Effect of salbutamol on respiratory muscle strength in spinal muscular atrophy

Publication date: Available online 20 April 2017
Source:Pediatric Neurology
Author(s): Sonia Khirani, Ivana Dabaj, Alessandro Amaddeo, Jorge Olmo Arroyo, Jacques Ropers, Stéphane Tirolien, Véronique Coudert, Brigitte Estournet, Brigitte Fauroux, Susana Quijano-Roy
PurposeOral 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.Basic proceduresLung 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 controls were compared with data of SMA II patients receiving salbutamol.Main findingsSeven children (6.4±2.0 yrs old, range 4-10; 1 SMA I, 5 SMA II, and 1 SMA III) treated by 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 to controls (p<.05).ConclusionLong term oral salbutamol showed benefits in respiratory function in SMA children and appeared to increase the strength of the inspiratory muscles in a small cohort of SMA II patients.



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