D-penicillamine in Wilson's disease presenting as acute liver failure with hemolysis.
Wilson's disease in a young woman presenting with an acute course is described. The clinical manifestations were fulminant hepatic failure associated with marked intravascular hemolysis. Immediate D-penicillamine and high-dose steroid therapy did not influence the course of the disease. Necropsy revealed an increased hepatic copper content and cirrhosis with extensive necrosis of the liver.
D000208:Acute Disease; D000293:Adolescent; D002570:Ceruloplasmin / Q000097:blood; D003300:Copper / Q000032:analysis / Q000652:urine; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006461:Hemolysis; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use*
D005938:Glucocorticoids; D003300:Copper; D002570:Ceruloplasmin; D010396:Penicillamine