Total number (n) | SMA type 1c (n) | SMA type 2a (n) | SMA type 2b (n) | SMA type 3a (n) | SMA type 3b (n) | |
---|---|---|---|---|---|---|
Participants | ||||||
Patients | 80 | 6 | 32 | 22 | 16 | 4 |
Female gender | 52 | 3 | 20 | 14 | 12 | 3 |
SMN2 copies | ||||||
2 | 4 | 1 | 1 | 1 | 1 | – |
3 | 66 | 5 | 29 | 18 | 12 | 2 |
4 | 10 | – | 2 | 3 | 3 | 2 |
Tests of respiratory muscle strength | ||||||
Peak expiratory flow (PEF) | ||||||
Patients | 79 | 6 | 31 | 22 | 16 | 4 |
Tests | 651 | 67 | 297 | 156 | 114 | 17 |
Follow-up (years) [IQR] | 6.7 [1.2–12] | 6.8 [3.5–8.2] | 7.3 [1.3–12.2] | 6.2 [1.2–11.8] | 2.1 [0.4–9.8] | 11.1 [7.7–15.3] |
Peak cough flow (PCF) | ||||||
Patients | 61 | 4 | 27 | 19 | 9 | 2 |
Tests | 288 | 27 | 144 | 76 | 35 | 6 |
Follow-up (years) [IQR] | 3.6 [0.3–8.1] | 6.6 [4.7–8.8] | 5.6 [0.8–7.9] | 3.4 [0.3–8.3] | 0.9 [0.0–2.2] | 2.0 [1.0–3.0] |
Maximum expiratory pressure (PE)max | ||||||
Patients | 75 | 6 | 28 | 22 | 15 | 4 |
Tests | 586 | 59 | 261 | 148 | 102 | 16 |
Follow-up (years) [IQR] | 5.8 [1.1–10.2] | 4.4 [1.8–7.1] | 6.1 [1.5–11.2] | 6.2 [1.0–11.5] | 3.0 [0.4–9.2] | 7.3 [4.9–9.6] |
Maximum inspiratory pressure (PI)max | ||||||
Patients | 76 | 6 | 28 | 22 | 16 | 4 |
Tests | 590 | 60 | 263 | 148 | 103 | 16 |
Follow-up (years) [IQR] | 6.3 [1.1–10.6] | 6.7 [3.5–7.6] | 6.6 [1.5–13.0] | 6.2 [1.0–11.5] | 2.5 [0–9.2] | 7.3 [4.9–9.6] |
PEmax/PImax ratio | ||||||
Patients | 75 | 6 | 28 | 22 | 15 | 4 |
Tests | 582 | 57 | 259 | 147 | 103 | 16 |
Follow-up (years) [(IQR] | 5.8 [1.1–10.2] | 4.4 [1.8–7.1] | 6.1 [1.5–11.2] | 6.2 [1.0–11.5] | 3.0 [0.4–9.2] | 7.3 [4.9–9.6] |
Sniff inspiratory pressure (SNIP) | ||||||
Patients | 57 | 3 | 22 | 19 | 11 | 2 |