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Pediatric Gastrointestinal and Liver Disease E-Book

Pediatric Gastrointestinal and Liver Disease E-Book

Robert Wyllie | Jeffrey S. Hyams

(2010)

Additional Information

Book Details

Abstract

Pediatric Gastrointestinal and Liver Disease, by Drs. Robert Wyllie and Jeffrey S. Hyams provides the comprehensive reference you need to treat GI diseases in children. Review the latest developments in the field and get up-to-date clinical information on hot topics like polyps, capsule endoscopy, and pancreatic treatments. With expert guidance from an expanded international author base and online access to 475 board-review-style questions, this latest edition is a must-have for every practicing gastroenterologist.

  • Confirm each diagnosis by consulting a section, organized by symptoms, that presents the full range of differential diagnoses and treatment options for each specific condition.
  • Recognize disease processes at a glance with detailed diagrams that accurately illustrate complex concepts.
  • Stay current with advances in the field by reviewing new chapters on Polyps and Polyposis Syndromes, Capsule Endoscopy and Small Bowel Enteroscopy, Small Bowel Transplantation, IBD, Short Gut Syndrome, Steatosis and Non-Alcoholic Fatty Liver Disease, and Pancreatic and Islet Cell Transplants.
  • Gain fresh global perspectives from an expanded list of expert international contributors.
  • Sharpen your visual recognition by accessing a color-plate section that displays additional endoscopy images.
  • Prepare for certification or recertification with 475 online board review-style questions, answers, and rationales.

Table of Contents

Section Title Page Action Price
Front Cover\r Cover
Pediatric Gastrointestinal and Liver Disease iii
Copyright iv
Dedication v
Contributors vii
Preface xix
Contents xxi
Section 1 Biologic Aspects of Gastrointestinal Function\r 1
Chapter 1 - Development of the Gastrointestinal Tract 2
MORPHOGENESIS 2
MOLECULAR MECHANISMS \r 5
REFERENCES 9
Chapter 2 - Basic Aspects of Digestion and Absorption 10
CARBOHYDRATES \r 10
PROTEINS \r 12
LIPIDS \r 14
DIGESTION AND ABSORPTION IN INFANTS \r 17
VITAMINS AND MINERALS \r 18
MINERALS AND TRACE ELEMENTS \r 19
ACKNOWLEDGMENT \r 19
REFERENCES 19
FURTHER READINGS 19
Chapter 3 - Bile Acid Physiology and Alterations in the Enterohepatic Circulation 20
BIOSYNTHESIS 20
ENTEROHEPATIC CIRCULATION 20
MATURATION OF THE ENTEROHEPATIC CIRCULATION 22
ALTERATIONS IN THE ENTEROHEPATIC CIRCULATION 22
PEROXISOMAL DISORDERS 25
ALTERATION OF HEPATIC BILE ACID TRANSPORT 25
ALTERATION IN CHOLESTASIS (GENERAL) 26
ALTERATION OF THE ENTEROHEPATIC CIRCULATION OF BILE ACIDS 26
MECHANISM OF BILE ACID-INDUCED DIARRHEA \r 27
SUMMARY \r 27
REFERENCES 27
Chapter 4 - Indigenous Flora 28
UNDERSTANDING THE INDIGENOUS FLORA BY STUDYING GERM-FREE ANIMALS \r 28
ESTABLISHING THE INDIGENOUS FLORA \r 29
BACTERIAL FLORA WITHIN THE VARIOUS SECTIONS OF THE GASTROINTESTINAL TRACT \r 30
CONTROLLING THE GROWTH OF THE INDIGENOUS POPULATION \r 31
SYMBIOSIS BETWEEN HOST AND FECAL FLORA \r 32
BACTERIAL FLORA IN ILLNESS \b\r 33
BACTERIAL FLORA AND ALLERGY \r 34
BACTERIAL FLORA AND ANTIBIOTICS \r 34
BACTERIAL OVERGROWTH \r 34
TROPICAL SPRUE \r 35
PROBIOTICS \r 35
PREBIOTICS \r 37
SYNBIOTICS \r 38
REFERENCES\r 38
Chapter 5 - Physiology of Gastrointestinal Motility 39
ESOPHAGEAL MOTILITY \r 39
GASTRIC MOTILITY \r 41
MOTILITY OF THE SMALL INTESTINE AND COLON \r 41
ADDITIONAL MOTOR FUNCTIONS \r 46
SUMMARY \r 47
REFERENCES 49
Chapter 6 - Gastrointestinal Mucosal Immunology and Mechanisms of Inflammation 50
HIERARCHY OF GUT IMMUNE RESPONSES \r 50
GENERATION OF INFLAMMATION \r 51
MECHANISMS THAT PREVENT INFLAMMATORY REACTIONS TO GUT LUMINAL CONTENTS \r 51
ORGANIZATION OF THE MUCOSAL IMMUNE SYSTEM \r 53
COMPONENTS OF THE MUCOSAL IMMUNE SYSTEM \r 54
