中国组织工程研究

中国组织工程研究 ›› 2012, Vol. 16 ›› Issue (31): 5886-5890.doi: 10.3969/j.issn. 2095-4344.2012.31. 037

• 器官移植综述 organ transplantation review • 上一篇    

肝移植治疗肝脏遗传性出血性毛细血管扩张症1例

朱雄伟，刘 煜，王 毅，路 宾，陈新国，陈 虹，沈中阳，臧运金   

  1. 武装警察部队总医院肝移植研究所，北京市 100039
  • 收稿日期:2011-12-14 修回日期:2011-12-19 出版日期:2012-07-29 发布日期:2012-07-29
  • 作者简介:朱雄伟☆，男，1972年生，浙江省义乌市人，汉族，2004年解放军第二军医大学毕业，博士，主治医师，主要从事肝、肾等器官移植、肝胆胰外科研究。

Liver transplantation treats hepatic hereditary hemorrhagic telangiectasia in one case

Zhu Xiong-wei, Liu Yu, Wang Yi, Lu Bin, Chen Xin-guo, Chen Hong, Shen Zhong-yang, Zang Yun-jin   

  1. Institute of Liver Transplantation, General Hospital of Chinese People’s Armed Police Forces, Beijing 100039, China
  • Received:2011-12-14 Revised:2011-12-19 Online:2012-07-29 Published:2012-07-29
  • About author:Zhu Xiong-wei☆, Doctor, Attending physician, Institute of Liver Transplantation, General Hospital of Chinese People’s Armed Police Forces, Beijing 100039, China zhuxiongwei@126. com

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摘要：

背景：肝脏遗传性出血性毛细血管扩张症是一种少见病，可引起多种威胁生命的并发症。肝移植是惟一根治性选择，但有关肝移植治疗肝脏遗传性出血性毛细血管扩张症的疗效罕有报道。
目的：探讨肝移植对肝脏遗传性出血性毛细血管扩张症的疗效。
方法：回顾性分析了中国武警总医院肝移植研究所1例肝脏遗传性出血性毛细血管扩张症女性59岁患者行肝移植的临床资
料，患者肝移植后共随访8个月，对肝移 植后8个月内的肝功能和腹部超声进行严密观察。
结果与结论：患者肝移植后身体状况良好。肝移植对肝脏遗传性出血性毛细血管扩张症的治疗有效，对其长期生存效果满意，且应在该疾病出现威胁生命的症状前进行肝移植。尽量避免对肝脏遗传性出血性毛细血管扩张症进行姑息性介入治疗，尤其是对肝动脉的介入治疗措施，因为姑息性介入治疗可能增加其并发症发生率，但远期疗效有待于进一步评估。

关键词: 遗传性出血性毛细血管扩张症, 肝脏, 肝移植, 诊断, 治疗, 预后

Abstract:

BACKGROUND: Hepatic hereditary hemorrhagic telangiectasia (HHHT) is a rare disease, which can lead to life-threatening complications. Liver transplantation has been an only curative option; however, the therapeutic effect of liver transplantation in HHHT is rarely reported.
OBJECTIVE: To explore the therapeutic effect of liver transplantation in patients with HHHT.
METHODS: The clinical data of one 59-year-old female patient with HHHT, who had undergone orthotropic liver transplantation at the Institute of Liver Transplantation of General Hospital of Chinese People’s Armed Police Forces, China was analyzed retrospectively. The period of follow-up was 8 months. Abdominal ultrasonography and liver function were observed after operation.
RESULTS AND CONCLUSION: This female patient had been in good health during 8-month follow-up after liver transplantation. She resumed family daily life. Liver transplantation can offer an effective therapy for patients with HHHT, and can provide satisfactory postoperative long-term results. Liver transplantation should be proposed earlier in the course of symptomatic HHHT presenting with life-threatening conditions. Palliative interventions, especially on the hepatic artery, should be avoided in view of their high (infectious) complication rate. However, the sample size was small with relatively short time follow-up. Thus, the long-term therapeutic effect still needs to be explored.

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