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Skeletal muscle contributions to reduced fitness in cystic fibrosis youth

BACKGROUND: Increased maximal oxygen uptake (V̇O(2max)) 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 potent...

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Autores principales: Tomlinson, Owen William, Barker, Alan Robert, Fulford, Jonathan, Wilson, Paul, Shelley, James, Oades, Patrick John, Williams, Craig Anthony
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10300555/
https://www.ncbi.nlm.nih.gov/pubmed/37388288
http://dx.doi.org/10.3389/fped.2023.1211547
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author Tomlinson, Owen William
Barker, Alan Robert
Fulford, Jonathan
Wilson, Paul
Shelley, James
Oades, Patrick John
Williams, Craig Anthony
author_facet Tomlinson, Owen William
Barker, Alan Robert
Fulford, Jonathan
Wilson, Paul
Shelley, James
Oades, Patrick John
Williams, Craig Anthony
author_sort Tomlinson, Owen William
collection PubMed
description BACKGROUND: Increased maximal oxygen uptake (V̇O(2max)) 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 V̇O(2max), although exact mechanisms remain unknown. This study utilises gold-standard methodologies to control for the residual effects of muscle size from V̇O(2max) 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 V̇O(2max) 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 V̇O(2max), once mCSA and TMV were controlled for. RESULTS: V̇O(2max) 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 V̇O(2max) 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.
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spelling pubmed-103005552023-06-29 Skeletal muscle contributions to reduced fitness in cystic fibrosis youth Tomlinson, Owen William Barker, Alan Robert Fulford, Jonathan Wilson, Paul Shelley, James Oades, Patrick John Williams, Craig Anthony Front Pediatr Pediatrics BACKGROUND: Increased maximal oxygen uptake (V̇O(2max)) 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 V̇O(2max), although exact mechanisms remain unknown. This study utilises gold-standard methodologies to control for the residual effects of muscle size from V̇O(2max) 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 V̇O(2max) 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 V̇O(2max), once mCSA and TMV were controlled for. RESULTS: V̇O(2max) 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 V̇O(2max) 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. Frontiers Media S.A. 2023-06-14 /pmc/articles/PMC10300555/ /pubmed/37388288 http://dx.doi.org/10.3389/fped.2023.1211547 Text en © 2023 Tomlinson, Barker, Fulford, Wilson, Shelley, Oades and Williams. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . 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.
spellingShingle Pediatrics
Tomlinson, Owen William
Barker, Alan Robert
Fulford, Jonathan
Wilson, Paul
Shelley, James
Oades, Patrick John
Williams, Craig Anthony
Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
title Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
title_full Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
title_fullStr Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
title_full_unstemmed Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
title_short Skeletal muscle contributions to reduced fitness in cystic fibrosis youth
title_sort skeletal muscle contributions to reduced fitness in cystic fibrosis youth
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10300555/
https://www.ncbi.nlm.nih.gov/pubmed/37388288
http://dx.doi.org/10.3389/fped.2023.1211547
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