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肝移植救治严重多囊肝1例报告并文献复习(英文)
引用本文:张海斌,司马辉,付雍,杨宁,杨广顺.肝移植救治严重多囊肝1例报告并文献复习(英文)[J].器官移植,2012,3(1):20-23.
作者姓名:张海斌  司马辉  付雍  杨宁  杨广顺
作者单位:第二军医大学附属东方肝胆外科医院肝外五科,上海,200438
基金项目:Medical Guide Foundation of Shanghai(10411963200)~~
摘    要:目的探讨肝移植治疗常染色体显性多囊肝(autosomal dominant polycystic liver disease,APLD)重症病例的疗效。方法 1例严重的APLD患者(只有右叶边缘2个肝段仍有肝组织),经皮穿刺乙醇硬化治疗效果不佳,予肝部分切除并开窗术,但出现严重的术后并发症。首次术后8个月患者接受了原位肝移植。结果肝移植术后随访26个月,患者恢复良好。结论对于肝部分切除并开窗术治疗无效的严重APLD患者,原位肝移植是有效的治疗方法。

关 键 词:常染色体显性多囊肝  肝切除  开窗术  肝移植

Severe polycystic liver disease rescued by liver transplantation: a case report and literature review
ZHANG Hai-bin,SIMA Hui,FU Yong,YANG Ning,YANG Guang-shun.Severe polycystic liver disease rescued by liver transplantation: a case report and literature review[J].Ogran Transplantation,2012,3(1):20-23.
Authors:ZHANG Hai-bin  SIMA Hui  FU Yong  YANG Ning  YANG Guang-shun
Institution:Department of 5th Hepatic Surgery,Eastern Hepatobiliary Hospital,the Second Military Medical University,Shanghai 200438,China
Abstract:Objective To evaluate the efficacy of liver transplantation for severe autosomal dominant polycystic liver disease (APLD).Methods A patient with severe APLD (only two liver segments left in the periphery of the right lobe)was treated by percutaneous aspiration and ethanol sclerotheraphy but in vain.She was then treated by partial hepatectomy and fenestration, but developed severe postoperative complications.Orthotopic liver transplantation (OLT) had to be performed 8 months after the first operation.Results A 26-month follow-up showed that the patient recovered well.Conclusions OLT is an effective method to cure severe APLD patient,who treated by partial hepatectomy and fenestration but in vain.
Keywords:Autosomal dominant polycyst liver disease  Partial hepatectomy  Fenestration  Liver transplantation
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