原发性胆汁性肝硬化41例临床病例分析

Clinical features of 41 patients with primary biliary cirrhosis

目的分析原发性胆汁性肝硬化(PBC)患者的临床特征,提高对该病的认识。方法回顾性分析41例原发性胆汁性肝硬化患者患者的临床特征、生化指标和组织学特点以及熊去氧胆酸的治疗反应。结果41例原发性胆汁性肝硬化患者中女性与男性之比为9:1,主要临床表现为皮肤瘙痒、乏力、纳差和黄疸,所有患者碱性磷酸酶(ALP)与γ-谷氨酰转肽酶(GGT)均明显升高。40例(97.6%)患者的抗线粒体抗体(AMA)及AMA-M2亚型抗体阳性。共有24例患者行肝穿刺病理检查,早期(Ⅰ、Ⅱ期)占83.3%,晚期(Ⅲ、Ⅳ期)占16.7%。41例患者经过熊去氧胆酸(UDCA)治疗6月后,生化指标较治疗前均有明显改善。结论PBC主要累及中年女性,其特征为碱性磷酸酶与γ-谷氨酰转肽酶升高和AMA及M2亚型阳性,熊去氧胆酸治疗能有效改善肝功能。

Objective To improve diagnosis and treatment of primary biliary cirrhosis （PBC）, clinical features of the patients with PBC were analyzed. Methods Clinical data of 41 cases with primary biliary cirrhosis were reviewed including the clinical manifestation, 1 aboratory test, pathological finding, and the response to UDCA therapy. Results The ratio of female to male was 9： 1. The most frequently re-ported symptoms were pruritus ,fatigue, anorexia and jaundice. The levels of ALP and GGT of all patients were elevated. 40 patients （97.6%） have positive antimitochondrial antibody （AMA）/AMA-M2. Of 24 patients undergone liver biopsy ,20 patients were in stage I or Ⅱ and 4 were in stage Ⅲ or Ⅳ. After treat- ment with Ursodeoxycholie acid （UDCA） for 6 months,liver function was significantly improved. Conclusions PBC most frequently affects middle-aged women, characterised with elevated ALP, GGT and positive AMA/AMA-M2. Treatment with UDCA could significantly improve liver function of PBC.