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目的对70例原发性胆汁性肝硬化(PBC)患者的临床特征进行分析,提高对该病的认识。方法观察分析70例PBC患者的临床特征、各项生化指标和组织学特点并进行统计学分析。结果70例PBC患者中女性与男性之比为8∶1,平均年龄(53.1±10.4)岁。临床主要表现为皮肤瘙痒(60.0%)、乏力(42.9%)、纳差(41.4%)、黄疸(45.7%)。所有患者碱性磷酸酶(ALP)与γ谷氨酰转肽酶(GGT)均明显升高,65例患者(92.9%)胆红素有不同程度的升高。44例患者(62.9%)血清IgM升高。98.6%的患者抗线粒体抗体(AMA)及AMAM2亚型抗体阳性。共有41例患者行肝穿刺病理检查,早期(Ⅰ、Ⅱ期)占82.9%,晚期(Ⅲ、Ⅳ期)占17.1%。结论PBC主要累及中年女性,主要表现为皮肤瘙痒、乏力、纳差和不同程度黄疸,血清ALP和GGT水平升高、AMA及AMAM2亚型抗体阳性、血清IgM、IgG升高,该病应早诊断、早治疗。
Objective To analyze the clinical features of 70 patients with primary biliary cirrhosis (PBC) to improve their understanding of the disease. Methods The clinical features, biochemical and histological features of 70 patients with PBC were observed and analyzed statistically. Results The ratio of female to male in 70 PBC patients was 8: 1, with an average age of (53.1 ± 10.4) years. Clinical manifestations of pruritus (60.0%), fatigue (42.9%), anorexia (41.4%), jaundice (45.7%). Alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (GGT) were significantly increased in all patients, and 65 patients (92.9%) had different degrees of bilirubin. Serum IgM was elevated in 44 patients (62.9%). 98.6% patients were positive for anti-mitochondrial antibody (AMA) and AMAM2 subtype antibodies. A total of 41 patients underwent liver biopsy, accounting for 82.9% in early stages (stage I and stage II) and 17.1% in late stage (stages III and IV). Conclusions PBC mainly affects middle-aged women. The main manifestations are pruritus, fatigue, anorexia and jaundice, serum ALP and GGT levels are elevated, AMA and AMAM2 subtype antibodies are positive, and serum IgM and IgG are elevated. The disease should be early Diagnosis, early treatment.