mary K. Donovan 338 Alia Y. Antoon and Mary K. Donovan Chapter 64 Withdrawal or Withholding of Life Support, Brain Death, and Organ Procurement 340 Lorry R. Frankel and Lawrence H. Mathers Chapter 65 Anesthesia and Perioperative Care 342 376 H. Eugene Hoyme Chapter 70 Chromosomal Clinical Abnormalities GeneTherapy 391 Mark A. Kay Chapter 58 Acute (Adult) Respiratory Distress Syndrome (ARDS) 316 Randall C. Wetzel H. Eugene Hoyme Chapter 71 57.1 Pediatric Emergencies and Resuscitation 279 Lawrence H. Mathers and Lorry R. Frankel 57.2 Shock 296 Lorry R. Frankel and Lawrence H.Mathers 57.3 Respiratory Distress and Failure 301 Lorry R. Frankel 57.4 Mechanical Ventilation 303 Lorry R. Frankel 57.5 Renal Stabilization 306 Joseph V. DiCarlo 57.6 Nutritional Stabilization 307 Joseph V. DiCarlo 57.7 Neurologic Stabilization 308 Joseph V. DiCarlo and Lorry R. Frankel , 57.8 Acute Care of the Multiple Trauma Victim 312 Peter S. Dayan and Bruce L. Klein Chapter 63 Cold Injuries Chapter 67 The Molecular Basis of Genetic Disorders 367 Judith G. Hall Chapter 57 Stabilization of the Critically III Child 279 Chapter 62 Burn Injuries 367 Chapter 69 Patterns of Inheritance Chapter 56 Scoring Systems and Predictors of Mortality 277 Chapter 61 Part IX Human Genetics Chapter 72 Genetic Counseling 395 Judith G. Hall PartX Metabolic Diseases 397 Chapter 73 An Approach to Inborn Errors of Metabolism 397 Iraj Rezvani Chapter 74 Defects in Metabolism of Amino Acids 398 74.1 Phenylalanine 398 Iraj Rezvani 74.2 Tyrosine 402 Iraj Rezvani „ 74.3 Methionine 405 Iraj Rezvani and David S. Rosenblatt 74.4 Cysteine/Cystine 407 Iraj Rezvani 74.5 Tryptophan 408 Iraj Rezvani 74.6 Valine, Leucine, Isoleucine, and Related Organic Acidemias 409 Iraj Rezvani and David S. Rosenblatt 74.7 Glycine 418 Iraj Rezvani 74.8 Serine 421 Iraj Rezvani 74.9 Proline and Hydroxyproline 421 Iraj Rezvani 74.10 GlutamicAdd 422 Iraj Rezvani 74.11 Urea Cycle and Hyperammonemia (Arginine, Citrulline, Omithine) 424 Iraj Rezvani 74.12 Histidine 429 Iraj Rezvani 74.13 Lysine 429 Iraj Rezvani 74.14 Aspartic Acid (Canavan Disease) 430 Reuben K. Matalon 382 XXVi Contents Chapter 75 Defects in Metabolism of Lipids 75.1 Disorders of Mitochondrial Fatty Acid Oxidation 433 433 Chapter 85 The Fetus 437 75.3 Disorders of Lipoprotein Metabolism and Transport 445 Andrew M. Tershakovec and Daniel J. Rader 459 Margaret M. McGovern and Robert J. Desnick 75.5 Mucolipidoses 467 Margaret M. McGovern and Robert J. Desnick Chapter 76 Defects in Metabolism of Carbohydrates 468 76.1 Glycogen Storage Diseases 469 Chapter 86 The High-Risk Infant Yuan-Tsong Chen 76.2 Defects in Galactose Metabolism 475 Yuan-Tsong Chen 76.3 Defects in Fructose Metabolism 536 Barbara J. Stoll and Robert M. Kliegman 85.1 Fetal Growth and Maturity 536 85.2 Fetal Distress 536 85.3 Maternal Disease and the Fetus 539 85.4 Maternal Medication and Toxin Exposure and the Fetus 540 85.5 Teratogens 542 85.6 Radiation 542 85.7 Intrauterine Diagnosis of Fetal Disease 542 85.8 Treatment and Prevention of Fetal Disease 545 Hugo W. Moser 75.4 Lipidoses 532 Barbara J. Stoll and Robert M. Kliegman Charles P. Venditti and Charles A. Stanley 75.2 Disorders of Very Long Chain Fatty Acids Chapter 84 High-Risk Pregnancies 476 Yuan-Tsong Chen Id A Defects in Intermediary Carbohydrate Metabolism Associated with Lactic Acidosis 477 Yuan-Tsong Chen 76.5 Deficiency of Xylulose Dehydrogenase (Essential Benign Pentosuria) 480 Yuan-Tsong Chen 76.6 Disorders of Glycoprotein Degradation and Structure 480 547 Barbara J. Stoll and Robert M. Kliegman 86.1 Multiple Pregnancies 548 86.2 Prematurity and Intrauterine Growth Retardation 550 86.3 Post-Term Infants 558 86.4 Large for Gestational Age 558 86.5 Infant Transport 559 Chapter 87 Clinical Manifestations of Diseases in the Newborn Period 559 Barbara J. Stoll and Robert M. Kliegman Chapter 78 Disorders of Purine and Pyrimidine Metabolism 486 James C. Harris Chapter 88 Nervous System Disorders 561 Barbara J. Stoll and Robert M. Kliegman 88.1 The Cranium 562 88.2 Intracranial-Intraventricular Hemorrhage and Periventricular Leukomalacia 562 88.3 Spine and Spinal Cord 564 88.4 Peripheral Nerve Injuries 565 88.5 Hypoxia-Ischemia 566 88.6 Intrauterine Infection 568 Chapter 79 Chapter 89 Delivery Room Emergencies Margaret M. McGovern and Robert J. Desnick Chapter 77 Mucopolysaccharidoses 482 Robert M. Kliegman and Joseph L. Muenzer Progeria 494 W. Ted Brown Chapter 80 The Porphyrias 495 Shigeru Sassa Chapter 81 Hypoglycemia 569 Barbara J. Stoll and Robert M. Kliegman 505 Mark A. Sperling Part XI The Fetus and the Neonatal Infant 519 Section 1: Noninfectious Disorders 519 Chapter 82 Overview of Mortality and Morbidity 519 Barbara J. Stoll and Robert M. Kliegman Chapter 83 The Newborn Infant 523 Barbara J. Stoll and Robert M. Kliegman 83.1 History in Neonatal Pediatrics 523 83.2 Physical Examination of the Newborn Infant 83.3 Routine Delivery Room Care 527 83.4 Nursery Care 529 83.5 Parent-Infant Bonding 529 573 Chapter 90 Respiratory Tract Disorders Barbara J. Stoll and Robert M. Kliegman 90.1 Transition to Pulmonary Respiration 573 90.2 Apnea 573 90.3 Hyaline Membrane Disease (Respiratory Distress Syndrome) 575 90.4 Transient Tachypnea of the Newborn 583 90.5 Aspiration of Foreign Material (Fetal Aspiration Syndrome, Aspiration Pneumonia) 583 90.6 Meconium Aspiration 583 90.7 Persistent Pulmonary Hypertension of the Newborn (Persistent Fetal Circulation) 584 90.8 Extrapulmonary Extravasation of Air (Pneumothorax, Pneumomediastinum, Pulmonary Interstitial Emphysema) 586 90.9 Pulmonary Hemorrhage 587 Chapter 91 Digestive System Disorders 524 Barbara J. Stoll and Robert M. Kliegman 91.1 Meconium Ileus in Cystic Fibrosis 589 91.2 Neonatal Necrotizing Enterocolitis (NEC) 590 588 Contents 91.3 Jaundice and Hyperbilirubinemia in the Newborn ,592 91.4 Kernicterus 596 Chapter 92 Blood Disorders 599 Barbara J. Stoll and Robert M. Kliegman 92.1 Anemia in the Newborn Infant 599 92.2 Hemolytic Disease of theNewborn (Erythroblastosis Fetalis) 601 92.3 Plethora in the Newborn Infant (Polycythemia) 92.4 Hemorrhage in the Newborn Infant 606 Chapter 93 Genitourinary System Barbara J. Stoll and Robert M. Kliegman 608 Chapter 94 The Umbilicus 608 Barbara J. Stoll and Robert M. Kliegman Chapter 95 Metabolic Disturbances Barbara J. Stoll and Robert M. Kliegman Chapter 96 The Endocrine System 609 613 Barbara J. Stoll and Robert M. Kliegman 96.1 Infants of Diabetic Mothers 613 96.2 Hypoglycemia 614 Chapter 97 Dysmorphology Kenneth Lyons Jones 616 Section 2: Infections of the Neonatal Infant 623 Barbara J. Stoll Chapter 98 Pathogenesis and Epidemiology 623 98.1 Modes of Transmission and Pathogenesis 623 98.2 Immunity 625 98.3 Etiology of Fetal and Neonatal Infection 626 98.4 Epidemiology of Early- and Late-Onset Neonatal Infections 627 98.5 Clinical Manifestations of Transplacental Intrauterine Infections 630 98.6 Diagnosis 633 98.7 Treatment 636 98.8 Complications and Prognosis 638 98.9 Prevention 638 Chapter 102 Suicide Renee R. Jenkins XXVII 648 Chapter 103 Violent Behavior 649 Renee R. Jenkins Chapter 104 Anorexia Nervosa and Bulimia 652 IrisF.Litt 606 Chapter 105 Substance Abuse 653 Renee R. Jenkins 105.1 105.2 105.3 105.4 105.5 105.6 105.7 105.8 105.9 Alcohol 657 Marijuana 657 Tobacco 658 Volatile Substances 658 Hallucinogens 659 Cocaine 660 Amphetamines 660 Opiates 660 Anabolic Steroids 661 Chapter 106 The Breast 662 Renee R. Jenkins Chapter 107 Menstrual Problems 663 Renee R. Jenkins 107.1 Amenorrhea 664 107.2 Abnormal Uterine Bleeding 665 107.3 Dysmenorrhea 666 107.4 Premenstrual Syndrome 667 Chapter 108 Contraception 667 Renee R. Jenkins 108.1 Barrier Methods 668 108.2 Spermicides 668 108.3 Combination Methods 668 108.4 Hormonal Methods 668 108.5 Emergency Contraception 671 108.6 Intrauterine Devices 671 Chapter 109 Pregnancy 671 Renee R. Jenkins Chapter 110 SexuallyTransmitted Diseases 673 Renee R. Jenkins Special Health Problems During Adolescence 64i Chapter 99 The Epidemiology of Adolescent Health Problems 641 Renee R. Jenkins Chapter 100 Delivery of Health Care to Adolescents 643 Chapter 101 Depression Renee R. Jenkins 677 PartXIII The Immunologic System and Disorders 681 Section 1: Evaluation of the Immune System 681 Rebecca H. Buckley Renee R. Jenkins 100.1 Legal Issues 644 100.2 Screening Procedures 100.3 Health Enhancement Chapter 111 Chronic Fatigue Syndrome Hal B. Jenson _ , 645 646 646 Chapter 112 Evaluation of Suspected Immunodeficiency 681 Section 2: The T-, B-, and NK-Cell Systems 683 Rebecca H. Buckley XXViii Contents Chapter 113 T Lymphocytes, B Lymphocytes, and Natural Killer Cells 683 Part XIV Allergic Disorders 743 Chapter 114 Primary Defects of Antibody Production 689 114.1 Treatment of B-Cell Defects 693 Chapter 130 Allergy and the Immunologic Basis of Atopic Disease 743 Chapter 115 Primary Defects of Cellular Immunity 693 Chapter 131 Chapter 116 Primary Combined Antibody and Cellular Immunodeficiencies 695 116.1 Severe Combined Immunodeficiency (SCID) 116.2 Combined Immunodeficiency (CID) 697 116.3 Treatment of Cellular or Combined Immunodeficiency 700 Chapter 132 Principles of Treatment of Allergic Disease 752 Section 3: The Phagocytic System Chapter 117 Neutrophils Donald Y. M. Leung 695 Chapter 134 Childhood Asthma 701 Chapter 135 Atopic Dermatitis (Atopic Eczema) 774 707 Donald Y. M. Leung Disorders of Phagocyte Function 710 Leukocytosis