BOOK
Pediatric Gastrointestinal and Liver Disease E-Book
Robert Wyllie | Jeffrey S. Hyams | Marsha Kay
(2015)
Additional Information
Book Details
Abstract
Consult the leading text in the field that delivers the information you need to diagnose and treat pediatric gastrointestinal and liver diseases effectively. In one convenient and comprehensive volume, Drs. Robert Wyllie, Jeffrey S. Hyams, and Marsha Kay provide all the latest details on the most effective new therapies, new drugs, and new techniques in the specialty. In addition, the new two-color design throughout helps you find what you need quickly and easily.
- Full-color endoscopy images to help improve your visual recognition
- Definitive guidance from renowned international contributors who share their knowledge and expertise in this complex field
- Detailed diagrams that accurately illustrate complex concepts and provide at-a-glance recognition of disease processes
- More than 400 board review-style questions, answers, and rationales
- New therapies for hepatitis B and C, new drugs for the treatment of inflammatory bowel disease, and an expanded discussion of the newest endoscopic and motility techniques available for pediatric patients
- The most current information on diagnosing and treating abnormalities of protein, fat, and carbohydrate metabolism
- New chapters on pancreatic transplantation and liver pathology
- The latest surgical techniques for children with gastrointestinal conditions
Table of Contents
| Section Title | Page | Action | Price |
|---|---|---|---|
| Front Cover | cover | ||
| Inside Front Cover | ifc1 | ||
| Pediatric Gastrointestinal and Liver Disease | i | ||
| Copyright Page | ii | ||
| Dedication | iii | ||
| Contributors | iv | ||
| Preface | xv | ||
| Table Of Contents | xvi | ||
| 1 Biologic Aspects of Gastrointestinal Function | 1 | ||
| 1 Development of the Gastrointestinal Tract | 2 | ||
| Early Development: Patterning the Gastrointestinal Tract | 2 | ||
| Three Germ Layers Contribute to Gastrointestinal Development | 2 | ||
| The Gastrointestinal Tract Develops Along Four Axes | 2 | ||
| Anteroposterior and Dorsoventral Axis | 2 | ||
| Left–Right Axis | 3 | ||
| Radial Axis | 5 | ||
| Later Morphologic and Functional Development: Differentiation | 5 | ||
| Esophagus | 5 | ||
| Stomach and Duodenum | 6 | ||
| Small Intestine and Colon | 6 | ||
| Development of the Gastrointestinal Epithelium | 6 | ||
| Crypts and Villi: Complementary Roles in Development and Homeostasis | 6 | ||
| Later Development and Postnatal Maturation of the Intestine | 7 | ||
| The Microbiota | 7 | ||
| The Enteric Immune System | 7 | ||
| Stem Cells and Recent Applications | 8 | ||
| Conclusion and Future Perspectives | 8 | ||
| Selected References | 8 | ||
| References | 9.e2 | ||
| Questions | 9.e1 | ||
| Answers | 9.e1 | ||
| 2 Basic Aspects of Digestion and Absorption | 10 | ||
| Key Principles of Digestion and Absorption | 10 | ||
| Carbohydrates | 10 | ||
| Dietary Forms | 10 | ||
| Luminal Digestion | 11 | ||
| Brush Border Digestion | 12 | ||
| Transport After Digestion | 13 | ||
| Nondigestible Carbohydrates | 13 | ||
| Proteins | 13 | ||
| Protein Sources | 13 | ||
| Luminal Digestion | 13 | ||
| Gastric Phase | 13 | ||
| Intestinal Phase | 14 | ||
| Brush Border and Intracellular Digestion | 14 | ||
| Brush Border | 14 | ||
| Cytoplasm | 15 | ||
| Transport After Digestion | 15 | ||
| Amino Acids | 15 | ||
| Dipeptides and Tripeptides | 15 | ||
| Exit From the Enterocyte | 15 | ||
| Lipids | 15 | ||
| Dietary Forms | 15 | ||
| Luminal Digestion | 16 | ||
| Gastric Phase | 16 | ||
| Small Intestinal Phase | 16 | ||
| Enterohepatic Bile Circulation | 17 | ||
| Transport of Fat Digestion Products | 18 | ||
| Intracellular Phase of Fat Assimilation | 18 | ||
| Exit From the Enterocyte | 18 | ||
| Digestion and Absorption in Infants | 18 | ||
| Carbohydrates | 19 | ||
| Proteins | 19 | ||
| Lipids | 19 | ||
| Vitamins and Minerals | 19 | ||
| Fat-Soluble Vitamins | 19 | ||
| Vitamin A | 19 | ||
| Vitamin D | 19 | ||
| Vitamin K and Vitamin E | 20 | ||
| Water-Soluble Vitamins | 20 | ||
| Vitamin B12 | 20 | ||
| Folate | 20 | ||
| Vitamin C | 20 | ||
| Other Water-Soluble Vitamins | 20 | ||
| Minerals and Trace Elements | 20 | ||
| Calcium | 21 | ||
| Iron | 21 | ||
| Magnesium, Phosphorus, Zinc, and Copper (Table 2-7) | 21 | ||
| Acknowledgment | 21 | ||
| Selected References | 21 | ||
| References | 21.e2 | ||
| Questions | 21.e1 | ||
| Answers | 21.e1 | ||
| 3 Bile Acid Physiology and Alterations in the Enterohepatic Circulation | 22 | ||
| Biosynthesis | 22 | ||
| Enterohepatic Circulation | 22 | ||
| Maturation of the Enterohepatic Circulation | 24 | ||
| Alterations in the Enterohepatic Circulation | 24 | ||
| Alteration of Primary Bile Acid Biosynthesis | 24 | ||
| Cerebrotendinous Xanthomatosis | 25 | ||
| 3β-Hydroxy-C27-Steroid Oxidoreductase Deficiency | 25 | ||
| Δ4-3-Oxosteroid 5β-Reductase Deficiency | 25 | ||
| Oxysterol 7α-Hydroxylase Deficiency | 26 | ||
| 2-Methylacyl-CoA Racemase Deficiency | 26 | ||
| THCA-CoA Oxidase Deficiency | 26 | ||
| Bile Acid–CoA Ligase Deficiency and Defective Amidation | 26 | ||
| Cholesterol 7α-Hydroxylase Deficiency | 27 | ||
| Peroxisomal Disorders | 27 | ||
| Alteration of Hepatic Bile Acid Transport | 27 | ||
| Alteration in Cholestasis (General) | 28 | ||
| Alteration of the Enterohepatic Circulation of Bile Acids | 28 | ||
| Mechanism of Bile Acid–Induced Diarrhea | 29 | ||
| Summary | 29 | ||
| Selected References | 30 | ||
| References | 30.e2 | ||
| Questions | 30.e1 | ||
| Answers | 30.e1 | ||
| 4 Indigenous Flora | 31 | ||
| Understanding the Indigenous Flora by Studying Germ-Free Animals | 31 | ||
| Establishing the Indigenous Flora | 32 | ||
| Bacterial Flora within the Various Sections of the Gastrointestinal Tract | 34 | ||
| Oral Flora | 34 | ||
| Esophageal Flora | 34 | ||
| Stomach Flora | 34 | ||
| Small Bowel Flora | 34 | ||
| Colonic Flora | 35 | ||
| Controlling the Growth of the Indigenous Population | 35 | ||
| Symbiosis Between Host and Fecal Flora | 36 | ||
| Bacterial Flora in Illness | 37 | ||
| Bacterial Flora and Allergy | 37 | ||
| Bacterial Flora and Antibiotics | 38 | ||
| Bacterial Overgrowth | 38 | ||
| Tropical Sprue | 39 | ||
| Probiotics | 39 | ||
| Probiotics and Promotion of Health | 40 | ||
| Immunomodulation | 40 | ||
| Cholesterol Levels | 40 | ||
| Probiotics and Disease | 40 | ||
| Diarrhea | 40 | ||
| Allergy | 41 | ||
| Inflammatory Bowel Disease | 41 | ||
| Prebiotics | 41 | ||
| Synbiotics | 42 | ||
| Selected References | 42 | ||
| References | 42.e3 | ||
| Questions | 42.e1 | ||
| Answers | 42.e1 | ||
| 5 Gastrointestinal Mucosal Immunology and Mechanisms of Inflammation | 43 | ||
| The Components of Gut Immune Responses | 43 | ||
| The Generation of Inflammation | 44 | ||
| Mechanisms That Prevent Inflammatory Reactions to Gut Luminal Contents | 44 | ||
| Epithelial Integrity | 44 | ||
| IgA Production | 45 | ||
| Regulatory Lymphocytes | 46 | ||
| Coordinated Immune Responses | 46 | ||
| The Gut Flora | 46 | ||
| Micronutrients | 46 | ||
| Organization of the Mucosal Immune System | 46 | ||
| Components of the Mucosal Immune System | 47 | ||
| Innate Immunity within the Intestine | 47 | ||
| Dendritic Cells within the Intestine | 48 | ||
| Conserved Pattern-Recognition Receptors and Dendritic Cell Function | 49 | ||
| Subgroups of Dendritic Cells | 49 | ||
| Dendritic Cells and Induction of Immune Tolerance within the Intestine | 49 | ||
| Dendritic Cells and Effector Immune Responses to Pathogens | 50 | ||
| Intestinal Macrophages | 50 | ||
| Polymorphonuclear Neutrophils | 50 | ||
| Eosinophils, Basophils, and Mast Cells | 51 | ||
| Adaptive Immunity within the Intestine | 51 | ||
| Archaic Lymphocyte Populations | 51 | ||
| B-Lymphocyte Populations | 51 | ||
| Homing and Recruitment of B Lymphocytes | 52 | ||
| Induction of Mucosal IgG and IgE Responses | 52 | ||
| T-Cell Populations in the Intestine | 52 | ||
| T Helper Cells (CD4+ Cells) | 52 | ||
| T Cytotoxic Cells (CD8+ Cells) | 53 | ||
| T Regulatory Cells | 53 | ||
| The Establishment and Maintenance of Oral Tolerance to Antigens and the Flora | 54 | ||
| Mechanisms of Oral Tolerance | 54 | ||
| The Importance of Early Life Exposures | 54 | ||
| Patterns of Inflammatory Response within the Intestine | 55 | ||
| Acute Inflammation Induced by Pathogens | 55 | ||
| Chronic Immune-Mediated Inflammation | 56 | ||
| TH1- or TH17-Dominated Responses | 56 | ||
| TH2-Dominated Responses | 56 | ||
| Inflammation Induced by Vascular or Neural Damage | 56 | ||
| Conclusions | 57 | ||
| Selected References | 57 | ||
| References | 57.e3 | ||
| Questions | 57.e1 | ||
| Answers | 57.e1 | ||
| 2 Clinical Problems | 58 | ||
| 6 Chronic Abdominal Pain of Childhood and Adolescence | 59 | ||
| Pathophysiology of AP-FGIDs | 59 | ||
| Nature versus Nurture | 60 | ||
| Psychological Comorbidities | 61 | ||
| Autonomic Dysfunction | 62 | ||
| Early Life Events and AP-FGIDs | 62 | ||
| Role of Infections | 63 | ||
| Role of Visceral Hypersensitivity | 64 | ||
| Alterations in Motility | 64 | ||
| Increased Permeability | 64 | ||
| Altered Microbiota | 65 | ||
| Immune Dysfunction | 65 | ||
| Food and IBS | 66 | ||
| Clinical Evaluation | 68 | ||
| Treatment | 69 | ||
| Elimination Diets in AP-FGIDs | 70 | ||
| Psychological Treatments | 71 | ||
| Prognosis | 72 | ||
| Summary | 72 | ||
| Selected References | 72 | ||
| References | 73.e2 | ||
| Questions | 73.e1 | ||
| Answers | 73.e1 | ||
| 7 Approach to the Child With a Functional Gastrointestinal Disorder | 74 | ||
| Biomedical Model | 74 | ||
| What are the Defining Characteristics of Functional Disorders? | 74 | ||
| Biopsychosocial Model | 75 | ||
| Early Learning: Developmental Aspects of Functional Gastrointestinal Disorders | 75 | ||
| First Visit | 76 | ||
| Effective Reassurance | 76 | ||
| Biomedical Versus Biopsychosocial Models | 77 | ||
| Treatment | 78 | ||
| Approach to the Child or Adolescent with Pain-Associated Disability Syndrome | 79 | ||
| Feeding Disorders of Infants and Young Children | 79 | ||
| What should a Pediatric Gastroenterology Consultation Accomplish? | 81 | ||
| Pediatric Functional Gastrointestinal Disorders | 81 | ||
| Conclusion | 83 | ||
| Selected References | 83 | ||
| References | 83.e2 | ||
| Questions | 83.e1 | ||
| Answers | 83.e1 | ||
| 8 Vomiting and Nausea | 84 | ||
| Vomiting Event | 84 | ||
| Definition | 84 | ||
| Physical Description | 84 | ||
| Gastrointestinal Motor Activity During Nausea and Vomiting | 85 | ||
| Emetic Reflex | 85 | ||
| Neurochemical Basis | 86 | ||
| Clinical Aspects of Vomiting | 86 | ||
| Temporal Patterns | 86 | ||
| Differential Diagnosis | 87 | ||
| Clinical Clues to Diagnosis | 90 | ||
| Evaluation | 91 | ||
| Complications | 92 | ||
| Pharmacologic Treatment | 93 | ||
| Nausea | 93 | ||
| Clinical Clues and Differential Diagnosis | 96 | ||
| Evaluation | 96 | ||
