Baseline participant characteristics
| Male | 33 (56%) |
| Age years | 28 (17–59) |
| Genotype | |
| F508del homozygous | 23 (39%) |
| F508del heterozygous | 19 (32%) |
| Other | 6 (10%) |
| Unknown | 11 (19%) |
| Comorbidities | |
| Pancreatic insufficiency | 54 (92%) |
| CF-related diabetes mellitus | 32 (54%) |
| CF-related liver disease | 13 (22%) |
| ABPA | 8 (14%) |
| Asthma | 2 (3%) |
| GORD | 24 (41%) |
| Maintenance therapies | |
| Nebulised colistin | 37 (63%) |
| Nebulised tobramycin | 6 (10%) |
| Nebulised dornase alpha | 35 (59%) |
| Nebulised hypertonic saline | 20 (34%) |
| Oral corticosteroids | 10 (17%) |
| Oral azithromycin | 48 (81%) |
| Duration of Pseudomonas aeruginosa infection years | 7.7 (0.3–14.4) |
| Baseline sputum microbiology | |
| P. aeruginosa | 52 (88%) |
| MSSA | 8 (14%) |
| MRSA | 3 (5%) |
| Candida albicans | 4 (7%) |
| Aspergillus fumigatus | 2 (3%) |
| Haemophilus influenzae | 1 (2%) |
| No respiratory pathogens | 3 (5%) |
| Intravenous antibiotic administered | |
| Tobramycin | 31 (53%) |
| Ceftazidime | 28 (47%) |
| Colomycin | 24 (41%) |
| Meropenem | 24 (41%) |
| Aztreonam | 7 (12%) |
| Piptazobactam | 5 (8%) |
| Other | 5 (8%) |
| Spirometry | |
| FEV1# L | 1.70±0.71 |
| FEV1# % predicted | 47.0±17.0 |
| Anthropometric measures | |
| Height m | 1.68±0.09 |
| BMI kg·m−2 | 21.9±3.7 |
| Change in weight kg | +1.00 (−0.10– +2.30) |
Data are presented as n (%), median (range) or mean±sd. CF: cystic fibrosis; ABPA: allergic bronchopulmonary aspergillosis; GORD: gastro-oesophageal reflux disease; MSSA: methicillin-sensitive Staphylococcus aureus; MRSA: methicillin-resistant S. aureus; BMI: body mass index. #: n=57.