Chapter 137 Anaphylaxis 781 Hugh A. Sampson and Donald Y. M. Leung 717 Chapter 138 Serum Sickness Laurence A. Boxer Chapter 122 Donald Y. M. Leung Chapter 136 Urticaria and Angioedema (Hives) 778 709 Laurence A. Boxer Leukopenia 760 Andrew H. Liu, Joseph D. Spahn, and Donald Y. M. Leung Laurence A. Boxer Chapter 121 759 Henry Milgrom and Donald Y. M. Leung 701 Chapter 118 Monocytesand Macrophages Richard B. Johnston, Jr. Chapter 120 747 Dan Atkins and Donald Y. M. Leung Chapter 133 Allergic Rhinitis Laurence A. Boxer Chapter 119 Eosinophils Diagnosis of Allergic Disease Dan Atkins and Donald Y. M. Leung 782 Scott H. Sicherer and Donald Y. M. Leung 723 Chapter 139 Adverse Reactions to Drugs Laurence A. Boxer Section 4: The Complement System 783 Mark Boguniewicz 725 Chapter 140 Insect Allergy 786 Scott H. Sicherer and Donald Y. M. Leung Richard B. Johnston, Jr. Chapter 141 Ocular Allergies Chapter 123 The Complement System 725 Chapter 124 Disorders of the Complement System 728 124.1 Evaluation 728 124.2 Primary Deficiencies of Complement Components 728 124.3 Deficiencies of Plasma, Membrane, or Serosal Complement Control Proteins 730 124.4 Secondary Disorders of Complement 730 124.5 Treatment of Complement Disorders 731 788 Mark Boguniewicz Chapter 142 Adverse Reactions to Foods 789 Hugh A. Sampson and Donald Y. M. Leung Rheumatic Diseases of Childhood (Connective Tissue Diseases, Collagen Vascular Diseases) 793 Section 5: Hematopoietic Stem Cell Transplantation 732 Chapter 143 Evaluation of Suspected Rheumatic Disease 793 Kent A. Robertson Chapter 125 Clinical Indications . Michael L. Miller 732 Chapter 126 Matching and Rejection Chapter 144 Treatment of Rheumatic Diseases 795 737 Daniel J. Lovell, Michael L. Miller, and James T. Cassidy Chapter 127 Graft versus Host Disease (GVHD) Chapter 128 Principles of Immunosuppression 740 Chapter 129 Late Effects of Stem Cell Transplantation 741 738 Chapter 145 Juvenile Rheumatoid Arthritis 799 Michael L. Miller and James T. Cassidy Chapter 146 Ankylosing Spondylitis and Other Spondyloarthropathies 805 Michael L. Miller and Ross E. Petty Contents Chapter 147 Postinfectious Arthritis and Related Conditions 808 Michael L. Miller and James T. Cassidy Chapter 164 Infections in Immunocompromised Persons 851 Chapter 148 Systemic Lupus Erythematosus Chapter 165 Infection Associated with Medical Devices 858 809 Marisa S. Klein-Gitelman and Michael L. Miller 148.1 Neonatal Lupus , 813 813 Lauren M. Pachman Section 2: Gram-Positive Bacterial Infections Chapter 166 Staphylococcus 816 819 Chapter 152 Sjogren Syndrome 820 Abraham Gedalia Chapter 153 Familial Mediterranean Fever 821 Abraham Gedalia Michael A. Gerber 168.1 Rheumatic Fever 822 Abraham Gedalia Chapter 155 Sarcoidosis 879 Catherine S. Lachenauer and Michael R. Wessels Margaret W. Leigh Chapter 156 Kawasaki Disease 870 874 Chapter 169 Group B Streptococcus 822 866 Chapter 167 Streptococcus pneumoniae (Pneumococcus) 867 Jon S. Abramson and Gary D. Overturf Chapter 168 Group A Streptococcus Chapter 154 Amyloidosis 861 861 James K. Todd 166.1 Staphylococcus aureus 861 166.2 Toxic Shock Syndrome 865 166.3 Coagulase-Negative Staphylococci Michael L. Miller Chapter 151 Behqet Disease Abraham Gedalia Marian G. Michaels and Michael Green Patricia M. Flynn and Fred F. Barrett Chapter 149 Juvenile Dermatomyositis Chapter 150 Scleroderma xxix Chapter 170 Non-GroupAor B Streptococci 883 823 Anne H. Rowley and Stanford T. Shulman Michael A. Gerber Chapter 157 Vasculitis Syndromes ~ 826 Michael L. Miller and Lauren M. Pachman 157.1 Henoch-SchonleinPurpura 826 157.2 Takayasu Arteritis 828 157.3 Polyarteritis Nodosa 829 157.4 Wegener Granulomatosis 830 157.5 Other Vasculitic Syndromes 831 Chapter 171 884 Enterococcus David B. Haslam Chapter 172 Diphtheria (Corynebacterium diphtheriae) 886 Sarah S. Long Chapter 173 Listeria monocytogenes Chapter 158 Musculoskeletal Pain Syndromes 831 Michael L. Miller 890 Robert S. Baltimore Chapter 174 Actinomyces 158.1 Reflex Sympathetic Dystrophy 158.2 Erythromelalgia 832 892 Richard F. Jacobs and Gordon E. Schutze 832 Chapter 175 Nocardia Chapter 159 Miscellaneous Conditions Associated with Arthritis 832 Michael L. Miller 894 Richard F. Jacobs and Gordon E. Schutze Section 3: Gram-Negative Bacterial Infections 896 Chapter 176 Neisseria meningitidis (Meningococcus) 896 Charles R. Woods Infectious Diseases Section 1: General Considerations 835 835 Chapter 160 Diagnostic Microbiology Toni Darville 835 Anita K. M. Zaidi and Donald A. Goldmann Chapter 161 Fever 839 Chapter 179 Chancroid (Haemophilus ducreyi) Chapter 162 FeverWithout a Focus Keith R. Powell Anne Stormorken and Keith R. Powell Chapter 178 Haemophilus influenzae 846 904 Robert S. Daum Keith R.Powell Chapter 163 Sepsis and Shock Chapter 177 Neisseria gonorrhoeae (Gonococcus) 899 841 908 Parvin Azimi Chapter 180 Pertussis [Bordetella pertussis and ; B. parapertussis) 908 Sarah S. Long <* XXX Contents Chapter 181 Salmonella 912 Chapter 196 Other Anaerobic Infections 181.1 181.2 Nontyphoidal Salmonellosis Enteric Fever 916 Chapter 182 Shigella 954 Margaret C. Fisher Thomas G. Cleary 912 Section 5: Mycobacterial Infections Chapter 197 Tuberculosis tuberculosis) 958 919 Thomas G. Cleary 958 (Mycobacterium Flor M. Munoz and Jeffrey R. Starke Chapter 183 Escherichia coli 921 Chapter 198 Hansen Disease leprae) 972 Dwight A.Powell Theresa J. Ochoa and Thomas G. Cleary Chapter 184 Cholera (Vibrio cholerae) 924 Gloria P. Heresi and James R. Murphy Chapter 199 Nontuberculous Mycobacteria Chapter 185 Campylobacter 926 Gloria P. Heresi and James R. Murphy Chapter 186 Yersinia Section 6: Spirochetal Infections 929 932 933 188.1 Pseudomonas aeruginosa 188.2 Burkholderia 936 188.3 Stenotrophomonas 936 934 985 990 Dwight A. Powell Chapter 205 Mycoplasma 943 Chapter 206 Genital Mycoplasmas (Mycoplasma hominis and Ureaplasma urealyticum) 992 947 (Clostridium Section 8: Chlamydial Infections 994 Margaret R. Hammerschlag Chapter 207 Chlamydia pneumoniae 994 Chapter 208 Chlamydia 995 208.1 208.2 208.3 208.4 trachomatis Trachoma 995 Genital Tract Infections 996 Conjunctivitis and Pneumonia in Newborns Lymphogranuloma Venereum (LGV) 998 Chapter 209 Psittacosis (Chlamydia psittaci) Robert Schechter and Stephen S. Arnon Chapter 194 Tetanus (Clostridium pneumoniae 990 941 Section 4: Anaerobic Bacterial Infections Section 9: Rickettsial Infections tetani) 951 Stephen S. Arnon Margaret C. Fisher 983 Section 7: Mycoplasmal Infections 939 Barbara W. Stechenberg 192.1 Bartonellosis {Bartonella bacilliformis) 943 192.2 Cat-Scratch Disease (Bartonella henselae) 944 192.3 Trench Fever (Bartonella quintana) 946 192.4 Bacillary Angiomatosis and Bacillary Peliosis Hepatis (Bartonella henselae and Bartonella quintana) 946 Chapter 195 Clostridium Diarrhea 953 Chapter 202 Leptospira Chapter 204 Lyme Disease (Borrelia burgdorferi) 986 Eugene D. Shapiro Lucy Tompkins Chapter 193 Botulism botulinum) 947 Parvin Azimi 201.1 Yaws (Treponemapertenue) 982 201.2 Bejel (Endemic Syphilis) (Treponemapallidum subsp. endemicum) 983 201.3 Pinta (Treponema carateum) 983 Parvin Azimi Gordon E. Schutze and Richard F. Jacobs Bartonella NonvenerealTreponemal 982 Chapter 203 Relapsing Fever (Borrelia) Chapter 189 Tularemia {Francisella tularensis) 937 Gordon E. Schutze and Richard F. Jacobs Chapter 192 Chapter 201 Infections Parvin Azimi Robert S. Baltimore Legionella 978 Parvin Azimi Chapter 188 Pseudomonas, Burkholderia, and Stenotrophomonas 933 Chapter 191 978 Chapter 200 Syphilis (Treponemapallidum) Chapter 187 Aeromonas and Plesiomonas James R. Murphy and Gloria P. Heresi Chapter 190 Brucella 975 Dwight A. Powell James R. Murphy and Gloria P. Heresi 186.1 Yersinia enterocolitica 929 186.2 Yersinia pseudotuberculosis 930 186.3 Plague (Yersiniapestis) 930 187.1 Aeromonas 932 187.2 Plesiomonas shigelloides (Mycobacterium difficile-Assodated 999 J. Stephen Dumler Chapter 210 Spotted Fever Group Rickettsioses 999 210.1 Rocky Mountain Spotted Fever (Rickettsia rickettsii) 999 997 998 Contents 210.2 Mediterranean Spotted Fever or Boutonneuse Fever (Rickettsia conorii) 1003 210.3 Rickettsialpox (Rickettsia akari) 1003 Chapter 211 ScrubTyphus (Orientia tsutsugamushf) 1004 Ehrlichiosisand Anaplasmosis Chapter 214 Q Fever (Coxiella burnetii) 1006 1009 Chapter 215 Candida 1011 Martin E. Weisse and Stephen C. Aronoff 215.1 Neonatal Infections 1012 215.2 Infections in Immunocompetent Children and Adolescents 1013 215.3 Infections in Immunocompromised Children and Adolescents 1013 215.4 Chronic Mucocutaneous Candidiasis 1014 Cryptococcus 1014 neoformans Malassezia 1016 Chapter 230 Parvovirus B19 William C. Koch 1048 Chapter 231 Herpes SimplexVirus Steve Kohl 1051 Chapter 233 Epstein-BarrVirus 1062 Hal B. Jenson Chapter 234 Cytomegalovirus 1066 Sergio Stagno Chapter 235 Roseola (Human Herpesviruses 6 and 7) 1069 Charles T. Leach 1072 Influenza Viruses 1072 Peter Wright 1016 Chapter 218 Aspergillus Stephen C. Aronoff 218.1 Hypersensitivity Syndromes 1016 218.2 Saprophytic (Noninvasive) Syndromes 218.3 Invasive Disease 1017. Chapter 238 Parainfluenza Viruses 1075 Peter Wright 1017 Chapter 219 Histoplasmosis (Histoplasma capsulatum) 1018 Stephen C. Aronoff Chapter 220 Blastomycosis (Blastomyces dermatitidis) 1020 Robin B. Churchill and Stephen C. Aronoff Chapter 239 Respiratory Syncytial Virus Kenneth Mclntosh Chapter 240 Adenoviruses Kenneth Mclntosh Chapter 241 