原发性胆汁性肝硬化的临床分析：42例报告

Dai Yun, Liang Ying-hui, Xie Peng-yan, Chen Bao-wen, Liu Xin-guang

Department of Gastroenterology, Peking University First Hospital, Beijing 100034, China.

Beijing Da Xue Xue Bao Yi Xue Ban. 2005 Aug 18;37(4):410-4.

OBJECTIVE

To study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate cognition of the disease.

METHODS

Clinical data of 42 patients clinically and/or histologically diagnosed with PBC were reviewed. Anti mitochondrial antibody (AMA) negative/positive patients as well as the patients who were/were not associated with Sjogren Syndrome (SS) were compared in terms of clinical, biochemical and immunological features.

RESULTS

Among the 42 patients, 78.6% (33/42) of the cases were females; the mean age at diagnosis was (61.1+/-10.8) years. The most frequent symptoms were fatigue. Serum alkaline phosphatase (ALP), gamma-glutamyltranspeptidase (gamma-GT) and total bile acid (TBA) levels were markedly elevated in the majority of the patients, whereas ALT and AST levels were mildly to moderately elevated. Thirty-one patients had a total bilirubin (TBil) level above normal. The levels of TBil and prothrombin time had positive correlationship with years of the course (P=0.000, r=0.696; P=0.005, r=0.424), whereas serum albumin level had negative correlationship with years of the course (P=0.002, r=-0.462). Thirty-seven patients had elevated serum IgM and 34 patients were AMA/AMA-M(2) positive. AMA negative and AMA positive patients were similar in terms of clinical manifestations and liver biochemistries findings. Serum IgM and IgA levels were significantly lower, whereas total cholesterol level was higher in AMA negative patients when compared with AMA positive cases. Fifteen cases were associated with SS, which were similar in terms of clinical, biochemical and immunological features when compared with the PBC patients were not associated with SS.

CONCLUSION

PBC is mostly found in middle aged and old women. Elevated serum ALP, TBA and gamma-GT levels together with positive AMA/AMA-M(2) can help to diagnose PBC. AMA negative PBC patients are characterized by relatively lower serum IgM and IgA levels and higher total cholesterol level. PBC patients who are associated with SS have not substantial differences in the clinical, biochemical and immunological spectra of the disease.

目的

研究原发性胆汁性肝硬化（PBC）的临床特征，以增进对该疾病的认识。

方法

回顾42例临床和/或组织学诊断为PBC患者的临床资料。比较抗线粒体抗体（AMA）阴性/阳性患者以及合并/未合并干燥综合征（SS）患者的临床、生化和免疫学特征。

结果

42例患者中，78.6%（33/42）为女性；诊断时的平均年龄为（61.1±10.8）岁。最常见的症状是乏力。大多数患者血清碱性磷酸酶（ALP）、γ-谷氨酰转肽酶（γ-GT）和总胆汁酸（TBA）水平显著升高，而ALT和AST水平轻度至中度升高。31例患者总胆红素（TBil）水平高于正常。TBil水平和凝血酶原时间与病程呈正相关（P = 0.000，r = 0.696；P = 0.005，r = 0.424），而血清白蛋白水平与病程呈负相关（P = 0.002，r = -0.462）。37例患者血清IgM升高，34例患者AMA/AMA-M(2)阳性。AMA阴性和AMA阳性患者在临床表现和肝脏生化检查结果方面相似。与AMA阳性患者相比，AMA阴性患者血清IgM和IgA水平显著降低，而总胆固醇水平较高。15例患者合并SS，与未合并SS的PBC患者相比，其临床、生化和免疫学特征相似。