Table 1.
Demographic and Clinical Characteristics, by Mutation
| Characteristic | F/F (n = 11,916) | F/RF (all) (n = 1242) | P-valuea | F/RF (excluding R117H) (n = 889) | P-valueb |
|---|---|---|---|---|---|
| Age, years, mean (SD) | 18.0 (9.6) | 23.0 (12.1) | < 0.001 | 23.6 (11.8) | < 0.001 |
| Female, n (%) | 5698 (47.8) | 645 (51.9) | 0.006 | 473 (53.2) | 0.002 |
| Height-for-age z-score, mean (SD) | −0.49 (1.03) | 0.04 (1.03) | < 0.001 | −0.01 (1.02) | < 0.001 |
| Weight-for-age z-score, mean (SD) | −0.48 (1.11) | 0.36 (1.14) | < 0.001 | 0.29 (1.15) | < 0.001 |
| BMI-for-age z-score, mean (SD) | −0.29 (1.08) | 0.36 (1.09) | < 0.001 | 0.29 (1.10) | < 0.001 |
| ppFEV1 at baseline visit, %, mean (SD) | 73.4 (26.5) | 80.4 (24.8) | < 0.001 | 78.6 (25.3) | < 0.001 |
| Patients with positive microbiology n (%)c | |||||
| Staphylococcus aureus | 5386 (45.2) | 524 (42.2) | 0.042 | 402 (45.2) | 0.99 |
| Pseudomonas aeruginosa | 4131 (34.7) | 277 (22.3) | < 0.001 | 234 (26.3) | < 0.001 |
| Haemophilus influenzae | 662 (5.6) | 69 (5.6) | 1.00 | 49 (5.5) | 0.96 |
| Aspergillus species | 688 (5.8) | 42 (3.4) | < 0.001 | 32 (3.6) | 0.007 |
| Stenotrophomonas maltophilia | 677 (5.7) | 48 (3.9) | 0.008 | 38 (4.3) | 0.078 |
| Burkholderia cepacia | 237 (2.0) | 7 (0.6) | < 0.001 | 7 (0.8) | 0.012 |
BMI body mass index, F F508del, ppFEV1 percent predicted forced expiratory volume in 1 s, RF residual function
aF/F versus F/RF (all)
bF/F versus F/RF (excluding R117H)
cAt first visit in the 2-year period