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 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 (…
Viral hepatitis continues to be a major health problem in both developing and developed countries; there has been significant progress in efforts to recognize and to treat infected subjects. This disorder is caused by the 5 pathogenic hepatotropic viruses recognized…
Metabolic liver diseases in children, although individually rare, altogether represent a significant cause of morbidity and mortality. This is because the liver has a central role in synthetic, degradative, and regulatory pathways involving carbohydrate, protein, lipid, trace element, and vitamin…
Pathologic Manifestations Congenital and acquired alterations in hepatic structure and function (acute or chronic) can be manifest by varying patterns of reaction of the liver to cell injury. Hepatocyte injury can be caused by viral infection, drugs or toxins, hypoxia,…
During the early embryonic process of gastrulation, the 3 embryonic germ layers (endoderm, mesoderm, and ectoderm) are formed. The liver and biliary system arises from cells of the ventral foregut endoderm; their development can be divided into 3 distinct processes…
Cholestasis, a potentially serious condition that indicates hepatobiliary dysfunction, is defined as a serum conjugated bilirubin fraction greater than 1.0 mg/dL if the total serum bilirubin level is less than 5 mg/dL, or a value of conjugated bilirubin more than…
Abbreviations A1AT α 1 -antitrypsin ABC ATP-binding cassette AGS Alagille syndrome ASBT apical sodium-dependent bile acid transporter BRIC benign recurrent intrahepatic cholestasis CMV cytomegalovirus FIC1 familial intrahepatic cholestasis 1 JAG1 jagged-1 gene MCT medium-chain triglyceride MDR3 multidrug resistance protein 3 MMR…
Metabolic liver diseases may manifest as acute, life-threatening illnesses in the neonatal period or as chronic liver disease in adolescence or adulthood, with progression to liver failure, cirrhosis, or HCC. In a 2015 report of the Scientific Registry of Transplant…