ESTABLISHMENT AND MAINTENANCE OF ORAL TOLERANCE TO ANTIGENS AND THE FLORA \r 59
PATTERNS OF INFLAMMATORY RESPONSE WITHIN THE INTESTINE \r 60
CONCLUSIONS \r 62
REFERENCES 63
Section 2 Clinical Problems 65
Chapter 7 - Chronic Abdominal Pain of Childhood and Adolescence 66
EVALUATION OF THE CHILD WITH CHRONIC ABDOMINAL PAIN \r 68
DIFFERENTIAL DIAGNOSIS \r 69
DIAGNOSIS OF CHILDHOOD FUNCTIONAL ABDOMINAL PAIN DISORDERS \r 72
THERAPEUTIC STRATEGY DEVELOPMENT \r 73
DIET \r 75
PHARMACOTHERAPY \r 75
PSYCHOLOGICAL THERAPIES \r 77
PROGNOSIS \r 78
PREVENTION \r 79
CONCLUSION \r 79
REFERENCES 79
Chapter 8 - Approach to the Child With a Functional Gastrointestinal Disorder 80
BIOMEDICAL MODEL \r 80
BIOPSYCHOSOCIAL MODEL \r 81
EARLY LEARNING-DEVELOPMENTAL ASPECTS OF FUNCTIONAL GASTROINTESTINAL DISORDERS \r 81
EFFECTIVE REASSURANCE \r 82
BIOMEDICAL VERSUS BIOPSYCHOSOCIAL MODELS \r 83
TREATMENT \r 84
APPROACH TO THE CHILD OR ADOLESCENT WITH PAIN-ASSOCIATED DISABILITY SYNDROME \r 84
WHAT SHOULD A PEDIATRIC GASTROENTEROLOGY CONSULTATION ACCOMPLISH? \r 85
PEDIATRIC FUNCTIONAL GASTROINTESTINAL DISORDERS \r 85
CONCLUSION \r 87
REFERENCES 87
Chapter 9 - Vomiting and Nausea 88
VOMITING EVENT \r 88
CLINICAL ASPECTS OF VOMITING \r 90
CLINICAL ASPECTS OF NAUSEA \r 96
SPECIFIC VOMITING DISORDERS \r 101
REFERENCES 105
Chapter 10 - Diarrhea 106
PHYSIOLOGY OF INTESTINAL CONTENT HANDLING \r 106
DEFINITIONS OF DIARRHEA \r 106
CLINICAL OBSERVATIONS OF TYPES OF DIARRHEA \r 107
PATHOPHYSIOLOGY OF SECRETORY DIARRHEA \r 107
PATHOPHYSIOLOGY OF OSMOTIC DIARRHEA \r 108
PATHOPHYSIOLOGY OF INFLAMMATORY DIARRHEA \r 108
CLINICAL RELEVANCE OF ACUTE INFECTIOUS DIARRHEA \r 109
DIFFERENTIAL DIAGNOSIS \r 109
APPROACH TO THE CHILD WITH DIARRHEA \r 114
CLINICAL MANAGEMENT \r 117
REFERENCES 118
Chapter 11 - Colic and Gastrointestinal Gas 119
GASTROINTESTINAL GAS \r 119
COLIC \r 121
CONCLUSIONS \r 125
REFERENCES 126
Chapter 12 - Constipation and Fecal Incontinence 127
PATHOPHYSIOLOGY \r 127
FUNCTIONAL CONSTIPATION IN INFANTS AND TODDLERS \r 127
FUNCTIONAL CONSTIPATION IN CHILDREN AND ADOLESCENTS \r 128
COMPLICATIONS OF CONSTIPATION \r 128
FUNCTIONAL FECAL INCONTINENCE \r 128
INVESTIGATIONS \r 130
TREATMENT \r 131
OUTCOME \r 134
REFERENCES 135
Chapter 13 - Failure to Thrive 136
EPIDEMIOLOGY \r 136
DEFINITION \r 136
NORMAL GROWTH PATTERNS \r 137
PATHOGENESIS \r 138
CAUSES AND CONTRIBUTORY FACTORS \r 138
EVALUATION OF FAILURE TO THRIVE \r 140
INVESTIGATIONS \r 141
MANAGEMENT \r 141
OUTCOME \r 143
GROWTH OUTCOME \r 143
CONCLUSION \r 144
ACKNOWLEDGMENTS \r 144
REFERENCES 144
Chapter 14 - Gastrointestinal Hemorrhage 146
THERAPY \r 149
CONCLUSIONS \r 153
REFERENCES 153
Chapter 15 - Obesity 154
DEFINITIONS \r 154
EPIDEMIOLOGY \r 155
ETIOLOGY AND PATHOGENESIS \r 155
ASSESSMENT OF THE OBESE CHILD \r 158
TREATMENT \r 162
CONCLUSIONS \r 165
REFERENCES 165
Chapter 16 - Eating Disorders in Children and Adolescents 166
CHANGING FACE OF THE EPIDEMIOLOGY \r 166
ASPECTS OF THE HISTORY AND PHYSICAL EXAMINATION \r 168
MEDICAL COMPLICATIONS OF EATING DISORDERS \r 169
AMENORRHEA AND OSTEOPENIA \r 171
PROGNOSIS: BONE, BRAINS, AND BABIES (OR FUTURE FERTILITY) \r 173
NEXT STEP \r 174
PREVENTION: HOW CAN WE DO BETTER? \r 174
REFERENCES 174
Chapter 17 - Jaundice 176
BILIRUBIN METABOLISM \r 176
ASSESSMENT OF JAUNDICE \r 178
NEONATAL JAUNDICE 179
JAUNDICE IN INFANTS AND OLDER CHILDREN \r 185
REFERENCES 186
Chapter 18 - Ascites 187
ETIOLOGY \r 187
PATHOPHYSIOLOGY \r 191
DIAGNOSIS \r 192
TREATMENT \r 193
SPONTANEOUS BACTERIAL PERITONITIS \r 195
References 196
Chapter 19 - Caustic Ingestion and Foreign Bodies 197
INGESTION OF CAUSTIC AGENTS \r 197
MECHANISMS OF DAMAGE TO THE GASTROINTESTINAL TRACT \r 197
LOCATION OF INJURY \r 198
MANAGEMENT OF CAUSTIC INGESTION \r 199
FOLLOW-UP AND LONG-TERM MANAGEMENT \r 200
INGESTION OF FOREIGN BODIES \r 200
ACUTE MANAGEMENT OF FOREIGN BODIES \r 203
