Manual de Gastroenterología, Hepatología y Nutrición Pediátrica

SECTION 1: Gastroenterology
1. Developmental Anatomy and Physiology of the Digestive Tract………………………………………………………….3
Yogesh Waikar
Applied developmental anatomy and physiology of esophagus 3
Applied developmental anatomy and physiology of stomach 3
Applied developmental anatomy and physiology of intestines 4
Applied developmental anatomy of pancreas 5
2. Common Gastrointestinal Symptoms……………………………………………………………………………………………………7
Wafa’a Al-Qabandi
Vomiting 7
Diarrhea 14
Abdominal pain 22
Constipation 27
Gastrointestinal bleeding 33
Abdominal distension 42
Failure to thrive 48
3. Disorders of the Esophagus ……………………………………………………………………………………………………………….. 59
SK Mittal
Gastroesophageal reflux disease (gerd) 59
Management of gerd 64
Pharmacologic therapies 65
Acquired esophageal stricture 69
Esophageal achalasia 71
4. Neurogastroenterology and Motility Disorders…………………………………………………………………………………. 78
Paolo Quitadamo, Efstratios Saliakellis, Osvaldo Borrelli, Nikhil Thapar
Abdominal pain-related functional gastrointestinal disorders 78
Functional constipation 81
Chronic intestinal pseudo-obstruction 83
Gastrointestinal and nutritional problems in neurologically impaired children 85
Cyclical vomiting syndrome 87
5. Disorders of the Stomach and Small Intestine …………………………………………………………………………………… 93
Ravinder Goyal, BR Thapa, Rimjhim Srivastava, Srikanth KP
Congenital anomalies of the stomach 93
Gastritis: H. pylori and peptic ulcer disease, and non-H. pylori ulcers 96
Congenital anomalies of small intestine 101
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6. Acute Diarrhea…………………………………………………………………………………………………………………………………..111
AK Patwari
Epidemiology 111
Etiology 111
Pathogenesis 111
Clinical features 112
Principles of treatment 112
7. Chronic and Persistent Diarrhea ……………………………………………………………………………………………………….119
Sarah Paul, John Matthai
Chronic diarrhea 119
Persistent diarrhea 122
8. Celiac Disease…………………………………………………………………………………………………………………………………….125
Bhupinder Sandhu, Nkem Onyeador
Definition 125
Historical background 125
Epidemiology 126
Genetics 126
Pathogenesis 127
Clinical features and patterns of disease 127
Investigations 128
Screening for celiac disease 130
Management of celiac disease 131
Prognosis 132
Complications of celiac disease 133
Refractory celiac disease 133
Future research 133
Sources of patient/parent information 134
9. Inflammatory Bowel Disease …………………………………………………………………………………………………………….137
Boosba Vivatvakin, Palittiya Sintusek
Inflammatory bowel disease 137
10. Disorders of the Pancreas………………………………………………………………………………………………………………….147
A Riyaz
Congenital anomalies 147
Genetic disorders 148
Acute pancreatitis 149
Chronic pancreatitis 154
Tumors of pancreas 156
11. Short Bowel Syndrome ……………………………………………………………………………………………………………………..159
Olivier Goulet
Etiology and incidence 159
Pathophysiology 163
Management 166
Long-term outcome and growth of patients 173
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12. Surgical Disorders of the Gastrointestinal Tract ……………………………………………………………………………….181.
