Sex (male–female) | 3−7 | 8−10 |
Mean (SD) age, year | 65.9 (10.5) | 29.3 (9.9)* |
CFTR genotype, n (%) | ND | 18 (100) |
| | |
F508 del/F508 del | — | 8 (44.4) |
F508 del/other mutation | — | 6 (33.3) |
Other/other mutations | — | 2 (11.1) |
Other/ unidentified mutations | — | 2 (11.1) |
| | |
Mean (SD) FEV1, % predicted | 96.8 (19.1)% | 19.5 (6.6)%* |
Mean (SD) FVC, % predicted | 101.3 (23.0)% | 27.2 (9.4)%* |
| | |
Airway colonisation, n (%) | 0 | 18 (100) |
Pseudomonas aeruginosa
| 0 | 17 (94.4) |
Staphylococcus aureus
| 0 | 6 (33.3) |
Haemophilus influenzae
| 0 | 0 |
Other Gram negative bacteria | 0 | 6 (33.3) |
Aspergillus fumigatus
| 0 | 4 (22.2) |