CAJ | 학술논문

目的评估亲属活体及儿童心脏死亡器官捐赠(DCD)供肝儿童肝移植51例的临床疗效.方法回顾性分析2006年6月至2013年3月期间单中心51例儿童肝移植的临床资料,其中,2006年6月至2009年3月间完成的亲属活体部分供肝肝移植42例,2011年11月至2013年3月间完成的儿童DCD供肝肝移植9例.51例受者中,男性患儿24例,女性患儿27例,患几年龄为80d至14岁,体质量为3.1～45.0 kg.患儿原发疾病分别为胆道闭锁34例,肝豆状核变性6例,肝糖原贮积症5例,门静脉高压3例及重型肝炎2例.术后随访时间为2～83个月,其中42例亲属活体供肝肝移植受者随访41～83个月,9例DCD肝移植受者随访2～18个月.结果 42位亲属活体供者均顺利出院并健康存活.51例受者围手术期死亡8例(15.7％),其中肝动脉血栓形成5例,门静脉血栓形成1例,肺部曲霉感染1例,移植物功能丧失死亡1例;随访期死亡4例,其中肝静脉狭窄死亡3例,并发噬血综合征死亡1例,均死于术后1年内;意外死亡2例.随访截止时健康生活37例,存活最长者达83个月,移植后1、3、5年受者存活率均为72.5％.结论亲属活体及儿童DCD供肝肝移植是治疗儿童终末期肝病的有效方法,血管并发症是影响术后疗效的主要原因,探索新的手术技术,减少术后血管并发症能明显提高受者存活率.
목적 평고친속활체급인동심장사망기관연증(DCD)공간인동간이식51례적림상료효.방법 회고성분석2006년6월지2013년3월기간단중심51례인동간이식적림상자료,기중,2006년6월지2009년3월간완성적친속활체부분공간간이식42례,2011년11월지2013년3월간완성적인동DCD공간간이식9례.51례수자중,남성환인24례,녀성환인27례,환궤년령위80d지14세,체질량위3.1～45.0 kg.환인원발질병분별위담도폐쇄34례,간두상핵변성6례,간당원저적증5례,문정맥고압3례급중형간염2례.술후수방시간위2～83개월,기중42례친속활체공간간이식수자수방41～83개월,9례DCD간이식수자수방2～18개월.결과 42위친속활체공자균순리출원병건강존활.51례수자위수술기사망8례(15.7％),기중간동맥혈전형성5례,문정맥혈전형성1례,폐부곡매감염1례,이식물공능상실사망1례;수방기사망4례,기중간정맥협착사망3례,병발서혈종합정사망1례,균사우술후1년내;의외사망2례.수방절지시건강생활37례,존활최장자체83개월,이식후1、3、5년수자존활솔균위72.5％.결론 친속활체급인동DCD공간간이식시치료인동종말기간병적유효방법,혈관병발증시영향술후료효적주요원인,탐색신적수술기술,감소술후혈관병발증능명현제고수자존활솔.
Objective To summarize the experience of pediatric liver transplantation (LT) in our hospital and evaluate the outcome.Methods Fifty-one pediatric patients with end-stage liver diseases who underwent LT between June 2006 and May 2013 in our hospital were analyzed retrospectively.These 51 recipients (24 boys and 27 girls) aged from 80 days to 14 years whose body weight at the time of operation was 3.1 to 45.0 kg.The underlying diseases were biliary atresia (35 cases),hepatolenticular degeneration diseases (6 cases),glycogen storage diseases (5 cases),cavernous transformation of the portal vein (3 cases) and fulminant liver failure (2 cases).All recipients were followed up from 2 to 83 months.In 51 cases of pediatric LT,42 received living related liver transplantation (LRLT) from April 2006 to December 2009,and 9 LT using donation after cardiac death donors (DCD) from November 2011 to March 2013.Results The 42 donors in LDLT resumed to their pre-transplantation live soon after the operation without occurrence of any complication.Thirty-seven of the 51 children have survived for 2-83 months (mean 58 months) now.Eight cases died from postoperative complications including hepatic artery thrombus (5 cases),aspergillus pulmonary infection (one case),portal vein thrombosis (one case) and graft nonfunction (GNF) in perioperative term (one case).The perioperative mortality rate of recipients was 15.7％ (8/51).During the follow-up term,4 died,including one case of hemophagocytic syndrome and 3 cases of stricture of hepatic vein in the first year after operation.In addition,2 recipients died of accidental asphyxia and food poisoning.The longest survival time was 83 months.At present,37 recipients were survival,and the 1-,3-and 5-year survival rate was 72.5％.Conclusion LT is an effective method to children with end-stage liver disease.But the postoperative vascular complications are the predominant cause of deseasing the survival rate of pediatric recipients.Exploring a new technique to minimize vascular complications is helpful to raise the survival rate.