Clinical features of forty patients with primary biliary cirrhosis.
OBJECTIVE
To study the clinical features of patients with primary biliary cirrhosis (PBC) in order to improve the doctors' awareness of the disease.


METHODS
General status, clinical manifestations and laboratory findings of 40 patients with PBC were reviewed.Thirty-seven patients were females (37/40), and the mean age at diagnosis was 50.5 +/- 7.8 years. The time interval from initial symptoms or preliminary diagnosis to final diagnosis was 24.0 +/- 23.6 months.


RESULTS
The most frequently reported symptoms were fatigue (67.5%, 27/40), jaundice (60%, 24/40) and pruritus (32.5%, 17/40). Eight patients (20%) had associated auto-immune diseases (Sjogren's syndrome and/or rheumatoid a(c)arthritis). Serum alkaline phosphatase (ALP) and gamma glutamyl transpeptidase (gamma-GT) levels were markedly elevated (520.3 +/- 382.3 IU/L and 648.6 +/- 529.1 IU/L, respectively) in all patients, while alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were mildly elevated (82.6 +/- 54.5 IU/L and 100.7 +/- 47.2 IU/L, respectively). Twenty-four patients (60%) had a total bilirubin level >/= 34.2 micromol/L. Thirty-five patients (87.5%) had elevated serum immunoglobin M,and 97.5% of patients (39/40 ) were anti-mitochondrial antibody (AMA)/AMA-M2 positive.


CONCLUSIONS
Elevated serum ALP and gamma-GT levels, together with a positive AMA/AMA-M2, can help the diagnosis of PBC. Liver biopsy is useful to confirm the diagnosis and to differentiate histopathological stages.
Liver Research Center, Beijing Friendship Hospital, Capital University of Medical Sciences, China. frankliver@yahoo.com.cn
Fukui
F
Zhang
Jidong
J
Jia
Rutao
R
Cui
Baoen
B
Wang
Huiji
H
Wang
D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D005260:Female; D019694:Hepatitis B, Chronic / Q000209:etiology; D006801:Humans; D008099:Liver / Q000473:pathology / Q000503:physiopathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged