Table 1.
Characteristics of patients at inclusion (n = 299).
| Characteristics | Missing Data |
n or Median or Mean |
% or [IQR] or (SD) |
|---|---|---|---|
| Age (years) | 0 | 23.7 | (10.9) |
| Gender (female) | 0 | 143 | 47.8 |
| CFTR mutations | 0 | ||
| F508del/F508del | 119 | 39.8 | |
| F508del/other | 136 | 45.5 | |
| Other/other or unknown | 44 | 14.7 | |
| Comorbidities | 0 | ||
| Exocrine pancreatic insufficiency | 235 | 78.6 | |
| Diabetes mellitus | 83 | 27.8 | |
| Gastroesophageal reflux | 73 | 24.4 | |
| ABPA | 69 | 23.1 | |
| Chronic sinusitis | 58 | 19.4 | |
| Haemoptysis | 55 | 18.4 | |
| Concomitant asthma | 34 | 11.4 | |
| Pneumothorax | 9 | 3.0 | |
| Pulmonary hypertension | 5 | 1.7 | |
| BMI (kg/m2) | 1 | 19.9 | [17.6–21.3] |
| Shwachman–Kulczycki score | 28 | 74.6 | [65–90] |
| Number of days of hospitalization during the last 12 months * | 10 | 7 | [1–115] |
| Sputum bacterial cultures positive with | |||
| Staphylococcus aureus (methicillin susceptible) | 80 | 27.4 | |
| Pseudomonas aeruginosa (non-mucoid strains) | 75 | 26.4 | |
| Pseudomonas aeruginosa (mucoid strains) | 77 | 22.6 | |
| Haemophilus influenzae | 28 | 9.6 | |
| Staphylococcus aureus (methicillin resistant) | 22 | 7.5 | |
| Non-pneumoniae Streptococcus species | 19 | 6.5 | |
| Stenotrophomonas maltophilia | 16 | 5.5 | |
| Alkaligenes xylosoxidans | 12 | 4.1 | |
| Streptococcus pneumoniae | 5 | 1.7 | |
| Bulkhoderia cepatia complex | 3 | 1.0 | |
| Mycobacterium avium complex | 4 | 1.4 | |
| Mycobacterium abscessus complex | 5 | 1.7 | |
| Sputum fungal cultures positive with | 10 | ||
| Candida albicans | 136 | 45.5 | |
| Aspergillus fumigatus | 80 | 26.8 | |
| Other moulds (Cladosporium sp., Penicillium sp.) | 20 | 6.7 | |
| Scedosporium species | 10 | 4.0 | |
| Aspergillus flavus | 9 | 3.0 | |
| Candida parapsilosis | 5 | 1.7 | |
| Other Aspergillus species | 4 | 1.3 | |
| Candida glabrata | 3 | 1.0 | |
| Other Candida species | 3 | 1.0 | |
| Exophiala dermatitidis | 2 | 0.7 | |
| Lomentospora prolificans | 1 | 0.3 | |
| Medications | 0 | ||
| Non-invasive ventilation | 21 | 7.2 | |
| Long-term oxygen | 32 | 10.7 | |
| Nebulized rhDNase | 178 | 59.5 | |
| Inhaled antibiotics | 86 | 28.8 | |
| Inhaled steroids | 168 | 56.2 | |
| Azithromycin | 162 | 54.2 | |
| Oral antibiotic courses during the last 12 months | |||
| 0 | 75 | 25.1 | |
| 1 to 3 | 161 | 53.8 | |
| >3 | 63 | 21.1 | |
| Intravenous antibiotics during the last 12 months | 153 | 51.2 | |
| Mean number of antibiotic courses | 1 | [0–2] | |
| Systemic corticosteroids during the last 12 months | 70 | 23.4 | |
| Other immunosuppressive treatment | 3 | 1.0 | |
| Antifungal treatment during the last 6 months | 61 | 20.4 |
ABPA, allergic bronchopulmonary aspergillosis; BMI, body mass index. Antifungal treatment was: itraconazole in 48, voriconazole in 14, fluconazole in 4, and posaconazole in 5 of the cases. * Excluding patients without hospitalization.