肝移植治疗肝豆状核变性的效果观察

毛家玺; 邹游; 郭闻渊

doi:10.3969/j.issn.1001-5256.2017.10.026

肝移植治疗肝豆状核变性的效果观察

DOI: 10.3969/j.issn.1001-5256.2017.10.026

第二军医大学长征医院器官移植科全军器官移植研究所第二军医大学器官移植中心

基金项目:

国家自然科学基金面上项目(31370896)；

详细信息

中图分类号: R657.3;R742.4
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出版历程
- 收稿日期: 2017-04-24
- 出版日期: 2017-10-20

Clinical effect of liver transplantation in the treatment of hepatolenticular degeneration

Department of Organ Transplantation, Changzheng Hospital, Second Military Medical University, PLA Institute of Organ Transplantation, Center of Organ Transplantation, Second Military Medical University

Research funding:

摘要

摘要:
目的观察肝移植治疗肝豆状核变性（HLD）的疗效及预后。方法对第二军医大学长征医院2003年6月-2017年1月因HLD行肝移植手术的15例患者的临床资料进行回顾性分析。计量资料采用多个相关样本的非参数检验中的Friedman检验,组内采用两相关样本的非参数检验中的Wilcoxon秩检验。结果 15例患者中行经典原位式肝移植13例,改良背驮式肝移植2例。围手术期因多器官功能衰竭死亡1例,2例患者术后半年后失访,1例患者术后存活14个月,1例患者术后存活5年8个月,余10例患者存活至今。术后发生急性排斥反应6例,予以常规激素冲击治疗后4例好转,余2例中1例合并巨细胞病毒感染,予以更昔洛韦抗病毒之后好转出院,另1例激素冲击疗效不佳,后又出现慢性排斥反应,于术后5个月行再次肝移植,术后恢复良好出院。术前10例K-F环阳性患者皆有不同程度改善,消失或变淡。术前3例严重精神神经症状的患者也得到了不同程度的缓解,术后半年至1年基本恢复正常。除1例围手术期死亡外,余14例术后1个月肝功能较术前明显改善,血清铜蓝蛋白水平明显升高,恢复至正常水平。结论原位肝移植术能有效改善HLD患者的铜代谢功能、肝功能状...
- 肝豆状核变性 /
- 肝移植 /
- 治疗结果
Abstract:
Objective To investigate the clinical effect of liver transplantation in the treatment of hepatolenticular degeneration ( HLD) and the prognosis of HLD patients. Methods A retrospective analysis was performed for the clinical data of 15 HLD patients who underwent liver transplantation in Changzheng Hospital of Second Military Medical University from June 2003 to January 2017. Friedman test of K related samples test was used for comparison of data between groups, and Wilcoxon one-sample test of 2 related samples test was used for comparison of data between two groups. Results Of all patients, 13 underwent standard orthotopic liver transplantation, and 2 underwent modified piggyback liver transplantation. One patient died of multiple organ failure during the perioperative period and 2 were lost to follow-up at half a year after surgery. Of all patients, one lived for 14 months, one lived for 5 years and 8 months, and the other ten patients were still alive up to now. Six patients suffered from acute rejection reaction ( AR) , among whom four were improved after conventional hormone pulse therapy, one was improved and discharged after antiviral therapy with ganciclovir for cytomegalovirus infection, and the other one patient was improved and discharged after the second liver transplantation at 5 months after surgery due to poor response to hormone pulse therapy and chronic rejection reaction. Ten patients had varying degrees of improvement in positive Kayser-Fleischer ring, and three patients achieved varying degrees of improvement in severe neuropsychiatric symptoms and had a basically normal neuropsychiatric state at half a year to one year after surgery.One patient died in the perioperative period, and the other 14 patients had a significant improvement in liver function at 1 month after surgery, and the level of ceruloplasmin gradually increased to a normal level. Conclusion Orthotopic liver transplantation can effectively improve copper metabolism, liver function, and neuropsychiatric symptoms in HLD patients and increase their quality of life and long-term survival rates.
- hepatolenticular degeneration /
- liver transplantation /
- treatment outcome

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