Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
dc.contributor.author | Tomlinson, OW | |
dc.contributor.author | Barker, AR | |
dc.contributor.author | Fulford, J | |
dc.contributor.author | Wilson, P | |
dc.contributor.author | Shelley, J | |
dc.contributor.author | Oades, PJ | |
dc.contributor.author | Williams, CA | |
dc.date.accessioned | 2023-06-14T08:14:48Z | |
dc.date.issued | 2023-06-14 | |
dc.date.updated | 2023-06-14T08:05:49Z | |
dc.description.abstract | Background: Increased maximal oxygen uptake (V̇ O2max) is beneficial in children with cystic fibrosis (CF) but remains lower compared to healthy peers. Intrinsic metabolic deficiencies within skeletal muscle (muscle “quality”) and skeletal muscle size (muscle “quantity”) are both proposed as potential causes for the lower V̇ O2max, although exact mechanisms remain unknown. This study utilises gold-standard methodologies to control for the residual effects of muscle size from V̇ O2max to address this “quality” vs. “quantity” debate. Methods: Fourteen children (7 CF vs. 7 age- and sex-matched controls) were recruited. Parameters of muscle size – muscle cross-sectional area (mCSA) and thigh muscle volume (TMV) were derived from magnetic resonance imaging, and V̇ O2max obtained via cardiopulmonary exercise testing. Allometric scaling removed residual effects of muscle size, and independent samples t-tests and effect sizes (ES) identified differences between groups in V̇ O2max, once mCSA and TMV were controlled for. Results: V̇ O2max was shown to be lower in the CF group, relative to controls, with large ES being identified when allometrically scaled to mCSA (ES = 1.76) and TMV (ES = 0.92). Reduced peak work rate was also identified in the CF group when allometrically controlled for mCSA (ES = 1.18) and TMV (ES = 0.45). Conclusions: A lower V̇ O2max was still observed in children with CF after allometrically scaling for muscle size, suggesting reduced muscle “quality” in CF (as muscle “quantity” is fully controlled for). This observation likely reflects intrinsic metabolic defects within CF skeletal muscle. | en_GB |
dc.description.sponsorship | National Institute for Health Research | en_GB |
dc.description.sponsorship | Royal Devon & Exeter CF Research Charitable Fund | en_GB |
dc.description.sponsorship | University of Exeter | en_GB |
dc.identifier.citation | Vol. 11, article 1211547 | en_GB |
dc.identifier.doi | https://doi.org/10.3389/fped.2023.1211547 | |
dc.identifier.grantnumber | CRF/2016/10027 | en_GB |
dc.identifier.uri | http://hdl.handle.net/10871/133378 | |
dc.identifier | ORCID: 0000-0003-4063-7682 (Tomlinson, Owen William) | |
dc.language.iso | en | en_GB |
dc.publisher | Frontiers Media | en_GB |
dc.rights | © 2023 Tomlinson, Barker, Fulford, Wilson, Shelley, Oades and Williams. This is an openaccess article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. | en_GB |
dc.subject | exercise capacity | en_GB |
dc.subject | modelling | en_GB |
dc.subject | adolescence | en_GB |
dc.subject | respiratory disease | en_GB |
dc.subject | musculoskeletal | en_GB |
dc.title | Skeletal muscle contributions to reduced fitness in cystic fibrosis youth | en_GB |
dc.type | Article | en_GB |
dc.date.available | 2023-06-14T08:14:48Z | |
dc.description | This is the final version. Available from Frontiers Media via the DOI in this record. | en_GB |
dc.description | Data availability statement: The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation. | en_GB |
dc.identifier.eissn | 2296-2360 | |
dc.identifier.journal | Frontiers in Pediatrics | en_GB |
dc.relation.ispartof | Frontiers in Pediatrics, 11 | |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | en_GB |
dcterms.dateAccepted | 2023-06-02 | |
rioxxterms.version | VoR | en_GB |
rioxxterms.licenseref.startdate | 2023-06-14 | |
rioxxterms.type | Journal Article/Review | en_GB |
refterms.dateFCD | 2023-06-14T08:11:59Z | |
refterms.versionFCD | VoR | |
refterms.dateFOA | 2023-06-14T08:14:52Z | |
refterms.panel | A | en_GB |
refterms.dateFirstOnline | 2023-06-14 |
Files in this item
This item appears in the following Collection(s)
Except where otherwise noted, this item's licence is described as © 2023 Tomlinson, Barker, Fulford, Wilson,
Shelley, Oades and Williams. This is an openaccess article distributed under the terms of the
Creative Commons Attribution License (CC BY).
The use, distribution or reproduction in other
forums is permitted, provided the original
author(s) and the copyright owner(s) are
credited and that the original publication in this
journal is cited, in accordance with accepted
academic practice. No use, distribution or
reproduction is permitted which does not
comply with these terms.