Professional Documents
Culture Documents
PEDIATRICS
Richard E. Behrman, MD
Executive Chair
Federation of Pediatric Organizations, Inc.
Pediatric Education Steering Committee
Palo Alto, California
and
Clinical Professor of Pediatrics
Stanford University School of Medicine
University of California, San Francisco, School of Medicine
Robert M. Kliegman, MD
Professor and Chair
Department of Pediatrics
Medical College of Wisconsin
Pediatrician in Chief
Pam and Les Muma Chair in Pediatrics
Children's Hospital of Wisconsin
Milwaukee, Wisconsin
Hal B. Jenson, MD
Professor and Chair, Department of Pediatrics
Director, Center for Pediatric Research
Eastern Virginia Medical School and Children's Hospital of The King's Daughters
Senior Vice President for Academic Affairs
Children's Hospital of The King's Daughters
Norfolk, Virginia
17th Edition
SAUNDERS
An Imprint of Elsevier Science
Contents
Chapter 101 Depression 646 Section 2: The T-, B-, and NK-Cell Systems 683
Renee R. Jenkins Rebecca H. Buckley
XXViii Contents
Section 3: The Phagocytic System 701 Chapter 134 Childhood Asthma 760
Andrew H. Liu, Joseph D. Spahn, and Donald Y. M. Leung
Chapter 117 Neutrophils 701
Laurence A. Boxer Chapter 135 Atopic Dermatitis (Atopic
Eczema) 774
Chapter 118 Monocytesand Macrophages 707 Donald Y. M. Leung
Richard B. Johnston, Jr.
Chapter 136 Urticaria and Angioedema
Chapter 119 Eosinophils 709 (Hives) 778
Laurence A. Boxer Donald Y. M. Leung
Chapter 120 Disorders of Phagocyte Function 710 Chapter 137 Anaphylaxis 781
Laurence A. Boxer Hugh A. Sampson and Donald Y. M. Leung
Chapter 121 Leukopenia 717 Chapter 138 Serum Sickness 782
Laurence A. Boxer Scott H. Sicherer and Donald Y. M. Leung .
Chapter 122 Leukocytosis 723 Chapter 139 Adverse Reactions to Drugs 783
Laurence A. Boxer Mark Boguniewicz
Section 4: The Complement System 725 Chapter 140 Insect Allergy 786
Scott H. Sicherer and Donald Y. M. Leung
Richard B. Johnston, Jr.
Chapter 141 Ocular Allergies 788
Chapter 123 The Complement System 725 Mark Boguniewicz
Chapter 124 Disorders of the Complement
Chapter 142 Adverse Reactions to Foods 789
System 728
Hugh A. Sampson and Donald Y. M. Leung
124.1 Evaluation 728
124.2 Primary Deficiencies of Complement
Components 728
124.3 Deficiencies of Plasma, Membrane, or Serosal Rheumatic Diseases of Childhood
Complement Control Proteins 730
124.4 Secondary Disorders of Complement 730 (Connective Tissue Diseases,
124.5 Treatment of Complement Disorders 731 Collagen Vascular Diseases) 793
Section 5: Hematopoietic Stem Cell
Transplantation 732 Chapter 143 Evaluation of Suspected Rheumatic
Disease 793
Kent A. Robertson
Michael L. Miller
Chapter 125 Clinical Indications 732 Chapter 144 Treatment of Rheumatic
Chapter 126 Matching and Rejection 737 Diseases 795
Daniel J. Lovell, Michael L. Miller, and James T. Cassidy
Chapter 127 Graft versus Host Disease (GVHD) 738
Chapter 145 Juvenile Rheumatoid Arthritis 799
Chapter 128 Principles of Immuno- Michael L. Miller and James T. Cassidy
suppression 740
Chapter 146 Ankylosing Spondylitis and Other
Chapter 129 Late Effects of Stem Cell Spondyloarthropathies 805
Transplantation 741 Michael L. Miller and Ross E. Petty
Contents xxix
Chapter 147 Postinfectious Arthritis and Related Chapter 164 Infections in Immunocompromised
Conditions 808 Persons 851
Michael L. Miller and James T. Cassidy Marian G. Michaels and Michael Green
Chapter 148 Systemic Lupus Erythematosus 809 Chapter 165 Infection Associated with Medical
Marisa S. Klein-Gitelman and Michael L. Miller Devices 858
148.1 Neonatal Lupus , 813 Patricia M. Flynn and Fred F. Barrett
Chapter 149 Juvenile Dermatomyositis 813 Section 2: Gram-Positive Bacterial Infections 861
Lauren M. Pachman
Chapter 166 Staphylococcus 861
Chapter 150 Scleroderma 816
James K. Todd
Michael L. Miller
166.1 Staphylococcus aureus 861
Chapter 151 Behqet Disease 819 166.2 Toxic Shock Syndrome 865
Abraham Gedalia 166.3 Coagulase-Negative Staphylococci 866
Chapter 152 Sjogren Syndrome 820 Chapter 167 Streptococcus pneumoniae
Abraham Gedalia (Pneumococcus) 867
Jon S. Abramson and Gary D. Overturf
Chapter 153 Familial Mediterranean Fever 821
Abraham Gedalia Chapter 168 Group A Streptococcus 870
Michael A. Gerber
Chapter 154 Amyloidosis 822
Abraham Gedalia 168.1 Rheumatic Fever 874
Chapter 181 Salmonella 912 Chapter 196 Other Anaerobic Infections 954
Thomas G. Cleary Margaret C. Fisher
181.1 Nontyphoidal Salmonellosis 912
181.2 Enteric Fever 916 Section 5: Mycobacterial Infections 958
Cl Chapter 248 OtherViral Hemorrhagic Fevers 1096 Chapter 266 Toxoplasmosis (Toxoplasma
Scott B. Halstead gondii) 1144
Rima McLeod and Jack S. Remington
