Oral bile acids in cystic fibrosis-associated liver disease.

Le résumé fourni par la source

Introduction With improved survival ofpatients with cystic fibrosis (CF), complications of the disease which can affect prognosis have received increased attention. There is growing evidence ofthe impact ofchronic liver disease in patients with CF1. In this population its prevalence has been increasing with time, and it has been recognized as a risk factor for early death2. In the absence of any specific or effective treatment, therapeutic efforts have so far been devoted to complications of cirrhosis and more recently have involved liver transplantation3. Ursodeoxycholic acid (UDCA) has been recently introduced for the medical management of chronic liver diseases4 and there is substantial evidence of its efficacy in those where cholestasis is a primary pathogenetic factor. The effects ofUDCA treatment in CF-associated liver disease are particularly promising513 and have suggested that CF could be considered one ofthe more appropriate indications for UDCA therapy. The purpose ofthis paper is to review the available clinical data and to report preliminary results on longterm UDCA treatment in our CF patients with liver disease.

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