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  • Title: Primary biliary cirrhosis in Southern Israel: a 20 year follow up study.
    Author: Delgado JS, Vodonos A, Delgado B, Jotkowitz A, Rosenthal A, Fich A, Novack V.
    Journal: Eur J Intern Med; 2012 Dec; 23(8):e193-8. PubMed ID: 23022449.
    Abstract:
    BACKGROUND: The epidemiology of primary biliary cirrhosis (PBC) in Israel is unknown. We aimed to determine the epidemiology, long-term survival and outcomes of PBC in Southern Israel from 1990 to 2010. METHODS: Case-finding methods and population-based administrative data were used to estimate and evaluate the incidence, prevalence and prognostic factors of outcome in our PBC cohort. RESULTS: 138 cases of PBC were identified. The average annual prevalence of PBC was 255 cases per million. The overall age/sex-adjusted annual incidence of PBC was 10 cases per million from 1990 through 1999 and 20 cases per million from 2000 to 2010. Among 138 incident cases with a total follow-up of 960 persons-years from diagnosis, 30 patients (21.7%) died. Survival in PBC patients was significantly lower than that of the age/sex-matched Israeli population. Mortality was significantly increased in patients with an initial MELD score greater than 8 (P<0.001), with portal hypertension (P<0.001), and in non-responders to ursodeoxycholic acid (UDCA) therapy according to Barcelona criteria (P=0.005). Out of 138 patients, 95 patients (68.0%) responded to UDCA therapy according to Barcelona and Paris criteria. None of the responders died during the follow-up period as opposed to 30 out of 43 (69.8%) of non-responders. In multivariate analysis the factors associated with response to UDCA were: albumin levels above 3.5 g/dL (P<0.001) and lower degree of fibrosis per liver biopsy (P=0.003). CONCLUSIONS: This study addresses the increasing burden of PBC in Israel and confirms the importance of some clinical and therapeutic factors as predictors of long-term prognosis.
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