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transplantation.Primary immunosuppression consisted of cyclosporine(n=2)or tacrolimus(Tac)(n=4).Corticosteroids were withdrawn six months after OLT,only 1 patient must use prednisolone perpetually for ever elevated serum bilirubin.3 cases suffered from episodes of acute cellular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。