亲属活体部分肝移植治疗肝豆状核变性的临床研究  

Living related liver transplantation for Wilson′s disease: clinical study of 32 cases

作　　者：王学浩 张峰 李相成 成峰 李国强 孙倍成 孔连宝 

机构地区：[1]南京医科大学第一附属医院肝脏外科,中国活体肝脏移植研究所,210029

出　　处：《中华医学杂志》2006年第46期3290-3293,共4页National Medical Journal of China

基　　金：江苏省医学重点学科建设基金资助项目(135-10);江苏省卫生厅科研课题基金资助项目(455EA9502)

摘　　要：目的总结亲属活体部分肝移植治疗肝豆状核变性供体的选择和外科治疗经验。方法自2001年1月至2006年3月，共完成亲属活体部分肝移植治疗肝豆状变性32例，其中儿童29例，〉14岁者3例，急性肝功能衰竭3例，慢性肝功能不全29例，13例伴有不同程度的神经系统功能障碍。供肝者7例为患者的父亲，25例为母亲，铜蓝蛋白均基本正常，24h尿铜〈100μg。施行术式：31例为原位部分肝移植，1例为原位辅助部分肝移植。结果随访3～63个月，现存活28例，围手术期死亡2例，另外2例分别死于胆漏和难以控制的排斥反应合并感染。2例发生肝动脉栓塞，需再次肝移植。1例术后期8个月出现胆总管吻合口狭窄而行胆肠吻合。2例术后22个月和28个月发生排斥反应，改用普乐可复后逆转。伴有神经系统功能障碍的患者中11例获得长期存活，术后锥体外系症状及语言和运动障碍明显好转。结论亲属活体部分肝移植是治疗肝豆状变性的有效方法，伴有神经系统功能障碍的患者，术后绝大多数有不同程度的改善。Objective To summarize the experience in donor selection and surgical outcomes of living related liver transplantation （LRLT） for Wilson＇s disease （WD）. Methods Thirty-two WD patients, 29 children and 3 being over 14, underwent LRLT because of chronic advanced liver disease （29 cases） or culminant hepatic failure （3 cases） among which 13 cases showed neurological dysfunction. The donors, including 7 fathers and 25 mothers, had their serum celuroplasmin levels within normal limit or slightly lower and their 24-hour urine copper 〈 100 μg. The modes of operation included orthotopic partial liver transplantation （31 cases） and orthotopic accessory partial liver transplantation （ 1 case）. The patients were followed p for 3 - 63 months postoperatively. Results Two patients die in the intraoperative period, and 2 died of bile leakage and severe rejection combined with infection. Twenty-eight patients survived, of which 2 had hepatic artery thrombosis and underwent retransplantation of cadeveric lever, one had anastomic stenosis 8 months after the original transplantation and underwent Roux-en-Y hepaticojejunostomy, 2 had chronic rejection 22 and 28 months after the LRLT respectively and were successfully rescued buy switching their primary immunosuppressor from cyclosporine A to FK506. Eleven patients with neurological dysfunctions all showed long-term survival and their extrapyramidal signs and speech disorders, and dyskinesia all were alleviated. Conclusion LRLT is the treatment of choice for WD patients with chronic advanced liver disease and culminant hepatic failure. After LRLT the disorders of the nervous system are alleviated.

关 键 词：肝豆状核变性 活体供者 肝移植 

分 类 号：R657.3[医药卫生—外科学]