Clinical Analysis Of Primary Biliary Cirrhosis: A Report Of146Cases

Objective：To better understand Primary Biliary cirrhosis(PBC)， improve thetherapeutic methods,decrease misdiagnosis rate and improve the prognosis by analyz-ing the clinical features of PBC and studying the changes of biochemical results andthe autoimmune.Methods：From the onset of symptoms or liver function changes to diagnosis,general information of146PBC cases were retrospectively analyzed, including theclinical manifestation，serum chemistry index，immunology，imaging,pathologicalchanges and treatment outcome．Further explore if it is significant between pre-cirrhosisstage group and the cirrhosis stage group in the biochemical and immunological resuls,the treatments of the patients.Results：Out of146patients，9were male and137were famale with the averageage of52years；Mean time from the onset of Symptoms or liver function change todiagnosis was48.5months，the longest was96months．the major symptoms in thesepatients were fatigue(45.89%），pruritus（37.67%），jaundice（30.14%）and abdominaldistention（26.71%）；the major signs included splenomegaly（37.67%）, hepatomegaly（17.81%）,liver palm（10.96%）,spider nevus（8.22%）, ascites（12.33%）andpigmentation（3.42%）；Laboratory tests include Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (GGT) levels weremarkedly elevated in all the patients [(354.88±275.23) IU/L and （391.85±400.34）IU/L,respectively],whereas ALT and AST levels were mildly to moderately elevated[(101.43±123.14)IU/L and (93.52±83.76)IU/L,respectively].serum antimitochondrialantibodies(AMA)were positive in (82．20％)of patients；AMA/AMA-M2positive rate was91.67％. total bilirubin(TBIL)，direct bilirubin(DBIL), total bile acid (TBA),serumalbumin(ALB),alanine amino transferase(ALT) and immunoglobin M，(IgM) in dif-ferent clinical stages were different significantly(P<0.05)．Conclusion： PBC mainly attacts middle-aged women with pruritus jaun-dice,fatigue and Hepatosplenomegaly as presenting manifestations．The disease courseis a slow process of progressive cholestasis. In the early course of the disease，thecholangitis may be dominant while it takes a long time to develop primary biliary cir-rhosis. Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (GGT) levelswere markedly elevated accompanied with hyperbilirubinemia characterized and hy-pergammaglobulinemia while ALT and AST levels were mildly to moderately elevated.High titer of AMA and AMA-M2Subtype are the main indices of Diagnosis. PBCshould be based on clinical manifestations and the results of laboratory test. It is themost important to do the liver biopsy when the immonological results are negative. Di-agnosis of PBC should be made by combining the Serum bilimbin levels with the clini-cal features and laboratory(especially GGT，ALT，auto-antibody)as well as histo-pathology examination．The therapies of PBC are very limited. UDCA is the only drugwhich is effective and now it is the first line therapeutic drug of PBC. In conclusion, thecourse of the disease, the level of ALB and TBIL，histological stages and the use ofUDCA have great revelance to the prognosis. The late stage of the patients have poorprognosis so that Early detection and treatment may retard the progressive of the diseaseand improve the prognosis.