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Textbook of Pediatric Gastroenterology, Hepatology and Nutrition
Anupam Sibal, Sarath Gopalan, Akshay Kapoor, Vidyut Bhatia
SECTION 1: GASTROENTEROLOGY
CHAPTER 1:
Developmental Anatomy and Physiology of the Digestive Tract
APPLIED DEVELOPMENTAL ANATOMY AND PHYSIOLOGY OF ESOPHAGUS
APPLIED DEVELOPMENTAL ANATOMY AND PHYSIOLOGY OF STOMACH
APPLIED DEVELOPMENTAL ANATOMY AND PHYSIOLOGY OF INTESTINES
APPLIED DEVELOPMENTAL ANATOMY OF PANCREAS
CHAPTER 2:
Common Gastrointestinal Symptoms
VOMITING
Pathophysiology
Phases of Vomiting
Causes of Vomiting in Children
Evaluation
History
Age of the Child
Color of Vomitus
When Does Vomiting Occur?
Relation to Food
Associated Gastrointestinal Symptoms
Systemic Review
Pattern of Vomiting
Physical Examination
Red Flags in a Vomiting Child
Investigations
Blood Work Up
Urine Tests
Imaging Studies
Endoscopy
Management
Pharmacotherapy
Antiemetics
Nonpharmacological Therapy
Regurgitation
Rumination
Rumination in Older Children and Adolescents
DIARRHEA
Definition
Absorption of Fluid and Electrolytes Through the Gastrointestinal Tract (GIT)
Absorption
Secretion
Pathophysiology
Osmotic
Secretory
Inflammation
Motility-related
Causes of Diarrhea
Acute Diarrhea
Chronic Diarrhea
Evaluation of Diarrhea
Physical Examination
Investigations
Acute Diarrhea
Chronic Diarrhea
Management
Acute Diarrhea
Chronic Diarrhea
ABDOMINAL PAIN
Types of Pain
Causes of Abdominal Pain
Acute Pain
Chronic Abdominal Pain
Evaluation
Location
Onset
Duration
Quality
Severity
Radiation of Pain
Relieving/Aggravating Factors
Associated Symptoms
Physical Examination
Investigations
First Line Tests
Second Line Tests
Third Line Tests
Management
CONSTIPATION
Normal Bowel Movements in Children
Definition of Constipation
Other Important Definitions
Soiling
Encopresis
Fecal Incontinence
When Does Constipation Start?
Epidemiology
Pathophysiology of Constipation
Functional Constipation
Evaluation of a Child with Constipation
History
Physical Examination
Investigations
Blood Work-up
Imaging Studies
Anorectal Manometry
Rectal Biopsy
Management
Education
Rectal Disimpaction
Rectal Enema
Treatment of the Perianal Area
Maintenance Therapy
Other Laxatives
Probiotics
Dietary Modification
Behavioral Therapy
Sacral Neuromodulation
Surgery for Functional Constipation
GASTROINTESTINAL BLEEDING
Presentations of GIT Bleeding
Other Presentations
Causes of GI Bleeding
Approach to a Child with GI Bleeding
Upper GI Bleeding
History
Neonates
Infants
Preschool-aged Children
Older Children/Adolescents
Physical Examination
Investigations of UGI Bleeding
Other Tests
Management of UGI Bleedingin Children (Flow chart 2.1)
Lower GI Bleeding
Causes of LGI Bleeding
Approach to a Child with LGI Bleeding
History
Physical Examination
Evaluation of LGI Bleeding
Management of LGI Bleed (Flow chart 2.2)
ABDOMINAL DISTENSION
Definition
General Causes of Abdominal Distension
Abdominal Distension Due toGaseous Distension
Composition of Intestinal Gas (Box 2.14)
Causes of Excessive Gaseous Distension
Abdominal Distension Due to Ascites
Abdominal Distension Caused by Organomegaly
Abdominal Distension Caused byIntra-abdominal Tumors
Abdominal Distension Due toAbdominal Wall Problems
Approach to a Child with Abdominal Distension
History
Examination
Investigations
In a Case of Ascites
Investigations of Abdominal DistensionDue to Organomegaly
Investigations of Abdominal Distension Caused by an Intra-abdominal Mass
Investigations of Abdominal DistensionCaused by Gaseous Distension
Management of Abdominal Distension
FAILURE TO THRIVE
Definition
Epidemiology
Causes of FTT
Approach of a Child with FTT
History
Physical Examination
Investigations
Management (Box 2.17)
Refeeding Syndrome
Prognosis
CHAPTER 3:
Disorders of the Esophagus
GASTROESOPHAGEAL REFLUX DISEASE (GERD)
Definition of GER and GERD
Pathophysiology
Clinical Manifestations
Diagnostic Tests for GERD
Diagnostic Tests Available
Barium Contrast Radiography
Esophageal and Gastric Ultrasonography
Nuclear Scintigraphy
Endoscopy and Biopsy
Esophageal pH Monitoring
Motility Studies
Combined Multiple Intraluminal Impedance (MII) and pH Monitoring
Tests on Ear, Lung and Esophageal Fluids
Empiric Trial of Acid Suppression as a Diagnostic Test
Summary of the Diagnostic Techniques
Simplified Approach to Diagnosis and Management of GERD Based on Symptoms and Signs (Flow charts 3.1 and 3.2)
MANAGEMENT OF GERD
Physiological GER
Positioning Therapy for Infants
Lifestyle Changes in Children and Adolescents
PHARMACOLOGIC THERAPIES
Histamine-2 Receptor Antagonists (H2RA)
Proton Pump Inhibitors
Prokinetics
Other Agents
Surgical Therapy
Corrosive Ingestion and Injury
Caustic Ingestion
Acute Corrosive Ingestion
Investigations
Radiology
Management
Subsequent Management
ACQUIRED ESOPHAGEAL STRICTURE
Etiology
Clinical Features
Diagnosis
Management
Esophageal Foreign Bodies
Pathophysiology of Injury
Definition
Pathogenesis
Clinical Features
Diagnosis
Management
Infections of the Esophagus
