亲体部分肝移植治疗Wilson病20例报告

Living-related liver transplantation for the treatment of Wilson's disease

目的探讨亲体部分肝移植治疗肝豆状核变性病 (Wilson病 )的价值。方法 2 0 0 1年 1月至 2 0 0 3年 10月 ,我院连续为 2 0例Wilson病患者成功施行亲体部分肝移植术 ,男性 8例 ,女性 12例 ,年龄 7～ 2 0岁 ,平均 11 1岁。 3例是暴发性肝功能衰竭 ,17例慢性进行性肝损害。供肝者为患者父亲或母亲。结果供受体手术顺利 ,术后 1个月肝功能和铜蓝蛋白恢复正常水平。 19例病人健康存活 ,1例术后 72d死于排斥反应。术后并发症包括 :醒状昏迷 1例 ,肝动脉血栓形成 1例 ,创面胆漏 1例 ,平均随访 18 9个月。结论亲体部分肝移植是治疗Wilson病并发肝功能衰竭的有效疗法。

Objective To evaluate living-related liver transplantation (LRLT) for the treatment of Wilson′s disease. MethodsBetween Jan. 2001 and Oct. 2003,LRLT was performed in 20 patients (mean age 11.1 years) of late staged Wilson′s disease. Among them emergency transplantation was performed in 3 patients. Donor livers were all from patient′s parents. ResultsSurgery was successful in all donors and recipients, liver function test and serum ceruloplasmin reached normal level one month after transplantation. One patient died of severe rejection. Discharged patients were followed up from 2～33 months (mean 18.9 months). [WT5”HZ]ConclusionsLiving related liver transplantation is an effective treatment for Wilson′s disease complicated with hepatic dysfunction.