| Pharmacologic Treatment | 97 | ||
| Chronic Idiopathic Nausea | 97 | ||
| Specific Vomiting Disorders | 98 | ||
| Cyclic Vomiting Syndrome and Abdominal Migraine | 98 | ||
| Postoperative Nausea and Vomiting | 99 | ||
| Chemotherapy-Induced Emesis | 102 | ||
| Functional Vomiting | 102 | ||
| Selected References | 103 | ||
| References | 103.e3 | ||
| Questions | 103.e1 | ||
| Answers | 103.e1 | ||
| 9 Diarrhea | 104 | ||
| Definition and Epidemiology | 104 | ||
| Pathophysiology of Stool Formation | 104 | ||
| Classification of Diarrhea | 105 | ||
| Pathophysiology of Secretory Diarrhea | 105 | ||
| Pathophysiology of Osmotic Diarrhea | 105 | ||
| Pathophysiology of Inflammatory Diarrhea | 106 | ||
| Differential Diagnosis | 107 | ||
| Infectious Causes | 107 | ||
| Malabsorptive Diarrheas | 108 | ||
| Carbohydrate Malabsorption | 108 | ||
| Fat Malabsorption | 108 | ||
| Cholestatic Conditions | 108 | ||
| Exocrine Pancreatic Insufficiency | 108 | ||
| Conditions Causing Decreased Enterohepatic Circulation of Bile Salts | 108 | ||
| Postenteritis Syndrome | 109 | ||
| Chronic Diarrhea of Inflammatory Origin | 109 | ||
| Microscopic or Collagenous Colitis | 109 | ||
| Eosinophilic Gastroenteritis | 109 | ||
| Celiac Disease | 109 | ||
| Irritable Bowel Syndrome | 110 | ||
| Intestinal Failure (Short Bowel Syndrome) | 110 | ||
| Congenital Diarrheas | 110 | ||
| Microvillus Inclusion Disease | 110 | ||
| Tufting Enteropathy | 110 | ||
| Autoimmune Enteropathy | 110 | ||
| Congenital Chloride Diarrhea | 110 | ||
| Congenital Sodium Diarrhea | 110 | ||
| Glucose–Galactose Malabsorption | 111 | ||
| Congenital Sucrase–Isomaltase Deficiency | 111 | ||
| Congenital Lactase Deficiency | 111 | ||
| Medication-induced Diarrhea | 111 | ||
| Neurohormonal Causes of Diarrhea | 111 | ||
| Approach to a Child With Diarrhea | 111 | ||
| Approach to a Child With Acute Diarrhea | 111 | ||
| Approach to a Child With Chronic Diarrhea | 112 | ||
| Watery Diarrhea (Osmotic/Secretory) | 113 | ||
| Inflammatory Diarrhea | 113 | ||
| Fatty Diarrhea | 114 | ||
| Summary | 114 | ||
| Acknowledgment | 114 | ||
| Selected References | 114 | ||
| References | 114.e2 | ||
| Questions | 114.e1 | ||
| Answers | 114.e1 | ||
| 10 Colic and Gastrointestinal Gas | 115 | ||
| Gastrointestinal Gas | 115 | ||
| Composition of Gastrointestinal Gas | 115 | ||
| Sources and Relative Distribution of Gastrointestinal Gases | 115 | ||
| Symptoms Attributable to Gastrointestinal Gas | 115 | ||
| Eructation | 116 | ||
| Abdominal Distension | 116 | ||
| Flatulence | 116 | ||
| Methane and Constipation | 117 | ||
| Colic | 117 | ||
| Proposed Etiologies of Infantile Colic | 118 | ||
| Excessive Gastrointestinal Gas | 118 | ||
| Carbohydrate Malabsorption | 118 | ||
| Mode of Feeding | 119 | ||
| Protein Allergy/Intolerance | 119 | ||
| Motility | 119 | ||
| Gastroesophageal Reflux | 119 | ||
| Gut Hormones | 120 | ||
| Altered Intestinal Flora | 120 | ||
| Psychosocial Factors | 120 | ||
| Management of Infantile Colic | 121 | ||
| Limiting Aerophagia | 121 | ||
| Dietary Management | 121 | ||
| Pharmacologic Therapy | 121 | ||
| Alternative Therapies | 122 | ||
| Behavioral Interventions | 122 | ||
| Complications and Long-Term Outcomes | 122 | ||
| Conclusions | 123 | ||
| Selected References | 123 | ||
| References | 123.e2 | ||
| Questions | 123.e1 | ||
| Answers | 123.e1 | ||
| 11 Functional Constipation and Incontinence | 124 | ||
| Definitions | 124 | ||
| Normal Stooling Pattern | 124 | ||
| 3 The Esophagus | 231 | ||
| 20 Congenital Malformations of the Esophagus | 232 | ||
| Congenital Lesions of the Esophagus | 232 | ||
| Congenital Esophageal Stenosis | 232 | ||
| Clinical Manifestations and Diagnosis | 232 | ||
| Treatment | 232 | ||
| Outcome | 233 | ||
| Esophageal Atresia and Tracheoesophageal Fistula | 233 | ||
| Anatomy | 233 | ||
| Associated Anomalies | 234 | ||
| Clinical Presentation and Diagnosis | 235 | ||
| Treatment | 235 | ||
| Special Situations | 237 | ||
| Postoperative Complications | 238 | ||
| Outcome | 239 | ||
| Laryngotracheoesophageal Clefts | 240 | ||
| Clinical Presentation and Diagnosis | 241 | ||
| Treatment | 241 | ||
| Outcome | 242 | ||
| Select References | 242 | ||
| References | 242.e2 | ||
| Questions | 242.e1 | ||
| Answers | 242.e1 | ||
| 21 Gastroesophageal Reflux | 243 | ||
| Introduction | 243 | ||
| Prevalence | 243 | ||
| Pathophysiology | 243 | ||
| Symptoms and Signs | 244 | ||
| GER and Regurgitation | 246 | ||
| GER(D) and Cow’s Milk Protein Allergy | 246 | ||
| GERD and Esophagitis | 246 | ||
| GER(D) and Eosinophilic Esophagitis | 247 | ||
| GER(D) and Heartburn | 247 | ||
| GER(D) and Distressed Behavior in Infants | 248 | ||
| GERD and Sandifer’s Syndrome | 248 | ||
| GER(D) and Respiratory Disorders | 248 | ||
| GER(D) and Apnea, ALTE, and SIDS | 248 | ||
| GER(D) and Cystic Fibrosis (CF) | 249 | ||
| GER(D) and Upper Airway Disease: ENT-Manifestations | 249 | ||
| GER(D) and Dental Erosions | 249 | ||
| GER(D) and Neurologic Impairment | 249 | ||
| GER(D) and Other Risk Groups | 250 | ||
| GERD and Complications | 250 | ||
| Diagnosis | 250 | ||
| Therapeutic Options | 252 | ||
| Complications of Nonintervention | 252 | ||
| Nonpharmacologic and Nonsurgical Therapies for GER | 253 | ||
| Pharmacologic Therapies for GER | 255 | ||
| Alginate (Antacids) and Mucosa protectors | 255 | ||
| Histamine-2 Receptor Antagonists and Proton Pump Inhibitors | 255 | ||
| Prokinetics and Other Non–Acid-Reducing/Blocking Medication | 256 | ||
| Surgery and Therapeutic Endoscopic Procedures | 257 | ||
| Conclusion | 258 | ||
| Selected References | 258 | ||
| References | 258.e2 | ||
| Questions | 258.e1 | ||
| Answers | 258.e1 | ||
| 22 Achalasia and Other Motor Disorders | 259 | ||
| Disorders of the Proximal Esophagus | 259 | ||
| Upper Esophageal Sphincter Achalasia | 259 | ||
| Cricopharyngeal Incoordination | 261 | ||
| Upper Esophageal Sphincter Hypotension | 261 | ||
| Disorders of the Distal Esophagus | 262 | ||
| Esophageal Achalasia | 262 | ||
| Incidence and Heredity | 262 | ||
| Pathogenesis | 262 | ||
| Associations | 263 | ||
| Triple A Syndrome. | 263 | ||
| Rozycki Syndrome. | 263 | ||
| Other Associations. | 263 | ||
| Diagnosis | 263 | ||
| Radiography. | 263 | ||
| Manometry. | 263 | ||
| Endoscopy. | 263 | ||
| Radionuclide Tests. | 263 | ||
| Differential Diagnosis | 264 | ||
| Treatment | 264 | ||
| Pneumatic Dilation. | 264 | ||
| Surgery. | 265 | ||
| Drug Therapy. | 265 | ||
| Botulinum Toxin Injection. | 265 | ||
| New Treatments | 265 | ||
| Nonachalasia Esophageal Motility Disorders | 266 | ||
| Diffuse Esophageal Spasm | 266 | ||
| Nutcracker Esophagus | 266 | ||
| Nonspecific Esophageal Motility Disorder | 266 | ||
| Treatment of Nonachalasia Esophageal Motility Disorders | 267 | ||
| Secondary Esophageal Motility Disorders | 267 | ||
| Intestinal Pseudoobstruction | 267 | ||
| Hirschsprung’s Disease | 267 | ||
| Connective Tissue Disease | 267 | ||
| Esophageal Atresia and Tracheoesophageal Fistula | 267 | ||
| Dysmotility Due to Esophageal Scar Formation | 267 | ||
| Selected References | 267 | ||
| References | 267.e3 | ||
| Questions | 267.e1 | ||
| Answers | 267.e2 | ||
| 23 Other Diseases of the Esophagus | 268 | ||
| Esophageal Symptoms | 268 | ||
| Medication-Induced Esophagitis | 268 | ||
| Treatment | 268 | ||
| Infections | 269 | ||
| Candida Esophagitis | 269 | ||
| Treatment | 270 | ||
| Herpes Simplex Esophagitis | 271 | ||
| Treatment | 271 | ||
| Immunocompetent | 271 | ||
| Immunocompromised | 272 | ||
| Cytomegalovirus Esophagitis | 272 | ||
| Treatment | 273 | ||
| Rare Infections | 273 | ||
| Other Esophageal Pathogens | 273 | ||
| Other Inflammatory Causes of “Esophageal Symptoms” | 273 | ||
| Graft Versus Host Disease | 273 | ||
| Radiation Induced Esophagitis/ Esophageal Strictures | 274 | ||
| Noninfectious Postchemotherapy Esophagitis | 274 | ||
| HIV-Associated Esophageal Ulcers | 274 | ||
| Epidermolysis Bullosa | 274 | ||
| Esophageal Tumors | 275 | ||
| Selected References | 275 | ||
| References | 275.e2 | ||
| Questions | 275.e1 | ||
| Answers | 275.e1 | ||
| 4 The Stomach | 276 | ||
| 24 Developmental Anatomy and Physiology of the Stomach | 277 | ||
| Histology | 277 | ||
| Neuromuscular Functions | 279 | ||
| Gastric Secretions | 280 | ||
| Gastric Acid Secretion | 280 | ||
| Hydrochloric Acid Secretion | 280 | ||
| Stimulants of Gastric Acid Secretion | 281 | ||
| Gastrin | 281 | ||
| Histamine | 281 | ||
| Acetylcholine | 281 | ||
| Other Stimulating Factors | 281 | ||
| Inhibitors of Gastric Acid Secretion | 282 | ||
| Somatostatin | 282 | ||
| Cholecystokinin | 282 | ||
| Prostaglandins | 282 | ||
| Secretin | 282 | ||
| Other Hormones and Peptides | 282 | ||
| Leptin | 282 | ||
| Ghrelin | 282 | ||
| Adrenomedullin | 282 | ||
| Nefstatin-1 | 282 | ||
| Calcitonin Gene–Related Peptide | 282 | ||
| Pepsinogen | 282 | ||
| Mucus and Bicarbonate Secretion | 283 | ||
| Gastric Lipase | 283 | ||
| Intrinsic Factor | 283 | ||
| Selected References | 283 | ||
| References | 283.e3 | ||
| Questions | 283.e1 | ||
| Answers | 283.e1 | ||
| 25 Congenital Anomalies and Surgical Disorders of the Stomach | 284 | ||
| Embryology | 284 | ||
| Hypertrophic Pyloric Stenosis | 284 | ||
| History, Incidence, and Etiology | 284 | ||
| Clinical Presentation | 284 | ||
| Diagnostic Evaluation | 285 | ||
| Treatment | 285 | ||
| Outcomes | 286 | ||
| Foveolar Hyperplasia | 286 | ||
| Congenital Gastric Outlet Obstruction | 287 | ||
| Clinical Presentation | 287 | ||
| Diagnostic Evaluation | 288 | ||
| Treatment | 288 | ||
| Gastric Volvulus | 288 | ||
| Etiology and Pathophysiology | 288 | ||
| Clinical Presentation | 289 | ||
| Diagnostic Evaluation | 290 | ||
| Treatment | 290 | ||
| Congenital Microgastria | 290 | ||
| Gastric Perforation | 290 | ||
| Gastric and Duodenal Duplications | 291 | ||
| Polyps | 291 | ||
| Tumors | 292 | ||
| Gastric foreign bodies | 292 | ||
| Acknowledgment | 293 | ||
| Selected References | 293 | ||
| References | 293.e2 | ||
| Questions | 293.e1 | ||
| Answers | 293.e1 | ||
| 26 Gastritis, Gastropathy, and Ulcer Disease | 294 | ||
| Incidence | 294 | ||
| Anatomy, Histology, and Pathophysiology | 294 | ||
| Clinical Presentation | 295 | ||
| Diagnostic Testing | 296 | ||
| Radiographic Imaging | 296 | ||
| Endoscopy | 296 | ||
| Pathologic Features of Gastritis | 297 | ||
| Etiology and Types of Gastritis, Gastropathies, and Ulcer Disease | 298 | ||
| Helicobacter Pylori Gastritis | 298 | ||
| Stress Gastropathy | 298 | ||
| Neonatal Gastropathy | 298 | ||
| Trauma | 299 | ||
| Exercise-Induced Gastropathy | 299 | ||
| Zollinger-Ellison Syndrome and Other Hypersecretory States | 299 | ||
| Autoimmune Gastritis | 299 | ||
| Inflammatory Bowel Disease | 300 | ||
| Celiac Disease | 300 | ||
| Eosinophilic and Allergic Gastritis | 300 | ||
| Granulomatous Gastritis | 301 | ||
| Infectious | 301 | ||
