title: [Clinical characteristics of primary biliary cirrhosis: a report of 45 cases].
abstract: OBJECTIVE
  To study the clinical features of primary biliary cirrhosis (PBC) in order to facilitate cognition of the disease.
  
  
  METHODS
  General status, clinical manifestations and laboratory findings of 45 patients with PBC were reviewed.
  
  
  RESULTS
  Among the 45 patients, 42 were females and the mean age at diagnosis was (50.8 +/- 8.1) years. The mean time interval between the first visit to physicians to the time of correct diagnosis was about 2 years. The most frequent symptoms were fatigue (66.7%, 30/45), then jaundice (55.6%, 25/45) and pruritus (40%, 18/45). Nine patients (20%) were asymptomatic. Nine patients (20%) were associated with other auto-immune diseases (Sjogren's syndrome and/or rheumatoid arthritis). Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (gamma-GT) levels were markedly elevated in all the patients [(498.5 +/- 369.8) IU/L and (595.2 +/- 518.4) IU/L, respectively], whereas ALT and AST levels were mildly to moderately elevated [(83.9 +/- 58.8) IU/L and (100.8 +/- 48.8) IU/L, respectively]. 25 patients (55.6%) had a total bilirubin level >/= 34.2 micromol/L. 40 patients (88.9%) had elevated serum IgM and 43 patients (95.6%) were anti-mitochondrial antibody (AMA)/AMA-M2 positive.
  
  
  CONCLUSIONS
  In China, PBC is probably not so rare as it was thought before. It is mostly found in middle-aged women. The most frequent symptom is fatigue and some patients are asymptomatic at early stage. Elevated serum ALP and gamma-GT levels together with positive AMA/AMA-M2 can help to diagnose PBC. Liver biopsy is useful to confirm the diagnosis and differentiate histopathological stages.
authors.affiliation: Liver Research Center, Beijing Friendship Hospital, Capital University of Medical Sciences, Beijing 100050, China.
authors.forename: Fukui
authors.initials: F
authors.lastname: Zhang
authors.forename: Jidong
authors.initials: J
authors.lastname: Jia
authors.forename: Baoen
authors.initials: B
authors.lastname: Wang
authors.forename: Linxue
authors.initials: L
authors.lastname: Qian
authors.forename: Shanshan
authors.initials: S
authors.lastname: Yin
authors.forename: Yu
authors.initials: Y
authors.lastname: Wang
authors.forename: Yan
authors.initials: Y
authors.lastname: Cui
authors.forename: Hong
authors.initials: H
authors.lastname: You
authors.forename: Hong
authors.initials: H
authors.lastname: Ma
authors.forename: Huiji
authors.initials: H
authors.lastname: Wang
authors.forename: Changhuai
authors.initials: C
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000368:Aged; D000410:Alanine Transaminase / Q000097:blood; D000469:Alkaline Phosphatase / Q000097:blood; D000626:Aminopeptidases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D043384:Glutamyl Aminopeptidase; D019694:Hepatitis B, Chronic; D006801:Humans; D008099:Liver / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D005723:gamma-Glutamyltransferase; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase; D000626:Aminopeptidases; D043384:Glutamyl Aminopeptidase; D001663:Bilirubin