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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。方法分析38例原发性胆汁性肝硬化患者的一般资料、临床表现、生化、免疫学及病理学等改变。结果本组患者中女性33例,确诊时的平均年龄为57.8±2.2岁。症状以黄疸最为多见(76.3%),其次为皮肤瘙痒(44.7%)和乏力(42.1%),14例患者(36.8%)合并腹水,9例患者(23.7%)食道静脉曲张,8例患者(21.1%)伴随其他自身免疫性疾病,其中5例干燥综合征,3例Graves病。所有患者血清碱性磷酸酶、γ谷氨酰转肽酶及胆红素水平明显升高,分别为523.1±72.6u/L、759.0±47.1u/L和290.0±50.8μmol/L,血清丙氨酸转氨酶(ALT),天冬氨酸转氨酶(AST)水平呈轻至中度升高,分别为101.6±27.1u/L及197.8±39.6u/L。患者血清IgM升高(3.9±0.4g/L),行线粒体抗体检查者76.5%(26/34)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γ-谷氨酰转肽酶水平升高,抗线粒体抗体阳性,血清IgM升高有助于诊断本病。肝活检病理学检查有助于本病的进一步确诊及组织学分期。
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 38 cases of primary biliary cirrhosis patients with general information, clinical manifestations, biochemical, immunological and pathological changes. Results In this group of 33 women, the mean age at diagnosis was 57.8 ± 2.2 years. The symptoms were most common in jaundice (76.3%), followed by pruritus (44.7%) and fatigue (42.1%), ascites in 14 patients (36.8%), esophageal varices in 9 patients (23.7%), (21.1%) with other autoimmune diseases, including 5 Sjogren’s syndrome and 3 Graves’ disease. Serum levels of alkaline phosphatase, γ-glutamyl transpeptidase and bilirubin were significantly higher in all patients (523.1 ± 72.6u / L, 759.0 ± 47.1u / L and 290.0 ± 50.8μmol / L, respectively) The levels of ALT and AST were mild to moderate (101.6 ± 27.1u / L and 197.8 ± 39.6u / L respectively). Patients with elevated serum IgM (3.9 ± 0.4g / L), line mitochondrial antibody test 76.5% (26/34) positive. Conclusions Primary biliary cirrhosis mainly affects middle-aged women. Serum alkaline phosphatase and γ-glutamyl transpeptidase levels are elevated. Anti-mitochondrial antibodies and elevated serum IgM are helpful for the diagnosis of this disease. Liver biopsy pathological examination contribute to the further diagnosis of this disease and histological staging.