Cargando…

Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction

BACKGROUND: Cystic fibrosis (CF) is characterized by CF transmembrane conductance regulator (CFTR) dysfunction. CFTR protein is expressed in human skeletal muscle; however, its impact on skeletal muscle is unknown. The objectives of this study were to compare quadriceps muscle size and quality betwe...

Descripción completa

Detalles Bibliográficos
Autores principales: Wu, Kenneth, Michalski, Anna, Sykes, Jenna, Batt, Jane, Stephenson, Anne L, Mathur, Sunita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9669672/
https://www.ncbi.nlm.nih.gov/pubmed/36380568
http://dx.doi.org/10.1177/14799731221131330
_version_ 1784832163005333504
author Wu, Kenneth
Michalski, Anna
Sykes, Jenna
Batt, Jane
Stephenson, Anne L
Mathur, Sunita
author_facet Wu, Kenneth
Michalski, Anna
Sykes, Jenna
Batt, Jane
Stephenson, Anne L
Mathur, Sunita
author_sort Wu, Kenneth
collection PubMed
description BACKGROUND: Cystic fibrosis (CF) is characterized by CF transmembrane conductance regulator (CFTR) dysfunction. CFTR protein is expressed in human skeletal muscle; however, its impact on skeletal muscle is unknown. The objectives of this study were to compare quadriceps muscle size and quality between adults with various severities of CFTR protein dysfunction. METHODS: We conducted a prospective, cross-sectional study comparing 34 adults with severe versus 18 with mild CFTR protein dysfunction, recruited from a specialized CF centre. Ultrasound images of rectus femoris cross-sectional area (RF-CSA) and quadriceps layer thickness for muscle size, and rectus femoris echogenicity (RF-ECHO) (muscle quality) were obtained. Multivariable linear regression models were developed using purposeful selection technique. RESULTS: People with severe CFTR protein dysfunction had larger RF-CSA by 3.22 cm(2), 95% CI (1.03, 5.41) cm(2), p=.0049], after adjusting for oral corticosteroid use and Pseudomonas aeruginosa colonization. However, a sensitivity analysis indicated that the result was influenced by the specific confounders being adjusted for in the model. We did not find any significant differences in quadriceps layer thickness or RF-ECHO between the two groups. CONCLUSION: We found no differential impact of the extent of diminished CFTR protein activity on quadriceps muscle size or quality in our study cohort. Based on these findings, CFTR mutation status cannot be used differentiate leg muscle size or quality in people with CF.
format Online
Article
Text
id pubmed-9669672
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher SAGE Publications
record_format MEDLINE/PubMed
spelling pubmed-96696722022-11-18 Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction Wu, Kenneth Michalski, Anna Sykes, Jenna Batt, Jane Stephenson, Anne L Mathur, Sunita Chron Respir Dis Original Paper BACKGROUND: Cystic fibrosis (CF) is characterized by CF transmembrane conductance regulator (CFTR) dysfunction. CFTR protein is expressed in human skeletal muscle; however, its impact on skeletal muscle is unknown. The objectives of this study were to compare quadriceps muscle size and quality between adults with various severities of CFTR protein dysfunction. METHODS: We conducted a prospective, cross-sectional study comparing 34 adults with severe versus 18 with mild CFTR protein dysfunction, recruited from a specialized CF centre. Ultrasound images of rectus femoris cross-sectional area (RF-CSA) and quadriceps layer thickness for muscle size, and rectus femoris echogenicity (RF-ECHO) (muscle quality) were obtained. Multivariable linear regression models were developed using purposeful selection technique. RESULTS: People with severe CFTR protein dysfunction had larger RF-CSA by 3.22 cm(2), 95% CI (1.03, 5.41) cm(2), p=.0049], after adjusting for oral corticosteroid use and Pseudomonas aeruginosa colonization. However, a sensitivity analysis indicated that the result was influenced by the specific confounders being adjusted for in the model. We did not find any significant differences in quadriceps layer thickness or RF-ECHO between the two groups. CONCLUSION: We found no differential impact of the extent of diminished CFTR protein activity on quadriceps muscle size or quality in our study cohort. Based on these findings, CFTR mutation status cannot be used differentiate leg muscle size or quality in people with CF. SAGE Publications 2022-11-15 /pmc/articles/PMC9669672/ /pubmed/36380568 http://dx.doi.org/10.1177/14799731221131330 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Original Paper
Wu, Kenneth
Michalski, Anna
Sykes, Jenna
Batt, Jane
Stephenson, Anne L
Mathur, Sunita
Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
title Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
title_full Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
title_fullStr Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
title_full_unstemmed Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
title_short Comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
title_sort comparison of quadriceps muscle size and quality in adults with cystic fibrosis with different severities of cystic fibrosis transmembrane conductance regulator protein dysfunction
topic Original Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9669672/
https://www.ncbi.nlm.nih.gov/pubmed/36380568
http://dx.doi.org/10.1177/14799731221131330
work_keys_str_mv AT wukenneth comparisonofquadricepsmusclesizeandqualityinadultswithcysticfibrosiswithdifferentseveritiesofcysticfibrosistransmembraneconductanceregulatorproteindysfunction
AT michalskianna comparisonofquadricepsmusclesizeandqualityinadultswithcysticfibrosiswithdifferentseveritiesofcysticfibrosistransmembraneconductanceregulatorproteindysfunction
AT sykesjenna comparisonofquadricepsmusclesizeandqualityinadultswithcysticfibrosiswithdifferentseveritiesofcysticfibrosistransmembraneconductanceregulatorproteindysfunction
AT battjane comparisonofquadricepsmusclesizeandqualityinadultswithcysticfibrosiswithdifferentseveritiesofcysticfibrosistransmembraneconductanceregulatorproteindysfunction
AT stephensonannel comparisonofquadricepsmusclesizeandqualityinadultswithcysticfibrosiswithdifferentseveritiesofcysticfibrosistransmembraneconductanceregulatorproteindysfunction
AT mathursunita comparisonofquadricepsmusclesizeandqualityinadultswithcysticfibrosiswithdifferentseveritiesofcysticfibrosistransmembraneconductanceregulatorproteindysfunction