Re-evaluation of the indications for liver transplantation in Wilson's disease based on the outcomes of patients referred to a transplant center

被引：11

作者：

Ohya, Yuki ^{[1
]}

Okajima, Hideaki ^{[2
]}

Honda, Masaki ^{[1
]}

Hayashida, Shintaro ^{[1
]}

Suda, Hiroko ^{[1
]}

Matsumoto, Shiro ^{[3
]}

Lee, Kwang-Jong ^{[1
]}

Yamamoto, Hidekazu ^{[1
]}

Takeichi, Takayuki ^{[1
]}

Mitsubuchi, Hiroshi ^{[3
]}

Asonuma, Katsuhiro ^{[1
]}

Endo, Fumio ^{[3
]}

Inomata, Yukihiro ^{[1
]}

机构：

[1] Kumamoto Univ, Grad Sch Med Sci, Dept Transplantat & Pediat Surg, Kumamoto 8608556, Japan

[2] Kyoto Prefectural Univ Med, Grad Sch Med Sci, Dept Transplantat & Regenerat Surg, Kyoto, Japan

[3] Kumamoto Univ, Grad Sch Med Sci, Dept Pediat, Kumamoto 8608556, Japan

来源：

PEDIATRIC TRANSPLANTATION | 2013年 / 17卷 / 04期

关键词：

indication; liver transplantation; Wilson's disease; FULMINANT HEPATIC-FAILURE; CLINICAL PRESENTATION; CHILDREN; DIAGNOSIS; SURVIVAL; SCORE; MODEL;

D O I：

10.1111/petr.12085

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

The aim of this study was to re-evaluate the indications and timing of LT for WD. From 2000 to 2009, eight patients with WD who had been referred to our institution for LT were enrolled in this study. The mean patient age was 15.9yr (range, 737yr). Four patients could not receive LT, because there were no available donors. All four patients were treated with chelating agent medication. Three of them (two of two patients with fulminant WD and one of two with cirrhotic WD) who did not undergo LT are still alive and doing well with stable liver functional tests. Only one of the patients with cirrhotic WD who did not undergo LT died of hepatic failure. Even among the four patients who underwent LT, one with fulminant WD recovered from hepatic encephalopathy with apheresis therapy and chelating agent. He later required LT because of severe neutropenia from d-penicillamine. The other three patients who underwent LT recovered and have been doing well. Some of the patients with WD can recover and avoid LT with medical treatment. Even when WD has progressed liver cirrhosis and/or fulminant hepatic failure at the time of diagnosis, medical treatment should be tried before considering LT.

引用

页码：369 / 373

页数：5

共 17 条

[11] WILSONS-DISEASE - CLINICAL PRESENTATION AND USE OF PROGNOSTIC INDEX [J].