Rajeev Redkar, Parag Karkera
Congenital Anomalies 181
Esophageal atresia and tracheoesophageal fistula 181
Infantile hypertrophic pyloric stenosis 183
Duodenal atresia 184
Ileal and jejunal atresia 184
Meconium ileus 185
Malrotation of gut 186
Abdominal wall defects (omphalocele and gastrochisis) 188
Hirschsprung’s disease 189
Anorectal malformations 190
Acute Emergencies 193
Appendicitis 193
Intussusception 194
Meckel’s diverticulum 196
Intestinal obstruction 197
Inflammatory bowel disease 198
Rarer Issues and Recent Advances 199
Annular pancreas 199
Pancreas divisum 199
Gastroesophageal reflux disease 199
Bariatric surgery 200
SECTION 2: Nutrition
13. Nutritional Assessment in Health and Disease………………………………………………………………………………….207
K Vijayaraghavan
Goals 207
Guiding principles 207
Methods of nutrition assessment 208
Clinical examination 208
Nutritional anthropometry 211
Biochemical assessment 214
Dietary assessment 216
14. Food Allergy ………………………………………………………………………………………………………………………………………218
S Srinivas
Terminology and definitions 218
Incidence 218
Immunological basis of food allergy 218
Pathophysiology of food allergy 219
Immunopathogenesis of food allergies 219
Symptoms associated with food hypersensitivity 220
Clinical phenotypes/reactions seen in food allergy 220
Food allergy to specific foods 220
Cow’s milk allergy/cow’s milk protein allergy (cma/cmpa) 221
Natural history of food allergy 221
Descriptive case scenarios in food allergy 223
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15. Childhood Malnutrition …………………………………………………………………………………………………………………….226
Md Iqbal Hossain, Tahmeed Ahmed
Guidelines for the management of children with sam 228
Community-based management of sam 233
Micronutrient deficiencies in children 234
Iron deficiency 235
Vitamin A deficiency 236
Zinc deficiency 236
Iodine deficiency 237
16. Pediatric Enteral Nutrition…………………………………………………………………………………………………………………239
Sarath Gopalan
American Society of Parenteral and Enteral Nutrition recommendations 239
General principles of pediatric enteral nutrition 240
Indications for enteral nutrition 241
Pediatric enteral nutrition formulations 241
Enteral nutrition delivery—access techniques and devices 243
Complications associated with enteral feeding 246
17. Parenteral Nutrition…………………………………………………………………………………………………………………………..248
Mohammad Nurul Akhtar Hasan, Sandipan Sarkar, Garima Garg, Parvathi U Iyer
Preferred route of nutrient delivery 249
Total parenteral nutrition and partial parenteral nutrition 249
Route of administration 249
Indications for parenteral nutrition 250
Sources of energy 250
Carbohydrate requirement 251
Protein requirement 251
Total nutrient admixture (tna) 256
18. Probiotics in Gastrointestinal Disorders……………………………………………………………………………………………261
Juliet Sio Aguilar
Definitions 261
Mechanisms of action of probiotics 261
Strains with documented efficacy 262
SECTION 3: Hepatology
19. Anatomy, Development and Physiology of the Hepatobiliary System …………………………………………….273
Dhanasekhar Kesavelu
Functional anatomy: sectors and segments 273
Anatomy of the biliary tract and bile duct epithelial cells 274
Development of hepatic vasculature 274
Liver physiology including hepatocellular function 274
Hepatocyte death and regeneration 275
20. Approach to Common Liver: Symptoms and Signs…………………………………………………………………………..276
Siham Al Sinani
Jaundice 276
Pruritus 280
Hepatomegaly 282
Splenomegaly 284
Contents xxi
Ascites 286
Transaminitis 289
Upper gastrointestinal bleeding 290
21. Interpretation of Liver Function Tests ……………………………………………………………………………………………….294
BR Thapa, Ravinder Goyal, Srikanth KP
Uses 294
Limitations 294
Classification of lft 294
Tests of the liver’s capacity to transport organic anions 294
Tests for hepatocytic necrosis 297
Tests of the liver’s biosynthetic capacity 301
22. Neonatal Cholestasis …………………………………………………………………………………………………………………………309
James Huang, Marion M Aw
Etiopathogenesis of neonatal cholestasis 309
Approach to an infant with cholestasis 311
History 311
Physical examination 312
A diagnostic approach 312
General principles of management 316
Approach to the premature infant with cholestasis 316
Extrahepatic causes of cholestasis 317
Familial (inherited) causes of intrahepatic cholestasis 320
23. Acute Hepatitis ………………………………………………………………………………………………………………………………….326
Ramaswamy Ganesh, Malathi Sathiyasekaran
Etiology 326
Epidemiology 326
Etiopathogenesis 327
Atypical manifestations and complications of acute viral hepatitis 330
Differential diagnosis 331
Management of acute viral hepatitis 333
Treatment for acute hepatitis due to other nonhepatotropic viruses 334
24. Chronic Viral Hepatitis……………………………………………………………………………………………………………………….337
Neelam Mohan, Deepak Goyal
