Please use this identifier to cite or link to this item: https://dora.health.qld.gov.au/qldresearchjspui/handle/1/4943
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dc.contributor.authorColombo, C.en
dc.contributor.authorLewindon, P. J.en
dc.contributor.authorRamm, G. A.en
dc.contributor.authorOoi, C. Y.en
dc.contributor.authorAlghisi, F.en
dc.contributor.authorKashirskaya, N.en
dc.contributor.authorKondratyeva, E.en
dc.contributor.authorCorti, F.en
dc.contributor.authorPadoan, R.en
dc.contributor.authorAsherova, I.en
dc.contributor.authorEvans, H.en
dc.contributor.authorde Monestrol, I.en
dc.contributor.authorStrandvik, B.en
dc.contributor.authorLindblad, A.en
dc.contributor.authorOliver, M.en
dc.contributor.authorAlicandro, G.en
dc.date.accessioned2022-11-07T23:57:52Z-
dc.date.available2022-11-07T23:57:52Z-
dc.date.issued2022en
dc.identifier.citation21, (2), 2022, p. 220-226en
dc.identifier.otherRISen
dc.identifier.urihttp://dora.health.qld.gov.au/qldresearchjspui/handle/1/4943-
dc.description.abstractBackground: The efficacy and safety of ursodeoxycholic acid (UDCA) for the treatment of liver disease associated with cystic fibrosis (CF) are under discussion, and clinical practice varies among centers. The study aimed at evaluating if the incidence of severe liver disease differs between CF centers routinely prescribing or not prescribing UDCA. Methods: We carried out a retrospective multicenter cohort study including 1591 CF patients (1192 patients from UDCA-prescribing centers and 399 from non-prescribing centers) born between 1990 and 2007 and followed from birth up to 31 December 2016. We computed the crude cumulative incidence (CCI) of portal hypertension (PH) at the age of 20 years in the two groups and estimated the subdistribution hazard ratio (HR) through a Fine and Gray model. Results: Over the observation period, 114 patients developed PH: 90 (7.6%) patients followed-up in UDCA prescribing centers and 24 (6.0%) in non-prescribing centers. The CCI of PH at 20 years was 10.1% (95% CI: 7.9-12.3) in UDCA-prescribing and 7.7% (95% CI: 4.6-10.7) in non-prescribing centers. The HR among patients followed in prescribing centers indicated no significant difference in the rate of PH either in the unadjusted model (HR: 1.21, 95% CI: 0.69-2.11) or in the model adjusted for pancreatic insufficiency (HR: 1.28, 95% CI: 0.77-2.12). Conclusions: CF patients followed-up in UDCA prescribing centers did not show a lower incidence of PH as compared to those followed in centers not prescribing UDCA. These results question the utility of UDCA in reducing the occurrence of severe liver disease in CF.L20115776282021-04-13 <br />en
dc.language.isoenen
dc.relation.ispartofJournal of Cystic Fibrosisen
dc.titleUrsodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort studyen
dc.typeArticleen
dc.identifier.doi10.1016/j.jcf.2021.03.014en
dc.subject.keywordsoverall survivalen
dc.subject.keywordsportal hypertensionen
dc.subject.keywordsretrospective studyen
dc.subject.keywordssubstitution therapyen
dc.subject.keywordstotal parenteral nutritionen
dc.subject.keywordsursodeoxycholic acidadolescenten
dc.subject.keywordsadulten
dc.subject.keywordsarticleen
dc.subject.keywordscause of deathen
dc.subject.keywordschilden
dc.subject.keywordsclinical practiceen
dc.subject.keywordscohort analysisen
dc.subject.keywordscomparative studyen
dc.subject.keywordscontrolled studyen
dc.subject.keywordscumulative incidenceen
dc.subject.keywordscystic fibrosisen
dc.subject.keywordsdisease associationen
dc.subject.keywordsdrug efficacyen
dc.subject.keywordsdrug safetyen
dc.subject.keywordsechographyen
dc.subject.keywordsfemaleen
dc.subject.keywordsfollow upen
dc.subject.keywordsgene mutationen
dc.subject.keywordshospital admissionen
dc.subject.keywordshumanen
dc.subject.keywordsinfanten
dc.subject.keywordsinformed consenten
dc.subject.keywordsliver diseaseen
dc.subject.keywordsliver transplantationen
dc.subject.keywordsmajor clinical studyen
dc.subject.keywordsmaleen
dc.subject.keywordsmaximum permissible doseen
dc.subject.keywordsmulticenter studyen
dc.relation.urlhttps://www.embase.com/search/results?subaction=viewrecord&id=L2011577628&from=exporthttp://dx.doi.org/10.1016/j.jcf.2021.03.014 |en
dc.identifier.risid2648en
dc.description.pages220-226en
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.cerifentitytypePublications-
item.fulltextNo Fulltext-
item.grantfulltextnone-
item.openairetypeArticle-
item.languageiso639-1en-
Appears in Sites:Children's Health Queensland Publications
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