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Effect of Salbutamol on Respiratory Muscle Strength in Spinal Muscular Atrophy

Sonia Khirani1, Ivana Dabaj2, Alessandro Amaddeo3

  • 1ASV Santé, Gennevilliers, France; AP-HP, Hôpital Necker-Enfants Malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.

Pediatric Neurology
|July 3, 2017
PubMed

Insights

Oral salbutamol improved respiratory muscle strength in children with spinal muscular atrophy (SMA). Long-term treatment enhanced inspiratory muscle strength and lung function in SMA II patients.

Area of Science:

  • Pediatric Neurology
  • Respiratory Medicine
  • Genetics

Background:

  • Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder affecting motor neurons.
  • Oral salbutamol has demonstrated potential clinical benefits in managing SMA.
  • Respiratory muscle weakness is a significant complication in SMA, impacting patient prognosis.

Purpose of the Study:

  • To investigate the effects of long-term oral salbutamol on respiratory muscle strength in pediatric SMA patients.
  • To compare respiratory function between SMA II patients treated with salbutamol and historical control subjects.

Main Methods:

  • Prospective assessment of lung and respiratory muscle function in children with SMA receiving daily oral salbutamol for over one year.
  • Comparison of respiratory parameters including maximal static inspiratory pressure, sniff nasal inspiratory pressure, and slow vital capacity.
  • Analysis of data from seven children (one SMA I, five SMA II, one SMA III) with a treatment duration of 23 ± 8 months.

Main Results:

  • Children with SMA II treated with oral salbutamol showed statistically significant improvements in maximal static inspiratory pressure, sniff nasal inspiratory pressure, and slow vital capacity compared to age-matched controls (P < 0.05).
  • The study included a small cohort of seven children aged 4-10 years with varying SMA types.

Conclusions:

  • Long-term administration of oral salbutamol demonstrates beneficial effects on respiratory function in children with spinal muscular atrophy.
  • The findings suggest that oral salbutamol may enhance inspiratory muscle strength in SMA II patients.
Abstract

Keywords:
lung functionmotor functionrespiratory musclessalbutamolspinal muscular atrophy

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