Nelson TEXTBOOK OF PEDIATRICS Richard E. Behrman, MD
Nelson TEXTBOOK OF PEDIATRICS Richard E. Behrman, MD
Nelson TEXTBOOK OF PEDIATRICS Richard E. Behrman, MD
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<strong>Nelson</strong> <strong>TEXTBOOK</strong> <strong>OF</strong><br />
<strong>PEDIATRICS</strong><br />
<strong>Richard</strong> E. <strong>Behrman</strong>, <strong>MD</strong><br />
Executive Chair<br />
Federation of Pediatric Organizations, Inc.<br />
Pediatric Education Steering Committee<br />
Palo Alto, California<br />
and<br />
Clinical Professor of Pediatrics<br />
Stanford University School of Medicine<br />
University of California, San Francisco, School of Medicine<br />
Robert M. Kliegman, <strong>MD</strong><br />
Professor and Chair<br />
Department of Pediatrics<br />
Medical College of Wisconsin<br />
Pediatrician in Chief<br />
Pam and Les Muma Chair in Pediatrics<br />
Children's Hospital of Wisconsin<br />
Milwaukee, Wisconsin<br />
Hal B. Jenson, <strong>MD</strong><br />
Professor and Chair, Department of Pediatrics<br />
Director, Center for Pediatric Research<br />
Eastern Virginia Medical School and Children's Hospital of The King's Daughters<br />
Senior Vice President for Academic Affairs<br />
Children's Hospital of The King's Daughters<br />
Norfolk, Virginia<br />
17th Edition<br />
SAUNDERS<br />
An Imprint of Elsevier Science
Contents<br />
«Part I The Field of Pediatrics 1<br />
Chapter 1 Overview of Pediatrics 1<br />
<strong>Richard</strong> E. <strong>Behrman</strong><br />
Chapter 2 Ethics in Pediatric Care 6<br />
Robert M. <strong>Nelson</strong> and Norman Fast<br />
Chapter 3 Cultural Issues in Pediatric Care 10<br />
JillE.Korbin<br />
Chapter 4 Child Health in the Developing World 12<br />
Kristine Torjesen and Karen Olness<br />
Chapter 5 Preventive Pediatrics 14<br />
Theodore C. Sectish<br />
Chapter 6 The Well Child<br />
Paul L. McCarthy<br />
20<br />
Part II Growth and Development 23<br />
Robert D. Needlman<br />
Chapter 7 Overview and Assessment of<br />
Variability 23<br />
Chapter 8 Fetal Growth and Development 27<br />
Chapter 9 The Newborn 30<br />
Chapter 10 The First Year 31<br />
Chapter 11 TheSecondYear 38<br />
Chapter 12 PreschoolYears 44<br />
Chapter 13 Middle Childhood 51<br />
Chapter 14 Adolescence 53<br />
Chapter 15 Assessment of Growth 58<br />
Chapter 16 Developmental Assessment 62<br />
Part-Ill Psychologic Disorders 67<br />
Chapter 17 Assessment and Interviewing 67<br />
17.1 The Clinical Interview (History) 67<br />
Neil W.'Boris, Marc A. Forman, and Jorge H. Daruna<br />
17.2 Psychosocial Problems 70<br />
<strong>Richard</strong> Dalton and Marc A. Forman<br />
Chapter 18 Psychiatric Considerations of Central<br />
Nervous System Injury 71<br />
Jorge H. Daruna, Marc A. Forman, and Neil W. Boris<br />
Chapter 19 Psychosomatic Illness 72<br />
Alessandra N. Kazura, Neil W. Boris, and <strong>Richard</strong> Dalton<br />
Chapter 20 Vegetative Disorders<br />
20.1 Rumination Disorder 73<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
20.2 Pica 73<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
20.3 Enuresis (Bed-wetting) 74<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
20.4 Encopresis 75<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
20.5 Sleep Disorders 75<br />
Judith A. Owens<br />
Chapter 21 Habit Disorders<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
80<br />
73<br />
Chapter 22 Anxiety Disorders 81<br />
Brian Stafford, Neil W. Boris, and <strong>Richard</strong> Dalton<br />
Chapter 23 Mood Disorders 84<br />
Neil W. Boris, <strong>Richard</strong> Dalton, and Marc A. Forman<br />
23.1 Major Depression 84<br />
23.2 Dysthymic Disorder 85<br />
23.3 Bipolar Disorder 85<br />
Chapter 24 Suicide and Attempted Suicide<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
Chapter 25 Disruptive Behavioral Disorders<br />
Neil W. Boris and <strong>Richard</strong> Dalton<br />
Chapter 26 Sexual Behavior and Its Variations<br />
<strong>Richard</strong> Dalton<br />
26.1 Gender Identity Disorder 91<br />
26.2 Homosexuality 91<br />
Chapter 27 Pervasive Developmental Disorders and<br />
Childhood Psychosis 93<br />
<strong>Richard</strong> Dalton, Marc A. Forman, and Neil W. Boris<br />
27.1 Autistic Disorder 93<br />
27.2 Asperger Disorder 94<br />
27.3 Childhood Disintegrative Disorder 94<br />
27.4 Rett Disorder 94<br />
27.5 Childhood Schizophrenia 94<br />
Chapter 28 Psychologic Treatment of Children and<br />
Adolescents 95<br />
<strong>Richard</strong> Dalton<br />
xxiii<br />
86<br />
88<br />
90
XXIV Contents<br />
• 28.1 Illness and Death 95<br />
28.2 Psychopharmacology 96<br />
28.3 Psychotherapy 100<br />
28.4 Psychiatric Hospitalization 100<br />
Chapter 29 Neurodevelopmental Dysfunction in the<br />
School-Aged Child 100<br />
29.1 Patterns of Development and Function 100<br />
Desmond P. Kelly<br />
29.2 Attention-Defidt/Hyperactivity Disorder 107<br />
Mark D. Simms<br />
29.3 Specific Reading Disability (Dyslexia) 110<br />
G. ReidLyon, Sally E. Shaywitz, and Bennett A. Shaywitz<br />
Part IV Social Issues 113<br />
Chapter 30 Adoption 113<br />
Mark D. Simms andMadelyn Freundlich<br />
Chapter 31 Foster Care 114<br />
Mark D. Simms and Madelyn Freundlich<br />
Chapter 32 Child Care<br />
Paul H. Dworkin<br />
115<br />
Chapter 33 Separation, Loss, and<br />
Bereavement 116<br />
33.1 Separation and Loss 116<br />
<strong>Richard</strong> Dalton<br />
33.2 Grief and Bereavement 117<br />
Linda Sayler Gudas and Gerald P. Koocher<br />
Chapter 34 Impact of Violence on Children<br />
Marilyn Augustyn and Barry Zuckerman<br />
Chapter 35 Abuse and Neglect of Children<br />
Charles F. Johnson<br />
35.1 Sexual Abuse 126<br />
35.2 Nonorganic Failure to Thrive 129<br />
35.3 Munchausen Syndrome by Proxy (MSBP)<br />
Part V Children with Special Health<br />
Needs 133<br />
Chapter 36 Failure toThrive<br />
Howard Bauchner<br />
133<br />
120<br />
121<br />
130<br />
Chapter 37 Developmental Disabilities and Chronic<br />
Illness 134<br />
James M. Perrin<br />
37.1 Chronic Illness in Childhood 135<br />
James M. Perrin<br />
37.2 Mental Retardation 138<br />
Bruce K. Shapiro and Mark L. Batshaw<br />
Chapter 38 Pediatric Palliative Care:The Care of<br />
Children with Life-Limiting Illness 143<br />
Stephen Liben<br />
Chapter 39 Children at Special Risk 148<br />
<strong>Richard</strong> E. <strong>Behrman</strong><br />
Part VI Nutrition 153<br />
Chapter 40 Nutritional Requirements 153<br />
William C. Heird<br />
Chapter 41 The Feeding of Infants and<br />
Children 157<br />
William C. Heird<br />
Chapter 42 Food Insecurity, Hunger, and<br />
Undernutrition 167<br />
William C. Heird<br />
Chapter 43 Obesity<br />
Patricia A. Donohoue<br />
173<br />
Chapter 44 Vitamin Deficiencies and Excesses<br />
William C. Heird<br />
177<br />
. Part VII Pathophysiology of Body Fluids and<br />
; Fluid Therapy 191<br />
Larry A. Greenbaum<br />
Chapter 45 Electrolyte and Acid-Base<br />
Disorders 191<br />
45.1 Composition of Body Fluids 191<br />
45.2 Regulation of Osmolality and Volume 193<br />
45.3 Sodium 196<br />
45.4 Potassium 202<br />
45.5 Calcium 208<br />
45.6 Magnesium 217<br />
45.7 Phosphorus 219<br />
45.8 Acid-Base Balance 223<br />
Chapter 46 Maintenance and Replacement<br />
Therapy 242<br />
Chapter 47 DeficitTherapy 245<br />
Chapter 48 Fluids and Electrolyte Treatment of<br />
Specific Disorders 249<br />
48.1 Acute Diarrhea and Oral Rehydration 249<br />
48.2 Diarrhea in Chronically Malnourished Children 250<br />
48.3 Pyloric Stenosis 251<br />
48.4 Perioperative Fluids 251<br />
PartVili The Acutely III Child 253<br />
Chapter 49 Evaluation of the Sick Child in the Office<br />
and Clinic 253<br />
Paul L. McCarthy<br />
Chapter 50 Injury Control 256<br />
Frederick P. Rivara and David Grossman<br />
Chapter 51 Emergency Medical Services for<br />
Children 263<br />
M. Denise Dowd and Frederick P. Rivara
Chapter 52 Pediatric Critical Care:<br />
An Overview 268<br />
Lorry R. Frankel<br />
Chapter 53 InterfacilityTransfer of the Critically III<br />
Infant and Child 269<br />
Lorry R. Frankel<br />
Chapter 54 Effective Communication with Families<br />
inthePICU 270<br />
Lawrence H. Mathers and Lorry R. Frankel<br />
Chapter 55 MonitoringTechniques for the Critically<br />
III Infant and Child 272<br />
Lorry R. Frankel<br />
Chapter 56 Scoring Systems and Predictors of<br />
Mortality 277<br />
Joseph V. DiCarlo and Lorry R. Frankel<br />
Chapter 57 Stabilization of the Critically III<br />
Child 279<br />
57.1 Pediatric Emergencies and Resuscitation 279<br />
Lawrence H. Mathers and Lorry R. Frankel<br />
57.2 Shock 296<br />
Lorry R. Frankel and Lawrence H.Mathers<br />
57.3 Respiratory Distress and Failure 301<br />
Lorry R. Frankel<br />
57.4 Mechanical Ventilation 303<br />
Lorry R. Frankel<br />
57.5 Renal Stabilization 306<br />
Joseph V. DiCarlo<br />
57.6 Nutritional Stabilization 307<br />
Joseph V. DiCarlo<br />
57.7 Neurologic Stabilization 308<br />
Joseph V. DiCarlo and Lorry R. Frankel ,<br />
57.8 Acute Care of the Multiple Trauma Victim 312<br />
Peter S. Dayan and Bruce L. Klein<br />
Chapter 58 Acute (Adult) Respiratory Distress<br />
Syndrome (ARDS) 316<br />
Lorry R. Frankel and Joseph V. DiCarlo<br />
Chapter 59 Continuous Hemofiltration 317<br />
Joseph V. DiCarlo<br />
Chapter 60 Transplantation Issues in<br />
thePICU 318<br />
Lawrence H. Mathers and Lorry R. Frankel<br />
Chapter 61 Drowning and Near-Drowning 321<br />
Harry J. Kallas<br />
Chapter 62 Burn Injuries 330<br />
Alia Y. Antoon and Mary K. Donovan<br />
Chapter 63 Cold Injuries 338<br />
Alia Y. Antoon and Mary K. Donovan<br />
Chapter 64 Withdrawal or Withholding of Life<br />
Support, Brain Death, and Organ<br />
Procurement 340<br />
Lorry R. Frankel and Lawrence H. Mathers<br />
Chapter 65 Anesthesia and Perioperative<br />
Care 342<br />
Randall C. Wetzel<br />
Chapter 66 Pediatric Pain Management 358<br />
Brenda Bursch and Lonnie K. Zeltzer<br />
Part IX Human Genetics 367<br />
Chapter 67 The Molecular Basis of Genetic<br />
Disorders 367<br />
H. Eugene Hoyme<br />
Chapter 68 Molecular Diagnosis of Genetic<br />
Diseases 371<br />
H. Eugene Hoyme<br />
Chapter 69 Patterns of Inheritance<br />
H. Eugene Hoyme<br />
376<br />
Chapter 70 Chromosomal Clinical Abnormalities<br />
Judith G. Hall<br />
Chapter 71 GeneTherapy<br />
Mark A. Kay<br />
391<br />
Chapter 72 Genetic Counseling<br />
Judith G. Hall<br />
395<br />
PartX Metabolic Diseases 397<br />
Chapter 73 An Approach to Inborn Errors of<br />
Metabolism 397<br />
Iraj Rezvani<br />
Contents XXV<br />
Chapter 74 Defects in Metabolism of<br />
Amino Acids 398<br />
74.1 Phenylalanine 398<br />
Iraj Rezvani<br />
74.2 Tyrosine 402<br />
Iraj Rezvani „<br />
74.3 Methionine 405<br />
Iraj Rezvani and David S. Rosenblatt<br />
74.4 Cysteine/Cystine 407<br />
Iraj Rezvani<br />
74.5 Tryptophan 408<br />
Iraj Rezvani<br />
74.6 Valine, Leucine, Isoleucine, and Related Organic<br />
Acidemias 409<br />
Iraj Rezvani and David S. Rosenblatt<br />
74.7 Glycine 418<br />
Iraj Rezvani<br />
74.8 Serine 421<br />
Iraj Rezvani<br />
74.9 Proline and Hydroxyproline 421<br />
Iraj Rezvani<br />
74.10 GlutamicAdd 422<br />
Iraj Rezvani<br />
74.11 Urea Cycle and Hyperammonemia (Arginine,<br />
Citrulline, Omithine) 424<br />
Iraj Rezvani<br />
74.12 Histidine 429<br />
Iraj Rezvani<br />
74.13 Lysine 429<br />
Iraj Rezvani<br />
74.14 Aspartic Acid (Canavan Disease) 430<br />
Reuben K. Matalon<br />
382
XXVi Contents<br />
Chapter 75 Defects in Metabolism of Lipids 433<br />
75.1 Disorders of Mitochondrial Fatty Acid<br />
Oxidation 433<br />
Charles P. Venditti and Charles A. Stanley<br />
75.2 Disorders of Very Long Chain Fatty Acids 437<br />
Hugo W. Moser<br />
75.3 Disorders of Lipoprotein Metabolism and<br />
Transport 445<br />
Andrew M. Tershakovec and Daniel J. Rader<br />
75.4 Lipidoses 459<br />
Margaret M. McGovern and Robert J. Desnick<br />
75.5 Mucolipidoses 467<br />
Margaret M. McGovern and Robert J. Desnick<br />
Chapter 76 Defects in Metabolism of<br />
Carbohydrates 468<br />
76.1 Glycogen Storage Diseases 469<br />
Yuan-Tsong Chen<br />
76.2 Defects in Galactose Metabolism 475<br />
Yuan-Tsong Chen<br />
76.3 Defects in Fructose Metabolism 476<br />
Yuan-Tsong Chen<br />
Id A Defects in Intermediary Carbohydrate Metabolism<br />
Associated with Lactic Acidosis 477<br />
Yuan-Tsong Chen<br />
76.5 Deficiency of Xylulose Dehydrogenase (Essential<br />
Benign Pentosuria) 480<br />
Yuan-Tsong Chen<br />
76.6 Disorders of Glycoprotein Degradation and<br />
Structure 480<br />
Margaret M. McGovern and Robert J. Desnick<br />
Chapter 77 Mucopolysaccharidoses<br />