| Cytomegalovirus | 301 | ||
| Other Viruses | 302 | ||
| Parasites | 302 | ||
| Fungi | 302 | ||
| Bacteria | 302 | ||
| Phlegmonous Gastritis and Emphysematous Gastritis | 303 | ||
| Medications | 303 | ||
| NSAIDs | 303 | ||
| Corticosteroids | 303 | ||
| Proton Pump Inhibitor Gastritis | 303 | ||
| Other Medications | 304 | ||
| Vascular Gastropathies | 304 | ||
| Portal Hypertensive Gastropathy | 304 | ||
| Gastric Antral Vascular Ectasia | 304 | ||
| Lymphocytic Gastritis | 304 | ||
| Graft versus Host Disease | 304 | ||
| Uremic Gastropathy | 305 | ||
| Henoch-Schönlein Gastritis | 305 | ||
| Bile Gastropathy | 305 | ||
| Corrosive Gastropathy and Caustic Ingestions | 305 | ||
| Collagenous | 306 | ||
| Ménétrier’s Disease | 306 | ||
| Chronic Varioliform Gastritis | 306 | ||
| Radiation Gastritis | 306 | ||
| Cystinosis | 306 | ||
| Treatment | 306 | ||
| Acid Suppression | 306 | ||
| Endoscopy | 307 | ||
| The Microbiome and Gastritis | 307 | ||
| Selected References | 308 | ||
| References | 308.e2 | ||
| Questions | 308.e1 | ||
| Answers | 308.e1 | ||
| 27 Helicobacter pylori in Childhood | 309 | ||
| Epidemiology | 309 | ||
| Socioeconomic Risk Factors | 309 | ||
| Transmission of Infection | 309 | ||
| Prevalence | 309 | ||
| Incidence | 310 | ||
| Spontaneous Bacterial Clearance | 310 | ||
| Reinfection and Recrudescence | 310 | ||
| Host Factors and Responses | 310 | ||
| Host Genotype | 310 | ||
| The Host Immune Response | 311 | ||
| Innate Immune Receptors | 311 | ||
| Autophagy | 311 | ||
| Myeloid Cells—Macrophages | 312 | ||
| Myeloid Cells—Dendritic Cells | 312 | ||
| Myeloid Cells—B Lymphocytes | 312 | ||
| Myeloid Cells—T Lymphocytes | 313 | ||
| Apoptosis and Neoplasia | 313 | ||
| Acid Homeostasis | 313 | ||
| Putative Hormonal Effects | 314 | ||
| Bacterial Factors and Adaptations | 314 | ||
| Genetic Diversity | 314 | ||
| Colonization and Environmental Adaptation | 314 | ||
| Mucosal Adherence | 314 | ||
| cag Pathogenicity Island | 315 | ||
| The Plasticity Region | 315 | ||
| Vacuolating Cytotoxin A, VacA | 316 | ||
| Pathoepigenetics | 316 | ||
| Host Immune Evasion and Manipulation | 316 | ||
| Antibiotic Resistance—The Bacterial Perspective | 318 | ||
| Disease Associations | 318 | ||
| Gastrointestinal Manifestations | 318 | ||
| Gastritis | 318 | ||
| Ulcer Disease | 318 | ||
| Gastroesophageal Reflux and Reflux Disease | 319 | ||
| Gastric Carcinoma | 319 | ||
| Mucosa-Associated Lymphoid Tissue Lymphoma | 319 | ||
| Extraintestinal Manifestations | 319 | ||
| Iron Deficiency Anemia | 319 | ||
| Short Stature | 320 | ||
| Other Suggested Associations | 320 | ||
| Benefits of Disease | 320 | ||
| Diagnosis | 321 | ||
| Invasive Tests | 321 | ||
| Endoscopy and Biopsy | 321 | ||
| Culture | 322 | ||
| Rapid Urease Test | 322 | ||
| Histopathology | 322 | ||
| Noninvasive Tests | 323 | ||
| Urea Breath Test | 323 | ||
| Serologic Tests | 323 | ||
| Stool Antigen Test | 324 | ||
| Summary | 324 | ||
| When is Testing Indicated? | 324 | ||
| Treatment | 324 | ||
| Conventional Regimens | 324 | ||
| Sequential Regimens | 325 | ||
| Treatment Failure | 326 | ||
| Antibiotic Resistance—A Clinical Perspective | 326 | ||
| Adjunctive Therapies | 326 | ||
| Vaccination | 326 | ||
| Conclusion | 327 | ||
| Selected References | 327 | ||
| References | 327.e3 | ||
| Questions | 327.e1 | ||
| Answers | 327.e1 | ||
| Multiple Choice Questions | 327.e2 | ||
| Answers | 327.e2 | ||
| 28 Gastric Motility Disorders | 328 | ||
| Gastric Motor Physiology | 328 | ||
| Evaluation of Gastric Motor and Sensory Disorders | 328 | ||
| Gastric Emptying Scintigraphy | 328 | ||
| Ultrasonography | 330 | ||
| Magnetic Resonance Imaging (MRI) | 330 | ||
| 13C Breath Testing | 330 | ||
| Single Photon Emission Computed Tomography | 330 | ||
| Nutrient Drinking Test | 330 | ||
| Wireless Motility Capsule | 330 | ||
| Antroduodenal Manometry | 330 | ||
| Electrogastrogram | 331 | ||
| Barostat | 331 | ||
| Rumination Syndrome | 332 | ||
| Infancy | 332 | ||
| Childhood and Adolescence | 332 | ||
| Gastroparesis | 334 | ||
| Dumping Syndrome | 336 | ||
| Functional Dyspepsia | 337 | ||
| Selected References | 339 | ||
| References | 339.e2 | ||
| Questions | 339.e1 | ||
| Answers | 339.e1 | ||
| 29 Bezoars | 340 | ||
| Definitions | 340 | ||
| Epidemiology | 340 | ||
| Classification and Pathogenesis | 340 | ||
| Phytobezoars | 340 | ||
| Diospyrobezoars (Persimmon Bezoars) | 340 | ||
| Trichobezoars | 340 | ||
| Lactobezoars | 340 | ||
| Paper Bezoars | 341 | ||
| Pharmacobezoars | 341 | ||
| Cement Bezoars | 341 | ||
| Yeast Bezoars | 341 | ||
| Shellac Bezoars | 341 | ||
| Polybezoars | 341 | ||
| Chewing Gum Bezoars | 341 | ||
| Clinical Presentation | 341 | ||
| Diagnosis | 342 | ||
| Treatment | 342 | ||
| Lavage/Dissolution | 342 | ||
| Retrieval | 343 | ||
| Fragmentation | 343 | ||
| Prevention | 343 | ||
| Suggested References | 343 | ||
| References | 343.e2 | ||
| Questions | 343.e1 | ||
| Answers | 343.e1 | ||
| 5 The Small and Large Intestines | 344 | ||
| 30 Anatomy and Physiology of the Small and Large Intestines | 345 | ||
| Intestinal Anatomy | 345 | ||
| Development of the Intestines | 345 | ||
| Anatomy of the Small Intestine | 345 | ||
| Anatomy of the Large Intestine | 346 | ||
| Cecum | 347 | ||
| Vermiform Appendix | 347 | ||
| Ascending Colon | 347 | ||
| Transverse Colon | 348 | ||
| Descending Colon | 348 | ||
| Sigmoid Colon | 348 | ||
| Rectum | 348 | ||
| Anal Canal | 348 | ||
| Intestinal Histology and Cellular Morphology | 348 | ||
| Serosa | 349 | ||
| Muscularis Propria | 349 | ||
| Submucosa | 349 | ||
| Mucosa | 350 | ||
| Absorptive Cells | 352 | ||
| Surface Epithelial Cell | 354 | ||
| Goblet Cells | 354 | ||
| Gut Endocrine Cells | 354 | ||
| Paneth Cells | 355 | ||
| Cup Cells | 355 | ||
| Tuft Cells | 355 | ||
| Microfold Cells | 356 | ||
| Physiology of Water and Ion Transport | 356 | ||
| Absorption | 356 | ||
| Secretion | 357 | ||
| Daily Gastrointestinal Tract Fluid Fluxes | 358 | ||
| Endogenous Neuroendocrine and Paracrine Regulation of Absorption and Secretion | 358 | ||
| Selected References | 359 | ||
| References | 359.e2 | ||
| Questions | 359.e1 | ||
| Answers | 359.e1 | ||
| 31 Maldigestion and Malabsorption | 360 | ||
| Lipids | 360 | ||
| Decreased Chylomicron Formation | 361 | ||
| Proteins and Amino Acids | 361 | ||
| Compromised Intraluminal Digestion | 362 | ||
| Compromised Mucosal Digestion and Absorption of Oligopeptides and Amino Acids | 362 | ||
| Congenital Disorders of Proteolytic Enzyme Secretion and Amino Acid Transport (Table 31-1) | 362 | ||
| Carbohydrates | 362 | ||
| Defective Intraluminal Hydrolysis of Carbohydrates | 364 | ||
| Disaccharidase Deficiencies (Table 31-3) | 365 | ||
| Sucrase-Isomaltase Deficiency | 365 | ||
| Carbohydrate Transport Defects (Table 31-3) | 366 | ||
| Vitamins and Minerals | 366 | ||
| Fat-Soluble Vitamins (Table 31-4) | 366 | ||
| Water-Soluble Vitamins (Table 31-4) | 366 | ||
| Minerals (Table 31-4) | 368 | ||
| Special Situations | 369 | ||
| Malabsorption after Bariatric Surgery | 369 | ||
| Maldigestion and Malabsorption in Immunodeficiency States | 369 | ||
| Medication- and Supplement-Associated Malabsorption | 369 | ||
| Diagnostic Considerations | 370 | ||
| The Physical Examination | 371 | ||
| Laboratory Evaluation | 371 | ||
| Stool Assessment | 372 | ||
| Rarely Used Laboratory Methods | 374 | ||
| Imaging of the Gastrointestinal Tract and Digestive Organs | 374 | ||
| Abdominal Computerized Tomography and Magnetic Resonance Imaging | 374 | ||
| Video Capsule Endoscopy and Device-Assisted Enteroscopy | 375 | ||
| Breath Testing for Hydrogen and Methane | 375 | ||
| Endoscopy with Mucosal Biopsy | 375 | ||
| Conclusion | 375 | ||
| Acknowledgments | 375 | ||
| Selected References | 375 | ||
| References | 375.e2 | ||
| Questions | 375.e1 | ||
| Answers | 375.e1 | ||
| 32 Protracted Diarrhea | 376 | ||
| Definition and Epidemiology | 376 | ||
| Classification of Protracted Diarrhea | 376 | ||
| Assessment of the Patient with Protracted Diarrhea | 378 | ||
| Clinical History and Physical Examination | 378 | ||
| Diagnostic Stool Studies | 379 | ||
| Hematologic Studies | 380 | ||
| Hydrogen Breath Test | 380 | ||
| Sweat Test | 380 | ||
| Endoscopy and Histologic Sampling | 381 | ||
| Causes of Protracted Diarrhea | 381 | ||
| Infection-Induced Enteropathies | 381 | ||
| Immune-Mediated Enteropathies | 382 | ||
| Inflammatory Enteropathies | 383 | ||
| Specific Absorption Defects | 383 | ||
| Functional Diarrheal Disorders | 383 | ||
| Congenital Diarrheal Disorders | 384 | ||
| Structural Abnormalities | 385 | ||
| Tufting Enteropathy/ Epithelial Dysplasia | 385 | ||
| Microvillus Inclusion Disease | 385 | ||
| Disorders of Lipid Transport: Chylomicron Retention Disease, Hypobetalipoproteinemia, and Abetalipoproteinemia | 385 | ||
| Primary Lymphangiectasia | 385 | ||
| Tricho-Hepatic-Enteric Syndrome | 386 | ||
| Autoimmune, Infectious, and Allergic | 386 | ||
| Congenital Disorders of Glycosylation | 386 | ||
| Neonatal Diarrhea with Normal Crypt-Villus Structure | 386 | ||
| Ion Transport | 387 | ||
| Carbohydrate Malabsorption | 387 | ||
| Pancreatic Insufficiency | 387 | ||
| Enteric Endocrine Dysgenesis | 387 | ||
| Anatomic Abnormalities | 388 | ||
| Selected References | 388 | ||
| References | 388.e2 | ||
| Questions | 388.e1 | ||
| Answers | 388.e1 | ||
| 33 Protein-Losing Enteropathy | 389 | ||
| Definition | 389 | ||
| Pathophysiology | 389 | ||
| Some Causes of PLE | 389 | ||
| Intestinal lymphangiectasia (OMIM 152800) | 389 | ||
| Ménétrier’s Disease | 392 | ||
| Ulcerative Lesions of the Gastrointestinal Tract | 393 | ||
| Miscellaneous Conditions | 393 | ||
| Diagnosis | 393 | ||
| Therapy | 394 | ||
| Selected References | 394 | ||
| References | 394.e2 | ||
| Questions | 394.e1 | ||
| Answers | 394.e1 | ||
| 34 Celiac Disease | 395 | ||
| Definition | 395 | ||
| Historical Background | 395 | ||
| Cereal Proteins and Other Environmental Factors | 395 | ||
| Cereal Proteins | 395 | ||
| Other Environmental Factors | 395 | ||
| Genetics | 396 | ||
| Family Studies | 396 | ||
| Genetic Markers | 396 | ||
| Epidemiology | 396 | ||
| Pathogenesis | 397 | ||
| Gliadin Resistance to Enzymes and Passage Through the Epithelium | 397 | ||
| Activation of the Innate Immune System | 398 | ||
| CD4+ T-Cell Activation in the Lamina Propria: the Adaptive Immune Response | 398 | ||
| Cytokine Production | 398 | ||
| Intraepithelial Lymphocytes | 398 | ||
| Autoimmune Phenomena in Celiac Disease | 399 | ||
| Clinical Presentation | 399 | ||
| Associated Diseases | 400 | ||
| Laboratory and Pathology Findings | 400 | ||
| Serologic Tests | 400 | ||
| HLA | 401 | ||
| Biopsy | 401 | ||
| Diagnosis | 402 | ||
| Follow-Up and Challenge Procedures | 402 | ||
| Therapy | 403 | ||
| Gluten-Free Diet | 403 | ||
| Therapeutic Strategies for the Future | 404 | ||
| Selected References | 404 | ||
