36例原发性胆汁性肝硬化的临床特征分析

Clinical characteristic analysis of 36 patients with primary biliary cirrhosis

目的探讨原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)患者的临床特征,以提高对PBC的诊断与治疗水平。方法对具有完整资料的36例PBC的一般资料、从出现症状或肝功改变到确诊的时间、临床表现、血清化学指标、免疫学、影像学、病理学改变及治疗转归等进行回顾性分析。结果本组36例PBC患者中女性33例(91.7%)。从出现症状或肝功改变至确诊的平均时间为18.95个月,最长82个月。临床表现主要为乏力(80.6%)、皮肤瘙痒(69.4%);最主要的体征为黄疸(61.1%)和脾大(61.1%);AMA/AMA-M2阳性率达91.67%;r-谷胺酰转肽酶(GGT)、总胆红素(TBIL)、直接胆红素(DBIL)和血清白蛋白(ALB)在不同的临床分期中差异有统计学意义(P<0.05)。肝硬化前期组经熊去氧胆酸(UDCA)治疗前后肝功的对比差异有统计学意义(P<0.05),而肝硬化期组此项对比差异无统计学意义(P>0.05)。结论 PBC以中年女性多见,高滴度AMA及AMA-M2亚型是诊断PBC的主要指标。肝硬化期GGT、TBIL和DBIL的水平明显高于肝硬化前期,而ALB水平反而明显降低。该病病程较长,在早期主要表现为原发性胆汁性胆管炎,需较长时间才发展为原发性胆汁性肝硬化。以UDCA为主的综合治疗能够明显改善早期患者的症状和血清胆汁淤积指标。因此早期发现、早期治疗效果较好。

Objective To study the clinical features of primary biliary cirrhosis （PBC）, and to improve the clinical diagnosis and therapy. Methods General information of 36 cases with complete data PBC, time from the onset of symp- toms or liver function changes to diagnosis, clinical manifestation, serum chemistry index, immunology, imaging, patho- logical changes and treatment outcome were retrospectively analyzed. Results 33 patients were female （91. 7% ）. Mean time from the onset of symptoms or liver function change to diagnosis was 18.95 months, the longest was 82 months. The most frequent symptoms were fatigue （80.6%） , pruritus （69.4%） ; the major signs included jaundice （61. 1% ） , splenomegaly （61. 1% ） ; AMA/AMA-M2 positive rate was 91. 67% , r-glutamic acid transpeptidase （GGT） , total bilirubin （TBIL） , direct bilirubin （DBIL） and serum albumin （ALB） in different clinical stages had sig- nificant difference （P 〈 0.05）. Liver function contrast had significant difference before and after treatment by ursode- oxycholic acid （UDCA） in early liver cirrhosis （P 〈 0.05） , while in the cirrhosis stage group, this comparison showed no statistical difference （P 〉 0.05）. Conclusion Middle-aged women in PBC is common, high titer of AMA and AMA-M2 subtype are the main indices of PBC diagnosis. Liver GGT, TBIL and DBIL levels in cirrhosis were significant- ly higher in early liver cirrhosis, but the level of ALB decreased obviously. The disease course is long. UDCA based in- tegrated treatment can significantly improve patients with early symptoms and serum cholestasis index.