Copyright â 2014 Springer Science+Business Media New York
Preface to the First Edition
1:
Anatomy and Development of the Liver
Anatomical Lobes and Ligaments
Blood Supply and Lymphatic Drainage
Cross-Sectional Imaging of the Liver: Anatomical Aspects
Liver Resection: Anatomical Aspects
Paediatric Liver Transplantation: Anatomical Aspects
Portal Hypertension: Anatomical Aspects
2: Normal Functional Biology of the Liver
Sinusoidal Endothelial Cells
Glycogen Synthesis (Glycogenesis)
The Pentose-Phosphate Pathway
Lipogenesis (Fatty Acid Synthesis)
Lipolysis (Fatty Acid Breakdown)
Bilirubin and Bile Acid Metabolism
Bile Acid Metabolism and Transport
Secretion, Bile Flow, and Enterohepatic Circulation
3: Laboratory Assessment of Hepatic Injury and Function
Baseline Investigations: Biochemical Liver Function Tests
Second-Line Investigations
Liver Disease in Older Children
Liver Biopsy and Histopathology
Complications of Percutaneous Liver Biopsy
Endoscopic Retrograde Cholangiopancreatography (ERCP)
Genetic Tests (Chromosome and DNA)
Metabolic Investigations (Table 3.11)
Ophthalmology (Table 3.12)
Skin Biopsy with Fibroblast Culture
4: Mechanisms of Liver Injury
General Structure and Function of the Liver
Inherited Metabolic Disorders
Disorders of Amino Acid Metabolism
Hereditary Tyrosinemia Type 1
Disorders of Carbohydrate Metabolism
Carbohydrate Deficiency Glycoprotein Syndrome
Glycogen Storage Diseases
Hereditary Fructose Intolerance
Disorders of Lipid or Cholesterol Metabolism/Lysosomal Storage Diseases
Cholesterol Ester Storage Disease
Acquired Metabolic Disorders
Nonalcoholic Fatty Liver Disease (NAFLD)
Parenteral Nutrition-Associated Liver Disease
Disorders of Iron Metabolism
Disorders of Copper Metabolism
α 1 -Antitrypsin Deficiency
Immune-Mediated Liver Diseases
Gestational Alloimmune Liver Disease (Neonatal Hemochromatosis)
Primary Biliary Cirrhosis
Primary Sclerosing Cholangitis
Infections Causing Neonatal Cholestasis
Drug-Induced Liver Injury
Disorders of Bile Acid Synthesis
Progressive Familial Intrahepatic Cholestasis
Congenital Hepatic Fibrosis/Caroli Disease
5: Radiology of the Liver in Children
Congenital Neonatal Cholestasis
Diffuse Hepatic Parenchymal Disease
Liver Transplantation Imaging
Image-Guided Hepatic Interventions
6: Phenotypes of Liver Diseases in Infants, Children, and Adolescents
Clinical Assessment: The Importance of a Careful History and an Expert Physical Examination
The Stable Infant with Liver Disease
Young Child with Liver Disease (1
4 Years)
Older Child/Adolescent with Liver Disease (>5 Years)
Acute Liver Disease and Failure
Consult from Other Services with Liver Dysfunction in Association with Known Disease
Why Diagnosing Liver Disease May Be Difficult
7: Psychosocial, Cognitive, and Quality of Life Considerations in the Child with Liver Disease and Their Family
Minimal Hepatic Encephalopathy (MHE)
Psychosocial and HRQOL Outcomes
Psychosocial and HRQOL Outcomes
Psychosocial and HRQOL Outcomes
Inherited Cholestatic Diseases
Psychosocial and HRQOL Outcomes
Psychosocial and HRQOL Outcomes
Psychosocial and HRQOL Outcomes
Psychosocial and HRQOL Outcomes
Psychosocial and HRQOL Outcomes
Implications for Practice and Research Opportunities
8: Metabolic Liver Disease: Part 1
Disorders of Bilirubin Metabolism
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Disorders of Carbohydrate Metabolism
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Hereditary Fructose Intolerance
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Fructose-1,6-Bisphosphatase Deficiency