| References | 404.e3 | ||
| Questions | 404.e1 | ||
| Answers | 404.e1 | ||
| 35 Short Bowel Syndrome | 405 | ||
| Definition | 405 | ||
| Etiology | 405 | ||
| Intestinal Function and the Impact of Intestinal Loss | 405 | ||
| Proximal Intestinal Loss | 405 | ||
| Distal Intestinal Loss | 406 | ||
| Colon Loss | 406 | ||
| Factors That Determine Prognosis of SBS | 406 | ||
| Small Intestinal Length and Absorptive Function | 406 | ||
| The Impact of Colon Anatomy | 407 | ||
| Function of Remnant Gut | 407 | ||
| Intestinal Adaptation | 408 | ||
| Clinical Management of SBS | 409 | ||
| The Early Postoperative Phase | 409 | ||
| Initiation of Feeding | 409 | ||
| Impact of Intestinal Anatomy | 410 | ||
| Advancement of EN and Reduction in PN | 410 | ||
| Home PN in SBS | 412 | ||
| Additional Medical Interventions | 412 | ||
| Complications of SBS and PN | 412 | ||
| Hepatobiliary Disease | 412 | ||
| Small Intestinal Bacterial Overgrowth | 413 | ||
| Enterocolitis | 413 | ||
| Anatomic and Functional Bowel Obstruction | 414 | ||
| Pancreatitis | 415 | ||
| Renal Disease | 415 | ||
| Impaired Growth | 416 | ||
| Complications Associated with Central Venous Catheters | 416 | ||
| Survival and Its Costs in Pediatric SBS | 416 | ||
| Referral for Intestinal Transplantation | 416 | ||
| Summary | 417 | ||
| Selected References | 417 | ||
| References | 417.e2 | ||
| Questions | 417.e1 | ||
| Answers | 417.e1 | ||
| 36 Intestinal and Multivisceral Transplantation: | 418 | ||
| Pathophysiology of Gut Failure | 418 | ||
| Complications of Short Gut Syndrome | 418 | ||
| Principles of Gut Rehabilitation | 419 | ||
| Medical Management | 419 | ||
| Surgical Interventions | 420 | ||
| Autologous Reconstruction and Bowel Lengthening | 420 | ||
| Intestinal and Multivisceral Transplantation | 421 | ||
| Current Clinical Practice | 421 | ||
| Nomenclature | 421 | ||
| Indications | 421 | ||
| New Trends | 422 | ||
| Indications for Transplantation | 422 | ||
| Early Transplantation | 423 | ||
| Retransplantation | 423 | ||
| Contraindications | 423 | ||
| Listing | 424 | ||
| Global Trends | 424 | ||
| Technical Advances | 424 | ||
| Donor Procurement | 424 | ||
| Recipient Operation | 424 | ||
| Advances in Immunosuppression | 425 | ||
| Postoperative management | 425 | ||
| Graft-versus-Host Disease | 426 | ||
| Discharge Planning | 426 | ||
| Long-Term Outcome | 426 | ||
| Quality of Life | 427 | ||
| Summary | 428 | ||
| Selected References | 428 | ||
| References | 428.e3 | ||
| Questions | 428.e1 | ||
| Answers | 428.e1 | ||
| 37 Allergic and Eosinophilic Gastrointestinal Disease | 429 | ||
| Overview | 429 | ||
| Food Allergy or Hypersensitivity | 429 | ||
| IgE-Mediated Allergy and Anaphylaxis | 429 | ||
| Food Protein–Induced Enterocolitis Syndrome | 430 | ||
| Eosinophilic Gastroenteropathies | 430 | ||
| Eosinophilic Esophagitis | 430 | ||
| Etiology | 430 | ||
| Clinical Manifestations | 431 | ||
| Evaluation and Diagnosis | 431 | ||
| Management | 434 | ||
| Eosinophilic Gastroenteritis | 435 | ||
| Etiology | 435 | ||
| Clinical Manifestations | 435 | ||
| Evaluation and Diagnosis | 435 | ||
| Management | 436 | ||
| Eosinophilic Proctocolitis | 436 | ||
| Etiology | 437 | ||
| Clinical Manifestations | 437 | ||
| Evaluation and Diagnosis | 438 | ||
| Management | 438 | ||
| Other Manifestations of Gastrointestinal Allergy | 438 | ||
| Gastroesophageal Reflux | 438 | ||
| Infantile Colic | 439 | ||
| Diarrhea | 439 | ||
| Constipation | 439 | ||
| Approach to the Potentially Allergic Infant With Nonspecific GI Symptoms | 439 | ||
| Conclusion | 440 | ||
| Selected References | 440 | ||
| References | 440.e3 | ||
| Questions | 440.e1 | ||
| Answers | 440.e1 | ||
| 38 Infectious Diarrhea | 441 | ||
| Viral Gastroenteritis | 441 | ||
| Rotavirus | 441 | ||
| Virology | 441 | ||
| Epidemiology | 441 | ||
| Clinical Manifestations | 442 | ||
| Pathophysiology | 442 | ||
| Diagnosis | 442 | ||
| Treatment | 442 | ||
| Prevention | 443 | ||
| Small Round Structured Viruses | 443 | ||
| Caliciviruses | 443 | ||
| Norovirus | 443 | ||
| Epidemiology. | 443 | ||
| Pathophysiology. | 444 | ||
| Clinical Manifestations. | 444 | ||
| Diagnosis and Treatment. | 444 | ||
| Enteric Adenovirus | 444 | ||
| Astrovirus | 445 | ||
| Other Viruses | 445 | ||
| Bacterial Gastroenteritis | 445 | ||
| Host-Defense Factors | 445 | ||
| Mechanisms of Bacterial Disease Production | 446 | ||
| Salmonella | 446 | ||
| Microbiology | 446 | ||
| Epidemiology | 446 | ||
| Pathogenesis | 447 | ||
| Clinical Manifestations | 447 | ||
| Diagnosis and Treatment | 447 | ||
| Typhoid Fever | 448 | ||
| Shigella | 448 | ||
| Microbiology | 448 | ||
| Epidemiology | 448 | ||
| Clinical Manifestations | 449 | ||
| Pathogenesis | 449 | ||
| Diagnosis and Treatment | 450 | ||
| Campylobacter | 450 | ||
| Epidemiology | 450 | ||
| Pathogenesis | 450 | ||
| Clinical Manifestations | 450 | ||
| Diagnosis and Treatment | 451 | ||
| Yersinia | 451 | ||
| Microbiology | 451 | ||
| Epidemiology | 451 | ||
| Pathogenesis | 451 | ||
| Clinical Manifestations | 452 | ||
| Diagnosis and Treatment | 452 | ||
| Cholera | 452 | ||
| Microbiology | 452 | ||
| Epidemiology | 452 | ||
| Pathogenesis | 453 | ||
| Clinical Manifestations | 454 | ||
| Diagnosis and Treatment | 454 | ||
| Other Vibrios | 454 | ||
| Escherichia coli | 454 | ||
| Enteropathogenic Escherichia coli | 454 | ||
| Enterotoxigenic Escherichia coli | 455 | ||
| Enteroinvasive Escherichia coli | 456 | ||
| Shiga Toxin–(Stx) Producing Escherichia coli | 456 | ||
| Enteroaggregative and Diffusely Adherent Escherichia coli | 458 | ||
| Clostridium difficile | 458 | ||
| Epidemiology | 458 | ||
| Pathogenesis | 458 | ||
| Clinical Manifestations | 459 | ||
| Diagnosis and Treatment | 459 | ||
| Aeromonas | 460 | ||
| Plesiomonas | 460 | ||
| Mycobacterium avium complex | 461 | ||
| Potential Diarrheagenic Organisms | 461 | ||
| Enterotoxigenic Bacteroides fragilis | 461 | ||
| Brachyspira aalborgi | 461 | ||
| Hafnia alvei | 461 | ||
| Listeria monocytogenes | 461 | ||
| Probiotics and Diarrhea | 461 | ||
| Background | 461 | ||
| Acute Infectious Diarrhea | 462 | ||
| Probiotics and C. difficile Infection | 462 | ||
| Probiotics and Other Disease Processes | 462 | ||
| Conclusion | 462 | ||
| Select References | 462 | ||
| References | 462.e2 | ||
| Questions | 462.e1 | ||
| Answers | 462.e1 | ||
| 39 Enteric Parasites | 463 | ||
| Epidemiology | 463 | ||
| Host Factors | 463 | ||
| Clinical Presentations | 463 | ||
| Diagnosis | 464 | ||
| Stool Examination | 464 | ||
| Immunoassay | 464 | ||
| Macroscopic Examination | 464 | ||
| Serology | 464 | ||
| Eosinophilia | 464 | ||
| Intestinal Fluid and Biopsy | 464 | ||
| Benefits of Parasites | 465 | ||
| Pathogenic Organisms | 465 | ||
| Protozoa | 465 | ||
| Giardia Intestinalis | 465 | ||
| Entamoeba Histolytica | 466 | ||
| Dientamoeba Fragilis | 470 | ||
| Blastocystis Species | 471 | ||
| Balantidium Coli | 471 | ||
| Cryptosporidium | 471 | ||
| Other Coccidia | 472 | ||
| Nematodes (Roundworms) | 473 | ||
| Ascaris Lumbricoides | 473 | ||
| Trichuris Trichiura | 473 | ||
| Necator Americanus and Ancylostoma Duodenale (Hookworms) | 474 | ||
| Strongyloides Stercoralis | 474 | ||
| Capillaria Philippinensis | 475 | ||
| Angiostrongylus Costaricensis | 475 | ||
| Enterobius Vermicularis (Pinworm) | 475 | ||
| Cestodes (Tapeworms) | 475 | ||
| Taenia Solium | 476 | ||
| Taenia Saginata | 476 | ||
| Hymenolepis Nana | 476 | ||
| Flukes | 476 | ||
| Schistosomiasis | 476 | ||
| Liver Flukes | 476 | ||
| Zoonotic Infections | 476 | ||
| Cutaneous Larva Migrans | 476 | ||
| Anisakiasis | 477 | ||
| Ancylostoma Caninum | 477 | ||
| Visceral Larva Migrans | 477 | ||
| Selected References | 477 | ||
| References | 477.e2 | ||
| Questions | 477.e1 | ||
| Answers | 477.e1 | ||
| 40 Gastrointestinal Manifestations of Immunodeficiency | 478 | ||
| Innate Versus Adaptive Immunity | 478 | ||
| Components of the Adaptive Immune Response | 481 | ||
| Humoral Immunodeficiencies | 484 | ||
| Selective IgA Deficiency | 484 | ||
| X-linked agammaglobulinemia | 485 | ||
| Hyper-IgM Syndrome | 485 | ||
| Transient Hypogammaglobulinemia of Infancy | 486 | ||
| Cellular Immunodeficiencies | 487 | ||
| DiGeorge Syndrome | 487 | ||
| Chronic Mucocutaneous Candidiasis and APECED Syndrome | 487 | ||
| Combined Cellular–Humoral Immunodeficiencies | 487 | ||
| Common Variable Immunodeficiency | 487 | ||
| Severe Combined Immunodeficiency (SCID) | 488 | ||
| NF-κB Essential Modifier Mutations | 489 | ||
| Disorders of Phagocyte Function | 489 | ||
| Chronic Granulomatous Disease | 489 | ||
| Leukocyte-Adhesion Deficiency | 491 | ||
| Other Primary Immunodeficiencies | 491 | ||
| Wiskott-Aldrich Syndrome | 491 | ||
| X-linked Lymphoproliferative Disease | 491 | ||
| Immune Dysfunction, Polyendocrinopathy, Enteropathy, X-linked | 492 | ||
| IL-10 Receptor Mutations | 492 | ||
| Newly Identified Mutations Leading to Immunodeficiency and IBD | 492 | ||
| Secondary Immunodeficiencies | 492 | ||
| Human Immunodeficiency Virus Infection | 492 | ||
| Malnutrition and Micronutrient Deficiencies | 494 | ||
| Selected References | 495 | ||
| References | 495.e2 | ||
| Questions | 495.e1 | ||
| Answers | 495.e1 | ||
| 41 Pediatric Polyposis Syndromes | 496 | ||
| Polyp Types | 496 | ||
| Clinical Presentation of Gastrointestinal Polyps | 496 | ||
| The Single Hamartomatous Polyp (The Juvenile Polyp) | 496 | ||
| Hamartomatous Polyposis Syndromes | 496 | ||
| Juvenile Polyposis Syndrome | 497 | ||
| Cancer Risk in Juvenile Polyposis Syndrome | 497 | ||
| Genetics of Juvenile Polyposis | 498 | ||
| Screening and Follow-up | 498 | ||
| PTEN Hamartoma Tumor Syndrome | 499 | ||
| Peutz-Jeghers Syndrome | 499 | ||
| Clinical Features and Diagnosis | 499 | ||
| Genetics of Peutz-Jeghers Syndrome | 499 | ||
| Screening, Management, and Complications | 500 | ||
| Malignancy Risk | 501 | ||
| Adenomatous Polyposis Syndromes | 501 | ||
| Familial Adenomatous Polyposis | 501 | ||
| Clinical Features | 502 | ||
| Genetics of FAP | 502 | ||
| Diagnosis—Interpretation of the Genetic Test and Clinical Screening in FAP | 502 | ||
| Management of FAP | 504 | ||
| Desmoid Disease | 505 | ||
| Future Chemoprevention | 505 | ||
| Prognosis | 505 | ||
| MYH-Associated Polyposis (MAP) | 505 | ||
| Lynch Syndrome | 506 | ||
| Other Polyposis Syndromes | 506 | ||
| The Role of a Polyposis Registry | 506 | ||
| Summary | 506 | ||
| Acknowledgements | 507 | ||
| Select References | 507 | ||
| References | 507.e3 | ||
| Questions | 507.e1 | ||
| Answers | 507.e1 | ||
| 42 Crohn’s Disease | 508 | ||
| Epidemiology of CD | 508 | ||
| Classification of CD | 508 | ||
| Diagnosis of CD | 509 | ||
| Diagnostic Work-Up of Children with Suspected CD | 511 | ||
| Very Early Onset CD | 513 | ||
| Natural History of CD | 514 | ||
| Measures of Disease Activity and Outcomes in CD | 514 | ||
| Etiology | 516 | ||