Chapter 249 Lymphocytic Choriomeningitis
Virus 1099 Chapter 267 Pneumocystis carinii 1154
Cl Hal B. Jenson Walter T. Hughes
Chapter 284 Child Care and Communicable Chapter 301 Obstructing and Motility Disorders of
Diseases 1187 the Esophagus 1220
Larry K. Pickering
Chapter 302 Dysmotility 1220
Chapter 285 Health Advice for ChildrenTraveling
Internationally 1189 Chapter 303 Hiatal Hernia 1221
Chandy C. John and Robert A. Salata Chapter 304 Gastroesophageal Reflux Disease
(GERD) 1222
304.1 Complications of GERD 1224
Part XVII The Digestive System 1197 Chapter 305 Non-GERDEsophagitis 1224
Chapter 318 Food Allergy (Food Chapter 330 Disorders of the Exocrine
Hypersensitivity) 1255 Pancreas 1299
Jeffrey Hyams
Chapter 331 Treatment of Pancreatic
Chapter 319 Eosinophilic Gastroenteritis 1257 Insufficiency 1300
Jeffrey Hyams
Chapter 332 Pancreatitis 1300
Chapter 320 Malabsorptive Disorders 1257 332.1 Acute Pancreatitis 1300
Manuel Garda-Careaga and John A. Kerner 332.2 Chronic Pancreatitis 1302
320.1 Evaluation of Children with Suspected Intestinal
Malabsorption 1257 Chapter 333 Pseudocyst of the Pancreas 1303
320.2 Chronic Malnutrition 1261
320.3 Liver and Biliary Disorders 1261 Chapter 334 PancreaticTumors 1303
320.4 Intestinal Infections 1261 Section 6: The Liver and Biliary System 1304
320.5 Immunodeficiency 1262
320.6 Stagnant Loop Syndrome (Blind Loop Syndrome: Chapter 335 Development and Function of the Liver
Bacterial Overgrowth Syndrome) 1262 and Biliary System 1304
320.7 Short Bowel Syndrome 1262 Michael D. Bates and William F. Balistreri
320.8 Gluten-Sensitive Enteropathy (Celiac Disease,
Celiac Sprue) 1264 Chapter 336 Manifestations of Liver Disease 1308
320.9 Immunoproliferative Small Intestinal Vicky Lee Ng and William F. Balistreri
Disease 1266 336.1 Evaluation of Patients with Possible Liver
320.10 Other Malabsorptive Snydromes 1266 Dysfunction 1312
320.11 Enzyme Deficiencies 1267 Chapter 337 Cholestasis 1314
320.12 Defects of Absorption or Transport 1268 Hassan H. A-Kaderand William F. Balistreri
Chapter 321 Gastroenteritis 1272 337.1 Neonatal Cholestasis 1314
Larry K. Pickering and John D. Snyder 337.2 Cholestasis in the Older Child 1319
Chapter 322 Chronic Diarrhea 1276 Chapter 338 Metabolic Diseases of the Liver 1319
Fayez K. Ghishan Jeffrey A. Rudolph and William F. Balistreri
338.1 Inherited Deficient Conjugation of Bilirubin
Chapter 323 Recurrent Abdominal Pain of (Familial Nonhemolytic Unconjugated
Childhood 1281 Hyperbilirubinemia) 1320
Robert Wyllie 338.2 Wilson Disease 1321
Chapter 324 Acute Appendicitis 1283 338.3 Hepatic Copper Overload Syndrome 1323
Gary E. Hartman
338.4 Indian Childhood Cirrhosis 1323
338.5 Neonatal Iron Storage Disease (NISD) 132 3
Chapter 325 Surgical Conditions of the Anus, 338.6 Miscellaneous Metabolic Diseases of the Liver 1323
Rectum, and Colon 1285 Chapter 339 Viral Hepatitis 1324
Alberto Pena
John D. Snyder and Larry K. Pickering
325.1 Anorectal Malformations 1285 339.1 Hepatitis in Neonates 1331
325.2 Anal Fissure 1288
325.3 Perianal Abscess and Fistula 1288 Chapter 340 Liver Abscess 1332
325.4 Hemorrhoids 1289 Jeffrey Schwimmer and William F. Balistreri
Contents XXXV
Chapter 341 Liver Disease Associated with Systemic Chapter 356 Regulation of Respiration 1359
Disorders 1333 Gabriel G. Haddad
Jeffrey Schwimmer and William F. Balistreri
Chapter 357 Respiratory Pathophysiology 1362
Chapter 342 Reye Syndrome and the Mitochondrial J. Julio Perez Fontdn and Gabriel G. Haddad
Hepatopathies 1335 357.1 Respiratory Failure 1370
Jeffrey A. Rudolph and William F. Balistreri J. Julio Perez Fontdn and Gabriel G. Haddad
357.2 Congenital Central Hypoventilation Syndrome
Chapter 343 Autoimmune (Chronic) Hepatitis 1337 (Ondine Curse) 1372
Benjamin L. Shneider and Frederick J. Suchy Gabriel G. Haddad
Chapter 344 Drug- andToxin-lnduced Liver Chapter 358 Defense Mechanisms and Metabolic
Injury 1339 Functions of the Lung 1373
Frederick J. Suchy Gabriel G. Haddad and J. Julio Perez Fontdn
358.1 Defense Mechanisms 1373
Chapter 345 Fulminant Hepatic Failure 1341 358.2 Metabolic Functions 1375
Frederick J. Suchy
Chapter 359 Diagnostic Approach to Respiratory
Chapter 346 Cystic Diseases of the Biliary Tract and
Disease 1375
Liver 1343 Gabriel G. Haddad and Regina M. Palazzo
Frederick J. Suchy
Chapter 360 Sudden Infant Death Syndrome 1380
Chapter 347 Diseases of the Gallbladder 1345 Carl E. Hunt and Fern R. Hauck
Frederick J. Suchy
Section 2: Upper Respiratory Tract 1386
Chapter 348 Portal Hypertension and
Varices 1346 Chapter 361 Congenital Disorders of the
Frederick J. Suchy Nose 1386
Joseph Haddad Jr.
