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Updated: Apr 17, 2026

Repeated Measurement of Respiratory Muscle Activity and Ventilation in Mouse Models of Neuromuscular Disease
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The value of respiratory muscle testing in children with neuromuscular disease

Brigitte Fauroux1, Susana Quijano-Roy2, Isabelle Desguerre3

  • 1Pediatric Noninvasive Ventilation and Sleep Unit, Necker University Hospital, AP-HP, Paris; Research Unit Inserm U955 Equipe 13, Créteil.

Chest
|February 4, 2015
PubMed

Insights

Simple breathing tests can assess respiratory muscle strength in children with neuromuscular disease (NMD). This monitoring aids in understanding disease progression and guiding clinical trials for better management.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Respiratory Physiology

Background:

  • Standard lung function tests rely on volitional maneuvers, challenging for young children with neuromuscular disease (NMD).
  • Assessing respiratory muscle strength is crucial for managing NMD in pediatric populations.

Purpose of the Study:

  • To evaluate the utility of nonvolitional respiratory maneuvers for assessing respiratory muscle function in children with NMD.
  • To explore how monitoring respiratory muscles can inform NMD natural history and clinical trial design.

Main Methods:

  • Measurement of esophageal and gastric pressures during spontaneous breathing.
  • Analysis of simple, natural maneuvers like sniffing and coughing.
  • Application in pediatric patients diagnosed with neuromuscular disease.

Main Results:

  • Nonvolitional maneuvers provide valuable insights into respiratory muscle strength and endurance in young children.
  • This approach offers an alternative when volitional testing is not feasible.
  • Data gathered can enhance understanding of NMD progression.

Conclusions:

  • Monitoring respiratory muscle function through natural maneuvers is beneficial for children with NMD.
  • This method aids in evaluating disease severity and guiding clinical management.
  • It can improve the identification of optimal endpoints and patient selection for clinical trials.

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