Variables | Study sample n = 52 | Patients with Vef and without DH n = 10 (19%) | Patients with Vin and without DH n = 13 (25%) | Patients with Vef and with DH n = 14 (27%) | Patients with Vin and with DH n = 15 (29%) | p value a |
---|---|---|---|---|---|---|
Age, years | 29.1 ± 8.1 | 30.9 ± 10.1 | 29.8 ± 8 | 26.3 ± 6 | 30 ± 8.7 | 0.494 |
Male, % | 58 | 80 | 46 | 50 | 60 | 0.373 |
BMI, kg/m2 | 21.4 [19.5; 22.5] | 22.3 [21.7; 24.3] | 20.8 [19; 22.8] | 20.9 [19.8; 21.9] | 20.9 [18.8; 23.3] | 0.230 |
FFMI, kg/m2 | 17.1 [15.4; 18] | 17.6 [15.7; 18.3] | 16.4 [14.5; 17.4] | 17 [15.6; 18.2] | 17.4 [15.3; 18] | 0.402 |
Diabetes, % | 31 | 50 | 23 | 29 | 27 | 0.523 |
CFTR mutations, % | 0.972 | |||||
F508del/F508del | 42 | 40 | 39 | 36 | 54 | |
F508del/Other | 41 | 40 | 46 | 43 | 33 | |
Other/Other | 17 | 20 | 15 | 21 | 13 | |
Pseudomonas aeruginosa colonization, % | 94 | 80 | 92 | 100 | 100 | 0.133 |
LTOT, % | 8 | 0 | 0 | 14 | 13 | 0.328 |
Domiciliary NIMV, % | 4 | 0 | 0 | 7 | 7 | 0.647 |
FEV1, % pred. | 69.2 ± 22.3 | 85.2 ± 22.3 | 82.1 ± 20 | 61.7 ± 17.4 b c | 54.3 ± 15.6 b c | < 0.001 |
FVC, % pred. | 89.6 ± 18.3 | 98.6 ± 17.1 | 100.4 ± 20 | 86 ± 12.8 | 77.5 ± 14.1 b c | 0.001 |
FEV1/FVC, % | 64.8 ± 12.2 | 72.8 ± 9.1 | 70.1 ± 11.8 | 59.9 ± 11.1 b | 59.6 ± 11.3 b | 0.005 |
IC at rest, % pred. | 77.5 [61.5; 91.7] | 76.5 [69.2; 104.5] | 76 [57.5; 103] | 78 [63.7; 89.5] | 81 [57; 87] | 0.977 |
VT at rest, L | 0.64 [0.48; 0.84] | 0.61 [0.37; 0.71] | 0.86 [0.67; 0.95] b | 0.50 [0.44; 0.81] c | 0.61 [0.54; 0.71] c | 0.015 |
RR at rest, breath/min | 17.5 [13; 21.5] | 18 [16.6; 20.7] | 13 [11.8; 18] | 17 [11.7; 24] | 20 [15.9; 26] | 0.510 |