Chapter 349 Liver Transplantation 1349
Kenneth L. Cox Chapter 362 Acquired Disorders of the
Nose 1387
Section 7: Peritoneum 1351
Joseph Haddad Jr.
Chapter 350 Malformations 1351 362.1 Foreign Body 1387
Jeffrey S: Hyams 362.2 Epistaxis 1387
: XVIII: The Respiratory System 1357 Section 3: Disorders of the Lungs and Lower
Airways 1401
Section 1: Development and Function 1357 Thomas P. Green and Susanna A. McColley
Chapter 355 Development of the Respiratory Chapter 370 Chronic or Recurrent Respiratory,
System 1357 Symptoms 1401 '•
Gabriel G. Haddad and J. Julio Perez Fontdn Thomas F. Boat '
XXXVI Contents
Chapter 371 Acute Inflammatory Upper Airway Chapter 381 o^-Antitrypsin Deficiency and
Obstruction 1405 Emphysema 1421
Genie E. Roosevelt Glenna B. Winnie and Steven Boas
371 :i Infectious Upper Airway Obstruction 1405
371.2 Bacterial Tracheitis 1409 Chapter 382 Other Distal Airway Diseases 1422
Steven Boas
Chapter 372 Congenital Anomalies of the 382.1 Bronciolitis Obliterans 1422
Larynx 1409 382.2 Follicular Bronchitis 1422
Lauren D. Holinger 382.3 Pulmonary Alveolar Microlithiasis 1422
372.1 Laryngomalacia 1409
Chapter 383 Congenital Disorders of the
372.2 Congenital Subglottic Stenosis 1409
372.3 Vocal Cord Paralysis 1410 Lung 1423
Thomas P. Green and Jonathan D. Finder
372.4 Congenital Laryngeal Webs and Atresia 1410
372.5 Congenital SubglotticHemangioma 1410 383.1 Pulmonary Agenesis and Aplasia 1423
372.6 Laryngoceles and Saccular Cysts 1410 383.2 Pulmonary Hypoplasia 1423
372.7 Posterior Laryngeal Cleft and 383.3 Cystic Adenomatoid Malformation 1424
Laryngotracheoesophageal Cleft 1410 383.4 Pulmonary Sequestration 1424
383.5 Bronchogenic Cysts 1425
Chapter 373 Foreign Bodies of the Airway 1410 383.6 Congenital Pulmonary Lymphangiectasia 1425
Lauren D. Holinger 383.7 Lung Hernia 1425
373.1 Laryngeal Foreign B odies 1411 383.8 Other Congenital Malformations of
373.2 Tracheal Foreign Bodies 1411 the Lung 1425
373.3 Bronchial Foreign Bodies 1411
Chapter 384 Pulmonary Edema 1426
Chapter 374 Laryngotracheal Stenosis, Subglottic Robert Mazor and Thomas P. Green
Stenosis 1412
Lauren D. Holinger
Chapter 385 Aspiration Syndromes 1427
John L. Colombo
374.1 Congenital Subglottic Stenosis 1412
374.2 AcquiredXaryngotracheal Stenosis 1412 Chapter 386 Chronic Recurrent Aspiration 1428
John L. Colombo
Chapter 375 Congenital Anomalies of theTrachea
and Bronchi 1412 Chapter 387 Gastroesophageal Reflux and
Lauren D. Holinger Respiratory Disorders 1430
375.1 Vascular and Cardiac Anomalies 1412 John L. Colombo
375.2 Trachea] Stenoses, Webs, and Atresia 1412
375.3 Foregut Cysts 1413 Chapter 388 Parenchymal Disease with Prominent
Hypersensitivity, Eosinophilic Infiltration, or
Chapter 376 Bronchomalacia and Toxin-Mediated Injury 1430
Tracheomalacia 1413 Oren Lakser
Jonathan D. Finder 388.1 Hypersensitivity to Inhaled Materials 1430
Chapter 377 Neoplasms of the Larynx,Trachea, and 388.2 Silo Filler Disease 1431
Bronchi 1413 388.3 Paraquat Lung 1431
388.4 Eosinophilic Lung Disease (Formerly Loffler
Lauren D. Holinger
Syndrome) 1432
377.1 Vocal Nodules 1413
377.2 Recurrent Respiratory Papillomatosis Chapter 389 Pneumonia 1432
(RRP) 1413 Theodore C. Sectish and Charles G. Prober
377.3 Congenital Subglottic Hemangioma 1414
377.4 Vascular Anomalies . 1414 Chapter 390 Slowly Resolving Pneumonia 1435
377.5 Other Laryngeal Neoplasms 1414 Oren Lakser
377.6 Tracheal Neoplasms 1414
Chapter 391 Bronchiectasis 1436
377.7 Bronchial Tumors 1414
Oren Lakser
Chapter 378 Inflammatory Disorders of the Small
Chapter 392 Pulmonary Abscess 1437
Airways 1414 Oren Lakser
Denise Goodman
378.1 Bronchitis 1414 Chapter 393 Cystic Fibrosis 1437
378.2 Bronchiolitis 1415 Thomas F. Boat
Chapter 379 Wheezing in Infants 1417 Chapter 394 Primary Ciliary Dyskinesia (Immotile
Marzena Krawiec and Robert F. Lemanske, Jr. Cilia Syndrome) 1450
Gabriel G. Haddad
Chapter 380 .Emphysema and
Overinflation 1419 Chapter 395 Interstitial Lung Diseases 1451
Steven Boas and Glenna B. Winnie Michelle S. Howenstine
\
Contents XXXVil
Chapter 421 Acyanotic Congenital Heart Disease: Chapter 427 General Principles of Treatment of
Regurgitant Lesions 1522 Congenital Heart Disease 1551
421.1 Pulmonary Valvular Insufficiency and Congenital Section 4: Cardiac Arrhythmias 1554
Absence of the Pulmonary Valve 1522
421.2 Congenital Mitral Insufficiency 1522 Anne Dubin