Robert M. Kliegman and Joseph L. Muenzer<br />
482<br />
Chapter 78 Disorders of Purine and Pyrimidine<br />
Metabolism 486<br />
James C. Harris<br />
Chapter 79 Progeria<br />
W. Ted Brown<br />
494<br />
Chapter 80 The Porphyrias<br />
Shigeru Sassa<br />
Chapter 81 Hypoglycemia<br />
Mark A. Sperling<br />
495<br />
505<br />
Part XI The Fetus and the Neonatal Infant 519<br />
Section 1: Noninfectious Disorders 519<br />
Chapter 82 Overview of Mortality and<br />
Morbidity 519<br />
Barbara J. Stoll and Robert M. Kliegman<br />
Chapter 83 The Newborn Infant 523<br />
Barbara J. Stoll and Robert M. Kliegman<br />
83.1 History in Neonatal Pediatrics 523<br />
83.2 Physical Examination of the Newborn Infant<br />
83.3 Routine Delivery Room Care 527<br />
83.4 Nursery Care 529<br />
83.5 Parent-Infant Bonding 529<br />
524<br />
Chapter 84 High-Risk Pregnancies<br />
Barbara J. Stoll and Robert M. Kliegman<br />
532<br />
Chapter 85 The Fetus 536<br />
Barbara J. Stoll and Robert M. Kliegman<br />
85.1 Fetal Growth and Maturity 536<br />
85.2 Fetal Distress 536<br />
85.3 Maternal Disease and the Fetus 539<br />
85.4 Maternal Medication and Toxin Exposure and the<br />
Fetus 540<br />
85.5 Teratogens 542<br />
85.6 Radiation 542<br />
85.7 Intrauterine Diagnosis of Fetal Disease 542<br />
85.8 Treatment and Prevention of Fetal Disease 545<br />
Chapter 86 The High-Risk Infant 547<br />
Barbara J. Stoll and Robert M. Kliegman<br />
86.1 Multiple Pregnancies 548<br />
86.2 Prematurity and Intrauterine Growth<br />
Retardation 550<br />
86.3 Post-Term Infants 558<br />
86.4 Large for Gestational Age 558<br />
86.5 Infant Transport 559<br />
Chapter 87 Clinical Manifestations of Diseases<br />
in the Newborn Period 559<br />
Barbara J. Stoll and Robert M. Kliegman<br />
Chapter 88 Nervous System Disorders 561<br />
Barbara J. Stoll and Robert M. Kliegman<br />
88.1 The Cranium 562<br />
88.2 Intracranial-Intraventricular Hemorrhage and<br />
Periventricular Leukomalacia 562<br />
88.3 Spine and Spinal Cord 564<br />
88.4 Peripheral Nerve Injuries 565<br />
88.5 Hypoxia-Ischemia 566<br />
88.6 Intrauterine Infection 568<br />
Chapter 89 Delivery Room Emergencies<br />
Barbara J. Stoll and Robert M. Kliegman<br />
569<br />
Chapter 90 Respiratory Tract Disorders 573<br />
Barbara J. Stoll and Robert M. Kliegman<br />
90.1 Transition to Pulmonary Respiration 573<br />
90.2 Apnea 573<br />
90.3 Hyaline Membrane Disease (Respiratory Distress<br />
Syndrome) 575<br />
90.4 Transient Tachypnea of the Newborn 583<br />
90.5 Aspiration of Foreign Material (Fetal Aspiration<br />
Syndrome, Aspiration Pneumonia) 583<br />
90.6 Meconium Aspiration 583<br />
90.7 Persistent Pulmonary Hypertension of<br />
the Newborn (Persistent Fetal<br />
Circulation) 584<br />
90.8 Extrapulmonary Extravasation of Air<br />
(Pneumothorax, Pneumomediastinum,<br />
Pulmonary Interstitial Emphysema) 586<br />
90.9 Pulmonary Hemorrhage 587<br />
Chapter 91 Digestive System Disorders<br />
Barbara J. Stoll and Robert M. Kliegman<br />
91.1 Meconium Ileus in Cystic Fibrosis 589<br />
91.2 Neonatal Necrotizing Enterocolitis<br />
(NEC) 590<br />
588
91.3 Jaundice and Hyperbilirubinemia in the<br />
Newborn ,592<br />
91.4 Kernicterus 596<br />
Chapter 92 Blood Disorders 599<br />
Barbara J. Stoll and Robert M. Kliegman<br />
92.1 Anemia in the Newborn Infant 599<br />
92.2 Hemolytic Disease of theNewborn<br />
(Erythroblastosis Fetalis) 601<br />
92.3 Plethora in the Newborn Infant (Polycythemia)<br />
92.4 Hemorrhage in the Newborn Infant 606<br />
Chapter 93 Genitourinary System<br />
Barbara J. Stoll and Robert M. Kliegman<br />
Chapter 94 The Umbilicus 608<br />
Barbara J. Stoll and Robert M. Kliegman<br />
Chapter 95 Metabolic Disturbances<br />
Barbara J. Stoll and Robert M. Kliegman<br />
Chapter 96 The Endocrine System<br />
Barbara J. Stoll and Robert M. Kliegman<br />
96.1 Infants of Diabetic Mothers 613<br />
96.2 Hypoglycemia 614<br />
Chapter 97 Dysmorphology<br />
Kenneth Lyons Jones<br />
616<br />
608<br />
Section 2: Infections of the Neonatal Infant<br />
Barbara J. Stoll<br />
Chapter 98 Pathogenesis and<br />
Epidemiology 623<br />
609<br />
613<br />
623<br />
98.1 Modes of Transmission and Pathogenesis 623<br />
98.2 Immunity 625<br />
98.3 Etiology of Fetal and Neonatal Infection 626<br />
98.4 Epidemiology of Early- and Late-Onset Neonatal<br />
Infections 627<br />
98.5 Clinical Manifestations of Transplacental<br />
Intrauterine Infections 630<br />
98.6 Diagnosis 633<br />
98.7 Treatment 636<br />
98.8 Complications and Prognosis 638<br />
98.9 Prevention 638<br />
Special Health Problems During<br />
Adolescence 64i<br />
Chapter 99 The Epidemiology of Adolescent<br />
Health Problems 641<br />
Renee R. Jenkins<br />
Chapter 100 Delivery of Health Care to<br />
Adolescents 643<br />
Renee R. Jenkins<br />
100.1 Legal Issues 644<br />
100.2 Screening Procedures 645<br />
100.3 Health Enhancement 646<br />
Chapter 101 Depression<br />
Renee R. Jenkins<br />
646<br />
606<br />
Chapter 102 Suicide<br />
Renee R. Jenkins<br />
648<br />
Chapter 103 Violent Behavior<br />
Renee R. Jenkins<br />
649<br />
Chapter 104 Anorexia Nervosa and<br />
Bulimia 652<br />
IrisF.Litt<br />
Chapter 105 Substance Abuse<br />
Renee R. Jenkins<br />
105.1 Alcohol 657<br />
105.2 Marijuana 657<br />
105.3 Tobacco 658<br />
105.4 Volatile Substances 658<br />
105.5 Hallucinogens 659<br />
105.6 Cocaine 660<br />
105.7 Amphetamines 660<br />
105.8 Opiates 660<br />
105.9 Anabolic Steroids 661<br />
Chapter 106 The Breast<br />
Renee R. Jenkins<br />
662<br />
653<br />
Chapter 107 Menstrual Problems 663<br />
Renee R. Jenkins<br />
107.1 Amenorrhea 664<br />
107.2 Abnormal Uterine Bleeding 665<br />
107.3 Dysmenorrhea 666<br />
107.4 Premenstrual Syndrome 667<br />
Chapter 108 Contraception 667<br />
Renee R. Jenkins<br />
108.1 Barrier Methods 668<br />
108.2 Spermicides 668<br />
108.3 Combination Methods 668<br />
108.4 Hormonal Methods 668<br />
108.5 Emergency Contraception 671<br />
108.6 Intrauterine Devices 671<br />
Chapter 109 Pregnancy<br />
Renee R. Jenkins<br />
671<br />
Chapter 110 SexuallyTransmitted<br />
Diseases 673<br />
Renee R. Jenkins<br />
Chapter 111 Chronic Fatigue Syndrome<br />
Hal B. Jenson _ ,<br />
Contents XXVII<br />
677<br />
PartXIII The Immunologic System and<br />
Disorders 681<br />
Section 1: Evaluation of the Immune System 681<br />
Rebecca H. Buckley<br />
Chapter 112 Evaluation of Suspected<br />
Immunodeficiency 681<br />
Section 2: The T-, B-, and NK-Cell Systems 683<br />
Rebecca H. Buckley
XXViii Contents<br />
Chapter 113 T Lymphocytes, B Lymphocytes, and<br />
Natural Killer Cells 683<br />
Chapter 114 Primary Defects of Antibody<br />
Production 689<br />
114.1 Treatment of B-Cell Defects 693<br />
Chapter 115 Primary Defects of Cellular<br />
Immunity 693<br />
Chapter 116 Primary Combined Antibody and<br />
Cellular Immunodeficiencies 695<br />
116.1 Severe Combined Immunodeficiency (SCID) 695<br />
116.2 Combined Immunodeficiency (CID) 697<br />
116.3 Treatment of Cellular or Combined<br />
Immunodeficiency 700<br />
Section 3: The Phagocytic System 701<br />
Chapter 117 Neutrophils 701<br />
Laurence A. Boxer<br />
Chapter 118 Monocytesand Macrophages 707<br />
<strong>Richard</strong> B. Johnston, Jr.<br />
Chapter 119 Eosinophils 709<br />
Laurence A. Boxer<br />
Chapter 120 Disorders of Phagocyte Function 710<br />
Laurence A. Boxer<br />
Chapter 121 Leukopenia 717<br />
Laurence A. Boxer<br />
Chapter 122 Leukocytosis 723<br />
Laurence A. Boxer<br />
Section 4: The Complement System 725<br />
<strong>Richard</strong> B. Johnston, Jr.<br />
Chapter 123 The Complement System 725<br />
Chapter 124 Disorders of the Complement<br />
System 728<br />
124.1 Evaluation 728<br />
124.2 Primary Deficiencies of Complement<br />
Components 728<br />
124.3 Deficiencies of Plasma, Membrane, or Serosal<br />
Complement Control Proteins 730<br />
124.4 Secondary Disorders of Complement 730<br />
124.5 Treatment of Complement Disorders 731<br />
Section 5: Hematopoietic Stem Cell<br />
Transplantation 732<br />
Kent A. Robertson<br />
Chapter 125 Clinical Indications 732<br />
Chapter 126 Matching and Rejection 737<br />
Chapter 127 Graft versus Host Disease (GVHD) 738<br />
Chapter 128 Principles of Immunosuppression<br />
740<br />
Chapter 129 Late Effects of Stem Cell<br />
Transplantation 741<br />
Part XIV Allergic Disorders 743<br />
Chapter 130 Allergy and the Immunologic Basis of<br />
Atopic Disease 743<br />
Donald Y. M. Leung<br />
Chapter 131 Diagnosis of Allergic Disease<br />
Dan Atkins and Donald Y. M. Leung<br />
Chapter 132 Principles of Treatment of Allergic<br />
Disease 752<br />
Dan Atkins and Donald Y. M. Leung<br />
Chapter 133 Allergic Rhinitis 759<br />
Henry Milgrom and Donald Y. M. Leung<br />
Chapter 134 Childhood Asthma 760<br />
Andrew H. Liu, Joseph D. Spahn, and Donald Y. M. Leung<br />
Chapter 135 Atopic Dermatitis (Atopic<br />
Eczema) 774<br />
Donald Y. M. Leung<br />
Chapter 136 Urticaria and Angioedema<br />
(Hives) 778<br />
Donald Y. M. Leung<br />
Chapter 137 Anaphylaxis 781<br />
Hugh A. Sampson and Donald Y. M. Leung<br />
Chapter 138 Serum Sickness 782<br />
Scott H. Sicherer and Donald Y. M. Leung .<br />
Chapter 139 Adverse Reactions to Drugs<br />
Mark Boguniewicz<br />
Chapter 140 Insect Allergy 786<br />
Scott H. Sicherer and Donald Y. M. Leung<br />
Chapter 141 Ocular Allergies<br />
Mark Boguniewicz<br />
788<br />
Chapter 142 Adverse Reactions to Foods<br />
Hugh A. Sampson and Donald Y. M. Leung<br />
747<br />
783<br />
789<br />
Rheumatic Diseases of Childhood<br />
(Connective Tissue Diseases,<br />
Collagen Vascular Diseases) 793<br />
Chapter 143 Evaluation of Suspected Rheumatic<br />
Disease 793<br />
Michael L. Miller<br />
Chapter 144 Treatment of Rheumatic<br />
Diseases 795<br />
Daniel J. Lovell, Michael L. Miller, and James T. Cassidy<br />
Chapter 145 Juvenile Rheumatoid Arthritis<br />
Michael L. Miller and James T. Cassidy<br />
Chapter 146 Ankylosing Spondylitis and Other<br />
Spondyloarthropathies 805<br />
Michael L. Miller and Ross E. Petty<br />
799
Chapter 147 Postinfectious Arthritis and Related<br />
Conditions 808<br />
Michael L. Miller and James T. Cassidy<br />
Chapter 148 Systemic Lupus Erythematosus 809<br />
Marisa S. Klein-Gitelman and Michael L. Miller<br />
148.1 Neonatal Lupus , 813<br />
Chapter 149 Juvenile Dermatomyositis 813<br />
Lauren M. Pachman<br />
Chapter 150 Scleroderma 816<br />
Michael L. Miller<br />
Chapter 151 Behqet Disease 819<br />
Abraham Gedalia<br />
Chapter 152 Sjogren Syndrome 820<br />
Abraham Gedalia<br />
Chapter 153 Familial Mediterranean Fever<br />
Abraham Gedalia<br />
Chapter 154 Amyloidosis 822<br />
Abraham Gedalia<br />
Chapter 155 Sarcoidosis 822<br />
Margaret W. Leigh<br />
Chapter 156 Kawasaki Disease 823<br />
Anne H. Rowley and Stanford T. Shulman<br />
Chapter 157 Vasculitis Syndromes ~ 826<br />
Michael L. Miller and Lauren M. Pachman<br />
157.1 Henoch-SchonleinPurpura 826<br />
157.2 Takayasu Arteritis 828<br />
157.3 Polyarteritis Nodosa 829<br />
157.4 Wegener Granulomatosis 830<br />
157.5 Other Vasculitic Syndromes 831<br />
Chapter 158 Musculoskeletal Pain<br />
Syndromes 831<br />
Michael L. Miller<br />
158.1 Reflex Sympathetic Dystrophy<br />
158.2 Erythromelalgia 832<br />
832<br />
821<br />
Chapter 159 Miscellaneous Conditions Associated<br />
with Arthritis 832<br />
Michael L. Miller<br />
Infectious Diseases 835<br />
Section 1: General Considerations 835<br />
Chapter 160 Diagnostic Microbiology<br />
Anita K. M. Zaidi and Donald A. Goldmann<br />
Chapter 161 Fever<br />
Keith R.Powell<br />
839<br />
Chapter 162 FeverWithout a Focus<br />
Keith R. Powell<br />
Chapter 163 Sepsis and Shock<br />
Anne Stormorken and Keith R. Powell<br />
846<br />
841<br />
835<br />
Contents xxix<br />
Chapter 164 Infections in Immunocompromised<br />
Persons 851<br />
Marian G. Michaels and Michael Green<br />
Chapter 165 Infection Associated with Medical<br />
Devices 858<br />
Patricia M. Flynn and Fred F. Barrett<br />
Section 2: Gram-Positive Bacterial Infections 861<br />
Chapter 166 Staphylococcus 861<br />
James K. Todd<br />
166.1 Staphylococcus aureus 861<br />
166.2 Toxic Shock Syndrome 865<br />
166.3 Coagulase-Negative Staphylococci 866<br />
Chapter 167 Streptococcus pneumoniae<br />
(Pneumococcus) 867<br />
Jon S. Abramson and Gary D. Overturf<br />
Chapter 168 Group A Streptococcus 870<br />
Michael A. Gerber<br />
168.1 Rheumatic Fever 874<br />
Chapter 169 Group B Streptococcus 879<br />
Catherine S. Lachenauer and Michael R. Wessels<br />
Chapter 170 Non-GroupAor B<br />
Streptococci 883<br />
