TY - JOUR T1 - Lung clearance index response in patients with CF with class III CFTR mutations JF - Thorax JO - Thorax SP - 476 LP - 477 DO - 10.1136/thoraxjnl-2015-207894 VL - 71 IS - 5 AU - Mica Kane AU - Tanja Gonska AU - Renee Jensen AU - Julie Avolio AU - Michelle Klingel AU - Sanja Stanojevic AU - Felix Ratjen Y1 - 2016/05/01 UR - http://thorax.bmj.com/content/71/5/476.abstract N2 - Ivacaftor (KALYDECO) is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that increases transmembrane chloride flux in vitro and leads to significant benefits in patients with cystic fibrosis (CF) with class III gating mutations.1–5 Ivacaftor is associated with sustained improvement in FEV1 and weight as well as reduced time to next pulmonary exacerbation.5–7 It has also been shown that 4 weeks of ivacaftor improves the lung clearance index (LCI) in patients with CF with preserved lung function.8It is presently unclear whether LCI, a measure of ventilation inhomogeneity, provides additional information among patients with more impaired lung function as well as whether the sustained effectiveness of ivacaftor as demonstrated by improvements in the previously mentioned outcomes is also evident in the LCI response. The aim of this observational study was to assess the LCI before and after initiation of ivacaftor treatment over 6 months in patients with CF with a wider range of … ER -