Table 1.
Baseline characteristics of the patients.
| Patient characteristics |
CFTR variants |
||
|---|---|---|---|
| F508del/ F508del |
Gating$/ other |
G551D/ other |
|
| Pancreatic Insufficient (n = 4,045) | n = 2,143 | n = 119 | n = 72 |
| Sex (male/female) | 1,125/1018 | 64/55 | 42/30 |
| European origin, n (%) | 2,061 (96) | 105 (88) | 67 (93) |
| Treated by ivacaftor (n = 81) | |||
| FEV1pp (baseline) $ | n = 60 | n = 40 | |
| >90% | 17 | 16 | |
| 40–90% | 30 | 16 | |
| <40% | 13 | 8 | |
| FEV1pp change analysis (n = 30) | n = 30 | n = 16 | |
| Age, mean (SD) | 20.0 (14.0) | 17.6 (12.4) | |
| Sex (male/female) | 18/12 | 10/6 | |
$Ivacaftor-approved CFTR gating mutations: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, and S549R; ∗∗FEV1pp (forced expiratory volume in 1 s percent-predicted) value in the 3 months before treatment. Data are means (SD) or numbers (%) unless otherwise indicated. CFTR: Cystic Fibrosis Transmembrane Conductance Regulator.