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目的对原发性胆汁性肝硬化患者的临床特征进行回顾性分析,以提高对该病诊治的认识。方法对原发性胆汁性肝硬化患者的临床资料进行分析。结果本组患者女性13例,男性3例,确诊的平均年龄为(52±6)岁,症状以黄疸最为多见(13/16,81%),其次是皮肤瘙痒(10/16,62%)和乏力(8/16,50%)。6例患者(37%)食道静脉曲张,4例患者(25%)合并腹水,5例(31%)伴发其他自身免疫性疾病,其中3例管干燥综合征,2例为G raves病。所有患者血清碱性磷酸酶、谷氨酰转肽酶及胆红素水平明显升高,分别为(532±172)U/L、(359±120)U/L和(127±39)U/L。患者血清IgM升高(4.7±1.8)g/L。行抗线粒体抗体检查94%(15/16)为阳性。结论原发性胆汁性肝硬化主要累及中年女性,血清碱性磷酸酶及谷氨酰转肽酶水平升高,抗线粒体抗体阳性,血清IgM升高有助于本病的诊断。
Objective To retrospectively analyze the clinical features of patients with primary biliary cirrhosis so as to raise awareness of the diagnosis and treatment of the disease. Methods The clinical data of patients with primary biliary cirrhosis were analyzed. Results The patients were 13 females and 3 males. The mean age at diagnosis was (52 ± 6) years old, the most common symptom was jaundice (13 / 16,81%), followed by pruritus (10/16, 62% ) And fatigue (8/16, 50%). Six patients (37%) had esophageal varices, four (25%) had ascites and five (31%) had other autoimmune diseases, three of which had Sjogren’s syndrome and two had G raves disease. Serum levels of alkaline phosphatase, glutamyl transpeptidase and bilirubin were significantly higher in all patients (532 ± 172 U / L, 359 ± 120 U / L and 127 ± 39 U / L. Patients with elevated serum IgM (4.7 ± 1.8) g / L. Line anti-mitochondrial antibody test 94% (15/16) was positive. Conclusions Primary biliary cirrhosis mainly affects middle-aged women. Serum levels of alkaline phosphatase and glutamyl transpeptidase are elevated. Anti-mitochondrial antibodies and elevated serum IgM are helpful for the diagnosis of this disease.