摘要
Abstract
Objective To summarize the diagnosis and management of patients with primary biliary cirrhosis (PBC).Methods Clinical data in 462 patients with PBC were reviewed and the clinical manifestations,the results of laboratory tests,and response to ursodeoxycholic acid(UDCA)therapy were analyzed retrospectively.Results Out of the 462 patients,418 (90.5％)were female with the average age of 46.4±9.4years;the major symptoms in these patients were fatigue,dry mouth,pruritus,jaundice and right epigastric discomfort;the major signs included hepatomegaly,splenomegaly and ascites; serum alkaline phosphatase (ALP),gamma glutamyl transpeptidase (GGT),bilirubin,and gammaglobulinemia levels increased in most of the patients;serum antimitochondrial antibodies(AMA)were positive in 90.9％ of patients;UDCA treatment reduced the serum levels of ALP and bilirubin in most patients;some patients accompanied with other autoimmune diseases received glucocorticoids or immunosuppressants therapy and the budesonide was effective with less systemic toxicity;ten patients had liver transplantation because of liver failure;six other patients were detected hepatocellular carcinoma during ten year followed-up.Conclusion PBC mainly involves middle-aged women with hepatosplenomegaly,jaundice,pruritus,fatigability and dry mouth as presenting manifestations.The liver function tests typically reveals a cholestatic pattern accompanied by hypergammaglobulinemia and a positive AMA or AMA-M2 subtype.UDCA can improve the abnormal liver function tests and clinical symptoms in patients with PBC.Some refractory patients need glucocorticoids or immunosuppressants treatment.Liver transplantation is vital for patients with terminal liver failure.关键词
原发性胆汁性肝硬化/临床特征/抗线粒体抗体Key words
Primary biliary cirrhosis/Clinical features/Antimitochondrial antibodies
