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1原发性胆汁性肝硬化153例分析显示文摘目的 探讨原发性胆汁性肝硬化 (PBC)的临床、免疫学及病理学特点。方法 观察 15 3例PBC的临床表现 ,并分析有关实验室检查资料 ,35例行肝组织学检查。结果 有典型的皮肤瘙痒者占 43 79% ,肝肿大占 46 41% ,脾肿大者占 39 87% ,有皮肤、巩膜黄染者占 84 30 % ,随病情进展血清胆红素 (SB)逐步上升 ,而胆碱酯酶 (CHE)逐步下降 (P <0 0 1) ;丙氨酸转氨酶 (ALT)、人血白蛋白在病程晚期明显下降 (P <0 0 5 ) ,ALT、CHE与SB呈酶 胆分离现象 ;天冬氨酸转氨酶 (AST)在病情晚期反而上升 (P <0 0 5 ) ;碱性磷酸酶 (ALP)、γ 谷氨酰转移酶 (GGT)在病情早期即明显升高 ,随病情进展到中期ALP明显下降 (P <0 0 5 ) ,到晚期GGT明显下降 (P <0 0 1)。抗线粒体抗体 (AMA)阳性者占83 7% ,抗线粒体抗体M2亚型 (M2 )阳性者占 6 9 3%。 35例经肝组织学检查确诊病例中 ,10例为AMA阳性者 ,2 5例为血清学检测全阴者。结论 5 0 %以上的PBC无典型临床症状 ;80 %以上的PBC可以根据临床表现结合血清自身抗体检测确诊 ;部分依临床资料不能确诊病例 ,应行肝脏组织学检查确诊。陈国凤 李莉 高君 李梵 李冰 2002中华医学杂志2002,82,23:6
240例原发性胆汁性肝硬化的临床特征(英文)显示文摘Objective To study the clinical features of patients with primary biliary cirrhosis (PBC) in order to improve the doctors’ awareness of the disease. Methods General status, clinical manifestations and laboratory findings of 40 patients with PBC were reviewed. Thirty-seven patients were females (37/40), and the mean age at diagnosis was 50.5±7.8 years. The time interval from initial symptoms or preliminary diagnosis to final diagnosis was 24.0±23.6 months. Results The most frequently reported symptoms were fatigue (67.5%, 27/40), jaundice (60%, 24/40) and pruritus (32.5%, 17/40). Eight patients (20%) had associated auto-immune diseases (Sjogren’s syndrome and/or rheumatoid arthritis). Serum alkaline phosphatase (ALP) and gamma glutamyl transpeptidase (γ-GT) levels were markedly elevated (520.3±382.3 IU/L and 648.6±529.1 IU/L, respectively) in all patients, while alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were mildly elevated (82.6±54.5 IU/L and 100.7±47.2 IU/L, respectively). Twenty-four patients (60%) had a total bilirubin level ≥34.2 μmol/L. Thirty-five patients (87.5%) had elevated serum immunoglobin M, and 97.5% of patients (39/40) were anti-mitochondrial antibody (AMA)/AMA-M2 positive. Conclusion Elevated serum ALP and γ-GT levels, together with a positive AMA/AMA-M2, can help the diagnosis of PBC. Liver biopsy is useful to confirm the diagnosis and to differentiate histopathological stages.张福奎 贾继东 崔儒涛 王宝恩 王惠吉 2002Chinese Medical Journal2002,,6:2
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