Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis

Galabert, C.; Montet, Jean-Claude; Lengrand, D.; Lecuire, A.; Sotta, C; Figarella, Catherine; Chazalette, J. P.

doi:10.1016/s0022-3476(05)82561-2

Cited by 63 publications

(25 citation statements)

References 10 publications

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“…This study confirms the previously reported beneficial effect of UDCA on liver biochemistry in CF patients with cholestatic liver disease (1)(2)(3)(4)(5)(6)(7)(8)(9). In our population the improvement in liver enzyme values was established within 3 months and then stabilized or further improved slightly during a 1-year follow-up period.…”

Section: Discussionsupporting

confidence: 92%

“…The current arguments to treat CF patients with UDCA are mainly based on the beneficial effect of UDCA on increased liver enzyme values in CF (1)(2)(3)(4)(5)(6)(7)(8)(9). A similar effect is seen in other cholestatic liver diseases, such as primary biliary cirrhosis and primary sclerosing cholangitis (16).…”

Section: Discussionmentioning

confidence: 99%

“…The hydrophilic bile salt ursodeoxycholic acid (UDCA) improves liver biochemistry values in patients with cystic fibrosis (CF)-related cholestasis (1)(2)(3)(4)(5)(6)(7)(8)(9). When treated with a standard dose of UDCA (10-15 mg/kg/day), CF patients appear to have a relatively low biliary enrichment in comparison with patients with other liver diseases or gallstone patients treated with UDCA (10).…”

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confidence: 99%

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

Meeberg

Houwen

Sinaasappel

et al. 1997

Scandinavian Journal of Gastroenterology

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VanBerge-Henegouwen GP. Low-dose versus high-dose ursodeoxycholic acid in cystic fibrosis-related cholestatic liver disease. Results of a randomized study with 1-year follow-up. Scand J Gastroenterol 1997;32:369-373.Background: Ursodeoxycholic acid (UDCA) is beneficial in cholestasis related to cystic fibrosis (CF). High-dose treatment has been recommended to compensate for bile salt malabsorption. We compared the results of low-dose (10 mg/kg/day) and high-dose (20 mg/kg/day) UDCA treatment on liver biochemistry after 3 and 12 months' treatment. Methods: Thirty CF patients (age >5 years) with biochemical cholestasis and compensated liver disease were randomized for low-dose (n = 17) or high-dose (n = 13) UDCA. Baseline clinical variables were comparable. Results: After 1 year one patient had died of liver failure (low dose), and three had dropped out because of pruritus (one in each group) or personal choice (low dose). In the high-dose group improvement in y-glutamyl transferase values was more pronounced after 3 months and 1 year (P < 0.004), and improvement of alanine aminotransferase was better after 1 year ( P < 0.02). Improvement of alkaline phosphatase and aspartate aminotransferase was comparable. Complete normalization of liver enzymes and bilirubin occurred more often in the high-dose group. Conclusion: High-dose UDCA induces a better response of liver biochemistry values than low-dose UDCA in CF patients with cholestatic liver disease. confirmed by the presence of typical pulmonary and digestive abnormalities and a sweat chloride concentration of more than 60 mmol/l. The presence of cholestatic liver disease was documented at separate occasions at least 3 months apart and Scand J Gastroenterol Downloaded from informahealthcare.com by University of Pennsylvania on 04/13/15 For personal use only. Scand J Gastroenterol Downloaded from informahealthcare.com by University of Pennsylvania on 04/13/15 For personal use only. Scand J Gastroenterol Downloaded from informahealthcare.com by University of Pennsylvania on 04/13/15 For personal use only.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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

Meeberg

Houwen

Sinaasappel

et al. 1997

Scandinavian Journal of Gastroenterology

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“…l h r effect of supplementation with UDCA on liver function test5 in C F has been unequivocal in all studies (32)(33)(34)(35)(36)(37)(38). but has been more pronounced with higher dosage.…”

Section: R Stwndivk B a L~~~d H L U C Lmentioning

confidence: 78%

Cystic Fibrosis Is Treatment with Ursodeoxycholic Acid of Value?