References 206
Chapter 20 - Developmental Anatomy and Physiology of the Esophagus 207
DEVELOPMENTAL ANATOMY \r 207
GENETIC AND MOLECULAR ASPECTS OF TRACHEOESOPHAGEAL DEVELOPMENT \r 209
ANATOMY \r 210
PHYSIOLOGY \r 212
PATHOPHYSIOLOGY \r 218
SUMMARY \r 220
REFERENCES 220
Section 3 The Esophagus 221
Chapter 21 - Congenital Malformations of the Esophagus 222
CONGENITAL LESIONS OF THE ESOPHAGUS \r 222
CONGENITAL ESOPHAGEAL STENOSIS \r 222
ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA \r 222
LARYNGOTRACHEOESOPHAGEAL CLEFTS \r 230
Chapter 22 - Gastroesophageal Reflux 232
DEFINITIONS \r 232
ENVIRONMENTAL AND GENETIC FACTORS \r 232
PATHOPHYSIOLOGY \r 233
SYMPTOMS AND SIGNS \r 234
DIAGNOSIS \r 240
TREATMENT OPTIONS \r 241
CONCLUSION \r 246
References 247
Chapter 23 - Achalasia and Other Motor Disorders 248
DISORDERS OF THE PROXIMAL ESOPHAGUS \r 248
DISORDERS OF THE DISTAL ESOPHAGUS \r 250
REFERENCES 254
Chapter 24 - Other Diseases of the Esophagus 255
ESOPHAGEAL SYMPTOMS \r 255
ESOPHAGEAL INFECTIONS \r 255
ESOPHAGEAL TUMORS \r 260
REFERENCES 260
Section 4 The Stomach 261
Chapter 25 - Developmental Anatomy and Physiology of the Stomach 262
HISTOLOGY \r 262
NEUROMUSCULAR FUNCTIONS\r 264
GASTRIC SECRETIONS \r 265
GASTRIC ACID SECRETION \r 265
OTHER HORMONES AND PEPTIDES \r 267
PEPSINOGEN \r 267
MUCUS AND BICARBONATE SECRETION\r 267
GASTRIC LIPASE \r 268
INTRINSIC FACTOR \r 268
REFERENCES 268
Chapter 26 - Congenital Anomalies and Surgical Disorders of the Stomach 269
EMBRYOLOGY \r 269
HYPERTROPHIC PYLORIC STENOSIS \r 269
FOVEOLAR HYPERPLASIA \r 271
GASTRIC OUTLET OBSTRUCTION \r 271
GASTRIC VOLVULUS \r 273
CONGENITAL MICROGASTRIA \r 274
GASTRIC PERFORATION \r 274
GASTRIC AND DUODENAL DUPLICATIONS \r 275
POLYPS \r 275
TUMORS \r 276
REFERENCES 276
Chapter 27 - Gastritis, Gastropathy, and Ulcer Disease 277
CLINICAL PRESENTATION \r 277
DIAGNOSTIC METHODS \r 278
APPROACH TO GASTRITIS \r 280
GASTRITIS \r 281
PEPTIC ULCER DISEASE \r 288
DIAGNOSIS \r 291
MANAGEMENT \r 291
REFERENCES 292
Chapter 28 - Helicobacter pylori in Childhood 293
EPIDEMIOLOGY \r 293
HOST FACTORS AND RESPONSES \r 294
BACTERIAL FACTORS AND ADAPTATIONS \r 298
DISEASE ASSOCIATIONS \r 301
DIAGNOSIS \r 303
TREATMENT \r 306
CONCLUSION \r 308
REFERENCES 308
Chapter 29 - Gastric Motility Disorders 309
GASTRIC MOTOR PHYSIOLOGY AND GASTRIC EMPTYING \r 309
DISORDERS OF GASTRIC FUNCTION \r 311
ASSESSMENT OF GASTRIC MOTOR AND SENSORY FUNCTION \r 314
TREATMENT \r 316
REFERENCES 318
Chapter 30 - Bezoars 319
DEFINITIONS \r 319
EPIDEMIOLOGY \r 319
ETIOLOGY AND PATHOGENESIS \r 319
CLINICAL PRESENTATION \r 320
DIAGNOSIS \r 321
TREATMENT \r 321
PREVENTION \r 322
REFERENCES\r 322
Section 5 The Small and Large Intestine 323
Chapter 31 - Anatomy and Physiology of the Small and Large Intestines 324
INTESTINAL ANATOMY \r 324
PHYSIOLOGY OF WATER AND ION TRANSPORT \r 333
REFERENCES 336
Chapter 32 - Maldigestion and Malabsorption 337
CLASSES OF NUTRIENTS \r 337
DIAGNOSTIC APPROACH \r 346
MANAGEMENT \r 349
REFERENCES 349
Chapter 33 - Protracted Diarrhea 350
Definitions, Epidemiology, and Risk Factors \r 350
PATHOPHSYSIOLOGY OF PROTRACTED DIARRHEA \r 350
ASSESSMENT OF THE PATIENT WITH PROTRACTED DIARRHEA 357
ACKNOWLEDGMENTS \r 359
REFERENCES 359
Chapter 34 - Protein-Losing Enteropathy 360
PATHOPHYSIOLOGY \r 360
CAUSES OF PLE \r 360
DIAGNOSIS \r 364
TREATMENT \r 364
REFERENCES 365
Chapter 35 - Celiac Disease 366
DEFINITION \r 366
HISTORICAL BACKGROUND \r 366
CEREAL PROTEINS AND OTHER ENVIRONMENTAL FACTORS \r 366
GENETICS OF CELIAC DISEASE \r 367
EPIDEMIOLOGY \r 367
PATHOGENESIS \r 368
PATHOLOGY \r 370
CLINICAL PRESENTATION \r 370
ASSOCIATED DISEASES \r 371
LABORATORY FINDINGS \r 372
DIAGNOSIS \r 372
THERAPY \r 373
REFERENCES 373
Chapter 36 - Short Bowel Syndrome 374
DEFINITION \r 374
ETIOLOGY \r 374
INTESTINAL FUNCTION AND THE IMPACT OF INTESTINAL LOSS \r 374
