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Pediatr Neurol. 2017 Aug;73:78-87.e1. doi: 10.1016/j.pediatrneurol.2017.04.013. Epub 2017 Apr 20.

Effect of Salbutamol on Respiratory Muscle Strength in Spinal Muscular Atrophy.

Author information

1
ASV Santé, Gennevilliers, France; AP-HP, Hôpital Necker-Enfants Malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.
2
AP-HP, Hôpital Raymond Poincaré, Hôpitaux Universitaires Paris-Ile-de-France Ouest, Pôle pédiatrique, Service de Pédiatrie, Garches, France; Centre de Référence Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), Garches, France; Réseau National de Maladies Neuromusculaires FILNEMUS, Garches, France.
3
AP-HP, Hôpital Necker-Enfants Malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France; INSERM U955, Créteil, France; Paris Descartes University, Paris, France.
4
AP-HP, Hôpital Necker-Enfants Malades, Pediatric Noninvasive Ventilation and Sleep Unit, Paris, France.
5
Hôpitaux Universitaires Paris Île-de-France Ouest, Unité de Recherche Clinique, Boulogne, France.
6
AP-HP, Hôpital Raymond Poincaré, Hôpitaux Universitaires Paris-Ile-de-France Ouest, Pôle pédiatrique, Service de Pédiatrie, Garches, France.
7
AP-HP, Hôpital Raymond Poincaré, Hôpitaux Universitaires Paris-Ile-de-France Ouest, Pôle pédiatrique, Service de Pédiatrie, Garches, France; Centre de Référence Maladies Neuromusculaires Garches-Necker-Mondor-Hendaye (GNMH), Garches, France; Réseau National de Maladies Neuromusculaires FILNEMUS, Garches, France; INSERM U1179, Université Versailles Saint-Quentin (UVSQ), UFR des sciences de la santé Simone Veil, Montigny, France. Electronic address: susana.quijano-roy@aphp.fr.

Abstract

BACKGROUND:

Oral salbutamol has shown clinical benefits in spinal muscular atrophy (SMA). We studied its effect on the respiratory muscle strength in children with different types of SMA.

METHODS:

Lung and respiratory muscle functions were assessed in children receiving daily oral salbutamol for at least one year. The respiratory data of age-matched SMA II historical control subjects were compared with data of SMA II patients receiving salbutamol.

RESULTS:

Seven children (6.4 ± 2.0 years old, range four to ten; one SMA I, five SMA II, and one SMA III) treated with salbutamol (duration 23 ± 8 months) were assessed. Maximal static inspiratory pressure, sniff nasal inspiratory pressure, and slow vital capacity were significantly better in the salbutamol-treated SMA II group compared with control subjects (P < 0.05).

CONCLUSIONS:

Long-term oral salbutamol showed benefits in respiratory function in children with SMA and appeared to increase the strength of the inspiratory muscles in a small cohort of SMA II patients.

KEYWORDS:

lung function; motor function; respiratory muscles; salbutamol; spinal muscular atrophy

[Indexed for MEDLINE]

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