Table 1 Patient clinical and demographic characteristics

Age, years

30.7 ± 8.0 (20–49)

Female, n (%)

18 (35)

Genotype, n (%)

F508del homozygous

38 (73)

F508del heterozygous

14 (27)

CFTR modulator therapy, n (%)

None

16 (31)

Tezacaftor/ivacaftor

33 (63)

Lumacaftor/ivacaftor

3 (6)

Body mass index, kg/m²

21.5 ± 3.3 (15.6–31.2)

FEV₁, L

2.1 ± 0.8 (1.0-4.8)

FEV₁, % predicted

52.1 ± 14.8 (30.0–96.0)

FVC, L

3.4 ± 1.1 (1.5–6.2)

FVC, % predicted

69.5 ± 16.2 (37.0-105.0)

Residual volume, L

3.0 ± 0.9 (1.1–5.6)

Residual volume, % predicted

179.9 ± 41.9 (99.0-283.0)

Pancreatic insufficiency, n (%)

51 (98)

Pseudomonas aeruginosa positive, n (%)

28 (54)

Cystic fibrosis-related diabetes, n (%)

12 (23)

Oxygen supplementation, n (%)

2 (4)