The Impact of Elexacaftor/Tezacaftor/Ivacaftor on Bone Mineral Density in Children and Adults with Cystic Fibrosis: A Systematic Review

The Impact of Elexacaftor/Tezacaftor/Ivacaftor on Bone Mineral Density in Children and Adults with Cystic Fibrosis: A Systematic Review | Authorea try { document.documentElement.classList.add('js'); } catch (e) { } var _gaq = _gaq || []; _gaq.push(['_setAccount', 'G-8VDV14Y67G']); _gaq.push(['_trackPageview']); (function() { var ga = document.createElement('script'); ga.type = 'text/javascript'; ga.async = true; ga.src = ('https:' == document.location.protocol ? 'https://ssl' : 'http://www') + '.google-analytics.com/ga.js'; var s = document.getElementsByTagName('script')[0]; s.parentNode.insertBefore(ga, s); })(); Skip to main content Preprints Collections Wiley Open Research IET Open Research Ecological Society of Japan All Collections About About Authorea FAQs Contact Us Quick Search anywhere Search for preprint articles, keywords, etc. Search Search ADVANCED SEARCH SCROLL This is a preprint and has not been peer reviewed. Data may be preliminary. 12 November 2025 V1 Latest version Share on The Impact of Elexacaftor/Tezacaftor/Ivacaftor on Bone Mineral Density in Children and Adults with Cystic Fibrosis: A Systematic Review Authors : O’Keeffe D [email protected] , Duggan C , and Cox DW Authors Info & Affiliations https://doi.org/10.22541/au.176296330.01619369/v1 209 views 116 downloads Contents Abstract Supplementary Material Information & Authors Metrics & Citations View Options References Figures Tables Media Share Abstract Background Low bone mineral density (BMD) and heightened fracture risk are recognised complications in individuals with cystic fibrosis (CF). The effect of the highly effective cystic fibrosis transmembrane conductance regulator (CFTR) therapy Elexacaftor/Tezacaftor/Ivacaftor (ETI) on BMD remains uncertain. Methods A systematic review was conducted following the PRISMA 2020 guidelines. Eligible studies included paediatric and/or adult CF patients who underwent BMD assessment before and after CFTR modulator therapy. Risk of bias was evaluated using the Risk of Bias in Non-Randomised Studies of Interventions (ROBINS-I). Results 37 studies were identified, of which 6 met the inclusion criteria, comprising 3 prospective and 3 retrospective studies. Five of these six studies observed improvements or stabilisation in BMD following CFTR modulator therapy, especially in adults. Additional findings included enhancements in bone biomarkers and body composition in some studies. However, the methodologies and follow-up durations varied considerably, and sample sizes were small. Conclusion This systematic review emphasises the potential benefits of CFTR modulators on bone health in individuals with CF. The limited number of studies available highlights a significant gap in the literature. It underscores the need for further, larger, multicentre studies assessing the effects of ETI on bone health in both children and adults with cystic fibrosis. Supplementary Material File (paeds pul dok.docx) Download 421.63 KB File (table_1_-_description_of_studies_2.docx) Download 182.79 KB Information & Authors Information Version history V1 Version 1 12 November 2025 Copyright This work is licensed under a Non Exclusive No Reuse License. Keywords bone mineral density cystic fibrosis elexacaftor/tezacaftor/ivacaftor Authors Affiliations O’Keeffe D [email protected] Children's Health Ireland at Crumlin Respiratory and Cystic Fibrosis Unit View all articles by this author Duggan C Children's Health Ireland at Crumlin Respiratory and Cystic Fibrosis Unit View all articles by this author Cox DW Children's Health Ireland at Crumlin Respiratory and Cystic Fibrosis Unit View all articles by this author Metrics & Citations Metrics Article Usage 209 views 116 downloads .FvxKWukQNSOunydq8rnd { width: 100px; } Citations Download citation O’Keeffe D, Duggan C, Cox DW. The Impact of Elexacaftor/Tezacaftor/Ivacaftor on Bone Mineral Density in Children and Adults with Cystic Fibrosis: A Systematic Review. Authorea . 12 November 2025. DOI: https://doi.org/10.22541/au.176296330.01619369/v1 If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. 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