| Serology | 516 | ||
| Genetics | 516 | ||
| Microbiome | 517 | ||
| Immunology | 518 | ||
| Therapy | 520 | ||
| Pharmacologic Therapy | 520 | ||
| Nutritional Therapy | 521 | ||
| Active Disease | 521 | ||
| Maintenance Therapy | 521 | ||
| Other Benefits | 521 | ||
| Corticosteroids | 521 | ||
| Induction | 521 | ||
| Maintenance and Postoperative Therapy | 522 | ||
| 6 Pediatric Surgical Disorders | 606 | ||
| 51 Intussusception in Infants and Children | 607 | ||
| Incidence | 607 | ||
| Pathophysiology | 607 | ||
| Clinical Presentation | 607 | ||
| Therapeutic Options | 608 | ||
| Nonoperative Management | 608 | ||
| Surgical Management | 611 | ||
| Open Approach | 611 | ||
| Laparoscopic Approach | 612 | ||
| Special Considerations | 612 | ||
| Recurrence | 612 | ||
| Postoperative Intussusception | 612 | ||
| Henoch-Schönlein Purpura | 612 | ||
| Cystic Fibrosis | 613 | ||
| Rotavirus Vaccine | 613 | ||
| Acknowledgments | 613 | ||
| Select References | 613 | ||
| References | 613.e2 | ||
| Questions | 613.e1 | ||
| Answers | 613.e1 | ||
| 52 Inguinal Hernias and Hydroceles | 614 | ||
| Embryology | 614 | ||
| Clinical Presentation | 614 | ||
| Management | 615 | ||
| Elective | 615 | ||
| Incarcerated | 615 | ||
| Contralateral Side | 615 | ||
| Premature Infants | 616 | ||
| Laparoscopic | 616 | ||
| Complications | 616 | ||
| Select References | 617 | ||
| References | 617.e2 | ||
| Questions | 617.e1 | ||
| Answers | 617.e1 | ||
| 53 Meckel’s Diverticula and Other Omphalomesenteric Duct Remnants | 618 | ||
| Embryology and Anatomy | 618 | ||
| Clinical Presentation | 618 | ||
| Diagnosis | 619 | ||
| Treatment | 621 | ||
| Summary | 621 | ||
| Select References | 621 | ||
| References | 621.e3 | ||
| Questions | 621.e1 | ||
| Answers | 621.e1 | ||
| 54 Hirschsprung’s Disease | 622 | ||
| Epidemiology and Genetics | 622 | ||
| Pathophysiology | 622 | ||
| Clinical Presentation | 623 | ||
| Differential Diagnosis | 623 | ||
| Diagnosis | 624 | ||
| Contrast Enema | 624 | ||
| Anorectal Manometry | 625 | ||
| Rectal Biopsy | 625 | ||
| Treatment | 626 | ||
| The Swenson Procedure | 626 | ||
| The Duhamel-Martin Procedure | 627 | ||
| The Soave Procedure | 627 | ||
| The Role of Minimally Invasive Techniques in the Treatment of HD | 627 | ||
| Treatment of HD and the Length of the Aganglionic Segment | 627 | ||
| Hirschsprung’s Disease–Associated Enterocolitis | 628 | ||
| Results of Surgical Therapy and Long Term Outcomes | 628 | ||
| Summary | 629 | ||
| Select References | 630 | ||
| References | 630.e3 | ||
| Questions | 630.e1 | ||
| Answer | 630.e1 | ||
| 55 Imperforate Anus | 631 | ||
| Classification and Description of Defects | 631 | ||
| Anorectal Malformation in Male Patients | 631 | ||
| Perineal Fistula | 631 | ||
| Rectourethral Fistula | 631 | ||
| Rectobladderneck Fistula | 631 | ||
| Imperforate Anus Without Fistula | 631 | ||
| Rectal Atresia/Stenosis | 633 | ||
| Anorectal Malformation in Female Patients | 633 | ||
| Perineal Fistula | 633 | ||
| Vestibular Fistula | 633 | ||
| Cloaca | 633 | ||
| Associated Defects | 634 | ||
| Early Management and Diagnosis | 635 | ||
| Main Repair | 636 | ||
| Anoplasty for the Treatment of a Perineal Fistula | 636 | ||
| Posterior-Sagittal Anorectoplasty | 637 | ||
| Functional Sequelae | 637 | ||
| Elements Required for Bowel Control | 637 | ||
| Anal Canal and Rectal Sensation | 637 | ||
| Sphincters | 637 | ||
| Rectosigmoid Motility | 637 | ||
| Fecal Incontinence | 638 | ||
| Soiling | 638 | ||
| Constipation | 638 | ||
| Urinary Incontinence | 639 | ||
| Selected References | 639 | ||
| References | 639.e2 | ||
| Questions | 639.e1 | ||
| Answers | 639.e1 | ||
| 56 Abnormal Rotation and Fixation of the Intestine | 640 | ||
| Embryology | 640 | ||
| Anatomy | 640 | ||
| Nonrotation | 641 | ||
| Incomplete (Mixed) Rotation | 642 | ||
| Reversed Rotation | 642 | ||
| Mesocolic Hernia | 643 | ||
| Clinical Presentation | 643 | ||
| Diagnosis | 643 | ||
| Treatment | 644 | ||
| Ladd’s Procedure | 644 | ||
| The Asymptomatic Patient | 645 | ||
| Obligate Malrotation | 646 | ||
| Heterotaxy Syndrome | 646 | ||
| Mesocolic Hernia | 646 | ||
| Complications, Results, Outcome | 646 | ||
| Summary | 647 | ||
| Selected References | 647 | ||
| References | 647.e2 | ||
| Questions | 647.e1 | ||
| Answers | 647.e1 | ||
| 57 Atresia and Stenosis of the Bowel | 648 | ||
| Duodenum | 648 | ||
| Embryology | 648 | ||
| Incidence | 648 | ||
| Clinical Presentation | 648 | ||
| Classification (Figure 57-2) | 649 | ||
| Treatment | 649 | ||
| Jejunoileum | 650 | ||
| Embryology | 650 | ||
| Incidence | 650 | ||
| Clinical Presentation | 650 | ||
| Classification (Figure 57-4) | 650 | ||
| Treatment | 651 | ||
| Colon | 652 | ||
| Embryology | 652 | ||
| Incidence | 652 | ||
| Clinical Presentation | 652 | ||
| Treatment | 652 | ||
| Summary | 652 | ||
| Select References | 653 | ||
| References | 653.e2 | ||
| Questions | 653.e1 | ||
| Answer | 653.e1 | ||
| 58 Newborn Abdominal Wall Defects | 654 | ||
| Historical Background | 654 | ||
| Embryogenesis | 654 | ||
| Normal Embryology of the Abdominal Wall | 654 | ||
| Defects in Embryogenesis | 654 | ||
| Etiology | 655 | ||
| The Evolution of Epidemiology | 655 | ||
| A Spectrum of Defects | 656 | ||
| Omphalocele | 656 | ||
| Gastroschisis | 656 | ||
| Associated Anomalies | 656 | ||
| Prenatal Management | 657 | ||
| Imaging | 657 | ||
| Amniotic Fluid and Maternal Serum Studies | 657 | ||
| Obstetric Delivery | 658 | ||
| Immediate Postnatal Management | 658 | ||
| Surgical Management | 659 | ||
| General Principles | 659 | ||
| Surgical Management of Gastroschisis | 659 | ||
| Postoperative Management after Gastroschisis Repair | 662 | ||
| Surgical Management of Omphalocele | 662 | ||
| Postoperative Management after Omphalocele Repair | 663 | ||
| Postoperative Complications | 664 | ||
| Outcomes | 664 | ||
| Cost of Treatment | 665 | ||
| Selected References | 665 | ||
| References | 665.e2 | ||
| Questions | 665.e1 | ||
| Answers | 665.e1 | ||
| 59 Stomas of the Small and Large Intestine | 666 | ||
| Historical Perspective | 666 | ||
| Considerations for the Child with an Intestinal Stoma | 666 | ||
| Types of Intestinal Stomas and Technical Aspects in the Pediatric Population | 667 | ||
| Basic Technical Steps | 667 | ||
| Jejunostomies | 668 | ||
| Other Intestinal Stomas and Special Considerations | 669 | ||
| Appendicostomies | 671 | ||
| Colostomies and Surgery in Hirschsprung’s Disease | 672 | ||
| Colostomies for Anorectal Malformations | 673 | ||
| Key References | 674 | ||
| References | 674.e3 | ||
| Questions | 674.e1 | ||
| Answers | 674.e1 | ||
| 7 Gastrointestinal Procedures | 675 | ||
| 60 Esophagogastroduodenoscopy and Related Techniques | 676 | ||
| Personnel | 676 | ||
| Facilities | 676 | ||
| Equipment | 677 | ||
| Indications | 678 | ||
| Contraindications | 678 | ||
| Antibiotic Prophylaxis | 679 | ||
| Preparation | 680 | ||
| Esophagogastroduodenoscopy | 680 | ||
| Sedation and Monitoring | 680 | ||
| Anatomy | 681 | ||
| Esophagogastroduodenoscopy | 681 | ||
| Technique | 682 | ||
| Upper Endoscopy | 682 | ||
| Biopsy Technique | 684 | ||
| Esophagus | 684 | ||
| Stomach | 685 | ||
| Small Intestine | 686 | ||
| Therapeutic Endoscopy | 687 | ||
| Injection | 687 | ||
| Thermocoagulation | 689 | ||
| Heater Probe | 689 | ||
| Electrocoagulation | 689 | ||
| Argon Plasma Coagulation | 691 | ||
| Endoscopic Hemostatic Devices Including Bands and Clips | 691 | ||
| Band Ligation | 691 | ||
| Hemostatic Clips | 691 | ||
| Endoloops | 692 | ||
| Hemostatic Powders | 692 | ||
| Laser Photocoagulation | 693 | ||
| Stricture Dilation | 693 | ||
| Small Bowel Enteroscopy | 694 | ||
| Wireless Capsule Endoscopy | 695 | ||
| Percutaneous Endoscopic Gastrostomy | 695 | ||
| Indications | 695 | ||
| Technique | 696 | ||
| Percutaneous Endoscopic Jejunostomy (PEJ) | 698 | ||
| Polypectomy | 698 | ||
| Special Endoscopic Techniques | 699 | ||
| Pyloric Balloon Dilation | 699 | ||
| Endoscopic Intrapyloric Injection of Botulinum Toxin | 699 | ||
| Endoscopic Therapy for Achalasia | 699 | ||
| Placement of Motility Catheters | 699 | ||
| Endoscopic Therapy for Gastroesophageal Reflux | 700 | ||
| Esophageal Stent Placement | 700 | ||
| Endoscopic Closure of Fistulas | 700 | ||
| Endoscopic Mucosal Resection | 701 | ||
| Endoscopic Ultrasonography | 701 | ||
| Chromoendoscopy and Narrow Band Imaging | 701 | ||
| Confocal Laser Endomicroscopy (CLE) | 701 | ||
| New Techniques | 702 | ||
| Complications | 702 | ||
| Selected References | 704 | ||
| References | 704.e3 | ||
| Questions | 704.e1 | ||
| Answers | 704.e1 | ||
| 61 Colonoscopy, Polypectomy, and Related Techniques | 705 | ||
| Personnel, Sedation, and Antibiotic Prophylaxis | 705 | ||
| Equipment | 705 | ||
| Indications | 705 | ||
| Contraindications | 707 | ||
| Preparation | 707 | ||
| Anatomy | 710 | ||
| Technique | 710 | ||
| Colonoscopy | 711 | ||
| Flexible Proctosigmoidoscopy | 715 | ||
| Anoscopy and Rigid Proctosigmoidoscopy | 715 | ||
| Biopsy Technique | 715 | ||
| Therapeutic Colonoscopy | 716 | ||
| Injection | 717 | ||
| Thermocoagulation: Heater Probe, Multipolar Probes, and Hot Biopsy Forceps | 717 | ||
| Argon Plasma Coagulation (APC) | 719 | ||
| Endoscopic Hemostatic Devices Including Bands and Clips | 719 | ||
| Band Ligation | 719 | ||
| Hemostatic Clips | 719 | ||
| Endoloops | 720 | ||
| Laser Photocoagulation | 720 | ||
| Stricture Dilation | 721 | ||
| Percutaneous Cecostomy | 721 | ||
| Polypectomy | 721 | ||
| Special Endoscopic Techniques and Equipment | 724 | ||
| Colonoscope 3D Imaging Probe | 724 | ||
| Placement of Motility Catheters | 724 | ||
| Endoscopic Mucosal Resection | 724 | ||
| Third-Eye Retroscope | 725 | ||
| Water-Immersion Colonoscopy | 725 | ||
| Advanced Imaging Techniques | 725 | ||
| Complications | 725 | ||
| Selected References | 726 | ||
| References | 726.e3 | ||
| Questions | 726.e1 | ||
| Answers | 726.e1 | ||
| 62 Endoscopic Retrograde Cholangiopancreatography | 727 | ||
| Pancreaticobiliary Imaging Methods | 727 | ||
| Indications for ERCP | 728 | ||
| Contraindications to ERCP | 728 | ||
| Preparation for ERCP | 729 | ||
| Technique of ERCP | 729 | ||
| Biliary Disorders | 730 | ||
| Diagnosis of Biliary Atresia and Neonatal Hepatitis | 730 | ||
| Choledocholithiasis | 731 | ||
| Choledochal Cyst | 732 | ||
| Biliary Strictures | 733 | ||
| Primary Sclerosing Cholangitis (PSC) | 733 | ||
| Post-Liver Transplantation Strictures | 734 | ||
| Biliary Leaks | 734 | ||
| Unusual Biliary Infections | 734 | ||
| Biliary Dyskinesia | 734 | ||
| Pancreatic Disorders | 736 | ||
| Congenital Malformations | 736 | ||
| Acquired Disorders | 736 | ||
| Abdominal Trauma | 737 | ||
| Pancreatic Pseudocysts | 737 | ||
| Acute Pancreatitis | 737 | ||
| Chronic Pancreatitis | 738 | ||
| Complications of ERCP | 738 | ||
| Summary | 738 | ||
| Selected References | 738 | ||