Rhinoviruses Kenneth Mclntosh Chapter 221 Coccidioidomycosis (Coccidioides immitis) 1021 Demosthenes Pappagianis 1076 1079 1080 Chapter 242 Rotavirus and Other Agents of Viral Gastroenteritis 1081 Dorsey M. Bass Chapter 243 Human Papillomaviruses Anna-Barbara Moscicki Chapter 222 Paracoccidioides brasiliensis 1023 1084 Chapter 244 Arboviral Encephalitis in North America 1086 Robin B. Churchill and Stephen C. Aronoff Chapter 223 Sporotrichosis Scott B. Halstead (Sporothrix schenckii) 1024 Robin B. Churchill and Stephen C. Aronoff Chapter 245 Arboviral Encephalitis Outside North America 1089 1025 Scott B. Halstead Robin B. Churchill and Stephen C. Aronoff Section 11: Viral Infections 1042 Mark J. Abzug Chapter 237 Martin E. Weisse Chapter 224 Zygomycosis 1036 Chapter 236 Human Herpesvirus8 Charles T. Leach Stephen C. Aronoff Chapter 217 Chapter 228 Polioviruses Eric A. F. Simoes Chapter 232 Varicella-ZosterVirus 1057 Martin G. Myers, Lawrence R. Stanberry, and Jane F. Seward Section 10: Mycotic Infections 1011 Chapter 216 1035 Chapter 229 Nonpolio Enteroviruses 1005 Chapter 212 Typhus Group Rickettsioses 212.1 Murine Typhus (Rickettsia typhi) 1005 212.2 Epidemic Typhus (Rickettsia prowazekii) 1006 Chapter 213 Chapter 227 Mumps Yvonne Maldonado 245.1 Venezuelan Equine Encephalitis 245.2 Japanese Encephalitis 1090 245.3 Tick-Borne Encephalitis 1091 1026 Chapter 225 Measles 1026 Yvonne Mdldonado 225.1 Subacute Sclerosing Panencephalitis Chapter 226 Rubella 1032 Yvonne Maldonado 226.1 Progressive Rubella Panencephalitis XXXI 1030 Chapter 246 Dengue Fever and Dengue Hemorrhagic Fever 1092 Scott B. Halstead 1034 1089 Chapter 247 Yellow Fever Scott B. Halstead 1095 X) XXXII Contents Cl Chapter 248 OtherViral Hemorrhagic Fevers 1096 Scott B. Halstead Rima McLeod and Jack S. Remington Chapter 249 Lymphocytic Choriomeningitis Virus 1099 Cl Hantaviruses 1100 Section 13: Helminthic Diseases Rabies William G. Adams Cl Chapter 253 Transmissible Spongiform Encephalopathies 1105 1105 Chapter 269 Hookworms (Ancylostoma and Necator americanus) 1156 Peter Hotez 269.1 Cutaneous Larva Migrans David M. Asher Chapter 271 Enterobiasis (Enterobius vermicularis) 1159 Ram Yogev and Ellen Gould Chadwick Sheral S. Patel and James W. Kazura Hal B. Jenson Section 12: Protozoan Diseases Sheral S. Patel and James W. Kazura 1123 Chandy C. John and Robert A. Salata Chapter 258 Giardiasis and Balantidiasis 1125 Larry K. Pickering 258.1 Giardia lamblia 258.2 Balantidiasis Cl Cl Chapter 277 Patricia M. Flynn Chapter 278 Intestinal) 1129 Robert A. Bonomo and Robert A. Salata 1169 Ronald Blanton Chapter 280 Chapter 262 AfricanTrypanosomiasis (Sleeping Sickness; Trypanosoma brucei complex) 1134 Cysticercosis 1171 Ronald Blanton Chapter 281 Echinococcosis (Echinococcus granulosusand E. multilocularis) 1173 Ronald Blanton Chapter 263 AmericanTrypanosomiasis (Chagas Disease; Trypanosoma cruzi) 1136 Robert A. Bonomo and Robert A. Salata Chapter 264 Malaria (Plasmodium) Babesiosis (Babesia) Peter J. Krause Section 14: Preventive Measures Chapter 282 1139 Peter J. Krause Chapter 265 1161 Flukes (Liver, Lung, and 1168 Chapter 279 Adult Tapeworm Infections 1130 Peter C. Melby Cl Schistosomiasis (Schistosoma) Charles H. King Chapter 261 Leishmaniasis (Leishmania) Cl 1166 Charles H. King Chandy C. John and Robert A. Salata Cl Sheral S. Patel and James W. Kazura Sheral S. Patel and James W. Kazura Chapter 260 Trichomoniasis (Trichomonas 5. Chapter 275 Toxocariasis (Visceral and Ocular Larva Migrans) 1164 Chapter 276 Trichinosis (Trichinella spiralis) 1125 1127 Chapter 259 Spore-Forming Intestinal Protozoa 1128 vagina I is) . 1162 Chapter 274 Other Tissue Nematodes Martin E. Weisse and Stephen C. Aronoff Cl stereo ra Iis) 1160 Sheral S. Patel and James W. Kazura Sheral S. Patel and James W. Kazura 1122 Chapter 257 Amebiasis Chapter 272 Strongyloidiasis (Strongyloides Chapter 273 Lymphatic Filariasis (Brugia malayi, Brugia timori, and Wuchereria bancrofti) 1161 1122 Primary Amebic , Meningoencephalitis 1158 Sheral S. Patel and James W. Kazura Chapter 255 HumanT-Cell LymphotropicViruses Types I and II 1121 Cl 1158 Chapter 270 Trichuriasis (Trichuris trichiura) Chapter 254 Acquired Immunodeficiency Syndrome (Human Immunodeficiency Virus) 1109 Chapter 256 1155 Sheral S. Patel and James W. Kazura Chapter 252 Polyomaviruses Hal B. Jenson Cl 1154 Chapter 268 Ascariasis (Ascaris lumbricoides) 1155 1101 Cl Cl Pneumocystis carinii Walter T. Hughes Scott B. Halstead Chapter 251 Cl Chapter 267 Hal B. Jenson Chapter 250 Cl Chapter 266 Toxoplasmosis (Toxoplasma gondii) 1144 1143 1174 Immunization Practices Georges Peter Chapter 283 Infection Control and Prophylaxis 1184 Margaret C. Fisher 1174 , Contents Chapter 284 Child Care and Communicable Diseases 1187 Larry K. Pickering Chapter 285 Health Advice for ChildrenTraveling Internationally 1189 Chandy C. John and Robert A. Salata Chapter 301 Obstructing and Motility Disorders of the Esophagus 1220 Chapter 302 Dysmotility Chapter 303 Hiatal Hernia 1220 1221 Chapter 304 Gastroesophageal Reflux Disease (GERD) 1222 304.1 Complications of GERD Part XVII The Digestive System 1197 Section 1: Clinical Manifestations of Gastrointestinal Disease 1197 Robert Wyllie Chapter 287 Major Symptoms and Signs of DigestiveTract Disorders 1198 1204 Chapter 288 Development and Developmental Anomalies of theTeeth 1204 Chapter 289 Disorders of the Oral Cavity Associated with Other Conditions 1206 Chapter 290 Malocclusion Chapter 291 Cleft Lip and Palate 1206 1207 Chapter 292 Syndromes with Oral Manifestations 1209 Chapter 293 Dental Caries Chapter 294 Periodontal Diseases Chapter 295 DentalTrauma 1209 1212 1213 Chapter 296 Common Lesions of the Oral Soft Tissues 1214 Chapter 297 Diseases of the Salivary Glands and Jaws 1215 Chapter 298 Diagnostic Radiology in Dental Assessment 1216 1217 Susan Orenstein, John Peters, Seema Khan, Nader Youssef, and Sunny Zaheed Hussain Chapter 299 Embryology, Anatomy, and Function of the Esophagus 1217 299.1 Common Clinical Manifestations and Diagnostic Aids 1218 Chapter 300 Congenital Anomalies: Esophageal Atresia and Tracheoesophageal Fistula 1219 300.1 Chapter 305 Non-GERDEsophagitis 1224 Chapter 306 Esophageal Perforation 1225 Chapter 307 Esophageal Varices 1225 Section 4: Stomach and Intestines Chapter 309 Function 1225 1228 Normal Development, Structure, and 1228 Robert Wyllie Norman Tinanoff Section 3: The Esophagus 1224 Chapter 308 Ingestions 1225 308.1 Foreign Bodies in the Esophagus 308.2 Caustic Ingestions 1226 Chapter 286 Normal DigestiveTract Phenomena 1197 Section 2: The Oral Cavity XXXIII Congenital Anomalies: Laryngotracheoesophageal Clefts 1220 Chapter 310 Pyloric Stenosis and Congenital Anomalies of the Stomach 1229 Robert Wyllie 310.1 Hypertrophic Pyloric Stenosis 1229 310.2 Congenital Gastric Outlet Obstruction 1231 310.3 Gastric Duplication 1231 310.4 Gastric Volvulus 1231 310.5 Hypertrophic Gastropathy 1231 Chapter 311 Intestinal Atresia, Stenosis, and Malrotation 1232 Robert Wyllie 311.1 Duodenal Obstruction 1233 311.2 Jejunal and Heal Atresia and Obstruction 1234 311.3 Malrotation 1235 Chapter 312 Intestinal Duplications, Meckel Diverticulum, and Other Remnants of the Omphalomesenteric Duct 1236 Robert Wyllie 312.1 Intestinal Duplication 1236 312.2 Meckel Diverticulum and Other Remnants of the Omphalomesenteric Duct 1236 Chapter 313 Motility Disorders and Hirschsprung Disease 1237 Robert Wyllie 313.1 Chronic Intestinal Pseudo-Obstruction 1237 313.2 Functional Constipation 1238 313.3 C ongenital Aganglionic Megacolon (Hirschsprung Disease) 1239 313.4 Superior Mesenteric Artery Syndrome (Wilkie Syndrome, Cast Syndrome, Arteriomesenteric Duodenal Compression Syndrome) 1241 Chapter 314 Ileus, Adhesions, Intussusception, and Closed-Loop Obstructions 1241 Robert Wyllie \ 314.1 Ileus 1241 C 314.2 Adhesions 1241 •* xxxiv Contents 314.3 Intussusception 1242 314.4 Closed-Loop Obstructions 325.5 Rectal Prolapse 1289 325.6 Pilonidal Sinus and Abscess 1243 Chapter 315 Foreign Bodies and Bezoars 1244 Robert Wyllie 315.1 Foreign Bodies in the Stomach and Intestine 1244 315.2 Bezoars 1244 1246 1248 1252 1257 Jeffrey Hyams Chapter 320 Malabsorptive Disorders 1257 Manuel Garda-Careaga and John A. Kerner 320.1 Evaluation of Children with Suspected Intestinal Malabsorption 1257 320.2 Chronic Malnutrition 1261 320.3 Liver and Biliary Disorders 1261 320.4 Intestinal Infections 1261 320.5 Immunodeficiency 1262 320.6 Stagnant Loop Syndrome (Blind Loop Syndrome: Bacterial Overgrowth Syndrome) 1262 320.7 Short Bowel Syndrome 1262 320.8 Gluten-Sensitive Enteropathy (Celiac Disease, Celiac Sprue) 1264 320.9 Immunoproliferative Small Intestinal Disease 1266 320.10 Other Malabsorptive Snydromes 1266 320.11 Enzyme Deficiencies 1267 320.12 Defects of Absorption or Transport 1268 1272 Larry K. Pickering and John D. Snyder Chapter 322 Chronic Diarrhea 1276 Chapter 323 Recurrent Abdominal Pain of Childhood 1281 Robert Wyllie 1283 Gary E. Hartman Chapter 325 Surgical Conditions of the Anus, Rectum, and Colon 1285 Alberto Pena 325.1 325.2 325.3 325.4 Inguinal Hernias 1292 1293 Section 5: Exocrine Pancreas 1298 Chapter 328 Embryology, Anatomy, and Physiology 1298 328.1 Anatomic Abnormalities 1298 328.2 Physiology 1298 1299 Disorders of the Exocrine 1299 Chapter 331 Treatment of Pancreatic Insufficiency 1300 Chapter 332 Pancreatitis 1300 332.1 Acute Pancreatitis 1300 332.2 Chronic