FACTORS THAT DETERMINE PROGNOSIS OF SBS \r 375
CLINICAL MANAGEMENT OF SBS \r 378
ADDITIONAL MEDICAL INTERVENTIONS \r 380
COMPLICATIONS OF SBS AND PN 380
SURVIVAL AND ITS COSTS IN PEDIATRIC SBS \r 384
SUMMARY \r 384
REFERENCES 385
Chapter 37 - Small Bowel Transplant 386
HISTORY \r 386
INDICATIONS AND REFERRAL \r 386
PRETRANSPLANT NUTRITIONAL MANAGEMENT \r 387
CHOICE OF ALLOGRAFT \r 387
DONOR AND RECIPIENT OPERATIONS \r 388
POSTOPERATIVE MANAGEMENT \r 389
POSTTRANSPLANT NUTRITIONAL MANAGEMENT \r 391
NURSING IMPLICATIONS \r 391
OUTCOMES \r 392
FUTURE PROSPECTS \r 394
REFERENCES 394
Chapter 38 - Allergic and Eosinophilic Gastrointestinal Disease 395
FOOD ALLERGY OR HYPERSENSITIVITY \r 395
EOSINOPHILIC GASTROENTEROPATHIES \r 395
EOSINOPHILIC ESOPHAGITIS \r 396
EOSINOPHILIC GASTROENTERITIS \r 399
EOSINOPHILIC PROCTOCOLITIS \r 401
OTHER MANIFESTATIONS OF GASTROINTESTINAL ALLERGY \r 402
CONCLUSION \r 403
REFERENCES 404
Chapter 39 - Infectious Diarrhea 405
VIRAL GASTROENTERITIS \r 405
BACTERIAL GASTROENTERITIS \r 409
POTENTIAL DIARRHEAGENIC ORGANISMS \r 422
CONCLUSION \r 422
REFERENCES 422
Chapter 40 - Enteric Parasites 423
EPIDEMIOLOGY \r 423
HOST FACTORS \r 423
CLINICAL PRESENTATIONS \r 423
DIAGNOSIS \r 423
BENEFITS OF PARASITES \r 424
PATHOGENIC ORGANISMS \r 425
REFERENCES 434
Chapter 41 - Gastrointestinal Manifestations of Primary Immunodeficiency 435
INNATE VERSUS ADAPTIVE IMMUNITY \r 435
COMPONENTS OF THE ADAPTIVE IMMUNE RESPONSE \r 435
HUMORAL IMMUNODEFICIENCIES\r 439
CELLULAR IMMUNODEFICIENCIES\r 441
COMBINED CELLULAR-HUMORAL IMMUNODEFICIENCIES \r 442
DISORDERS OF PHAGOCYTE FUNCTION \r 444
OTHER PRIMARY IMMUNODEFICIENCIES \r 445
References 446
Chapter 42 - Gastrointestinal Complications of Secondary Immunodeficiency Syndromes 447
PEDIATRIC HIV INFECTION \r 447
HIV AND THE CELLULAR COMPONENTS OF THE GASTROINTESTINAL TRACT \r 447
STRUCTURE AND FUNCTION OF THE INTESTINAL TRACT IN HIV INFECTION \r 448
INFECTIONS OF THE GASTROINTESTINAL TRACT \r 450
INFECTIONS OF THE GASTROINTESTINAL TRACT IN REGIONS WITH LOW HAART ACCESSIBILITY OR IN PATIENTS WITH CD4 COUNTS BELOW 200 CELLS/...\r 450
INFECTIONS OF THE GASTROINTESTINAL TRACT IN REGIONS WITH HIGH HAART ACCESSIBILITY AND SUCCESSFUL HIV VIRAL SUPPRESSION \r 455
MOTILITY OF THE GASTROINTESTINAL TRACT IN HIV INFECTION \r 455
IDIOPATHIC ESOPHAGEAL ULCERATION \r 457
CLINICAL MANAGEMENT OF GASTROINTESTINAL DISORDERS IN HIV INFECTION AND OTHER IMMUNODEFICIENCIES \r 457
OTHER SECONDARY IMMUNODEFICIENCIES \r 458
LIVER COMPLICATIONS IN SECONDARY IMMUNODEFICIENCY \r 459
SUMMARY \r 460
References 461
Chapter 43 - Pediatric Polyposis Syndromes\r 462
Histopathologic Classification \r 462
Clinical Management \r 462
FAMILIAL ADENOMATOUS POLYPOSIS \r 462
JUVENILE POLYP (SOLITARY AT DIAGNOSIS) \r 467
JUVENILE POLYPOSIS SYNDROME \r 467
PTEN – HAMARTOMA TUMOR SYNDROME \r 468
PEUTZ-JEGHERS SYNDROME (PJS) \r 469
OTHER POLYPOSIS SYNDROMES\r 471
SUMMARY \r 471
References 471
Chapter 44 - Crohn’s Disease\r 472
EPIDEMIOLOGY \r 472
ETIOLOGY \r 472
PATHOLOGY \r 475
PATHOPHYSIOLOGY OF GASTROINTESTINAL SYMPTOMS \r 476
CLASSIFICATION OF SUBGROUPS IN CROHN’S DISEASE \r 476
CLINICAL FEATURES \r 478
DIAGNOSIS \r 481
DIFFERENTIAL DIAGNOSIS \r 483
THERAPY \r 483
NATURAL HISTORY \r 489
ACKNOWLEDGMENT \r 489
References 489
Chapter 45 - Ulcerative Colitis in Children and Adolescents\r 490
EPIDEMIOLOGY \r 490
GENETICS \r 490
ETIOLOGY \r 491
PATHOLOGY \r 492
CLINICAL FEATURES \r 493
EXTRAINTESTINAL MANIFESTATIONS \r 494
COMPLICATIONS \r 495
DIAGNOSIS \r 497
DIFFERENTIAL DIAGNOSIS \r 498
MEDICAL THERAPIES \r 498
SURGERY \r 503
COURSE AND PROGNOSIS \r 504
References 504
Chapter 46 - Chronic Intestinal \rPseudo-obstruction 505
ETIOLOGY \r 505
PATHOLOGY \r 505
CLINICAL FEATURES \r 507
TREATMENT \r 509
OUTCOMES \r 511
REFERENCES 511
Chapter 47 - Neonatal Necrotizing Enterocolitis 512
EPIDEMIOLOGY \r 512
