1.1 Study Population
We enrolled 23 consecutive children, aged 0-18 years, with type1 or 2 SMA, regularly followed at our Unit. The study is part of a larger natural history study on SMA approved by the Ethics Committee of our Institution, and a fully informed consent from the parents of each participant was obtained before the study.
For all the participating families, information on type of SMA, degree of neuroscoliosis/chest deformities, type of treatment, number of previous lung infections/ICU admissions, type of ventilation (non-invasive vs mechanical vs spontaneous breathing) were collected in a dedicated form.