Pancreatitis 1302 Chapter 333 Pseudocyst of the Pancreas Chapter 334 PancreaticTumors 1303 1303 Section 6: The Liver and Biliary System 1304 Chapter 335 Development and Function of the Liver and Biliary System 1304 Michael D. Bates and William F. Balistreri Chapter 336 Manifestations of Liver Disease 1308 Vicky Lee Ng and William F. Balistreri 336.1 Evaluation of Patients with Possible Liver Dysfunction 1312 Chapter 337 Cholestasis 1314 Hassan H. A-Kaderand William F. Balistreri 337.1 Neonatal Cholestasis 1314 337.2 Cholestasis in the Older Child 1319 Chapter 338 Metabolic Diseases of the Liver 1319 Jeffrey A. Rudolph and William F. Balistreri Fayez K. Ghishan Chapter 324 Acute Appendicitis Chapter 327 Chapter 329 Pancreatic FunctionTests Jeffrey Hyams Chapter 321 Gastroenteritis 326.1 Diarrhea from Hormone-Secreting Tumors Chapter 330 Pancreas Eosinophilic Gastroenteritis 1289 Joel Shilyansky Steven L. Werlin Chapter 318 Food Allergy (Food Hypersensitivity) 1255 Chapter 319 Chapter 326 Tumors of the DigestiveTract John J. Aiken Chapter 316 Peptic Ulcer Disease 1244 Francisco A. Sylvester 316.1 Secondary or Stress (Peptic) Ulcer Disease 316.2 Zollinger-Ellison Syndrome 1247 Chapter 317 Inflammatory Bowel Disease Jeffrey Hyams 317.1 Chronic Ulcerative Colitis 1249 317.2 Crohn Disease (Regional Enteritis, Regional lleitis, Granulomatous Colitis) 317.3 Behcet Syndrome 1255 1289 Anorectal Malformations 1285 Anal Fissure 1288 Perianal Abscess and Fistula 1288 Hemorrhoids 1289 338.1 Inherited Deficient Conjugation of Bilirubin (Familial Nonhemolytic Unconjugated Hyperbilirubinemia) 1320 338.2 Wilson Disease 1321 1323 338.3 Hepatic Copper Overload Syndrome 338.4 Indian Childhood Cirrhosis 1323 338.5 Neonatal Iron Storage Disease (NISD) 132 3 338.6 Miscellaneous Metabolic Diseases of the Liver 1323 Chapter 339 Viral Hepatitis 1324 John D. Snyder and Larry K. Pickering 339.1 Hepatitis in Neonates Chapter 340 Liver Abscess 1331 1332 Jeffrey Schwimmer and William F. Balistreri Contents Chapter 341 Disorders Liver Disease Associated with Systemic 1333 Jeffrey Schwimmer and William F. Balistreri Chapter 342 Reye Syndrome and the Mitochondrial Hepatopathies 1335 Chapter 343 Autoimmune (Chronic) Hepatitis 1337 Benjamin L. Shneider and Frederick J. Suchy Fulminant Hepatic Failure 1341 Frederick J. Suchy Chapter 346 Cystic Diseases of the Biliary Tract and Liver 1343 Frederick J. Suchy Diseases of the Gallbladder 1345 Frederick J. Suchy Liver Transplantation Ascites 1351 Chapter 359 Diagnostic Approach to Respiratory Disease 1375 Gabriel G. Haddad and Regina M. Palazzo Chapter 360 Sudden Infant Death Syndrome 1380 Carl E. Hunt and Fern R. Hauck Chapter 363 1351 Nasal Polyps 1388 Joseph Haddad Jr. 1351 Chapter 364 The Common Cold Chapter 365 Sinusitis 1391 Diane E. Poppas and J. Owen Hendley Chapter 366 Acute Pharyngitis 1393 Gregory F. Hayden and Ronald B. Turner Chapter 353 Diaphragmatic Hernia 1353 Gary E. Hartman 353.1 Foramen of Morgagni Hernia 1355 353.2 Paraesophageal Hernia 1355 353.3 Eventration 1355 Epigastric Hernia 1389 Ronald B. Turner and Gregory F. Hayden Chapter 352 Peritonitis 1351 Jeffrey S. Hyams 352.1 Acute Primary Peritonitis 1352 352.2 Acute Secondary Peritonitis 1352 352.3 Acute S econdary Localized Peritonitis (Peritoneal Abscess) 1353 Chapter 354 Chapter 358 Defense Mechanisms and Metabolic Functions of the Lung 1373 Gabriel G. Haddad and J. Julio Perez Fontdn 358.1 Defense Mechanisms 1373 358.2 Metabolic Functions 1375 Chapter 362 Acquired Disorders of the Nose 1387 Joseph Haddad Jr. 362.1 Foreign Body 1387 362.2 Epistaxis 1387 Jeffrey S. Hyams 351.1 Chylous Ascites 357.2 Congenital Central Hypoventilation Syndrome (Ondine Curse) 1372 Joseph Haddad Jr. 1349 Section 7: Peritoneum 1351 Chapter 351 1362 Chapter 361 Congenital Disorders of the Nose 1386 Kenneth L. Cox Chapter 350 Malformations Jeffrey S: Hyams Chapter 357 Respiratory Pathophysiology J. Julio Perez Fontdn and Gabriel G. Haddad 357.1 Respiratory Failure 1370 Section 2: Upper Respiratory Tract 1386 Chapter 348 Portal Hypertension and Varices 1346 Frederick J. Suchy Chapter 349 1359 Gabriel G. Haddad Chapter 344 Drug- andToxin-lnduced Liver Injury 1339 Frederick J. Suchy Chapter 347 Regulation of Respiration Gabriel G. Haddad J. Julio Perez Fontdn and Gabriel G. Haddad Jeffrey A. Rudolph and William F. Balistreri Chapter 345 Chapter 356 XXXV Chapter 367 Retropharyngeal Abscess, Lateral Pharyngeal (Parapharyngeal) Abscess, and Peritonsillar Cellulitis/Abscess 1394 Diane E. Poppas and J. Owen Hendley Chapter 368 Tonsils and Adenoids 1355 1396 Ralph F. Wetmore Gary E. Hartman 354.1 Incisional Hernia Chapter 369 Obstructive Sleep Apnea and Hypoventilation 1397 1356 Carol L. Rosen, Lewis J. Kass, and Gabriel G. Haddad : XVIII: The Respiratory System Section 1: Development and Function 1357 1357 Chapter 355 Development of the Respiratory System 1357 Gabriel G. Haddad and J. Julio Perez Fontdn Section 3: Disorders of the Lungs and Lower Airways 1401 Thomas P. Green and Susanna A. McColley Chapter 370 Chronic or Recurrent Respiratory, Symptoms 1401 '• Thomas F. Boat ' XXXVI Contents Chapter 371 Acute Inflammatory Upper Airway Obstruction 1405 Glenna B. Winnie and Steven Boas Genie E. Roosevelt 371 :i Infectious Upper Airway Obstruction 371.2 Bacterial Tracheitis 1409 1405 Chapter 372 Congenital Anomalies of the Larynx 1409 Lauren D. Holinger 372.1 372.2 372.3 372.4 372.5 372.6 372.7 Laryngomalacia 1409 Congenital Subglottic Stenosis 1409 Vocal Cord Paralysis 1410 Congenital Laryngeal Webs and Atresia 1410 Congenital SubglotticHemangioma 1410 Laryngoceles and Saccular Cysts 1410 Posterior Laryngeal Cleft and Laryngotracheoesophageal Cleft 1410 Chapter 373 Foreign Bodies of the Airway 1410 Lauren D. Holinger 373.1 Laryngeal Foreign B odies 1411 373.2 Tracheal Foreign Bodies 1411 373.3 Bronchial Foreign Bodies 1411 Chapter 374 Stenosis Chapter 381 o^-Antitrypsin Deficiency and Emphysema 1421 Laryngotracheal Stenosis, Subglottic 1412 Chapter 382 Other Distal Airway Diseases 1422 Steven Boas 382.1 Bronciolitis Obliterans 1422 382.2 Follicular Bronchitis 1422 382.3 Pulmonary Alveolar Microlithiasis 1422 Chapter 383 Congenital Disorders of the Lung 1423 Thomas P. Green and Jonathan D. Finder 383.1 383.2 383.3 383.4 383.5 383.6 383.7 383.8 Pulmonary Agenesis and Aplasia 1423 Pulmonary Hypoplasia 1423 Cystic Adenomatoid Malformation 1424 Pulmonary Sequestration 1424 Bronchogenic Cysts 1425 Congenital Pulmonary Lymphangiectasia 1425 Lung Hernia 1425 Other Congenital Malformations of the Lung 1425 Chapter 384 Pulmonary Edema 1426 Robert Mazor and Thomas P. Green Lauren D. Holinger Chapter 385 Aspiration Syndromes 374.1 Congenital Subglottic Stenosis 1412 374.2 AcquiredXaryngotracheal Stenosis 1412 Chapter 386 Chapter 375 Congenital Anomalies of theTrachea and Bronchi 1412 Lauren D. Holinger 375.1 Vascular and Cardiac Anomalies 1412 375.2 Trachea] Stenoses, Webs, and Atresia 1412 375.3 Foregut Cysts 1413 Chapter 376 Bronchomalacia and Tracheomalacia 1413 Chapter 377 Neoplasms of the Larynx,Trachea, and Bronchi 1413 Lauren D. Holinger 377.1 Vocal Nodules 1413 377.2 Recurrent Respiratory Papillomatosis (RRP) 1413 377.3 Congenital Subglottic Hemangioma 1414 377.4 Vascular Anomalies . 1414 377.5 Other Laryngeal Neoplasms 1414 377.6 Tracheal Neoplasms 1414 377.7 Bronchial Tumors 1414 Chapter 378 Inflammatory Disorders of the Small Airways 1414 Denise Goodman John L. Colombo Chapter 388 Parenchymal Disease with Prominent Hypersensitivity, Eosinophilic Infiltration, or Toxin-Mediated Injury 1430 388.1 388.2 388.3 388.4 Hypersensitivity to Inhaled Materials 1430 Silo Filler Disease 1431 Paraquat Lung 1431 Eosinophilic Lung Disease (Formerly Loffler Syndrome) 1432 Chapter 389 Pneumonia 1432 Theodore C. Sectish and Charles G. Prober Chapter 390 Slowly Resolving Pneumonia 1435 Oren Lakser Chapter 391 Bronchiectasis 1436 Oren Lakser Chapter 392 Pulmonary Abscess 1437 Oren Lakser Cystic Fibrosis 1437 Thomas F. Boat 1417 Marzena Krawiec and Robert F. Lemanske, Jr. Steven Boas and Glenna B. Winnie 1428 Chapter 387 Gastroesophageal Reflux and Respiratory Disorders 1430 Chapter 393 378.1 Bronchitis 1414 378.2 Bronchiolitis 1415 Chapter 380 .Emphysema and Overinflation 1419 Chronic Recurrent Aspiration John L. Colombo Oren Lakser Jonathan D. Finder Chapter 379 Wheezing in Infants 1427 John L. Colombo Chapter 394 Primary Ciliary Dyskinesia (Immotile Cilia Syndrome) 1450 Gabriel G. Haddad Chapter 395 Interstitial Lung Diseases 1451 Michelle S. Howenstine \ Contents Chapter 396 Pulmonary Alveolar Proteinosis 1453 Aaron Hamvas, Lawrence M. Nogee, and F. Sessions Cole Chapter 397 Inherited Disorders of Surfactant Protein Metabolism 1454 Aaron Hamvas, Lawrence M. Nogee, andF. Sessions Cole Chapter 398 Pulmonary Hemosiderosis 1455 Dorr G. Dearborn Chapter 399 Pulmonary Hemorrhage, Embolism, and Infarction 1457 Daniel Sloniewsky and Thomas P. Green 399.1 Pulmonary Embolism and Infarction 399.2 Hemoptysis 1458 Chapter 400 Atelectasis 1457 1459 Pleurisy Section V. Developmental Biology of the Cardiovascular System 1475 Daniel Bernstein Chapter 413 Cardiac Development 413.1 413.2 413.3 413.4 413.5 413.6 414.1 414.2 414.3 414.4 1461 Susanna A. McColley and Paul R. Haut Chapter 402 Part XIX The Cardiovascular System 1475 1461 Glenna B. Winnie 402.1 Dry or Plastic Pleurisy 1461 402.2 Serofibrinous Pleurisy 1462 402.3 Purulent Pleurisy (Empyema) Chapter 403 Pneumothorax Chapter 415 History and Physical Examination 1481 1465 Glenna B. Winnie Chapter 416 Laboratory Evaluation Chapter 405 Hydrothorax Glenna B. Winnie 1465 Chapter 406 Hemothorax Glenna B. Winnie 1465 Chapter 407 Chylothorax 1466 416.1 416.2 416.3 416.4 416.5 416.6 Glenna B. Winnie Chapter 408 The Fetal Circulation 1479 The Transitional Circulation 1479 The Neonatal Circulation 1479 Persistent Pulmonary Hypertension of the Neonate (Persistence of Fetal Circulatory Pathways) 1481 Daniel Bernstein 1463 Pneumomediastinum 1478 Section 2: Evaluation of the Cardiovascular System 1481 1462 Glenna B. Winnie Chapter 404 1475 Early Cardiac Morphogenesis 1475 Cardiac Looping 1475 Cardiac Septation 1475 Aortic Arch Development 1477 Cardiac Differentiation 1478 Developmental Changes in Cardiac Function Chapter 414 The Fetal-to-Neonatal Circulatory Transition 1479 Ranna A. Rozenfeld Chapter 401 Pulmonary Tumors XXXVil Bronchopulmonary Dysplasia 1466 Steven Lestrud Chapter 409 Skeletal Diseases Influencing Pulmonary Function 1467 Steven Boas 409.1 Pectus Excavatum (Funnel Chest) 1467 409.2 Pectus Carinatum and Fissuri Sterni 1468 409.3 Asphyxiating Thoracic Dystrophy (Thoracic-Pelvic-Phalangeal Dystrophy) 1468 409.4 Achondroplasia 1468 409.5 Kyphoscoliosis: Adolescent Idiopathic Scoliosis and Congential Scoliosis 1469 409.6 Congenital Rib Anomalies 1469 Chapter 410 Neuromuscular Diseases with Pulmonary Consequences 1469 David Gozal Chapter 411 Extrapulmonary Diseases with Pulmonary Manifestations 1471 Susanna A. McColley Chapter 412 Chronic Severe Respiratory Insufficiency 1472 Zehava Noah and Cynthia Budek 416.7 1488 Radiologic Assessment 1488 Electrocardiography 1489 Hematologic Data 1493 Echocardiography 1493 Exercise Testing 1495 MRI, Electron Beam CT, and Radionuclide Studies 1496 Cardiac Catheterization 1496 Section 3: Congenital Heart Disease 1499 Daniel Bernstein Chapter 417 Epidemiology and Genetic Basis of Congenital Heart Disease 1499 Chapter 418 Evaluation of the Infant or Child with Congenital Heart Disease 1502 Chapter 419 Acyanotic Congenital Heart Disease: The Left-to-Right Shunt Lesions 1503 419.1 419.2 419.3 419.4 419.5 419.6 419.7 419.8 419.9 Atrial Septal Defect 1503 Ostium Secundum Defect 1504 Sinus Venosus Atrial Septal Defect 1505 Partial Anomalous Pulmonary Venous Return 1505 Atrioventricular Septal Defects (Ostium Primum and Atrioventricular Canal or Endocardial Cushion Defects) 1506 Ventricular Septal Defect 1508 Supracristal Ventricular Septal Defect with Aortic Insufficiency 1510 t, Patent Ductus Arteriosus 1510 ',"» Aorticopulmonary Window Defect 1512 .'-\ xxxviii Contents 419.10 Coronary-Arteriovenous Fistula (Coronary-Cameral Fistula) 1512 419.11 Ruptured Sinus of Valsalva Aneurysm 1512 Chapter 420 Acyanotic Congenital Heart Disease: The Obstructive Lesions 1513 420.1 Pulmonary Valve Stenosis with Intact Ventricular Septum 1513 420.2 Infundibular Pulmonary Stenosis and DoubleChamber Right Ventricle 1515 420.3 Pulmonary Stenosis in Combination with an Intracardiac Shunt 1515 420.4 Peripheral Pulmonary Stenosis 1515 420.5 Aortic Stenosis 1516 420.6 Coarctation of the Aorta 1518 420.7 Coarctation with Ventricular Septal Defect 1520 420.8 Coarctation with Other Cardiac Anomalies and Interrupted Aortic Arch 1520 420.9 Congenital Mitral Stenosis 1520 42 0.10 Pulmonary Venous Hypertension 1521 Chapter 421 Acyanotic Congenital Heart Disease: Regurgitant Lesions 1522 421.1 Pulmonary Valvular Insufficiency and Congenital Absence of the Pulmonary Valve 1522 421.2 Congenital Mitral Insufficiency 1522 421.3 Mitral Valve Prolapse 1522 421.4 Tricuspid Regurgitation 1523 Chapter 422 Cyanotic Congenital Heart Disease: Evaluation of the Critically III Neonate with Cyanosis and Respiratory Distress 1523 Chapter 423 Cyanotic Congenital Heart Lesions: Lesions Associated with Decreased Pulmonary Blood Flow 1524 423.1 Tetralogy of Fallot 1524 42 3.2 Pulmonary Atresia with Ventricular Septal Defect 1528 423.3 Pulmonary Atresia with Intact Ventricular Septum 1529 423.4 Tricuspid Atresia 1530 423.5 Double-Outlet Right Ventricle with Pulmonary Stenosis 1532 42 3.6 Transposition of the Great Arteries with Ventricular Septal Defect and Pulmonary Stenosis 1532 423.7 Ebstein Anomaly of the Tricuspid Valve 1532 Chapter 424 Cyanotic Congenital Heart Disease: Lesions Associated with Increased Pulmonary Blood Flow 1534 424.1 d-Transposition of the Great Arteries 1534 424.2 d-Transposition of the Great Arteries with Intact Ventricular Septum 1535 424.3 Transposition of the Great Arteries with Ventricular Septal Defect 1536 424.4 1-Transposition of the Great Arteries (Corrected ^ Transposition) 1537 424.5 Double-Outlet Right Ventricle Without Pulmonary Stenosis 1538 424.6 Double-Outlet Right Ventricle with Transposition of the Great Arteries (Taussig-Bing Anomaly) 1538 424.7 Total Anomalous Pulmonary Venous Return 1538 424.8 Truncus Arteriosus 1540 424.9 Single Ventricle (Double-Inlet Ventricle, Univentricular Heart) 1541 424.10 Hypoplastic Left Heart Syndrome 1541 424.11 Abnormal Positions of the Heart and the Heterotaxy Syndromes (Asplenia, Polysplenia) 1544 Chapter 425 Other Congenital Heart and Vascular Malformations 1545 42 5.1 Anomalies of the Aortic Arch 1545 42 5.2 Anomalous Origin of the Coronary Arteries 1545 42 5.3 Pulmonary Arteriovenous Fistula 1547 425.4 Ectopia Cordis 1548 42 5.5 Diverticulum of the Left Ventricle 1548 Chapter 426 Pulmonary Hypertension 1548 426.1 Primary Pulmonary Hypertension 1548 426.2 Pulmonary Vascular Disease (Eisenmenger Syndrome) 1549 Chapter 427 General Principles of Treatment of Congenital Heart Disease 1551 Section 4: Cardiac Arrhythmias 1554 Anne Dubin Chapter 428 Disturbances of Rate and Rhythm of the Heart 1554 428.1 Sinus Arrhythmias and Extrasystoles 1554 428.2 Supraventricular Tachycardia 1559 428.3 Ventricular Tachyarrhythmias 1561 428.4 Long Q-T Syndrome 1562 428.5 Bradyarrhythmias 1562 428.6 Sick Sinus Syndrome 1563 428.7 Sudden Death 1563 Section 5:AcquiredHeartDisease 1565 Daniel Bernstein Chapter 429 Infective Endocarditis 1565 Chapter 430 Rheumatic Heart Disease 1570 Section 6: Diseases of the Myocardium and Pericardium 1572 Daniel Bernstein Chapter 431 Diseases of the Myocardium 431.1 Dilated Cardiomyopathy 15 72 431.2 Hypertrophic Cardiomyopathy 1575 431.3 Restrictive Cardiomyopathy 1576 431.4 Myocarditis 1576 431.5 Nonviral Causes of Myocarditis 1577 431.6 Endocardial Fibroelastosis 1578 Chapter 432 432.1 Diseases of the Pericardium Constrictive Pericarditis 1581 Chapter 433 Tumorsof the Heart 1581 Section 7: Cardiac Therapeutics 1582 Daniel Bernstein Chapter 434 434.1 1572 Heart Failure Cardiogenic Shock 1582 1586 1579 , : Contents Chapter 435 Pediatric Heart and Heart-Lung Transplantation 1587 435.1 Pediatric Heart Transplantation 1587 435.2 Heart-Lung and Lung Transplantation 1590 Chapter 451 Section 8: Diseases of the Peripheral Vascular System 1591 Chapter 453 Chapter 452 Hereditary Stomatocytosis 1622 George B. Segel Other Membrane Defects 1622 Chapter 454 Hemoglobin Disorders 1623 Keith Quircdo and Elliott Vichinsky 454.1 Sickle Cell Disease 1624 454.2 Sickle Cell Trait (Hemoglobin AS) 1628 454.3 Other Hemoglobinopathies 1629 454.4 Unstable Hemoglobin Disorders 1629 454.5 Abnormal Hemoglobins with Increased Oxygen Affinity 1629 454.6 Abnormal Hemoglobins Causing Cyanosis 1629 454.7 Hereditary Methemoglobinemia 1629 454.8 Syndromes of Hereditary Persistence of Fetal Hemoglobin (HPFH) 1630 454.9 Thalassemia Syndromes 1630 Chapter 436 Disease of the Blood Vessels (Aneurysms and Fistulas) 1591 436.1 Kawasaki Disease 1591 436.2 Arteriovenous Fistulas 1591 Chapter 437 Systemic Hypertension 1592 Diseases of the Blood 1599 1599 Chapter 438 Development of the Hematopoietic System 1599 Robin K. Ohls and Robert D. Christensen Chapter 439 The Anemias 1622 George B. Segel George B. Segel Daniel Bernstein Section 1: The Hematopoietic System Hereditary Elliptocytosis XXXJX Chapter 455 Enzymatic Defects 1635 George B. Segel 455.1 Pyruvate Kinase (PK) Deficiency 1635 455.2 Other Glycolytic Enzyme Deficiencies 1636 455.3 Glucose-6-Phosphate Dehydrogenase (G6PD) and Related Deficiencies 1636 1604 Bertil Glader Section 2: Anemias of Inadequate Production 1606 Bertil Glader Chapter 456 Hemolytic Anemias Resulting from Extracellular Factors 1638 George B. Segel Chapter 440 Congenital Hypoplastic Anemia (Diamond-Blackfan Anemia) 1606 Chapter 457 Hemolytic Anemias Secondary to Other Extracellular Factors 1640 Chapter 441 Pearson Marrow-Pancreas Syndrome 1607 George B. Segel Chapter 442 Acquired Pure Red Blood Cell Anemias 1608 Section 4: Polycythemia (Erythrocytosis) Chapter 443 Anemia of Chronic Disorders (ACD) and Renal Disease 1609 Chapter 458 Primary Polycythemia (Polycythemia RubraVera) 1641 Chapter 444 Congenital Dyserythropoietic Anemias (CDA) 1609 Chapter 459 Chapter 445 1610 Chapter 446 Megaloblastic Anemias 1611 446.1 Folic Acid Deficiency 1611 446.2 Vitamin B 12 (Cobalamin) Deficiency 1612 Chapter 447 Iron-Deficiency Anemia Chapter 448 Other Microcytic Anemias Section 3: Hemolytic Anemias 1613 1614 1616 1617 Chapter 449 Definitions and Classification of Hemolytic Anemias 1617 George B. Segel 1620 1641 1642 Chapter 460 The Constitutional Pancytopenias 1642 Alan D. D'Andrea Chapter 461 The Acquired Pancytopenias 1644 Jeffrey D. Hard Section 6: Risks of Blood Component Transfusions 1646 Ronald G. Strauss