Table 1.
Data of anthropometrics, body composition, pulmonary function, and resting energy expenditure, before and after ivacaftor treatment in seven individuals with CF and S1251N mutation
| Variable | Pre‐ivacaftor (mean, SD) | Post‐ivacaftor (mean, SD) | P value (two‐tailed) | 95% CI (lower; upper) |
|---|---|---|---|---|
| Anthropometrics and body composition | ||||
| Height/(Z‐score) §, cm | 160.9 (18.5)/−0.4 (0.8) | 164.8 (15.2)/−0.2 (0.8) | ||
| Weight/(Z‐score) §, kg | 54.0 (19.7)/0.2 (0.7) | 59.2 (18.6)/0.6 (0.4) | ||
| BMI/(Z‐score), kg/m2 | 19.9 (3.4)/0.4 (0.7) | 21.2 (3.4)/0.6 (0.8) | *.027/.22 | 0.2; 2.4/−0.2; 0.6 |
| Fat mass, kg | 12.5 (6.1) | 15.0 (5.5) | .16 | −1.4; 6.4 |
| Lean mass, kg | 34.9 (13.9) | 37.4 (12.2) | .07 | −0.3; 5.4 |
| Pulmonary function | ||||
| ppFEV1, % | 81.7 (17.8) | 97.7 (19.6) | .015* | 4.4; 27.6 |
| RV, §, l | 1.5 (0.5) | 0.9 (0.3) | ||
| RV/TLC, % | 31.5 (10.5) | 16.8 (5.4) | .016* | 21.0; 3.8 |
| Resting energy expenditure | ||||
| REE, kcal/d | 1966.8 (305.9) | 1751.7 (530.9) | .43 | −859.5; 429.2 |
Note: § = not assessed with paired‐samples t test. P < .05 is considered statistically significant as indicated in bold and with *.
Abbreviations: BMI, body mass index; CI, confidence interval; ppFEV1, forced expiratory volume in 1 second, in percentage predicted; REE, resting energy expenditure; RV, residual volume; TLC, total lung capacity.