421.3 Mitral Valve Prolapse 1522 Chapter 428 Disturbances of Rate and Rhythm
421.4 Tricuspid Regurgitation 1523
of the Heart 1554
Chapter 422 Cyanotic Congenital Heart Disease: 428.1 Sinus Arrhythmias and Extrasystoles 1554
Evaluation of the Critically III Neonate with 428.2 Supraventricular Tachycardia 1559
Cyanosis and Respiratory Distress 1523 428.3 Ventricular Tachyarrhythmias 1561
428.4 Long Q-T Syndrome 1562
Chapter 423 Cyanotic Congenital Heart Lesions: 428.5 Bradyarrhythmias 1562
Lesions Associated with Decreased Pulmonary 428.6 Sick Sinus Syndrome 1563
Blood Flow 1524 428.7 Sudden Death 1563
423.1 Tetralogy of Fallot 1524
42 3.2 Pulmonary Atresia with Ventricular Section 5:AcquiredHeartDisease 1565
Septal Defect 1528 Daniel Bernstein
423.3 Pulmonary Atresia with Intact Ventricular
Septum 1529 Chapter 429 Infective Endocarditis 1565
423.4 Tricuspid Atresia 1530 Chapter 430 Rheumatic Heart Disease 1570
423.5 Double-Outlet Right Ventricle with Pulmonary
Stenosis 1532 Section 6: Diseases of the Myocardium and
42 3.6 Transposition of the Great Arteries with Ventricular Pericardium 1572
Septal Defect and Pulmonary Stenosis 1532 Daniel Bernstein
423.7 Ebstein Anomaly of the Tricuspid Valve 1532
Chapter 431 Diseases of the Myocardium 1572
Chapter 424 Cyanotic Congenital Heart Disease: 431.1 Dilated Cardiomyopathy 15 72
Lesions Associated with Increased Pulmonary 431.2 Hypertrophic Cardiomyopathy 1575
Blood Flow 1534 431.3 Restrictive Cardiomyopathy 1576
424.1 d-Transposition of the Great Arteries 1534 431.4 Myocarditis 1576
424.2 d-Transposition of the Great Arteries with Intact 431.5 Nonviral Causes of Myocarditis 1577
Ventricular Septum 1535 431.6 Endocardial Fibroelastosis 1578
424.3 Transposition of the Great Arteries with Ventricular
Septal Defect 1536 Chapter 432 Diseases of the Pericardium 1579
424.4 1-Transposition of the Great Arteries (Corrected 432.1 Constrictive Pericarditis 1581 , :
^ Transposition) 1537 Chapter 433 Tumorsof the Heart 1581
424.5 Double-Outlet Right Ventricle Without Pulmonary
Stenosis 1538 Section 7: Cardiac Therapeutics 1582
424.6 Double-Outlet Right Ventricle with
Daniel Bernstein
Transposition of the Great Arteries (Taussig-Bing
Anomaly) 1538 Chapter 434 Heart Failure 1582
424.7 Total Anomalous Pulmonary Venous Return 1538 434.1 Cardiogenic Shock 1586
Contents XXXJX
Chapter 435 Pediatric Heart and Heart-Lung Chapter 451 Hereditary Elliptocytosis 1622
Transplantation 1587 George B. Segel
435.1 Pediatric Heart Transplantation 1587
Chapter 452 Hereditary Stomatocytosis 1622
435.2 Heart-Lung and Lung Transplantation 1590
George B. Segel
Section 8: Diseases of the Peripheral Vascular Chapter 453 Other Membrane Defects 1622
System 1591 George B. Segel
Daniel Bernstein
Chapter 454 Hemoglobin Disorders 1623
Chapter 436 Disease of the Blood Vessels Keith Quircdo and Elliott Vichinsky
(Aneurysms and Fistulas) 1591 454.1 Sickle Cell Disease 1624
436.1 Kawasaki Disease 1591 454.2 Sickle Cell Trait (Hemoglobin AS) 1628
436.2 Arteriovenous Fistulas 1591 454.3 Other Hemoglobinopathies 1629
454.4 Unstable Hemoglobin Disorders 1629
Chapter 437 Systemic Hypertension 1592 454.5 Abnormal Hemoglobins with Increased Oxygen
Affinity 1629
454.6 Abnormal Hemoglobins Causing
Cyanosis 1629
Diseases of the Blood 1599 454.7 Hereditary Methemoglobinemia 1629
454.8 Syndromes of Hereditary Persistence of Fetal
Section 1: The Hematopoietic System 1599
Hemoglobin (HPFH) 1630
454.9 Thalassemia Syndromes 1630
Chapter 438 Development of the Hematopoietic
System 1599 Chapter 455 Enzymatic Defects 1635
George B. Segel
Robin K. Ohls and Robert D. Christensen
455.1 Pyruvate Kinase (PK) Deficiency 1635
Chapter 439 The Anemias 1604 455.2 Other Glycolytic Enzyme Deficiencies 1636
Bertil Glader 455.3 Glucose-6-Phosphate Dehydrogenase (G6PD) and
Related Deficiencies 1636
Section 2: Anemias of Inadequate Production 1606
Chapter 456 Hemolytic Anemias Resulting from
Bertil Glader Extracellular Factors 1638
Chapter 440 Congenital Hypoplastic Anemia George B. Segel
(Diamond-Blackfan Anemia) 1606 Chapter 457 Hemolytic Anemias Secondary to Other
Chapter 441 Pearson Marrow-Pancreas Extracellular Factors 1640
George B. Segel
Syndrome 1607
Chapter 442 Acquired Pure Red Blood Cell Section 4: Polycythemia (Erythrocytosis) 1641
Anemias 1608 Bruce M. Camitta
