中国组织工程研究

中国组织工程研究 ›› 2013, Vol. 17 ›› Issue (18): 3267-3274.doi: 10.3969/j.issn.2095-4344.2013.18.006

• 肝移植 liver transplantation • 上一篇    下一篇

肝移植治疗肝豆状核变性

于秀涛1，江  艺1，2，张  坤2，杨  芳2，蔡秋程2   

  1. 1 福建医科大学福总临床医学院，福建省福州市  350025
    2 解放军南京军区福州总医院肝胆外科，福建省福州市  350025
  • 收稿日期:2012-09-04 修回日期:2013-01-14 出版日期:2013-04-30 发布日期:2013-04-30
  • 通讯作者: 江艺，博士，教授，博士生导师，福建医科大学福总临床医学院，福建省福州市 350025；解放军南京军区福州总医院肝胆外科，福建省福州市 350025 jiangyi183@yahoo.com.cn
  • 作者简介:于秀涛★，男，1984年生，山东省临沂市沂水县人，汉族，福建医科大学在读硕士，主要从事器官移植及门静脉动脉化的相关研究。 xiutao0522@126.com
  • 基金资助:

    福建省自然基金重点项目(2011y0046)。

Liver transplantation for the treatment of hepatolenticular degeneration  

Yu Xiu-tao1, Jiang Yi1, 2, Zhang Kun2, Yang Fang2, Cai Qiu-cheng2   

  1. 1 School of Clinical Medicine, Fuzhou General Hospital of Nanjing Military Region, Fujian Medical University, Fuzhou  350025, Fujian Province, China
    2 Department of Hepatobiliary Surgery, Fuzhou General Hospital of Nanjing Military Command, Fuzhou  350025, Fujian Province, China
  • Received:2012-09-04 Revised:2013-01-14 Online:2013-04-30 Published:2013-04-30
  • Contact: Jiang Yi, Doctor, Professor, Doctoral supervisor, School of Clinical Medicine, Fuzhou General Hospital of Nanjing Military Region, Fujian Medical University, Fuzhou 350025, Fujian Province, China; Department of Hepatobiliary Surgery, Fuzhou General Hospital of Nanjing Military Command, Fuzhou 350025, Fujian Province, China jiangyi183@yahoo.com.cn
  • About author:Yu Xiu-tao★, Studying for master’s degree, School of Clinical Medicine, Fuzhou General Hospital of Nanjing Military Region, Fujian Medical University, Fuzhou 350025, Fujian Province, China xiutao0522@126.com
  • Supported by:

    Key Project of Fujian Natural Science Foundation, No. 2011y0046

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596

被引次数

1

摘要：

背景：尽管近年来肝移植治疗肝豆状核变性取得了较大的进展，并且少量研究也乐观地提示这种改善能够持续稳定存在。但由于患者、医生以及其他不可控等因素，移植后患者的恢复情况也有所不同。
目的：总结解放军南京军区福州总医院肝移植病例资料，并对患者移植前伴随的神经系统的损害进行了长期随访。
方法：回顾性分析2005年7月至2010年5月因肝豆状核变性在解放军南京军区福州总医院接受肝移植治疗的9例患者临床资料，其中4例男性，5例女性，年龄14-44岁，平均年龄24.5岁。移植前9例患者的血清铜蓝蛋白均低于正常值(200-600 mg/L)，移植前5例患者存在神经病学症状。
结果与结论：8例行经典原位肝移植，1例行活体肝移植，其中1例原位肝移植患者于围手术期死亡，死因为多器官功能衰竭。其余8例患者肝功能均恢复顺利，移植后1个月总胆红素水平显著下降(P < 0.05)，白蛋白、血清铜蓝蛋白水平及血小板计数均显著升高(P值均< 0.05)；血铜水平有升高趋势，但差异无显著性意义(P > 0.05)；合并的神经病学症状得到不同程度的改善甚至消失；5例患者角膜K-F环移植后1个月有不同程度变淡，其中2例患者分别于移植后7个月和11个月角膜K-F环消失。结果可见通过全肝移植或活体肝移植，不仅能改善肝豆状核变性患者铜代谢，还能有效缓解神经病学症状。

关键词: 器官移植, 肝移植, 原位肝移植, 活体肝移植, 肝豆状核变性, K-F环, 铜转运蛋白, 围手术期, 肝功能, Child-Pugh分级, 多器官功能衰竭, 省级基金

Abstract:

BACKGROUND: In recent years, the treatment of hepatolenticular degeneration with liver transplantation has achieved great improvement, and a small amount of researches also optimistic reminder that the improvement can be sustained stably. Because of the patients, doctors, and other uncontrollable factors, the recovery of the patients after transplantation is different.
OBJECTIVE: To review the cases with liver transplantation in Department of Hepatobiliary Surgery, Fuzhou General Hospital of Nanjing Military Command, and to long-term follow-up the patients with nerves system damage before transplantation. 
METHODS: From July 2005 to May 2010, nine cases (four males and five females) with a mean age of 24.5 years (14-44 years) undergoing liver transplantation for hepatolenticular degeneration were selected from Fuzhou General Hospital of Nanjing Military Command, and the clinical data of the nine patients were retrospective analyzed. All the recipients had low serum levels of ceruloplasmin (200-600 mg/L) before transplantation, and five of them demonstrated the neurological manifestations.
RESULTS AND CONCLUSION: There were eight patients underwent orthotopic liver transplantation and one patient received living donor liver transplantation in this study. Eight recipients recovered satisfactorily with excellent liver functions, while one patient died of multiple organ failure during perioperative period. Compared to the levels before transplantationthe, total bilirubin levels of the eight patients were significantly declined (P < 0.05), the serum levels of albumin, ceruloplasmin and platelet were obviously increased (P < 0.05); the serum copper levels were increased, but the difference was not significant (P > 0.05); the neurological manifestations were also partly relieved or disappeared after liver transplantation. K-P loop was receded in five patients at 1 month after transpantation, and K-P loop was disappeared in two patients at 7 and 11 months after transplantation. Orthotopic liver transplantation or living donor liver transplantation cannot only improve the copper metabolism of the patients with hepatolenticular degeneration, but also can significantly relieve the severe neurological manifestations.

Key words: organ transplantation, liver transplantation, orthotopic liver transplantation, living donor liver transplantation, hepatolenticular degeneration, K-P loop, human copper transporter 1, perioperative period, liver function, Child-Pugh classification, multiple organ dysfunction syndrome, provincial grants-supported paper

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