Eosinophilic Esophagitis
Esophageal Atresia and Tracheoesophageal Fistula
Embryology
Pathology
Associated Defects
Clinical Presentation
Management
Surgery
Postoperative Care
CHAPTER 4:
Neurogastroenterology and Motility Disorders
INTRODUCTION
ABDOMINAL PAIN-RELATED FUNCTIONAL GASTROINTESTINAL DISORDERS
Introduction
Epidemiology
Pathophysiology
Clinical Presentation
Diagnostic Investigations
Treatment
Prognosis
Conclusion
FUNCTIONAL CONSTIPATION
Introduction
Pathophysiology
Diagnosis
Investigations
Treatment
Prognosis and Long-term Outcomes
CHRONIC INTESTINAL PSEUDO-OBSTRUCTION
Introduction
Pathophysiology
Clinical Presentation
Diagnosis
Treatment
Prognosis
Summary and Future Perspectives
GASTROINTESTINAL AND NUTRITIONAL PROBLEMS IN NEUROLOGICALLY IMPAIRED CHILDREN
Introduction
Feeding and Nutritional Aspects
Gastrointestinal Problems
Dysphagia
Gastroesophageal Reflux
Constipation
CYCLICAL VOMITING SYNDROME
Introduction
Pathophysiology
Clinical presentation
Diagnosis
Management
Prognosis
CHAPTER 5:
Disorders of the Stomachand Small Intestine
CONGENITAL ANOMALIES OF THE STOMACH
Gastric Atresia or Stenosis
Clinical Features
Diagnosis
Management
Gastric Duplication
Clinical Features
Diagnosis
Management
Gastric Volvulus
Clinical Features
Diagnosis
Management
Inantile Hypertrophic Pyloric Stenosis
Clinical Features
Diagnosis
Management
Microgastria
Clinical Features
Diagnosis
Management
GASTRITIS: H. PYLORI AND PEPTIC ULCER DISEASE, AND NON-H. PYLORI ULCERS
Helicobacter pylori
Gastritis and Peptic Ulcer
Epidemiology
Transmission
Pathogenesis
Clinical Features
Diagnosis
Treatment of H. pylori-related Gastritis and PUD
Prognosis
Helicobacter pylori -Negative Gastritis
Etiology
Gastric Neoplasms
Gastric Lymphoma
Adenocarcinoma
Gastrointestinal Stromal Tumor
Gastric Teratoma
Gastric Polyps
Conclusion
CONGENITAL ANOMALIES OF SMALL INTESTINE
Duodenal Atresia and Stenosis
Types
Clinical Features
Diagnosis
Management
Small Intestinal Duplication
Histologic Criteria for Duplication Cysts62
Types70
Clinical Features
Diagnosis
Management
Malrotation and Midgut Volvulus
Clinical Features
Diagnosis
Management
Jejunal and Ileal Atresia
Types
Clinical Features
Diagnosis
Management
Megacystis-Microcolon-Malrotation-Intestinal-Hypoperistalsis Syndrome (MMMIHS)
Clinical Features
Diagnosis
Management
Small Intestinal Bacterial Overgrowth
Pathophysiology
Clinical Features
Diagnosis of SIBO
Breath Tests
Treatment
CHAPTER 6:
Acute Diarrhea
INTRODUCTION
EPIDEMIOLOGY
ETIOLOGY
PATHOGENESIS
CLINICAL FEATURES
Approach to a Patient with Acute Diarrhea
PRINCIPLES OF TREATMENT
Assessment of Dehydration
Compensatory Mechanisms
Prevention and Treatment of Dehydration
Nutritional Management During and After a Diarrheal Episode
Zinc Supplementation for Treatment of Diarrhea
Treatment of Associated Problems
Risk Factors for Diarrheal Morbidity and Mortality
Correction of Electrolyte and Acid-base Imbalance
Hypoglycemia
Metabolic Acidosis
Acute Renal Failure
Hemolytic Uremic Syndrome (HUS)
CHAPTER 7:
Chronic and Persistent Diarrhea
INTRODUCTION
Definitions
Persistent Diarrhea
Chronic Diarrhea
Malabsorption
Protracted Diarrhea
CHRONIC DIARRHEA
Epidemiology
Evaluation of a Child with Chronic Diarrhea
Investigations
General
Specific Tests
Specific Etiology
Prognosis and Outcome
PERSISTENT DIARRHEA
CHAPTER 8:
Celiac Disease
DEFINITION
HISTORICAL BACKGROUND
EPIDEMIOLOGY
GENETICS
PATHOGENESIS
CLINICAL FEATURES AND PATTERNS OF DISEASE
Clinical Features
Patterns of Disease
Classical Celiac Disease
Nonclassical Celiac Disease
INVESTIGATIONS (FLOW CHART 8.1)
Serological Tests
Antigliadin Antibodies (AGA)
Anti-tissue Transglutaminase (tTG) and Anti-endomysial Antibodies (EMA)
HLA Typing
Endoscopy and Histology
SCREENING FOR CELIAC DISEASE
Immunoglobulin A (IgA) Deficiency
Dermatitis Herpetiformis
When to Screen Asymptomatic but at Risk Groups
Categories of Celiac Disease Identified on Screening
Asymptomatic Celiac Disease
Subclinical Celiac Disease
Potential Celiac Disease
MANAGEMENT OF CELIAC DISEASE
Acute Management
Long-term Management
Monitoring
Gluten Challenge
PROGNOSIS
COMPLICATIONS OF CELIAC DISEASE
REFRACTORY CELIAC DISEASE
FUTURE RESEARCH
SOURCES OF PATIENT/PARENT INFORMATION
CHAPTER 9:
Inflammatory Bowel Disease
INTRODUCTION
INFLAMMATORY BOWEL DISEASE
Genetic Susceptibility
Microbial Factors
Immune Factors9
Environmental Factors9
Clinical Manifestations
Crohn‘s Disease
Investigation10
Imaging Studies
Ulcerative Colitis
Investigations
Endoscopy16,20
Imaging Study
Severity of Disease
Activity Index
Nutritional Management27–30
Pharmacological Treatment of IBD
5-Aminosalicylic Acid Compound (ASA)31-34
Corticosteroid28,34,41
Immunomodulators
Antibiotics15,16
Biological Drugs41-50
Surgery51-54
Other Support
CHAPTER 10:
Disorders of the Pancreas
CONGENITAL ANOMALIES
Pancreas Divisum
Annular Pancreas
Ectopic Pancreas (Pancreatic Rests/Heterotopic Pancreas)
GENETIC DISORDERS
Cystic Fibrosis11
Shwachman-Diamond Syndrome
