Comparison of general characteristics and lung function measurements of cystic fibrosis(CF) patients
| ED- | ED+ | p-value | |
| Subjects n | 9 | 14 | |
| Age yrs | 27±3 | 27±4 | ns |
| Male:female | 6:3 | 8:6 | ns |
| CFTR mutation n | |||
| Delta F508/F508 | 5 | 9 | |
| Delta F508/1066C | 1 | 2 | |
| Delta F508/E60X | 1 | 1 | |
| 1677 DES TA/D 614G | 1 | ||
| G 542 X heteroz | 2 | 1 | |
| Spirometry | |||
| FEV1 % pred | 28±2 | 31±2 | ns |
| FVC % pred | 44±3 | 48±2 | ns |
| FEV1/FVC % | 65±4 | 66±5 | ns |
| Ppa | |||
| Systolic Ppa mmHg | 27±2 | 44±3# | <0.001 |
| PH n | 0 | 10¶ | <0.001 |
Data are presented as mean±se, unless otherwise stated. ED-: patients without pulmonary endothelial dysfunction; ED+: patients with pulmonary endothelial dysfunction; CFTR: cystic fibrosis transmembrane conductance regulator; FEV1: forced expiratory volume in 1 s; % pred: % predicted; FVC: forced vital capacity; Ppa: pulmonary arterial pressure; PH: pulmonary hypertension; ns: nonsignificant. #: Mann–Whitney U-test; ¶: Chi-squared test.