Table 2.
Clinical characteristics of participants with restrictive lung involvement (FSHD1 and FSHD2 inclusive)
| Characteristic | Restrictive Pattern | Non-restrictive Pattern | P-value |
|---|---|---|---|
| Number (%) | 6 (9.8%) | 55 (90.2%) | * |
| Age years (SD) | 48.9(13.3) | 54.8 (14.6) | 0.31 |
| Gender female (%) | 3 (50.0%) | 16 (29.0%) | 0.36 |
| Age at symptom onset (SD) | 23.6 (14.5) | 36.3 (11.09) | 0.09 |
| FSHD1 (%) | 4 (66.7%) | 49 (89.0%) | 0.17 |
| CSS (SD) | 7.5 (1.4) | 4.9(2.1) | 0.005 |
| FVC supine-sitting (SD) | −2.3 (7.4) | −4.3 (13.3) | 0.56 |
| Self-reported breathing problem (%) | 4 (66.7%) | 8 (14.5%) | 0.01 |
| History of asthma (%) | 2 (33.3%) | 4 (7.3%) | 0.10 |
| History of Smoking (%) | 4 (66.7%) | 21 (38.2%) | 0.38 |
| Use of Wheelchair (%) | 3 (50.0%) | 3 (5.4%) | 0.01 |
| Composite MMT score (SD) | 4.0 (0.8) | 4.5 (0.6) | 0.12 |
| Hip Flexion (SD) | 3.7 (0.8) | 4.6 (0.5) | 0.0007 |
| Knee Extension (SD) | 4.2 (0.8) | 4.8 (0.5) | 0.02 |
| Knee Flexion (SD) | 3.5 (1.6) | 4.4 (0.7) | 0.01 |
| Ankle Dorsiflexion (SD) | 3.0 (1.5) | 3.7 (1.5) | 0.34 |
| Shoulder ROM degrees (SD) | 55.0 (22.1) | 103.5 (48.8) | 0.02 |
*
not applicable