亲属活体供肝移植治疗肝豆状核变性时供者的选择

Selection of donors of living related liver transplantation for Wilson’s disease

目的总结亲属活体供肝移植治疗肝豆状核变性时选择供者的经验。方法29例肝豆状核变性患者接受亲属活体供肝移植,共有70名供者入选筛查,筛查内容包括肝功能、血清肝炎标记物、肝体积的估测及肝血管和胆管系统解剖结构的预测,以及年龄、体型、供受者配型、伦理学方面的评估等。结果23名供者被排除,10名(43.5%)志愿者是因为受家庭、亲属和社会上的传统观念的影响而最终放弃捐肝意愿;6名(26.1%)因铜代谢指标异常,血清铜蓝蛋白<200mg/L,24h尿铜>100μg;4名(17.4%)因为脂肪肝;3名(13%)因为乙型肝炎。10例为右叶供肝,19例为左叶供肝。供肝重量与受者体重之比为(1.38±0.44)%。所有供者术后恢复正常生活。3名供者术后发生外科相关并发症,其中2例为一过性胆漏,经穿刺引流后治愈,1例为肝断面渗血,经保守治疗痊愈。结论影响供肝选择的主要因素有传统观念、供者铜代谢指标的异常、乙型肝炎及脂肪肝;而术前对供者进行全面评估及供肝切取技术的改进,是保证供者安全和供肝质量的关键。

Objective To summarize experience of selection of donors of living related liver transplantation （LRLT） for Wilson＇s Disease. Methods Twenty-nine cases of Wilson＇s disease were subjected to LRLT in our center. A retrospective data analysis was carried on 70 potential donors. Results Twenty-three of the 70 potential donors were excluded. The causes were as follows： family and traditional background （n = 10, 43.5%）, copper metabolism abnormality, serum ceruloplasmin 〈200 mg/L, 24 h urinary copper ）100μg （n=6, 26. 1%）, hepatic steatosis （n=4, 17. 4%） and hepatitis B （n = 3.13%）. The grafts were right liver （n = 10） and left liver （n = 19）. Graft-recipient weight ratio was （1.38±. 44） %. All donors recovered completely. Perioperative surgical complica- tions occurred in 3 donors and were cured without reoperation, temporary biliary leakages （n = 2） as well as postoperative bleeding from the wound of liver （n= 1）. Conclusions Major impact on living relative donors for Wilson＇ s Disease is traditional background, copper metabolism abnormalities, hepatitis B and hepatic steatosis. Comprehensive preoperative evaluation of potential donors and harvesting liver surgery innovation ensure the donor safety and graft quality.