Johanson-Blizzard Syndrome (JBS)
Pearson’s Marrow-Pancreas Syndrome
Jeune’s Syndrome (Asphyxiating Thoracic Dystrophy/Thoracic-Pelvic-Phalangeal Dystrophy)
ACUTE PANCREATITIS
Protective Mechanisms
Etiology
Atlanta Classification of Pancreatitis21
Clinical Presentation
Diagnosis
Serum Lipase
Serum Amylase
Role of Imaging
USG Findings
Nonstandard Imaging Tests
Medical Management of Acute Pancreatitis
Nutritional Support
Metabolic Complications
Local Complications
Surgical Management of Acute Pancreatitis
CHRONIC PANCREATITIS
Etiology of Chronic Pancreatitis
Genetic Causes
Metabolic Causes
Autoimmune
Obstructive
Miscellaneous
Genetic Causes of Chronic Pancreatitis
Clinical Features
Pain
Weight Loss
Malabsorption
Diagnosis
Management
Pain Relief
Pancreatic Insufficiency
Endoscopic and Surgical Treatment
Juvenile Tropical Pancreatitis43
TUMORS OF PANCREAS
Multiple Endocrine Neoplasia
Other Tumors
CHAPTER 11:
Short Bowel Syndrome
ETIOLOGY AND INCIDENCE
Intestinal Atresia
Gastroschisis
Intestinal Aganglionosis
Malrotation and Midgut Volvulus
Necrotizing Enterocolitis
PATHOPHYSIOLOGY
Consequences of Intestinal Resection
Resection of the Jejunum
Resection of the Ileum
Ileocecal Valve Resection
Resection of the Colon
Associated Disorders
Adaptation after Extensive Intestinal Resection
MANAGEMENT
Initial Surgery
Medical Therapy
Parenteral Nutrition
Enteral Feeding
Risks and Consequences of Microbiological Disorders
Hormonal Therapy and Other Adaptive Treatments
Nontransplant Surgery
LONG-TERM OUTCOME AND GROWTH OF PATIENTS
CONCLUSION
CHAPTER 12:
Surgical Disorders of the Gastrointestinal Tract
ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA
Classification (Gross 1953)3 (Fig. 12.1)
Clinical Presentation1
Associated Anomalies4-6
Diagnosis1
Treatment1,6
Preoperative Management
Operative Management
Outcome7,8
Complication after Surgery1
INFANTILE HYPERTROPHIC PYLORIC STENOSIS
Epidemiology
Pathogenesis
Clinical Presentation9
Diagnosis
Treatment
Preoperative Management9
Surgical Management
DUODENAL ATRESIA
Etiology
Incidence
Associated Conditions
Clinical Presentation21,22
Diagnosis23
Management24
Duodenoduodenostomy
ILEAL AND JEJUNAL ATRESIA
Classification of Intestinal Atresia (Grosfeld)26 [Fig. 12.8]
Associated Conditions
Clinical Presentation27,28
Diagnosis29
Management26
MECONIUM ILEUS
Clinical Presentation31
Imaging Studies
Management
Nonoperative Treatment
Surgical Treatment
Postsurgical Management31
MALROTATION OF GUT
Clinical Presentation39
Diagnosis37,40
Surgical Management41
ABDOMINAL WALL DEFECTS (OMPHALOCELE AND GASTROCHISIS)
Omphalocele
Gastroschisis
Embryology43
Associated Anomalies42
Omphalocele
Gastroschisis
Management
Surgical Treatment42,44,45
HIRSCHSPRUNG’S DISEASE
Etiology46,48
Clinical Presentation
Neonatal Obstruction
Chronic Constipation
Enterocolitis
Diagnosis
Radiological Examination
Rectal Biopsy
Management46
Definitive Treatment
Complications of the Disease and Treatment
ANORECTAL MALFORMATIONS
Incidence
Associated Malformations (50–60%)55
Classification
Male Defects58(12.21)
Female Defects58 (Fig. 12.22)
Imaging59
Management
Colostomy60
Perineal Procedures58
Definitive Surgery
Posterior Sagittal Anorectoplasty61
Anterior Sagittal Anorectoplasty62
Sacroperineal Pull-through63
Laparoscopic-assisted Pull-through64
Complications65
ACUTE EMERGENCIES
APPENDICITIS
Pathophysiology66
Clinical Features66
Clinical Examination66
Investigations
Ultrasonography68
Computed Tomography (CT)69
Treatment
Appendectomy—Open or Laparoscopic70
Complications66
INTUSSUSCEPTION
Incidence and Demographics75,76
Types77
Lead Points
Clinical Findings and Physical Examination80-82
Diagnostic Evaluation
Ultrasonography (US)83
Contrast Enema84
Treatment
Radiological Reduction
Hydrostatic Reduction87,88
Operative Management81,86
MECKEL’S DIVERTICULUM
Features (“Rule of 2”)92
Clinical Features92
Investigations
Management95
INTESTINAL OBSTRUCTION
Clinical Features97
Diagnosis
Radiological Imaging
Contrast Enema99
Treatment of Acute Intestinal Obstruction96
Principles of Surgical Intervention for Obstruction
INFLAMMATORY BOWEL DISEASE
Surgical Options
Ulcerative Colitis
Indications for Surgery107
Operations
Proctocolectomy and Ileostomy108
Ileostomy with a Continent Intra-abdominal Pouch (Kock’s Procedure)109
Restorative Proctocolectomy with an Ileoanal Pouch (Parks)108
RARER ISSUES AND RECENT ADVANCES
ANNULAR PANCREAS
Clinical Presentation
Treatment
PANCREAS DIVISUM112-114
Incidence
Investigations
Pathology
Treatment
GASTROESOPHAGEAL REFLUX DISEASE
Risk Factors
Clinical History116
Investigations116
Treatment
Medical Therapy
Surgical Treatment117-119
Surgical Technique
Complications116
BARIATRIC SURGERY
Definition
Recommendations for Patient Selection127
Surgical Options
Roux-en-Y Gastric Bypass128,129
Vertical-banded Gastroplasty and Adjustable Gastric Banding130
Sleeve Gastrectomy131
Biliopancreatic Diversion with or without Duodenal Switch132
SECTION 2: NUTRITION
CHAPTER 13:
Nutritional Assessment in Health and Disease.
