title: Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N.
abstract: In patients with Cystic Fibrosis and a type III mutation, ivacaftor (Kalydeco(®), Vertex) can increase the opening time of the CFTR channel and improve chloride transport. Research showed significant improvement of lung function and increase in weight following ivacaftor use. However, ivacaftor showed to have adverse events on the sinonasal system as well, such as upper respiratory tract infections, nasal congestion and headaches. This case report showed a positive effect of ivacaftor on the sinonasal pathology in a 17 year old patient with CF. After 5 months of ivacaftor use, the CT-sinus showed complete resolution of the opacification of the paranasal sinuses and a decrease in symptoms of sinonasal disease. This positive effect of ivacaftor on sinonasal pathology seems promising, therefore more research is needed to evaluate the effect of ivacaftor on the upper airways in CF.
authors.affiliation: Department of Pulmonology, Haga Teaching Hospital, Leyweg 275, 2545 CH The Hague, The Netherlands.
authors.forename: C L
authors.initials: CL
authors.lastname: Vreede
authors.affiliation: Department of Pulmonology, Haga Teaching Hospital, Leyweg 275, 2545 CH The Hague, The Netherlands. Electronic address: m.berkhout@hagaziekenhuis.nl.
authors.forename: M C
authors.initials: MC
authors.lastname: Berkhout
authors.affiliation: Department of Pediatrics, Haga Teaching Hospital, Sportlaan 600, 2566 MJ The Hague, The Netherlands.
authors.forename: A J
authors.initials: AJ
authors.lastname: Sprij
authors.affiliation: Department of Otorhinolaryngology, Academic Medical Centre Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
authors.forename: W J
authors.initials: WJ
authors.lastname: Fokkens
authors.affiliation: Department of Pulmonology, Haga Teaching Hospital, Leyweg 275, 2545 CH The Hague, The Netherlands.
authors.forename: H G M
authors.initials: HG
authors.lastname: Heijerman
mesh_terms: D000293:Adolescent; D000627:Aminophenols / Q000008:administration & dosage*; D003550:Cystic Fibrosis / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism; D004247:DNA / Q000235:genetics; D005260:Female; D005541:Forced Expiratory Volume; D005838:Genotype; D006801:Humans; D017136:Ion Transport / Q000235:genetics; D009154:Mutation; D010254:Paranasal Sinus Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D010256:Paranasal Sinuses / Q000000981:diagnostic imaging*; D015363:Quinolones / Q000008:administration & dosage*; D014057:Tomography, X-Ray Computed
chemical_list: D000627:Aminophenols; D015363:Quinolones; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; C545203:ivacaftor; D004247:DNA
keywords: CFTR potentiator; Computed Tomography; Cystic Fibrosis; Ivacaftor; Rhinosinusitis