Inborn Metabolic Disorders Treated by Liver Transplantation

In the European Liver Transplant Registry issued in October 1993 a metabolic disorder was recorder as a indication for transplantation in 18% of 1248 children treated by liver transplantation from May 1988 to June 1993. Two groups can be distinguished, as discussed below.

Liver disorders associated with severe liver damage.The majority of liver transplantations reported in the literature were performed in children with hereditary liver diseases resulting in liver cell failure,

cirrhosis, or a risk of hepatocellular carcinoma and for which there was no other effective treatment avaliable.

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Alpha-1-Antiterypsin Defictency. Liver transplantation is the only effective therapy for children with cirrhosis and the protein inhibitor (PI) Z phenotype. Serum levels of alpha-1-antitrypsin (AAT) return to normal; the PI phenotype of the donor's liver becomes detectable in the recipient's serum within 24h of transplantation. It may be hoped that this will prevent pulmonary emphisema during adulthood.

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