Michael A. Gerber<br />
Chapter 171 Enterococcus<br />
David B. Haslam<br />
884<br />
Chapter 172 Diphtheria (Corynebacterium<br />
diphtheriae) 886<br />
Sarah S. Long<br />
Chapter 173 Listeria monocytogenes 890<br />
Robert S. Baltimore<br />
Chapter 174 Actinomyces 892<br />
<strong>Richard</strong> F. Jacobs and Gordon E. Schutze<br />
Chapter 175 Nocardia 894<br />
<strong>Richard</strong> F. Jacobs and Gordon E. Schutze<br />
Section 3: Gram-Negative Bacterial Infections 896<br />
Chapter 176 Neisseria meningitidis<br />
(Meningococcus) 896<br />
Charles R. Woods<br />
Chapter 177 Neisseria gonorrhoeae<br />
(Gonococcus) 899<br />
Toni Darville<br />
Chapter 178 Haemophilus influenzae 904<br />
Robert S. Daum<br />
Chapter 179 Chancroid<br />
(Haemophilus ducreyi) 908<br />
Parvin Azimi<br />
Chapter 180 Pertussis [Bordetella pertussis and;<br />
B. parapertussis) 908<br />
Sarah S. Long < *
XXX Contents<br />
Chapter 181 Salmonella 912<br />
Thomas G. Cleary<br />
181.1 Nontyphoidal Salmonellosis<br />
181.2 Enteric Fever 916<br />
Chapter 182 Shigella<br />
Thomas G. Cleary<br />
919<br />
Chapter 183 Escherichia coli 921<br />
Theresa J. Ochoa and Thomas G. Cleary<br />
912<br />
Chapter 184 Cholera (Vibrio cholerae)<br />
Gloria P. Heresi and James R. Murphy<br />
Chapter 185 Campylobacter 926<br />
Gloria P. Heresi and James R. Murphy<br />
Chapter 186 Yersinia 929<br />
James R. Murphy and Gloria P. Heresi<br />
186.1 Yersinia enterocolitica 929<br />
186.2 Yersinia pseudotuberculosis 930<br />
186.3 Plague (Yersiniapestis) 930<br />
924<br />
Chapter 187 Aeromonas and Plesiomonas 932<br />
James R. Murphy and Gloria P. Heresi<br />
187.1 Aeromonas 932<br />
187.2 Plesiomonas shigelloides 933<br />
Chapter 188 Pseudomonas, Burkholderia, and<br />
Stenotrophomonas 933<br />
Robert S. Baltimore<br />
188.1 Pseudomonas aeruginosa 934<br />
188.2 Burkholderia 936<br />
188.3 Stenotrophomonas 936<br />
Chapter 189 Tularemia {Francisella<br />
tularensis) 937<br />
Gordon E. Schutze and <strong>Richard</strong> F. Jacobs<br />
Chapter 190 Brucella 939<br />
Gordon E. Schutze and <strong>Richard</strong> F. Jacobs<br />
Chapter 191 Legionella<br />
Lucy Tompkins<br />
941<br />
Chapter 192 Bartonella 943<br />
Barbara W. Stechenberg<br />
192.1 Bartonellosis {Bartonella bacilliformis) 943<br />
192.2 Cat-Scratch Disease (Bartonella henselae) 944<br />
192.3 Trench Fever (Bartonella quintana)<br />
192.4 Bacillary Angiomatosis and Bacillary<br />
946<br />
Peliosis Hepatis (Bartonella henselae and<br />
Bartonella quintana) 946<br />
Section 4: Anaerobic Bacterial Infections 947<br />
Chapter 193 Botulism (Clostridium<br />
botulinum) 947<br />
Robert Schechter and Stephen S. Arnon<br />
Chapter 194 Tetanus (Clostridium tetani)<br />
Stephen S. Arnon<br />
Chapter 195 Clostridium difficile-Assodated<br />
Diarrhea 953<br />
Margaret C. Fisher<br />
951<br />
Chapter 196 Other Anaerobic Infections 954<br />
Margaret C. Fisher<br />
Section 5: Mycobacterial Infections 958<br />
Chapter 197 Tuberculosis (Mycobacterium<br />
tuberculosis) 958<br />
Flor M. Munoz and Jeffrey R. Starke<br />
Chapter 198 Hansen Disease (Mycobacterium<br />
leprae) 972<br />
Dwight A.Powell<br />
Chapter 199 Nontuberculous Mycobacteria 975<br />
Dwight A. Powell<br />
Section 6: Spirochetal Infections 978<br />
Chapter 200 Syphilis (Treponemapallidum) 978<br />
Parvin Azimi<br />
Chapter 201 NonvenerealTreponemal<br />
Infections 982<br />
Parvin Azimi<br />
201.1 Yaws (Treponemapertenue) 982<br />
201.2 Bejel (Endemic Syphilis) (Treponemapallidum<br />
subsp. endemicum) 983<br />
201.3 Pinta (Treponema carateum) 983<br />
Chapter 202 Leptospira<br />
Parvin Azimi<br />
983<br />
Chapter 203 Relapsing Fever (Borrelia)<br />
Parvin Azimi<br />
Chapter 204 Lyme Disease<br />
(Borrelia burgdorferi) 986<br />
Eugene D. Shapiro<br />
Section 7: Mycoplasmal Infections 990<br />
Dwight A. Powell<br />
Chapter 205 Mycoplasma pneumoniae<br />
985<br />
990<br />
Chapter 206 Genital Mycoplasmas (Mycoplasma<br />
hominis and Ureaplasma urealyticum) 992<br />
Section 8: Chlamydial Infections 994<br />
Margaret R. Hammerschlag<br />
Chapter 207 Chlamydia pneumoniae 994<br />
Chapter 208 Chlamydia<br />
208.1 Trachoma 995<br />
trachomatis 995<br />
208.2 Genital Tract Infections 996<br />
208.3 Conjunctivitis and Pneumonia in Newborns<br />
208.4 Lymphogranuloma Venereum (LGV) 998<br />
Chapter 209 Psittacosis (Chlamydia psittaci)<br />
Section 9: Rickettsial Infections 999<br />
J. Stephen Dumler<br />
Chapter 210 Spotted Fever Group<br />
Rickettsioses 999<br />
210.1 Rocky Mountain Spotted Fever<br />
(Rickettsia rickettsii) 999<br />
997<br />
998
210.2 Mediterranean Spotted Fever or Boutonneuse<br />
Fever (Rickettsia conorii) 1003<br />
210.3 Rickettsialpox (Rickettsia akari) 1003<br />
Chapter 211 ScrubTyphus<br />
(Orientia tsutsugamushf) 1004<br />
Chapter 212 Typhus Group Rickettsioses<br />
212.1 Murine Typhus (Rickettsia typhi) 1005<br />
212.2 Epidemic Typhus (Rickettsia prowazekii)<br />
1005<br />
1006<br />
Chapter 213 Ehrlichiosisand Anaplasmosis 1006<br />
Chapter 214 Q Fever (Coxiella burnetii) 1009<br />
Section 10: Mycotic Infections<br />
1011<br />
Chapter 215 Candida 1011<br />
Martin E. Weisse and Stephen C. Aronoff<br />
215.1 Neonatal Infections 1012<br />
215.2 Infections in Immunocompetent Children and<br />
Adolescents 1013<br />
215.3 Infections in Immunocompromised Children and<br />
Adolescents 1013<br />
215.4 Chronic Mucocutaneous Candidiasis 1014<br />
Chapter 216 Cryptococcus neoformans<br />
Stephen C. Aronoff<br />
Chapter 217 Malassezia<br />
Martin E. Weisse<br />
1016<br />
1014<br />
Chapter 218 Aspergillus 1016<br />
Stephen C. Aronoff<br />
218.1 Hypersensitivity Syndromes 1016<br />
218.2 Saprophytic (Noninvasive) Syndromes 1017<br />
218.3 Invasive Disease 1017.<br />
Chapter 219 Histoplasmosis (Histoplasma<br />
capsulatum) 1018<br />
Stephen C. Aronoff<br />
Chapter 220 Blastomycosis (Blastomyces<br />
dermatitidis) 1020<br />
Robin B. Churchill and Stephen C. Aronoff<br />
Chapter 221 Coccidioidomycosis (Coccidioides<br />
immitis) 1021<br />
Demosthenes Pappagianis<br />
Chapter 222 Paracoccidioides<br />
brasiliensis 1023<br />
Robin B. Churchill and Stephen C. Aronoff<br />
Chapter 223 Sporotrichosis<br />
(Sporothrix schenckii) 1024<br />
Robin B. Churchill and Stephen C. Aronoff<br />
Chapter 224 Zygomycosis 1025<br />
Robin B. Churchill and Stephen C. Aronoff<br />
Section 11: Viral Infections 1026<br />
Chapter 225 Measles 1026<br />
Yvonne Mdldonado<br />
225.1 Subacute Sclerosing Panencephalitis 1030<br />
Chapter 226 Rubella 1032<br />
Yvonne Maldonado<br />
226.1 Progressive Rubella Panencephalitis 1034<br />
Chapter 227 Mumps 1035<br />
Yvonne Maldonado<br />
Chapter 228 Polioviruses 1036<br />
Eric A. F. Simoes<br />
Chapter 229 Nonpolio Enteroviruses 1042<br />
Mark J. Abzug<br />
Chapter 230 Parvovirus B19 1048<br />
William C. Koch<br />
Chapter 231 Herpes SimplexVirus 1051<br />
Steve Kohl<br />
Contents XXXI<br />
Chapter 232 Varicella-ZosterVirus 1057<br />
Martin G. Myers, Lawrence R. Stanberry, and Jane F. Seward<br />
Chapter 233 Epstein-BarrVirus 1062<br />
Hal B. Jenson<br />
Chapter 234 Cytomegalovirus 1066<br />
Sergio Stagno<br />
Chapter 235 Roseola (Human Herpesviruses<br />
6 and 7) 1069<br />
Charles T. Leach<br />
Chapter 236 Human Herpesvirus8<br />
Charles T. Leach<br />
Chapter 237<br />
Peter Wright<br />
Influenza Viruses 1072<br />
Chapter 238 Parainfluenza Viruses<br />
Peter Wright<br />
1072<br />
1075<br />
Chapter 239 Respiratory Syncytial Virus 1076<br />
Kenneth Mclntosh<br />
Chapter 240 Adenoviruses 1079<br />
Kenneth Mclntosh<br />
Chapter 241 Rhinoviruses 1080<br />
Kenneth Mclntosh<br />
Chapter 242 Rotavirus and Other Agents of Viral<br />
Gastroenteritis 1081<br />
Dorsey M. Bass<br />
Chapter 243 Human Papillomaviruses 1084<br />
Anna-Barbara Moscicki<br />
Chapter 244 Arboviral Encephalitis in<br />
North America 1086<br />
Scott B. Halstead<br />
Chapter 245 Arboviral Encephalitis Outside<br />
North America 1089<br />
Scott B. Halstead<br />
245.1 Venezuelan Equine Encephalitis 1089<br />
245.2 Japanese Encephalitis 1090<br />
245.3 Tick-Borne Encephalitis 1091<br />
Chapter 246 Dengue Fever and Dengue<br />
Hemorrhagic Fever 1092<br />
Scott B. Halstead<br />
Chapter 247 Yellow Fever<br />
Scott B. Halstead<br />
1095
X)<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
Cl<br />
5.<br />
Cl<br />
Cl<br />
Cl<br />
XXXII Contents<br />
Chapter 248 OtherViral Hemorrhagic Fevers<br />
Scott B. Halstead<br />
Chapter 249 Lymphocytic Choriomeningitis<br />
Virus 1099<br />
Hal B. Jenson<br />
Chapter 250 Hantaviruses 1100<br />
Scott B. Halstead<br />
Chapter 251 Rabies<br />
William G. Adams<br />
1101<br />
Chapter 252 Polyomaviruses<br />
Hal B. Jenson<br />
1105<br />
Chapter 253 Transmissible Spongiform<br />
Encephalopathies 1105<br />
David M. Asher<br />
Chapter 254 Acquired Immunodeficiency<br />
Syndrome (Human Immunodeficiency<br />
Virus) 1109<br />
Ram Yogev and Ellen Gould Chadwick<br />
Chapter 255 HumanT-Cell LymphotropicViruses<br />
Types I and II 1121<br />
Hal B. Jenson<br />
Section 12: Protozoan Diseases 1122<br />
Chapter 256 Primary Amebic<br />
, Meningoencephalitis 1122<br />
Martin E. Weisse and Stephen C. Aronoff<br />
Chapter 257 Amebiasis 1123<br />
Chandy C. John and Robert A. Salata<br />
Chapter 258 Giardiasis and Balantidiasis 1125<br />
Larry K. Pickering<br />
258.1 Giardia lamblia 1125<br />
258.2 Balantidiasis 1127<br />
Chapter 259 Spore-Forming Intestinal<br />
Protozoa 1128<br />
Patricia M. Flynn<br />
Chapter 260 Trichomoniasis (Trichomonas<br />
vagina I is) . 1129<br />
Chandy C. John and Robert A. Salata<br />
Chapter 261 Leishmaniasis (Leishmania) 1130<br />
Peter C. Melby<br />
Chapter 262 AfricanTrypanosomiasis<br />
(Sleeping Sickness; Trypanosoma brucei<br />
complex) 1134<br />
Robert A. Bonomo and Robert A. Salata<br />
Chapter 263 AmericanTrypanosomiasis (Chagas<br />
Disease; Trypanosoma cruzi) 1136<br />
Robert A. Bonomo and Robert A. Salata<br />
Chapter 264 Malaria (Plasmodium) 1139<br />
Peter J. Krause<br />
Chapter 265 Babesiosis (Babesia) 1143<br />
Peter J. Krause<br />
1096<br />
Chapter 266 Toxoplasmosis (Toxoplasma<br />
gondii) 1144<br />
Rima McLeod and Jack S. Remington<br />
Chapter 267 Pneumocystis carinii 1154<br />
Walter T. Hughes<br />
Section 13: Helminthic Diseases 1155<br />
Chapter 268 Ascariasis (Ascaris<br />
lumbricoides) 1155<br />
Sheral S. Patel and James W. Kazura<br />
Chapter 269 Hookworms (Ancylostoma and<br />
Necator americanus) 1156<br />
Peter Hotez<br />
269.1 Cutaneous Larva Migrans 1158<br />
Chapter 270 Trichuriasis (Trichuris trichiura)<br />
Sheral S. Patel and James W. Kazura<br />
Chapter 271 Enterobiasis (Enterobius<br />
vermicularis) 1159<br />
Sheral S. Patel and James W. Kazura<br />
Chapter 272 Strongyloidiasis (Strongyloides<br />
stereo ra I is) 1160<br />
Sheral S. Patel and James W. Kazura<br />
1158<br />
Chapter 273 Lymphatic Filariasis (Brugia malayi,<br />
Brugia timori, and Wuchereria bancrofti) 1161<br />
Sheral S. Patel and James W. Kazura<br />
Chapter 274 Other Tissue Nematodes<br />
Sheral S. Patel and James W. Kazura<br />
1162<br />
Chapter 275 Toxocariasis (Visceral and Ocular<br />
Larva Migrans) 1164<br />
Sheral S. Patel and James W. Kazura<br />
Chapter 276 Trichinosis (Trichinella spiralis) 1166<br />
Sheral S. Patel and James W. Kazura<br />
Chapter 277 Schistosomiasis (Schistosoma) 1161<br />
Charles H. King<br />
Chapter 278 Flukes (Liver, Lung, and<br />
Intestinal) 1168<br />
Charles H. King<br />
Chapter 279 Adult Tapeworm Infections 1169<br />
Ronald Blanton<br />
Chapter 280 Cysticercosis<br />
Ronald Blanton<br />
1171<br />
Chapter 281 Echinococcosis (Echinococcus<br />
granulosusand E. multilocularis) 1173<br />
Ronald Blanton<br />
Section 14: Preventive Measures 1174<br />
Chapter 282 Immunization Practices 1174<br />
Georges Peter ,<br />
Chapter 283 Infection Control and<br />
Prophylaxis 1184<br />
Margaret C. Fisher
Chapter 284 Child Care and Communicable<br />
Diseases 1187<br />
Larry K. Pickering<br />
Chapter 285 Health Advice for ChildrenTraveling<br />
Internationally 1189<br />
Chandy C. John and Robert A. Salata<br />
Part XVII The Digestive System 1197<br />
Section 1: Clinical Manifestations of Gastrointestinal<br />
Disease 1197<br />
Robert Wyllie<br />
Chapter 286 Normal DigestiveTract<br />
Phenomena 1197<br />
Chapter 287 Major Symptoms and Signs of<br />
DigestiveTract Disorders 1198<br />
Section 2: The Oral Cavity 1204<br />
Norman Tinanoff<br />
Chapter 288 Development and Developmental<br />
Anomalies of theTeeth 1204<br />
