Inherited metabolic diseases of the liver

Many inherited metabolic diseases affect the liver in neonates, children, or adults. The histopathologic changes are diverse and may be acute or chronic. They can be considered primary (when the injury is from the cytopathic effect of an accumulated metabolite) or secondary (e.g., an infection caused by an immune deficiency). All forms of liver disease are described: for example, intrahepatic cholestasis, neonatal hepatitis with giant-cell transformation, paucity of bile ducts, steatosis, steatohepatitis, necroinflammatory diseases (acute or chronic), fibrosis, cirrhosis, and neoplasms (benign or malignant). Familiarity with the morphologic changes is important in clinicopathologic correlation, diagnosis, and understanding of pathogenetic mechanisms.