Article
Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2017
Fidler Meredith C, Beusmans Jack, Panorchan Paul, Van Goor Fredrick
Abstract excerpt
Ivacaftor, a CFTR potentiator that enhances chloride transport by acting directly on CFTR to increase its channel gating activity, has been evaluated in patients with different CFTR mutations. Several previous analyses have reported no statistical correlation between change from baseline in ppFEV1 and reduction in sweat chloride levels for individuals treated with ivacaftor. The objective of the post hoc analysis...
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