CAJ | 학술논문

目的探讨肝移植治疗Caroli病的临床效果.方法回顾性分析1999年9月至2008年2月期间7例弥漫型Caroli病患者行肝移植治疗后的临床资料.对其临床特征、手术方式、并发症类型以及随访情况进行分析.结果 7例患者术前均表现为复发性胆管炎症状并最终确诊为弥散型Caroli病.其中男性3例,女性4例;年龄10～31岁,平均16岁.6例患者肝移植术前接受保守治疗,1例曾行胆囊切除、T型管引流术.4例患者行劈离式肝移植,2例行全肝移植,1例行亲属活体供肝肝移植.其中2例患者术中采用静脉-静脉转流术.手术平均耗时9.1 h.术后并发症包括:肺部感染3例、急性排斥反应2例、胸腔积液2例、肝断面胆汁漏1例.经抗感染、调整免疫抑制方案及用量、胸腔穿刺抽吸及经皮穿刺引流等对症保守治疗后症状得到控制.1例肝移植受者术后19 d死于急性肾功能衰竭合并多器官功能衰竭,其余6例受者均存活.随访发现,存活的6例受者健康状况良好,其中最长存活已达7年.结论应用肝移植治疗弥散型Caroli病具有良好的临床效果.
목적 탐토간이식치료Caroli병적림상효과.방법 회고성분석1999년9월지2008년2월기간7례미만형Caroli병환자행간이식치료후적림상자료.대기림상특정、수술방식、병발증류형이급수방정황진행분석.결과 7례환자술전균표현위복발성담관염증상병최종학진위미산형Caroli병.기중남성3례,녀성4례;년령10～31세,평균16세.6례환자간이식술전접수보수치료,1례증행담낭절제、T형관인류술.4례환자행벽리식간이식,2례행전간이식,1례행친속활체공간간이식.기중2례환자술중채용정맥-정맥전류술.수술평균모시9.1 h.술후병발증포괄:폐부감염3례、급성배척반응2례、흉강적액2례、간단면담즙루1례.경항감염、조정면역억제방안급용량、흉강천자추흡급경피천자인류등대증보수치료후증상득도공제.1례간이식수자술후19 d사우급성신공능쇠갈합병다기관공능쇠갈,기여6례수자균존활.수방발현,존활적6례수자건강상황량호,기중최장존활이체7년.결론 응용간이식치료미산형Caroli병구유량호적림상효과.
Objective To investigate the clinical effects of liver transplantation including living related liver transplantation for Caroli's disease (CD). Methods Seven consecutive patients with diffused type of Caroli's disease had undergone liver transplantation (LT) from September 1999 to February 2007 in our single center. The clinical characteristics and survival of these patients were retrospectively reviewed. Results All 7 patients were diagnosed as Caroli's disease with diffused type which manifested recurrent cholangitis in clinical symptoms. Among them, 4 were female and 3 male.The mean age was 16 years old (ranging from 10 to 31 years old). Six patients were subjected to conservative therapy and only one patient had previously undergone cholecystectomy and T tube drainage before transplantation. In types of surgery, 4 patients accepted split liver transplantation with right liver lobe, two got whole liver transplantation and only one underwent living related liver transplantation. In two patients venovenous bypass was done during the operation. The mean duration of surgery was 9. 1 h. Post-transplant complications included pulmonary infection (3 cases), acute rejection (2 cases), pleural effusion (2 cases) and biliary leakage in the split section of donor liver (1 case). One patient died within 19 days caused by acute renal failure and multiple organs dysfunction.The rest six patients are alive without any signs of recurrence of protopathy and the longest survival time is 7 years. Conclusion Liver transplantation is a valuable treatment to Caroli's disease with diffused type. Due to the organ shortage, living related liver transplantation may own identical effects on LT.