Chapter 443 Anemia of Chronic Disorders Chapter 458 Primary Polycythemia (Polycythemia
(ACD) and Renal Disease 1609 RubraVera) 1641
Chapter 444 Congenital Dyserythropoietic Chapter 459 Secondary Polycythemia 1641
Anemias (CDA) 1609
Section 5: The Pancytopenias 1642
Chapter 445 Physiologic Anemia of Infancy 1610
Chapter 460 The Constitutional
Chapter 446 Megaloblastic Anemias 1611 Pancytopenias 1642
446.1 Folic Acid Deficiency 1611 Alan D. D'Andrea
446.2 Vitamin B 12 (Cobalamin) Deficiency 1612
Chapter 461 The Acquired Pancytopenias 1644
446.3 Other Rare Megaloblastic Anemias 1613 Jeffrey D. Hard
Chapter 469 von Willebrand Disease 1662 Chapter 484 Molecular and Cellular Biology of
Cancer 1681
Chapter 470 Hereditary Predisposition to Laura L. Worth
Thrombosis 1664
Chapter 485 Principles of Diagnosis 1684
Chapter 471 Acquired Thrombotic Disorders 1665 Archie Bleyer
471.1 Anticoagulant and Thrombolytic Therapy 1666
Chapter 486 Principles of Treatment 1688
Chapter 472 Postneonatal Vitamin K Archie Bleyer
Deficiency 1668
Chapter 487 The Leukemias 1694
Chapter 473 Liver Disease 1668 David G. Tubergen and Archie Bleyer
487.1 Acute Lymphoblastic Leukemia 1694
Chapter 474 Acquired Inhibitors of 487.2 Acute Myelogenous Leukemia 1696
Coagulation 1668 487.3 Down Syndrome and Acute Leukemia and
Chapter 475 Disseminated Intravascular Myeloproliferation 1697
Coagulation (Consumptive Coagulopathy) 1669 487.4 Chronic Myelogenous Leukemia 1697
487.5 Juvenile Chronic Myelogenous
Chapter 476 Platelet and Blood Vessel Leukemia 1698
Disorders 1670 487.6 Infant Leukemia 1698
476.1 Idiopathic Thrombocytopenic Purpura 1670
Chapter 488 Lymphoma 1698
476.2 Drug-Induced Thrombocytopenia 1671
GeraldS. Gilchrist
476.3 Nonimmune Platelet Destruction 1671
488.1 Hodgkin Disease 1698
476.4 Hemolytic-Uremic Syndrome (HUS) 1671
488.2 Non-Hodgkin Lymphoma 1701
476.5 Thrombotic Thrombocytopenic Purpura (TTP) 1672
476.6 Kasabach-Merritt Syndrome 1672 Chapter 489 BrainTumors in Childhood 1702
476.7 Sequestration 1672 John F. Kuttesch, Jr. and Joann L. Ater
476.8 Congenital Thrombocytopenic Syndromes 1672
476.9 Neonatal Thrombocytopenia 1672 Chapter 490 Neuroblastoma 1709
476.10 Thrombocytopenia Due to Acquired Disorders Joann L. Ater
Causing Decreased Production 1673 Chapter 491 Neoplasms of the Kidney 1711
476.11 Platelet Function Disorders 1673 Norman Jaffe and Vicki Huff
476.12 Acquired Disorders of Platelet Function 1673
476.13 Congenital Abnormalities of Platelet Function 1673 Chapter 492 SoftTissue Sarcomas 1714
476.14 Disorders of the Blood Vessels 1674 Carola A. S. Arndt
Contents xli
Chapter 523 Bartter/Gitelman Syndromes and Other Chapter 540 History and Physical
InheritedTubularTransport Abnormalities 1763 Examination 1827
Katherine MacRae Dell and Ellis D. Avner
523.1 Banter Syndrome 1763 Chapter 541 Vulvovaginitis 1828
523.2 Gitelman Syndrome 1764 •
Chapter 542 Bleeding 1833
523.3 Other Inherited Tubular Transport
Abnormalities 1764 Chapter 543 Breast Disorders 1833
Chapter 524 Tubulointerstitial Nephritis 1764 Chapter 544 Hirsutism and Polycystic Ovarian
Katherine MacRae Dell and Ellis D. Avner Syndrome 1835
SectionS: Toxic Nephropathies—Renal Failure 1766 Chapter 545 Neoplasms 1837
Chapter 525 Toxic Nephropathy 1766 Chapter 546 Vulvovaginal and Mullerian
Beth A. Vogt and Ellis D. Avner Anomalies 1839
Chapter 526 Cortical Necrosis 1767 Chapter 547 Athletic Gynecologic Problems 1842
Beth A. Vogt and Ellis D. Avner
Chapter 548 Special Gynecologic Needs 1843
Chapter 527 Renal Failure 1767
Beth A. Vogt and Ellis D. Avner Chapter 549 Gynecologic Imaging 1843
527.1 Acute Renal Failure 1767
527.2 Chronic Renal Failure 1771
527.3 End-Stage Renal Disease 1774
Part XXV The Endocrine System 1845
Chapter 528 RenalTransplantation 1775
Rodrigo E. Urizar Section 1: Disorders of the Hypothalamus and Pituitary
Gland 1845
Chapter 550 Hormones of the Hypothalamus and
Part XXI11 Urologic Disorders in Infants and Pituitary 1845
John S. Parks
Children 1783
Chapter 551 Hypopituitarism 1847
Jack S. Elder John S. Parks
Chapter 529 Congenital Anomalies and Dysgenesis Chapter 552 Diabetes Insipidus 1853
of the Kidneys 1783 David T. Breault and Joseph A. Majzoub
552.1 Causes of Hypernatremia 1854
Chapter 530 Urinary Tract Infections 1785
Chapter 553 Other Abnormalities of Arginine
Chapter 531 Vesicoureteral Reflux 1790 Vasopressin Metabolism and Action 1855
