D-penicillamine in Wilson's disease presenting as acute liver failure with hemolysis

Dig Dis Sci. 1982 Dec;27(12):1126-9. doi: 10.1007/BF01391452.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Adolescent
  • Ceruloplasmin / blood
  • Copper / analysis
  • Copper / urine
  • Female
  • Glucocorticoids / therapeutic use
  • Hemolysis*
  • Hepatolenticular Degeneration / complications
  • Hepatolenticular Degeneration / diagnosis
  • Hepatolenticular Degeneration / drug therapy*
  • Humans
  • Liver Diseases / diagnosis*
  • Liver Diseases / etiology
  • Middle Aged
  • Penicillamine / therapeutic use*

Substances

  • Glucocorticoids
  • Copper
  • Ceruloplasmin
  • Penicillamine