Clinical–Liver, Pancreas, and Biliary TractSurvival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: Follow-up for up to 28 years☆
Section snippets
Materials and methods
The methods used to identify the Northeast England cohort of patients with PBC have previously been described in detail.13 This cohort included all incident and prevalent cases of definite or probable PBC who were alive between January 1987 and December 1994 within a geographically defined area of Northeast England. Boundaries within the region were verified from the Office of National Statistics, postal code (ZIP code), and local health authority data.
Definite PBC was defined as all 3 of the
Results
A total of 770 patients were diagnosed with PBC within the geographic and temporal boundary of the sampling frame; 302 patients were diagnosed before January 1, 1987 (i.e., prevalent cases), and 468 patients were incident cases between January 1987 and December 1994. Data to analyze survival adjusted for the effect of prevalent cases were available for 765 (99%) patients. Full clinical follow-up by interview and note review to ascertain symptom status was possible for 708 (91.9%) patients.
A
Discussion
We have reported the follow-up of a large geographically based cohort of patients with PBC. Unlike all previous reports of outcome, this study was not limited to a few health care providers, nor was it based in a tertiary referral center. These data may provide the most accurate picture of the outcome of PBC in an unselected population.
Most patients were asymptomatic at the time of initial diagnosis. This case series is the first to formally analyze the progression of symptoms in a
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Address requests for reprints to: Martin Prince, M.R.C.P., Centre for Liver Research, Fourth Floor, William Leech Building, The Medical School, Framlington Place, Newcastle-Upon-Tyne, NE2 4HH, England. Fax: (44) 191-222-0723.