Demographic and clinical characteristics of groups according to genotype*
| Genotype I–II/I–II(n = 37) | Genotype I–II/III, IV or V(n = 37) | p value | |
|---|---|---|---|
| BMI, body mass index. | |||
| *Data obtained at the first visit to adult unit. | |||
| Sex (no (%) male) | 18 (48.6%) | 22 (59.5%) | 0.484 |
| Mean (SD) age (years) | 22.5 (4.9) | 30.9 (8.8) | <0.001 |
| Mean (SD) age at diagnosis (years) | 4.2 (5.3) | 21.9 (13.4) | <0.001 |
| Adult age at diagnosis, n (%) | 1 (2.7%) | 28 (75.7%) | <0.001 |
| Mean (SD) follow up (years) | 3.7 (3.7) | 5.3 (1.9) | 0.024 |
| Mean (SD) BMI (kg/m2) | 18.4 (2.9) | 23 (3) | <0.001 |
| Mean (SD) sweat chloride concentration (mEq/l) | 108 (23) | 89 (22) | 0.001 |
| Digestive symptoms at diagnosis, n (%) | 28 (73.7%) | 9 (24.3%) | 0.010 |
| Pancreatic insufficiency, n (%) | 36 (97.3%) | 9 (24.3%) | <0.001 |
| Pulmonary symptoms at diagnosis, n (%) | 25 (68.4%) | 28 (75.7%) | 0.439 |
| P aeruginosa colonisation, n (%) | 32 (86.5%) | 16 (43.2%) | <0.001 |
| S aureus colonisation, n (%) | 22 (59.5%) | 12 (32.4%) | 0.019 |
| End-stage lung disease, n (%) | 15 (40.5%) | 1 (2.7%) | 0.001 |
| Lung transplantation, n (%) | 9 (24.3%) | 1 (2.7%) | 0.010 |
| Dead patients, n (%) | 11 (29.7%) | 1 (2.4%) | 0.012 |