Chapter 289 Disorders of the Oral Cavity Associated<br />
with Other Conditions 1206<br />
Chapter 290 Malocclusion 1206<br />
Chapter 291 Cleft Lip and Palate 1207<br />
Chapter 292 Syndromes with Oral<br />
Manifestations 1209<br />
Chapter 293 Dental Caries 1209<br />
Chapter 294 Periodontal Diseases 1212<br />
Chapter 295 DentalTrauma 1213<br />
Chapter 296 Common Lesions of the Oral Soft<br />
Tissues 1214<br />
Chapter 297 Diseases of the Salivary Glands and<br />
Jaws 1215<br />
Chapter 298 Diagnostic Radiology in Dental<br />
Assessment 1216<br />
Section 3: The Esophagus 1217<br />
Susan Orenstein, John Peters, Seema Khan, Nader Youssef, and<br />
Sunny Zaheed Hussain<br />
Chapter 299 Embryology, Anatomy, and Function of<br />
the Esophagus 1217<br />
299.1 Common Clinical Manifestations and<br />
Diagnostic Aids 1218<br />
Chapter 300 Congenital Anomalies: Esophageal<br />
Atresia and Tracheoesophageal Fistula 1219<br />
300.1 Congenital Anomalies: Laryngotracheoesophageal<br />
Clefts 1220<br />
Contents XXXIII<br />
Chapter 301 Obstructing and Motility Disorders of<br />
the Esophagus 1220<br />
Chapter 302 Dysmotility 1220<br />
Chapter 303 Hiatal Hernia 1221<br />
Chapter 304 Gastroesophageal Reflux Disease<br />
(GERD) 1222<br />
304.1 Complications of GERD 1224<br />
Chapter 305 Non-GERDEsophagitis 1224<br />
Chapter 306 Esophageal Perforation 1225<br />
Chapter 307 Esophageal Varices 1225<br />
Chapter 308 Ingestions 1225<br />
308.1 Foreign Bodies in the Esophagus 1225<br />
308.2 Caustic Ingestions 1226<br />
Section 4: Stomach and Intestines 1228<br />
Chapter 309 Normal Development, Structure, and<br />
Function 1228<br />
Robert Wyllie<br />
Chapter 310 Pyloric Stenosis and Congenital<br />
Anomalies of the Stomach 1229<br />
Robert Wyllie<br />
310.1 Hypertrophic Pyloric Stenosis 1229<br />
310.2 Congenital Gastric Outlet Obstruction 1231<br />
310.3 Gastric Duplication 1231<br />
310.4 Gastric Volvulus 1231<br />
310.5 Hypertrophic Gastropathy 1231<br />
Chapter 311 Intestinal Atresia, Stenosis, and<br />
Malrotation 1232<br />
Robert Wyllie<br />
311.1 Duodenal Obstruction 1233<br />
311.2 Jejunal and Heal Atresia and Obstruction 1234<br />
311.3 Malrotation 1235<br />
Chapter 312 Intestinal Duplications, Meckel<br />
Diverticulum, and Other Remnants of the<br />
Omphalomesenteric Duct 1236<br />
Robert Wyllie<br />
312.1 Intestinal Duplication 1236<br />
312.2 Meckel Diverticulum and Other Remnants<br />
of the Omphalomesenteric Duct 1236<br />
Chapter 313 Motility Disorders and Hirschsprung<br />
Disease 1237<br />
Robert Wyllie<br />
313.1 Chronic Intestinal Pseudo-Obstruction 1237<br />
313.2 Functional Constipation 1238<br />
313.3 C ongenital Aganglionic Megacolon<br />
(Hirschsprung Disease) 1239<br />
313.4 Superior Mesenteric Artery Syndrome (Wilkie<br />
Syndrome, Cast Syndrome, Arteriomesenteric<br />
Duodenal Compression Syndrome) 1241<br />
Chapter 314 Ileus, Adhesions, Intussusception, and<br />
Closed-Loop Obstructions 1241<br />
Robert Wyllie \<br />
314.1 Ileus 1241 C<br />
314.2 Adhesions 1241 •*
xxxiv Contents<br />
314.3 Intussusception 1242<br />
314.4 Closed-Loop Obstructions 1243<br />
Chapter 315 Foreign Bodies and Bezoars 1244<br />
Robert Wyllie<br />
315.1 Foreign Bodies in the Stomach and Intestine 1244<br />
315.2 Bezoars 1244<br />
Chapter 316 Peptic Ulcer Disease 1244<br />
Francisco A. Sylvester<br />
316.1 Secondary or Stress (Peptic) Ulcer Disease 1246<br />
316.2 Zollinger-Ellison Syndrome 1247<br />
Chapter 317 Inflammatory Bowel Disease 1248<br />
Jeffrey Hyams<br />
317.1 Chronic Ulcerative Colitis 1249<br />
317.2 Crohn Disease (Regional Enteritis,<br />
Regional lleitis, Granulomatous Colitis) 1252<br />
317.3 Behcet Syndrome 1255<br />
Chapter 318 Food Allergy (Food<br />
Hypersensitivity) 1255<br />
Jeffrey Hyams<br />
Chapter 319 Eosinophilic Gastroenteritis<br />
Jeffrey Hyams<br />
1257<br />
Chapter 320 Malabsorptive Disorders 1257<br />
Manuel Garda-Careaga and John A. Kerner<br />
320.1 Evaluation of Children with Suspected Intestinal<br />
Malabsorption 1257<br />
320.2 Chronic Malnutrition 1261<br />
320.3 Liver and Biliary Disorders 1261<br />
320.4 Intestinal Infections 1261<br />
320.5 Immunodeficiency 1262<br />
320.6 Stagnant Loop Syndrome (Blind Loop Syndrome:<br />
Bacterial Overgrowth Syndrome) 1262<br />
320.7 Short Bowel Syndrome 1262<br />
320.8 Gluten-Sensitive Enteropathy (Celiac Disease,<br />
Celiac Sprue) 1264<br />
320.9 Immunoproliferative Small Intestinal<br />
Disease 1266<br />
320.10 Other Malabsorptive Snydromes 1266<br />
320.11 Enzyme Deficiencies 1267<br />
320.12 Defects of Absorption or Transport 1268<br />
Chapter 321 Gastroenteritis 1272<br />
Larry K. Pickering and John D. Snyder<br />
Chapter 322 Chronic Diarrhea<br />
Fayez K. Ghishan<br />
Chapter 323<br />
Childhood<br />
Robert Wyllie<br />
1276<br />
Recurrent Abdominal Pain of<br />
1281<br />
Chapter 324 Acute Appendicitis<br />
Gary E. Hartman<br />
1283<br />
Chapter 325 Surgical Conditions of the Anus,<br />
Rectum, and Colon 1285<br />
Alberto Pena<br />
325.1 Anorectal Malformations 1285<br />
325.2 Anal Fissure 1288<br />
325.3 Perianal Abscess and Fistula 1288<br />
325.4 Hemorrhoids 1289<br />
325.5 Rectal Prolapse 1289<br />
325.6 Pilonidal Sinus and Abscess 1289<br />
Chapter 326 Tumors of the DigestiveTract 1289<br />
Joel Shilyansky<br />
326.1 Diarrhea from Hormone-Secreting Tumors 1292<br />
Chapter 327 Inguinal Hernias 1293<br />
John J. Aiken<br />
Section 5: Exocrine Pancreas 1298<br />
Steven L. Werlin<br />
Chapter 328 Embryology, Anatomy, and<br />
Physiology 1298<br />
328.1 Anatomic Abnormalities 1298<br />
328.2 Physiology 1298<br />
Chapter 329 Pancreatic FunctionTests 1299<br />
Chapter 330 Disorders of the Exocrine<br />
Pancreas 1299<br />
Chapter 331 Treatment of Pancreatic<br />
Insufficiency 1300<br />
Chapter 332 Pancreatitis 1300<br />
332.1 Acute Pancreatitis 1300<br />
332.2 Chronic Pancreatitis 1302<br />
Chapter 333 Pseudocyst of the Pancreas 1303<br />
Chapter 334 PancreaticTumors 1303<br />
Section 6: The Liver and Biliary System 1304<br />
Chapter 335 Development and Function of the Liver<br />
and Biliary System 1304<br />
Michael D. Bates and William F. Balistreri<br />
Chapter 336 Manifestations of Liver Disease<br />
Vicky Lee Ng and William F. Balistreri<br />
336.1 Evaluation of Patients with Possible Liver<br />
Dysfunction 1312<br />
Chapter 337 Cholestasis 1314<br />
Hassan H. A-Kaderand William F. Balistreri<br />
337.1 Neonatal Cholestasis 1314<br />
337.2 Cholestasis in the Older Child 1319<br />
Chapter 338 Metabolic Diseases of the Liver<br />
Jeffrey A. Rudolph and William F. Balistreri<br />
338.1 Inherited Deficient Conjugation of Bilirubin<br />
(Familial Nonhemolytic Unconjugated<br />
338.2<br />
338.3<br />
338.4<br />
338.5<br />
338.6<br />
1308<br />
1319<br />
Hyperbilirubinemia) 1320<br />
Wilson Disease 1321<br />
Hepatic Copper Overload Syndrome 1323<br />
Indian Childhood Cirrhosis 1323<br />
Neonatal Iron Storage Disease (NISD) 132 3<br />
Miscellaneous Metabolic Diseases of the Liver 1323<br />
Chapter 339 Viral Hepatitis 1324<br />
John D. Snyder and Larry K. Pickering<br />
339.1 Hepatitis in Neonates 1331<br />
Chapter 340 Liver Abscess 1332<br />
Jeffrey Schwimmer and William F. Balistreri
Chapter 341 Liver Disease Associated with Systemic<br />
Disorders 1333<br />
Jeffrey Schwimmer and William F. Balistreri<br />
Chapter 342 Reye Syndrome and the Mitochondrial<br />
Hepatopathies 1335<br />
Jeffrey A. Rudolph and William F. Balistreri<br />
Chapter 343 Autoimmune (Chronic) Hepatitis<br />
Benjamin L. Shneider and Frederick J. Suchy<br />
Chapter 344 Drug- andToxin-lnduced Liver<br />
Injury 1339<br />
Frederick J. Suchy<br />
Chapter 345 Fulminant Hepatic Failure<br />
Frederick J. Suchy<br />
1341<br />
1337<br />
Chapter 346 Cystic Diseases of the Biliary Tract and<br />
Liver 1343<br />
Frederick J. Suchy<br />
Chapter 347 Diseases of the Gallbladder<br />
Frederick J. Suchy<br />
Chapter 348 Portal Hypertension and<br />
Varices 1346<br />
Frederick J. Suchy<br />
Chapter 349 Liver Transplantation<br />
Kenneth L. Cox<br />
Section 7: Peritoneum 1351<br />
Chapter 350 Malformations 1351<br />
Jeffrey S: Hyams<br />
Chapter 351 Ascites<br />
Jeffrey S. Hyams<br />
351.1 Chylous Ascites<br />
1351<br />
1351<br />
1349<br />
Chapter 352 Peritonitis 1351<br />
Jeffrey S. Hyams<br />
352.1 Acute Primary Peritonitis 1352<br />
352.2 Acute Secondary Peritonitis 1352<br />
352.3 Acute S econdary Localized Peritonitis<br />
(Peritoneal Abscess) 1353<br />
Chapter 353 Diaphragmatic Hernia 1353<br />
Gary E. Hartman<br />
353.1 Foramen of Morgagni Hernia 1355<br />
353.2 Paraesophageal Hernia 1355<br />
353.3 Eventration 1355<br />
Chapter 354 Epigastric Hernia<br />
Gary E. Hartman<br />
354.1 Incisional Hernia 1356<br />
1355<br />
1345<br />
: XVIII: The Respiratory System 1357<br />
Section 1: Development and Function 1357<br />
Chapter 355 Development of the Respiratory<br />
System 1357<br />
Gabriel G. Haddad and J. Julio Perez Fontdn<br />
Chapter 356 Regulation of Respiration<br />
Gabriel G. Haddad<br />
Contents XXXV<br />
1359<br />
Chapter 357 Respiratory Pathophysiology 1362<br />
J. Julio Perez Fontdn and Gabriel G. Haddad<br />
357.1 Respiratory Failure 1370<br />
J. Julio Perez Fontdn and Gabriel G. Haddad<br />
357.2 Congenital Central Hypoventilation Syndrome<br />
(Ondine Curse) 1372<br />
Gabriel G. Haddad<br />
Chapter 358 Defense Mechanisms and Metabolic<br />
Functions of the Lung 1373<br />
Gabriel G. Haddad and J. Julio Perez Fontdn<br />
358.1 Defense Mechanisms 1373<br />
358.2 Metabolic Functions 1375<br />
Chapter 359 Diagnostic Approach to Respiratory<br />
Disease 1375<br />
Gabriel G. Haddad and Regina M. Palazzo<br />
Chapter 360 Sudden Infant Death Syndrome<br />
Carl E. Hunt and Fern R. Hauck<br />
Section 2: Upper Respiratory Tract 1386<br />
Chapter 361 Congenital Disorders of the<br />
Nose 1386<br />
Joseph Haddad Jr.<br />
Chapter 362 Acquired Disorders of the<br />
Nose 1387<br />
Joseph Haddad Jr.<br />
362.1 Foreign Body 1387<br />
362.2 Epistaxis 1387<br />
Chapter 363 Nasal Polyps 1388<br />
Joseph Haddad Jr.<br />
Chapter 364 The Common Cold 1389<br />
Ronald B. Turner and Gregory F. Hayden<br />
Chapter 365 Sinusitis 1391<br />
Diane E. Poppas and J. Owen Hendley<br />
Chapter 366 Acute Pharyngitis 1393<br />
Gregory F. Hayden and Ronald B. Turner<br />
Chapter 367 Retropharyngeal Abscess, Lateral<br />
Pharyngeal (Parapharyngeal) Abscess, and<br />
Peritonsillar Cellulitis/Abscess 1394<br />
Diane E. Poppas and J. Owen Hendley<br />
Chapter 368 Tonsils and Adenoids 1396<br />
Ralph F. Wetmore<br />
Chapter 369 Obstructive Sleep Apnea and<br />
Hypoventilation 1397<br />
Carol L. Rosen, Lewis J. Kass, and Gabriel G. Haddad<br />
Section 3: Disorders of the Lungs and Lower<br />
Airways 1401<br />
Thomas P. Green and Susanna A. McColley<br />
Chapter 370 Chronic or Recurrent Respiratory,<br />
Symptoms 1401 '•<br />
Thomas F. Boat '<br />
1380
XXXVI Contents<br />
Chapter 371 Acute Inflammatory Upper Airway<br />
Obstruction 1405<br />
Genie E. Roosevelt<br />
371 :i Infectious Upper Airway Obstruction 1405<br />
371.2 Bacterial Tracheitis 1409<br />
Chapter 372 Congenital Anomalies of the<br />
Larynx 1409<br />
Lauren D. Holinger<br />
372.1 Laryngomalacia 1409<br />
372.2 Congenital Subglottic Stenosis 1409<br />
372.3 Vocal Cord Paralysis 1410<br />
372.4 Congenital Laryngeal Webs and Atresia 1410<br />
372.5 Congenital SubglotticHemangioma 1410<br />
372.6 Laryngoceles and Saccular Cysts 1410<br />
372.7 Posterior Laryngeal Cleft and<br />
Laryngotracheoesophageal Cleft 1410<br />
Chapter 373 Foreign Bodies of the Airway 1410<br />
Lauren D. Holinger<br />
373.1 Laryngeal Foreign B odies 1411<br />
373.2 Tracheal Foreign Bodies 1411<br />
373.3 Bronchial Foreign Bodies 1411<br />
Chapter 374 Laryngotracheal Stenosis, Subglottic<br />
Stenosis 1412<br />
Lauren D. Holinger<br />
374.1 Congenital Subglottic Stenosis 1412<br />
374.2 AcquiredXaryngotracheal Stenosis 1412<br />
Chapter 375 Congenital Anomalies of theTrachea<br />
and Bronchi 1412<br />
Lauren D. Holinger<br />
375.1 Vascular and Cardiac Anomalies 1412<br />
375.2 Trachea] Stenoses, Webs, and Atresia 1412<br />
375.3 Foregut Cysts 1413<br />
Chapter 376 Bronchomalacia and<br />
Tracheomalacia 1413<br />
Jonathan D. Finder<br />
Chapter 377 Neoplasms of the Larynx,Trachea, and<br />
Bronchi 1413<br />
Lauren D. Holinger<br />
377.1 Vocal Nodules 1413<br />
377.2 Recurrent Respiratory Papillomatosis<br />
(RRP) 1413<br />
377.3 Congenital Subglottic Hemangioma 1414<br />
377.4 Vascular Anomalies . 1414<br />
377.5 Other Laryngeal Neoplasms 1414<br />
