Baseline characteristics of study cohort
| Patients with CF | Control subjects | ||
|---|---|---|---|
| Included | Not included | ||
| N | 63 | 23 | 57 |
| Age in years, median (range) | 12.4 (5.3–18.8) | 14.0 (4.5–19.6) | 12.4 (4.4–17.9) |
| <12 years | 31 (49%) | 10 (43%) | 27 (47%) |
| Male | 33 (52%) | 10 (43%) | 28 (48%) |
| F508del homozygote | 40 (63%) | 12 (52%) | |
| Pancreatic sufficient | 6 (10%) | 4 (17%) | |
| Colonisation status with Pseudomonas aeruginosa† | |||
| Chronic | 8 (13%) | 2 (9%) | |
| Intermittent | 12 (19%) | 3 (13%) | |
| Weight z-score | −0.51 (0.94) | −0.40 (1.17) | 0.04 (0.74)* |
| Height z-score | −0.41 (0.98) | −0.73 (1.21) | 0.37 (0.98)* |
| BMI z-score | −0.34 (0.90) | 0.04 (0.95) | −0.19 (0.69) |
| FEV1% predicted | 89.4 (15.7) | 86.3 (25.7) | 102.7 (10.2)* |
| FEV1 z-score | −0.88 (1.29) | −1.09 (2.10) | 0.23 (0.85)* |
| FEF25–75% predicted | 79.9 (30.5) | 74.3 (42.1) | 94.3 (21.2)* |
| FEF25–75 z-score | −1.04 (1.53) | −1.37 (2.08) | −0.3 (1.0)* |
| LCI | 10.78 (3.09) | 7.30 (0.50)* | |
| LCI z-score | 6.97 (6.20) | 0.00 (1.00)* | |
| CFQ-Rresp, median (range) | 89 (34–100) | ||
| CFCS, median (range) | 15 (10–30) | ||
Data presented as n (%) or mean (SD) unless otherwise specified.
*t Test, p≤0.01 control subjects vs patients with CF.
†During the previous 12 months according to the definition of Lee et al.23
BMI, body mass index; CF, cystic fibrosis; CFCS, Cystic Fibrosis Clinical Score; CFQ-Rresp, respiratory domain of the Cystic Fibrosis Questionnaire-Revised; FEF25–75, forced expiratory flow between 25% and 75% of forced vital capacity; FEV1, forced expiratory volume in 1 s; LCI, lung clearance index.