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目的了解原发性胆汁性肝硬化(Prim ary b iliary c irrhosis,PBC)的临床特点以提高对PBC的认识。方法分析具有完整资料的22例PBC患者的临床表现、实验室检查及治疗转归。结果22例PBC中女性21例,确诊年龄平均为52岁。临床表现主要为乏力、皮肤瘙痒、黄疸,体征包括肝脾肿大、黄色瘤。实验室检查以高ALP、GGT、高胆红素血症、高球蛋白血症以及存在自身抗体如AMA、AMA-M2等为特征。熊去氧胆酸可延缓病程、改善胆汁淤积指标。结论PBC好发于中老年女性,以乏力、瘙痒、黄疸为主要临床表现。肝功能以胆汁淤积为主,伴有高球蛋白血症,高滴度AMA及AMA-M2亚型是诊断PBC的主要指标。以熊去氧胆酸(UDCA)为主的综合治疗能延缓病程,总体预后欠佳,治疗手段有限。
Objective To understand the clinical features of primary biliary cirrhosis (PBC) to improve the understanding of PBC. Methods The clinical manifestations, laboratory tests and treatment outcomes of 22 PBC patients with complete data were analyzed. Results 21 cases of PBC in 22 cases, the average age at diagnosis of 52 years old. The main clinical manifestations of fatigue, skin itching, jaundice, signs, including hepatosplenomegaly, xanthoma. Laboratory tests are characterized by high ALP, GGT, hyperbilirubinemia, hypergammaglobulinemia and the presence of autoantibodies such as AMA, AMA-M2 and the like. Ursodeoxycholic acid can delay the course of disease, improve the cholestasis index. Conclusion PBC occurs in middle-aged and elderly women, with fatigue, itching, jaundice as the main clinical manifestations. Cholestatic liver function mainly with hypergammaglobulinemia, high titer AMA and AMA-M2 subtype is the main indicator of diagnosis of PBC. Ursodeoxycholic acid (UDCA) based comprehensive treatment can delay the course of the overall poor prognosis, limited treatment.