Characteristic | N = 921 |
---|---|
Sex | |
Males | 60 (65.2%) |
Females | 32 (34.8%) |
Age (years) | |
Median (IQR) | 38 (33; 43) |
Age category | |
< 40 years | 53 (57.6%) |
≥ 40 years | 39 (42.4%) |
Residual function mutation | 12 (13.0%) |
Pancreatic insufficiency | 64 (69.6%) |
Chronic P. aeruginosa infection | 59 (64.1%) |
ppFEV1 | |
Median (IQR) | 50.5 (39.0; 70.8) |
Unknown | 2 |
ppFEV1 < 40 | 25 (27.8%) |
Unknown | 2 |
CFRD | 23 (25.0%) |
Previous CFTR modulator therapy | |
None | 69 (75.0%) |
Lumacaftor/Ivacaftor | 21 (22.8%) |
Tezacaftor/Ivacaftor | 1 (1.1%) |
Ivacaftor | 1 (1.1%) |
Height (cm) | |
Median (IQR) | 170 (163; 175) |
Weight (kg) | |
Median (IQR) | 60.0 (55.7; 70.0) |
BMI (kg/m2) | |
Median (IQR) | 21.8 (19.1; 23.5) |
BMI category | |
Underweight | 10 (10.9%) |
Normal weight | 72 (78.3%) |
Overweight | 10 (10.9%) |