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目的分析原发性胆汁性肝硬化(PBC)临床特征。方法对29例PBC患者的一般资料、临床症状和体征、生化指标、免疫指标及肝组织病理特征进行分析。结果临床表现为黄疸19例,乏力15例,皮肤瘙痒12例,肝区疼痛5例,脾肿大27例,肝肿大22例,无症状2例。伴有自身免疫性疾病干燥综合征4例、类风湿性关节炎2例、系统性红斑狼疮1例。血清GGT和ALP明显升高,GGT升高平均为正常值上限的5.8倍,ALP升高平均为正常值上限的3.1倍,而血清ALT、AST轻中度升高,分别为平均正常值上限的1.6倍和2.7倍,19例TBil水平升高(28.2~427.2μmol/L)。24例抗线粒体抗体(AMA)或AMA-M2阳性,13例抗核抗体(ANA)阳性,1例抗双链DNA(ds-DNA)抗体阳性,4例抗SSA/SSB型,2例类风湿因子(RF)阳性。17例免疫球蛋白Ig M阳性,升高平均为正常值上限的1.8倍。15例肝脏病理检查示1例Ⅰ期,4例Ⅱ期,7例Ⅲ期,3例Ⅳ期。结论PBC主要累及中年女性,常表现为黄疸、乏力、皮肤搔痒,血清GGT、ALP水平升高,AMA或M2亚型阳性,Ig M阳性有助于诊断本病,肝活检病理学检查可用于进一步确诊该病。
Objective To analyze the clinical features of primary biliary cirrhosis (PBC). Methods The general data, clinical symptoms and signs, biochemical indexes, immune indexes and liver histopathological features of 29 PBC patients were analyzed. Results The clinical manifestations were jaundice in 19 cases, weakness in 15 cases, pruritus in 12 cases, liver pain in 5 cases, splenomegaly in 27 cases, hepatomegaly in 22 cases and asymptomatic in 2 cases. 4 cases of Sjogren’s syndrome accompanied by autoimmune diseases, 2 cases of rheumatoid arthritis and 1 case of systemic lupus erythematosus. Serum GGT and ALP were significantly higher, the average rise of GGT 5.8 times the upper limit of normal, ALP increased 3.1 times the upper limit of normal, and serum ALT, AST mild to moderate increase, respectively, the upper limit of the average normal 1.6 times and 2.7 times, 19 cases of TBil levels (28.2 ~ 427.2μmol / L). 24 were positive for anti-mitochondrial antibody (AMA) or AMA-M2, 13 were positive for antinuclear antibody (ANA), 1 was positive for anti-dsDNA, 4 were anti-SSA / SSB and 2 were rheumatoid Factor (RF) positive. 17 cases of immunoglobulin Ig M positive, elevated average of 1.8 times the upper limit of normal. Fifteen cases of liver biopsy showed 1 case of stage Ⅰ, 4 cases of stage Ⅱ, 7 cases of stage Ⅲ and 3 cases of stage Ⅳ. Conclusions PBC mainly affects middle-aged women. Jaundice, fatigue, pruritus, elevated serum GGT and ALP levels, positive AMA or M2 subtypes and positive IgM are helpful to diagnose the disease. Pathological examination of liver biopsy can be used to detect Further confirmed the disease.