【摘要】 目的分析56例原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)的临床、生化及病理学特点,探讨免疫学指标和肝穿病理对临床诊断的意义。方法对56例临床诊断为原发性胆汁性肝硬化患者的一般资料、临床表现、生化指标、免疫学及病理学等进行分析。结果 56例单纯PBC中女性占80.4%,平均年龄(49.1±13.1)岁。临床主要表现为乏力(26/56,46.4%)、黄疸(20/56,35.7%)、右上腹不适(19/56,33.9%),皮肤瘙痒(9/56,16.1%)。其中9例无明显自觉症状,AMA-M2阳性检出率为55.4%(31/56)。其中AMA-M2阳性组和AMA-M2阴性组均以ALP、γ-GT、lgM升高为主,两组的生化及免疫学指标(除ALP、γ-GT、IgA外)比较无统计学意义;病理学表现为胆管周围炎和小胆管损伤,两组皆以Ⅰ、Ⅱ期改变为主(54.8%&52%)。结论 AMA-M2阴性PBC(AIC)可能是AMA-M2阳性PBC的另一表现形式,而不是另一种不同的疾病。GP210对AMA-M2阳性和AMA-M2阴性的PBC特异性均强。对于AMA-M2阴性PBC和AMA-M2阳性PBC,肝穿病理学检查具有重要临床价值。更多还原

【Abstract】 Objective To review the clinical,biochemical and pathological characters of patients with primary biliary cirrhosis(PBC).Methods Fifty-six cases with PBC were analyzed including the clinical features,biochemical results,immune detection and pathological examination.Results In all 56 cases of PBC,there were 45 females(80.4%),with an average age of(49.1±13.1) years.The clinical symptoms included fatigue(26/56,46.4%),jaundice(20/56,35.7%),and uncomfortableness in the right upper abdominal(19/56,33.9%).All cases had no clinical signs.The positive rate of AMA-M2 detection was 55.4%(31/56).Serum,ALP,γ-GT,and IgM were increased in both AMA-M2 positive group and AMA-M2 negative patients.There was no significant difference between the positive group and negative group in biochemical and immune measurements(expect ALP,γ-GT and IgA) between two groups.The pathological examination showed perio-cholangitis and small bile ducts injury,I stage andⅡstage were most common in both two groups(54.8%and 52%).Conclusion AMA-M2 negative PBC(AIC) may be another form of AMA-M2 positive PBC,it is not diagnosed with another disease.GP210 detection has specificity to both AMA-M2 positive and AMA-M2 PBC.Liver biopsy and pathological examination are important procedures in diagnosis of AMA-M2 positive and negative PBC.更多还原