David T. Breault and Joseph A. Majzoub
Chapter 532 Obstructions of the Urinary Tract 1794
Chapter 554 Hyperpituitarism,Tall Stature, and
Chapter 533 Anomalies of the Bladder 1804
Overgrowth Syndromes 1857
Chapter 534 Neuropathic Bladder 1806 Pinchas Cohen
Contents xliii
Chapter 555 Physiology of Puberty 1862 Section 4: Disorders of the Adrenal Glands 1898
Luigi Garibaldi Lenore S. Levine and Perrin C. White
Chapter 556 Disorders of Pubertal Chapter 568 The Physiology of the Adrenal
Development 1863 Gland 1898
Luigi Garibaldi
568.1 Histology and Embryology 1898
556.1 Gonadotropin-Dependent Precocious 568.2 Adrenal Steroid Biosynthesis 1899
Puberty 1863 568.3 Regulation of the Adrenal Cortex 1900
556.2 Precocious Puberty Resulting from Organic Brain 568.4 Adrenal Steroid Hormone Actions 1901
Lesions 1865 568.5 Adrenal Medulla 1904
556.3 Precocious Puberty Following Irradiation of the
Brain 1866 Chapter 569 Adrenocortical Insufficiency 1904
556.4 Syndrome of Precocious Puberty and 569.1 Primary Adrenal Insufficiency 1904
Hypothyroidism 1866 569.2 Secondary Adrenal Insufficiency 1908
556.5 Gonadotropin-Secreting Tumors 1867
556.6 McCune-Albright Syndrome (Precocious Puberty Chapter 570 Congenital Adrenal Hyperplasia and
with Polyostotic Fibrous Dysplasia and Abnormal Related Disorders 1909
Pigmentation) 1867 570.1 Congenital Adrenal Hyperplasia due to
556.7 Familial Male Gonadotropin-Independent 21 -Hydroxylase Deficiency 1909
Precocious Puberty 1868 570.2 Congenital Adrenal Hyperplasia due to
556.8 Incomplete (Partial) Precocious 11 P-Hydroxylase Deficiency 1913
Development 1868 570.3 Congenital Adrenal Hyperplasia due to 3(3-
556.9 Medicational Precocity 1869 Hydroxysteriod Dehydrogenase Deficiency 1913
570.4 Congenital Adrenal Hyperplasia due to
Section 2: Disorders of the Thyroid Gland 1870 17-Hydroxylase Deficiency 1914
Stephen LaFranchi 570.5 Lipoid Adrenal Hyperplasia 1914
570.6 Aldosterone Synthase Deficiency 1915
Chapter 557 Thyroid Development and 570.7 Glucocorticoid-Suppressible
Physiology 1870 Hyperaldosteronism 1915
557.1 Thyroid Hormone Studies 1871
Chapter 571 Cushing Syndrome 1916
Chapter 558 Defects of Thyroxine-Binding
Chapter 572 Primary Aldosteronism 1918
Globulin 1872
Chapter 573 AdrenalTumors 1918
Chapter 559 Hypothyroidism 1872
573.1 Virilizing Adrenocortical Tumors 1918
Chapter 560 Thyroiditis 1879
573.2 Feminizing Adrenal Tumors 1919
Chapter 561 Goiter 1881
Chapter 574 Pheochromocytoma 1919
561.1 Congenital Goiter 1881
561.2 Endemic Goiter and Cretinism 1882 Chapter 575 Adrenal Masses 1920
561.3 Sporadic Goiter 1883 575.1 Adrenal Incidentaloma 1920
561.4 Intratracheal Goiter 1883 575.2 Adrenal Calcification 1921
Chapter 562 Hyperthyroidism 1884 Section 5: Disorders of the Gonads 1921
562.1 Graves Disease 1884 Robert Rapaport
562.2 Congenital Hyperthyroidism 1886
Chapter 576 Development and Function of the
Chapter 563 Carcinoma of the
Gonads 1921
Thyroid 1887 '
563.1 Solitary Thyroid Nodule 1888 Chapter 577 Hypofunction of theTestes 1924
563.2 Medullary Carcinoma 1889 577.1 Hypergonadotropic Hypogonadism in the Male
(Primary Hypogonadism) 1924
Section 3: Disorders of the Parathyroid 1890 5 77.2 Hypogonadotropic Hypogonadism in the Male
Daniel A. Doyle and Angelo M. DiGeorge (Secondary Hypogonadism) 1927
Chapter 564 Hormones and Peptides of Chapter 578 Pseudoprecocity Resulting from
Calcium Homeostasis and Bone Tumors of theTestes 1930
Metabolism 1890 Chapter 579 Gynecomastia 1930
Chapter 565 Hypoparathyroidism 1892 Chapter 580 Hypofunction of the Ovaries 1931
Chapter 566 Pseudohypoparathyroidism 580.1 Hypergonadotropic Hypogonadism in the Female •
(Albright Hereditary Osteodystrophy) 1894 (Primary Hypogonadism) 1931 ,
580.2 Hypogonadotropic Hypogonadism in the Female
Chapter 567 Hyperparathyroidism 1895 (Secondary Hypogonadism) 1935
xliv Contents
Chapter 581 Pseudoprecocity Due to Lesions of the 589.3 Sturge-Weber Disease 2017
Ovary 1937 589.4 von Hippel-Lindau Disease 2019
589.5 Linear Nevus Syndrome 2019
Chapter 582 Intersex 1939 589.6 PHACE Syndrome 2019
582.1 46,XX Intersex (46,XX with Virilization) 1940
582.2 46,XYIntersex (46,XY with Undervirilization) 1940 Chapter 590 Movement Disorders 2019
582.3 True Gonadal Intersex 1945 Michael V. Johnston
590.1 Ataxias 2019
