Long-term results of liver transplantation for Wilson's disease.
Wilson's disease is a hereditary defect in hepatic copper metabolism, causing hepatic, neurological and/or psychiatric manifestations. For patients with severe disease, liver transplantation is the treatment of choice. The aim of this study was to report the long-term outcome of patients who underwent liver transplantation for Wilson's disease.


METHODS
Thirteen patients with Wilson's disease, transplanted in Lyon France between January 1987 and May 2006, were including in this study: eight women and five men, aged eight to 53 years (median 20 years, seven children and six adults). The diagnosis of Wilson's disease was established before liver transplantation.


RESULTS
The indication for liver transplantation was chronic (69%) or fulminant liver failure (31%). The median follow-up after liver transplantation was 10 years with 100% patient survival. Copper metabolism returned to normal in all patients. None of the patients with exclusive liver disease required chelation treatment after liver transplantation and none developed neurological symptoms of Wilson's disease.


CONCLUSIONS
Liver transplantation totally reverses the abnormalities of copper metabolism and subsequent hepatic failure, but the course of neurological symptoms remains unpredictable. Long-term patient survival can be excellent without occurrence of neurological complications.
Pabón
V
V
Centre de Référence Maladies Rares-Maladie de Wilson, Hospices Civils de Lyon, Lyon, France.
Dumortier
J
J
Gincul
R
R
Baulieux
J
J
Ducerf
C
C
Trépo
C
C
Souquet
J-C
JC
Zoulim
F
F
Paliard
P
P
Boillot
O
O
Bost
M
M
Lachaux
A
A
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006527:Hepatolenticular Degeneration / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D016896:Treatment Outcome