Skeletal muscle function in young patients with cystic fibrosis

Publikation: Beitrag in FachzeitschriftArtikelForschungPeer-Review

Autoren

  • Lothar Stein
  • Constanze Pacht
  • Sibylle Junge
  • Tobias S. Kaeding
  • Momme Kück
  • Norbert Maassen
  • Torge Wittke
  • Vladimir Shushakov

Organisationseinheiten

Externe Organisationen

  • Medizinische Hochschule Hannover (MHH)
Forschungs-netzwerk anzeigen

Details

OriginalspracheEnglisch
Seiten (von - bis)364-373
Seitenumfang10
FachzeitschriftPediatric Exercise Science
Jahrgang28
Ausgabenummer3
PublikationsstatusVeröffentlicht - Aug. 2016

Abstract

Purpose: Defects in the gene encoding the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) cause CF. Absence of the CFTR may result in skeletal muscle dysfunction. Here, we tested skeletal muscle function in male adolescent patients with CF. Methods: Ten CF and 10 control participants (age: 16.8 ± 0.6 years) performed 7 repetitive sets of maximum voluntary contractions (MVCs) and underwent an isometric fatigue test of the knee extensors. Electromyography (EMG) activity was recorded from the m. vastus lateralis (VL) and m. vastus medialis (VM). Results: In CF, the MVC torque was lower and correlated with the predicted forced expiratory volume in one second (r = .73, p = .012, n = 10). The M-wave in the VL was shorter in CF than in controls (18.6 ± 0.5 vs. 20.3 ± 0.5 ms, p < .028). In the VM, both the M-wave (4.96 ± 0.61 vs. 7.97 ± 0.60 mV, p = .001) and the EMG (0.29 ± 0.04 vs. 0.47 ± 0.04 mV, p = .004) amplitudes were smaller in CF. Conclusion: The differences in the VL and VM EMG signals between the groups indicate that the lower MVC torque in CF did not result from the direct impact of a CFTR defect on the sarcolemmal excitability; the differences more likely resulted from the less developed musculature in the patients with CF.

Zitieren

Skeletal muscle function in young patients with cystic fibrosis. / Stein, Lothar; Pacht, Constanze; Junge, Sibylle et al.
in: Pediatric Exercise Science, Jahrgang 28, Nr. 3, 08.2016, S. 364-373.

Publikation: Beitrag in FachzeitschriftArtikelForschungPeer-Review

Stein, L, Pacht, C, Junge, S, Kaeding, TS, Kück, M, Maassen, N, Wittke, T & Shushakov, V 2016, 'Skeletal muscle function in young patients with cystic fibrosis', Pediatric Exercise Science, Jg. 28, Nr. 3, S. 364-373. https://doi.org/10.1123/pes.2015-0184
Stein, L., Pacht, C., Junge, S., Kaeding, T. S., Kück, M., Maassen, N., Wittke, T., & Shushakov, V. (2016). Skeletal muscle function in young patients with cystic fibrosis. Pediatric Exercise Science, 28(3), 364-373. https://doi.org/10.1123/pes.2015-0184
Stein L, Pacht C, Junge S, Kaeding TS, Kück M, Maassen N et al. Skeletal muscle function in young patients with cystic fibrosis. Pediatric Exercise Science. 2016 Aug;28(3):364-373. doi: 10.1123/pes.2015-0184
Stein, Lothar ; Pacht, Constanze ; Junge, Sibylle et al. / Skeletal muscle function in young patients with cystic fibrosis. in: Pediatric Exercise Science. 2016 ; Jahrgang 28, Nr. 3. S. 364-373.
Download
@article{81e5a8601f9948c38c5c94220644ead2,
title = "Skeletal muscle function in young patients with cystic fibrosis",
abstract = "Purpose: Defects in the gene encoding the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) cause CF. Absence of the CFTR may result in skeletal muscle dysfunction. Here, we tested skeletal muscle function in male adolescent patients with CF. Methods: Ten CF and 10 control participants (age: 16.8 ± 0.6 years) performed 7 repetitive sets of maximum voluntary contractions (MVCs) and underwent an isometric fatigue test of the knee extensors. Electromyography (EMG) activity was recorded from the m. vastus lateralis (VL) and m. vastus medialis (VM). Results: In CF, the MVC torque was lower and correlated with the predicted forced expiratory volume in one second (r = .73, p = .012, n = 10). The M-wave in the VL was shorter in CF than in controls (18.6 ± 0.5 vs. 20.3 ± 0.5 ms, p < .028). In the VM, both the M-wave (4.96 ± 0.61 vs. 7.97 ± 0.60 mV, p = .001) and the EMG (0.29 ± 0.04 vs. 0.47 ± 0.04 mV, p = .004) amplitudes were smaller in CF. Conclusion: The differences in the VL and VM EMG signals between the groups indicate that the lower MVC torque in CF did not result from the direct impact of a CFTR defect on the sarcolemmal excitability; the differences more likely resulted from the less developed musculature in the patients with CF.",
author = "Lothar Stein and Constanze Pacht and Sibylle Junge and Kaeding, {Tobias S.} and Momme K{\"u}ck and Norbert Maassen and Torge Wittke and Vladimir Shushakov",
note = "Publisher Copyright: {\textcopyright} 2016 Human Kinetics, Inc.",
year = "2016",
month = aug,
doi = "10.1123/pes.2015-0184",
language = "English",
volume = "28",
pages = "364--373",
journal = "Pediatric Exercise Science",
issn = "0899-8493",
publisher = "Human Kinetics Publishers Inc.",
number = "3",

