原发性胆汁性肝硬化16例临床分析并文献复习

目的 :对原发性胆汁性肝硬化 (PBC)患者的临床特征进行总结及分析 ,以提高对该病诊治的认识。方法 :分析 16例原发性胆汁性肝硬化患者的一般资料、临床表现、血清学、免疫学、影像学、治疗结果等改变。结果 :本组患者中女性∶男性为 14∶ 2 ,平均年龄为 (45± 13)岁。最常见的临床症状为黄疸 (75 % ) ,其次为皮肤瘙痒 (5 0 % )和乏力 (31% ) ,4例患者 (2 5 % ) B超提示门脉高压 ,3例患者 (18.7% )内镜发现食管静脉曲张 ,3例患者 (18.7% )出现腹水 ,2例患者 (12 .4 % )有眼睑黄色瘤。所有患者均有血清碱性磷酸酶 (AL P)和 γ-谷氨酰转肽酶 (γ- GT)及总胆红素 (TBIL)水平明显升高 ,分别为 (345± 2 34) u/ L (341± 175 ) u/ L和 (188± 16 0 ) umol/ L ,AL T、AST水平呈轻至中度升高 ,分别为 (85±4 2 ) u/ L 和 (12 5± 5 8) u/ L,血清 Ig M水平升高 ,平均 (3.5± 1.8) g/ L,行线粒体抗体检查者 93.75 % (15 / 16 )阳性 ,31.2 0%患者早期合用熊去氧胆酸治疗 ,短期内显示一定疗效。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、总胆红素水平明显升高、抗线粒体抗体阳性、血清 Ig M升高有助于诊断本病 ,肝活检病理学检查有助于进一步确诊

Analysis on 16 Cases of Primary Biliary Cirrhosis and Review of Associated Issues

Objective To study the clinical feature,diagnosis and treatment of primary biliary cirrhosis. Methods The clinical feature,results of serology,immunology,radiology and treatment of 16 primary biliary cirrhosis were retrospectively analyzed.Results The ratio of female vs male was 14∶2.Average age was (45±13) years.The first three symptoms were jaundice(75 %),itch of skin (50 %)and fatigue(31 %).4 patients (25 %)were found portal hypertension by ultrasound,3(18.7 %)were found with esophageal varicosis by endoscope.3(18.7 %)with hydroperitonia and 2(12.4 %)with xanthoma palpebrarum.The value of serum ALP(345±234)u/L γ GT(341±175) u/L and TBIL(188±160) μmol/L showed elevated,ALT(85±42)u/L and AST(125±58)u/L also elevated mildly to moderately. Serum IgM elevlated (3.5±1.8). The positive rate of antimitochondria antibody; was 93.75%(15/16). 31.20 % of patients were treated with UDCA and achieved good results.Conclusion Primary biliary cirrhosis mostly occur among middle aged female. The increase of serum ALP,γ GT.Tbil and IgM, and positive antimitochondria antibody,were with help to the diagnosis of Primary biliary cirrhosis. However,the biopsy of liver was of final diagnosis.