Liver transplantation may be necessary for a patient with Wilson's disease and severe hepatic involvement. A patient with Wilson's disease may enjoy long-term survival and a significantly improved quality of life following liver transplantation.
Indications for liver transplantation in a patient with Wilson's disease:
(1) fulminant hepatitis
(2) progressive hepatic insufficiency with hemolytic anemia following discontinuation of chelation therapy
(3) cirrhosis AND hepatic decompensation AND failure to respond to an adequate trial of therapy (2-3 months chelation therapy and metabolic management)
(4) cirrhosis AND hepatic decompensation AND unable to tolerate chelation therapy because of side effects
The serum transaminase levels are a poor criterion for transplantation, since the elevations may be modest despite severe liver disease. Delay in transplantation can be fatal.
Some investigators believe that liver transplantation may benefit patients with severe neurological complications, even in the absence of significant liver disease.
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