Respiratory muscle testing: a valuable tool for children with neuromuscular disorders

Frédéric Nicot1, Nicholas Hart, Véronique Forin

  • 1AP-HP, Hopital Armand Trousseau, Pediatric Pulmonary Department, Research Unit INSERM U 719, Université Pierre et Marie Curie-Paris 6, Paris, France.

Insights

Simple volitional tests effectively assess respiratory muscle strength in children with neuromuscular disorders. These methods correlate well with nonvolitional tests and lung capacity measurements, offering a valuable clinical tool.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Respiratory Physiology

Background:

  • Limited data exists on respiratory muscle performance in pediatric patients diagnosed with neuromuscular disorders.
  • Assessing respiratory muscle strength is crucial for managing these conditions.

Purpose of the Study:

  • To evaluate respiratory muscle strength using both volitional and nonvolitional testing methods.
  • To compare the results of these tests with forced vital capacity measurements in children.

Main Methods:

  • Inspiratory muscle strength was measured via transdiaphragmatic and esophageal pressures during volitional and nonvolitional maneuvers.
  • Expiratory muscle strength was assessed by measuring gastric pressure during a cough.
  • Lung volumes were determined using forced vital capacity measurements.

Main Results:

  • Forty-one pediatric patients (ages 2-18) with Duchenne muscular dystrophy, spinal amyotrophy, or congenital myopathy participated.
  • A positive correlation was found between volitional (sniff maneuver) and nonvolitional (phrenic nerve stimulation) tests.
  • Forced vital capacity correlated significantly with sniff transdiaphragmatic pressure and cough gastric pressure.

Conclusions:

  • Volitional respiratory muscle tests demonstrate strong correlation with nonvolitional tests and forced vital capacity.
  • Simple volitional tests are a valuable and practical tool for assessing respiratory muscle strength in pediatric patients with neuromuscular disorders.
Abstract

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