}

Download

TY - JOUR

T1 - Skeletal muscle function in young patients with cystic fibrosis

AU - Stein, Lothar

AU - Pacht, Constanze

AU - Junge, Sibylle

AU - Kaeding, Tobias S.

AU - Kück, Momme

AU - Maassen, Norbert

AU - Wittke, Torge

AU - Shushakov, Vladimir

N1 - Publisher Copyright: © 2016 Human Kinetics, Inc.

PY - 2016/8

Y1 - 2016/8

N2 - Purpose: Defects in the gene encoding the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) cause CF. Absence of the CFTR may result in skeletal muscle dysfunction. Here, we tested skeletal muscle function in male adolescent patients with CF. Methods: Ten CF and 10 control participants (age: 16.8 ± 0.6 years) performed 7 repetitive sets of maximum voluntary contractions (MVCs) and underwent an isometric fatigue test of the knee extensors. Electromyography (EMG) activity was recorded from the m. vastus lateralis (VL) and m. vastus medialis (VM). Results: In CF, the MVC torque was lower and correlated with the predicted forced expiratory volume in one second (r = .73, p = .012, n = 10). The M-wave in the VL was shorter in CF than in controls (18.6 ± 0.5 vs. 20.3 ± 0.5 ms, p < .028). In the VM, both the M-wave (4.96 ± 0.61 vs. 7.97 ± 0.60 mV, p = .001) and the EMG (0.29 ± 0.04 vs. 0.47 ± 0.04 mV, p = .004) amplitudes were smaller in CF. Conclusion: The differences in the VL and VM EMG signals between the groups indicate that the lower MVC torque in CF did not result from the direct impact of a CFTR defect on the sarcolemmal excitability; the differences more likely resulted from the less developed musculature in the patients with CF.

AB - Purpose: Defects in the gene encoding the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) cause CF. Absence of the CFTR may result in skeletal muscle dysfunction. Here, we tested skeletal muscle function in male adolescent patients with CF. Methods: Ten CF and 10 control participants (age: 16.8 ± 0.6 years) performed 7 repetitive sets of maximum voluntary contractions (MVCs) and underwent an isometric fatigue test of the knee extensors. Electromyography (EMG) activity was recorded from the m. vastus lateralis (VL) and m. vastus medialis (VM). Results: In CF, the MVC torque was lower and correlated with the predicted forced expiratory volume in one second (r = .73, p = .012, n = 10). The M-wave in the VL was shorter in CF than in controls (18.6 ± 0.5 vs. 20.3 ± 0.5 ms, p < .028). In the VM, both the M-wave (4.96 ± 0.61 vs. 7.97 ± 0.60 mV, p = .001) and the EMG (0.29 ± 0.04 vs. 0.47 ± 0.04 mV, p = .004) amplitudes were smaller in CF. Conclusion: The differences in the VL and VM EMG signals between the groups indicate that the lower MVC torque in CF did not result from the direct impact of a CFTR defect on the sarcolemmal excitability; the differences more likely resulted from the less developed musculature in the patients with CF.

UR - http://www.scopus.com/inward/record.url?scp=84979680187&partnerID=8YFLogxK

U2 - 10.1123/pes.2015-0184

DO - 10.1123/pes.2015-0184

M3 - Article

C2 - 27045385

AN - SCOPUS:84979680187

VL - 28

SP - 364

EP - 373

JO - Pediatric Exercise Science

JF - Pediatric Exercise Science

SN - 0899-8493

IS - 3

ER -