377.6 Tracheal Neoplasms 1414<br />
377.7 Bronchial Tumors 1414<br />
Chapter 378 Inflammatory Disorders of the Small<br />
Airways 1414<br />
Denise Goodman<br />
378.1 Bronchitis 1414<br />
378.2 Bronchiolitis 1415<br />
Chapter 379 Wheezing in Infants 1417<br />
Marzena Krawiec and Robert F. Lemanske, Jr.<br />
Chapter 380 .Emphysema and<br />
Overinflation 1419<br />
Steven Boas and Glenna B. Winnie<br />
Chapter 381 o^-Antitrypsin Deficiency and<br />
Emphysema 1421<br />
Glenna B. Winnie and Steven Boas<br />
Chapter 382 Other Distal Airway Diseases 1422<br />
Steven Boas<br />
382.1 Bronciolitis Obliterans 1422<br />
382.2 Follicular Bronchitis 1422<br />
382.3 Pulmonary Alveolar Microlithiasis 1422<br />
Chapter 383 Congenital Disorders of the<br />
Lung 1423<br />
Thomas P. Green and Jonathan D. Finder<br />
383.1 Pulmonary Agenesis and Aplasia 1423<br />
383.2 Pulmonary Hypoplasia 1423<br />
383.3 Cystic Adenomatoid Malformation 1424<br />
383.4 Pulmonary Sequestration 1424<br />
383.5 Bronchogenic Cysts 1425<br />
383.6 Congenital Pulmonary Lymphangiectasia 1425<br />
383.7 Lung Hernia 1425<br />
383.8 Other Congenital Malformations of<br />
the Lung 1425<br />
Chapter 384 Pulmonary Edema<br />
Robert Mazor and Thomas P. Green<br />
Chapter 385 Aspiration Syndromes<br />
John L. Colombo<br />
1426<br />
1427<br />
Chapter 386 Chronic Recurrent Aspiration<br />
John L. Colombo<br />
Chapter 387 Gastroesophageal Reflux and<br />
Respiratory Disorders 1430<br />
John L. Colombo<br />
1428<br />
Chapter 388 Parenchymal Disease with Prominent<br />
Hypersensitivity, Eosinophilic Infiltration, or<br />
Toxin-Mediated Injury 1430<br />
Oren Lakser<br />
388.1 Hypersensitivity to Inhaled Materials 1430<br />
388.2 Silo Filler Disease 1431<br />
388.3 Paraquat Lung 1431<br />
388.4 Eosinophilic Lung Disease (Formerly Loffler<br />
Syndrome) 1432<br />
Chapter 389 Pneumonia 1432<br />
Theodore C. Sectish and Charles G. Prober<br />
Chapter 390 Slowly Resolving Pneumonia 1435<br />
Oren Lakser<br />
Chapter 391 Bronchiectasis 1436<br />
Oren Lakser<br />
Chapter 392 Pulmonary Abscess 1437<br />
Oren Lakser<br />
Chapter 393 Cystic Fibrosis 1437<br />
Thomas F. Boat<br />
Chapter 394 Primary Ciliary Dyskinesia (Immotile<br />
Cilia Syndrome) 1450<br />
Gabriel G. Haddad<br />
Chapter 395 Interstitial Lung Diseases<br />
Michelle S. Howenstine<br />
1451<br />
\
Chapter 396 Pulmonary Alveolar Proteinosis<br />
Aaron Hamvas, Lawrence M. Nogee, and F. Sessions Cole<br />
Chapter 397 Inherited Disorders of Surfactant<br />
Protein Metabolism 1454<br />
Aaron Hamvas, Lawrence M. Nogee, andF. Sessions Cole<br />
Chapter 398 Pulmonary Hemosiderosis<br />
Dorr G. Dearborn<br />
1455<br />
Chapter 399 Pulmonary Hemorrhage, Embolism,<br />
and Infarction 1457<br />
Daniel Sloniewsky and Thomas P. Green<br />
399.1 Pulmonary Embolism and Infarction 1457<br />
399.2 Hemoptysis 1458<br />
Chapter 400 Atelectasis 1459<br />
Ranna A. Rozenfeld<br />
Chapter 401 Pulmonary Tumors 1461<br />
Susanna A. McColley and Paul R. Haut<br />
Chapter 402 Pleurisy 1461<br />
Glenna B. Winnie<br />
402.1 Dry or Plastic Pleurisy 1461<br />
402.2 Serofibrinous Pleurisy 1462<br />
402.3 Purulent Pleurisy (Empyema) 1462<br />
Chapter 403 Pneumothorax<br />
Glenna B. Winnie<br />
1463<br />
Chapter 404 Pneumomediastinum<br />
Glenna B. Winnie<br />
Chapter 405 Hydrothorax 1465<br />
Glenna B. Winnie<br />
Chapter 406 Hemothorax 1465<br />
Glenna B. Winnie<br />
Chapter 407 Chylothorax 1466<br />
Glenna B. Winnie<br />
1465<br />
Chapter 408 Bronchopulmonary Dysplasia<br />
Steven Lestrud<br />
Chapter 409 Skeletal Diseases Influencing<br />
Pulmonary Function 1467<br />
1453<br />
1466<br />
Steven Boas<br />
409.1 Pectus Excavatum (Funnel Chest) 1467<br />
409.2 Pectus Carinatum and Fissuri Sterni 1468<br />
409.3 Asphyxiating Thoracic Dystrophy<br />
(Thoracic-Pelvic-Phalangeal Dystrophy) 1468<br />
409.4 Achondroplasia 1468<br />
409.5 Kyphoscoliosis: Adolescent Idiopathic Scoliosis and<br />
Congential Scoliosis 1469<br />
409.6 Congenital Rib Anomalies 1469<br />
Chapter 410 Neuromuscular Diseases with<br />
Pulmonary Consequences 1469<br />
David Gozal<br />
Chapter 411 Extrapulmonary Diseases with<br />
Pulmonary Manifestations 1471<br />
Susanna A. McColley<br />
Chapter 412 Chronic Severe Respiratory<br />
Insufficiency 1472<br />
Zehava Noah and Cynthia Budek<br />
Contents XXXVil<br />
Part XIX The Cardiovascular System 1475<br />
Section V. Developmental Biology of the Cardiovascular<br />
System 1475<br />
Daniel Bernstein<br />
Chapter 413 Cardiac Development 1475<br />
413.1 Early Cardiac Morphogenesis 1475<br />
413.2 Cardiac Looping 1475<br />
413.3 Cardiac Septation 1475<br />
413.4 Aortic Arch Development 1477<br />
413.5 Cardiac Differentiation 1478<br />
413.6 Developmental Changes in Cardiac Function 1478<br />
Chapter 414 The Fetal-to-Neonatal Circulatory<br />
Transition 1479<br />
414.1 The Fetal Circulation 1479<br />
414.2 The Transitional Circulation 1479<br />
414.3 The Neonatal Circulation 1479<br />
414.4 Persistent Pulmonary Hypertension of the<br />
Neonate (Persistence of Fetal Circulatory<br />
Pathways) 1481<br />
Section 2: Evaluation of the Cardiovascular System 1481<br />
Daniel Bernstein<br />
Chapter 415 History and Physical<br />
Examination 1481<br />
Chapter 416 Laboratory Evaluation 1488<br />
416.1 Radiologic Assessment 1488<br />
416.2 Electrocardiography 1489<br />
416.3 Hematologic Data 1493<br />
416.4 Echocardiography 1493<br />
416.5 Exercise Testing 1495<br />
416.6 MRI, Electron Beam CT, and Radionuclide<br />
Studies 1496<br />
416.7 Cardiac Catheterization 1496<br />
Section 3: Congenital Heart Disease<br />
Daniel Bernstein<br />
1499<br />
Chapter 417 Epidemiology and Genetic Basis of<br />
Congenital Heart Disease 1499<br />
Chapter 418 Evaluation of the Infant or Child with<br />
Congenital Heart Disease 1502<br />
Chapter 419 Acyanotic Congenital Heart Disease:<br />
The Left-to-Right Shunt Lesions 1503<br />
419.1 Atrial Septal Defect 1503<br />
419.2 Ostium Secundum Defect 1504<br />
419.3 Sinus Venosus Atrial Septal Defect 1505<br />
419.4 Partial Anomalous Pulmonary Venous<br />
Return 1505<br />
419.5 Atrioventricular Septal Defects (Ostium Primum and<br />
Atrioventricular Canal or Endocardial Cushion<br />
Defects) 1506<br />
419.6 Ventricular Septal Defect 1508<br />
419.7 Supracristal Ventricular Septal Defect with Aortic<br />
Insufficiency 1510 t,<br />
419.8 Patent Ductus Arteriosus 1510 ',"»<br />
419.9 Aorticopulmonary Window Defect 1512 .'-\
xxxviii Contents<br />
419.10 Coronary-Arteriovenous Fistula<br />
(Coronary-Cameral Fistula) 1512<br />
419.11 Ruptured Sinus of Valsalva Aneurysm 1512<br />
Chapter 420 Acyanotic Congenital Heart Disease:<br />
The Obstructive Lesions 1513<br />
420.1 Pulmonary Valve Stenosis with Intact Ventricular<br />
Septum 1513<br />
420.2 Infundibular Pulmonary Stenosis and Double-<br />
Chamber Right Ventricle 1515<br />
420.3 Pulmonary Stenosis in Combination with an<br />
Intracardiac Shunt 1515<br />
420.4 Peripheral Pulmonary Stenosis 1515<br />
420.5 Aortic Stenosis 1516<br />
420.6 Coarctation of the Aorta 1518<br />
420.7 Coarctation with Ventricular Septal Defect 1520<br />
420.8 Coarctation with Other Cardiac Anomalies and<br />
Interrupted Aortic Arch 1520<br />
420.9 Congenital Mitral Stenosis 1520<br />
42 0.10 Pulmonary Venous Hypertension 1521<br />
Chapter 421 Acyanotic Congenital Heart Disease:<br />
Regurgitant Lesions 1522<br />
421.1 Pulmonary Valvular Insufficiency and Congenital<br />
Absence of the Pulmonary Valve 1522<br />
421.2 Congenital Mitral Insufficiency 1522<br />
421.3 Mitral Valve Prolapse 1522<br />
421.4 Tricuspid Regurgitation 1523<br />
Chapter 422 Cyanotic Congenital Heart Disease:<br />
Evaluation of the Critically III Neonate with<br />
Cyanosis and Respiratory Distress 1523<br />
Chapter 423 Cyanotic Congenital Heart Lesions:<br />
Lesions Associated with Decreased Pulmonary<br />
Blood Flow 1524<br />
423.1 Tetralogy of Fallot 1524<br />
42 3.2 Pulmonary Atresia with Ventricular<br />
Septal Defect 1528<br />
423.3 Pulmonary Atresia with Intact Ventricular<br />
Septum 1529<br />
423.4 Tricuspid Atresia 1530<br />
423.5 Double-Outlet Right Ventricle with Pulmonary<br />
Stenosis 1532<br />
42 3.6 Transposition of the Great Arteries with Ventricular<br />
Septal Defect and Pulmonary Stenosis 1532<br />
423.7 Ebstein Anomaly of the Tricuspid Valve 1532<br />
Chapter 424 Cyanotic Congenital Heart Disease:<br />
Lesions Associated with Increased Pulmonary<br />
Blood Flow 1534<br />
424.1 d-Transposition of the Great Arteries 1534<br />
424.2 d-Transposition of the Great Arteries with Intact<br />
Ventricular Septum 1535<br />
424.3 Transposition of the Great Arteries with Ventricular<br />
Septal Defect 1536<br />
424.4 1-Transposition of the Great Arteries (Corrected<br />
^ Transposition) 1537<br />
424.5 Double-Outlet Right Ventricle Without Pulmonary<br />
Stenosis 1538<br />
424.6 Double-Outlet Right Ventricle with<br />
Transposition of the Great Arteries (Taussig-Bing<br />
Anomaly) 1538<br />
424.7 Total Anomalous Pulmonary Venous Return 1538<br />
424.8 Truncus Arteriosus 1540<br />
424.9 Single Ventricle (Double-Inlet Ventricle,<br />
Univentricular Heart) 1541<br />
424.10 Hypoplastic Left Heart Syndrome 1541<br />
424.11 Abnormal Positions of the Heart and the<br />
Heterotaxy Syndromes (Asplenia,<br />
Polysplenia) 1544<br />
Chapter 425 Other Congenital Heart and Vascular<br />
Malformations 1545<br />
42 5.1 Anomalies of the Aortic Arch 1545<br />
42 5.2 Anomalous Origin of the Coronary Arteries 1545<br />
42 5.3 Pulmonary Arteriovenous Fistula 1547<br />
425.4 Ectopia Cordis 1548<br />
42 5.5 Diverticulum of the Left Ventricle 1548<br />
Chapter 426 Pulmonary Hypertension 1548<br />
426.1 Primary Pulmonary Hypertension 1548<br />
426.2 Pulmonary Vascular Disease (Eisenmenger<br />
Syndrome) 1549<br />
Chapter 427 General Principles of Treatment of<br />
Congenital Heart Disease 1551<br />
Section 4: Cardiac Arrhythmias<br />
Anne Dubin<br />
1554<br />
Chapter 428 Disturbances of Rate and Rhythm<br />
of the Heart 1554<br />
428.1 Sinus Arrhythmias and Extrasystoles 1554<br />
428.2 Supraventricular Tachycardia 1559<br />
428.3 Ventricular Tachyarrhythmias 1561<br />
428.4 Long Q-T Syndrome 1562<br />
428.5 Bradyarrhythmias 1562<br />
428.6 Sick Sinus Syndrome 1563<br />
428.7 Sudden Death 1563<br />
Section 5:AcquiredHeartDisease 1565<br />
Daniel Bernstein<br />
Chapter 429 Infective Endocarditis 1565<br />
Chapter 430 Rheumatic Heart Disease 1570<br />
Section 6: Diseases of the Myocardium and<br />
Pericardium 1572<br />
Daniel Bernstein<br />
Chapter 431 Diseases of the Myocardium 1572<br />
431.1 Dilated Cardiomyopathy 15 72<br />
431.2 Hypertrophic Cardiomyopathy 1575<br />
431.3 Restrictive Cardiomyopathy 1576<br />
431.4 Myocarditis 1576<br />
431.5 Nonviral Causes of Myocarditis 1577<br />
431.6 Endocardial Fibroelastosis 1578<br />
Chapter 432 Diseases of the Pericardium 1579<br />
432.1 Constrictive Pericarditis 1581 , :<br />
Chapter 433 Tumorsof the Heart 1581<br />
Section 7: Cardiac Therapeutics 1582<br />
Daniel Bernstein<br />
Chapter 434 Heart Failure 1582<br />
434.1 Cardiogenic Shock 1586
Chapter 435 Pediatric Heart and Heart-Lung<br />
Transplantation 1587<br />
435.1 Pediatric Heart Transplantation 1587<br />
435.2 Heart-Lung and Lung Transplantation 1590<br />
Section 8: Diseases of the Peripheral Vascular<br />
System 1591<br />
Daniel Bernstein<br />
Chapter 436 Disease of the Blood Vessels<br />
(Aneurysms and Fistulas) 1591<br />
436.1 Kawasaki Disease 1591<br />
436.2 Arteriovenous Fistulas 1591<br />
Chapter 437 Systemic Hypertension 1592<br />
Diseases of the Blood 1599<br />
Section 1: The Hematopoietic System 1599<br />
Chapter 438 Development of the Hematopoietic<br />
System 1599<br />
Robin K. Ohls and Robert D. Christensen<br />
Chapter 439 The Anemias 1604<br />
Bertil Glader<br />
Section 2: Anemias of Inadequate Production 1606<br />
Bertil Glader<br />
Chapter 440 Congenital Hypoplastic Anemia<br />
(Diamond-Blackfan Anemia) 1606<br />
Chapter 441 Pearson Marrow-Pancreas<br />
Syndrome 1607<br />
Chapter 442 Acquired Pure Red Blood Cell<br />
Anemias 1608<br />
Chapter 443 Anemia of Chronic Disorders<br />
(ACD) and Renal Disease 1609<br />
Chapter 444 Congenital Dyserythropoietic<br />
Anemias (CDA) 1609<br />
Chapter 445 Physiologic Anemia of Infancy 1610<br />
Chapter 446 Megaloblastic Anemias 1611<br />
446.1 Folic Acid Deficiency 1611<br />
446.2 Vitamin B12 (Cobalamin) Deficiency 1612<br />
446.3 Other Rare Megaloblastic Anemias 1613<br />
Chapter 447 Iron-Deficiency Anemia 1614<br />
