TABLE 2.
Effects of ivacaftor on clinical parameters and inflammationa
| Parameter | Mean | No. | Change from baseline |
||
|---|---|---|---|---|---|
| Mean | 95% CI | P value (adj) | |||
| Sweat chloride (mM) | |||||
| Baseline | 80.44 | 9 | |||
| 2 days | 50.33 | 9 | −30.11 | (−41.70 to −18.53) | 0.0002 |
| 7 days | 55.88 | 8 | −24.57 | (−35.99 to −13.14) | 0.0008 |
| 2.5 yrs | 42.13 | 8 | −38.32 | (−62.61 to −14.03) | 0.0050 |
| FEV1 (% predicted) | |||||
| Baseline | 64.98 | 10 | |||
| 2 days | 68.87 | 10 | 3.88 | (0.35 to 7.41) | 0.03 |
| 7 days | 71.98 | 10 | 7.00 | (1.68 to 12.33) | 0.01 |
| 2.5 yrs | 71.14 | 8 | 6.16 | (−1.34 to 13.66) | 0.11 |
| Neutrophil elastase (log10 ug/mL) | |||||
| Baseline | 1.76 | 10 | |||
| 2 days | 1.51 | 10 | −0.25 | (−1.12 to 0.60) | 0.86 |
| 7 days | 1.07 | 9 | −0.69 | (−1.39 to −0.003) | 0.049 |
| 2.5 yrs | 1.04 | 7 | −0.72 | (−2.17 to 0.72) | 0.38 |
| IL-1β (log10 pg/mL) | |||||
| Baseline | 3.69 | 10 | |||
| 2 days | 3.66 | 10 | −0.03 | (−0.92 to 0.85) | 0.99 |
| 7 days | 3.38 | 9 | −0.31 | (−1.21 to 0.60) | 0.79 |
| 2.5 yrs | 2.60 | 7 | −1.09 | (−2.63 to 0.44) | 0.17 |
| IL-8 (log10 pg/mL) | |||||
| Baseline | 4.68 | 10 | |||
| 2 days | 4.70 | 10 | 0.02 | (−0.71 to 0.75) | >0.99 |
| 7 days | 4.50 | 9 | −0.19 | (−1.08 to 0.71) | 0.97 |
| 2.5 yrs | 4.16 | 7 | −0.52 | (−1.64 to 0.60) | 0.48 |
| MacConkey (log10 CFU/mL) (presumptive Pa) | |||||
| Baseline | 6.62 | 5 | |||
| 2 days | 6.26 | 5 | −0.37 | (−2.50 to 1.77) | 0.96 |
| 7 days | 5.61 | 4 | −1.02 | (−3.59 to 1.56) | 0.41 |
| 2.5 yrs | 3.34 | 3 | −3.28 | (−8.42 to 1.85) | 0.12 |
| MSA (log10 CFU/mL) (presumptive Sa) | |||||
| Baseline | 5.31 | 7 | |||
| 2 days | 4.98 | 7 | −0.33 | (−2.35 to 1.69) | 0.99 |
| 7 days | 4.19 | 6 | −1.11 | (−2.11 to −0.12) | 0.03 |
| 2.5 yrs | 3.49 | 4 | −1.82 | (−6.33 to 2.68) | 0.40 |
CI, confidence interval; adj, adjusted for multiple comparisons; FEV1, forced expiratory volume in 1 second; IL, interleukin; Pa, Pseudomonas aeruginosa; MSA, mannitol salt agar; Sa, Staphylococcus aureus.