P041 Correlation of improvements in sweat chloride and percent predicted FEV1 across twenty studies examining different corrector therapies for F508del

Abstract

Objectives: Cystic fibrosis is caused by a variant in the cystic fibrosis transmembrane conductance regulator protein, of which the class II variant, F508del, is the commonest. A number of therapies aiming to correct the F508del defect have been assessed in clinical trials using change in FEV1 and sweat chloride as outcome measures. We aim to establish if there is a correlation between changes in sweat chloride and FEV1 in studies included in a Cochrane systematic review evaluating corrector therapies for people with class II variants.