Table 1. General characteristics of the sample (n= 48).
| Characteristics | Data |
|---|---|
| Gender | |
| Male, n (%) | 28 (58.3) |
| Female, n (%) | 20 (41.7) |
| Age at diagnosis (years), median (IQR) | 1.0 (2.0) |
| F508del homozygosity, n (%) | 10 (20.8) |
| Family history, n (%) | 12 (25) |
| CF related complications before 18 years | |
| Pancreatic insufficiency, n (%) | 39 (81.3) |
| CFRDM, n (%) | 3 (6.3) |
| Massive hemoptysis, n (%) | 5 (10.4) |
| MSSA, n (%) | 29 (60.4) |
| MRSA, n (%) | 9 (18.8) |
| Pseudomonas aeruginosa, n (%) | 41 (85.4) |
| Burkholderia cepacia, n (%) | 13 (27.1) |
| Hospital admission, n (%) | 40 (83.3) |
| ICU admission, n (%) | 4 (8.3) |
| Invasive mechanical ventilation, n (%) | 5 (10.4) |
| Clinical data at age 18 years | |
| Body mass index (kg/m2), mean (SD) | 20.4 (2.3) |
| FEV1 (%), mean (SD) | 64 (22.2) |
| FVC (%), mean (SD) | 73.8 (18.5) |
| FEV1/FVC (%), mean (SD) | 87 (12.4) |
| Death, n (%) | 16 (33.3) |
| Death <30 years, n (%) | 8 (16.7) |
| Age at death, mean (SD) | 31.3 (5.6) |
Data are reported as n (%), median (interquartile range), or mean (SD). CF: cystic fibrosis; CFRDM: CF related diabetes mellitus; MSSA: methicillin-sensitive Staphylococcus aureus; MRSA: methicillin-resistant Staphylococcus aureus; ICU: intensive care unit; FEV1: first second of forced expiratory volume; FVC: forced vital capacity.