Median values and range of the lung function tests performed in the studied population at baseline and follow-up visits
| Baseline visit | Follow-up 1 visit | Follow-up 2 visit | Follow-up 3 visit | Follow-up 4 visit | Follow-up 5 visit | |||||||
| Time of treatment (months) | – | n | 4 (1–8) | n | 7 (4–16) | n | 14 (8–20) | n | 21 (11–27) | n | 27 (15–32) | n |
| FEV1/FVC | 85 (76–97) | 6 | 85 (78–93) | 6 | 83 (77–92) | 6 | 83 (77–92) | 5 | 83 (75–92) | 5 | 83 (75–88) | 5 |
| FVC (ml) | 1780 (880–1920) | 6 | 1940 (1080–2090) * | 6 | 1885 (1020–2050)* | 6 | 1880 (990–2010) | 5 | 1620 (930–2050) | 5 | 1780 (1040–1990) | 5 |
| FVC (%) | 50 (26–71) | 6 | 55 (33–80)* | 6 | 56 (31–79)* | 6 | 52 (30–75) | 5 | 47 (29–84) | 5 | 47 (32–83) | 5 |
| MIP (%) | 38 (12–47) | 6 | 38 (30–45) | 6 | 37 (24–49) | 6 | 34 (26–53) | 5 | 38 (24–47) | 3 | 40 (21–50) | 3 |
| MEP (%) | 51 (19–113) | 6 | 62 (24–115) | 6 | 48 (27–85) | 6 | 43 (26–134) | 5 | 36 (27–137) | 3 | 124 (30–125) | 3 |
| SNIP (- cmH2O) | 14 (8–19) | 2 | NA | 0 | NA | 0 | 13 | 1 | 22 (15–28) | 2 | 39 | 1 |
| CPF (l/min) | 192 (133–250) | 2 | 135 | 1 | 185 (120–249) | 2 | 225 (100–350) | 2 | 113 | 1 | 235 (150–320) | 2 |
| SpO2 nocturnal mean (%) | 94 (92–95) | 2 | 92 | 1 | 95 | 1 | 92 | 1 | 96 | 1 | 93 | 1 |
| CT90 (%) | 14 (6–21) | 2 | 3 | 1 | 2 | 1 | 4 | 1 | 4 | 1 | 0 | 1 |
*P < 0.05 between values of follow-up and baseline visits (Wilcoxon signed-rank test).
CPF, cough peak flow; CT90, proportion of sleep time with SpO2 under 90%; FEV1, Forced Expiratory Volume in 1 second; FVC, forced vital capacity; MEP, maximum expiratory pressure; MIP, maximum inspiratory pressure; NA, not available; SNIP, sniff nasal inspiratory pressure; SpO2, oxygen pulse oximeter saturation.