Work

Liver transplantation for hereditary tyrosinemia in the presence of hepatocellular carcinoma.

Year: 1989

Type: article

Abstract: Hereditary tyrosinemia is a metabolic disorder characterized by deficiency of the enzyme fummarylacetoacetate, resulting in the accumulation of tyrosine metabolites, which are toxic to the liver. Hepatic dysfunction, associated with tyrosinemia, varies from an acute form of liver failure to a chronic, progressive form that leads to cirrhosis and hepatocellular carcinoma (HCC).1,2 Liver transplantation is the treatment of choice for end-stage hepatic disease brought about by several metabolic disorders, including tyrosinemia. Liver replacement has also been offered to patients with primary hepatic malignancies.3 Despite the fact that liver transplantation cures the underlying metabolic defect in patients with tyrosinemia,4 little is known about the proper timing for transplantation in such patients and the potential consequences of replacing the liver in the presence of HCC. The purpose of this investigation is to report our experience with hepatic transplantation for tyrosinemia in the presence of HCC.

Source: PubMed