Characteristic | Patients without MH | Patients with MH | Hazard ratio (95% CI) | p-value |
Subjects n | 47 | 15 | ||
Age years median (IQR) | 22.0 (20.5–30.0) | 19.0 (16.0–22.0) | 1.01 (0.96–1.04) | 0.93 |
Male | 28 (59.6%) | 6 (40.0%) | 0.97 (0.33–2.85) | 0.96 |
Homozygous ΔF508/ΔF508 | 18 (38.3%) | 6 (40.0%) | 1.04 (0.36–3.01) | 0.94 |
Heterozygous ΔF508 | 18 (38.3%) | 5 (33.3%) | 1.62 (0.53–4.97) | 0.40 |
Body mass index kg·m−2 | 20.1±2.2 | 18.5 ±2.75 | 0.79 (0.61– 1.02) | 0.075 |
Severe malnutrition | 8 (17.0%) | 7 (46.7%) | 2.69 (0.94–7.68) | 0.064 |
FEV1 % predicted | 57.3±24.6 | 71.9 ±19.8 | 1.01 (0.98–1.03) | 0.55 |
FVC % predicted | 74.8±20.6 | 79.7 ±19.6 | 0.99 (0.96–1.03) | 0.69 |
EPI | 41 (87.2%) | 12 (80.0%) | 1.12 (0.25–5.04) | 0.88 |
ABPA | 12 (25.5%) | 9 (60.0%) | 6.03 (1.97–18.45) | 0.002 |
Liver cirrhosis | 1 (2.1%) | 4 (25.0%) | ND | |
Osteoporosis | 4 (8.5%) | 1 (6.7%) | ND | |
Diabetes | 1(2.1%) | 5 (33.3%) | 5.66 (1.84–17.42) | 0.002 |
Thrombocytopenia | 1 (2.1%) | 3 (20.0%) | ND | |
Bacteriology | ||||
Pseudomonas aeruginosa | 34 (72.3%) | 8 (53.3%) | 0.46 (0.15–1.38) | 0.17 |
MSSA | 22 (46.8%) | 8 (53.3%) | 1.53 (0.53–4.42) | 0.43 |
MRSA | 9 (19.1%) | 1 (6.7%) | 1.17 (0.14–9.41) | 0.88 |
Aspergillus | 10 (21.3%) | 2 (13.3%) | 0.88 (0.20–3.96) | 0.87 |
Stenotrophomonas | 3 (6.4%) | 1 (6.7%) | ND | |
Achromobacter | 2 (4.3%) | 1 (6.7%) | ND | |
Long-term treatment | ||||
RhDNase | 37 (78.7%) | 13 (86.7%) | 2.19 (0.28–16.85) | 0.45 |
Inhaled tobramycin | 9 (19.1%) | 3 (20.0%) | 1.26 (0.34–4.71) | 0.73 |
Inhaled colimycin | 12 (25.5%) | 4 (25.0%) | 1.05 (0.33–3.36) | 0.93 |
Triazole | 9 (19.1%) | 6 (40.0%) | 4.13 (1.41–12.07) | 0.009 |
>4 i.v. antibiotic courses | 11 (23.9%) | 3 (20.0%) | 1.08 (0.30–3.90) | 0.90 |
Exacerbations per year n (range) | 2 (0–3) | 1 (0–3) | 1.07 (0.85–1.35) | 0.57 |
Data are presented as mean±sd or n (%), unless otherwise stated. Hazard ratios were calculated using Cox regression models. Multivariate analysis was not performed due to the limited sample size and MH event number. Bold font indicates statistically significant p-values. Genotype was known for 61 out of the 62 patients. IQR: interquartile range; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; EPI: exocrine pancreatic insufficiency; ABPA: allergic bronchopulmonary aspergillosis; MSSA: methicillin-sensitive Staphylococcus aureus; MRSA: methicillin-resistant S. aureus; RhDNase: recombinant human deoxyribonuclease (dornase alfa); ND: not done.