Kasahara Mureo, Kiuchi Tetsuya, Inomata Yukihiro, Uryuhara Kenji, Sakamoto Seisuke, Ito Takashi, Fujimoto Yasuhiro, Ogura Yasuhiro, Oike Fumitaka, Tanaka Koichi
Organ Transplant Unit, Department of Transplant Surgery, Kyoto University Hospital, Kyoto, Japan. mureo@kuhp. kyoto-u.ac.jp
Transplantation. 2003 Jun 27;75(12):2147-50. doi: 10.1097/01.TP.0000066804.33006.17.
Alagille syndrome (AGS) is an autosomal dominant genetic disorder characterized by chronic cholestasis, congenital heart disease, peculiar facies, butterfly-like vertebrae, and posterior embryotoxon. Liver dysfunction is the common presentation of AGS, and liver transplantation may be indicated. This study examines the outcome of living-related liver transplantation (LRLT) for AGS. Twenty patients with AGS (median age 5.0 years, range 0.6-12.9) underwent LRLT at Kyoto University Hospital between June 1990 and February 2002. Five potential donors were excluded because of paucity of intrahepatic bile ducts diagnosed by preoperative liver biopsy and one because of a hepatic vascular anomaly. The overall 5-year patient survival was 80.4%. Three patients died as the result of the following: complications related to surgery, heart failure caused by progressive pulmonary artery stenosis, and a graft with unsuspected bile duct paucity. Liver dysfunction was improved in all successful cases, and catch-up growth occurred in 90% of patients. LRLT is an efficacious treatment modality for AGS if donors are selected by cautious evaluation to rule out unsuspected bile duct paucity.
阿拉吉耶综合征（AGS）是一种常染色体显性遗传病，其特征为慢性胆汁淤积、先天性心脏病、特殊面容、蝶形椎骨及后胚胎毒素。肝功能障碍是AGS的常见表现，可能需要进行肝移植。本研究探讨了亲属活体肝移植（LRLT）治疗AGS的效果。1990年6月至2002年2月期间，20例AGS患者（中位年龄5.0岁，范围0.6 - 12.9岁）在京都大学医院接受了LRLT。5名潜在供体因术前肝活检诊断肝内胆管稀少而被排除，1名因肝血管异常被排除。患者总体5年生存率为80.4%。3例患者死于以下情况：手术相关并发症、进行性肺动脉狭窄导致的心力衰竭以及移植肝存在未被怀疑的胆管稀少。所有成功病例的肝功能障碍均得到改善，90%的患者实现了追赶生长。如果通过谨慎评估选择供体以排除未被怀疑的胆管稀少，LRLT是治疗AGS的一种有效治疗方式。
