Journal of Hepatology, 60(3):579–589, March, 2014.
doi abstract bibtex
doi abstract bibtex
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 (\textless1%), which achieves an excellent long-term outcome, including renal function.
@article{guillaud_long_2014,
title = {Long term results of liver transplantation for {Wilson}'s disease: experience in {France}},
volume = {60},
issn = {1600-0641},
shorttitle = {Long term results of liver transplantation for {Wilson}'s disease},
doi = {10.1016/j.jhep.2013.10.025},
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 ({\textless}1\%), which achieves an excellent long-term outcome, including renal function.},
language = {eng},
number = {3},
journal = {Journal of Hepatology},
author = {Guillaud, Olivier and Dumortier, Jérôme and Sobesky, Rodolphe and Debray, Dominique and Wolf, Philippe and Vanlemmens, Claire and Durand, François and Calmus, Yvon and Duvoux, Christophe and Dharancy, Sébastien and Kamar, Nassim and Boudjema, Karim and Bernard, Pierre Henri and Pageaux, Georges-Philippe and Salamé, Ephrem and Gugenheim, Jean and Lachaux, Alain and Habes, Dalila and Radenne, Sylvie and Hardwigsen, Jean and Chazouillères, Olivier and Trocello, Jean-Marc and Woimant, France and Ichai, Philippe and Branchereau, Sophie and Soubrane, Olivier and Castaing, Denis and Jacquemin, Emmanuel and Samuel, Didier and Duclos-Vallée, Jean-Charles},
month = mar,
year = {2014},
pages = {579--589}
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