LIVER-TRANSPLANTATION FOR WILSONS-DISEASE - INDICATIONS AND OUTCOME

被引:190
作者
SCHILSKY, ML
SCHEINBERG, IH
STERNLIEB, I
机构
[1] ALBERT EINSTEIN COLL MED,MARION BESSIN LIVER RES CTR,BRONX,NY 10461
[2] NATL CTR STUDY WILSONS DIS INC,NEW YORK,NY 10019
关键词
D O I
10.1002/hep.1840190307
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
The objective of this study was to determine the indications for and results of liver transplantation in patients with Wilson's disease on the basis of results of a survey with retrospective review of data obtained on 55 transplants performed at centers in the United States and Europe. The study group comprised 32 females and 23 males, aged 8.5 to 51 yr, with features diagnostic of Wilson's disease. Indication for orthotopic liver transplantation included hepatic insufficiency (n = 32), wilsonian fulminant hepatitis (n = 21), intractable neurological Wilson's disease (n = 1) and gastrointestinal hemorrhage (n = 1). Forty-three patients have survived, at this writing, from 3 mo to 20 yr. Mean and median survival after orthotopic liver transplantation were 2.7 and 2.5 yr, respectively. Survival at 1 yr was 79%. Nonfatal complications occurred in five patients. Of the seven patients given transplants for hepatic insufficiency who manifested neurological and/or psychiatric manifestations at the time of orthotopic liver transplantation, four showed improvement of these symptoms. One patient given a transplant for intractable neurological disease improved but died of a vascular complication. Our data demonstrate that liver transplantation is life-saving but not without risk for patients with wilsonian fulminant hepatitis or chronic severe hepatic insufficiency unresponsive to medical therapy. Furthermore, neurological or psychiatric symptoms due to Wilson's disease may improve after liver transplantation; however, the role of this procedure in the management of patients with neurological Wilson's disease in the absence of hepatic insufficiency is still uncertain.
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页码:583 / 587
页数:5
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