PATHOLOGY \r 513
PATHOGENESIS \r 514
CLINICAL MANIFESTATIONS \r 516
DIAGNOSIS \r 517
PREVENTION \r 519
MANAGEMENT \r 519
OUTCOME \r 520
References 520
Chapter 48 - Disorders of the Anorectum: 521
ANAL FISSURE \r 521
RECTAL PROLAPSE \r 521
FISTULAS \r 522
HEMORRHOIDS \r 524
TAGS AND MISCELLANEOUS CONDITIONS \r 526
REFERENCES 527
Chapter 49 - Neoplasms of the Gastrointestinal Tract and Liver 528
NEOPLASMS OF THE LUMINAL GASTROINTESTINAL TRACT \r 528
NEOPLASMS OF LYMPHOID ORIGIN \r 528
NEOPLASMS OF EPITHELIAL ORIGIN \r 531
NEOPLASMS OF MESENCHYMAL ORIGIN \r 534
NEOPLASMS OF THE LIVER \r 535
REFERENCES 539
Chapter 50 - Other Diseases of the Small Intestine and Colon 540
HENOCH-SCHÖNLEIN PURPURA 540
ACRODERMATITIS ENTEROPATHICA \r 541
HEMOLYTIC UREMIC SYNDROME \r 542
RADIATION ENTERITIS \r 543
MALAKOPLAKIA \r 544
BEHÇET’S SYNDROME \r 545
TYPHLITIS \r 546
MICROSCOPIC COLITIS \r 547
EHLERS-DANLOS SYNDROME \r 547
PNEUMATOSIS INTESTINALIS \r 548
SOLITARY RECTAL ULCER SYNDROME AND COLITIS CYSTICA PROFUNDA \r 549
AMYLOIDOSIS \r 549
INTESTINAL GRAFT-VERSUS-HOST DISEASE \r 550
REFERENCES 550
Chapter 51 - Appendicitis 551
EMBRYOLOGY AND ANATOMY 551
PATHOPHYSIOLOGY \r 551
HISTOLOGY \r 551
CLINICAL PRESENTATION \r 551
PATIENT EVALUATION \r 552
DIAGNOSTIC TESTS \r 553
MANAGEMENT OPTIONS \r 553
DIFFERENTIAL DIAGNOSIS \r 554
COMPLICATIONS \r 555
CHRONIC APPENDICEAL PAIN \r 556
REFERENCES 556
Section 6 Pediatric Surgical Disorders 557
Chapter 52 - Intussusception in Infants and Children 558
PATHOPHYSIOLOGY \r 558
ETIOLOGY \r 559
CLINICAL PRESENTATION \r 559
DIAGNOSTIC WORK-UP \r 560
THERAPEUTIC OPTIONS \r 562
RECURRENCE \r 565
SPECIAL CONSIDERATIONS \r 565
REFERENCES 566
Chapter 53 - Inguinal Hernias and Hydroceles 568
PATHOGENESIS \r 568
CLINICAL PRESENTATION \r 568
MANAGEMENT \r 570
COMPLICATIONS \r 571
REFERENCES 571
Chapter 54 - Meckel’s Diverticulum and Other Omphalomesenteric Duct Remnants\r 572
EMBRYOLOGY AND ANATOMY \r 572
CLINICAL PRESENTATION \r 572
DIAGNOSIS \r 573
TREATMENT \r 574
SUMMARY \r 574
REFERENCES 575
Chapter 55 - Hirschsprung’s Disease 576
EPIDEMIOLOGY AND GENETICS \r 576
PATHOPHYSIOLOGY \r 576
CLINICAL PRESENTATION \r 576
DIFFERENTIAL DIAGNOSIS \r 577
DIAGNOSIS \r 578
TREATMENT \r 579
RESULTS OF SURGICAL THERAPY \r 581
SUMMARY \r 581
REFERENCES 582
Chapter 56 - Imperforate Anus 583
CLASSIFICATION AND DESCRIPTION OF DEFECTS \r 583
ASSOCIATED DEFECTS \r 586
EARLY MANAGEMENT AND DIAGNOSIS \r 586
MAIN REPAIR \r 587
FUNCTIONAL SEQUELAE \r 588
References 590
Chapter 57 - Abnormal Rotation and Fixation of the Intestine 591
EMBRYOLOGY \r 591
ANATOMY \r 591
CLINICAL PRESENTATION \r 594
DIAGNOSIS \r 594
TREATMENT \r 595
COMPLICATIONS, RESULTS, OUTCOME \r 596
SUMMARY \r 597
References 597
Chapter 58 - Small and Large Bowel Stenosis and Atresias 598
DUODENAL STENOSIS AND ATRESIA \r 598
SMALL BOWEL ATRESIA \r 600
COLONIC ATRESIA \r 601
SUMMARY \r 602
References 602
Chapter 59 - Newborn Abdominal Wall Defects 603
HISTORICAL BACKGROUND AND TERMINOLOGY \r 603
EMBRYOGENESIS DICTATES THE TYPE OF DEFECT \r 603
FACTORS THAT MAY CAUSE THE DEFECT \r 604
A CHANGING EPIDEMIOLOGY \r 604
A SPECTRUM OF DEFECTS \r 604
ASSOCIATED ANOMALIES (THE MAJOR DETERMINANT OF MORBIDITY AND MORTALITY) \r 605
PRENATAL MANAGEMENT \r 607
INITIAL MANAGEMENT AFTER DELIVERY \r 607
SPECIFIC MANAGEMENT OF THE DEFECT \r 608
POSTOPERATIVE MANAGEMENT \r 611
ALTERNATIVE METHODS OF CLOSURE \r 612
EXPECTED MORTALITY \r 613
INCREASING HOSPITAL COSTS \r 613
POSTOPERATIVE COMPLICATIONS \r 613
LATE MORBIDITY AND QUALITY OF LIFE \r 614
References 614
Chapter 60 - Stomas of the Small and Large Intestine 615
HISTORICAL NOTE \r 615
CHILD WITH A STOMA \r 615
TYPES OF INTESTINAL STOMA AND THEIR APPLICATIONS \r 616
INDICATIONS FOR ENTEROSTOMIES IN CHILDREN \r 617
CHOICE OF ENTEROSTOMY \r 618
TECHNICAL ASPECTS \r 621
STOMA CARE \r 622