INTRODUCTION
GOALS
GUIDING PRINCIPLES
METHODS OF NUTRITION ASSESSMENT
CLINICAL EXAMINATION
Protein–Energy Malnutrition (PEM)
Kwashiorkor
Marasmus
Marasmic Kwashiorkor
Vitamin A Deficiency
Nutritional Anemia
Iodine Deficiency Disorders (IDD)
Vitamin B Complex Deficiency
Riboflavin Deficiency
Thiamine Deficiency and Beriberi
Pellagra
Rickets
Scurvy
Fluorosis
NUTRITIONAL ANTHROPOMETRY
Anthropometric Measurements
Body Weight
Height
Measurement of Standing Height
Measurement of Recumbent Length
Mid-upper Arm Circumference
Fat Fold at Triceps
Age Assessment
Reference Standards
Anthropometric Indicators
Underweight
Stunting
Wasting
Overweight
Body Mass Index
Interpretation of Data
Percentile Chart
Z-scores
Percent of Expected Measurement
BIOCHEMICAL ASSESSMENT
DIETARY ASSESSMENT
Household Level
Weighment Method
Family Questionnaire Method
Individual Label
Duplicate Sample or Chemical Analysis
CHAPTER 14:
Food Allergy
INTRODUCTION
TERMINOLOGY AND DEFINITIONS
INCIDENCE
IMMUNOLOGICAL BASIS OF FOOD ALLERGY
PATHOPHYSIOLOGY OF FOOD ALLERGY
IMMUNOPATHOGENESIS OF FOOD ALLERGIES
IgE-mediated Reactions
Non-IgE-mediated (T Cell-mediated) Reactions
SYMPTOMS ASSOCIATED WITH FOOD HYPERSENSITIVITY
CLINICAL PHENOTYPES/REACTIONS SEEN IN FOOD ALLERGY
FOOD ALLERGY TO SPECIFIC FOODS
COW’ S MILK ALLERGY/COW’ S MILK PROTEIN ALLERGY (CMA/CMPA)
NATURAL HISTORY OF FOOD ALLERGY
Diagnosis of Food Allergy Presenting with Gastrointestinal Manifestations
History
Laboratory Tests12
A Practical and Simple Diagnostic Approach to Gastrointestinal Allergy
Management
Medical Position Paper by the Espghan on Diagnosis and Management of CMPA14
Prevention of Food Allergy
DESCRIPTIVE CASE SCENARIOS IN FOOD ALLERGY
CONCLUSION
CHAPTER 15:
Childhood Malnutrition
INTRODUCTION
Management of Severe Acute Malnutrition: Combining Facility-based and Community-based Care
GUIDELINES FOR THE MANAGEMENT OF CHILDREN WITH SAM
Phases of Management of SAM
Acute Phase
Nutritional Rehabilitation Phase
Follow-up
Treatment of Complications/Associate Illnesses
COMMUNITY-BASEDMANAGEMENT OF SAM
MICRONUTRIENT DEFICIENCIESIN CHILDREN
IRON DEFICIENCY
VITAMIN A DEFICIENCY
ZINC DEFICIENCY
IODINE DEFICIENCY
CHAPTER 16:
Pediatric Enteral Nutrition
INTRODUCTION
AMERICAN SOCIETY OF PARENTERAL AND ENTERAL NUTRITION RECOMMENDATIONS
Practice Guidelines
Malnutrition and its Consequences
GENERAL PRINCIPLES OF PEDIATRIC ENTERAL NUTRITION
INDICATIONS FOR ENTERAL NUTRITION
PEDIATRIC ENTERAL NUTRITION FORMULATIONS
Formulations for Infants and Young Children with Cow’s Milk Protein Allergy
ENTERAL NUTRITION DELIVERY—ACCESS TECHNIQUES AND DEVICES
Noninvasive Techniques of Enteral Access
Invasive Techniques of Enteral Access
COMPLICATIONS ASSOCIATED WITH ENTERAL FEEDING
CHAPTER 17:
Parenteral Nutrition
INTRODUCTION
PREFERRED ROUTE OF NUTRIENT DELIVERY
TOTAL PARENTERAL NUTRITION AND PARTIAL PARENTERAL NUTRITION
ROUTE OF ADMINISTRATION
INDICATIONS FOR PARENTERAL NUTRITION
SOURCES OF ENERGY
CARBOHYDRATE REQUIREMENT
PROTEIN REQUIREMENT
What Happens if Lipid is not Given?
Electrolytes
Are Micronutrients Required in PICU Patients?
Vitamins, Minerals and Trace Elements
Administration of Multivitamins in Children 12
Practical Aspects of Multivitamin Administration in Children
Trace Elements
Manganese (Mn)
Chromium (Cr)
Iron
Copper
Zinc
Is Anything Else We Should Add to PN?
Carnitine
Cysteine
Selenium
Fluid Requirements
Complications with the Use of PN
Known Causes of Risk Factors of Parenteral Nutrition Associated Cholestatis (PNAC)12-14
Prevention and Treatment of PNAC12-14
Practice Guidelines for Administration of PN12-14,53
How Should We Monitor Infants Receiving PN?
How Should We Wean PN ?
TOTAL NUTRIENT ADMIXTURE (TNA)
Parenteral Nutrition Admixtures: Advantages and Drawbacks (Table 17.18)
CONCLUSION
ACKNOWLEDGMENTS
CONFLICTS OF INTEREST
CHAPTER 18:
Probiotics in Gastrointestinal Disorders
INTRODUCTION
DEFINITIONS
MECHANISMS OFACTION OF PROBIOTICS
Characteristics and Dose
STRAINS WITH DOCUMENTED EFFICACY
Acute Diarrhea
Persistent Diarrhea
Diarrhea Prevention
Necrotizing Enterocolitis
Helicobacter pylori Eradication
Infantile Colic
Functional Constipation
Irritable Bowel Syndrome
Inflammatory Bowel Diseases
SECTION 3: HEPATOLOGY
CHAPTER 19:
Anatomy, Development and Physiology of the Hepatobiliary System
INTRODUCTION
FUNCTIONAL ANATOMY: SECTORS AND SEGMENTS
Couinaud Classification (Fig. 19.1)
The Numbering of Segments
ANATOMY OF THE BILIARY TRACT AND BILE DUCT EPITHELIAL CELLS
DEVELOPMENT OF HEPATIC VASCULATURE
LIVER PHYSIOLOGY INCLUDING HEPATOCELLULAR FUNCTION
The Liver as Metabolic Organ—the Liver as Endocrine and Exocrine Liver
The Liver Filter
The Liver as Hematopoietic Organ
HEPATOCYTE DEATH AND REGENERATION
CHAPTER 20:
Approach to Common Liver: Symptoms and Signs
INTRODUCTION
JAUNDICE
Definition 1
Bilirubin Metabolism
Definition of Neonatal Cholestasis
Jaundice in Infants and Older Children
Complications of Cholestasis
Evaluation
Management of Cholestatic Jaundice3,4
PRURITUS
Definition
Pathogenesis
Bile Acids (BA)
Endogenous Opioids
Histamine
Serotonin
Lysophosphatidic Acid and Autotaxin
Evaluation
Treatment
Rifampin
Phenobarbital
Ursodeoxycholic Acid
Opioid Antagonists
Antihistamine
Bile-binding Resins
Other Medical Therapeutic Options
HEPATOMEGALY
Pathophysiology
Evaluation
SPLENOMEGALY
Definition
Spleen’s Functions
Normal Spleen Size 26
Prevalence
Signs of Abnormal Splenomegaly
Causes of Splenomegaly (Table 20.3):S-P-L-E-E-N 28
Evaluation
Complications of Splenomegaly
Management
When to Refer to Specialist
ASCITES
Definition
Pathogenesis
Etiology
Evaluation29,31,32
Paracentesis Location
Treatment30,32
TRANSAMINITIS
Definition
Note
Differential Diagnosis
Evaluation
Management
UPPER GASTROINTESTINAL BLEEDING
Definition
Epidemiology34,35
Presentation34,35
Causes of UGIB
Portal Hypertension Related UGIB
Variceal Bleeding
Portal Hypertensive Gastropathy
Evaluation
Management34,35
Endoscopic Intervention36,37
CHAPTER 21:
Interpretation of Liver Function Tests
INTRODUCTION
USES
LIMITATION5
CLASSIFICATION OF LFT
TESTS OF THE LIVER’S CAPACITY TO TRANSPORT ORGANIC ANIONS
Serum Bilirubin
Diagnostic Value of Bilirubin Levels
Hyperbilirubinemia Due to Hemolysis
Drugs
Prognostic Value of Bilirubin Levels
Urine Bilirubin
Urobilinogen
Bile Salts
Functions of Bile Salts
Inborn Error of Bile Acid Synthesis
TESTS FOR HEPATOCYTIC NECROSIS
Enzymes that Detect Hepatocellular Dysfunction
Aminotransferases
Mild, Moderate, and Severe Elevations of Aminotransferases
AST : ALT Ratio
Mitochondrial AST : Total AST Ratio
Other Enzymes of Hepatocellular Necrosis
Lactate Dehydrogenase (LDH)
Enzymes that Detect Cholestasis
Alkaline Phosphatase (ALP)
Elevated ALP
Low ALP
γ Glutamyl transpeptidase
Other Enzymes that Detect Cholestasis
TESTS OF THE LIVER’S BIOSYNTHETIC CAPACITY
Serum Proteins
Albumin
Prealbumin
Serum Ceruloplasmin
Procollagen III Peptide
α1 Antitrypsin
α Fetoprotein
Prothrombin Time (PT)
Liver Biopsy
Prerequisites for Liver Biopsy
Indications of Liver Biopsy
Contraindications
Methods of Liver Biopsy
Percutaneous Liver Biopsy
Transvenous Liver Biopsy
Laparoscopic and Surgical Biopsies
Fine Needle Aspiration (FNA)
Limitations and Complications
Noninvasive Tests of Liver Disease
Specific Tests
Noninvasive Tests
Direct Serological Markers of Fibrosis
Indirect Serological Markers of Fibrosis
Imaging
Conventional Imaging Studies
Elastography
Ultrasound Elastography
Magnetic Resonance Elastography (MRE)
Limitation
CONCLUSION
CHAPTER 22:
Neonatal Cholestasis
INTRODUCTION
ETIOPATHOGENESIS OF NEONATAL CHOLESTASIS
Physiology of Bile Acid Synthesis and Transport
