论文部分内容阅读
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。结论 亲体部分肝移植是根治Wilson’s病等代谢性疾病的有效方法
Objective To treat Wilson’s disease (Wilson’s disease) in children through partial liver transplantation and to explore the method of radical treatment of Wil son’s disease. Methods Partial liver transplantation was performed on the children with Wilson’s disease (1 males and 4 females) who were diagnosed preoperatively. The average age of children was 9 years old. This group of donors are children with mothers. Child classification: B grade in 4 cases, C grade in 1 case. Except for one case where the blood type was type B and the donor type was type O, the rest were of the same donor blood type. All patients with Wilson’s disease were retrospectively analyzed with clinical data. Results All donor and recipient surgeries were successful and postoperative recovery was good. Five patients with Wilson’s disease returned to normal liver function 3 weeks after operation, and the copper oxidase was normal. The K F ring was lighter and the neurological symptoms improved. Postoperative complications: Hepatic artery embolism occurred in one case (VGTD was performed again on the 6th day after operation). The patients were followed up for 12.5 months, 11 months, 10 months, 9 months and 7.5 months respectively. Are now discharged from hospital. Conclusion Partial liver transplantation is an effective method to cure Wilson’s disease and other metabolic diseases