Clinical features of chronic viral hepatitis 337
Hepatitis B 337
Hepatitis C 343
25. Autoimmune Hepatitis………………………………………………………………………………………………………………………348
M Geetha
Epidemiology 348
Genetics 348
Pathogenesis 349
Types 349
Clinical patterns of presentation 350
Natural history 351
Autoantibodies 351
Liver biopsy 353
Imaging 353
Diagnostic criteria 353
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Treatment 353
Standard treatment 354
Treatment decisions: when to start treatment 356
Relapse 357
Treatment failure 357
Prognosis 357
Transplantation 357
Post-transplant recurrent aih 357
Post liver transplantation de novo aih 357
Autoimmune sclerosing cholangitis (asc) 358
Overlap syndrome 358
Management 358
26. Wilson’s Disease…………………………………………………………………………………………………………………………………363
Dinesh Banur
Pathogenesis and metabolism of copper 363
Pathogenesis 363
Clinical features 363
Diagnosis 365
Treatment 366
27. Metabolic Disorders in the Infant ……………………………………………………………………………………………………..373
Suresh Vijay
Metabolic liver disease 373
Acute liver failure 377
Isolated organomegaly or when associated with other clinical features 379
Hydrops fetalis 384
28. Metabolic Disorders in the Older Child……………………………………………………………………………………………..386
Wikrom Karnsakul, Kathleen B Schwarz
Defects in bile acid synthesis 386
Progressive familial intrahepatic cholestasis (pfic)— types 1, 2, and 3 387
Alagille syndrome (ags) 388
Storage disorders affecting the liver 388
Cystic fibrosis 391
Disorders of heme metabolism and the liver 392
29. Nonalcoholic Fatty Liver Disease ………………………………………………………………………………………………………397
Vidyut Bhatia
Definition 397
Epidemiology 397
Risk factors 397
Natural history and pathogenesis 398
Symptoms 398
Histopathology and scoring systems 398
Ultrasonography 398
Computed tomography/magnetic resonance imaging 399
Transient elastography 399
Treatment 399
30. Chronic Liver Disease ………………………………………………………………………………………………………………………..401
B Sumathi
Definition 401
Causes of chronic liver disease other than neonatal cholestasis syndrome 401
Clinical presentation of chronic liver disease 402
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Signs of chronic liver failure 402
Clinical clues for etiology 402
Complications 404
Renal complications and hepatorenal syndrome 405
Ascites and spontaneous bacterial peritonitis 405
Evaluation 405
Treatment 406
Management of variceal bleed 406
Spontaneous bacterial peritonitis 407
Diet in chronic liver disease 407
31. Surgical Disorders of the Hepatobiliary System ……………………………………………………………………………….408
Sujit Chowdhary
Biliary atresia 408
Choledochal cyst 410
Portal hypertension 412
Intrahepatic obstruction 413
Cholelithiasis 413
Liver tumors 414
Mesenchymal hamartoma 414
Focal nodular hyperplasia 414
Hepatocellular adenoma 415
Malignant primary liver tumors 415
Hepatoblastoma 415
Undifferentiated embryonal sarcoma 415
Hepatocellular carcinoma 416
32. Drug-induced Liver Injury …………………………………………………………………………………………………………………418
Seema Alam, Sanjiv Verma
Epidemiology 418
Development of drug metabolism in liver 419
Pathogenesis 419
Diagnosis of dili 419
Commonly incriminated drugs in children 420
Treatment of dili 421
Liver transplantation in dili 422
33. Acute Liver Failure……………………………………………………………………………………………………………………………..424
Deirdre Kelly
Pathogenesis 424
Pathology 424
Etiology 425
Acute liver failure in infancy 425
Metabolic disease 426
Acute liver failure in older children 428
Drug and toxin-related hepatic injury 429
Selection for liver transplantation 433
34. Portal Hypertension ………………………………………………………………………………………………………………………….437
Sankaranarayanan VS, Srinivas S
Definition 437
Pathophysiology 437
Classification 438
Etiology 438
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Clinical features 439
Diagnosis 440
Management of portal hypertension in children 441
Management of esophageal varices—endoscopic therapy 441
Main indications for surgery 445
35. Liver Transplantation ………………………………………………………………………………………………………………………..447
Akshay Kapoor, Subash Gupta, Anupam Sibal, Vidyut Bhatia,
Neerav Goyal, Manav Wadhawan, Sarath Gopalan
Indications 447
Pretransplant evaluation 449
Transplant surgery 450
Liver transplantation in India 454
36. Liver Tumors………………………………………………………………………………………………………………………………………458
Huma Cheema, Manas Kalra, Amita Mahajan
Hepatoblastoma 458
Hepatocellular carcinoma 461
37. Liver in Systemic Illness……………………………………………………………………………………………………………………..464
Hanifah Oswari
Critically ill patient 464
Infection 464
Liver in the presence of altered systemic circulation 465
Hematologic disorders 466
Collagen vascular disease 466
Nutritional disorders 468
Index ……………………………………………………………………………………………………………………………………………………………………………………………………………473