Differential Diagnoses of Neonatal Cholestasis
APPROACH TO AN INFANT WITH CHOLESTASIS
HISTORY
PHYSICAL EXAMINATION
A DIAGNOSTIC APPROACH
Liver Function Test, Including Gamma Glutamyltranspeptidase (Ggt) Level
Gamma-glutamyltranspeptidase (Gamma-GT)
Albumin Level, Coagulation Profile
Full Blood Count/Complete Blood Count
Second-line Investigations
RADIOLOGICAL INVESTIGATIONS
Ultrasound Scan of the Hepatobiliary System
Hepatobiliary Iminodiacetic Acid Scan (HIDA) Scan
Cholangiography in Defining Biliary Anatomy
Histopathological Investigations
GENERAL PRINCIPLES OF MANAGEMENT
APPROACH TO THE PREMATURE INFANT WITH CHOLESTASIS
EXTRAHEPATIC CAUSES OF CHOLESTASIS
Biliary Atresia
Pathophysiology
Diagnostic Approach to Biliary Atresia
Mimics of Biliary Atresia
Surgical Management
Postoperative Complications
Ascending Cholangitis
Progressive Liver Disease
Medical Management
Promoting Biliary Drainage
Nutritional Support
Choledochal Cyst
Inspissated Bile Syndrome
Spontaneous Perforation of the Bile Duct
Intrahepatic Causes of Cholestasis
Infectious Causes
Endocrinopathies
Drug Effect/Toxic Effect
FAMILIAL (INHERITED) CAUSES OF INTRAHEPATIC CHOLESTASIS
Alagille Syndrome (AS)(Arteriohepatic Dysplasia)
Clinical Presentation
Pathogenesis
Diagnosis
Management
Progressive Familial Intrahepatic Cholestasis (PFIC)
PFIC Type 1
Etiopathogenesis
Clinical Features
Management
PFIC Type 2
Etiopathogenesis
Clinical Features
Management
Benign Recurrent Intrahepatic Cholestasis (BRIC) Type 1 and Type 2
Progressive Familial Intrahepatic Cholestasis Type 3
Etiopathogenesis
Clinical Features
Management
Disorders of Bile Acid Biosynthesis
Etiopathogenesis
Clinical Features
Diagnosis
Management
Metabolic Disorders
Alpha-1 Antitrypsin Deficiency
Cystic Fibrosis
Galactosemia/Tyrosinemia
Citrin Deficiency
Neonatal Hematochromatosis
Etiopathogenesis
Management
SUMMARY
CHAPTER 23:
Acute Hepatitis
INTRODUCTION
ETIOLOGY
EPIDEMIOLOGY
ETIOPATHOGENESIS
Clinical Manifestations of AVH
Hepatitis A
Hepatitis E
Hepatitis B
Hepatitis C
Hepatitis D
ATYPICAL MANIFESTATIONS AND COMPLICATIONS OF ACUTE VIRAL HEPATITIS
Diagnosis
Biochemical Investigations
Serological Tests
Acute HDV Infection
DIFFERENTIAL DIAGNOSIS
Nonhepatotropic Viruses
Epstein Barr Virus
Measles Virus
Cytomegalovirus
Parvovirus B19
Herpes Simplex 1 and 2
Dengue Virus
Human Herpes Virus-6
Varicella Zoster
Human Immunodeficiency Virus
Severe Acute Respiratory Syndrome-Coronavirus
Exotic Viruses
Infective Nonviral Causes: Bacterial Hepatitis
Salmonella
Bacterial Sepsis
Tuberculosis
Brucellosis
Leptospirosis
Scrub Typhus
Infective Nonviral Causes: Protozoal Hepatitis
Malaria
Noninfective Causes
Drug Induced Liver Injury
Autoimmune Hepatitis (AIH)
Obstructive Jaundice
Glycogen Storage Disease
Wilson’s Disease
Hemophagocytic Lymphohistiocytosis (HLH)
MANAGEMENT OFACUTE VIRAL HEPATITIS
Supportive Therapy
Specific Therapy
Acute Hepatitis A and E
Acute Hepatitis B
Acute Hepatitis C
Acute Hepatitis D
TREATMENT FOR ACUTE HEPATITIS DUE TO OTHER NONHEPATOTROPIC VIRUSES
Prevention
Hepatitis A
Hepatitis B
Hepatitis C and D
SUMMARY
CHAPTER 24:
Chronic Viral Hepatitis
INTRODUCTION
CLINICAL FEATURES OF CHRONIC VIRAL HEPATITIS
HEPATITIS B
Epidemiology
Virus Characteristics
Hepatitis B Virus Genotypes
Mutations of the Hepatitis B Virus Genome
Hepatitis B Surface Antigen Mutants
Mutations in the Pre-core, Basal Core Promoter and Core Genes
Hepatitis B Virus DNA Polymerase Mutants
Pathogenesis
Perinatal Transmission
Diagnosis
Treatment
Initial Management4
Important Definitions in Management (Table 24.2)
Treatment Endpoints
Factors Influencing the Choice of Agents
Guidelines for the Management of Hepatitis B
Antiviral Agents
Newer Drugs
Prevention
Passive Immunization
Active Immunization
Preventing Perinatal Transmission
HEPATITIS C
Viral Characteristics
Genotypes
Epidemiology
Perinatal Transmission
Pathogenesis
Natural History of HCV Infection in Infants and Children
Diagnosis
Serologic Assay
HCV Genotyping
Treatment
Prevention
CHAPTER 25:
Autoimmune hepatitis
INTRODUCTION
EPIDEMIOLOGY
GENETICS
PATHOGENESIS
Molecular Mimicry
TYPES
CLINICAL PATTERNS OF PRESENTATION
NATURAL HISTORY
AUTOANTIBODIES
Antinuclear Antibody (ANA)
Smooth Muscle Antibody (SMA)
Liver Kidney Microsomal Type I Antibody
