[Clinical analysis of 22 patients with primary biliary cirrhosis].
OBJECTIVE
To improve the diagnosis and management of primary biliary cirrhosis (PBC).


METHODS
Clinical data of 22 cases of PBC were reviewed including the clinical manifestation, laboratory test, and the response to therapy.


RESULTS
There were 20 female patients with an average of 50 years old in total of 22 PBC patients. The major symptoms were pruritus, fatigue, anorexia, and abdominal discomfort. The major signs included jaundice, hepatomegaly, splenmegaly, 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 level was only slightly elevated but the AST/ALT ratio was reversed. It took 8 months (ranging from 2 months to 5 years) to confirm the diagnosis after the clinical manifestation onset. Ursodeoxycholic acid could decrease the serum levels of ALP and bilirubin in 80% of the patients (12/15) and improve the symptom of pruritus and fatigue in 72.7% of the patients (11/15).


CONCLUSIONS
PBC mainly affects middle-aged women and the main clinical manifestations are hepatosplenomegaly, jaundice, pruritus, and fatigue. Liver function test typically reveal a cholestatic pattern accompanied by hypergammagloblinemia and a positive antimitochondrial antibody (AMA) including M2 subtype (AMA-M2). Ursodeoxycholic acid could improve the abnormal liver function tests and clinical features in PBC patients
Yao
Dingkang
D
Department of Gastroenterology, Changzheng Hospital, Second Military Medical University, Shanghai 200003, China.
Xie
Weifen
W
Wang
Lei
L
D000469:Alkaline Phosphatase; D001663:Bilirubin; D002779:Cholestasis; D005260:Female; D006529:Hepatomegaly; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000378:metabolism / Q000503:physiopathology; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D014580:Ursodeoxycholic Acid
D014580:Ursodeoxycholic Acid; D000469:Alkaline Phosphatase; D001663:Bilirubin