Fulminant hepatic failure without evidence of cirrhosis in a case of Wilson's disease.

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We treated a sixteen-year old Japanese girl with fulminant hepatic failure in Wilson's disease. The diagnosis of Wilson's disease was made immediately after the admission because of low serum copper and ceruloplasmin levels with high urinary copper excretion. Her hepatic failure was accompanied by bouts of hemolytic crisis. In spite of the administration of D-penicillamine and repeated plasmapheresis, she died of hepatic failure four months later. At autopsy, the surface of the liver was smooth. The histology of the liver showed massive necrosis. There were only a few remaining scattered hepatocytes, in which copper was revealed by Rhodanine staining. There was no evidence of cirrhosis. The livers of the previously reported cases of Wilson's disease accompanied by fulminant hepatic failure were all cirrhotic. Our case indicated that Wilson's disease could occur as true fulminant hepatic failure without preceeding neurological and hepatological signs and the evidence of cirrhosis.
社団法人　日本内科学会の論文