原发性胆汁性肝硬化26例临床特征及疗效回顾分析  

Retrospective analysis of clinical characteristics and curative effect of 26 patients with primary biliary cirrhosis

作　　者：龚晓蓉[1] 钟碧慧[1] 张盛洪[1] 陈旻湖[1] 

机构地区：[1]中山大学附属第一医院消化内科,广东广州510080

出　　处：《胃肠病学和肝病学杂志》2009年第9期859-862,共4页Chinese Journal of Gastroenterology and Hepatology

摘　　要：目的总结原发性胆汁性肝硬化（PBC）患者的临床特征及诊治经验,以提高对该病的认识。方法对我院1996年7月-2005年12月收治的26例PBC患者的临床特征、生化、免疫学及组织学等资料进行回顾性分析。结果26例PBC患者中男6例,女20例,中位年龄47岁（36-76岁）,临床表现主要为黄疸（100%,26/26）,贫血（84.6%,22/26）,皮肤瘙痒（69.2%,18/26）。血清总胆红素[（208.0±119.7）μmol/L,26/26]、总胆汁酸[（128.7±94.9）μmol/L,26/26]、碱性磷酸酶[（467.1±289.0）U/L,25/26]、γ-谷氨酰转肽酶[（476.8±469.1）U/L,23/26];65.4%（17/26）患者IgM明显升高（4.2±1.8）g/L,抗线粒体抗体（AMA）阳性率76.2%（16/21）。19例患者行肝穿刺病理检查,早期（Ⅰ-Ⅱ期）占47.4%（9/19）,进展期（Ⅲ-Ⅳ期）占52.6%（10/19）;26例患者经常规护肝加熊去氧胆酸治疗,23例病情好转,有效率达88.5%。结论PBC以中年女性多见,黄疸为患者最主要表现,血清总胆红素、总胆汁酸、碱性磷酸酶、γ-谷氨酰转肽酶水平及AMA等有助于疾病的诊断,肝穿病理学检查可进一步确诊疾病及组织学分期,常规护肝加熊去氧胆酸治疗效果确切。Objective To summarize the clinical characteristics and experience in the diagnosis and treatment of primary biliary cirrhosis（PBC）.Methods Clinical data of 26 patients with PBC including the clinical manifestations,laboratory tests,pathological findings and the response to therapy were analyzed retrospectively.Results Twenty patients were females（76.9%）,and 6 were males（23.1%）.The median age at diagnosis was 47 years（range： 3676 years）.The most common symptoms were jaundice（100%,26/26）,followed by anaemia（84.6%,22/26）,pruitus（69.2%,18/26）,anorexia（65.4%,17/26） and fatigue（61.5%,16/26）.Total bile acid（TBA）,serum alkaline phosphatase（ALP） and glutamyl transpeptidase（GGT） levels were obviously elevated in most patients [（128.7±94.9）μmol/L,（467.1±289.0）U/L,（476.8±469.1）U/L,respectively].Serum IgM levels were elevated in 17 patients（65.4%）.Anti-mitochondrial antibody（AMA）/AMA-M2 were positive in 16 patients（61.5%）.Nineteen patients underwent pathological examinations,with 47.4%（9/19） in early stage（stage Ⅰ～Ⅱ） and 52.6%（10/19） in advanced stage（stage Ⅲ～Ⅳ）.All patients undertook the conventional therapy plus ursodeoxycholic acid（UDCA）,and the improvement rate was 88.5%.Conclusion PBC is subjective to occur in middle-aged women.Jaundice is the most common presentation and the biochemical test such as TBA,ALP,GGT and AMA are helpful in diagnosis.Liver biopsy is useful for confirming the diagnosis and differentiating histopathological stages.The conventional therapy of protecting liver function plus UDCA has achieved a effective result.

关 键 词：胆汁性肝硬化 自身抗体 肝活检 熊去氧胆酸 

分 类 号：R575.22[医药卫生—消化系统]