原发性胆汁性肝硬化16例临床分析

Clinical analysis of 16 cases of primary biliary cirrhosis

目的分析原发性胆汁性肝硬化(PBC)的临床特征,提高对于该病诊治的认识。方法分析16例PBC患者的一般资料、临床表现、血清学、免疫学指标等改变。结果本组患者中男女之比为1∶4.3,确诊时平均年龄(52.6±9.9)岁。最常见症状为乏力(68.8%)、皮肤瘙痒(43.8%)。所有患者碱性磷酸酶(ALP)及谷氨酰转肽酶(GGT)明显增高,分别为(452.44±365.45)U/L和(385.25±192.67)U/L,10例(62.5%)患者血清总胆红素(TB)增高,血清丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)水平呈轻至中度升高,分别为(94.13±48.38)U/L及(101.5±73.58)U/L。10例患者血清IgM增高,所有患者血清抗线粒体抗体(AMA)或AMA-M2亚型阳性。结论PBC好发于中老年女性,长期慢性乏力、皮肤瘙痒,血清ALP、GGT、TB及血清IgM水平升高及AMA/AMA-M2阳性有助于诊断本病。

Objective To study the clinical features of primary biliary cirrhosis （PBC） in order to better handle the treatment of the disease. Method The general conditions, clinical manifestations, and the results of serologic and immunologic tests were assessed in 16 patients. Results The ratio of male to female was 1：4.3, the mean age at definite diagnosis was （52.6 ±9.9） years. The most frequent symptoms were fatigue （68.8%） and pruritus （43.8%）. Serum alkline phosphatase （ALP） and glutamyl transpeptidase （GGT） were markedly elevated [ （452.44 ± 365.45 ）U/L and （385.25 ± 192.67）U/L, respectively]. Total bilirubin （TB） levels were elevated in 10 patients （62.5%）. ALT and AST levels were mildly or moderately elevated [ （94.13 ±48.38） U/L and （ 101.5 ± 73.58） U/L , respectively]. IgM was also elevated in 10 patients. The positive rate of antimitochondrial antibody （AMA） or AMA -M2 subtype was 100%. Conclusions PBC mainly affects middle - aged women. Chronic fatigue, pruritus and the increase of serum ALP, GGT,TB, IgM and positive AMA or AMA -M2 subtype help to establish the diagnosis of primary biliary cirrhosis.