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Glycogen Storage Diseases
GSD I (Von Gierke Disease)
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
GSD III (Forbes Disease, Cori Disease, Glycogen Debrancher Deficiency)
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
GSD IV (Andersen Disease, Glycogen Branching Enzyme Deficiency)
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Disorders of Amino Acid and Organic Acid Metabolism
Treatment, Management, and Outcomes
Methylmalonic Acid, Isovaleric Acid, HMG-CoA Lyase, and Maple Syrup Urine Disease
Treatment, Management, and Outcomes
Fatty Acid Oxidation Defects
MCAD (Medium-Chain Acyl-CoA Dehydrogenase) Deficiency
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
LCHAD (Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase) Deficiency
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
CACT (Carnitine-Acylcarnitine Translocase) Deficiency
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
HMG (3-Hydroxy-3-Methylglutaryl) CoA Synthase Deficiency
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Treatment, Management, and Outcomes
Bile Acid and Biliary Transport Defects
Biliary Transport Defects
Treatment, Management, and Outcomes
Inborn Errors of Bile Acid Metabolism
Treatment, Management, and Outcomes
Lysosomal Storage Disorders
Treatment, Management, and Outcome
Lysosomal Acid Lipase Deficiency (Wolman Disease and Cholesteryl Ester Storage Disease) (CESD)
Management, Outcome, and Prognosis
Management, Outcome, and Prognosis
Treatment, Management, and Outcomes
Mitochondrial Hepatopathies
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Alpers-Huttenlocher Syndrome
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
Clinical Manifestations and Diagnosis
Treatment, Management, and Outcomes
9: Metabolic Liver Disease: Part 2
Alpha-One Antitrypsin Deficiency
Function of Alpha-1 Antitrypsin
Structure of Alpha-1 Antitrypsin
Biosynthesis and Regulation
Central Nervous System Manifestations
Ophthalmologic Manifestations
Ophthalmologic Evaluation
Screening of Asymptomatic Relatives
Natural History and Clinical Outcome
Cystic Fibrosis Liver Disease
Cystic Fibrosis-Related Cirrhosis with and Without Portal Hypertension
Portal Hypertension Without Cirrhosis
Complications of Cirrhosis with Portal Hypertension
Liver Involvement Without Cirrhosis or Portal Hypertension
Elevations in AST, ALT, and/or GGT
Acoustic Transient Elastography
Screening for Liver Disease
Acoustic Radiation Forced Impulse Imaging
Management of CF Cirrhosis with Portal Hypertension
Esophageal and Gastric Varices
Hemochromatosis and Hemosiderosis
Physiology and Pathophysiology of Iron Overload
Genetics of Hereditary Hemochromatosis
Approach to the Child with a Parent with HHC
Iron Overload in Children with Liver Dysfunction
Juvenile Hemochromatosis (OMIM 602390)
10: Neonatal Hemochromatosis and Gestational Alloimmune Liver Disease
Etiology and Pathogenesis of NH
Clinical Presentation and Findings in NH and GALD
Clinical Manifestations and Management
Duct Paucity and Hepatic Histopathology
Liver Transplantation in ALGS
Indications for Liver Transplantation
Preoperative Assessment for Liver Transplantation
Facial Features and Skeletal and Ophthalmologic Involvement
Diagnostic Considerations
Prognosis of Alagille Syndrome
12: Idiopathic Neonatal Hepatitis and Its Differential Diagnoses
Neonatal Acute Liver Failure
Transient Neonatal Cholestasis
Chronic Neonatal Cholestasis