REESTABLISHING INTESTINAL CONTINUITY \r 623
COMPLICATIONS OF ENTEROSTOMIES AND THEIR MANAGEMENT \r 623
OSTOMY TRENDS \r 624
References 624
Section 7 GastrointestinalProcedures 625
Chapter 61 - Esophagogastroduodenoscopy and Related Techniques 626
PERSONNEL \r 626
FACILITIES \r 626
EQUIPMENT \r 627
INDICATIONS \r 628
CONTRAINDICATIONS \r 628
ANTIBIOTIC PROPHYLAXIS \r 628
PREPARATION \r 629
SEDATION AND MONITORING \r 629
ANATOMY \r 630
TECHNIQUE \r 631
BIOPSY TECHNIQUE \r 633
Stomach \r 634
THERAPEUTIC ENDOSCOPY \r 635
INJECTION \r 635
THERMOCOAGULATION \r 636
ELECTROCOAGULATION \r 636
STRICTURE DILATION \r 640
SMALL BOWEL ENTEROSCOPY \r 641
WIRELESS CAPSULE ENDOSCOPY \r 642
PERCUTANEOUS ENDOSCOPIC GASTROSTOMY \r 642
PERCUTANEOUS ENDOSCOPIC JEJUNOSTOMY (PEJ) \r 645
POLYPECTOMY \r 645
SPECIAL ENDOSCOPIC TECHNIQUES \r 645
ENDOSCOPIC ULTRASONOGRAPHY \r 647
CHROMOENDOSCOPY AND NARROW BAND IMAGING \r 647
CONFOCAL LASER MICROSCOPY (ENDOMICROSCOPY) \r 647
NEW TECHNIQUES \r 647
COMPLICATIONS \r 647
References 649
Chapter 62 - Colonoscopy, Polypectomy, and Related Techniques 650
PERSONNEL, SEDATION, AND ANTIBIOTIC PROPHYLAXIS \r 650
EQUIPMENT \r 650
INDICATIONS \r 650
CONTRAINDICATIONS \r 651
PREPARATION \r 652
ANATOMY \r 653
TECHNIQUE \r 654
BIOPSY TECHNIQUE \r 658
THERAPEUTIC COLONOSCOPY \r 659
INJECTION \r 659
THERMOCOAGULATION: HEATER PROBE, MULTIPOLAR PROBES, AND HOT BIOPSY FORCEPS \r 659
STRICTURE DILATION \r 663
PERCUTANEOUS CECOSTOMY \r 663
POLYPECTOMY \r 663
SPECIAL ENDOSCOPIC TECHNIQUES \r 665
COMPLICATIONS \r 666
REFERENCES\r 666
Chapter 63 - Endoscopic Retrograde Cholangiopancreatography 668
PANCREATICOBILIARY IMAGING METHODS \r 668
INDICATIONS FOR ERCP \r 668
CONTRAINDICATIONS TO ERCP \r 669
PREPARATION FOR ERCP \r 669
TECHNIQUE OF ERCP \r 670
BILIARY DISORDERS \r 670
CHOLEDOCHOLITHIASIS \r 671
CHOLEDOCHAL CYST \r 672
BILIARY STRICTURES \r 673
BILIARY LEAKS \r 674
UNUSUAL BILIARY INFECTIONS \r 674
BILIARY DYSKINESIA \r 674
PANCREATIC DISORDERS \r 675
COMPLICATIONS OF ERCP \r 677
SUMMARY \r 677
References 678
Chapter 64 - CAPSULE ENDOSCOPY AND SMALLBOWEL ENTEROSCOPY 679
EQUIPMENT 679
PERFORMANCE OF CE STUDY 679
INDICATIONS 680
LIMITATIONS 682
CONTRAINDICATIONS AND COMPLICATIONS 682
ENTEROSCOPY 682
COMPLICATIONS 684
INTRAOPERATIVE ENTEROSCOPY 684
CONCLUSION 684
REFERENCES 685
Chapter 65 - Gastrointestinal Motility Procedures 686
GASTOINTESTINAL MOTILITY STUDIES \r 686
ESOPHAGEAL MANOMETRY \r 686
ANTRODUODENAL MANOMETRY \r 689
COLONIC MANOMETRY \r 693
ANORECTAL MANOMETRY \r 696
SUMMARY \r 698
References 698
Chapter 66 - Gastrointestinal Pathology 699
ESOPHAGEAL BIOPSY \r 699
STOMACH BIOPSY \r 701
SMALL INTESTINAL BIOPSY \r 702
INTERPRETATION OF COLONIC MUCOSAL BIOPSY SPECIMENS IN THE EVALUATION OF INFLAMMATORY BOWEL DISEASE \r 706
EVALUATION OF RESECTION SPECIMENS IN INFLAMMATORY BOWEL DISEASE \r 709
DISORDERS OF INTESTINAL MOTILITY \r 711
GASTROINTESTINAL POLYPS AND POLYPOSIS SYNDROMES \r 713
ACKNOWLEDGMENTS \r 716
References 716
Section 8 The Liver and Bile Ducts 717
Chapter 67 - Developmental Anatomy and Physiology of the Liver and Bile Ducts 718
EARLY DEVELOPMENT OF THE LIVER IN HUMANS AND OTHER VERTEBRATES 718
DEVELOPMENT OF THE BILIARY TREE \r 720
DEVELOPMENT OF THE HEPATIC VASCULATURE \r 722
THE FUNCTIONAL UNIT OF THE LIVER: THE HEPATIC LOBULE AND SINUSOIDAL SYSTEM \r 722
FUNCTIONAL DEVELOPMENT OF THE LIVER \r 723
LIVER AS A METABOLIC ORGAN \r 724
LIVER AS A FILTER \r 725
LIVER AS A HEMATOPOIETIC ORGAN \r 725
LIVER AS AN IMMUNE ORGAN \r 725
STEM CELLS AND LIVER REGENERATION \r 725
CONCLUSION \r 727
ACKNOWLEDGMENTS \r 727
References 727
Chapter 68 - Neonatal Hepatitis 728
APPROACH TO THE INFANT WITH CHOLESTASIS \r 728
CHOLESTASIS ASSOCIATED WITH INFECTION \r 733
SYSTEMIC CONDITIONS ASSOCIATED WITH NEONATAL HEPATITIS \r 737
IDIOPATHIC NEONATAL HEPATITIS \r 738
MANAGEMENT OF NEONATAL CHOLESTASIS \r 738
