Low-Dose versus High-Dose Ursodeoxycholic Acid in Cystic Fibrosis-Related Cholestatic Liver Disease Results of a Randomized Study with 1-Year Follow-up

References

• O'Brien S, Fitzgerald MX, Hegarty JE. A controlled trial of ursodeoxycholic acid treatment in cystic fibrosis-related liver disease. Eur J Gastroenterol Hepatol 1992; 4: 857–63
   Google Scholar
• Bittner P, Posselt HG, Sailer T, Ott H, Magdorf K, Wahn U. The effect of treatment with ursodeoxycholic acid in cystic fibrosis and hepatopathy: a placebo controlled study. Bile acids as therapeutic agents: from basic science to clinical practice, G Paumgartner, A Stiehl, W Gerok. Kluwer Academic Publishers, Dordrecht 1991; 345–5
   Google Scholar
• Merli M, Bertasi S, Servi R, Diamanti S, Martino F, De Santis A. Effect of a medium dose of ursodeoxycholic acid with or without taurine supplementation on the nutritional status of patients with cystic fibrosis: a randomized, placebo-controlled, crossover trial. J Pediatr Gastroenterol Nutr 1994; 19: 198–203
   PubMedGoogle Scholar
• Cotting J, Lentze MJ, Reichen J. Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis. Gut 1990; 31: 918–21
   PubMedGoogle Scholar
• Colombo C, Setchell KDR, Podda M, Crosignani A, Roda A, Curcio L. Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis. J Pediatr 1990; 117: 482–9
   PubMed Web of Science ®Google Scholar
• Colombo C, Crosignani A, Assaisso M, Battezzati PM, Podda M, Giunta A. Ursodeoxycholic acid therapy in cystic fibrosis-associated liver disease: a dose-response study. Hepatology 1992; 16: 924–30
   PubMedGoogle Scholar
• Cotting J, Dufour JF, Lentze MJ, Paumgartner G, Reichen J. Ursodeoxycholate in the treatment of cholestasis in cystic fibrosis—a 2-year experience and review of the literature. Pediatric cholestasis: novel approaches to treatment, MJ Lentze, J Reichen. Kluwer Academic Publishers, Dordrecht 1992; 345–53
   Google Scholar
• Galabert C, Montet JC, Lengrand D, Lecuire A, Sotta C, Figarella C. Effects of ursodeoxycholic acid on liver function in cystic fibrosis and chronic cholestasis. J Pediatr 1992; 121: 138–41
   PubMedGoogle Scholar
• Colombo C, Battezzati PM, Podda M, Bettinardi N, Giunta A. Italian Group for the Study of Ursodeoxycholic Acid in Cystic Fibrosis. Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-blind multicenter trial. Hepatology 1996; 23: 1484–90
   PubMed Web of Science ®Google Scholar
• Nakagawa M, Colombo C, Setchell KDR. Comprehensive study of the biliary bile acid composition of patients with cystic fibrosis and associated liver disease before and after UDCA administration. Hepatology 1990; 12: 322–34
   PubMedGoogle Scholar
• Weber AM, Roy CC, Morin CL, Lasalle R. Malabsorption of bile acids in children with cystic fibrosis. N Engl J Med 1973; 289: 1001–5
   PubMed Web of Science ®Google Scholar
• O'Brien S, Mulcahy H, Fenlon H, O'Broin A, Casey M, Burke A. Intestinal bile acid malabsorption in cystic fibrosis. Gut 1993; 34: 1137–41
   PubMed Web of Science ®Google Scholar
• Lacerda MA, Lindor KD, Jorgensen RA, Rossi S, Hofmann A, Dickson ER. Ursodiol therapy in primary biliary cirrhosis (PBC): enrichment correlates with biochemical improvement. Gastroenterology 1993; 104: A933
   Google Scholar
• Jorgensen RA, Dickson ER, Hofmann AF, Rossi SS, Lindor KD. Characterisation of patients with a complete biochemical response to ursodeoxycholic acid. Gut 1995; 36: 935–8
   PubMedGoogle Scholar
• Van de Meeberg PC, Wolfhagen FHJ, VanBerge-Henegouwen GP, Salemans JMJI, Tangerman A, Van Buuren HR. Single or multiple dose ursodeoxycholic acid for cholestatic liver disease: relation with biliary enrichment and biochemical response. J Hepatol., In press
   Google Scholar
• Van de Meeberg PC, Van Erpecum KJ, VanBerge-Henegouwen GP. Therapy with ursodeoxycholic acid in cholestatic liver disease. Scand J Gastroenterol 1993; 28: 15–20, Suppl 200
   PubMedGoogle Scholar
• Colombo C, Castellani MR, Balistreri WF, Seregni E, Assaisso ML, Giunta A. Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with cystic fibrosis and associated liver disease. Hepatology 1992; 15: 677–84
   PubMed Web of Science ®Google Scholar
• Jazrawi RP, De Caestecker JS, Goggin PM, Britten AJ, Joseph AEA, Maxwell JD. Kinetics of hepatic bile acid handling in cholestatic liver disease: effect of ursodeoxycholic acid. Gastroenterology 1994; 106: 134–42
   PubMed Web of Science ®Google Scholar
• Lacaille F, Paradis K, Lenaerts C, Sénéchal L, Lepage G, Roy CC. Ursodeoxycholic acid (UDCA) improves essential fatty acid (EFA) deficiency in cystic fibrosis. Hepatology 1993; 18: 271, A.
   Google Scholar
• Poupon RE, Poupon R, Balkau B. UDCA-PBC Study Group. Ursodiol for the long-term treatment of primary biliary cirrhosis. N Engl J Med 1994; 330: 1342–7
   PubMed Web of Science ®Google Scholar
• Floreani A, Zappala F, Mazzetto M, Naccarato R, Plebani M, Chiaramonte M. Different response to ursodeoxycholic acid (UDCA) in primary biliary cirrhosis according to severity of disease. Dig Dis Sci 1994; 39: 9–14
   PubMed Web of Science ®Google Scholar
• Kneppelhout JC, Mulder CJJ, VanBerge-Henegouwen GP, De Vries RA, Brandt KH. Ursodeoxycholic acid treatment in primary biliary cirrhosis with the emphasis on late stage disease. Neth J Med 1992; 41: 11–6
   PubMedGoogle Scholar
• Hwang SJ, Chan CY, Lee SD. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis: a short-term, randomized, double-blind controlled, cross-over study with a long-term follow-up. J Gastroenterol Hepatol 1993; 8: 217–23
   PubMed Web of Science ®Google Scholar
• Vogel W, Kathrein H, Judmaier G, Braunsteiner H. Deterioration of primary biliary cirrhosis during treatment with urso-deoxycholic acid. Lancet 1988; 1: 1163
   PubMed Web of Science ®Google Scholar