title: Diminished survival in asymptomatic primary biliary cirrhosis. A prospective study.
abstract: Data from 73 asymptomatic patients with primary biliary cirrhosis were analyzed to determine clinical course and long-term survival. Of these, 44 entered a D-penicillamine treatment trial; 29 qualified but chose not to participate. Median follow-up was 7.6 yr (range, 2.8-12.2 yr). Liver biopsy at the initial visit showed advanced disease (fibrosis, cirrhosis) in 61% of the patients. During prospective clinical follow-up, which was available for 37 of the 44 study patients, one or more symptoms of liver disease developed in 33 (89%); esophageal varices were found in 15 (41%), and histologic progression to cirrhosis was found in 20 (67%) of the 30 precirrhotic patients. Significant (p less than 0.01) biochemical progression was reflected by a decrease in mean serum albumin concentrations and an increase in mean serum bilirubin levels in 32 patients followed for 4-6 yr. Survival data were available for all 73 patients; 17 died (11 secondary to liver failure), and 1 underwent liver transplantation. These patients had a 4-fold increase in mortality rate (p less than 0.001) compared with the U.S. population matched for age, race, and sex.
authors.affiliation: Division of Gastroenterology and Internal Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: K
authors.initials: K
authors.lastname: Balasubramaniam
authors.forename: P M
authors.initials: PM
authors.lastname: Grambsch
authors.forename: R H
authors.initials: RH
authors.lastname: Wiesner
authors.forename: K D
authors.initials: KD
authors.lastname: Lindor
authors.forename: E R
authors.initials: ER
authors.lastname: Dickson
mesh_terms: D000328:Adult; D000368:Aged; D001663:Bilirubin / Q000097:blood; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D010396:Penicillamine / Q000627:therapeutic use; D011446:Prospective Studies; D012709:Serum Albumin / Q000032:analysis; D015996:Survival Rate
chemical_list: D012709:Serum Albumin; D010396:Penicillamine; D001663:Bilirubin