22例原发性胆汁性肝硬化的临床分析

目的 研究原发性胆汁性肝硬化（PBC）的临床特点、实验室检查、治疗转归，提高对PBC的认识。方法 分析22例PBC的临床表现、实验室检查及治疗转归。结果 22例PBC中女性20例，发病平均年龄51岁，主要症状包括皮肤瘙痒、乏力、纳差、腹痛，主要体征包括黄疸、肝大、脾大、腹水，实验室检查以ALP、高GGT、高胆红素血症、高球蛋白血症、存在多种自身抗体如抗线粒体抗体（AMA）、AMA－M2及抗核抗体（ANA）等，多数患者血ALT、AST升高，所有患者血清AST高于ALT。出现症状至临床确诊时间为2月－5年，平均8个月。治疗采用以熊去氧胆酸（UCDA）为主的综合方法，治疗3个月后ALP及TBil下降达50％以上者有12例，72．7％症状改善，死亡2例。结论 PBC以中年女性多，以肝脾肿大、黄疸、瘙痒、乏力为主要临床表现，肝功能异常以胆汁淤积为主，伴有高球蛋白血症及自身抗体；UCDA能够改善患者的症状和部分肝功能异常。

Clinical analysis of 22 patients with primary biliary cirrhosis

Objective To improve the diagnosis and management of primary biliary cirrhosis (PBC). Methods Clinical data of 22 cases of PBC were reviewed including the clinical manifestation, laboratory test, and the response to therapy. Results There were 20 female patients with an average of 50 years old in total of 22 PBC patients. The major symptoms were pruritus, fatigue, anorexia, and abdominal discomfort. The major signs included jaundice, hepatomegaly, splenmegaly, and ascites. Very high levels of serum alkaline phosphatase (ALP) and serum gamma glutamyl transpeptidase (GGT), hyperbilirubinemia and hypergammaglobulinemia were also detected in most of the patients. The aminotransferase level was only slightly elevated but the AST/ALT ratio was reversed. It took 8 months (ranging from 2 months to 5 years) to confirm the diagnosis after the clinical manifestation onset. Ursodeoxycholic acid could decrease the serum levels of ALP and bilirubin in 80% of the patients (12/15) and improve the symptom of pruritus and fatigue in 72.7% of the patients (11/15). Conclusions PBC mainly affects middle-aged women and the main clinical manifestations are hepatosplenomegaly, jaundice, pruritus, and fatigue. Liver function test typically reveal a cholestatic pattern accompanied by hypergammagloblinemia and a positive antimitochondrial antibody (AMA) including M2 subtype (AMA-M2). Ursodeoxycholic acid could improve the abnormal liver function tests and clinical features in PBC patients.