The impact of ivacaftor on sinonasal pathology in S1251N-mediated cystic fibrosis patients

PloS One
Romee GostelieLucienne Speleman

Abstract

Sinonasal symptoms in patients suffering from cystic fibrosis can negatively influence the quality of life and sinuses can be a niche for pathogens causing infection and inflammation leading to a decrease of lung function. Ivacaftor, a potentiator of the Cystic Fibrosis Transmembrane Conductance Regulator protein, has shown improvement in pulmonary function in cystic fibrosis patients with different forms of class III gating mutations. However, the effects of ivacaftor on sinonasal pathology have hardly been studied. To determine the impact of ivacaftor therapy on sinonasal pathology in patients with cystic fibrosis with an S1251N mutation. Prospective observational mono-center cohort study, between June 2015 and December 2016. A tertiary referral center in Utrecht, The Netherlands. Eight patients with cystic fibrosis with an S1251N mutation, treated with the potentiator ivacaftor were investigated. Ivacaftor (Kalydeco, VX-770) therapy. Computed tomography imaging of paranasal sinuses. Nasal nitric oxide concentration measurements and nasal endoscopy. Primary outcome is opacification of paranasal sinuses examined with computed tomography scan analysis and scaled by the modified Lund-Mackay score before and one year after treatm...Continue Reading

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