| References | 739.e3 | ||
| Questions | 739.e1 | ||
| Answers | 739.e1 | ||
| 63 Capsule Endoscopy and Small Bowel Enteroscopy | 740 | ||
| Equipment | 740 | ||
| Performance of CE Study | 740 | ||
| Patient Preparation | 740 | ||
| Ingestion Versus Endoscopic Placement | 740 | ||
| Image Review | 741 | ||
| Indications | 741 | ||
| Crohn’s Disease | 741 | ||
| Obscure Gastrointestinal Bleeding | 742 | ||
| Celiac Disease | 742 | ||
| Polyposis | 742 | ||
| Graft-Versus-Host Disease | 743 | ||
| Unexplained Growth Failure | 743 | ||
| Recurrent Abdominal Pain | 743 | ||
| Protein-Losing Enteropathy/Intestinal Lymphangiectasia | 743 | ||
| Limitations | 743 | ||
| Contraindications and Complications | 743 | ||
| Enteroscopy | 744 | ||
| Introduction | 744 | ||
| Push Enteroscopy | 744 | ||
| Balloon Enteroscopy (Push and Pull Enteroscopy) | 744 | ||
| Double-Balloon Enteroscopy | 744 | ||
| Single-Balloon Enteroscopy | 745 | ||
| Spiral Enteroscopy | 746 | ||
| Complications | 746 | ||
| Intraoperative Enteroscopy | 746 | ||
| Conclusion | 747 | ||
| Selected References | 747 | ||
| References | 747.e3 | ||
| Questions | 747.e1 | ||
| Answer | 747.e1 | ||
| 64 Gastrointestinal Motility Procedures | 748 | ||
| Gastrointestinal Motility Studies | 748 | ||
| Esophageal Manometry | 749 | ||
| Normal Anatomy and Physiology | 749 | ||
| Before the Procedure | 750 | ||
| Catheter Placement | 750 | ||
| Procedure | 750 | ||
| Interpretation | 750 | ||
| Normal Motility | 750 | ||
| Indications and Clinical Utility | 750 | ||
| Primary Esophageal Motility Disorders | 751 | ||
| Gastroesophageal Reflux Disease | 752 | ||
| Connective Tissue Disorders | 752 | ||
| Chest Pain | 752 | ||
| Newer Techniques to Perform Esophageal Manometry | 752 | ||
| Multichannel Intraluminal Impedance Combined With Manometry | 752 | ||
| Antroduodenal Manometry | 753 | ||
| Normal Physiology | 753 | ||
| Fasting Motility | 753 | ||
| Postprandial Motility | 754 | ||
| Preparation for the Procedure | 754 | ||
| Equipment | 754 | ||
| Catheter Placement | 754 | ||
| Study Procedure | 755 | ||
| Interpretation | 756 | ||
| Manometric Diagnosis by Antroduodenal Manometry | 756 | ||
| Normal Motility | 756 | ||
| Chronic Intestinal Pseudo-Obstruction | 756 | ||
| Postprandial Antral Hypomotility | 756 | ||
| Mechanical Obstruction | 756 | ||
| Rumination | 757 | ||
| Indications and Clinical Utility | 757 | ||
| Future | 757 | ||
| Colonic Manometry | 758 | ||
| Normal Physiology | 758 | ||
| Before the Procedure | 758 | ||
| Equipment | 758 | ||
| Catheter Placement | 759 | ||
| Motility Study | 759 | ||
| Interpretation | 759 | ||
| Normal Patterns | 759 | ||
| Gastrocolonic Response | 760 | ||
| HAPCs | 760 | ||
| Indications and Clinical Significance | 760 | ||
| Constipation | 760 | ||
| Chronic Intestinal Pseudo-Obstruction | 761 | ||
| Hirschsprung’s Disease | 761 | ||
| Imperforate Anus | 761 | ||
| Outcome | 761 | ||
| Future | 761 | ||
| Anorectal Manometry | 761 | ||
| Normal Anorectal Anatomy and Physiology | 762 | ||
| Before the Procedure | 762 | ||
| Equipment | 762 | ||
| Procedure | 762 | ||
| Interpretation | 763 | ||
| Normal Values | 764 | ||
| Indications and Clinical Significance | 764 | ||
| Constipation | 764 | ||
| Postoperative Hirschsprung’s Disease | 765 | ||
| Pelvic Floor Dyssynergia (Anismus) | 765 | ||
| Fecal Incontinence | 765 | ||
| Imperforate Anus | 765 | ||
| Spinal Neuropathy | 765 | ||
| Summary | 765 | ||
| Selected References | 765 | ||
| References | 765.e2 | ||
| Questions | 765.e1 | ||
| Answers | 765.e1 | ||
| 65 Gastrointestinal Pathology | 766 | ||
| Esophageal Biopsy | 766 | ||
| Gastroesophageal Reflux | 766 | ||
| Histologic Changes—Squamous Mucosa | 766 | ||
| Histologic Changes—Glandular Mucosa | 767 | ||
| Differential Diagnosis | 767 | ||
| Infectious Esophagitis | 767 | ||
| Eosinophilic Esophagitis | 767 | ||
| Pill Esophagitis | 768 | ||
| Barrett’s Esophagus | 768 | ||
| Stomach Biopsy | 769 | ||
| Small Intestinal Biopsy | 770 | ||
| Specimen Procurement and Processing | 770 | ||
| Normal Small Intestinal Histology | 770 | ||
| Patterns of Abnormal Small Bowel Architecture | 770 | ||
| Entities Associated With a Diffuse Severe Villous Abnormality and Crypt Hyperplasia | 771 | ||
| Celiac Sprue. | 771 | ||
| Other Protein Allergies. | 772 | ||
| Entities Associated With a Variable Villous Abnormality and Crypt Hypoplasia | 772 | ||
| Marasmus and Kwashiorkor. | 772 | ||
| Megaloblastic Anemia–Radiation and Chemotherapy Effect. | 772 | ||
| Microvillus Inclusion Disease. | 772 | ||
| Entities Associated With a Nonspecific Variable Villous Abnormality | 772 | ||
| Entities Associated With Variable Villous Abnormalities Illustrating Specific Diagnostic Changes | 773 | ||
| Collagenous Sprue. | 773 | ||
| Immunodeficiency Syndromes (Excluding Acquired Immune Deficiency Syndrome). | 773 | ||
| Whipple’s Disease. | 774 | ||
| Eosinophilic Gastroenteritis. | 774 | ||
| Parasitic Infestations. | 774 | ||
| Intestinal Lymphangiectasia. | 774 | ||
| Abetalipoproteinemia. | 775 | ||
| Acrodermatitis Enteropathica. | 775 | ||
| Tufting Enteropathy. | 775 | ||
| Interpretation of Colonic Mucosal Biopsy Specimens | 775 | ||
| Active Colitis—Differential Diagnosis | 775 | ||
| Chronic Colitis—Differential Diagnosis | 776 | ||
| Diffuse Chronic Active Colitis | 776 | ||
| Focal Active Colitis | 776 | ||
| Acute Ischemic-Type Change | 777 | ||
| Prolapse/Trauma-Type Change | 777 | ||
| Apoptotic Colopathies | 777 | ||
| Acute Graft-versus-Host-Disease | 777 | ||
| Cord Colitis Syndrome | 778 | ||
| Specific Infectious Colitides | 778 | ||
| Common Bacterial Agents | 778 | ||
| Hemorrhagic Colitis Syndrome | 778 | ||
| Antibiotic-Associated Colitis and Pseudomembranous Colitis | 778 | ||
| Viral Agents | 779 | ||
| Specific Forms of Colitis | 779 | ||
| Eosinophilic Colitis/Proctitis | 779 | ||
| Evaluation of Resection Specimens in Inflammatory Bowel Disease | 779 | ||
| Crohn’s Disease and Ulcerative Colitis | 779 | ||
| Colitis–Type Indeterminate | 781 | ||
| Lesions Associated with Surgical Procedures | 781 | ||
| Diversion Colitis/Defunctionalized Bowel | 781 | ||
| Ileal Reservoirs (Pouches) and Pouchitis | 782 | ||
| Disorders of Intestinal Motility | 783 | ||
| Intestinal Pseudo-obstruction | 783 | ||
| Visceral Myopathies | 783 | ||
| Visceral Neuropathies | 783 | ||
| Ceroidosis: The “Brown Bowel Syndrome” | 783 | ||
| Melanosis Coli | 783 | ||
| Hirschsprung’s Disease and Allied Conditions | 783 | ||
| Hirschsprung’s Disease | 783 | ||
| Long-Segment Hirschsprung’s Disease | 785 | ||
| Ultrashort-Segment Hirschsprung’s Disease | 785 | ||
| Hypoganglionosis | 785 | ||
| Intestinal Neuronal Dysplasia Type B (Submucosal Hyperganglionosis) | 785 | ||
| Other Related Conditions | 786 | ||
| Gastrointestinal Polyps and Polyposis Syndromes | 786 | ||
| Familial Adenomatous Polyposis | 786 | ||
| Genetics of Familial Adenomatous Polyposis and Related Syndromes | 787 | ||
| Juvenile Polyps and Juvenile Polyposis Syndromes | 787 | ||
| PTEN Hamartoma Tumor Syndrome (Ruvalcaba-Myhre-Smith Syndrome [Bannayan-Riley-Ruvalcaba Syndrome] and Cowden’s Syndrome) | 788 | ||
| Ruvalcaba-Myhre-Smith Syndrome | 788 | ||
| Cowden’s Syndrome | 788 | ||
| Intestinal Ganglioneuromatosis | 789 | ||
| Peutz-Jeghers Syndrome | 789 | ||
| Select References | 789 | ||
| References | 789.e2 | ||
| Questions | 789.e1 | ||
| Answers | 789.e1 | ||
| 8 The Liver and Bile Ducts | 790 | ||
| 66 Liver Pathology | 791 | ||
| Functional Anatomy and Histology of the Liver | 791 | ||
| Neonatal Cholestasis | 791 | ||
| Extrahepatic Biliary Obstruction Pattern | 791 | ||
| Biliary Atresia | 791 | ||
| α1-Antitrypsin Deficiency | 792 | ||
| Total Parenteral Nutrition–Induced Liver Injury | 793 | ||
| Giant Cell Hepatitis Pattern | 793 | ||
| Idiopathic Neonatal Hepatitis | 794 | ||
| Hypopituitarism | 794 | ||
| Normal/Low GGT Disorders | 794 | ||
| Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC-2) | 794 | ||
| Bile Acid Synthetic Defects | 794 | ||
| Bland Cholestasis Pattern | 794 | ||
| Progressive Familial Intrahepatic Cholestasis Type 1 (PFIC-1) | 794 | ||
| Sepsis | 795 | ||
| Ductopenia Pattern | 795 | ||
| Defining Ductopenia | 795 | ||
| Alagille Syndrome | 795 | ||
| Nonsyndromic Bile Duct Paucity | 796 | ||
| Chronic Liver Disease in Older Children | 796 | ||
| Chronic Hepatitis | 796 | ||
| Autoimmune Hepatitis | 796 | ||
| Chronic Viral Hepatitis | 797 | ||
| Wilson’s Disease | 797 | ||
| Primary Sclerosing Cholangitis | 797 | ||
| Fatty Liver Disease | 798 | ||
| Liver Transplant Pathology | 799 | ||
| Assessment of Donor Liver Pretransplantation | 799 | ||
| Assessment of Liver Posttransplantation | 799 | ||
| Ischemia/Reperfusion Injury | 799 | ||
| Acute Cellular Rejection | 799 | ||
| De Novo Autoimmune Hepatitis | 799 | ||
| Chronic Rejection | 800 | ||
| Liver Tumors in Children | 800 | ||
| Hepatoblastoma | 800 | ||
| Hepatocellular Carcinoma | 805 | ||
| Hemangiomas | 806 | ||
| Mesenchymal Hamartoma of the Liver | 808 | ||
| Undifferentiated Embryonal Sarcoma | 808 | ||
| Hepatobiliary Rhabdomyosarcoma | 809 | ||
| Focal Nodular Hyperplasia | 809 | ||
| Hepatocellular Adenoma | 809 | ||
| Select References | 809 | ||
| References | 810.e3 | ||
| Questions | 810.e1 | ||
| Answers | 810.e1 | ||
| 67 Developmental Anatomy and Physiology of the Liver and Bile Ducts | 811 | ||
| Early Development of the Liver in Humans and Other Vertebrates | 811 | ||
| Competence | 811 | ||
| Specification | 811 | ||
| Morphogenesis: Emergence and Growth of the Liver Bud | 812 | ||
| Growth and Size Regulation of the Liver Bud | 813 | ||
| Development of Structures that Support Hepatocytes: Bile Ducts, Portal Tract Mesenchyme, and Vasculature | 813 | ||
| Bile Ducts | 813 | ||
| Portal Tract Mesenchyme | 816 | ||
| Vasculature | 816 | ||
| The Functional Unit of the Liver: The Hepatic Lobule and Sinusoidal System | 817 | ||
| Functional Development of the Liver | 818 | ||
| The Liver as a Metabolic Organ | 819 | ||
| The Endocrine Liver | 819 | ||
| Carbohydrates | 819 | ||
| Protein | 819 | ||
| Lipids | 819 | ||
| Coagulation Factors | 819 | ||
| Hormones | 819 | ||
| The Exocrine Liver | 819 | ||
| Bile Acids | 819 | ||
| Cholesterol and Phospholipids | 819 | ||
| The Liver as a Filter | 820 | ||
| The Liver as a Hematopoietic Organ | 820 | ||
| The Liver as an Immune Organ | 820 | ||
| Stem Cells and Liver Regeneration | 820 | ||
| Regeneration | 820 | ||
| Experimental Biology | 821 | ||
| Conclusion | 821 | ||
| Acknowledgments | 821 | ||
| Selected References | 821 | ||
| References | 822.e2 | ||
| Questions | 822.e1 | ||
| Answers | 822.e1 | ||
| 68 Neonatal Hepatitis | 823 | ||
| Approach to the Infant with Cholestasis | 823 | ||
| Clinical Features | 823 | ||
| Laboratory and Radiographic Evaluation | 826 | ||
| Cholestasis Associated with Infection | 828 | ||
| Bacterial Infections | 829 | ||