Chapter 448 Other Microcytic Anemias 1616<br />
Section 3: Hemolytic Anemias 1617<br />
Chapter 449 Definitions and Classification of<br />
Hemolytic Anemias 1617<br />
George B. Segel<br />
Chapter 450 Hereditary Spherocytosis 1620<br />
George B. Segel<br />
Chapter 451 Hereditary Elliptocytosis 1622<br />
George B. Segel<br />
Contents XXXJX<br />
Chapter 452 Hereditary Stomatocytosis 1622<br />
George B. Segel<br />
Chapter 453 Other Membrane Defects 1622<br />
George B. Segel<br />
Chapter 454 Hemoglobin Disorders 1623<br />
Keith Quircdo and Elliott Vichinsky<br />
454.1 Sickle Cell Disease 1624<br />
454.2 Sickle Cell Trait (Hemoglobin AS) 1628<br />
454.3 Other Hemoglobinopathies 1629<br />
454.4 Unstable Hemoglobin Disorders 1629<br />
454.5 Abnormal Hemoglobins with Increased Oxygen<br />
Affinity 1629<br />
454.6 Abnormal Hemoglobins Causing<br />
Cyanosis 1629<br />
454.7 Hereditary Methemoglobinemia 1629<br />
454.8 Syndromes of Hereditary Persistence of Fetal<br />
Hemoglobin (HPFH) 1630<br />
454.9 Thalassemia Syndromes 1630<br />
Chapter 455 Enzymatic Defects 1635<br />
George B. Segel<br />
455.1 Pyruvate Kinase (PK) Deficiency 1635<br />
455.2 Other Glycolytic Enzyme Deficiencies 1636<br />
455.3 Glucose-6-Phosphate Dehydrogenase (G6PD) and<br />
Related Deficiencies 1636<br />
Chapter 456 Hemolytic Anemias Resulting from<br />
Extracellular Factors 1638<br />
George B. Segel<br />
Chapter 457 Hemolytic Anemias Secondary to Other<br />
Extracellular Factors 1640<br />
George B. Segel<br />
Section 4: Polycythemia (Erythrocytosis) 1641<br />
Bruce M. Camitta<br />
Chapter 458 Primary Polycythemia (Polycythemia<br />
RubraVera) 1641<br />
Chapter 459 Secondary Polycythemia 1641<br />
Section 5: The Pancytopenias 1642<br />
Chapter 460 The Constitutional<br />
Pancytopenias 1642<br />
Alan D. D'Andrea<br />
Chapter 461 The Acquired Pancytopenias 1644<br />
Jeffrey D. Hard<br />
Section 6: Risks of Blood Component<br />
Transfusions 1646<br />
Ronald G. Strauss<br />
Chapter 462 Red Blood Cell Transfusions and<br />
ErythropoietinTherapy 1646<br />
Chapter 463 Platelet Transfusions 1647<br />
Chapter 464 Neutrophil (Granulocyte)<br />
Transfusions 1648 A
xl Contents<br />
Chapter 465 Fresh Frozen Plasma<br />
Transfusions 1649<br />
Chapter 466 Risks of BloodTransfusions 1650<br />
Section 7: Hemorrhagic and Thrombotic Diseases 1651<br />
Robert R. Montgomery and J. Paul Scott<br />
Chapter 467 Hemostasis 1651<br />
467.1 Hemostatic Mechanism 1651<br />
467.2 The Clinical and Laboratory Evaluation 1653<br />
Chapter 468 Hereditary Clotting Factor Deficiencies<br />
(Bleeding Disorders) 1657<br />
468.1 Factor VIII or Factor IX Deficiency<br />
(Hemophilia A or B) 1657<br />
468.2 Factor XI Deficiency (HemophiliaC) 1660<br />
468.3 Deficiencies of the Contact Factors<br />
(Nonbleeding Disorders) 1660<br />
468.4 Factor VII Deficiency 1661<br />
468.5 Factor X Deficiency 1661<br />
468.6 Prothrombin (Factor II) Deficiency 1661<br />
468.7 Factor V Deficiency 1661<br />
468.8 Combined Deficiency of Factors V and VIII 1661<br />
468.9. Fibrinogen Deficiency 1661<br />
468.10 Factor XIII Deficiency (Fibrin-Stabilizing Factor or<br />
Transglutaminase Deficiency) 1661<br />
468.11 Antiplasmin or Plasminogen Activator Inhibitor<br />
(PAI) Deficiency 1662<br />
Chapter 469 von Willebrand Disease 1662<br />
Chapter 470 Hereditary Predisposition to<br />
Thrombosis 1664<br />
Chapter 471 Acquired Thrombotic Disorders 1665<br />
471.1 Anticoagulant and Thrombolytic Therapy 1666<br />
Chapter 472 Postneonatal Vitamin K<br />
Deficiency 1668<br />
Chapter 473 Liver Disease 1668<br />
Chapter 474 Acquired Inhibitors of<br />
Coagulation 1668<br />
Chapter 475 Disseminated Intravascular<br />
Coagulation (Consumptive Coagulopathy) 1669<br />
Chapter 476 Platelet and Blood Vessel<br />
Disorders 1670<br />
476.1 Idiopathic Thrombocytopenic Purpura 1670<br />
476.2 Drug-Induced Thrombocytopenia 1671<br />
476.3 Nonimmune Platelet Destruction 1671<br />
476.4 Hemolytic-Uremic Syndrome (HUS) 1671<br />
476.5 Thrombotic Thrombocytopenic Purpura (TTP) 1672<br />
476.6 Kasabach-Merritt Syndrome 1672<br />
476.7 Sequestration 1672<br />
476.8 Congenital Thrombocytopenic Syndromes 1672<br />
476.9 Neonatal Thrombocytopenia 1672<br />
476.10 Thrombocytopenia Due to Acquired Disorders<br />
Causing Decreased Production 1673<br />
476.11 Platelet Function Disorders 1673<br />
476.12 Acquired Disorders of Platelet Function 1673<br />
476.13 Congenital Abnormalities of Platelet Function 1673<br />
476.14 Disorders of the Blood Vessels 1674<br />
Section 8: The Spleen 1675<br />
James French and Bruce M. Camitta<br />
Chapter 477 Anatomy and Function of the<br />
Spleen 1675<br />
Chapter 478 Splenomegaly 1675<br />
Chapter 479 Hyposplenism, Splenic Trauma, and<br />
Splenectomy 1676<br />
Section 9: The Lymphatic System 1677<br />
Bruce M. Camitta<br />
Chapter 480 Anatomy and Function of the<br />
Lymphatic System 1677<br />
Chapter 481 Abnormalities of Lymphatic<br />
Vessels 1677<br />
Chapter 482 Lymphadenopathy 1677<br />
Part XXI Cancer and Benign Tumors 1679<br />
Chapter 483 Epidemiology of Childhood and<br />
Adolescent Cancer 1679<br />
James G. Gurney and Melissa L. Bondy<br />
Chapter 484 Molecular and Cellular Biology of<br />
Cancer 1681<br />
Laura L. Worth<br />
Chapter 485 Principles of Diagnosis 1684<br />
Archie Bleyer<br />
Chapter 486 Principles of Treatment 1688<br />
Archie Bleyer<br />
Chapter 487 The Leukemias 1694<br />
David G. Tubergen and Archie Bleyer<br />
487.1 Acute Lymphoblastic Leukemia 1694<br />
487.2 Acute Myelogenous Leukemia 1696<br />
487.3 Down Syndrome and Acute Leukemia and<br />
Myeloproliferation 1697<br />
487.4 Chronic Myelogenous Leukemia 1697<br />
487.5 Juvenile Chronic Myelogenous<br />
Leukemia 1698<br />
487.6 Infant Leukemia 1698<br />
Chapter 488 Lymphoma 1698<br />
GeraldS. Gilchrist<br />
488.1 Hodgkin Disease 1698<br />
488.2 Non-Hodgkin Lymphoma 1701<br />
Chapter 489 BrainTumors in Childhood<br />
John F. Kuttesch, Jr. and Joann L. Ater<br />
Chapter 490 Neuroblastoma<br />
Joann L. Ater<br />
1709<br />
1702<br />
Chapter 491 Neoplasms of the Kidney 1711<br />
Norman Jaffe and Vicki Huff<br />
Chapter 492 SoftTissue Sarcomas<br />
Carola A. S. Arndt<br />
1714
Chapter 493 Neoplasms of Bone 1717<br />
CarolaA. S.Arndt<br />
49 3.1 Malignant Tumors of B one 1717<br />
493.2 Benign Tumors and Tumor-like Processes of<br />
Bone 1720<br />
Chapter 494 Retinoblastoma<br />
Cynthia E. Herzog<br />
1722<br />
Chapter 495 Gonadal and Germ Cell<br />
Neoplasms 1723<br />
Cynthia E. Herzog<br />
Chapter 496 Neoplasms of the Liver<br />
Cynthia E. Herzog<br />
Chapter 497 Benign VascularTumors<br />
Cynthia E. Herzog<br />
Chapter 498 Rare Tumors 1726<br />
Cynthia E. Herzog<br />
Chapter 499 Histiocytosis Syndromes of<br />
Childhood 1727<br />
Stephan Ladisch<br />
Part XXII Nephrology 1731<br />
Section 1:Glomerular Disease<br />
Ira D. Davis and Ellis D. Avner<br />
1731<br />
Chapter 500 Introduction to Glomerular<br />
Diseases 1731<br />
500.1 Anatomy of the Glomerulus 1731<br />
500.2 Glomerular Filtration 1732<br />
500.3 Glomerular Diseases 1734<br />
1725<br />
1726<br />
Section 2: Conditions Particularly Associated with<br />
Hematuria 1735<br />
Ira D. Davis and Ellis D. Avner<br />
Chapter 501 Clinical Evaluation of the Child with<br />
Hematuria 1735<br />
Chapter 502 Isolated Glomerular Diseases with<br />
Recurrent Gross Hematuria 1737<br />
502.1 IgA Nephropathy (BergerNephropathy) 1737<br />
502.2 Alport Syndrome 1738<br />
502.3 Thin Glomerular Basement Membrane<br />
Disease 1739<br />
Chapter 503 Glomerulonephritis Associated with<br />
Infections 1740<br />
503.1 Acute Poststreptococcal Glomerulonephritis 1740<br />
503.2 Other Chronic Infections 1741<br />
Chapter 504 Membranous Glomerulopathy<br />
(Glomerulonephritis) 1741<br />
Chapter 505 Membranoproliferative<br />
(Mesangiocapillary) Glomerulonephritis 1742<br />
Chapter 506 Glomerulonephritis Associated with<br />
Systemic Lupus Erythematosus 1743<br />
Chapter 507 Henoch-Schonlein Purpura<br />
Nephritis 1744<br />
Chapter 508 Rapidly Progressive (Crescentic)<br />
Glomerulonephritis 1745<br />
Chapter 509 Goodpasture Disease 1745<br />
Chapter 510 Hemolytic-Uremic<br />
Syndrome 1746<br />
Chapter 511 Upper Urinary Tract Causes of<br />
Hematuria 1747<br />
511.1 Interstitial Nephritis 1747<br />
511.2 Toxic Nephropathy 1747<br />
511.3 Cortical Necrosis 1747<br />
511.4 Pyelonephritis 1747<br />
511.5 Nephrocalcinosis 1747<br />
511.6 Vascular Abnormalities 1747<br />
511.7 Renal Vein Thrombosis 1747<br />
511.8 Idiopathic Hypercalciuria 1748<br />
Chapter 512 Hematologic Diseases Causing<br />
Hematuria 1748<br />
512.1 Sickle Cell Nephropathy 1748<br />
512.2 Coagulopathies and<br />
Thrombocytopenia 1749<br />
Contents xli<br />
Chapter 513 AnatomicAbnormalitiesAssociated<br />
with Hematuria 1749<br />
513.1 Congenital Anomalies 1749<br />
513.2 Autosomal Recessive Polycystic Kidney<br />
Disease 1749<br />
513.3 Autosomal Dominant Polycystic Kidney<br />
Disease 1750<br />
513.4 Trauma 1750<br />
513.5 Renal Tumors 1750<br />
Chapter 514 Lower Urinary Tract Causes of<br />
Hematuria 1751<br />
514.1 Infectious Causes of Cystitis and<br />
Urethritis 1751<br />
514.2 Hemorrhagic Cystitis 1751<br />
514.3 Heavy Exercise 1751<br />
514.4 Munchausen Syndrome by Proxy 1751<br />
Section 3: Conditions Particularly Associated with<br />
Proteinuria 1751<br />
Beth A. Vogt and Ellis D. Avner<br />
Chapter 515 Introduction to the Child with<br />
Proteinuria 1751<br />
Chapter 516 Transient Proteinuria 1752<br />
Chapter 517 Orthostatic (Postural)<br />
Proteinuria 1752<br />
Chapter 518 Fixed Proteinuria 1752<br />
518.1 Glomerular Proteinuria 1752<br />
518.2 Tubular Proteinuria 1753<br />
Chapter 519 Nephrotic Syndrome 1753<br />
519.1 Idiopathic Nephrotic Syndrome 1755<br />
519.2 Secondary Nephrotic Syndrome 1757<br />
519.3 Congenital Nephrotic Syndrome 1757
Xlii Contents<br />
Section 4: Tubular Disorders 1758<br />
Chapter 520 Tubular Function 1758<br />
Katherine MacRae Dell and Ellis D. Avner<br />
Chapter 521 RenalTubularAcidosis 1758<br />
521.1 Proximal (Type II) Renal Tubular Acidosis 1759<br />
Katherine MacRae Dell and Ellis D. Avner<br />
521.2 Distal (Type I) Renal Tubular Acidosis 1760<br />
Katherine MacRae Dell and Ellis D. Avner<br />
521.3 Hyperkalemic (Type IV) Renal Tubular<br />
Acidosis 1761<br />
Katherine MacRae Dell and Ellis D. Avner<br />
521.4 Rickets Associated with Renal Tubular<br />
Acidosis 1762<br />
Russell W. Chesney<br />
Chapter 522 Nephrogenic Diabetes Insipidus 1762<br />
Katherine MacRae Dell and Ellis D. Avner<br />
Chapter 523 Bartter/Gitelman Syndromes and Other<br />
InheritedTubularTransport Abnormalities 1763<br />
Katherine MacRae Dell and Ellis D. Avner<br />
523.1 Banter Syndrome 1763<br />
523.2 Gitelman Syndrome 1764 •<br />
523.3 Other Inherited Tubular Transport<br />
Abnormalities 1764<br />
Chapter 524 Tubulointerstitial Nephritis 1764<br />
Katherine MacRae Dell and Ellis D. Avner<br />
SectionS: Toxic Nephropathies—Renal Failure<br />
Chapter 525 Toxic Nephropathy 1766<br />
Beth A. Vogt and Ellis D. Avner<br />
Chapter 526 Cortical Necrosis<br />
Beth A. Vogt and Ellis D. Avner<br />
1767<br />
Chapter 527 Renal Failure 1767<br />
Beth A. Vogt and Ellis D. Avner<br />
527.1 Acute Renal Failure 1767<br />
527.2 Chronic Renal Failure 1771<br />
527.3 End-Stage Renal Disease 1774<br />
Chapter 528 RenalTransplantation<br />
Rodrigo E. Urizar<br />
1775<br />
1766<br />
Part XXI11 Urologic Disorders in Infants and<br />
Children 1783<br />
Jack S. Elder<br />
Chapter 529 Congenital Anomalies and Dysgenesis<br />
of the Kidneys 1783<br />
Chapter 530 Urinary Tract Infections 1785<br />
Chapter 531 Vesicoureteral Reflux 1790<br />
Chapter 532 Obstructions of the Urinary Tract 1794<br />
Chapter 533 Anomalies of the Bladder 1804<br />
Chapter 534 Neuropathic Bladder 1806<br />
Chapter 535 Voiding Dysfunction 1808<br />
Chapter 536 Anomalies of the Penis and<br />
Urethra 1812<br />
Chapter 537 Disorders and Anomalies of the Scrotal<br />
Contents 1817<br />
Chapter 538 Trauma to the Genitourinary<br />
Tract 1821<br />
Chapter 539 Urinary Lithiasis 1822<br />
Part XXIV Gynecologic Problems of<br />
Childhood 1827<br />
Joseph S. Sanfilippo<br />
Chapter 540 History and Physical<br />
Examination 1827<br />
Chapter 541 Vulvovaginitis 1828<br />
Chapter 542 Bleeding 1833<br />
Chapter 543 Breast Disorders 1833<br />
Chapter 544 Hirsutism and Polycystic Ovarian<br />
Syndrome 1835<br />
Chapter 545 Neoplasms 1837<br />
Chapter 546 Vulvovaginal and Mullerian<br />
Anomalies 1839<br />