Section 6: Diabetes Mellitus in Children 1947 590.2 Chorea, Athetosis, Tremor 2020
Ramin Alemzadeh and David T. Wyatt 590.3 Dystonia 2022
590.4 Tics 2023
Chapter 583 Diabetes Mellitus 1947
583.1 Introduction and Classification 1947 Chapter 591 Encephalopathies 2023
583.2 Type 1 Diabetes Mellitus (Immune Michael V. Johnston
Mediated) 1948 591.1 Cerebral Palsy 2024
583.3 Type 2 Diabetes Mellitus 1967 591.2 MitochondrialEncephalomyopathies 2025
583.4 Other Specific Types of Diabetes 1968 591.3 Other Encephalopathies 2027
Chapter 592 Neurodegenerative Disorders of
Childhood 2029
Part XXVI The Nervous System 1973 Michael V. Johnston
592.1 Sphingolipidoses 2029
Chapter 584 Neurologic Evaluation 1973 592.2 Neuronal Ceroid Lipofuscinoses 2032
Robert H. A. Haslam
592.3 Adrenoleukodystrophy 2032
592.4 Sialidosis 2033
Chapter 585 Congenital Anomalies of the Central 592.5 Miscellaneous Disorders 2033
Nervous System 1983
Chapter 593 Acute Stroke Syndromes 2035
Michael V. Johnston and Stephen Kinsman
Michael V. Johnston
585.1 Neural Tube Defects (Dysraphism) 1983
593.1 Arterial Thrombosis/Embolism 2035
585.2 Spina Bifida Occulta 1983
593.2 Venous Thrombosis 2036
585.3 Meningocele 1983
593.3 Intracranial Hemorrhage 2036
585.4 Myelomeningocele 1984
593.4 Differential Diagnosis of Strokelike
585.5 Encephalocele 1985
Events 2037
585.6 Anencephaly 1986
585.7 Disorders of Neuronal Migration 1986 Chapter 594 Central Nervous System
585.8 Agenesis of the Corpus Callosum 1987 Infections 2038
585.9 Agenesis of the Cranial Nerves 1988 Charles G. Prober
585.10 Microcephaly 1988 594.1 Acute Bacterial Meningitis Beyond the Neonatal
585.11 Hydrocephalus 1989 Period 2038
585.12 Craniosynostosis 1992 594.2 Viral Meningoencephalitis 2044
Chapter 586 Seizures in Childhood 1993 594.3 Eosinophilic Meningitis 2046
Michael V. Johnston Chapter 595 Brain Abscess 2047
586.1 Febrile Seizures 1994 Robert H. A. Haslam
586.2 Unprovoked Seizures 1994
586.3 Partial Seizures 1995 Chapter 596 Pseudotumor Cerebri 2048
586.4 Generalized Seizures 1997 Robert H. A. Haslam
586.5 Mechanisms of Seizures 1999
Chapter 597 Spinal Cord Disorders 2049
586.6 Treatment of Epilepsy 1999
Robert H. A. Haslam
586.7 Neonatal Seizures 2005
597.1 Spinal Cord Tumors 2049
586.8 Status Epilepticus 2007
597.2 Spinal Cord Trauma 2050
Chapter 587 Conditions that Mimic Seizures 2009 597.3 Tethered Cord 2050
Michael V. Johnston 597.4 Diastematomyelia 2051
597.5 Syringomyelia 2051
Chapter 588 Headaches 2012 597.6 Transverse Myelitis 2051
Robert H. A. Haslam 597.7 Arteriovenous Malformation 2052
588.1 Migraine 2012
588.2 Organic Headaches 2014
588.3 Tension or Stress Headaches 2015
Part XXVII Neuromuscular Disorders 2053
Chapter 589 Neurocutaneous Syndromes 2015
Robert H. A. Haslam Harvey B. Sarnat
589.1 Neurofibromatosis 2015
589.2 Tuberous Sclerosis 2017 Chapter 598 Evaluation and Investigation 2053
Contents xlv
Chapter 642 Hyperpigmented Lesions 2176 Chapter 665 Torsional and Angular
Deformities 2261
Chapter 643 Hypopigmented Lesions 2178 George H. Thompson
665.1 Normal Developmental Alignment 2261
Chapter 644 Vesiculobullous Disorders 2181
665.2 Internal Femoral Torsion 2262
Chapter 645 Eczematous Disorders 2188 665.3 Internal Tibial Torsion 2263
665.4 External Femoral Torsion 2263
Chapter 646 Photosensitivity 2191 665.5 External Tibial Torsion 2264
665.6 Genu Varum (Bowlegs) 2264
Chapter 647 Diseases of the Epidermis 2195
665.7 Genu Valgum (Knock-Knees) 2266
Chapter 648 Disorders of Keratinization 2200 665.8 Congenital Angular Deformities of the Tibia and
Fibula 2269
Chapter 649 Diseases of the Dermis 2204
Chapter 666 Leg-Length Discrepancy 2270
Chapter 650 Diseases of Subcutaneous George H. Thompson
Tissue 2210
Chapter 667 The Knee 2271
Chapter 651 Disorders of the Sweat George H. Thompson
Glands 2213 667.1 Discoid Lateral Meniscus 2272
Chapter 652 Disorders of Hair 2215 667.2 Popliteal Cyst 2272
667.3 Osteochondritis Dissecans 2272
Chapter 653 Disorders of the Nails 2219 667.4 Osgood-Schlatter Disease 2272
667.5 Idiopathic Adolescent Anterior Knee Pain
Chapter 654 Disorders of the Mucous Syndrome 2272
Membranes 2220 667.6 Patella Subluxation and Dislocation 2273
Chapter 655 Cutaneous Bacterial Chapter 668 The Hip 2273
Infections 2222 George H. Thompson
668.1 Developmental Dysplasia of the Hip 2273
Chapter 656 Cutaneous Fungal Infections 2230
668.2 Transient Monoarticular Synovitis 2276