| Congenital Syphilis | 829 | ||
| Perinatal Tuberculosis | 829 | ||
| Listeriosis | 830 | ||
| Viral Infections | 830 | ||
| Cytomegalovirus | 830 | ||
| Herpes Simplex Virus | 831 | ||
| Congenital Rubella | 831 | ||
| Enteroviruses | 831 | ||
| Hepatotropic Viruses | 832 | ||
| Human Immunodeficiency Virus | 832 | ||
| Other Viruses | 832 | ||
| Parasitic Infections | 833 | ||
| Toxoplasmosis | 833 | ||
| Systemic Conditions Associated with Neonatal Hepatitis | 833 | ||
| Endocrinopathies | 833 | ||
| Chromosomal Disorders | 834 | ||
| Autoimmune Conditions | 834 | ||
| Hepatic Ischemia | 834 | ||
| Parenteral Nutrition | 834 | ||
| Idiopathic Neonatal Hepatitis | 835 | ||
| Management of Neonatal Cholestasis | 835 | ||
| Summary | 837 | ||
| Acknowledgments | 837 | ||
| Select References | 837 | ||
| References | 837.e2 | ||
| Questions | 837.e1 | ||
| Answers | 837.e1 | ||
| 69 Biliary Atresia and Neonatal Disorders of the Bile Ducts | 838 | ||
| Surgically Correctable Disorders | 838 | ||
| Biliary Atresia | 838 | ||
| Clinical Features and Diagnosis | 838 | ||
| Etiopathogenesis | 839 | ||
| Genetic Factors/Defective Morphogenesis | 840 | ||
| Vascular Abnormalities | 840 | ||
| Viral Infection | 840 | ||
| Toxins | 841 | ||
| Immune Mechanisms | 841 | ||
| Treatment | 842 | ||
| Choledochal Cysts | 843 | ||
| Spontaneous Perforation of the Bile Duct | 844 | ||
| Neonatal Sclerosing Cholangitis | 845 | ||
| Alagille Syndrome (Paucity of Interlobular Bile Ducts) | 845 | ||
| Neonatal Gallstones | 847 | ||
| Progressive Familial Intrahepatic Cholestasis Syndrome | 847 | ||
| Fibrocystic Liver Disease | 849 | ||
| Selected References | 850 | ||
| References | 850.e2 | ||
| Questions | 850.e1 | ||
| Answers | 850.e1 | ||
| 70 Pediatric Cholestatic Liver Disease | 851 | ||
| Defects in Bile Acid Production | 851 | ||
| Bile Acid Synthetic Defects | 851 | ||
| Defects in Membrane Transporters | 851 | ||
| Progressive Familial Intrahepatic Cholestasis (PFIC) | 852 | ||
| PFIC-1: FIC1 Disease | 855 | ||
| PFIC-2: BSEP Disease | 855 | ||
| PFIC-3, Multidrug Resistance Gene-3 (MDR-3) Deficient Disease | 855 | ||
| Benign Recurrent Intrahepatic Cholestasis | 855 | ||
| Hereditary Cholestasis with Lymphedema: Aagenaes Syndrome | 856 | ||
| Arthrogryposis Multiplex Congenita, Renal Dysfunction, and Cholestasis Syndrome | 856 | ||
| Neonatal Ichthyosis–Sclerosing Cholangitis Syndrome | 856 | ||
| North American Indian Childhood Cirrhosis | 857 | ||
| Cystic Fibrosis | 858 | ||
| Disorders of Embryogenesis: Alagille Syndrome | 858 | ||
| Clinical Manifestations of Alagille Syndrome | 859 | ||
| Hepatic Manifestations of ALGS | 859 | ||
| Neonatal Cholestasis. | 859 | ||
| Chronic Cholestasis and Natural History. | 859 | ||
| Liver Transplantation. | 860 | ||
| Cardiac Manifestations | 860 | ||
| Skeletal Manifestations | 861 | ||
| Ocular Manifestations | 861 | ||
| Facial Features | 861 | ||
| Renal Involvement in Alagille Syndrome (ALGS) | 862 | ||
| Vascular Involvement in ALGS | 862 | ||
| Growth and Nutrition | 862 | ||
| Notch Pathway and Development | 863 | ||
| Disorder of Mitochondrial Function | 863 | ||
| Navajo Neurohepatopathy | 863 | ||
| GRACILE Syndrome | 863 | ||
| α1-Antitrypsin Deficiency | 864 | ||
| Genetic Cholestasis | 865 | ||
| Cholestatic Liver Conditions Without Genetic Explanations | 867 | ||
| Primary Sclerosing Cholangitis | 867 | ||
| Autoimmune Sclerosing Cholangitis or Overlap Syndrome | 867 | ||
| Associations. | 868 | ||
| Disease Therapy. | 868 | ||
| Secondary Sclerosing Cholangitis | 868 | ||
| Selected References | 869 | ||
| References | 869.e2 | ||
| Questions | 869.e1 | ||
| Answers | 869.e1 | ||
| 71 Mitochondrial Hepatopathies: | 870 | ||
| Defects in Fatty Acid Oxidation | 870 | ||
| Pathophysiology | 870 | ||
| Clinical Features and Diagnosis | 871 | ||
| Diagnosis | 871 | ||
| Fatty Acid Transport Defects | 872 | ||
| Carnitine Transporter Deficiency | 872 | ||
| Carnitine Palmitoyl Transferase 1 Deficiency | 872 | ||
| Carnitine-acylcarnitine Translocase Deficiency | 872 | ||
| Carnitine Palmitoyl Transferase 2 Deficiency | 872 | ||
| Multiple Acyl-coenzyme: A Dehydrogenase Defect (Glutaric Acidemia Type 2) | 872 | ||
| Very-long-chain Acyl-CoA Dehydrogenase (VLCAD) Deficiency | 873 | ||
| Long-chain 3-hydroxyacyl-CoA Dehydrogenase (LCHAD) and Mitochondrial Trifunctional Protein (MTP) Deficiencies | 873 | ||
| Medium-chain Acyl-CoA Dehydrogenase (MCAD) Deficiency | 873 | ||
| Short-chain Acyl-CoA Dehydrogenase (SCAD) Deficiency | 873 | ||
| Acyl-CoA Dehydrogenase 9 (ACAD) Deficiency | 873 | ||
| Treatment of FAO Disorders | 873 | ||
| Outcome | 874 | ||
| Disorders of the Mitochondrial Respiratory Chain | 874 | ||
| mtDNA Point Mutations | 874 | ||
| mtDNA Rearrangements | 874 | ||
| mtDNA Depletion Syndromes | 874 | ||
| Deoxyguanosine kinase | 875 | ||
| POLG | 875 | ||
| MPV17 | 875 | ||
| Twinkle | 875 | ||
| Nuclear Genes Encoding Components of the Respiratory Chain | 875 | ||
| Nuclear Genes Implicated in Respiratory Chain Assembly | 875 | ||
| Nuclear Genes Involved in Translation of mtDNA-encoded Proteins | 875 | ||
| Clinical Phenotypes (Box 71-2) | 875 | ||
| Acute Liver Failure | 875 | ||
| Chronic Liver Disease | 876 | ||
| Pearson Syndrome | 876 | ||
| Alpers Syndrome | 876 | ||
| Drug-Induced Mitochondrial Disease | 876 | ||
| Diagnostic Approach | 876 | ||
| Treatment | 877 | ||
| Selected References | 878 | ||
| References | 878.e2 | ||
| Questions | 878.e1 | ||
| Answers | 878.e1 | ||
| 72 Abnormalities of Hepatic Protein Metabolism | 879 | ||
| α1-Antitrypsin Deficiency | 879 | ||
| The Characteristics of the α1-Antitrypsin Protein | 879 | ||
| Phenotyping of α1-Antitrypsin | 879 | ||
| Genetics of α1-Antitrypsin Deficiency | 879 | ||
| Clinical Manifestations of α1-Antitrypsin Deficiency | 880 | ||
| Liver Disease in Children | 880 | ||
| Liver Disease in Adults | 880 | ||
| Lung Disease | 881 | ||
| Pathophysiology of Liver Disease in α1-Antitrypsin Deficiency | 881 | ||
| Pathology of Liver Disease in α1-Antitrypsin Deficiency | 881 | ||
| Diagnosis of α1-Antitrypsin Deficiency | 882 | ||
| Treatment of α1-Antitrypsin Deficiency | 882 | ||
| Tyrosinemia | 882 | ||
| Hepatorenal Tyrosinemia (Hereditary Tyrosinemia Type I) | 882 | ||
| Pathogenesis | 883 | ||
| Clinical Features | 883 | ||
| Diagnosis | 883 | ||
| Genetics and Prenatal Diagnosis | 884 | ||
| Treatment | 884 | ||
| Urea-cycle Defects | 885 | ||
| Clinical Features | 885 | ||
| Diagnosis | 885 | ||
| Differential Diagnosis | 886 | ||
| Genetics and Prenatal Diagnosis | 886 | ||
| Treatment | 887 | ||
| Select References | 889 | ||
| References | 889.e3 | ||
| Questions | 889.e1 | ||
| Answers | 889.e1 | ||
| 73 Abnormalities of Carbohydrate Metabolism and the Liver | 890 | ||
| Nonalcoholic Fatty Liver Disease | 890 | ||
| Clinical Presentation | 891 | ||
| Diagnosis and Treatment | 891 | ||
| Galactosemia | 892 | ||
| Clinical Presentation | 893 | ||
| Diagnosis and Treatment | 893 | ||
| Hereditary Fructose Intolerance | 893 | ||
| 9 The Pancreas | 990 | ||
| 80 Pancreatic Development | 991 | ||
| Buds Form and Fuse | 991 | ||
| Ductal System Forms | 991 | ||
| Differentiation of Pancreatic Progenitors | 991 | ||
| Programmed Development by Hierarchical Signals | 991 | ||
| Transcriptional Factors Mediate Pancreatic Development | 991 | ||
| PDX1 | 991 | ||
| PTF1a | 991 | ||
| MIST1 | 991 | ||
| SOX9 | 991 | ||
| Signals from Adjacent Structures and Tissues | 992 | ||
| Endothelial Cells | 992 | ||
| Pancreatic Mesenchyme and Stroma | 992 | ||
| Epigenetic Control | 992 | ||
| Regenerative Capacity of the Pancreas | 993 | ||
| Regenerative Capacity After Pancreatitis | 993 | ||
| Reemergence of Embryonic Transcriptional Factors | 993 | ||
| Significance of Pancreatic Development on Postnatal Pancreatic Disorders | 993 | ||
| Reemergence of Embryonic Transcriptional Factors or Their Forced Expression Can Result in Metaplasia | 993 | ||
| Summary on Pancreatic Development | 994 | ||
| Pancreatic Secretion and Exocrine Function | 994 | ||
| Overview of Pancreatic Physiology | 994 | ||
| Functional Anatomy of the Exocrine Pancreas | 994 | ||
| Formation of Pancreatic Juice | 995 | ||
| Fluid and Electrolytes | 995 | ||
| Pancreatic Enzymes | 995 | ||
| Proteases | 995 | ||
| Lipases | 996 | ||
| Amylases | 996 | ||
| Regulation of Pancreatic Secretion | 996 | ||
| Interdigestive Phase of Pancreatic Secretion | 996 | ||
| Postprandial Phase of Pancreatic Secretion | 997 | ||
| Secretin | 997 | ||
| Cholecystokinin (CCK) | 997 | ||
| Serotonin | 997 | ||
| Other Stimulatory Factors | 997 | ||
| Neural Regulation | 998 | ||
| Pancreatic Secretion Inhibitors | 998 | ||
| Selected References | 998 | ||
| References | 998.e2 | ||
| Questions | 998.e1 | ||
| Answers | 998.e1 | ||
| 81 Cystic Fibrosis and Congenital Anomalies of the Exocrine Pancreas | 999 | ||
| Cystic Fibrosis | 999 | ||
| Cystic Fibrosis Transmembrane Regulator | 999 | ||
| CFTR Function in the Pancreas | 1000 | ||
| Pancreatic Sufficiency | 1001 | ||
| Pancreatic Insufficiency | 1001 | ||
| Pancreatic Function Testing | 1001 | ||
| Pancreatic Enzyme–Replacement Therapy | 1002 | ||
| Fibrosing Colonopathy | 1003 | ||
| Meconium Ileus | 1003 | ||
| Distal Intestinal Obstruction Syndrome | 1003 | ||
| Rectal Prolapse | 1004 | ||
| Pancreatitis | 1005 | ||
| Abdominal Pain | 1005 | ||
| Cystic Fibrosis–Related Diabetes Mellitus | 1006 | ||
| Nutrition | 1006 | ||
| Vitamin A | 1007 | ||
| Vitamin D | 1007 | ||
| Vitamin E | 1007 | ||
| Vitamin K | 1007 | ||
| Calcium | 1008 | ||
| Iron | 1008 | ||
| Trace Metals | 1008 | ||
| Essential Fatty Acids | 1008 | ||
| Nutritional Management | 1009 | ||
| Hepatobiliary Complications of Cystic Fibrosis | 1010 | ||
| Pathophysiology | 1010 | ||
| Clinical Evaluation and Diagnosis | 1010 | ||
| Spectrum of Disease | 1010 | ||
| Treatment | 1011 | ||
| Structural Abnormalities of the Pancreas | 1011 | ||
| Embryology | 1011 | ||
| Ductal Abnormalities | 1012 | ||
| Pancreas Divisum | 1012 | ||
| Anomalous Pancreaticobiliary Union | 1013 | ||
| Abnormalities of Migration | 1013 | ||
| Ectopic Pancreas | 1013 | ||
| Annular Pancreas | 1013 | ||
| Abnormalities in Volume | 1013 | ||
| Functional Abnormalities of the Pancreas | 1014 | ||
| Shwachman-Diamond Syndrome | 1014 | ||
| Johanson-Blizzard Syndrome | 1015 | ||
| Pearson’s Marrow-Pancreas Syndrome | 1015 | ||
| Jeune’s Syndrome | 1015 | ||
| Selected References | 1015 | ||
| References | 1015.e2 | ||
| Questions | 1015.e1 | ||
| Answers | 1015.e1 | ||
| 82 Pancreatitis | 1016 | ||
| Acute and Chronic Pancreatitis | 1016 | ||
| Definition and Classification | 1016 | ||
| Acute Pancreatitis | 1016 | ||
| Pathophysiology | 1016 | ||
| Pathology | 1017 | ||
| Etiology | 1017 | ||
| Clinical Presentation | 1018 | ||
| Diagnosis | 1019 | ||
| Laboratory Studies | 1019 | ||
| Imaging | 1019 | ||
| Investigations of Etiology | 1020 | ||
