CAJ | 학술논문

目的:探讨亲属活体部分肝移植治疗肝豆状核变性手术适应证.方法:回顾性分析2001年1月至2007年2月37例接受亲属活体部分肝移植治疗的肝豆状核变性患者的临床资料.其中男性16例,女性21例,年龄7～21岁,中位年龄11岁.临床诊断包括:急性肝功能衰竭3例,慢性肝功能衰竭失代偿32例(其中伴有神经系统功能障碍13例),慢性肝功能衰竭代偿但伴有严重神经系统功能障碍2例.术前15例神经系统功能障碍患者的评分为(15.9±4.3)分.随访20～93个月,平均(48.1±27.6)个月.结果:本组37例患者获得随访,随访率100%.患者及移植物的1、3、5年存活率分别为91.9%、83.8%、75.7%和86.5%、78.4%、75.7%.供受者外科并发症包括:2例供者术后发生肝断而胆漏,经引流后治愈;3例受者发生血管并发症,其中2例为肝动脉栓塞,再次急诊行尸体肝移植后治愈;另1例为肝静脉吻合口狭窄,行介入球囊扩张治疗后治愈.神经系统功能障碍患者移植术后症状明显缓解,术后6、12、18、24和30个月评分分别为:[(17.5±3.7)分,n=13]、[(21.0±4.3)分,n=12]、[(23.9±3.9)分,n=10]、[(26.6±2.2)分,n=10]和[(28.1±1.9)分,n=7].结论:急性肝功能衰竭、慢性肝功能衰竭失代偿伴有(或不伴有)神经系统功能障碍均是亲属活体部分肝移植的手术适应证.对伴有严重神经系统功能障碍但肝功能相对稳定的患者,如内科治疗无效,仍可考虑行亲属活体部分肝移植.
목적:탐토친속활체부분간이식치료간두상핵변성수술괄응증.방법:회고성분석2001년1월지2007년2월37례접수친속활체부분간이식치료적간두상핵변성환자적림상자료.기중남성16례,녀성21례,년령7～21세,중위년령11세.림상진단포괄:급성간공능쇠갈3례,만성간공능쇠갈실대상32례(기중반유신경계통공능장애13례),만성간공능쇠갈대상단반유엄중신경계통공능장애2례.술전15례신경계통공능장애환자적평분위(15.9±4.3)분.수방20～93개월,평균(48.1±27.6)개월.결과:본조37례환자획득수방,수방솔100%.환자급이식물적1、3、5년존활솔분별위91.9%、83.8%、75.7%화86.5%、78.4%、75.7%.공수자외과병발증포괄:2례공자술후발생간단이담루,경인류후치유;3례수자발생혈관병발증,기중2례위간동맥전새,재차급진행시체간이식후치유;령1례위간정맥문합구협착,행개입구낭확장치료후치유.신경계통공능장애환자이식술후증상명현완해,술후6、12、18、24화30개월평분분별위:[(17.5±3.7)분,n=13]、[(21.0±4.3)분,n=12]、[(23.9±3.9)분,n=10]、[(26.6±2.2)분,n=10]화[(28.1±1.9)분,n=7].결론:급성간공능쇠갈、만성간공능쇠갈실대상반유(혹불반유)신경계통공능장애균시친속활체부분간이식적수술괄응증.대반유엄중신경계통공능장애단간공능상대은정적환자,여내과치료무효,잉가고필행친속활체부분간이식.
Objective To probe into indication of living-related liver transplantation (LRLT) for Wilson's Disease. Methods From January 2001 to February 2007, thirty-seven living-related liver transplants were performed. A retrospective analysis was carried on outcome of those patients. The indications for LRLT were acute hepatic failure in 3 patients and chronic advanced liver disease in 32 patients including 13 patients with Wilsonian neurological manifestations. Two patients presented with severe Wilsonian neurological manifestations even though their liver functions were stable. According to the scoring system for evaluation of the neurological impairment in Wilson disease based on neurological signs and functions (total score was 30 ), the pre-transplantation score of those patients with neurological manifestations was 15.9 ± 4.3 (n = 15). Results Thirty-seven patients were followed up for 20-93 months. The survival rates of post-transplant patients and grafts at 1,3, and 5 year were 91.9% , 83.8% , 75.7% , and 86.5%, 78.4% ,75.7%, respectively. Postoperative surgical complications occurred in 2 donors with bile leakage required drainage,in 2 recipients with hepatic thrombosis underwent retransplantation of cadeveric liver and in 1 recipient with hepatic stenosis required balloon dilatation. Neurological function was improved in all recipients and the score of posttransplantation at 6, 12,18,24, and 30 month was 17.5 ± 3.7 (n=13) ; 21.0±4.3(n=12);23.9±3.9(n=10);26.6±2.2(n= 10) and 28.1±1.9(n =7)respectively. Conclusions Patients with acute hepatic failure or patients with severe liver disease unresponsive to chelation treatment should be treated with LRLT. Early transplantation in patients with an unsatisfactory response medical treatment may prevent irreversible neurological deterioration even though their liver function is stable.