Strandvik

Lindblad

1994

Scandinavian Journal of Gastroenterology

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Strandvik B, Lindblad A. Cystic fibrosis: is treatment with ursodeoxycholic acid of value? Scand J Gastroenterol 1994;29 Suppl 204:65-7 Liver and biliary diseases are common complications in cystic fibrosis (CF) and may even be so severe that liver transplantation is indicated in cases with mild pulmonary disease. The most common complications are steatosis, fibrosis/cirrhosis, micro gallbladder, cholelithiasis, and sclerosing cholangitis. Ursodeoxycholic acid (UDCA) has been used in the treatment of gallstones and pathological liver function in CF. The effect on gallstones has been contradictory, and the effect on liver function tests has been shown to be dose-dependent. A significantly better effect has been shown on liver function tests during treatment over 1 year with doses of 15-20 mg/kg/day. Although supplementation with taurine did not further improve this effect, it has been used in many studies. The biliary bile acid pool was enriched by UDCA from about lo%, to 3 H O % , but a similar increase was not seen in serum. We have studied the long-term effect of UDCA in 10 patients with liver fibrosis or cirrhosis and/or sclerosing cholangitis. Liver function and liver morphology examined on liver biopsies by both light and transmission electron microscopy (TEM) were followed for 2 years. Preliminary results of this study are encouraging. UDCA is an interesting drug which binds to the hepatocyte membranes, stabilizing membrane structure in in vitro experiments and increasing the bicarbonate excretion in bile in pigs. The mechanism of the effect of UDCA in CF might not only be an increased choleresis; further studies will focus on these aspects.

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“…We recommend that future longitudinal studies should attempt to identify the underlying pathology of these abnormalities and patterns of early biochemical abnormalities that might more reliably predict evolving liver disease in CF, perhaps using the liver isoenzyme of alkaline phosphatase 19. The relation between liver abnormality and nutrition needs further study, both in CF and in other liver diseases.…”

Section: Discussionmentioning

confidence: 99%

The evolution of liver disease in cystic fibrosis

Ling

Wilkinson

Hollman

et al. 1999

Archives of Disease in Childhood

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Objectives-To describe prospectively the evolution of liver abnormalities in cystic fibrosis (CF), and to assess their impact on nutritional status. Study design-124 children (61 boys) with CF (median age, 5.4 years; range, 0.1-13.9) were followed longitudinally for a median of four years. Annual clinical examination, biochemistry, and ultrasound assessment were performed. Chrispin-Norman score, anthropometry, and bacterial colonisation of airway secretions were measured at each assessment. Results-At initial assessment, 45% of the patients had no liver abnormalities, 42% had biochemical abnormality, 35% ultrasound abnormality, and 6% had clinical abnormality of the liver. In this cross sectional analysis, abnormal biochemistry was present in 40% of children with ultrasound or clinical abnormalities, but when longitudinal follow up data were analysed, abnormal biochemistry preceded or coincided with abnormal ultrasound or clinical hepatosplenomegaly in three quarters of 53 children developing new abnormalities. Eighty four of 124 children (68%) showed ultrasound or clinical evidence of liver abnormality at some point during the four years of follow up. No association was found between liver disease and nutritional status. Conclusions-Hepatic abnormality was common in this group of children with CF, was often predicted by intermittent biochemical abnormalities, and was not associated with deterioration in nutritional status. (Arch Dis Child 1999;81:129-132)

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Effects of ursodeoxycholic acid on liver function in patients with cystic fibrosis and chronic cholestasis

Cited by 63 publications

References 10 publications

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

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

Cystic Fibrosis Is Treatment with Ursodeoxycholic Acid of Value?

The evolution of liver disease in cystic fibrosis

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