| Age (years), median (II) |
9.1 (4.5 - 19.4) |
| Children and adolescents, N (%) |
70 (76.1) |
| Gender, N (%) |
| Male |
51 (55.4) |
| Ethnicity, N (%) |
| Caucasian |
87 (94.6) |
| Age of diagnosis (years), median (II) |
0.35 (0.1 - 3.5) |
| Nutritional status (adults) |
| BMI (kg/m²), mean ± SD |
21.4 ± 4.0 |
| Nutritional Status (children and adolescents) |
| Percentile Weight / Age, median (II) |
35.8 (24.3 - 51.3) |
| Percentile BMI/ Age, median (II) |
65.6 (39.6 - 84.3) |
| Causing variants classification, N (%) |
| III-VI |
22 (23.9) |
| I-II |
70 (76.1) |
| Causing variants, N (%) |
| F508del homozygous |
33 (35.9) |
| F508del heterozygous |
31 (33.7) |
| Others |
28 (30.4) |
| Pancreatic Insufficiency, N (%) |
82 (90.1) |
| Bronchiectasis, N (%) |
35 (56.5) |
| Pulmonary Function, mean ± SD |
| Predicted FEV1 %, pre-bronchodilator |
80.2 ± 30.5 |
