Patient characteristics on entry to the trial
| Diagnosis | Age(years) | Diurnal Pao2(kPa) | Diurnal Paco2(kPa) | Vital capacity(l) |
|---|---|---|---|---|
| Values are shown as mean (SD) except age which is shown as median (range); mean (SD). | ||||
| DMD, Duchenne muscular dystrophy; CMD, congenital muscular dystrophy; BMD, Becker muscular dystrophy; CMT, Charcot Marie Tooth neuropathy; LGMD, limb girdle muscular dystrophy; KS, kyphoscoliosis; SMA II, spinal muscular atrophy type 2.; NIV, non-invasive ventilation Pao2, Paco2, arterial oxygen and carbon dioxide tensions. | ||||
| Group 1: Control | 18 (7–46); 21 (11) | 10.27 (1.4) | 6.03 (0.39) | 0.81 (0.61) |
| DMD (n = 3) | ||||
| CMD (n = 2) | ||||
| BMD (n = 1) | ||||
| CMT (n = 1) | ||||
| SMA II (n = 1) | ||||
| Beal’s syndrome (n = 1) | ||||
| Osteogenesis imperfecta (n = 1) | ||||
| Group 2: Randomised to NIV | 18 (11–51); 26 (14) | 10.70 (0.87) | 5.79 (0.66) | 1.01 (0.34) |
| DMD (n = 2) | ||||
| CMD (n = 3) | ||||
| Congenital KS (n = 3) | ||||
| Poland’s syndrome (n = 1) | ||||
| Group 3: Elective NIV | 22.5 (10–66); 28.3 (15.6) | 10.19 (2.23) | 7.47 (1.92) | 0.53 (0.19) |
| DMD (n = 8) | ||||
| CMD (n = 3) | ||||
| LGMD (n = 1) | ||||
| Congenital KS (n = 4) | ||||