| Management | 1021 | ||
| Fluid Management | 1021 | ||
| Pain Control | 1021 | ||
| Nutritional Therapy | 1021 | ||
| Outcome | 1022 | ||
| Chronic Pancreatitis | 1022 | ||
| Pathophysiology | 1022 | ||
| Pathology | 1023 | ||
| Etiology | 1023 | ||
| Clinical Presentation | 1023 | ||
| Diagnosis | 1023 | ||
| Imaging | 1024 | ||
| Pancreatic Insufficiency | 1024 | ||
| Genetic Testing | 1025 | ||
| Management | 1026 | ||
| Pain Relief | 1026 | ||
| Pancreatic Insufficiency | 1026 | ||
| Selected References | 1026 | ||
| References | 1026.e3 | ||
| Questions | 1026.e1 | ||
| Answers | 1026.e1 | ||
| 83 Total Pancreatectomy with Islet Autotransplantation and Pancreatic Allotransplantation | 1027 | ||
| Surgical Procedure of Total Pancreatectomy | 1029 | ||
| Islet Isolation and Reintroduction | 1030 | ||
| Results of Islet Autotransplantation | 1031 | ||
| Whole Organ Pancreas Allotransplantation | 1032 | ||
| Organ Procurement | 1033 | ||
| Islets of Langerhans Allotransplantation | 1039 | ||
| Selected References | 1041 | ||
| References | 1041.e2 | ||
| Questions | 1041.e1 | ||
| Answers | 1041.e1 | ||
| 84 Pancreatic Neuroendocrine Tumors | 1042 | ||
| Embryologic Origin of PNETs | 1042 | ||
| Classification of PNETs—Functional and Nonfunctional, Sporadic and Hereditary | 1042 | ||
| Grading and Staging | 1042 | ||
| Inherited Syndromes | 1043 | ||
| Multiple Endocrine Neoplasia Type 1 (MEN1) | 1043 | ||
| Von Hippel-Lindau (VHL) | 1044 | ||
| Neurofibromatosis Type 1 (NF1) | 1045 | ||
| Tuberous Sclerosis (TSC) | 1045 | ||
| Nesidioblastosis | 1045 | ||
| Insulinoma | 1045 | ||
| Epidemiology | 1045 | ||
| Diagnosis | 1046 | ||
| Localization | 1046 | ||
| Treatment and Outcomes | 1047 | ||
| Medical Management | 1047 | ||
| Surgical Management | 1047 | ||
| Gastrinoma | 1048 | ||
| Epidemiology | 1048 | ||
| Diagnosis | 1048 | ||
| Localization | 1048 | ||
| Treatment and Outcome | 1049 | ||
| Medical Management | 1049 | ||
| Surgical Management | 1049 | ||
| Locoregional Disease | 1049 | ||
| Locally Advanced/Metastatic Gastrinoma | 1049 | ||
| Rare FPNETs | 1050 | ||
| Glucagonoma | 1050 | ||
| VIPoma | 1050 | ||
| Somatostatinoma | 1050 | ||
| NFPNETs | 1050 | ||
| Locally Advanced/Metastatic PNET | 1051 | ||
| Surgical and Interventional Radiologic Techniques for Liver Metastases | 1051 | ||
| Systemic and Targeted Therapies | 1051 | ||
| Conclusion | 1052 | ||
| Selected References | 1052 | ||
| References | 1052.e4 | ||
| Questions | 1052.e1 | ||
| Answers | 1052.e2 | ||
| 85 Infant and Toddler Nutrition | 1053 | ||
| Growth | 1053 | ||
| Breast-Feeding | 1054 | ||
| Formula Feeding | 1058 | ||
| Complementary Feeding | 1059 | ||
| Supplements | 1059 | ||
| Toddlers | 1061 | ||
| Descriptive Information | 1061 | ||
| Nutrient Information | 1061 | ||
| Energy | 1061 | ||
| Fats | 1062 | ||
| Essential Fatty Acids (EFAs) | 1062 | ||
| Trans-Fats | 1062 | ||
| Proteins | 1063 | ||
| Carbohydrates | 1063 | ||
| Specific Nutrient Deficits | 1063 | ||
| Iron | 1063 | ||
| Depleted Iron Stores | 1064 | ||
| Iron Deficiency without Anemia | 1064 | ||
| Iron Deficiency Anemia | 1064 | ||
| Zinc | 1064 | ||
| Vitamin E | 1064 | ||
| Toddlers’ Nutrition and Stooling | 1064 | ||
| Diarrhea | 1065 | ||
| Constipation | 1065 | ||
| Psychology of Toddlers’ Nutrition | 1065 | ||
| Eating the Right Amount | 1065 | ||
| Meals and Snacks | 1065 | ||
| Food Security | 1065 | ||
| Conclusion | 1065 | ||
| Selected References | 1065 | ||
| References | 1066.e2 | ||
| Questions | 1066.e1 | ||
| Answers | 1066.e1 | ||
| 10 Nutrition | 1067 | ||
| 86 Nutritional Assessment | 1068 | ||
| Normal Growth and Body Composition in Children | 1068 | ||
| Prevalence and Classification of Malnutrition in Pediatric Patients | 1068 | ||
| Indications for Nutrition Assessment | 1069 | ||
| Components of a Nutritional Assessment | 1070 | ||
| Clinical History and Physical Assessment | 1070 | ||
| Anthropometric Measurements | 1070 | ||
| Body Mass Index (BMI) | 1074 | ||
| Triceps Skinfold/Mid-Arm Circumference (TSF/MAC) | 1074 | ||
| Detailed Diet History | 1074 | ||
| Biochemical Analyses | 1075 | ||
| Serum Proteins | 1075 | ||
| Focus on Specific Nutrients | 1075 | ||
| Iron | 1075 | ||
| Zinc | 1076 | ||
| Vitamin D | 1076 | ||
| Calcium | 1076 | ||
| Inflammation | 1076 | ||
| Treatment of Malnutrition | 1076 | ||
| Pathophysiology of Starvation | 1077 | ||
| Refeeding Syndrome | 1077 | ||
| Nutrition Rehabilitation | 1077 | ||
| Stabilization | 1078 | ||
| Rehabilitation | 1078 | ||
| Follow-up | 1078 | ||
| Future Focus | 1078 | ||
| Summary | 1078 | ||
| Selected References | 1078 | ||
| References | 1079.e2 | ||
| Questions | 1079.e1 | ||
| Answers | 1079.e1 | ||
| 87 Tubes for Enteric Access | 1080 | ||
| Tubes to Remove Contents (Suction Tubes) | 1080 | ||
| Tubes to Add Content (Feeding Tubes) | 1081 | ||
| Evaluation | 1082 | ||
| Considerations for Short-Term Access | 1084 | ||
| Considerations for Long-Term Access | 1084 | ||
| Gastroesophageal Reflux and Feeding Tubes | 1086 | ||
| Summary | 1087 | ||
| Selected References | 1087 | ||
| References | 1087.e2 | ||
| Questions | 1087.e1 | ||
| Answers | 1087.e1 | ||
| 88 Parenteral Nutrition | 1088 | ||
| Indications | 1088 | ||
| Route of Administration | 1088 | ||
| Parenteral Nutrition Components and Requirements | 1089 | ||
| Energy | 1089 | ||
| Protein | 1090 | ||
| Carbohydrates | 1093 | ||
| Lipids | 1093 | ||
| Electrolytes and Minerals | 1094 | ||
| Vitamins | 1095 | ||
| Trace Elements | 1096 | ||
| Additives | 1097 | ||
| Shortages | 1097 | ||
| Monitoring | 1097 | ||
| Complications | 1098 | ||
| Mechanical Complications | 1098 | ||
| Infectious Complications | 1098 | ||
| Metabolic Complications | 1099 | ||
| Total Nutrient Admixtures | 1100 | ||
| Formulating a Regimen | 1101 | ||
| Cyclic Parenteral Nutrition | 1101 | ||
| Home Parenteral Nutrition | 1102 | ||
| Monitoring the Home PN Patient | 1102 | ||
| Summary | 1103 | ||
| Selected References | 1103 | ||
| References | 1103.e2 | ||
| Questions | 1103.e1 | ||
| Answer | 1103.e1 | ||
| 89 Enteral Nutrition | 1104 | ||
| Background | 1104 | ||
| Metabolic Implications of Enteral Versus Parenteral Route | 1104 | ||
| Steps Preceding Initiation of Enteral Feeding | 1105 | ||
| An Approach to Selecting the Type of Enteral Feeding | 1107 | ||
| Special Formula Diets | 1107 | ||
| Principal Components of Specialized Formula Diets | 1109 | ||
| Energy Requirements | 1109 | ||
| Fat | 1110 | ||
| Carbohydrates | 1111 | ||
| Protein | 1112 | ||
| Vitamins and Minerals | 1112 | ||
| Administration of Formula Feedings | 1113 | ||
| Initiation of Feeds | 1117 | ||
| Home Tube Feeding | 1118 | ||
| Families of Children with Special Needs | 1119 | ||
| Discontinuing Enteral Feedings | 1119 | ||
| Summary | 1119 | ||
| Select References | 1119 | ||
| References | 1120.e3 | ||
| Questions | 1120.e1 | ||
| Answers | 1120.e1 | ||
| 90 Management of Diarrhea | 1121 | ||
| Management of Acute Diarrhea | 1121 | ||
| Pathophysiology of Diarrhea and the Evolution of ORS | 1121 | ||
| Super ORS | 1122 | ||
| Hypoosmolar ORS | 1122 | ||
| Early Feeding | 1123 | ||
| Treatment Strategies and Practical Guidelines | 1124 | ||
| Clinical Signs and Symptoms | 1124 | ||
| Workup and Laboratory Studies | 1125 | ||
| Management in the Home Setting (Pre-Hospital Care) | 1125 | ||
| Hospital Management | 1126 | ||
| Intravenous Therapy | 1126 | ||
| Hypernatremic Dehydration | 1126 | ||
| Complications | 1126 | ||
| Antimicrobial Therapy | 1127 | ||
| Probiotics | 1127 | ||
| Antidiarrheal Agents | 1127 | ||
| Prevention | 1128 | ||
| Vaccines | 1128 | ||
| Conclusion | 1129 | ||
| Management of Chronic Diarrhea | 1129 | ||
| Chronic Infectious Diarrhea | 1129 | ||
| Postenteritis Syndrome (or Persistent Diarrhea) | 1130 | ||
| Carbohydrate Intolerance | 1130 | ||
| Protein Intolerance | 1130 | ||
| Nonspecific Functional Diarrhea | 1130 | ||
| Toddler Diarrhea | 1130 | ||
| Irritable Bowel Syndrome | 1130 | ||
| Inflammatory Bowel Disease (IBD) | 1130 | ||
| Pancreatic Insufficiency | 1130 | ||
| Immunodeficiency Syndromes | 1130 | ||
| Severe and Protracted Diarrhea (Intractable Diarrhea) of Infancy | 1130 | ||
| Congenital Chloridorrhea | 1131 | ||
| Conclusion | 1131 | ||
| Select References | 1131 | ||
| References | 1131.e2 | ||
| Questions | 1131.e1 | ||
| Answers | 1131.e1 | ||
| 91 Effects of Digestive Diseases on Bone Metabolism | 1132 | ||
| Accretion of Bone Mass and Maintenance of Bone Mass | 1132 | ||
| Bone Modeling | 1132 | ||
| Bone Remodeling | 1133 | ||
| Bone Structure | 1133 | ||
| Peak Bone Mass | 1133 | ||
| Measurement of Bone Mass in Children | 1134 | ||
| Dual X-ray Absorptiometry (DXA) | 1134 | ||
| Peripheral Quantitative Computed Tomography | 1135 | ||
| Bone Biopsy | 1135 | ||
| Biomarkers of Bone Metabolic Activity | 1135 | ||
| Definition of Osteoporosis in Children | 1135 | ||
| Common Mechanisms by Which Digestive Diseases Affect Bone Mass | 1135 | ||
| Endocrine Dysfunction | 1135 | ||
| Nutritional Factors | 1136 | ||
| Decreased Physical Activity | 1136 | ||
| Cytokines | 1136 | ||
| RANKL and Osteoprotegerin | 1136 | ||
| Medications | 1137 | ||
| Selected Gastrointestinal and Liver Diseases Associated with Decreased Bone Mass | 1137 | ||
| Inflammatory Bowel Diseases | 1137 | ||
| Celiac Disease | 1138 | ||
| Cholestatic Liver Disease | 1138 | ||
| Liver Transplantation | 1138 | ||
| Cystic Fibrosis | 1139 | ||
| Hypertrophic Pulmonary Osteoarthropathy | 1139 | ||
| Treatment | 1140 | ||
| General Measures | 1140 | ||
| Vitamin D | 1140 | ||
| Calcium | 1140 | ||
| Bone-Active Agents | 1141 | ||
| Selected References | 1141 | ||
| References | 1141.e2 | ||
| Questions | 1141.e1 | ||
| Answers | 1141.e1 | ||
| 92 Nutrition and Feeding for Children with Developmental Disabilities | 1142 | ||
| Cerebral Palsy and Growth Failure | 1142 | ||
| Causes of Growth Failure | 1142 | ||
| Benefits of Nutritional Rehabilitation | 1143 | ||
| Developing a Nutritional Plan | 1143 | ||
| Nutritional Assessment | 1143 | ||
| Feeding History | 1143 | ||
| Anthropometrics | 1143 | ||
| Nutritional Needs | 1144 | ||
| Laboratory Evaluation | 1145 | ||
| Nutritional Goals | 1145 | ||
| Oropharyngeal Swallowing Assessment | 1145 | ||
| Evaluation for Supplemental Feeding Tube | 1146 | ||
| Therapeutic Goals | 1148 | ||
| Selected References | 1149 | ||
| References | 1149.e2 | ||
| Questions | 1149.e1 | ||
| Answers | 1149.e1 | ||
| Index | 1151 | ||
| A | 1151 | ||
| B | 1155 | ||
| C | 1157 | ||
| D | 1163 | ||
| E | 1165 | ||
| F | 1168 | ||
| G | 1170 | ||
| H | 1173 | ||
| I | 1176 | ||
| J | 1179 | ||
| K | 1179 | ||
| L | 1179 | ||
| M | 1181 | ||
| N | 1183 | ||
| O | 1185 | ||
| P | 1186 | ||
| Q | 1190 | ||
| R | 1190 | ||
| S | 1191 | ||
| T | 1194 | ||
| U | 1196 | ||
| V | 1196 | ||
| W | 1198 | ||
| X | 1198 | ||
| Y | 1198 | ||
| Z | 1198 | ||
| color insert | P1 | ||
| Inside Back Cover | ibc1 |