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肝移植术后桥脑中央髓鞘溶解症5例报告
引用本文:易述红,李敏如,易慧敏,蔡常洁,安玉玲,杨扬,陆敏强,陈规划.肝移植术后桥脑中央髓鞘溶解症5例报告[J].器官移植,2010,1(2):77-80,87.
作者姓名:易述红  李敏如  易慧敏  蔡常洁  安玉玲  杨扬  陆敏强  陈规划
作者单位:中山大学器官移植研究所,中山大学附属第三医院肝脏移植中心,广州,510630
摘    要:目的探讨肝移植术后并发桥脑中央髓鞘溶解症(central pontine myelinolysis,CPM)的诊断和治疗。方法回顾性分析5例肝移植后并发CPM的临床资料和治疗经过。结果5例CPM患者均为男性,平均年龄49岁。原发病为慢性重症肝炎4例,原发性肝细胞癌1例,其中3例并发Ⅲ~Ⅳ度肝性脑病,3例并发严重肺部感染。CPM发生于术后4~13 d,平均发生时间为术后7 d,临床表现主要为闭锁综合征、肢体痉挛性瘫痪、缄默等。术前有明显低钠血症患者4例,发病时他克莫司血药浓度正常者4例,明显增高者1例。5例患者均经头颅磁共振成像检查诊断为CPM。采取病因治疗和对症处理相结合的治疗措施,重点防治并发症,其中两例由于早期合并水电解质、酸碱平衡紊乱,给予持续的肾脏替代治疗(continuous renal replacement therapy,CRRT)。本组患者3例死亡,2例存活,但均存在神经系统损害。结论肝移植后并发CPM的原因是多方面的,预后差,尚无有效的治疗方法,早期发现并积极防治并发症是降低病死率的主要措施。

关 键 词:肝移植  桥脑中央髓鞘溶解症  桥脑  神经系统  脱髓鞘  低钠血症

Central pontine myelinolysis following liver transplantation:five cases report
Institution:YI Shu-hong, LI Min-ru, YI Hui-min, et al.( Liver Transplantation Center, Third Affiliated Hospital, Sun Yat-sen University, Guangzhou 510630, China)
Abstract:Objective To evaluate the diagnosis and treatment of central pontine myelinolysis (CPM) following liver transplantation. Methods The clinical data of five cases of CPM after liver transplantation were analyzed retrospectively. Results All of the 5 cases were male with a mean age of 49 years. The primary indications for liver transplantation were chronic severe hepatitis (n = 4 ) and hepatocellular carcinoma ( HCC, n = 1 ) . The patients developed CPM on day 4 to 13 days after transplantation. Clinical manifestation included locked-in syndrome, spastic paralysis, mutism and so on. Four cases had a significant byponatremia before the operation. All of the 5 cases were diagnosed as CPM by magnetic resonance image (MRI) , 2 cases cured, 1 cases died from severe infections and another 2 cases died from multiple organ dysfunction. Conclusion CPM after liver transplantation can be induced by multiple causes. There is no effective treatment for CPM at present and early diagnosis and prophylaxis of complications can reduce mortality.
Keywords:Liver transplantation  Central pontine myelinolysis  Pons  Nervous system  Myelinolysis  Hyponatremia
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