부산대학교 도서관

Metabolic liver disease refers to inherited disorders of metabolism that manifest prominently with liver disease. The 3 major inherited disorders that cause liver disease in adults are α1-antitrypsin deficiency, Wilson disease, and hereditary hemochromatosis. All are multisystem disorders that cause liver injury by various mechanisms but which can ultimately lead to cirrhosis and complications of liver failure. Also, various inborn errors of metabolism that cause liver disease manifest during childhood. These inborn errors of metabolism are, in aggregate, a major indication for pediatric liver transplant. As more patients who have childhood syndromes with liver involvement survive into adulthood, gastroenterologists who treat adults will need to become familiar with their care; however, the childhood syndromes rarely manifest initially in adulthood.