Chapter 462 Red Blood Cell Transfusions and ErythropoietinTherapy 1646 Chapter 463 George B. Segel Chapter 450 Hereditary Spherocytosis Secondary Polycythemia Section 5: The Pancytopenias Physiologic Anemia of Infancy 446.3 Other Rare Megaloblastic Anemias 1641 Bruce M. Camitta Platelet Transfusions 1647 Chapter 464 Neutrophil (Granulocyte) Transfusions 1648 A xl Contents Chapter 465 Fresh Frozen Plasma Transfusions 1649 Chapter 466 Section 8: The Spleen James French and Bruce M. Camitta Risks of BloodTransfusions 1650 Section 7: Hemorrhagic and Thrombotic Diseases Robert R. Montgomery and J. Paul Scott Chapter 467 Hemostasis 1651 467.1 Hemostatic Mechanism 1651 467.2 The Clinical and Laboratory Evaluation 1651 1653 Chapter 480 Anatomy and Function of the Lymphatic System 1677 Chapter 481 Abnormalities of Lymphatic Vessels 1677 Chapter 482 Lymphadenopathy 1677 Part XXI Cancer and Benign Tumors 1679 Chapter 483 Epidemiology of Childhood and Adolescent Cancer 1679 James G. Gurney and Melissa L. Bondy Chapter 485 1665 1666 Principles of Diagnosis 1684 Principles of Treatment 1688 Archie Bleyer Chapter 486 Archie Bleyer Chapter 487 The Leukemias 1668 Chapter 474 Acquired Inhibitors of Coagulation 1668 Chapter 475 Disseminated Intravascular Coagulation (Consumptive Coagulopathy) 1677 Bruce M. Camitta Laura L. Worth Chapter 472 Postneonatal Vitamin K Deficiency 1668 Liver Disease Section 9: The Lymphatic System Chapter 484 Molecular and Cellular Biology of Cancer 1681 Chapter 470 Hereditary Predisposition to Thrombosis 1664 Chapter 473 1675 Chapter 479 Hyposplenism, Splenic Trauma, and Splenectomy 1676 1662 Chapter 471 Acquired Thrombotic Disorders 471.1 Anticoagulant and Thrombolytic Therapy Chapter 477 Anatomy and Function of the Spleen 1675 Chapter 478 Splenomegaly Chapter 468 Hereditary Clotting Factor Deficiencies (Bleeding Disorders) 1657 468.1 Factor VIII or Factor IX Deficiency (Hemophilia A or B) 1657 468.2 Factor XI Deficiency (HemophiliaC) 1660 468.3 Deficiencies of the Contact Factors (Nonbleeding Disorders) 1660 468.4 Factor VII Deficiency 1661 468.5 Factor X Deficiency 1661 468.6 Prothrombin (Factor II) Deficiency 1661 468.7 Factor V Deficiency 1661 468.8 Combined Deficiency of Factors V and VIII 1661 468.9. Fibrinogen Deficiency 1661 468.10 Factor XIII Deficiency (Fibrin-Stabilizing Factor or Transglutaminase Deficiency) 1661 468.11 Antiplasmin or Plasminogen Activator Inhibitor (PAI) Deficiency 1662 Chapter 469 von Willebrand Disease 1675 1669 Chapter 476 Platelet and Blood Vessel Disorders 1670 476.1 Idiopathic Thrombocytopenic Purpura 1670 476.2 Drug-Induced Thrombocytopenia 1671 476.3 Nonimmune Platelet Destruction 1671 476.4 Hemolytic-Uremic Syndrome (HUS) 1671 476.5 Thrombotic Thrombocytopenic Purpura (TTP) 1672 476.6 Kasabach-Merritt Syndrome 1672 476.7 Sequestration 1672 476.8 Congenital Thrombocytopenic Syndromes 1672 476.9 Neonatal Thrombocytopenia 1672 476.10 Thrombocytopenia Due to Acquired Disorders Causing Decreased Production 1673 476.11 Platelet Function Disorders 1673 476.12 Acquired Disorders of Platelet Function 1673 476.13 Congenital Abnormalities of Platelet Function 1673 476.14 Disorders of the Blood Vessels 1674 1694 David G. Tubergen and Archie Bleyer 487.1 Acute Lymphoblastic Leukemia 1694 487.2 Acute Myelogenous Leukemia 1696 487.3 Down Syndrome and Acute Leukemia and Myeloproliferation 1697 487.4 Chronic Myelogenous Leukemia 1697 487.5 Juvenile Chronic Myelogenous Leukemia 1698 487.6 Infant Leukemia 1698 Chapter 488 Lymphoma 1698 GeraldS. Gilchrist 488.1 Hodgkin Disease 1698 488.2 Non-Hodgkin Lymphoma 1701 Chapter 489 BrainTumors in Childhood 1702 John F. Kuttesch, Jr. and Joann L. Ater Chapter 490 Neuroblastoma 1709 Joann L. Ater Chapter 491 Neoplasms of the Kidney 1711 Norman Jaffe and Vicki Huff Chapter 492 SoftTissue Sarcomas Carola A. S. Arndt 1714 Contents Chapter 493 CarolaA. S.Arndt Chapter 507 Nephritis 49 3.1 Malignant Tumors of B one 1717 493.2 Benign Tumors and Tumor-like Processes of Bone 1720 Chapter 508 Rapidly Progressive (Crescentic) Glomerulonephritis 1745 Chapter 494 Neoplasms of Bone Retinoblastoma 1717 Chapter 509 Goodpasture Disease 1722 Cynthia E. Herzog Chapter 511 Upper Urinary Tract Causes of Hematuria 1747 511.1 Interstitial Nephritis 1747 511.2 Toxic Nephropathy 1747 511.3 Cortical Necrosis 1747 511.4 Pyelonephritis 1747 511.5 Nephrocalcinosis 1747 511.6 Vascular Abnormalities 1747 511.7 Renal Vein Thrombosis 1747 511.8 Idiopathic Hypercalciuria 1748 Cynthia E. Herzog Neoplasms of the Liver 1725 Cynthia E. Herzog Chapter 497 Benign VascularTumors 1726 Cynthia E. Herzog Chapter 498 Rare Tumors 1726 Cynthia E. Herzog Chapter 499 Histiocytosis Syndromes of Childhood 1727 Chapter 512 Hematologic Diseases Causing Hematuria 1748 512.1 Sickle Cell Nephropathy 1748 512.2 Coagulopathies and Thrombocytopenia 1749 Stephan Ladisch Part XXII Nephrology 1745 Chapter 510 Hemolytic-Uremic Syndrome 1746 Chapter 495 Gonadal and Germ Cell Neoplasms 1723 Chapter 496 Henoch-Schonlein Purpura 1744 1731 Chapter 513 AnatomicAbnormalitiesAssociated with Hematuria 1749 513.1 Congenital Anomalies 1749 513.2 Autosomal Recessive Polycystic Kidney Disease 1749 513.3 Autosomal Dominant Polycystic Kidney Disease 1750 513.4 Trauma 1750 513.5 Renal Tumors 1750 Section 1:Glomerular Disease 1731 Ira D. Davis and Ellis D. Avner Chapter 500 Introduction to Glomerular Diseases 1731 500.1 Anatomy of the Glomerulus 1731 500.2 Glomerular Filtration 1732 500.3 Glomerular Diseases 1734 Section 2: Conditions Particularly Associated with Hematuria 1735 Ira D. Davis and Ellis D. Avner Chapter 501 Clinical Evaluation of the Child with Hematuria 1735 Chapter 502 Isolated Glomerular Diseases with Recurrent Gross Hematuria 1737 502.1 IgA Nephropathy (BergerNephropathy) 1737 502.2 Alport Syndrome 1738 502.3 Thin Glomerular Basement Membrane Disease 1739 Chapter 514 Lower Urinary Tract Causes of Hematuria 1751 514.1 Infectious Causes of Cystitis and Urethritis 1751 514.2 Hemorrhagic Cystitis 1751 514.3 Heavy Exercise 1751 514.4 Munchausen Syndrome by Proxy 1751 Section 3: Conditions Particularly Associated with Proteinuria 1751 Beth A. Vogt and Ellis D. Avner Chapter 515 Introduction to the Child with Proteinuria 1751 Chapter 503 Glomerulonephritis Associated with Infections 1740 503.1 Acute Poststreptococcal Glomerulonephritis 1740 503.2 Other Chronic Infections 1741 Chapter 516 Transient Proteinuria Chapter 504 Membranous Glomerulopathy (Glomerulonephritis) 1741 Chapter 518 Fixed Proteinuria 1752 518.1 Glomerular Proteinuria 1752 518.2 Tubular Proteinuria 1753 Chapter 505 Membranoproliferative (Mesangiocapillary) Glomerulonephritis 1742 Chapter 506 Glomerulonephritis Associated with Systemic Lupus Erythematosus 1743 1752 Chapter 517 Orthostatic (Postural) Proteinuria 1752 Chapter 519.1 519.2 519.3 519 Nephrotic Syndrome 1753 Idiopathic Nephrotic Syndrome 1755 Secondary Nephrotic Syndrome 1757 Congenital Nephrotic Syndrome 1757 xli Xlii Contents Section 4: Tubular Disorders 1758 Chapter 535 Voiding Dysfunction Chapter 520 Tubular Function 1758 Katherine MacRae Dell and Ellis D. Avner Chapter 536 Anomalies of the Penis and Urethra 1812 Chapter 521 521.1 521.2 521.3 521.4 RenalTubularAcidosis 1758 Proximal (Type II) Renal Tubular Acidosis 1759 Katherine MacRae Dell and Ellis D. Avner Distal (Type I) Renal Tubular Acidosis 1760 Katherine MacRae Dell and Ellis D. Avner Hyperkalemic (Type IV) Renal Tubular Acidosis 1761 Katherine MacRae Dell and Ellis D. Avner Rickets Associated with Renal Tubular Acidosis 1762 Russell W. Chesney Chapter 522 Nephrogenic Diabetes Insipidus Chapter 537 Contents 1808 Disorders and Anomalies of the Scrotal 1817 Chapter 538 Trauma to the Genitourinary Tract 1821 Chapter 539 Urinary Lithiasis 1822 Part XXIV Gynecologic Problems of Childhood 1827 1762 Joseph S. Sanfilippo Katherine MacRae Dell and Ellis D. Avner Chapter 523 Bartter/Gitelman Syndromes and Other InheritedTubularTransport Abnormalities 1763 Katherine MacRae Dell and Ellis D. Avner 523.1 Banter Syndrome 1763 523.2 Gitelman Syndrome 1764 • 523.3 Other Inherited Tubular Transport Abnormalities 1764 Chapter 540 History and Physical Examination 1827 Chapter 541 Vulvovaginitis Chapter 524 Tubulointerstitial Nephritis 1764 Katherine MacRae Dell and Ellis D. Avner 1828 Chapter 542 Bleeding 1833 Chapter 543 Breast Disorders 1833 Chapter 544 Hirsutism and Polycystic Ovarian Syndrome 1835 SectionS: Toxic Nephropathies—Renal Failure 1766 Chapter 545 Chapter 525 Toxic Nephropathy Chapter 546 Vulvovaginal and Mullerian Anomalies 1839 1766 Beth A. Vogt and Ellis D. Avner Chapter 526 Cortical Necrosis 1767 Beth A. 527.1 527.2 527.3 Renal Failure Chapter 548 Special Gynecologic Needs 1767 Chapter 549 Gynecologic Imaging Vogt and Ellis D. Avner Acute Renal Failure 1767 Chronic Renal Failure 1771 End-Stage Renal Disease 1774 Chapter 528 RenalTransplantation 1843 1843 Part XXV The Endocrine System 1845 Section 1: Disorders of the Hypothalamus and Pituitary Gland 1845 Part XXI11 Urologic Disorders in Infants and Children 1783 Chapter 550 Hormones of the Hypothalamus and Pituitary 1845 John S. Parks Chapter 551 Hypopituitarism 1847 John S. Parks Jack S. Elder Chapter 529 Congenital Anomalies and Dysgenesis of the Kidneys 1783 Urinary Tract Infections Chapter 531 Vesicoureteral Reflux 1785 Chapter 533 Anomalies of the Bladder Neuropathic Bladder 1804 1806 Chapter 552 Diabetes