“…Literature review (Online Table 5.4) Due to ethical constraints, the benefits on NIV have not been confirmed in randomized controlled trials and the published cohort studies mainly consist of a limited number of patients. NIV is associated with: o an increase in survival in patients with SMA type 1 [57,62,65,66,209] and Duchenne muscular dystrophy [71,210] o fewer hospitalizations in patients with SMA type 1 [57,61,62,64,209], and some NMD [211,212]); but no change in hospitalizations in other children with NMD [213] o improvement in SDB symptoms in patients with SMA type 2-3 [60], infantile Pompe disease [73], and other NMD [69,82,84] o improvement in nocturnal and daytime gas exchange in patients with juvenile Pompe disease [72], and NMD [82] o improvement in sleep quality/architecture and cyclic-alternating patterns in patients with SMA type 1-2 [60], SMA type 2 [214], and other NMD [69,82] o decrease in chest deformity in patients with SMA type 1 [58,63], and SMA type 1-3 [61] o transient improvement in predicted FVC and thoraco-abdominal asynchrony in patients with Duchenne muscular dystrophy [215] o improvement in cardiac function in patients with Duchenne muscular dystrophy [216] o improvement in QoL in children with NMD [217].…”