Anti-LC-1
Anti-SLA/LP
Peripheral Antineutrophil Cytoplasmic Antibody (pANCA)
Antibodies to the Asialoglycoprotein Receptor (Anti-ASGP-R)
Antibodies to Saccharomyces Cerevisiae (ASCA)
Antimitochondrial Antibody (AMA)
LIVER BIOPSY
IMAGING
DIAGNOSTIC CRITERIA
TREATMENT
STANDARD TREATMENT
Corticosteroids
Prednisolone
Budesonide
Azathioprine
Mycophenolate Mofetil
Cyclosporine
Tacrolimus
TREATMENT DECISIONS: WHEN TO START TREATMENT
Monitoring
When to Stop Treatment
RELAPSE
TREATMENT FAILURE
PROGNOSIS
TRANSPLANTATION
POST-TRANSPLANT RECURRENT AIH
POST LIVER TRANSPLANTATION DE NOVO AIH
AUTOIMMUNE SCLEROSING CHOLANGITIS (ASC)
OVERLAP SYNDROME
MANAGEMENT
CONCLUSION
CHAPTER 26:
Wilson’s Disease
INTRODUCTION
PATHOGENESIS AND METABOLISM OF COPPER
PATHOGENESIS
CLINICAL FEATURES
DIAGNOSIS
Family Screening for Wilson’s Disease
Acute Liver Failure and Wilson’s Disease
TREATMENT
Liver Transplantation and Wilson’s Disease
CHAPTER 27:
Metabolic Disorders in the Infant
INTRODUCTION
METABOLIC LIVER DISEASE1
Prolonged Neonatal Jaundice
Classical Galactosemia2,3
Investigation
Treatment
Prognosis
Hereditary Tyrosinemia Type 1 (HT1)
Diagnosis and management4
Treatment
Outcome
Citrin Deficiency/Citrullinemia Type II5-7
Investigations
Treatment
Outcome
Niemann-Pick Disease Type C8
Investigations
Treatment
Outcome
Wolman Disease (Lysosomal Acid Lipase (Lal) Deficiency)9-11
Investigations
Treatment
Outcome
Zellweger Syndrome12
Investigations
Treatment
Outcome
Congenital Disorders of Glycosylation (CDG)13
Investigations
Treatment
ACUTE LIVER FAILURE
Hereditary Fructose intolerance (HFI) (Aldolase B deficiency)
Investigations
Treatment
Outcome
Mitochondrial Liver Disease14,15
Investigations
Treatment
Defects of Long Chain Fatty Acid metabolism (LCFAODs)16,17
Disorders of Transport across the Mitochondrial Membranes
Long-chain Fatty Acid Oxidation Defects (LCFAODS)
Investigations
Treatment
Outcome
ISOLATED ORGANOMEGALY OR WHEN ASSOCIATED WITH OTHER CLINICAL FEATURES
Glycogen Storage Disorders (GSDs/Hepatic Glycogenoses)
Glycogen Storage Disease Type Ia (Glucose-6-Phosphatase Deficiency)18
Investigations
Treatment
Glycogen Storage Disease Type III (GSDIII) (Glycogen Debrancher Deficiency)19
Investigation
Treatment
Glycogen Storage Disease type IV (Glycogen Brancher Deficiency)20
Investigation
Treatment
Prognosis for Hepatic GSDs
Mucopolysaccharidoses (MPS)21
Investigations
Treatment
Outcome
Gaucher Disease (Glucocerebrosidase Deficiency)22
Investigations
Treatment
Prognosis
Niemann-Pick Disease A and B (NPC A and B)23
Investigations
Treatment
Prognosis
Other Disorders
Urea Cycle Enzyme Defects (UCEDs)
Investigations
Treatment
Outcome
HYDROPS FETALIS
CONCLUSION
CHAPTER 28:
Metabolic Disorders in the Older Child
DEFECTS IN BILE ACID SYNTHESIS
PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS (PFIC)— TYPES 1, 2, AND 3
ALAGILLE SYNDROME (AGS)
STORAGE DISORDERS AFFECTING THE LIVER
CYSTIC FIBROSIS
DISORDERS OF HEME METABOLISM AND THE LIVER
CHAPTER 29:
Nonalcoholic Fatty Liver Disease
INTRODUCTION
DEFINITION
EPIDEMIOLOGY
RISK FACTORS
NATURAL HISTORY AND PATHOGENESIS
SYMPTOMS
HISTOPATHOLOGY AND SCORING SYSTEMS
ULTRASONOGRAPHY
COMPUTED TOMOGRAPHY/MAGNETIC RESONANCE IMAGING
TRANSIENT ELASTOGRAPHY
TREATMENT
CHAPTER 30:
Chronic Liver Disease
INTRODUCTION
DEFINITION
CAUSES OF CHRONIC LIVER DISEASE OTHER THAN NEONATAL CHOLESTASIS SYNDROME
Chronic Hepatitis
Genetic/Metabolic Liver Disease
Copper and Iron Storage Disease
Venous Congestion/Vascular Congestion
Miscellaneous
Nonalcoholic Steatohepatitis
CLINICAL PRESENTATION OF CHRONIC LIVER DISEASE
SIGNS OF CHRONIC LIVER FAILURE
CLINICAL CLUES FOR ETIOLOGY
When to Suspect Inherited Disorders
General Examination
COMPLICATIONS
Portal Hypertension
Variceal Bleed3
Hepatic Encephalopathy
RENAL COMPLICATIONS AND HEPATORENAL SYNDROME2
ASCITES AND SPONTANEOUS BACTERIAL PERITONITIS
EVALUATION
Investigations for Complications
Liver Biopsy
TREATMENT
MANAGEMENT OF VARICEAL BLEED3,8
Medical Therapy
Secondary Prophylaxis
Surgical Modalities
Radiological Method
Management of Ascites
SPONTANEOUS BACTERIAL PERITONITIS
Specific Treatment
DIET IN CHRONIC LIVER DISEASE
Diseases Requiring Dietary Elimination
Liver Diseases Requiring Diet Modification
CHAPTER 31:
