原发性胆汁性肝硬化失代偿期的临床特征

Clinical characteristics of decompensated primary biliary cirrhosis

目的了解原发性胆汁性肝硬化(PBC)失代偿期的临床特征。方法回顾性分析204例PBC患者(包括代偿期113例,失代偿期91例)的人口统计学、实验室检查、临床表现及预后模型积分等,研究失代偿期PBC的临床特征,并且与乙型肝炎肝硬化失代偿期(乙肝组,51例)、丙型肝炎肝硬化失代偿期(丙肝组,20例)、酒精性肝硬化失代偿期(ALD组,51例)患者的临床特征进行比较。统计学处理采用t检验、方差分析及χ2检验。结果 (1)PBC失代偿期患者往往高龄,血细胞计数、血脂水平、白蛋白、胆碱酯酶及凝血酶原活动度明显降低,国际标准化比值、总胆红素及直接胆红素水平显著升高,Child-Pugh分级、终末期肝病模型、Mayo等模型积分均明显升高(P<0.05)。(2)与乙肝、丙肝及ALD组失代偿期肝硬化相比,PBC失代偿期患者女性比例较多,血清碱性磷酸酶显著升高,凝血酶原时间延长较少,更常见瘙痒症状(与ALD、丙肝组相比,P<0.05),上消化道出血发生率较高(与乙肝、丙肝组相比,P<0.05),更常合并干燥综合征、骨质疏松,但肝细胞癌发生率较低。结论 PBC多发于中老年女性,与其他病因所致肝硬化失代偿期患者相比,失代偿期PBC有一些显著不同的临床特征。

Objective To study the clinical characteristics of decompensated primary biliary cirrhosis（PBC）.Methods The clinical characteristics including demographics,laboratory tests,clinical manifestations,complications as well as scores of several prognostic models of 204 patients with PBC（113 patients with compensated cirrhosis and 91 patients with decompensated cirrhosis） were retrospectively studied.Then compared the clinical characteristics with 51 patients from decompensated hepatitis B（HBV group）,20 patients from hepatitis C（HCV group） and 51 patients from decompensated alcoholic liver disease（ALD group）.Results（1） The patients with decompensated PBC were older than those with compensated PBC and had significantly decreased blood cell counts,serum lipids,albumin and cholinesterase levels while increased international normalized ratio（INR）,total bilirubin level and direct bilirubin level.The model integration of Child-Pugh grading,model of end-stage liver disease（MELD） and Mayo significantly increased（P0.05）.（2） The patients with decompensated PBC were in mostly middle aged to elderly women and had higher serum alkaline phosphatase while the prothrombin time was shortened compared with those in HBV,HCV and ALD group.They had higher incidence of pruritus（compared with those in HCV and ALD group,P0.05）,upper gastrointestinal bleeding（compared with those in HBV and HCV group,P0.05）,more frequent complications with Sjogren＇s syndrome and osteoporosis and lower incidence of hepatocellular carcinoma.Conclusion PBC primarily affected middle aged to elderly women and the decompensated cirrhotic patients had significantly special clinical characteristics compared with those from other etiologies.