title: Liver transplantation for Wilson's disease.
abstract: Although Wilsons's disease (WD) may be treated with copper chelation (to remove copper) or zinc salts (to prevent absorption) to alleviate or prevent symptom development in most patients, there are WD patients for whom medical therapy is inadequate and survival would be unlikely without liver transplantation. Liver transplantation is indicated for the ∼5% of WD patients with acute liver failure as the first presentation of disease, most commonly in the second decade of life, or those who present with end-stage liver disease and severe hepatic insufficiency, most commonly in the third and fourth decades. Liver transplantation restores normal biliary copper excretion (thereby preventing disease recurrence) and promotes removal of copper from extrahepatic sites. Outcomes of liver transplantation for WD are excellent, including both cadaveric and living donors.
authors.affiliation: Sections of Digestive Disease and Immunology and Transplantation, Yale New Haven Transplant Center, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: Michael L
authors.initials: ML
authors.lastname: Schilsky
mesh_terms: D058625:End Stage Liver Disease / Q000209:etiology / Q000601:surgery; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D017114:Liver Failure, Acute / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation / Q000379:methods; D019520:Living Donors; D016896:Treatment Outcome
keywords: Wilson's disease; copper; liver failure; liver transplant