Diagnostic Approach (Fig. 12.4)
Etiology and Pathogenesis of Biliary Atresia
Screening for Cholestatic Infants and Early Identification of Biliary Atresia
Management for Biliary Atresia
Surgical Management for Biliary Atresia
Postoperative Complications and Management
Prognosis After Kasai Operation
14: Choledochal Cysts and Fibrocystic Diseases of the Liver
Normal Biliary Development and Cholangiocyte Biology
Congenital Hepatic Fibrosis
Caroli Disease/Caroli Syndrome
Physical Examination (PE)
15: Infections of the Liver
Bacterial Infection Involving the Liver
Gram-Positive and Gram-Negative Infections
Other Gram-Negative Infections
Parasitic Infections of the Liver
Fungal Infections of the Liver
Viral Infections of the Liver
Infectious Complications in Liver Transplantation
16: Autoimmune Hepatitis and Sclerosing Cholangitis
Epidemiology and Genetic Predisposition
Definition of Remission/Relapse
Treatment of Refractory Cases
Duration of Treatment and Prognosis
Autoimmune Sclerosing Cholangitis
De Novo Autoimmune Hepatitis After Liver Transplantation
17: Parenteral Nutrition-Associated Liver Disease in Pediatric Patients: Strategies for Treatment and Prevention
Manganese, Copper, and Aluminum
Non-pharmacologic Management Strategies
Cycled Parenteral Nutrition
Early Initiation of Enteral Feeding
Alternative Lipid Strategies
Use of Fish Oil-Based IVFE
Composite Intravenous Fat Emulsion Formulation
Prevention and Treatment of Sepsis
Autologous Intestinal Reconstruction Surgery
18: Nonalcoholic Fatty Liver Disease and Nonalcoholic Steatohepatitis
Adipokines and Inflammation
Screening Comorbidities of NAFLD
Antioxidant and Cytoprotective Agents
Drugs Targeting Insulin Resistance
19: Drug-Induced Liver Injury in Children: A Structured Approach to Diagnosis and Management
Suspicion of Drug-Induced Liver Injury
Clinical History (Table 19.1)
DILI: Do the Biochemical Data Fit the Pattern?
Medications and Patterns of Injury in DILI (Table 19.4)
Metabolism Characteristics
20: Gallstone Disease in Children
Epidemiology/Risk Factors
Total Parenteral Nutrition
Asymptomatic Cholelithiasis
Symptomatic Cholelithiasis
Gallstone-Related Complications
Acute Hydrops of the Gallbladder
Sphincter of Oddi Dysfunction
21: Vascular Liver Disease
Extrahepatic Portal Vein Obstruction
Clinical Presentation and Natural History
Acute Portal Vein Thrombosis
Chronic Portal Vein Thrombosis
Sinusoidal Obstruction Syndrome (Hepatic Veno-occlusive Disease)
Budd-Chiari Syndrome (Hepatic Venous Outflow Obstruction)
Idiopathic Non-cirrhotic Portal Hypertension
Definition of Idiopathic Non-cirrhotic Portal Hypertension
Congenital Vascular Malformations
Arteriovenous Malformation (AVM)
Liver Involvement in Hereditary Hemorrhagic Telangiectasia
Treatment of Liver Manifestations
Congenital Portosystemic Shunts
Infantile Hepatic Hemangiomas (IHH)
Hepatic Epithelioid Hemangioendothelioma
Infantile Hepatic Hemangioma (IHH)
Classification of Hepatic Hemangiomas [ 7 ]
Mesenchymal Hamartoma (MH)
Focal Nodular Hyperplasia (FNH)
Hepatocellular Adenoma (HA)
Nodular Regenerative Hyperplasia (NRH)
Hepatocellular Carcinoma (HCC)
Fibrolamellar Carcinoma (FLC)
Embryonal (Undifferentiated) Sarcoma of the Liver (ESL)
Epithelioid Hemangioendothelioma (EHE)
Hepatobiliary Rhabdomyosarcoma
Criteria for Acute Liver Failure
Immune/Inflammatory Dysregulation
Systemic Inflammatory Response Syndrome (SIRS)
Macrophage Activation Syndrome (MAS)
Infants Less Than 3 Months
Membrane Adsorbent Recirculating System (MARS)