Insipidus 1853 David T. Breault and Joseph A. Majzoub 552.1 Causes of Hypernatremia 1854 Chapter 553 Other Abnormalities of Arginine Vasopressin Metabolism and Action 1855 1790 Chapter 532 Obstructions of the Urinary Tract Chapter 534 1842 1775 Rodrigo E. Urizar Chapter 530 1837 Chapter 547 Athletic Gynecologic Problems Beth A. Vogt and Ellis D. Avner Chapter 527 Neoplasms 1794 David T. Breault and Joseph A. Majzoub Chapter 554 Hyperpituitarism,Tall Stature, and Overgrowth Syndromes 1857 Pinchas Cohen Contents Chapter 555 Physiology of Puberty 1862 Luigi Garibaldi Section 4: Disorders of the Adrenal Glands 1898 Lenore S. Levine and Perrin C. White Chapter 556 Disorders of Pubertal Development 1863 Luigi Garibaldi 556.1 Gonadotropin-Dependent Precocious Puberty 1863 556.2 Precocious Puberty Resulting from Organic Brain Lesions 1865 556.3 Precocious Puberty Following Irradiation of the Brain 1866 556.4 Syndrome of Precocious Puberty and Hypothyroidism 1866 556.5 Gonadotropin-Secreting Tumors 1867 556.6 McCune-Albright Syndrome (Precocious Puberty with Polyostotic Fibrous Dysplasia and Abnormal Pigmentation) 1867 556.7 Familial Male Gonadotropin-Independent Precocious Puberty 1868 556.8 Incomplete (Partial) Precocious Development 1868 556.9 Medicational Precocity 1869 Section 2: Disorders of the Thyroid Gland 1870 Stephen LaFranchi Chapter 557 Thyroid Development and Physiology 1870 557.1 Thyroid Hormone Studies 1871 Chapter 568 The Physiology of the Adrenal Gland 1898 568.1 Histology and Embryology 1898 568.2 Adrenal Steroid Biosynthesis 1899 568.3 Regulation of the Adrenal Cortex 1900 568.4 Adrenal Steroid Hormone Actions 1901 568.5 Adrenal Medulla 1904 Chapter 569 Adrenocortical Insufficiency 1904 569.1 Primary Adrenal Insufficiency 1904 569.2 Secondary Adrenal Insufficiency 1908 Chapter 570 Congenital Adrenal Hyperplasia and Related Disorders 1909 570.1 Congenital Adrenal Hyperplasia due to 21 -Hydroxylase Deficiency 1909 570.2 Congenital Adrenal Hyperplasia due to 11 P-Hydroxylase Deficiency 1913 570.3 Congenital Adrenal Hyperplasia due to 3(3Hydroxysteriod Dehydrogenase Deficiency 1913 570.4 Congenital Adrenal Hyperplasia due to 17-Hydroxylase Deficiency 1914 570.5 Lipoid Adrenal Hyperplasia 1914 570.6 Aldosterone Synthase Deficiency 1915 570.7 Glucocorticoid-Suppressible Hyperaldosteronism 1915 Chapter 571 Cushing Syndrome 1916 Chapter 558 Globulin Defects of Thyroxine-Binding 1872 Chapter 572 Chapter 559 Hypothyroidism Chapter 573 AdrenalTumors 1918 573.1 Virilizing Adrenocortical Tumors 1918 Chapter 560 Thyroiditis Chapter 561.1 561.2 561.3 561.4 xliii 1872 1879 561 Goiter 1881 Congenital Goiter 1881 Endemic Goiter and Cretinism Sporadic Goiter 1883 Intratracheal Goiter 1883 Primary Aldosteronism 573.2 Feminizing Adrenal Tumors Chapter 574 1882 Chapter 562 Hyperthyroidism 1884 562.1 Graves Disease 1884 562.2 Congenital Hyperthyroidism 1886 1918 1919 Pheochromocytoma 1919 Chapter 575 Adrenal Masses 1920 575.1 Adrenal Incidentaloma 1920 575.2 Adrenal Calcification 1921 Section 5: Disorders of the Gonads 1921 Robert Rapaport Chapter 576 Development and Function of the Gonads 1921 Chapter 563 Carcinoma of the Thyroid 1887 ' 563.1 Solitary Thyroid Nodule 1888 563.2 Medullary Carcinoma 1889 Section 3: Disorders of the Parathyroid 1890 Daniel A. Doyle and Angelo M. DiGeorge Chapter 577 Hypofunction of theTestes 1924 577.1 Hypergonadotropic Hypogonadism in the Male (Primary Hypogonadism) 1924 5 77.2 Hypogonadotropic Hypogonadism in the Male (Secondary Hypogonadism) 1927 Chapter 564 Hormones and Peptides of Calcium Homeostasis and Bone Metabolism 1890 Chapter 578 Pseudoprecocity Resulting from Tumors of theTestes 1930 Chapter 565 Chapter 580 Hypofunction of the Ovaries 1931 580.1 Hypergonadotropic Hypogonadism in the Female • (Primary Hypogonadism) 1931 , 580.2 Hypogonadotropic Hypogonadism in the Female (Secondary Hypogonadism) 1935 Hypoparathyroidism 1892 Chapter 566 Pseudohypoparathyroidism (Albright Hereditary Osteodystrophy) 1894 Chapter 567 Hyperparathyroidism 1895 Chapter 579 Gynecomastia 1930 xliv Contents Chapter 581 Pseudoprecocity Due to Lesions of the Ovary 1937 Chapter 582 Intersex 1939 582.1 46,XX Intersex (46,XX with Virilization) 1940 582.2 46,XYIntersex (46,XY with Undervirilization) 1940 582.3 True Gonadal Intersex 1945 Section 6: Diabetes Mellitus in Children 1947 Ramin Alemzadeh and David T. Wyatt Chapter 583 Diabetes Mellitus 1947 583.1 Introduction and Classification 1947 583.2 Type 1 Diabetes Mellitus (Immune Mediated) 1948 583.3 Type 2 Diabetes Mellitus 1967 583.4 Other Specific Types of Diabetes 1968 Part XXVI The Nervous System Chapter 584 Neurologic Evaluation 1973 1973 Chapter 585 Congenital Anomalies of the Central Nervous System 1983 Michael V. Johnston and Stephen Kinsman 585.1 Neural Tube Defects (Dysraphism) 1983 585.2 Spina Bifida Occulta 1983 585.3 Meningocele 1983 585.4 Myelomeningocele 1984 585.5 Encephalocele 1985 585.6 Anencephaly 1986 585.7 Disorders of Neuronal Migration 1986 585.8 Agenesis of the Corpus Callosum 1987 585.9 Agenesis of the Cranial Nerves 1988 585.10 Microcephaly 1988 585.11 Hydrocephalus 1989 585.12 Craniosynostosis 1992 Chapter 586 Seizures in Childhood 1993 Michael V. Johnston 586.1 Febrile Seizures 1994 586.2 Unprovoked Seizures 1994 586.3 Partial Seizures 1995 586.4 Generalized Seizures 1997 586.5 Mechanisms of Seizures 1999 586.6 Treatment of Epilepsy 1999 586.7 Neonatal Seizures 2005 586.8 Status Epilepticus 2007 Conditions that Mimic Seizures 2025 Chapter 592 Neurodegenerative Disorders of Childhood 2029 Michael V. Johnston 592.1 Sphingolipidoses 2029 592.2 Neuronal Ceroid Lipofuscinoses 2032 592.3 Adrenoleukodystrophy 2032 592.4 Sialidosis 2033 592.5 Miscellaneous Disorders 2033 Chapter 593 Acute Stroke Syndromes 2035 Michael V. Johnston 593.1 Arterial Thrombosis/Embolism 2035 593.2 Venous Thrombosis 2036 593.3 Intracranial Hemorrhage 2036 593.4 Differential Diagnosis of Strokelike Events 2037 Chapter 594 Central Nervous System Infections 2038 Charles G. Prober 594.1 Acute Bacterial Meningitis Beyond the Neonatal Period 2038 594.2 Viral Meningoencephalitis 2044 594.3 Eosinophilic Meningitis 2046 Brain Abscess 2047 Robert H. A. Haslam Chapter 596 Pseudotumor Cerebri 2048 Robert H. A. Haslam 2009 2015 Chapter 589 Neurocutaneous Syndromes Robert H. A. Haslam 589.1 Neurofibromatosis 2015 589.2 Tuberous Sclerosis 2017 Chapter 590 Movement Disorders 2019 Michael V. Johnston 590.1 Ataxias 2019 590.2 Chorea, Athetosis, Tremor 2020 590.3 Dystonia 2022 590.4 Tics 2023 Chapter 595 Michael V. Johnston Chapter 588 Headaches 2012 Robert H. A. Haslam 588.1 Migraine 2012 588.2 Organic Headaches 2014 588.3 Tension or Stress Headaches Sturge-Weber Disease 2017 von Hippel-Lindau Disease 2019 Linear Nevus Syndrome 2019 PHACE Syndrome 2019 Chapter 591 Encephalopathies 2023 Michael V. Johnston 591.1 Cerebral Palsy 2024 591.2 MitochondrialEncephalomyopathies 591.3 Other Encephalopathies 2027 Robert H. A. Haslam Chapter 587 589.3 589.4 589.5 589.6 2015 Chapter 597 Spinal Cord Disorders 2049 Robert H. A. Haslam 597.1 Spinal Cord Tumors 2049 597.2 Spinal Cord Trauma 2050 597.3 Tethered Cord 2050 597.4 Diastematomyelia 2051 597.5 Syringomyelia 2051 597.6 Transverse Myelitis 2051 597.7 Arteriovenous Malformation 2052 Part XXVII Neuromuscular Disorders 2053 Harvey B. Sarnat Chapter 598 Evaluation and Investigation 2053 Contents Chapter 599 Developmental Disorders of Muscle 2055 599.1 Myotubular Myopathy 2056 599.2 Congenital Muscle Fiber-Type Disproportion (CMFTD) 2057 599.3 Nemaline Rod Myopathy 2058 599.4 Central Core Disease 2059 599.5 Brain Malformations and Muscle Development 2059 599.6 Amyoplasia 2059 599.7 Muscular Dysgenesis (Proteus Syndrome Myopathy) 2059 599.8 Benign Congenital Hypotonia 2059 599.9 Arthrogryposis 2059 Chapter 600 Muscular Dystrophies 2060 600.1 Duchenne and Becker Muscular Dystrophies -2060 600.2 Emery-Dreifuss Muscular Dystrophy 2064 600.3 Myotonic Muscular Dystrophy 2064 600.4 Limb-Girdle Muscular Dystrophy 2066 600.5 Facioscapulohumeral Muscular Dystrophy • 2067 600.6 Congenital Muscular Dystrophy 2067 Chapter 601 Endocrine Myopathies 2069 PartXXVIII Disorders of the Eye 2083 Scott E. Olitsky and Leonard B. Nelson Chapter 609 Growth and Development Chapter 610 Examination of the Eye 2083 2084 Chapter 611 Abnormalities of Refraction and Accommodation 2086 Chapter 612 Disorders of Vision 2087 Chapter 613 Abnormalities of Pupil and Iris Chapter 615 Abnormalities of the Lids 2097 Chapter 616 Disorders of the Lacrimal System 2099 Chapter 617 Disorders of the Conjunctiva 2099 Chapter 618 Abnormalities of the Cornea Chapter 619 Abnormalities of the Lens 2103 2105 Chapter 620 Disorders of the UvealTract Chapter 603 Disorders of Neuromuscular Transmission and of Motor Neurons 2072 603.1 Myasthenia Gravis 2072 603.2 Spinal Muscular Atrophies 2075 603.3 Other Motor Neuron Diseases 2076 Chapter 623 Childhood Glaucoma 2122 Chapter 624 Orbital Abnormalities 2123 Chapter 605 Toxic Neuropathies 606.2 Other Autonomic Neuropathies Chapter 607 Guillain-Barre Syndrome Chapter 608 Bell Palsy 2081 Chapter 621 Vitreous 2111 Disorders of the Retina and 2113 Chapter 622 Abnormalities of the Optic Nerve 2119 Chapter 625 Injuries to the Eye Part XXIX The Ear 2124 2127 Chapter 626 Clinical Manifestations 2127 Joseph Haddad Jr. Chapter 627 Hearing Loss 2129 Joseph Haddad Jr. Chapter 628 Congenital Malformations 2135 