中国组织工程研究

中国组织工程研究 ›› 2011, Vol. 15 ›› Issue (53): 9897-9900.doi: 10.3969/j.issn.1673-8225.2011.53.005

• 肝移植 liver transplantation • 上一篇    下一篇

肝移植治疗肝豆状核变性

吕少诚，史宪杰，梁雨荣，何  蕾，纪文斌，芦  芳，陈永卫，罗  英   

  1. 解放军总医院肝胆外科，北京市  100853
  • 收稿日期:2011-10-28 修回日期:2011-11-12 出版日期:2011-12-31 发布日期:2011-12-31
  • 通讯作者: 史宪杰，主任医师，解放军总医院肝胆外科，北京市100853 shixianjie301@yahoo.com.cn
  • 作者简介:吕少诚★，男，1987年生，河南省驻马店市人，回族，解放军军医进修学院在读硕士，主要从事肝移植及围手术期处理研究。 shaocheng0502@163.com

Liver transplantation for Wilson’s disease

Lü Shao-cheng, Shi Xian-jie, Liang Yu-rong, He Lei, Ji Wen-bin, Lu Fang, Chen Yong-wei, Luo Ying   

  1. Department of Hepatobiliary Surgery, General Hospital of Chinese PLA, Beijing  100853, China
  • Received:2011-10-28 Revised:2011-11-12 Online:2011-12-31 Published:2011-12-31
  • Contact: Shi Xian-jie, Chief physician, Department of Hepatobiliary Surgery, General Hospital of Chinese PLA, Beijing 100853, China Shixiejie301@yahoo.com.cn
  • About author:Lü Shao-cheng★, Studying for master’s degree, Department of Hepatobiliary Surgery, General Hospital of Chinese PLA, Beijing 100853, China Shaocheng0502@163.com

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摘要：

背景：肝移植能够为肝豆状核变性患者提供正常的肝组织以纠正其遗传缺陷，改善铜代谢、肝硬化所致的各种临床症状及神经精神症状。
目的：观察肝移植治疗肝豆状核变性的效果。
方法：选择17例肝豆状核变性患者，3例患者行尸体肝移植，14例患者行亲体肝移植，所有肝移植患者动脉重建采用供肝动脉与受者肝固有动脉端端吻合，胆道重建均采用胆道端端吻合，术中均未放置胆道支架管支撑引流。
结果与结论：围手术期死亡1例，生存时间超过1年15例，超过3年10例。8例伴有神经系统功能障碍患者中，6例症状得到不同程度改善。生存时间超过1年的15例患者移植后肝功能较移植前明显改善，血清铜蓝蛋白水平明显升高 (P < 0.05)。证实肝移植可改变肝豆状核变性患者的代谢缺陷，是治疗肝豆状核变性的有效方法。

关键词: 肝豆状核变性, 肝移植, 治疗, 诊断, 铜蓝蛋白, 存活

Abstract:

BACKGROUND: Liver transplantation can provide healthy liver tissue for Wilson’s disease patients to correct their genetic defects, and it can relive clinical and neuropsychiatric symptoms induced by copper metabolism and cirrhosis.
OBJECTIVE: To investigate the effects of liver transplantation on Wilson’s disease.
METHODS: Seventeen cases were selected. Three cases were treated with cadaveric liver transplantation, and 14 cases were treated with living-related liver transplantation. In all the cases, artery reconstruction was conducted through end-to-end anastomosis between donor hepatic artery and recipient hepatic artery. The duct-to-duct biliary reconstruction was performed, and no support pipe was placed in the biliary duct for drainage during operation.
RESULTS AND CONCLUSION: One patient died of hemorrhage perioperatively. Fifteen patients survived more than 1 year and 10 patients survived more than 3 years. Of the eight patients with nervous system dysfunction, six patients had improved neuropsychic symptoms to various degrees after operation. The liver function and serum ceruloplasmin level were improved significantly in the 15 patients undergoing liver transplantation surviving more than 1 year (P < 0.05). It suggests that liver transplantation can correct patients’ metabolic defects, which is an effective therapy for Wilson’s disease.

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