Estimated ppFEV1 intercept and slope by CFTR mutation (R117H or homozygous F508del) (Tables 2 and 3). Panel A: Overall estimates. R117H-CFTR mutation n=156, F508del-CFTR mutation n=6,251. Panel B: Estimates separated by age group. R117H-CFTR mutation ages 6–12 years, n=36; ages 13–17 years, n=17; ages 18–24 years, n=18; ages ≥25 years, n=85). F508del-CFTR mutation ages 6–12 years, n=2,398; ages 13–17 years, n=1,447; ages 18–24 years, n=1,278; ages ≥25 years, n=1,128.
Solid lines = R117H-CFTR mutation. Dashed lines = homozygous F508del-CFTR mutation. Shaded areas represent 95% Confidence Intervals.
ppFEV1, percentage of predicted forced expiratory volume in 1 second.(9)