Joseph Haddad Jr. Chapter 629 Diseases of the External Ear 2136 Joseph Haddad Jr. Chapter 630 Otitis Media 2138 Jack L. Paradise 2078 Chapter 606 Autonomic Neuropathies 606.1 Familial Dysautonomia 2079 2089 Chapter 614 Disorders of Eye Movement and Alignment 2092 Chapter 602 Metabolic Myopathies 2070 602.1 Periodic Paralyses (Potassium-Related) 2070 602.2 Malignant Hyperthermia -2070 602.3 Glycogenoses 2070 602.4 Mitochondrial Myopathies 2071 602.5 Lipid Myopathies 2072 602.6 Vitamin E Deficiency Myopathy 2072 Chapter 604 Hereditary Motor-Sensory Neuropathies 2076 604.1 Peroneal Muscular Atrophy (Charcot-Marie-Tooth Disease; HMSN Type I) 2076 604.2 Peroneal Muscular Atrophy (Axonal Type) 2077 604.3 Dejerine-Sottas Disease (HMSN Type III) 2077 604.4 Roussy-Levy Syndrome 2077 604.5 Refsum Disease 2077 604.6 Fabry Disease 2077 604.7 Giant Axonal Neuropathy 2078 604.8 Congenital Hypomyelinating Neuropathy 2078 604.9 Tomaculous Neuropathy 2078 604.10 Leukodystrophies 2078 xlv 2079 2079 2080 Chapter 631 The Inner Ear and Diseases of the Bony Labyrinth 2149 i Joseph Haddad Jr. \ Chapter 632 Traumatic Injuries of the Ear and Temporal Bone 2150 Joseph Haddad Jr. , xlvi Contents Chapter 633 Tumors of the Ear andTemporal Bone 2151 Part XXXI Bone and Joint Disorders Joseph Haddad Jr. Section 1: Orthopedic Problems 2251 Chapter 662 Growth and Development The Skin 2153 Chapter 663 Evaluation of the Child George H. Thompson Chapter 634 Morphology of the Skin 2153 Chapter 635 Evaluation of the Patient Chapter 664 The Foot and Toes 2154 Cutaneous Manifestations of Systemic Diseases 2155 Chapter 636 Principles of Therapy 2159 Chapter 637 Diseases of the Neonate Chapter 638 Cutaneous Defects 2161 2164 Chapter 639 Ectodermal Dysplasias Chapter 640 Vascular Disorders Chapter 641 Cutaneous Nevi 2166 2167 2172 Chapter 642 Hyperpigmented Lesions 2176 Chapter 643 Hypopigmented Lesions 2178 Chapter 644 Vesiculobullous Disorders Chapter 645 Eczematous Disorders Chapter 646 Photosensitivity 2181 2188 2191 Chapter 647 Diseases of the Epidermis 2195 Chapter 648 Disorders of Keratinization Chapter 649 Diseases of the Dermis 2200 2204 Disorders of Hair Chapter 653 Disorders of the Nails 2215 2219 Chapter 668 The Hip Chapter 656 Cutaneous Fungal Infections Cutaneous Viral Infections 2230 2235 Chapter 658 Arthropod Bites and Infestations 2237 Chapter 661 George H. Thompson 665.1 Normal Developmental Alignment 2261 665.2 Internal Femoral Torsion 2262 665.3 Internal Tibial Torsion 2263 665.4 External Femoral Torsion 2263 665.5 External Tibial Torsion 2264 665.6 Genu Varum (Bowlegs) 2264 665.7 Genu Valgum (Knock-Knees) 2266 665.8 Congenital Angular Deformities of the Tibia and Fibula 2269 2270 2271 George H. Thompson 667.1 Discoid Lateral Meniscus 2272 667.2 Popliteal Cyst 2272 667.3 Osteochondritis Dissecans 2272 667.4 Osgood-Schlatter Disease 2272 667.5 Idiopathic Adolescent Anterior Knee Pain Syndrome 2272 667.6 Patella Subluxation and Dislocation 2273 Chapter 655 Cutaneous Bacterial Infections 2222 2273 George H. Thompson 668.1 Developmental Dysplasia of the Hip 2273 668.2 Transient Monoarticular Synovitis 2276 668.3 Legg-Calve-Perthes Disease 2276 668.4 Slipped Capital Femoral Epiphysis 2278 i Chapter 669 The Spine 2242 Chapter 660 Tumors of the Skin 2257 Chapter 665 Torsional and Angular Deformities 2261 Chapter 667 The Knee Chapter 654 Disorders of the Mucous Membranes 2220 Chapter 659 Acne 2254 George H. Thompson Chapter 651 Disorders of the Sweat Glands 2213 Chapter 652 2251 George H. Thompson 664.1 Metatarsus Adductus 2255 664.2 Calcaneovalgus Feet 2255 664.3 Talipes Equinovarus (Clubfoot) 2256 664.4 Congenital Vertical Talus 2256 664.5 Hypermobile Pes Planus (Flexible Flatfeet) 664.6 Tarsal Coalition 2257 664.7 CavusFeet 2257 664.8 Osteochondroses 2257 664.9 Puncture Wounds of the Foot 2258 664.10 Toe Deformities 2258 664.11 Painful Foot 2260 664.12 Shoes 2260 Chapter 666 Leg-Length Discrepancy Chapter 650 Diseases of Subcutaneous Tissue 2210 Chapter 657 2251 George H. Thompson Gary L. Darmstadt and Robert Sidbury 635.1 2251 2246 Nutritional Dermatoses 2248 2280 \ George H. Thompson i 669.1 Idiopathic Scoliosis 2280 » 669.2 Congenital Scoliosis 2282 669.3 Neuromuscular Scoliosis, Genetic Syndromes, and Compensatory Scoliosis 2283 xlvii Contents 669.4 669.5 669.6 669.7 669.8 669.9 Kyphosis (Round-Back) 2284 Back Pain in Children 2285 Spondylolysis and Spondylolisthesis 2285 Disk Space Infection 2286 Intervertebral Disk Herniation 2287 Tumors 2287 Chapter 670 The Neck 2289 2289 Chapter 672 Arthrogryposis 2314 Chapter 679 Female Athletes: Menstrual Problems and the Risk for Osteopenia 2315 2316 Section 3: The Skeletal Dysplasias 2320 Chapter 682 General Considerations 2320 William A. Horton and Jacqueline T. Hecht Chapter 683 Disorders Involving Cartilage Matrix Proteins 2324 William A. Horton and Jacqueline T. Hecht Chapter 684 Disorders InvolvingTransmembrane Receptors 2328 William A. Horton and Jacqueline T. Hecht 2292 2292 George H. Thompson Chapter 685 Disorders Involving lonTransporter 2330 William A. Horton and Jacqueline T. Hecht Chapter 686 Disorders InvolvingTranscription Factors 2332 2292 Chapter 673 Common Fractures 2294 George H. Thompson 673.1 673.2 673.3 672.4 673.5 673.6 673.7 673.8 673.9 673.10 673.11 Chapter 678 Heat Injuries 2313 Chapter 681 Specific Sports and Associated Injuries 2316 Chapter 671 The Upper Limb 2290 George H. Thompson 671.1 Sprengel Deformity 2290 671.2 Shoulder Dislocation 2290 671.3 Nursemaid's Elbow 2290 671.4 Panner Disease 2291 671.5 Ganglion 2291 671.6 Radial Clubhand 2291 671.7 Polydactyly 2292 671.8 Syndactyly' 2292 671.9 Congenital Trigger Thumb and Finger 672.1 Amyoplasia Head and Neck Injuries Chapter 680 ErgogenicAids 2288 George H. Thompson 670.1 Torticollis 2288 670.2 Klippel-Feil Syndrome 670.3 Atlantoaxial Instability Chapter 677 Pediatric Fracture Patterns 2294 Clavicular Fractures 2295 Proximal Humerus Fractures 2295 Distal Humerus Fractures 2295 Distal Radius and Ulna Fractures 2296 Phalangeal Fractures 2296 Toddler Fracture 2296 Lateral Malleolar Fractures 2296 Metatarsal Fractures 2296 Toe Phalangeal Fractures 2296 Operative Treatment 2297 William A. Horton and Jacqueline T. Hecht Chapter 687 Disorders Involving Defective Bone Resorption 2333 William A. Horton and Jacqueline T. Hecht Chapter 688 Disorders for Which Defects Are Poorly Understood or Unknown 2334 William A. Horton and Jacqueline T. Hecht Chapter 689 Osteogenesis Imperfecta 2336 John C. Marini Chapter 690 Marfan Syndrome 2338 Luther K. Robinson Chapter 674 Osteomyelitis and Suppurative Arthritis 2297 Richard M. Lampe Section 2: Sports Medicine Section 4: Metabolic Bone Disease 2341 Russell W. Chesney 2302 Albert C. Hergenroed'er and Joseph N. Chorley Chapter 691 Bone Structure, Growth, and Hormonal Regulation 2341 Chapter 675 Epidemiology and Prevention of Injuries 2302 Chapter 692 Primary Chondrodystrophy (Metaphyseal Dysplasia) 2343 Chapter 676 Management of Musculoskeletal Injury 2306 676.1 Growth Plate Injuries 2307 676.2 Shoulder Injuries 2308 676.3 Elbow Injuries 2308 676.4 Back Injuries 2309 676.5 Hip and Pelvis Injuries 2310 676.6 Knee Injuries 2310 676.7 Shin Splints and Stress Fractures of the Lower Leg 2312 676.8 Ankle Injuries 2312 676.9 Foot Injuries 2313 Chapter 693 Idiopathic Hypercalcemia Chapter 694 Hypophosphatasia Chapter 695 Hyperphosphatasia 2343 2344 2344 Chapter 696 Familial Hypophosphatemia (Vitamin D-Resistant Rickets, X-Linked Hypophosphatemia) 2345 Chapter 697 Vitamin D-Dependent Rickets (Pseudovitamin D Deficiency, Hypocalcemic Vitamin D-Resistant Rickets) 2346 * [ \ xlviii Contents Chapter 698 Oncogenous Rickets (Primary Hypophosphatemic Rickets Associated with Tumor) 2346 Chapter 699 Osteoporosis 2347 Chapter 705 Nonbacterial Food Poisoning DeniseA. Salerno and Stephen C. Aronoff 705.1 Mushroom Poisoning 2375 705.2 Solanine Poisoning 2376 705.3 Seafood Poisoning 2377 Chapter 706 2375 Biologic and ChemicalTerrorism 2378 Theodore J. Cieslak and Fred M. Henretig Part XXXII Environmental Health Hazards 2349 Chapter 700 Pediatric Radiation Injuries Chapter 707 Animal and Human Bites 2385 Charles M. Ginsburg Chapter 708 Envenomations 2349 2387 Steve Holve Fred A. Mettler, Jr. and Madelyn M. Stazzone Chapter 701 Chemical Pollutants 2353 Philip J. Landrigan and Joel A. Forman Chapter 702 Heavy Metal Intoxication Laboratory Medicine, Drug Therapy, and Reference Tables 2393 2355 Collin S. Goto Chapter 703 Lead Poisoning 2358 Morri Markowitz Chapter 704 Poisonings: Drugs, Chemicals, and Plants 2362 George C. Rodgers, Jr. and Nancy J. Matyunas 704.1 Epidemiology and Approach to Management 2362 704.2 Acetaminophen 2366 704.3 Salicylates 2367 704.4 Ibuprofen 2368 704.5 Antidepressants 2368 704.6 Clonidine 2369 704.7 Calcium Channel Blockers 2370 704.8 Iron 2370 704.9 Caustics 2371 704.10 Methanol and Ethylene Glycol 2371 704.11 Hydrocarbons 2372 704.12 Cholinesterase-Inhibiting Insecticides 2373 704.13 Toxic Gases 2374 704.14 Plants 2374 Chapter 709 Children Laboratory Testing in Infants and 2393 John F. Nicholson and Michael A. Pesce Chapter 710 Reference Ranges for Laboratory Tests and Procedures 2396 John F. Nicholson and Michael A. Pesce Chapter 711 Principles of DrugTherapy Michael D. Reed and Peter Gal Chapter 712 Medications 2432 Peter Gal and Michael D. Reed Chapter 713 Herbal Medicines Kathi J. Kemper and Paula Gardiner Index 2507 2502 2427