SUMMARY \r 740
ACKNOWLEGMENTS \r 740
References 740
Chapter 69 - Biliary Atresia and Neonatal Disorders of the Bile Ducts 741
SURGICALLY CORRECTABLE DISORDERS \r 741
References 751
Chapter 70 - Pediatric Cholestatic Liver Disease with Genetic Etiology 752
DEFECTS IN BILE ACID PRODUCTION \r 752
DEFECTS IN MEMBRANE TRANSPORTERS \r 752
DISORDERS OF EMBRYOGENESIS: ALAGILLE SYNDROME \r 758
DISORDER OF MITOCHONDRIAL FUNCTION \r 763
GENETIC CHOLESTASIS \r 764
References 766
Chapter 71 - Mitochondrial Hepatopathies: 767
FAO DISORDERS \r 767
PHYSIOLOGY OF NORMAL FAO \r 767
EPIDEMIOLOGY AND GENETICS OF FAO DISORDERS \r 771
CLINICAL PRESENTATIONS OF FAO DISORDERS \r 771
DIAGNOSIS OF FAO DISORDERS \r 774
TREATMENT OF DISORDERS OF FAO \r 776
RESPIRATORY CHAIN DISORDERS \r 778
MITOCHONDRIAL RC GENETICS AND BIOLOGY \r 778
EPIDEMIOLOGY OF RC DISORDERS \r 779
CLINICAL PRESENTATION \r 779
GENETICS OF MITOCHONDRIAL HEPATOPATHIES \r 781
DIAGNOSIS OF MITOCHONDRIAL HEPATOPATHIES \r 783
TREATMENT OF MITOCHONDRIAL HEPATOPATHIES \r 784
References 785
Chapter 72 - Abnormalities of Hepatic Protein Metabolism 786
α1-ANTITRYPSIN DEFICIENCY 786
TYROSINEMIA \r 789
UREA CYCLE DEFECTS \r 791
References 794
Chapter 73 - Abnormalities of Carbohydrate Metabolism and the Liver 795
NONALCOHOLIC FATTY LIVER DISEASE \r 795
GALACTOSEMIA \r 797
HEREDITARY FRUCTOSE INTOLERANCE \r 798
FRUCTOSE-1,6-BISPHOSPHATASE DEFICIENCY \r 799
GLYCOGEN STORAGE DISEASE \r 799
References 803
Chapter 74 - Nonalcoholic Fatty Liver Disease 804
DEFINITION AND CAUSES \r 804
PREVALENCE AND DEMOGRAPHIC PREDICTORS \r 804
PATHOPHYSIOLOGY \r 804
NATURAL HISTORY \r 805
SIGNS AND SYMPTOMS \r 805
DIAGNOSIS \r 806
TREATMENT \r 808
PREVENTION AND SCREENING \r 810
CONCLUSION \r 810
References 810
Chapter 75 - Acute and Chronic Hepatitis 811
EVALUATION OF THE CHILD WITH HEPATITIS \r 811
HEPATOTROPIC VIRUSES \r 811
AUTOIMMUNE HEPATITIS \r 823
NONALCOHOLIC FATTY LIVER DISEASE \r 825
TREATMENT OF HEPATITIS \r 827
SUMMARY \r 828
References 828
Chapter 76 - Portal Hypertension 829
PATHOGENESIS OF PORTAL HYPERTENSION \r 829
CLINICAL FEATURES \r 829
DIAGNOSIS \r 833
THERAPY \r 834
References 839
Chapter 77 - Liver Failure 840
CHRONIC LIVER FAILURE \r 840
ACUTE LIVER FAILURE \r 847
SUMMARY \r 852
References 852
Chapter 78 - Liver Transplantation in Children 853
INDICATIONS FOR LIVER TRANSPLANTATION \r 853
GENERAL CONTRAINDICATIONS FOR LIVER TRANSPLANTATION IN CHILDREN \r 855
EVALUATION OF PEDIATRIC LIVER TRANSPLANT CANDIDATES \r 855
PREPARATION FOR LIVER TRANSPLANTATION \r 856
ORGAN ALLOCATION \r 856
LIVER TRANSPLANTATION SURGERY \r 857
POSTTRANSPLANT MANAGEMENT AND COMPLICATIONS \r 859
GROWTH AND DEVELOPMENT AND QUALITY OF LIFE \r 862
NONADHERENCE WITH IMMUNOSUPPRESSION \r 863
SURVIVAL AFTER LIVER TRANSPLANTATION \r 863
LIVER TRANSPLANTATION IN SPECIAL GROUPS \r 863
WITHDRAWAL OF IMMUNOSUPPRESSION AND TOLERANCE \r 864
CONCLUSION \r 864
References 864
Chapter 79 - Diseases of the Gallbladder 866
EPIDEMIOLOGY OF GALLSTONE DISEASE \r 866
PATHOPHYSIOLOGY OF GALLSTONE DISEASE \r 866
CHOLESTEROL STONES \r 868
PIGMENT STONES \r 868
GALLSTONES IN INFANTS \r 869
TOTAL PARENTERAL NUTRITION–ASSOCIATED STONES \r 870
CYSTIC FIBROSIS–ASSOCIATED GALLSTONES \r 870
ILEAL DISEASE AND ILEAL RESECTION-ASSOCIATED STONES \r 870
DRUG-ASSOCIATED STONES \r 870
DIAGNOSIS OF GALLSTONE DISEASE \r 871
TREATMENT OF GALLSTONE DISEASE \r 871
CHOLECYSTITIS \r 872
CHOLEDOCHOLITHIASIS (COMMON BILE DUCT STONES) \r 873
ACALCULOUS CHOLECYSTITIS \r 874
ACUTE HYDROPS OF THE GALLBLADDER \r 875
UNUSUAL DISEASES OF THE GALLBLADDER \r 875
References 875
Section 9 The Pancreas 877
Chapter 80 - Pancreatic Development 878
BUDS FORM AND FUSE \r 878
DUCTAL SYSTEM FORMS \r 878
DIFFERENTIATION OF PANCREATIC PROGENITORS \r 878
PROGRAMMED DEVELOPMENT BY HIERARCHICAL SIGNALS \r 878
REGENERATIVE CAPACITY OF THE PANCREAS \r 881
