Value | Baseline (n = 6) | 12 months (n = 6) | 24 months (n = 5) | Change from Baseline | |
---|---|---|---|---|---|
12 months (n = 6) | 24 months (n = 5) | ||||
FEV1 | |||||
Least-square mean — liters | 1.59 ± 0.41 | 1.87 ± 0.47 | 1.65 ± 0.45 | 0.28 ± 0.08 (P<0.001) | 0.13 ± 0.05 (P = 0.004) |
Percent of predicted value | 58.51 ± 11.09 | 65.32 ± 12.32 | 59.89 ± 11.58 | 6.81 ± 2.28 (P<0.001) | 3.46 ± 1.57 (P = 0.008) |
FVC | |||||
Least-square mean — liters | 2.77 ± 0.56 | 3.20 ± 0.68 | 2.95 ± 0.66 | 0.43 ± 0.17 (P = 0.002) | 0.27 ± 0.14 (P = 0.013) |
Percent of predicted value | 79.90 ± 12.68 | 87.16 ± 12.63 | 84.36 ± 13.71 | 7.26 ± 2.16 (P<0.001) | 6.39 ± 1.36 (P<0.001) |
Total lung capacity | |||||
Least-square mean — liters | 5.16 ± 0.51 | 5.18 ± 0.44 | 5.14 ± 0.49 | 0.03 ± 0.12 (P = 0.62) | 0.04 ± 0.10 (P = 0.43) |
Percent of predicted value | 99.21 ± 7.01 | 99.70 ± 7.28 | 97.75 ± 7.24 | 0.49 ± 1.47 (P = 0.45) | −0.22 ± 0.78 (P = 0.57) |
Residual volume | |||||
Least-square mean — liters | 2.39 ± 0.10 | 1.98 ± 0.31 | 2.11 ± 0.29 | −0.41 ± 0.24 (P = 0.009) | −0.31 ± 0.23 (P = 0.036) |
Percent of predicted value | 124.56 ± 10.04 | 104.23 ± 8.85 | 112.30 ± 5.41 | −20.32 ± 1.62 (P<0.001) | −15.79 ± 2.38 (P<0.001) |
DLco | |||||
Least-square mean — ml/mmHg/min | 12.93 ± 2.42 | 13.00 ± 2.41 | 12.59 ± 2.39 | 0.07 ± 0.46 (P = 0.71) | −0.21 ± 0.55 (P = 0.43) |
Percent of predicted value | 53.40 ± 9.29 | 53.60 ± 8.70 | 54.27 ± 9.56 | 0.19 ± 1.39 (P = 0.74) | 1.68 ± 2.11 (P = 0.15) |