亲体部分肝移植治疗小儿肝豆状核变性病

Preliminary experience of living related liver transplantation for Wilson's disease in children

目的 通过亲体部分肝移植的方法治疗小儿肝豆状核变性病 (Wilson’s病 ) ,探讨Wil son’s病的根治方法。方法 对术前均明确诊断为Wilson’s病的患儿 (男 1例 ,女 4例 )施行亲体部分肝移植 ,患儿平均年龄 9岁。本组供肝者均为患儿母亲。Child分级 :B级 4例 ,C级 1例。除 1例患儿血型为B型 ,供体血型为O型外 ,其余均为供受体血型相同。结合临床资料对本组所有Wilson’s病患儿进行回顾性分析。结果 所有供、受体手术顺利 ,术后恢复良好。 5例Wilson’s病患儿术后 3周肝功能恢复正常 ,复查铜氧化酶均正常 ,K F环均不同程度变浅 ,神经系统症状有所好转。术后并发症 :1例出现肝动脉栓塞 (术后第 6d再次行减体积肝移植 ) ,术后分别随访 12 .5个月、11个月、10个月、9个月和 7.5个月 ,现均已康复出院。

Objective To investigate LRLT (living related liver transplantation) for Wilson's disease in children.Methods LRLT was performed on 5 children(mean age 9 years) with Wilson's disease (1 male and 4 females).All living donors were mothers.The Child stage were grade B in 4 and grade C in 1.Only 1 recipient blood type was B, donor blood type was O, the other donors and recipients blood types were the same, respectively. A retrospective analysis was made for all cases.Results All donors and recipients recovered satisfactorily. In 5 Wilson's patients, liver graft function and copper oxidase became normal 3 weeks post operatively. Kayser Fleischer ring and nervous system symptom were alleviated to various extents. One patient became complicated hepatic artery thrombosis (reduced size liver transplantation was performed 6?days after the first transplantation ). The patients were followed up for 12.5 ?months, 11?months, 10?months, 9?months and 7.5 ?months respectively.Conclusion LRLT is an effective therapy for Wilson's disease and other metabolic liver diseases.