Chapter 547 Athletic Gynecologic Problems 1842<br />
Chapter 548 Special Gynecologic Needs 1843<br />
Chapter 549 Gynecologic Imaging 1843<br />
Part XXV The Endocrine System 1845<br />
Section 1: Disorders of the Hypothalamus and Pituitary<br />
Gland 1845<br />
Chapter 550 Hormones of the Hypothalamus and<br />
Pituitary 1845<br />
John S. Parks<br />
Chapter 551 Hypopituitarism<br />
John S. Parks<br />
1847<br />
Chapter 552 Diabetes Insipidus 1853<br />
David T. Breault and Joseph A. Majzoub<br />
552.1 Causes of Hypernatremia 1854<br />
Chapter 553 Other Abnormalities of Arginine<br />
Vasopressin Metabolism and Action 1855<br />
David T. Breault and Joseph A. Majzoub<br />
Chapter 554 Hyperpituitarism,Tall Stature, and<br />
Overgrowth Syndromes 1857<br />
Pinchas Cohen
Chapter 555 Physiology of Puberty<br />
Luigi Garibaldi<br />
1862<br />
Chapter 556 Disorders of Pubertal<br />
Development 1863<br />
Luigi Garibaldi<br />
556.1 Gonadotropin-Dependent Precocious<br />
Puberty 1863<br />
556.2 Precocious Puberty Resulting from Organic Brain<br />
Lesions 1865<br />
556.3 Precocious Puberty Following Irradiation of the<br />
Brain 1866<br />
556.4 Syndrome of Precocious Puberty and<br />
Hypothyroidism 1866<br />
556.5 Gonadotropin-Secreting Tumors 1867<br />
556.6 McCune-Albright Syndrome (Precocious Puberty<br />
with Polyostotic Fibrous Dysplasia and Abnormal<br />
Pigmentation) 1867<br />
556.7 Familial Male Gonadotropin-Independent<br />
Precocious Puberty 1868<br />
556.8 Incomplete (Partial) Precocious<br />
Development 1868<br />
556.9 Medicational Precocity 1869<br />
Section 2: Disorders of the Thyroid Gland 1870<br />
Stephen LaFranchi<br />
Chapter 557 Thyroid Development and<br />
Physiology 1870<br />
557.1 Thyroid Hormone Studies 1871<br />
Chapter 558 Defects of Thyroxine-Binding<br />
Globulin 1872<br />
Chapter 559 Hypothyroidism 1872<br />
Chapter 560 Thyroiditis 1879<br />
Chapter 561 Goiter 1881<br />
561.1 Congenital Goiter 1881<br />
561.2 Endemic Goiter and Cretinism<br />
561.3 Sporadic Goiter 1883<br />
561.4 Intratracheal Goiter 1883<br />
1882<br />
Chapter 562 Hyperthyroidism 1884<br />
562.1 Graves Disease 1884<br />
562.2 Congenital Hyperthyroidism 1886<br />
Chapter 563 Carcinoma of the<br />
Thyroid 1887 '<br />
563.1 Solitary Thyroid Nodule 1888<br />
563.2 Medullary Carcinoma 1889<br />
Section 3: Disorders of the Parathyroid 1890<br />
Daniel A. Doyle and Angelo M. DiGeorge<br />
Chapter 564 Hormones and Peptides of<br />
Calcium Homeostasis and Bone<br />
Metabolism 1890<br />
Chapter 565 Hypoparathyroidism 1892<br />
Chapter 566 Pseudohypoparathyroidism<br />
(Albright Hereditary Osteodystrophy) 1894<br />
Chapter 567 Hyperparathyroidism 1895<br />
Contents xliii<br />
Section 4: Disorders of the Adrenal Glands 1898<br />
Lenore S. Levine and Perrin C. White<br />
Chapter 568 The Physiology of the Adrenal<br />
Gland 1898<br />
568.1 Histology and Embryology 1898<br />
568.2 Adrenal Steroid Biosynthesis 1899<br />
568.3 Regulation of the Adrenal Cortex 1900<br />
568.4 Adrenal Steroid Hormone Actions 1901<br />
568.5 Adrenal Medulla 1904<br />
Chapter 569 Adrenocortical Insufficiency<br />
569.1 Primary Adrenal Insufficiency 1904<br />
569.2 Secondary Adrenal Insufficiency 1908<br />
1904<br />
Chapter 570 Congenital Adrenal Hyperplasia and<br />
Related Disorders 1909<br />
570.1 Congenital Adrenal Hyperplasia due to<br />
21 -Hydroxylase Deficiency 1909<br />
570.2 Congenital Adrenal Hyperplasia due to<br />
11 P-Hydroxylase Deficiency 1913<br />
570.3 Congenital Adrenal Hyperplasia due to 3(3-<br />
Hydroxysteriod Dehydrogenase Deficiency 1913<br />
570.4 Congenital Adrenal Hyperplasia due to<br />
17-Hydroxylase Deficiency 1914<br />
570.5 Lipoid Adrenal Hyperplasia 1914<br />
570.6 Aldosterone Synthase Deficiency 1915<br />
570.7 Glucocorticoid-Suppressible<br />
Hyperaldosteronism 1915<br />
Chapter 571 Cushing Syndrome 1916<br />
Chapter 572 Primary Aldosteronism 1918<br />
Chapter 573 AdrenalTumors 1918<br />
573.1 Virilizing Adrenocortical Tumors 1918<br />
573.2 Feminizing Adrenal Tumors 1919<br />
Chapter 574 Pheochromocytoma 1919<br />
Chapter 575 Adrenal Masses 1920<br />
575.1 Adrenal Incidentaloma 1920<br />
575.2 Adrenal Calcification 1921<br />
Section 5: Disorders of the Gonads 1921<br />
Robert Rapaport<br />
Chapter 576 Development and Function of the<br />
Gonads 1921<br />
Chapter 577 Hypofunction of theTestes 1924<br />
577.1 Hypergonadotropic Hypogonadism in the Male<br />
(Primary Hypogonadism) 1924<br />
5 77.2 Hypogonadotropic Hypogonadism in the Male<br />
(Secondary Hypogonadism) 1927<br />
Chapter 578 Pseudoprecocity Resulting from<br />
Tumors of theTestes 1930<br />
Chapter 579 Gynecomastia 1930<br />
Chapter 580 Hypofunction of the Ovaries 1931<br />
580.1 Hypergonadotropic Hypogonadism in the Female •<br />
(Primary Hypogonadism) 1931 ,<br />
580.2 Hypogonadotropic Hypogonadism in the Female<br />
(Secondary Hypogonadism) 1935
xliv Contents<br />
Chapter 581 Pseudoprecocity Due to Lesions of the<br />
Ovary 1937<br />
Chapter 582 Intersex 1939<br />
582.1 46,XX Intersex (46,XX with Virilization) 1940<br />
582.2 46,XYIntersex (46,XY with Undervirilization) 1940<br />
582.3 True Gonadal Intersex 1945<br />
Section 6: Diabetes Mellitus in Children<br />
Ramin Alemzadeh and David T. Wyatt<br />
1947<br />
Chapter 583 Diabetes Mellitus 1947<br />
583.1 Introduction and Classification 1947<br />
583.2 Type 1 Diabetes Mellitus (Immune<br />
Mediated) 1948<br />
583.3 Type 2 Diabetes Mellitus 1967<br />
583.4 Other Specific Types of Diabetes 1968<br />
Part XXVI The Nervous System 1973<br />
Chapter 584 Neurologic Evaluation<br />
Robert H. A. Haslam<br />
1973<br />
Chapter 585 Congenital Anomalies of the Central<br />
Nervous System 1983<br />
Michael V. Johnston and Stephen Kinsman<br />
585.1 Neural Tube Defects (Dysraphism) 1983<br />
585.2 Spina Bifida Occulta 1983<br />
585.3 Meningocele 1983<br />
585.4 Myelomeningocele 1984<br />
585.5 Encephalocele 1985<br />
585.6 Anencephaly 1986<br />
585.7 Disorders of Neuronal Migration 1986<br />
585.8 Agenesis of the Corpus Callosum 1987<br />
585.9 Agenesis of the Cranial Nerves 1988<br />
585.10 Microcephaly 1988<br />
585.11 Hydrocephalus 1989<br />
585.12 Craniosynostosis 1992<br />
Chapter 586 Seizures in Childhood 1993<br />
Michael V. Johnston<br />
586.1 Febrile Seizures 1994<br />
586.2 Unprovoked Seizures 1994<br />
586.3 Partial Seizures 1995<br />
586.4 Generalized Seizures 1997<br />
586.5 Mechanisms of Seizures 1999<br />
586.6 Treatment of Epilepsy 1999<br />
586.7 Neonatal Seizures 2005<br />
586.8 Status Epilepticus 2007<br />
Chapter 587 Conditions that Mimic Seizures 2009<br />
Michael V. Johnston<br />
Chapter 588 Headaches 2012<br />
Robert H. A. Haslam<br />
588.1 Migraine 2012<br />
588.2 Organic Headaches 2014<br />
588.3 Tension or Stress Headaches 2015<br />
Chapter 589 Neurocutaneous Syndromes<br />
Robert H. A. Haslam<br />
589.1 Neurofibromatosis 2015<br />
589.2 Tuberous Sclerosis 2017<br />
2015<br />
589.3 Sturge-Weber Disease 2017<br />
589.4 von Hippel-Lindau Disease 2019<br />
589.5 Linear Nevus Syndrome 2019<br />
589.6 PHACE Syndrome 2019<br />
Chapter 590 Movement Disorders 2019<br />
Michael V. Johnston<br />
590.1 Ataxias 2019<br />
590.2 Chorea, Athetosis, Tremor 2020<br />
590.3 Dystonia 2022<br />
590.4 Tics 2023<br />
Chapter 591 Encephalopathies 2023<br />
Michael V. Johnston<br />
591.1 Cerebral Palsy 2024<br />
591.2 MitochondrialEncephalomyopathies 2025<br />
591.3 Other Encephalopathies 2027<br />
Chapter 592 Neurodegenerative Disorders of<br />
Childhood 2029<br />
Michael V. Johnston<br />
592.1 Sphingolipidoses 2029<br />
592.2 Neuronal Ceroid Lipofuscinoses 2032<br />
592.3 Adrenoleukodystrophy 2032<br />
592.4 Sialidosis 2033<br />
592.5 Miscellaneous Disorders 2033<br />
Chapter 593 Acute Stroke Syndromes 2035<br />
Michael V. Johnston<br />
593.1 Arterial Thrombosis/Embolism 2035<br />
593.2 Venous Thrombosis 2036<br />
593.3 Intracranial Hemorrhage 2036<br />
593.4 Differential Diagnosis of Strokelike<br />
Events 2037<br />
Chapter 594 Central Nervous System<br />
Infections 2038<br />
Charles G. Prober<br />
594.1 Acute Bacterial Meningitis Beyond the Neonatal<br />
Period 2038<br />
594.2 Viral Meningoencephalitis 2044<br />
594.3 Eosinophilic Meningitis 2046<br />
Chapter 595 Brain Abscess<br />
Robert H. A. Haslam<br />
2047<br />
Chapter 596 Pseudotumor Cerebri<br />
Robert H. A. Haslam<br />
2048<br />
Chapter 597 Spinal Cord Disorders 2049<br />
Robert H. A. Haslam<br />
597.1 Spinal Cord Tumors 2049<br />
597.2 Spinal Cord Trauma 2050<br />
597.3 Tethered Cord 2050<br />
597.4 Diastematomyelia 2051<br />
597.5 Syringomyelia 2051<br />
597.6 Transverse Myelitis 2051<br />
597.7 Arteriovenous Malformation 2052<br />
Part XXVII Neuromuscular Disorders 2053<br />
Harvey B. Sarnat<br />
Chapter 598 Evaluation and Investigation 2053
Chapter 599 Developmental Disorders of<br />
Muscle 2055<br />
599.1 Myotubular Myopathy 2056<br />
599.2 Congenital Muscle Fiber-Type Disproportion<br />
(CMFTD) 2057<br />
599.3 Nemaline Rod Myopathy 2058<br />
599.4 Central Core Disease 2059<br />
599.5 Brain Malformations and Muscle<br />
Development 2059<br />
599.6 Amyoplasia 2059<br />
599.7 Muscular Dysgenesis (Proteus Syndrome<br />
Myopathy) 2059<br />
599.8 Benign Congenital Hypotonia 2059<br />
599.9 Arthrogryposis 2059<br />
Chapter 600 Muscular Dystrophies 2060<br />
600.1 Duchenne and Becker Muscular<br />
Dystrophies -2060<br />
600.2 Emery-Dreifuss Muscular Dystrophy 2064<br />
600.3 Myotonic Muscular Dystrophy 2064<br />
600.4 Limb-Girdle Muscular Dystrophy 2066<br />
600.5 Facioscapulohumeral Muscular<br />
Dystrophy • 2067<br />
600.6 Congenital Muscular Dystrophy 2067<br />
Chapter 601 Endocrine Myopathies 2069<br />
Chapter 602 Metabolic Myopathies 2070<br />
602.1 Periodic Paralyses (Potassium-Related) 2070<br />
602.2 Malignant Hyperthermia -2070<br />
602.3 Glycogenoses 2070<br />
602.4 Mitochondrial Myopathies 2071<br />
602.5 Lipid Myopathies 2072<br />
602.6 Vitamin E Deficiency Myopathy 2072<br />
Chapter 603 Disorders of Neuromuscular<br />
Transmission and of Motor Neurons 2072<br />
603.1 Myasthenia Gravis 2072<br />
603.2 Spinal Muscular Atrophies 2075<br />
603.3 Other Motor Neuron Diseases 2076<br />
Chapter 604 Hereditary Motor-Sensory<br />
Neuropathies 2076<br />
604.1 Peroneal Muscular Atrophy (Charcot-Marie-Tooth<br />
Disease; HMSN Type I) 2076<br />
604.2 Peroneal Muscular Atrophy<br />
(Axonal Type) 2077<br />
604.3 Dejerine-Sottas Disease (HMSN Type III) 2077<br />
604.4 Roussy-Levy Syndrome 2077<br />
604.5 Refsum Disease 2077<br />
604.6 Fabry Disease 2077<br />
604.7 Giant Axonal Neuropathy 2078<br />
604.8 Congenital Hypomyelinating<br />
Neuropathy 2078<br />
604.9 Tomaculous Neuropathy 2078<br />
604.10 Leukodystrophies 2078<br />
Chapter 605 Toxic Neuropathies 2078<br />
Chapter 606 Autonomic Neuropathies 2079<br />
606.1 Familial Dysautonomia 2079<br />
606.2 Other Autonomic Neuropathies 2079<br />
Chapter 607 Guillain-Barre Syndrome 2080<br />
Chapter 608 Bell Palsy 2081<br />
PartXXVIII Disorders of the Eye 2083<br />
Scott E. Olitsky and Leonard B. <strong>Nelson</strong><br />
Contents xlv<br />
Chapter 609 Growth and Development 2083<br />
Chapter 610 Examination of the Eye 2084<br />
Chapter 611 Abnormalities of Refraction and<br />
Accommodation 2086<br />
Chapter 612 Disorders of Vision 2087<br />
Chapter 613 Abnormalities of Pupil and Iris 2089<br />
Chapter 614 Disorders of Eye Movement and<br />
Alignment 2092<br />
Chapter 615 Abnormalities of the Lids 2097<br />
Chapter 616 Disorders of the Lacrimal<br />
System 2099<br />
Chapter 617 Disorders of the Conjunctiva 2099<br />
Chapter 618 Abnormalities of the Cornea 2103<br />
Chapter 619 Abnormalities of the Lens 2105<br />
Chapter 620 Disorders of the UvealTract 2111<br />
Chapter 621 Disorders of the Retina and<br />
Vitreous 2113<br />
Chapter 622 Abnormalities of the Optic<br />
Nerve 2119<br />
Chapter 623 Childhood Glaucoma 2122<br />
Chapter 624 Orbital Abnormalities 2123<br />
Chapter 625 Injuries to the Eye 2124<br />
Part XXIX The Ear 2127<br />
Chapter 626 Clinical Manifestations 2127<br />
Joseph Haddad Jr.<br />
Chapter 627 Hearing Loss 2129<br />
Joseph Haddad Jr.<br />
Chapter 628 Congenital Malformations 2135<br />
Joseph Haddad Jr.<br />
Chapter 629 Diseases of the External Ear 2136<br />
Joseph Haddad Jr.<br />
Chapter 630 Otitis Media<br />
Jack L. Paradise<br />
2138<br />
Chapter 631 The Inner Ear and Diseases of the Bony<br />
Labyrinth 2149 i<br />
Joseph Haddad Jr. \<br />
Chapter 632 Traumatic Injuries of the Ear and ,<br />
Temporal Bone 2150<br />
Joseph Haddad Jr.
