肝移植术治疗原发性胆汁性肝硬化的疗效分析

Liver Transplantation for primary biliary cirrhosis

目的总结肝移植术治疗原发性胆汁性肝硬化的临床特点,提高肝移植术治疗该病的认识和疗效。方法回顾性分析2003年12月至2005年11月完成的7例原发性胆汁性肝硬化的患者资料并结合文献进行讨论。结果7例原发性胆汁性肝硬化患者,占同期施行肝移植治疗患者的1.55%(7/452)。女6例,男1例,平均年龄为50.7±10.9岁,术前平均血清胆红素345.8±88.8μmol/L,child分级B级2例,C级5例。3例在术后1月内出现急性排斥反应,加强免疫抑制治疗后治愈。激素撤退时间5例为术后6月,1例长期服用激素。随访5天～36月,6例痊愈,1例患者死于多器官功能衰竭,2年存活率为85.7%,未发现复发病例。结论肝移植术是治疗终末期原发性胆汁性肝硬化有效治疗手段。术后早期应加强免疫抑制治疗,减少急性排斥反应的发生。

Objective To evaluated the clinical features and outcome of liver transplantation (LT)for primary biliary cirrhrosis (PBC). Methods Clinicopathological data of 7 cases with PBC who underwent liver transplantation in our transpalatation center were analyzed retrospectively. Results Among 452 patients who underwent liver transplantation from Dec 2003 to Oct 2005, 7(1.55%) suffered from PBC. The median age of the 6 women and 1 men was (50.7±10.9)years. the mean serum bilirubin was 345.8±88.8μmol/L before liver transplantation. Primary immunosuppression consisted of cyclosporine (n = 2) or tacrolimus (Tac) (n = 4). Corticosteroids were withdrawn six months after OLT, only 1 patient must use prednisolone perpetually for ever elevated serum bilirubin. 3 cases suffered from episodes of acute cellular rejection within one month after liver transplantation but cured when strengthened immunosuppression therapy. The follow-up after liver transplantation was ranged from 5 days to 36months, 1 case died from multiple organ failure and 6 cases cured. Actuarial patient survival after 2 years was 85.7%, no recurrent PBC case was found. Conclusion Orthotopic liver transplantation is the only effective curative therapy for end-stage primary biliary cirrhosis. Immunosuppression therapy should be strengthened on early stage after transplantation to avoid episodes of acute cellular rejection。