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A systematic review of the impact of Elexacaftor/Tezacaftor/Ivacaftor on body composition in people with cystic fibrosis

European Journal of Clinical Nutrition volume 79, pages 715–722 (2025) Cite this article

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Abstract

Elexacaftor/Tezacaftor/Ivacaftor (ETI) has led to improved lung function, life expectancy, and body mass index for people with Cystic Fibrosis (CF). The aim of this systematic review was to evaluate the impact that ETI has had on body composition in people with CF. A systematic review was performed using MEDLINE, EMBASE, CINAHL, and the Cochrane Central Register of Controlled Trials. Quality assessment using the Joanna Briggs Institute critical appraisal tools were performed. Results were summarised narratively. Five observational cohort studies involving a total of 185 participants were reviewed. Three studies showed an increase in fat mass (7.0–8.6 kg, 13.2–14.3 kg, and 13.4–15.5 kg). Two studies reported an increase in fat-free mass (49.4–50.1 kg, 52.5–55 kg), while one reported a decrease (50.5–48.9 kg). Two studies found an increase in fat mass index (4.1–6.3 kgm/2 and 4.7–5.4 kg/m2) and fat-free mass index (17.4–17.7 kg/m2 and 18.1–18.8 kg/m2). Two studies observed an increase in percentage body fat mass (12.1–15.4% and 23.1–27.6%). Four studies were classified as low quality, while one was considered medium quality. This review suggest that commencing ETI results in changes in body composition. Firm conclusions about the type and distribution of change in body composition cannot be made due to limited studies, high heterogeneity, and methodical weaknesses. It highlights the necessity for higher quality and longer-term studies to explore the impact that ETI is having on body composition.

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Acknowledgements

Laura Kinsey was funded as part of an Applied Research Collaboration for Greater Manchester (ARC-GM) pre-doctoral fellowship.

Funding

LK was funded as part of an Applied Research Collaboration for Greater Manchester (ARC-GM) pre-doctoral fellowship.

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Authors and Affiliations

  1. Manchester Adult Cystic Fibrosis Centre, Manchester University NHS Foundation Trust, Wythenshawe Hospital, Manchester, M23 9LT, United Kingdom

    Laura Kinsey, Hannah Winterbottom, Lydia Hudson & Andrew M. Jones

  2. Intestinal Failure Unit, Salford Royal Infirmary, Northern Care Alliance Foundation Trust, Manchester, United Kingdom

    Simon Lal & Sorrel Burden

  3. School of Health Sciences, Faculty of Biology, Medicine, and Health, University of Manchester, Oxford Road, Manchester, M13 6PL, United Kingdom

    Sorrel Burden

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  1. Laura Kinsey
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  2. Hannah Winterbottom
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  3. Lydia Hudson
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Contributions

LK developed the search strategy, conducted the search. LK and HW undertook the study selection. LK undertook the data extract and HW checked data extraction. LK and LH conducted the risk of bias assessment. LK conducted data synthesis and collated results. SL and SB provided expert opinion on body composition and systematic reviews. AMJ provided expert opinion on Cystic Fibrosis. LK wrote the first draft of the manuscript. All authors provided feedback and approved the final manuscript.

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Correspondence to Laura Kinsey.

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Kinsey, L., Winterbottom, H., Hudson, L. et al. A systematic review of the impact of Elexacaftor/Tezacaftor/Ivacaftor on body composition in people with cystic fibrosis. Eur J Clin Nutr 79, 715–722 (2025). https://doi.org/10.1038/s41430-025-01589-y

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