Surgical Disorders of the Hepatobiliary System
BILIARY ATRESIA
Etiology
Classification
Diagnosis
Treatment
Outcome
Conclusion
CHOLEDOCHAL CYST
Etiology and Pathogenesis
Clinical Presentation
Diagnosis
Classification and Treatment
Outcome
PORTAL HYPERTENSION
Prehepatic Obstruction
INTRAHEPATIC OBSTRUCTION
Suprahepatic Obstruction
Clinical Presentation
Treatment
CHOLELITHIASIS
Conclusion
LIVER TUMORS
Benign Tumors of the Liver
Infantile Hemangioendothelioma
Treatment
MESENCHYMAL HAMARTOMA
FOCAL NODULAR HYPERPLASIA
HEPATOCELLULAR ADENOMA
MALIGNANT PRIMARY LIVER TUMORS
HEPATOBLASTOMA
UNDIFFERENTIATED EMBRYONAL SARCOMA
HEPATOCELLULAR CARCINOMA
CHAPTER 32:
Drug-induced Liver Injury
INTRODUCTION
EPIDEMIOLOGY
DEVELOPMENT OF DRUG METABOLISM IN LIVER
PATHOGENESIS
DIAGNOSIS OF DILI
COMMONLY INCRIMINATED DRUGS IN CHILDREN
TREATMENT OF DILI
LIVER TRANSPLANTATION IN DILI
CHAPTER 33:
Acute Liver Failure
INTRODUCTION
PATHOGENESIS
Liver Regeneration
Encephalopathy
PATHOLOGY
Recovery
ETIOLOGY
ACUTE LIVER FAILURE IN INFANCY
Hepatitis B
Herpes Simplex
Echovirus
Coxsackievirus
METABOLIC DISEASE
Galactosemia
Clinical Picture
Hepatic Changes
Diagnosis
Prognosis and Treatment
Neonatal Hemochromatosis (NNH)
Disorders of mitochondrial Energy Metabolism
Deficiencies of the Electron Transport Chain Enzyme
Mitochondrial DNA Depletion Syndromes
Tyrosinemia Type I
ACUTE LIVER FAILURE INOLDER CHILDREN
Virus Infection
Hepatitis A Virus (HAV)
Hepatitis B (HBV)
Hepatitis E Virus (HEV)
Nonviral Infectious Hepatitis
Indeterminate Hepatitis (Hepatitis non-A-E)
DRUG AND TOXIN-RELATEDHEPATIC INJURY
Acetaminophen (Paracetamol) Poisoning
Sodium Valproate
Autoimmune hepatitis
Metabolic Diseases
Clinical Manifestations of Acute Liver Failure
Laboratory Investigations
Diagnosis
Complications
Management (Table 33.5)
Prevention and Treatment of Complications
Hepatic Support
SELECTION FOR LIVER TRANSPLANTATION
Liver Transplantation
SUMMARY
CHAPTER 34:
Portal Hypertension
DEFINITION
PATHOPHYSIOLOGY3,5,6
CLASSIFICATION3
ETIOLOGY3,4,7-9,15
Presinusoidal PH
Intrahepatic Sinusoidal PH7-9
Postsinusoidal PH
CLINICAL FEATURES
DIAGNOSIS1-5,8,12
MANAGEMENT OF PORTAL HYPERTENSION IN CHILDREN1-5,8,11-16,18 (FLOW CHART 34.1)
MANAGEMENT OF ESOPHAGEAL VARICES—ENDOSCOPIC THERAPY
Endoscopic Sclerotherapy
Drug Therapy of Portal Hypertension (Guidelines)
Drugs to Stop Bleeding3,4,20
Combined Medical Therapy
Prevention of Recurrent Variceal Bleeding
Prevention of Formation of Varices (“Preprimary” Prevention)20
MAIN INDICATIONS FOR SURGERY3,4,11,12,18
CHAPTER 35:
Liver Transplantation
INTRODUCTION
INDICATIONS
Acute Liver Failure
End-stage Chronic Liver Failure
Pediatric End-stage Liver (PELD) Disease Scoring System
LT for Metabolic Disorders
PRETRANSPLANT EVALUATION
Pretransplant Assessment Guidelines for Recipient
Pretransplant Medical Management
Nutritional Rehabilitation
Immunization
Management of Complications of Chronic Liver Disease
Counseling
TRANSPLANT SURGERY
Technical Variants
Living Related Liver Transplantation
Split-liver Transplantation
Monosegmental Liver Transplantation
Auxiliary Partial Liver Transplantation
Postoperative Course
Immunosuppression
Complications
Retransplantation
Novel Therapies
Looking beyond Immunosuppression: Tolerance
Liver-assist Devices
Hepatocyte Transplantation
Life after Transplantation
Registries and Databases
LIVER TRANSPLANTATION IN INDIA
SUMMARY
CHAPTER 36:
Liver Tumors
INTRODUCTION
HEPATOBLASTOMA
Etiology and Epidemiology
Clinical Features
Investigations
Histology
Staging and Risk Stratification
Treatment
Chemotherapy
SIOPEL Studies
Surgery
Liver Transplant
HEPATOCELLULAR CARCINOMA
Benign Liver Tumors
CHAPTER 37:
Liver in Systemic Illness
INTRODUCTION
CRITICALLY ILL PATIENT
INFECTION
Sepsis
Dengue Hemorrhagic Fever
Typhoid Fever
LIVER IN THE PRESENCE OF ALTERED SYSTEMIC CIRCULATION
Ischemic Hepatitis
Congestive Hepatopathy
HEMATOLOGIC DISORDERS
Hemolytic Anemia
Leukemia
Lymphoma
COLLAGEN VASCULAR DISEASE
Systemic Lupus Erythematosus
Juvenile Rheumatoid Arthritis
NUTRITIONAL DISORDERS
Celiac Disease
Obesity
Total Parenteral Nutrition
IMMUNODEFICIENCY
Primary Immunodeficiencies
Hyper-immunoglobulin M Syndrome
Secondary Immunodeficiencies
HIV Infection
INDEX
TOC
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