xlvi Contents<br />
Chapter 633 Tumors of the Ear andTemporal<br />
Bone 2151<br />
Joseph Haddad Jr.<br />
The Skin 2153<br />
Gary L. Darmstadt and Robert Sidbury<br />
Chapter 634 Morphology of the Skin 2153<br />
Chapter 635 Evaluation of the Patient 2154<br />
635.1 Cutaneous Manifestations of Systemic<br />
Diseases 2155<br />
Chapter 636 Principles of Therapy 2159<br />
Chapter 637 Diseases of the Neonate 2161<br />
Chapter 638 Cutaneous Defects 2164<br />
Chapter 639 Ectodermal Dysplasias 2166<br />
Chapter 640 Vascular Disorders 2167<br />
Chapter 641 Cutaneous Nevi 2172<br />
Chapter 642 Hyperpigmented Lesions 2176<br />
Chapter 643 Hypopigmented Lesions 2178<br />
Chapter 644 Vesiculobullous Disorders 2181<br />
Chapter 645 Eczematous Disorders 2188<br />
Chapter 646 Photosensitivity 2191<br />
Chapter 647 Diseases of the Epidermis 2195<br />
Chapter 648 Disorders of Keratinization 2200<br />
Chapter 649 Diseases of the Dermis 2204<br />
Chapter 650 Diseases of Subcutaneous<br />
Tissue 2210<br />
Chapter 651 Disorders of the Sweat<br />
Glands 2213<br />
Chapter 652 Disorders of Hair 2215<br />
Chapter 653 Disorders of the Nails 2219<br />
Chapter 654 Disorders of the Mucous<br />
Membranes 2220<br />
Chapter 655 Cutaneous Bacterial<br />
Infections 2222<br />
Chapter 656 Cutaneous Fungal Infections 2230<br />
Chapter 657 Cutaneous Viral Infections 2235<br />
Chapter 658 Arthropod Bites and<br />
Infestations 2237<br />
Chapter 659 Acne 2242<br />
Chapter 660 Tumors of the Skin 2246<br />
Chapter 661 Nutritional Dermatoses 2248<br />
Part XXXI Bone and Joint Disorders 2251<br />
Section 1: Orthopedic Problems 2251<br />
Chapter 662 Growth and Development<br />
George H. Thompson<br />
Chapter 663 Evaluation of the Child 2251<br />
George H. Thompson<br />
2251<br />
Chapter 664 The Foot and Toes 2254<br />
George H. Thompson<br />
664.1 Metatarsus Adductus 2255<br />
664.2 Calcaneovalgus Feet 2255<br />
664.3 Talipes Equinovarus (Clubfoot) 2256<br />
664.4 Congenital Vertical Talus 2256<br />
664.5 Hypermobile Pes Planus (Flexible Flatfeet) 2257<br />
664.6 Tarsal Coalition 2257<br />
664.7 CavusFeet 2257<br />
664.8 Osteochondroses 2257<br />
664.9 Puncture Wounds of the Foot 2258<br />
664.10 Toe Deformities 2258<br />
664.11 Painful Foot 2260<br />
664.12 Shoes 2260<br />
Chapter 665 Torsional and Angular<br />
Deformities 2261<br />
George H. Thompson<br />
665.1 Normal Developmental Alignment 2261<br />
665.2 Internal Femoral Torsion 2262<br />
665.3 Internal Tibial Torsion 2263<br />
665.4 External Femoral Torsion 2263<br />
665.5 External Tibial Torsion 2264<br />
665.6 Genu Varum (Bowlegs) 2264<br />
665.7 Genu Valgum (Knock-Knees) 2266<br />
665.8 Congenital Angular Deformities of the Tibia and<br />
Fibula 2269<br />
Chapter 666 Leg-Length Discrepancy<br />
George H. Thompson<br />
2270<br />
Chapter 667 The Knee<br />
George H. Thompson<br />
2271<br />
667.1 Discoid Lateral Meniscus 2272<br />
667.2 Popliteal Cyst 2272<br />
667.3 Osteochondritis Dissecans 2272<br />
667.4 Osgood-Schlatter Disease 2272<br />
667.5 Idiopathic Adolescent Anterior Knee Pain<br />
Syndrome 2272<br />
667.6 Patella Subluxation and Dislocation 2273<br />
Chapter 668 The Hip 2273<br />
George H. Thompson<br />
668.1 Developmental Dysplasia of the Hip 2273<br />
668.2 Transient Monoarticular Synovitis 2276<br />
668.3 Legg-Calve-Perthes Disease 2276<br />
668.4 Slipped Capital Femoral Epiphysis 2278<br />
i<br />
Chapter 669 The Spine 2280 \<br />
George H. Thompson i<br />
669.1 Idiopathic Scoliosis 2280 »<br />
669.2 Congenital Scoliosis 2282<br />
669.3 Neuromuscular Scoliosis, Genetic Syndromes, and<br />
Compensatory Scoliosis 2283
669.4 Kyphosis (Round-Back) 2284<br />
669.5 Back Pain in Children 2285<br />
669.6 Spondylolysis and Spondylolisthesis<br />
669.7 Disk Space Infection 2286<br />
669.8 Intervertebral Disk Herniation 2287<br />
669.9 Tumors 2287<br />
Chapter 670 The Neck 2288<br />
George H. Thompson<br />
670.1 Torticollis 2288<br />
670.2 Klippel-Feil Syndrome<br />
670.3 Atlantoaxial Instability<br />
2289<br />
2289<br />
2285<br />
Chapter 671 The Upper Limb<br />
George H. Thompson<br />
2290<br />
671.1 Sprengel Deformity 2290<br />
671.2 Shoulder Dislocation 2290<br />
671.3 Nursemaid's Elbow 2290<br />
671.4 Panner Disease 2291<br />
671.5 Ganglion 2291<br />
671.6 Radial Clubhand 2291<br />
671.7 Polydactyly 2292<br />
671.8 Syndactyly' 2292<br />
671.9 Congenital Trigger Thumb and Finger 2292<br />
Chapter 672 Arthrogryposis<br />
George H. Thompson<br />
672.1 Amyoplasia 2292<br />
2292<br />
Chapter 673 Common Fractures 2294<br />
George H. Thompson<br />
673.1 Pediatric Fracture Patterns 2294<br />
673.2 Clavicular Fractures 2295<br />
673.3 Proximal Humerus Fractures 2295<br />
672.4 Distal Humerus Fractures 2295<br />
673.5 Distal Radius and Ulna Fractures 2296<br />
673.6 Phalangeal Fractures 2296<br />
673.7 Toddler Fracture 2296<br />
673.8 Lateral Malleolar Fractures 2296<br />
673.9 Metatarsal Fractures 2296<br />
673.10 Toe Phalangeal Fractures 2296<br />
673.11 Operative Treatment 2297<br />
Chapter 674 Osteomyelitis and Suppurative<br />
Arthritis 2297<br />
<strong>Richard</strong> M. Lampe<br />
Section 2: Sports Medicine 2302<br />
Albert C. Hergenroed'er and Joseph N. Chorley<br />
Chapter 675 Epidemiology and<br />
Prevention of Injuries 2302<br />
Chapter 676 Management of Musculoskeletal<br />
Injury 2306<br />
676.1 Growth Plate Injuries 2307<br />
676.2 Shoulder Injuries 2308<br />
676.3 Elbow Injuries 2308<br />
676.4 Back Injuries 2309<br />
676.5 Hip and Pelvis Injuries 2310<br />
676.6 Knee Injuries 2310<br />
676.7 Shin Splints and Stress Fractures of the<br />
Lower Leg 2312<br />
676.8 Ankle Injuries 2312<br />
676.9 Foot Injuries 2313<br />
Chapter 677 Head and Neck Injuries<br />
Chapter 678 Heat Injuries 2314<br />
Contents<br />
2313<br />
Chapter 679 Female Athletes: Menstrual Problems<br />
and the Risk for Osteopenia 2315<br />
Chapter 680 ErgogenicAids 2316<br />
Chapter 681 Specific Sports and Associated<br />
Injuries 2316<br />
Section 3: The Skeletal Dysplasias 2320<br />
Chapter 682 General Considerations 2320<br />
William A. Horton and Jacqueline T. Hecht<br />
Chapter 683 Disorders Involving Cartilage Matrix<br />
Proteins 2324<br />
William A. Horton and Jacqueline T. Hecht<br />
Chapter 684 Disorders InvolvingTransmembrane<br />
Receptors 2328<br />
William A. Horton and Jacqueline T. Hecht<br />
xlvii<br />
Chapter 685 Disorders Involving lonTransporter 2330<br />
William A. Horton and Jacqueline T. Hecht<br />
Chapter 686 Disorders InvolvingTranscription<br />
Factors 2332<br />
William A. Horton and Jacqueline T. Hecht<br />
Chapter 687 Disorders Involving Defective Bone<br />
Resorption 2333<br />
William A. Horton and Jacqueline T. Hecht<br />
Chapter 688 Disorders for Which Defects Are Poorly<br />
Understood or Unknown 2334<br />
William A. Horton and Jacqueline T. Hecht<br />
Chapter 689 Osteogenesis Imperfecta<br />
John C. Marini<br />
Chapter 690 Marfan Syndrome 2338<br />
Luther K. Robinson<br />
Section 4: Metabolic Bone Disease 2341<br />
Russell W. Chesney<br />
2336<br />
Chapter 691 Bone Structure, Growth, and Hormonal<br />
Regulation 2341<br />
Chapter 692 Primary Chondrodystrophy<br />
(Metaphyseal Dysplasia) 2343<br />
Chapter 693 Idiopathic Hypercalcemia 2343<br />
Chapter 694 Hypophosphatasia 2344<br />
Chapter 695 Hyperphosphatasia 2344<br />
Chapter 696 Familial Hypophosphatemia<br />
(Vitamin D-Resistant Rickets, X-Linked *<br />
Hypophosphatemia) 2345 [<br />
Chapter 697 Vitamin D-Dependent Rickets \<br />
(Pseudovitamin D Deficiency, Hypocalcemic<br />
Vitamin D-Resistant Rickets) 2346
xlviii Contents<br />
Chapter 698 Oncogenous Rickets (Primary<br />
Hypophosphatemic Rickets Associated with<br />
Tumor) 2346<br />
Chapter 699 Osteoporosis 2347<br />
Part XXXII Environmental Health<br />
Hazards 2349<br />
Chapter 700 Pediatric Radiation Injuries<br />
Fred A. Mettler, Jr. and Madelyn M. Stazzone<br />
Chapter 701 Chemical Pollutants<br />
Philip J. Landrigan and Joel A. Forman<br />
2353<br />
2349<br />
Chapter 702<br />
Collin S. Goto<br />
Heavy Metal Intoxication 2355<br />
Chapter 703 Lead Poisoning<br />
Morri Markowitz<br />
2358<br />
Chapter 704 Poisonings: Drugs, Chemicals, and<br />
Plants 2362<br />
George C. Rodgers, Jr. and Nancy J. Matyunas<br />
704.1 Epidemiology and Approach to<br />
Management 2362<br />
704.2 Acetaminophen 2366<br />
704.3 Salicylates 2367<br />
704.4 Ibuprofen 2368<br />
704.5 Antidepressants 2368<br />
704.6 Clonidine 2369<br />
704.7 Calcium Channel Blockers 2370<br />
704.8 Iron 2370<br />
704.9 Caustics 2371<br />
704.10 Methanol and Ethylene Glycol 2371<br />
704.11 Hydrocarbons 2372<br />
704.12 Cholinesterase-Inhibiting Insecticides 2373<br />
704.13 Toxic Gases 2374<br />
704.14 Plants 2374<br />
Chapter 705 Nonbacterial Food Poisoning 2375<br />
DeniseA. Salerno and Stephen C. Aronoff<br />
705.1 Mushroom Poisoning 2375<br />
705.2 Solanine Poisoning 2376<br />
705.3 Seafood Poisoning 2377<br />
Chapter 706 Biologic and ChemicalTerrorism<br />
Theodore J. Cieslak and Fred M. Henretig<br />
Chapter 707 Animal and Human Bites<br />
Charles M. Ginsburg<br />
Chapter 708<br />
Steve Holve<br />
Envenomations 2387<br />
2385<br />
Laboratory Medicine, Drug<br />
Therapy, and Reference<br />
Tables 2393<br />
Chapter 709 Laboratory Testing in Infants and<br />
Children 2393<br />
John F. Nicholson and Michael A. Pesce<br />
2378<br />
Chapter 710 Reference Ranges for Laboratory Tests<br />
and Procedures 2396<br />
John F. Nicholson and Michael A. Pesce<br />
Chapter 711 Principles of DrugTherapy<br />
Michael D. Reed and Peter Gal<br />
Chapter 712 Medications 2432<br />
Peter Gal and Michael D. Reed<br />
Chapter 713 Herbal Medicines<br />
Kathi J. Kemper and Paula Gardiner<br />
Index 2507<br />
2502<br />
2427
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