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Peritoneal Malformations
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Liver Transplantation
Survival rates for pediatric liver transplantation are now >90% in the United States, in large part to refinements made in the critical care management of children with liver failure and advances in perioperative care and immunosuppression management. Protocols for immune…
Portal Hypertension and Varices
Portal hypertension, defined as an elevation of portal pressure >10-12 mm Hg or a hepatic venous pressure gradient >4 mm Hg, is a major cause of morbidity and mortality in children with liver disease. Portal hypertension occurs when there is increased portal…
Diseases of the Gallbladder
The incidence of gallbladder disease, particularly cholelithiasis and biliary dyskinesia, has been increasing in children, and has been associated with a rise in the number of cholecystectomies. Anomalies The gallbladder is congenitally absent in approximately 0.1% of the population. Hypoplasia…
Cystic Diseases of the Biliary Tract and Liver
Cystic lesions of liver may be initially recognized during infancy and childhood. Hepatic fibrosis can also occur as part of an associated developmental defect ( Table 392.1 ). Cystic renal disease is usually associated and often determines the clinical presentation…
Acute Hepatic Failure
Acute liver failure is a clinical syndrome resulting from massive necrosis of hepatocytes or from severe functional impairment of hepatocytes. The synthetic, excretory, and detoxifying functions of the liver are all severely impaired. In adults, hepatic encephalopathy has been an…
Drug- and Toxin-Induced Liver Injury
The liver is the main site of drug metabolism and is particularly susceptible to structural and functional injury after the ingestion, parenteral administration, or inhalation of chemical agents, drugs, plant derivatives (home remedies), herbal or nutritional supplements, or environmental toxins.…
Autoimmune Hepatitis
Autoimmune Hepatitis Chronic Liver Disease Autoimmune hepatitis is a chronic hepatic inflammatory process manifested by elevated serum aminotransaminase concentrations, liver-associated serum autoantibodies, and/or hypergammaglobulinemia. The serological autoantibody profile defines 2 main types of autoimmune hepatitis: AIH type 1, with positivity…
Mitochondrial Hepatopathies
A wide variety of mitochondrial disorders are associated with liver disease. Hepatocytes contain a high density of mitochondria because the liver, with its biosynthetic and detoxifying functions, is highly dependent on adenosine triphosphate. Defects in mitochondrial function can lead to…
Liver Disease Associated With Systemic Disorders
Liver disease is found in a wide variety of systemic illnesses, both as a result of the primary pathologic process and as a secondary complication of the disease or associated therapy. Inflammatory Bowel Disease Ulcerative colitis and Crohn disease (…