[Hepatic Wilson's disease: clinical presentation and prognosis. Indications for liver transplantation]

Minerva Gastroenterol Dietol. 1994 Dec;40(4):191-5.
[Article in Italian]

Abstract

Wilson's disease is a hereditary disorder of biliary copper excretion. Most often the disease presents with hepatic and neurological involvement. In the hepatic forms, hypocerulo-plasminemia, the determination of eye copper and the dosage of copper in serum, urine and liver tissue are all leads to diagnosis. The presentation and the biochemistry may direct the diagnosis of the acute forms. Treatment differs according to the clinical patterns. Early diagnosis in the asymptomatic patient leads to chelating therapy to prevent copper overload. Chronic disease may benefit from chelation and liver transplant. Transplantation is the cure for fulminant forms. We report three young women with Wilson's disease; one had a fulminant form and was transplanted and the other two responded to chelation therapy. Family screening allowed the identification of an asymptomatic sibling.

Publication types

  • Case Reports
  • Comparative Study
  • English Abstract

MeSH terms

  • Adolescent
  • Chelating Agents / administration & dosage
  • Chelating Agents / therapeutic use
  • Female
  • Hepatolenticular Degeneration / diagnosis*
  • Hepatolenticular Degeneration / genetics
  • Hepatolenticular Degeneration / therapy
  • Humans
  • Liver Transplantation*
  • Pedigree
  • Prognosis
  • Time Factors

Substances

  • Chelating Agents