Patients with Cystic Fibrosis (CF) often develop liver disease and although the mechanism leading to the pathognomonic lesion (focal biliary cirrhosis) is still unknown, quantitative/qualitative alterations in bile secretion may of of major importance. Correction of the complex abnormalities of bile acid (BA) metabolism described in these patients, (BA malabsorption with predominance of relatively more hydrophobic glycine-conjugated BA), may thus slow the progress of liver involvement associated to CF. Recently, a shift of the BA pool composition towards more hydrophilic-less hepatotoxic components, induced by UDCA and, in one report, by T administration, has been shown to be beneficial in adults with Primary Biliary Cirrhosis and Chronic Hepatitis. In 6 CF patients (aged 8-12 years) with clinical and biochemical signs of liver involvement but no portal hypertension, T (30 mg/Kg/die) was administered one month before and during the successive treatment with UDCA (10-15 mg/Kg/die). Liver functin tests were determined before and during each period of treatment, fecal fat and BA (GLC) exretion and biliary BA composition (HPLC) before and after long term administration of UDCA and T. Before treatment, both mean coefficient of fat absorption n enzymatic therapy (83.7 ± 10%) and fecal BA excretion (12.3± 5.9 mg/Kg/die) were abnormal. Chenodeoxycholic acid was predominant among biliary BA (44.1 ± 9.1%) and the glycine to taurine conjugate ratio was 2.7 ± 2.0. T administration produced only inconsistent changes of lvier function tests from basal abnormal values, whereas in patients treated fo two months with T + UDCA a substantial improvement was observed: AST -28%, ALT -40%. GT -33%, Alkaline Phosphatase -19%. The effects of longer periods of treatment are currently investigated, with purpose of establishing their relationship with changes in the BA pool composition.
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