Chapter 657 Cutaneous Viral Infections 2235 668.3 Legg-Calve-Perthes Disease 2276
668.4 Slipped Capital Femoral Epiphysis 2278
Chapter 658 Arthropod Bites and i
Infestations 2237 Chapter 669 The Spine 2280 \
George H. Thompson i
Chapter 659 Acne 2242
669.1 Idiopathic Scoliosis 2280 »
Chapter 660 Tumors of the Skin 2246 669.2 Congenital Scoliosis 2282
669.3 Neuromuscular Scoliosis, Genetic Syndromes, and
Chapter 661 Nutritional Dermatoses 2248 Compensatory Scoliosis 2283
Contents xlvii
669.4 Kyphosis (Round-Back) 2284 Chapter 677 Head and Neck Injuries 2313
669.5 Back Pain in Children 2285
669.6 Spondylolysis and Spondylolisthesis 2285 Chapter 678 Heat Injuries 2314
669.7 Disk Space Infection 2286 Chapter 679 Female Athletes: Menstrual Problems
669.8 Intervertebral Disk Herniation 2287
and the Risk for Osteopenia 2315
669.9 Tumors 2287
Chapter 680 ErgogenicAids 2316
Chapter 670 The Neck 2288
George H. Thompson Chapter 681 Specific Sports and Associated
670.1 Torticollis 2288 Injuries 2316
670.2 Klippel-Feil Syndrome 2289
670.3 Atlantoaxial Instability 2289 Section 3: The Skeletal Dysplasias 2320
Chapter 671 The Upper Limb 2290 Chapter 682 General Considerations 2320
George H. Thompson William A. Horton and Jacqueline T. Hecht
671.1 Sprengel Deformity 2290
671.2 Shoulder Dislocation 2290 Chapter 683 Disorders Involving Cartilage Matrix
671.3 Nursemaid's Elbow 2290 Proteins 2324
671.4 Panner Disease 2291 William A. Horton and Jacqueline T. Hecht
671.5 Ganglion 2291
671.6 Radial Clubhand 2291 Chapter 684 Disorders InvolvingTransmembrane
671.7 Polydactyly 2292 Receptors 2328
671.8 Syndactyly' 2292 William A. Horton and Jacqueline T. Hecht
671.9 Congenital Trigger Thumb and Finger 2292 Chapter 685 Disorders Involving lonTransporter 2330
Chapter 672 Arthrogryposis 2292 William A. Horton and Jacqueline T. Hecht
George H. Thompson
Chapter 686 Disorders InvolvingTranscription
672.1 Amyoplasia 2292
Factors 2332
Chapter 673 Common Fractures 2294 William A. Horton and Jacqueline T. Hecht
George H. Thompson
673.1 Pediatric Fracture Patterns 2294 Chapter 687 Disorders Involving Defective Bone
673.2 Clavicular Fractures 2295 Resorption 2333
673.3 Proximal Humerus Fractures 2295 William A. Horton and Jacqueline T. Hecht
672.4 Distal Humerus Fractures 2295 Chapter 688 Disorders for Which Defects Are Poorly
673.5 Distal Radius and Ulna Fractures 2296
Understood or Unknown 2334
673.6 Phalangeal Fractures 2296
William A. Horton and Jacqueline T. Hecht
673.7 Toddler Fracture 2296
673.8 Lateral Malleolar Fractures 2296 Chapter 689 Osteogenesis Imperfecta 2336
673.9 Metatarsal Fractures 2296 John C. Marini
673.10 Toe Phalangeal Fractures 2296
673.11 Operative Treatment 2297 Chapter 690 Marfan Syndrome 2338
Luther K. Robinson
Chapter 674 Osteomyelitis and Suppurative
Arthritis 2297 Section 4: Metabolic Bone Disease 2341
Richard M. Lampe Russell W. Chesney
Section 2: Sports Medicine 2302 Chapter 691 Bone Structure, Growth, and Hormonal
Albert C. Hergenroed'er and Joseph N. Chorley Regulation 2341
Chapter 675 Epidemiology and Chapter 692 Primary Chondrodystrophy
Prevention of Injuries 2302 (Metaphyseal Dysplasia) 2343
Chapter 676 Management of Musculoskeletal Chapter 693 Idiopathic Hypercalcemia 2343
Injury 2306
676.1 Growth Plate Injuries 2307 Chapter 694 Hypophosphatasia 2344
676.2 Shoulder Injuries 2308 Chapter 695 Hyperphosphatasia 2344
676.3 Elbow Injuries 2308
676.4 Back Injuries 2309 Chapter 696 Familial Hypophosphatemia
676.5 Hip and Pelvis Injuries 2310 (Vitamin D-Resistant Rickets, X-Linked *
676.6 Knee Injuries 2310 Hypophosphatemia) 2345 [
676.7 Shin Splints and Stress Fractures of the
Lower Leg 2312 Chapter 697 Vitamin D-Dependent Rickets \
676.8 Ankle Injuries 2312 (Pseudovitamin D Deficiency, Hypocalcemic
676.9 Foot Injuries 2313 Vitamin D-Resistant Rickets) 2346
xlviii Contents
Chapter 698 Oncogenous Rickets (Primary Chapter 705 Nonbacterial Food Poisoning 2375
Hypophosphatemic Rickets Associated with DeniseA. Salerno and Stephen C. Aronoff
Tumor) 2346 705.1 Mushroom Poisoning 2375
705.2 Solanine Poisoning 2376
Chapter 699 Osteoporosis 2347 705.3 Seafood Poisoning 2377
Chapter 706 Biologic and ChemicalTerrorism 2378
Theodore J. Cieslak and Fred M. Henretig
Part XXXII Environmental Health Chapter 707 Animal and Human Bites 2385
Hazards 2349 Charles M. Ginsburg