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VPPI does indeed appear to have a positive effect on chest growth in children with type I SMA
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A French retrospective study assessed the effect of intermittent positive pressure ventilation (IPPV) using the Alpha 300® in seven children with type I spinal muscular atrophy, with a median age of 4.8 years, who were being treated with Zolgensma® (four of them) or Nusinersen®.
- After four months of daily use for around 20 minutes, the ratio of chest circumference to head circumference increased significantly.
- Only one child did not respond to IPPV.
- The duration of the study was too short to measure a significant effect on the number of hospitalisations due to exacerbations.
Randomised controlled trials involving larger cohorts will be required to confirm the results of this pilot study.
