Table 1 Clinical characteristics of the patients (n = 27)
Clinical variable | Value |
|---|---|
Age, y mean (SD) | 25.9 (6.7) |
Sex, man/woman (n) | 10/17 |
Lung function tests: mean (SD) | |
FVC, L | 3.0 (0.84) |
FVC, % predicted | 73.8 (18.8) |
FEV1, L | 1.9 (0.6) |
FEV1, % predicted | 53.2 (16.3) |
FEV1/FVC | 61.1 (11.3) |
Genetic mutation, n (%) | |
F508del/F508del | 8 (29.6) |
F508del/other | 15 (55.6) |
Other | 4 (14.8) |
Diabetes, n (%) | 5 (18.5) |
ABPA, n (%) | 11 (40.7) |
Pancreatic insufficiency, n (%) | 24 (88.9) |
Bacterial colonization, n (%) | |
Staphylococcus aureus | 18 (66.7) |
MRSA | 4 (14.8) |
Pseudomonas aeruginosa | 23 (85.2) |
Burkholderia cepacia | 3 (7.4) |
Achromobacter xylosoxidans | 6 (21.7) |
Haemophilus influenzae | 1 (3.7) |
Stenotrophomonas maltophilia | 1 (3.7) |
Treatment, n (%) | |
Macrolides | 22 (81.5) |
Inhaled corticosteroids | 21 (77.8) |
Oral corticosteroids | 5 (18.5) |
Nebulized antibiotics | 23 (85.2) |
DNase | 14 (51.9) |
Hypertonic saline solution | 13 (48.1) |
No. of exacerbations during the previous year treated with oral antibiotics, mean (SD) | 3.2 (1.9) |
No. of exacerbations during the previous year treated with intravenous antibiotics, mean (SD) | 1.77 (1.73) |