Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation Kris De Boeck, Anne Munck, Seth Walker, Albert Faro,

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Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation Kris De Boeck, Anne Munck, Seth Walker, Albert Faro, Peter Hiatt, Geoffrey Gilmartin, Mark Higgins Journal of Cystic Fibrosis Volume 13, Issue 6, Pages 674-680 (December 2014) DOI: 10.1016/j.jcf.2014.09.005 Copyright © 2014 European Cystic Fibrosis Society. Terms and Conditions

Fig. 1 Mean absolute change from baseline after 8weeks of treatment in (A) percent predicted FEV1; (B) BMI; (C) sweat chloride; and (D) CFQ-R respiratory domain score. BMI=body mass index; CFQ-R=Cystic Fibrosis Questionnaire-Revised. Journal of Cystic Fibrosis 2014 13, 674-680DOI: (10.1016/j.jcf.2014.09.005) Copyright © 2014 European Cystic Fibrosis Society. Terms and Conditions

Fig. 2 Mean absolute change from baseline in percent predicted FEV1 over 24weeks of ivacaftor treatment for patients from treatment sequence 2 (placebo→ivacaftor). Journal of Cystic Fibrosis 2014 13, 674-680DOI: (10.1016/j.jcf.2014.09.005) Copyright © 2014 European Cystic Fibrosis Society. Terms and Conditions