CAJ | 학술논문

目的总结儿童肝移植的临床疗效,探讨儿童肝移植在适应证、手术方式及术后处理等方面的特点.方法 2000年8月至2007年7月对20例儿童(＜14岁)施行肝移植手术23次,患儿年龄6个月～13岁,原发疾病主要包括先天性胆道闭锁、Wilson病、肝糖原累积症和尿素循环障碍.手术方式采用亲体部分肝移植4次,多米诺肝移植1次,劈离式肝移植5次,减体积肝移植10次和尸体全肝移植3次.除1例患儿应用环孢霉素外,其余19例患儿均应用他克莫司,联合激素及霉酚酸酯三联免疫抑制方案.结果患儿围手术期内死亡3例(15.0%),死亡原因分别为移植肝原发无功、心功能衰竭和腹腔感染.9例患儿术后出现不同的并发症,包括肝动脉血栓形成2例、门静脉血栓形成1例、急性排斥反应1例、胆漏3例、胆道狭窄2例、肠瘘2例、腹腔感染3例、肺感染1例和心功能衰竭1例.患儿术后6个月、1年和2年累积生存率分别为80.0%、73.9%和73.9%.结论肝移植是治疗儿童终末期肝病的有效方法,手术方式可根据患儿的年龄、体重进行选择.
목적 총결인동간이식적림상료효,탐토인동간이식재괄응증、수술방식급술후처리등방면적특점.방법 2000년8월지2007년7월대20례인동(＜14세)시행간이식수술23차,환인년령6개월～13세,원발질병주요포괄선천성담도폐쇄、Wilson병、간당원루적증화뇨소순배장애.수술방식채용친체부분간이식4차,다미낙간이식1차,벽리식간이식5차,감체적간이식10차화시체전간이식3차.제1례환인응용배포매소외,기여19례환인균응용타극막사,연합격소급매분산지삼련면역억제방안.결과 환인위수술기내사망3례(15.0%),사망원인분별위이식간원발무공、심공능쇠갈화복강감염.9례환인술후출현불동적병발증,포괄간동맥혈전형성2례、문정맥혈전형성1례、급성배척반응1례、담루3례、담도협착2례、장루2례、복강감염3례、폐감염1례화심공능쇠갈1례.환인술후6개월、1년화2년루적생존솔분별위80.0%、73.9%화73.9%.결론 간이식시치료인동종말기간병적유효방법,수술방식가근거환인적년령、체중진행선택.
Objective To summarize the clinical efficacy of pediatric liver transplantation,and investigate the characters of pediatric liver transplantation in their indications.surgical procedures and postoperative management.Methods From August 2000 to March 2007,23 liver transplantations were performed on 20 children,aging from 6 months to 13 years old.The most common indications were biliary atresia,Wilson's disease,glycogen storage disease and urea cycle defects.Surgical procedures included 4 living donor liver transplantations,1 Domino liver transplantation,5 split grafts,10 reduced liver grafts and 3 whole cadaveric grafts.The triple-drug(FK506,steroid and MMF)immunosuppressive regimen was used in 19 children,except one children using cyclosporine.Resuits Three children died of primary non-function,heart failure and abdominal infections respectively during peri-operative period,and the mortality was 15.0%.Nine children showed different post-operative complications including 2 hepatic artery thrombosis,1 portal vein thrombosis,1 acute rejection,3 biliary leakage,2 biliary stricture,2 intestinal fistula,3 abdominal infection,1 pulmonary infection and 1 heart failure.Cumulative patient survival rates at 6-month,1-and 2-year were 80.0%,73.9%and 73.9%,respectively.Conclusions Liver transplantation is an effective option to cure the liver disease of children with end-stage.Different surgieal procedure could be chosen according to the children's age and body weight.