影响难治型原发性胆汁性肝硬化疗效的因素分析
Baseline analysis of refractory primary biliary cirrhosis
目的 总结难治型原发性胆汁性肝硬化(PBC)的特点,比较其与典型PBC患者的异同,探索影响疗效的危险因素.方法 对60例2009年8月至2011年5月北京协和医院随诊1年以上的PBC患者,根据对熊去氧胆酸(UDCA)应答情况,应答良好患者为典型组(37例),应答不良者为难治型组(23例),分析初治时两组临床资料的异同.结果 难治型PBC患者初治时瘙痒(73.9%比45.9%)、乏力(60.9%比40.5%)、黄疸(43.4%比18.9%)发生率高于典型PBC患者(P<0.05);难治型PBC患者在初治时有较高的血清丙氨酸氨基转移酶(ALT)、门冬氨酸氨基转移酶(AST)、碱性磷酸酶(ALP)、γ-谷氨酰转肽酶(GGT)、总胆红素(TBIL)和直接胆红素(DBIL)水平,且IgM高于典型组,而IgG较低(P<0.05);难治型和典型PBC患者在年龄、性别、抗核抗体(ANA)、抗线粒体抗体(AMA)及M2亚型(AMA-M2)、其他相关抗体(如抗GP210、抗SP100等)以及肝脏病理分期方面无统计学差异.结论 初治时有瘙痒、乏力、黄疸等临床表现的,ALT、AST、ALP、GGT、TBIL和DBIL、IgM 水平较高的PBC患者可能对UDCA应答不良.
更多Objective To summarize the clinical characters of refractory primary biliary cirrhosis (PBC) versus typical ones and explore the risk factors of prognosis.Methods From 2009 to 2011,60 PBC patients on the therapy of ursodeoxycholic acid (UDCA) over 1 year at our clinic were recruited.According to the response to UDCA by the Paris criteria,they were divided into refractory group (n =23) and typical group (n =37) to analyze the basic clinical conditions,biochemical markers,antibodies and liver biopsies.Results (1) Compared with the typical patients,the refractory ones had more fatigue (73.9% vs 45.9%),pruritus (60.9% vs 40.5%) and jaundice (43.4% vs 18.9%); (2)the serum level of biomarkers (alanine aminotransferase (ALT),aspartate aminotransferase (AST),alkaline phosphatase (ALP),γ-glutamyl transpeptidase (GGT),total bilirubin (TBIL),direct bilirubin (DBIL) and immunoglobulin M (IgM)) were higher in the refractory group (P < 0.05) ; (3) no differences existed between two groups in age,gender,antibodies (antinuclear antibody (ANA),antimitochondrial antibody (AMA),AMA-M2,anti-GP210,anti-SP100 and anti-centromere antibody (ACA)) or liver biopsies.Conclusion Some clinical severe manifestation (fatigue,pruritus and jaundice) and higher biochemical markers levels (ALT,AST,ALP,GGT,TBIL,DBIL and IgM) seem to predict a worse response to UDCA and a rapid progression of disease in PBC patients.
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