Clinical analysis of 78 patients with primary biliary cirrhosis / 解放军医学杂志

ABSTRACT

Objective To improve the diagnosis and management of primary biliary cirrhosis (PBC). Methods Clincal data of 78 cases of PBC patients were reviewed. The clinical manifestations,the results of laboratory tests,and response to therapy in all the PBC patients were analysed retrospectively. Results Among 78 patients 68 were female. The mean age was 50.4?9.3years at the time when definite diagnosis was made. The major symptoms in these patients were pruritus,fatigability,and jaundice. The major signs included hepatomegaly,splenomegaly,and ascites. Very high levels of serum alkaline phosphatase (ALP) and serum gamma glutamyl transpeptidase (GGT),hyperbilirubinemia,and hypergammaglobulinemia were also detected in most of the patients. The aminotransferase levels were only slightly elevated,but the AST/ALT ratio was reversed. It took 12 months (ranging from 2 months to 10 years) to confirm the diagnosis after the onset of clinical manifestations. Ursodeoxycholic acid reduced the serum levels of ALP and bilirubin in 80% of patients and improved the symptom of pruritus and fatigability in 73.4% of patients. Conclusion PBC mainly affects middle-aged women with hepatosplenomegaly, jaundice,pruritus and fatigability as presenting features. Liver function tests typically revealed a cholestatic pattern accompanied by hypergammaglobulinemia and a positive antimitochondrial antibody (AMA) including M2 subtype (AMA-M2). Ursodeoxycholic acid could improve the abnormal liver function tests and clinical symptoms in PBC patients.