Table 2.
Participant characteristics
| n | % | Mean ± SD | Minimum | Median | Maximum | ||
|---|---|---|---|---|---|---|---|
| Age | 121 | 100 | 44.8 ± 13.0 | 21 | 43 | 85 | |
| Sex | Male | 55 | 44 | ||||
| Female | 66 | 55 | |||||
| Age at onset | 121 | 100 | 27.7 ± 9.6 | 15 | 26 | 61 | |
| Body mass index | 121 | 100 | 21.4 ± 4.4 | 12.1 | 21 | 37.8 | |
| Normal | 65 | 54 | |||||
| Underweight | 36 | 30 | |||||
| Obesity | 20 | 16 | |||||
| Walking capability | Ambulant without assistance | 24 | 20 | ||||
| Ambulant with assistance | 45 | 37 | |||||
| Non-ambulant | 52 | 43 | |||||
| Wheelchair use | Never | 43 | 36 | ||||
| Part-time | 28 | 23 | |||||
| Full time | 50 | 41 | |||||
| Respiratory function (%FVC) | Performed | 79 | 65 | 88.4 ± 23.7 | 16.4 | 92.8 | 130 |
| Not performed | 42 | 35 | |||||
| Normal (FVC ≥ 80%) | 26 | 33* | |||||
| Decreased | 53 | 67* | |||||
| Nocturnal NPPV | 2 | 2 | |||||
| Cardiac function | Performed | 35 | 29 | ||||
| (EF, ES) | Not performed | 86 | 71 | ||||
| Normal | 35 | 100* | |||||
| Decreased | 0 | 0* | |||||
| Grip power (kg) | Performed | 96 | 79 | 5.5 ± 7.2 | 0 | 2.9 | 30 |
| Not performed | 25 | 21 | |||||
| Grip power = 0 | 36 | 38* | |||||
| CK (Iu/L) | Performed | 120 | 99 | 357.8 ± 209.0 | 11 | 209 | 459 |
| Not performed | 1 | 1 |
*ratio of examined/performed.