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Lung clearance index response in patients with CF with class III CFTR mutations
  1. Mica Kane1,2,
  2. Tanja Gonska2,3,4,
  3. Renee Jensen1,2,
  4. Julie Avolio1,2,3,
  5. Michelle Klingel1,2,
  6. Sanja Stanojevic1,2,5,
  7. Felix Ratjen1,2,4
  1. 1Division of Respiratory Medicine, Department of Pediatrics, Toronto, Ontario, Canada
  2. 2Physiology & Experimental Medicine, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
  3. 3Division of Gastroenterology and Nutrition, Department of Pediatrics, Toronto, Ontario, Canada
  4. 4Department of Pediatrics, University of Toronto, Ontario, Canada
  5. 5Institute of Health Policy, Management and Evaluation, University of Toronto, Ontario, Canada
  1. Correspondence to Professor Felix Ratjen, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8; Felix.ratjen{at}sickkids.ca

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Background

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.8

It 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 …

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