Plasmapheresis/Plasma Exchange
Disease Severity Assessment
24: Intensive Care Management of Children with Liver Failure
Complications of End-Stage Liver Disease
Treatment of Hepatic Encephalopathy
Acute Kidney Injury/Hepatorenal Syndrome
Spontaneous Bacterial Peritonitis (SBP)
Hematologic Complications/Coagulopathy
Hepatopulmonary Syndrome (HPS)
Portopulmonary Hypertension
Extracorporeal Liver Support Systems
Acute Versus Chronic Liver Failure
ICU Care Following Liver Transplantation
Liver Transplantation Complications
Intra-abdominal Compartment Syndrome
Infections in the Immediate Posttransplant Period
Infections in the Late Posttransplant Period
Complications of Immunosuppression
25: Chronic Liver Disease, Cirrhosis, and Complications: Part 1 (Portal Hypertension, Ascites, Spontaneous Bacterial Peritonitis (SBP), and Hepatorenal Syndrome (HRS))
Causes of Portal Hypertension in Children (Table 25.1)
Clinical Features (Table 25.2)
Management of Portal Hypertension
Management of Ascites (Box 25.1)
Box 25.1. Management of Ascites
Spontaneous Bacterial Peritonitis
Pathophysiology and Microbiology
Clinical Features and Diagnosis
Clinical Features and Diagnosis
26: Chronic Liver Disease, Cirrhosis and Complications: Part 2: Hepatic Encephalopathy and Other Systemic Effects
Clinical Features of Hepatic Encephalopathy
Pathogenesis of Hepatic Encephalopathy
Role of Ammonia in Hepatic Encephalopathy
Role of Neurotoxins and Altered Neurotransmitters in Hepatic Encephalopathy
Inflammatory Mediators in Hepatic Encephalopathy
Critical Flicker Frequency
Neuropsychological Assessment
Management of Hepatic Encephalopathy
Assessment and Stabilisation
Reducing the Ammonia Load
Enhancing Alternative Pathways of Nitrogen Excretion
l -Ornithine l -Aspartate (LOLA)
Drugs of Doubtful Benefit
Extracorporeal Liver Support
Neurological Complications in CLD
Portopulmonary Hypertension
27: Nutrition in Children with Liver Disease: Evaluation and Management
The Role of the Healthy Liver in Nutrition Homeostasis
Chronic Liver Disease and Nutrition
Goals of Nutritional Therapy in Children with Liver Disease
Nutritional Assessment of the Children with Chronic Liver Disease
Anthropometric Evaluation
Nutritional Deficiencies in Children with Chronic Liver Disease
Fat and Essential Fatty Acids
The Role of Diet in the Treatment of Serious Complications of Liver Disease
The Bridge to Transplantation
28: Liver Transplantation
Biliary Atresia (See Also Chapter 13)
Inborn Errors of Metabolism
Other Cholestatic Liver Disease
Fulminant Hepatic Failure
Contraindications to Liver Transplantation
Immunizations Prior to Transplantation
Assessment and Treatment of Malnutrition
Hepatic Artery Thrombosis
Vascular Thromboses or Stenoses
Induction Immunosuppression
Maintenance Immunosuppression
Cognitive Outcomes, Family Function, and Quality of Life
Obesity, Dyslipidemia, and Metabolic Syndrome
Fertility and Pregnancy Posttransplantation
29: Liver in Systemic Disease
Pediatric Heart Disease and Chronic Liver Damage
Single-Ventricle Cardiac Physiology and the Liver
Hepatic Dysfunction with Systemic Infection
Juvenile Idiopathic Arthritis
Systemic Lupus Erythematosus
Drug-Induced Liver Injury in Rheumatologic Disease
Leukemia, Lymphoma, and Hepatosplenic T-cell Lymphoma
Graft-Versus-Host Disease
Hemophagocytic Lymphohistiocytosis
Macrophage Activation Syndrome
Hypopituitarism and Adrenal Insufficiency
Chronic Granulomatous Disease
Common Variable Immunodeficiency
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