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Clinical features and management of primary biliary cirrhosis

查看全文 作  者:Andrea [1]Crosignani;Pier Maria [1]Battezzati;Pietro [2]Invernizzi;Carlo [2]Selmi;Elena [1]Prina;Mauro [1]Podda 高影响力作者 机构地区:[1]Division of Internal Medicine and Liver Unit,San Paolo Hospital School of Medicine,University of Milan,Milano 20142,Italy;[2]Division of Internal Medicine and Liver Unit,San Paolo Hospital School of Medicine,University of Milan,Milano 20142,Italy Division of Rheumatology,Allergy,and Clinical Immunology,University of California at Davis,Davis,CA 95616,United States高影响力机构 出  处:《World Journal of Gastroenterology》索引2008年第14卷第21期,共15页高影响力期刊 摘  要:Primary biliary cirrhosis(PBC),which is characterised by progressive destruction of intrahepatic bile ducts,is not a rare disease since both prevalence and incidence are increasing during the last years mainly due to the improvement of case finding strategies.The prognosis of the disease has improved due to both the recognition of earlier and indolent cases,and to the wide use of ursodeoxycholic acid(UDCA).New indicators of prog-nosis are available that will be useful especially for the growing number of patients with less severe disease.Most patients are asymptomatic at presentation.Pruri-tus may represent the most distressing symptom and,when UDCA is ineffective,cholestyramine represents the mainstay of treatment.Complications of long-standing cholestasis may be clinically relevant only in very ad-vanced stages.Available data on the effects of UDCA on clinically relevant end points clearly indicate that the drug is able to slow but not to halt the progression of the disease while,in advanced stages,the only thera-peutic option remains liver transplantation. 关 键 词:胆管硬化 流行病学 临床特征 治疗方法
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