肝移植治疗原发性胆汁性肝硬化的远期效果观察

Long term follow-up of patients with primary biliary cirrhosis after liver transplantation

目的观察肝移植治疗原发性胆汁性肝硬化（PBC）的远期效果。方法对15例接受了肝移植的终末期PBC患者进行长期随访，中位随访时间为70个月（38-86个月），记录并总结随访期间患者的相关资料，分析肝移植术后患者的预后、新发疾病以及术后远期存活率。结果15例PBC患者共接受原位肝移植16例次，其中1例因原发性移植肝无功能进行了2次肝移植。术后患者均采用环孢素A（或他克莫司）＋霉酚酸酯＋激素的三联免疫抑制方案，部分患者使用了熊去氧胆酸。术后2周时，患者肝功能指标基本恢复正常，乏力、皮肤瘙痒及黄疸等症状缓解，患者的生存质量明显改善。术后有4例患者出现新发疾病（26．7％），1例为乙型肝炎病毒感染，2例为自身免疫性肝炎，1例为结肠癌，经治疗后2例好转，2例治疗无效死亡。肝移植术后，患者无PBC复发，1、2和5年的存活率分别为100％、100％和86．7％。结论肝移植可提高终末期PBC患者的生存质量和存活率，但一些少见的新发疾病对患者的远期存活率影响较大。

Objective To study the long term efficacy of orthotopic liver transplantation （OLT） to treat primary biliary cirrhosis （PBC）. Methods Fifteen patients after OLT because of end-stage PBC were followed up for a median time of 70 months （38-86 months）. The prognosis, de novo diseases and long term survival of the patients after OLT were analyzed. Results Fifteen PBC patients received OLT for 16 times, including one patient received re-transplantation because of primary transplant dysfunction. All patients were treated with immunosuppressive agents including cyclosporine A （or tacrolimus）, mycophenolate mofetil and prednisone after OLT, and some patients were treated with ursodeoxycholic acid at the same time. The liver function tests were normalized two weeks after OLT, and the symptoms of fatigue, pruritus and jaundice were improved significantly. All patients showed improved quality of life. During the follow up, there were 4 patients （26. 7 %） diagnosed with de novo diseases, including one patient with hepatitis B virus infection, 2 patients with autoimmune hepatitis, and one patient with colon cancer. Two patients were improved after the treatment, but the other two patients died. No PBC recurrence was found during the follow-up. The overall 1-, 2- and 5-year survival rate was 100 %, 100 % and 86. 7 %, respectively. Conclusions OLT can improve the quality of life and survival of end-stage PBC patients. Some rare de novo diseases after OLT may impact the long term survival of the patients.