Fulminant Wilsonian hepatitis unmasked by disease progression: report of a case and review of the literature

Dig Dis Sci. 1999 May;44(5):1054-8. doi: 10.1023/a:1026637403894.

Abstract

Among various hepatic manifestations of Wilson's disease, fulminant hepatic failure is the most uncommon entity and requires a detailed clinicopathological analysis for correct diagnosis. Left unrecognized and without proper therapy, in time the disease rapidly progresses to death. We describe a 24-year-old woman who died within five weeks of the onset of Wilson's disease, which presented with a dramatic course. Discriminating features of the disease are discussed with regard to the literature.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Ceruloplasmin / analysis
  • Copper / blood
  • Disease Progression
  • Fatal Outcome
  • Female
  • Hepatic Encephalopathy / diagnosis*
  • Hepatic Encephalopathy / etiology*
  • Hepatic Encephalopathy / pathology
  • Hepatitis / complications*
  • Hepatitis / pathology
  • Hepatolenticular Degeneration / complications*
  • Hepatolenticular Degeneration / pathology
  • Humans
  • Kidney Tubules, Proximal / pathology

Substances

  • Copper
  • Ceruloplasmin