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儿童肝移植:多伦多的初步经验。

Liver transplantation in children: the initial Toronto experience.

作者信息

Superina R A, Pearl R H, Roberts E A, Phillips M J, Graham N, Greig P D, Langer B

机构信息

Department of Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.

出版信息

J Pediatr Surg. 1989 Oct;24(10):1013-9. doi: 10.1016/s0022-3468(89)80205-2.

Abstract

The Hospital for Sick Children's initial 2-year experience with pediatric liver transplantation is reviewed. Patients are divided into high- and low-risk groups according to certain criteria. The high-risk group includes patients under 10 kg in weight, those with extrahepatic biliary atresia (EHBA), those with portal vein atresia or thrombosis, and those in hepatic coma. All others were considered low risk. Twenty-nine patients were assessed for transplantation: 18 were transplanted and 6 (21% of total referred) died while on the waiting list. Eighteen patients received 23 transplants. Of the 18 recipients, nine had EHBA, four had fulminant hepatic failure, two had tyrosinemia, one had glycogen storage disease, one had Indian childhood cirrhosis, and one had idiopathic cirrhosis. Seven of the 13 patients in the high-risk group survived (55% survival) with 1 to 23 month follow-up. Survival was significantly higher (80%) in the low-risk group (P less than 0.05). Four patients were retransplanted and two survived. Early deaths occurred from prolonged warm ischemia, recurrent portal vein thrombosis, and brain death in a patient who had been transplanted in hepatic coma. Late deaths occurred from cytomegalovirus (CMV) disease (2 patients), acute rejection (1 patient), and myocardial infarction (1 patient). The incidence of primary nonfunction was 4.3% (1 of 23) and of arterial thrombosis was 13% (3 of 23). Survival in patients transplanted for EHBA (67%) was slightly higher than it was for the rest of the group, although not as good as it was in the low-risk group.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

回顾了病童医院小儿肝移植最初两年的经验。根据某些标准将患者分为高风险组和低风险组。高风险组包括体重低于10公斤的患者、肝外胆道闭锁(EHBA)患者、门静脉闭锁或血栓形成患者以及肝昏迷患者。其他所有患者被视为低风险。对29名患者进行了移植评估:18名患者接受了移植,6名(占转诊总数的21%)在等待名单上死亡。18名患者接受了23次移植。在18名受者中,9名患有EHBA,4名患有暴发性肝衰竭,2名患有酪氨酸血症,1名患有糖原贮积病,1名患有印度儿童肝硬化,1名患有特发性肝硬化。高风险组的13名患者中有7名存活(存活率55%),随访时间为1至23个月。低风险组的存活率显著更高(80%)(P小于0.05)。4名患者接受了再次移植,2名存活。早期死亡原因包括长时间热缺血、复发性门静脉血栓形成以及一名在肝昏迷状态下接受移植的患者出现脑死亡。晚期死亡原因包括巨细胞病毒(CMV)疾病(2例)、急性排斥反应(1例)和心肌梗死(1例)。原发性无功能的发生率为4.3%(23例中的1例),动脉血栓形成的发生率为13%(23例中的3例)。因EHBA接受移植患者的存活率(67%)略高于该组其他患者,尽管不如低风险组。(摘要截选至250词)

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