Table 1.
Demographics for all subjects and stratified by modulator (lumacaftor/ivacaftor) use
| All subjects (n = 20) | CFTR modulator (n = 9) | No CFTR modulator (n = 11) |
P value |
|
| Age at MRI (yr) | 22.3 (11.3-39.0) | 26.4 (16.3-39.0) | 21.9 (11.3-36.1) | 0.29 |
| Genotype | ||||
| F508del/F508del | 10 | 9 | 1 | < 0.01 |
| F508del/other | 9 | 0 | 9 | < 0.01 |
| Other/other | 1 | 0 | 1 | < 0.01 |
| Male sex | 16 (80%) | 7 (78%) | 9 (82%) | 1.00 |
| BMI percentile | 39 (2-96) | 51 (3-77) | 23 (2-96) | 0.21 |
| % predicted FEV1 | 77 (33-105) | 73 (33-89) | 77 (48-105) | 0.24 |
| CFRD | 12 (60%) | 7 (78%) | 5 (45%) | 0.20 |
| Insulin therapy | 10 (50%) | 6 (67%) | 4 (36%) | 0.37 |
Data presented as median (range) or frequency (percent) as appropriate. BMI (percentile): body mass index percentile adjusted for age; FEV1: forced expiratory volume at 1 s; CFRD: cystic fibrosis related diabetes.