Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation Helge Hebestreit, Annette Sauer-Heilborn, Rainald Fischer,

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Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation Helge Hebestreit, Annette Sauer-Heilborn, Rainald Fischer, Manfred Käding, Jochen G. Mainz Journal of Cystic Fibrosis Volume 12, Issue 6, Pages 599-603 (December 2013) DOI: 10.1016/j.jcf.2013.05.006 Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions

Fig. 1 Change in FEV1, body weight and RV/TLC in 14 patients after the start of ivacaftor (day 0). The graphs on the right display the average of all available measurements obtained during the year prior to ivacaftor (“pre”) and the last measurement reported (“post”). Journal of Cystic Fibrosis 2013 12, 599-603DOI: (10.1016/j.jcf.2013.05.006) Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions

Fig. 2 Changes in FEV1 after the start of ivacaftor and its associates. A) There was no association between baseline FEV1 and ΔFEV1. B) The change in body weight was not significantly correlated with ΔFEV1. C) A decrease in RV/TLC was significantly associated with an increase in FEV1 in the 10 patients with RV and TLC measurements. Journal of Cystic Fibrosis 2013 12, 599-603DOI: (10.1016/j.jcf.2013.05.006) Copyright © 2013 European Cystic Fibrosis Society. Terms and Conditions