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Assessing Functional Performance in the Mdx Mouse Model
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Ventilator-induced diaphragmatic dysfunction in MDX mice.

Feng Liang1, Tong Li1, Ilan Azuelos1

  • 1Meakins-Christie Laboratories, and Translational Research in Respiratory Diseases Program, McGill University Health Centre and Research Institute, 1001 Decarie Boulevard, Montreal, Quebec, H4A 3J1, Canada.

Muscle & Nerve
|August 9, 2017
PubMed
Summary
This summary is machine-generated.

Mechanical ventilation (MV) rapidly impairs diaphragm function and causes biochemical changes in dystrophic mice, suggesting a risk of ventilator-induced diaphragmatic dysfunction (VIDD) in Duchenne muscular dystrophy (DMD) patients.

Keywords:
Duchenne muscular dystrophyVIDDdiaphragm disusehome mechanical ventilationmdx miceneuromuscular disorder

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Area of Science:

  • Biomedical Engineering
  • Respiratory Physiology
  • Muscle Biology

Background:

  • Duchenne muscular dystrophy (DMD) patients often require mechanical ventilation (MV) for respiratory support.
  • The impact of MV on the diaphragm in DMD has not been previously investigated.

Purpose of the Study:

  • To investigate the susceptibility of the dystrophic diaphragm to ventilator-induced diaphragmatic dysfunction (VIDD).

Main Methods:

  • Dystrophic mdx mice were subjected to 6 hours of MV or served as controls.
  • Diaphragmatic force production and biochemical markers of stress, metabolism, and proteolysis were assessed.

Main Results:

  • MV significantly reduced maximal diaphragmatic force production.
  • MV induced oxidative stress, STAT3 phosphorylation, and upregulated muscle wasting pathways (autophagy, E3 ubiquitin ligases, myostatin).

Conclusions:

  • Short-term MV causes rapid diaphragm dysfunction and biochemical alterations consistent with VIDD in mdx mice.
  • Findings suggest potential implications for managing MV in DMD patients.