[肝豆状核变性肝移植的适应症]

[Indications for liver transplantation in Wilson's disease].

作者信息

Gottrand F, Razemon M, Otte J B, Vigier J E, Farriaux J P

机构信息

Service de Génétique et Maladies Héréditaires du Métabolisme de l'Enfant, Hôpital Huriez, CHR, Lille.

出版信息

Arch Fr Pediatr. 1988 Mar;45(3):187-8.

PMID:3293546

Abstract

We report the case of a 12 year-old boy with Wilson's disease, presenting with decompensated liver cirrhosis. Medical treatment failed to prevent fulminant liver failure in less than one month. Emergency liver transplantation was successfully performed. This report led us to review the prognosis of Wilson's disease with liver failure according to the present results of liver transplantation.

摘要

我们报告了一例12岁患有威尔逊氏病的男孩，表现为失代偿性肝硬化。药物治疗未能在不到一个月的时间内预防暴发性肝衰竭。成功进行了紧急肝移植。根据目前肝移植的结果，本报告促使我们回顾威尔逊氏病合并肝衰竭的预后情况。