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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...

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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
Descripción
Sumario: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.