SUMMARY ON PANCREATIC DEVELOPMENT \r 882
PANCREATIC SECRETION AND EXOCRINE FUNCTION \r 882
References 889
Chapter 81 - Cystic Fibrosis and Congenital Anomalies of the Exocrine Pancreas 890
CYSTIC FIBROSIS \r 890
STRUCTURAL ABNORMALITIES OF THE PANCREAS \r 900
FUNCTIONAL ABNORMALITIES OF THE PANCREAS \r 903
References 904
Chapter 82 - Pancreatitis 905
ACUTE AND CHRONIC PANCREATITIS \r 905
ACUTE PANCREATITIS \r 905
CHRONIC PANCREATITIS \r 911
References 914
Chapter 83 - Total Pancreatectomy with Autoislet Transplantation, and Pancreatic Allotransplantation 915
TOTAL PANCREATECTOMY WITH AUTOISLET TRANSPLANTATION (TP-AIT) \r 915
WHOLE-ORGAN PANCREAS ALLOTRANSPLANTATION \r 919
PANCREATIC ISLET ALLOTRANSPLANTATION \r 924
References 926
Chapter 84 - Secretory Neoplasms of the Pancreas 927
MULTIPLE ENDOCRINE NEOPLASIA\r 927
INSULINOMA \r 928
CONGENITAL HYPERINSULINISM \r 929
GASTRINOMA: ZOLLINGER-ELLISON SYNDROME \r 929
VIPOMA \r 931
OTHER TUMORS \r 933
SUMMARY \r 933
References 934
Chapter 85 - Infant and Toddler Nutrition 935
GROWTH \r 935
BREAST-FEEDING \r 936
FORMULA FEEDING \r 939
COMPLEMENTARY FEEDING \r 940
SUPPLEMENTS \r 941
TODDLERS \r 942
DESCRIPTIVE INFORMATION \r 942
EXPERIMENTAL INFORMATION \r 943
SPECIFIC NUTRIENT DEFICITS \r 944
TODDLERS’ NUTRITON AND STOOLING \r 945
PSYCHOLOGY OF TODDLERS’ NUTRITION \r 945
References 946
Section 10 \rNutrition 947
Chapter 86 - Nutritional Assessment 948
EPIDEMIOLOGY OF NUTRITIONAL DISORDERS IN PEDIATRIC PATIENTS \r 948
CLINICAL SIGNIFICANCE OF NUTRITIONAL ASSESSMENT \r 948
CLINICAL FEATURES OF NORMAL GROWTH AND BODY COMPOSITION IN CHILDREN \r 948
INDICATIONS FOR NUTRITIONAL ASSESSMENT \r 950
COMPONENTS OF NUTRITIONAL ASSESSMENT \r 951
SUMMARY \r 956
ACKNOWLEDGMENTS \r 956
References 956
Chapter 87 - Tubes for Enteric Access 957
EVALUATION\r 957
CONSIDERATIONS FOR SHORT-TERM ACCESS\r 958
CONSIDERATIONS FOR LONG-TERM ACCESS\r 960
GASTROESOPHAGEAL REFLUX AND TUBES\r 962
SUMMARY\r 963
References 963
Chapter 88 - Parenteral Nutrition 964
INDICATIONS\r 964
ROUTE OF ADMINISTRATION\r 964
PARENTERAL NUTRITION COMPONENTS AND REQUIREMENTS\r 965
MONITORING\r 972
COMPLICATIONS\r 972
TOTAL NUTRIENT ADMIXTURES\r 974
FORMULATING A REGIMEN\r 975
CYCLIC PARENTERAL NUTRITION\r 975
HOME PARENTERAL NUTRITION\r 976
MONITORING THE HOME PN PATIENT\r 976
SUMMARY\r 976
References 977
Chapter 89 - Enteral Nutrition 978
MALNUTRITION IN THE HOSPITAL SETTING\r 978
INDICATIONS FOR ENTERAL FEEDING (TABLE 89-1)\r 978
GROWTH FAILURE ASSOCIATED WITH CHRONIC DISEASE\r 981
HYPERMETABOLIC STATES\r 982
CHOICE OF ENTERAL FEEDING ROUTE\r 983
CHOICE OF FEEDING METHOD (CONTINUOUS, NOCTURNAL, OR BOLUS FEEDING)\r 985
ENTERAL NUTRITION IN THE NEONATE\r 985
ENTERAL FEEDING IN THE INFANT AND CHILD\r 988
PRACTICAL ISSUES OF ENTERAL NUTRITION\r 994
SAFETY OF ENTERAL FEEDS\r 997
COMPLICATIONS\r 997
TRANSITION FROM PARENTERAL TO ENTERAL/ORAL NUTRITION\r 1000
SUMMARY\r 1001
References 1001
Chapter 90 - Management of Diarrhea 1002
MANAGEMENT OF ACUTE DIARRHEA\r 1002
MANAGEMENT OF CHRONIC DIARRHEA\r 1009
References 1011
Chapter 91 - Effects of Digestive Diseases on Bone Metabolism 1012
BONE BIOLOGY IN CHILDREN\r 1012
PEAK BONE MASS\r 1013
MEASUREMENT OF BONE METABOLISM AND BONE MASS IN CHILDREN\r 1014
DEFINITION OF OSTEOPOROSIS IN CHILDREN\r 1014
COMMON MECHANISMS BY WHICH DIGESTIVE DISEASES AFFECT BONE MASS\r 1015
SELECTED GASTROINTESTINAL DISEASES ASSOCIATED WITH DECREASED BMD\r 1016
LIVER DISEASES\r 1017
CYSTIC FIBROSIS\r 1018
TREATMENT\r 1018
References 1019
Chapter 92 - Nutrition and Feeding for Children with Developmental Disabilities 1020
NUTRITIONAL GOALS AND RISKS\r 1020
VARIABILITY IN NEEDS\r 1020
CONTRIBUTING FACTORS\r 1021
PRESENTATION AND EVALUATION\r 1024
INTERVENTION\r 1026
ORAL FEEDING\r 1027
ORAL FEEDING PROBLEMS\r 1029
ENTERAL DELIVERY\r 1030
SUMMARY\r 1031
References 1031
Index 1033
Color Plates\r CP1