Table 5.
Change in FEV1pp within 15–75 days and over 1 year on ivacaftor treatment according to SLC26A9 variants in patients carrying at least one ivacaftor-approved CFTR gating mutation.
| Period of evaluation | 15–75 days after ivacaftor treatment start |
Over the 1st year of ivacaftor treatment |
||||
|---|---|---|---|---|---|---|
| SLC26A9 variants$ | Gating$/other (n = 23) |
Gating$/other (n = 30) |
||||
| SNP | FEV1pp change∗ | P-value | Adjusted P-value∗∗ | FEV1pp change∗ | P-value∗ | Adjusted P-value∗∗ |
| rs7512462 | –5.9 ± 1.7 | 0.0031 | 0.0217 | –5.2 ± 1.8 | 0.0042 | 0.0294 |
| rs1874361 | 3.7 ± 1.3 | 0.0120 | 0.0840 | 3.9 ± 1.9 | 0.0479 | 0.3346 |
| rs12741299 | –2.8 ± 4.8 | 0.5656 | 1.0000 | –2.7 ± 4.2 | 0.5270 | 1.0000 |
| rs4077468 | –7.0 ± 2.1 | 0.0033 | 0.0231 | –7.3 ± 2.2 | 0.0030 | 0.0210 |
| rs4077469 | –7.8 ± 2.6 | 0.0070 | 0.0490 | –8.2 ± 2.6 | 0.0040 | 0.0280 |
| rs12047830 | –6.3 ± 2.4 | 0.0138 | 0.0966 | –6.6 ± 2.6 | 0.0161 | 0.1127 |
| rs7419153 | 6.3 ± 2.6 | 0.0233 | 0.1631 | 7.9 ± 2.5 | 0.0038 | 0.0266 |
$See description Supplementary Table S2; ∗linear regression of FEV1pp (forced expiratory volume in one second percent-predicted) change with additive model adjusted on baseline; ∗∗Bonferroni adjustment; $Ivacaftor-approved CFTR gating mutations: G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, and S549R.