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

ABSTRACT:

Importance

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.

Objective

To determine the impact of ivacaftor therapy on sinonasal pathology in patients with cystic fibrosis with an S1251N mutation.

Design

Prospective observational mono-center cohort study, between June 2015 and December 2016.

Setting</

SUBMITTER: Gostelie R 

PROVIDER: S-EPMC7371187 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature