Long term results of liver transplantation for Wilson's disease: experience in France.
J Hepatol
; 60(3): 579-89, 2014 Mar.
Article
en En
| MEDLINE
| ID: mdl-24211743
ABSTRACT
BACKGROUND & AIMS:
Liver transplantation (LT) is the therapeutic option for severe complications of Wilson's disease (WD). We aimed to report on the long-term outcome of WD patients following LT.METHODS:
The medical records of 121 French patients transplanted for WD between 1985 and 2009 were reviewed retrospectively. Seventy-five patients were adults (median age 29 years, (18-66)) and 46 were children (median age 14 years, (7-17)). The indication for LT was (1) fulminant/subfulminant hepatitis (n = 64, 53%), median age = 16 years (7-53), (2) decompensated cirrhosis (n = 50, 41%), median age = 31.5 years (12-66) or (3) severe neurological disease (n = 7, 6%), median age = 21.5 years (14.5-42). Median post-transplant follow-up was 72 months (0-23.5).RESULTS:
Actuarial patient survival rates were 87% at 5, 10, and 15 years. Male gender, pre-transplant renal insufficiency, non elective procedure, and neurological indication were significantly associated with poorer survival rate. None of these factors remained statistically significant under multivariate analysis. In patients transplanted for hepatic indications, the prognosis was poorer in case of fulminant or subfulminant course, non elective procedure, pretransplant renal insufficiency and in patients transplanted before 2000. Multivariate analysis disclosed that only recent period of LT was associated with better prognosis. At last visit, the median calculated glomerular filtration rate was 93 ml/min (33-180); 11/93 patients (12%) had stage II renal insufficiency and none had stage III.CONCLUSIONS:
Liver failure associated with WD is a rare indication for LT (<1%), which achieves an excellent long-term outcome, including renal function.Palabras clave
ALF; BMI; BP; CLD; CNI; FHF; HAT; HCC; LT; MMF; PNF; SFHF; WD; Wilson's disease; acute liver failure; blood pressure; body mass index; calcineurin-inhibitor; chronic liver disease; fulminant hepatic failure; hepatic artery thrombosis; hepatocellular carcinoma; liver transplantation; mycophenolate mofetil; n.a.; not available; primary non function; subfulminant hepatic failure
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Trasplante de Hígado
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Degeneración Hepatolenticular
Tipo de estudio:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Límite:
Adolescent
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Adult
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Aged
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Child
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Female
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Humans
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Male
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Middle aged
País como asunto:
Europa
Idioma:
En
Año:
2014
Tipo del documento:
Article