TY - JOUR
T1 - Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N
AU - Vreede, C. L.
AU - Berkhout, M. C.
AU - Sprij, A. J.
AU - Fokkens, W. J.
AU - Heijerman, H. G. M.
PY - 2015
Y1 - 2015
N2 - 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
AB - 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
U2 - https://doi.org/10.1016/j.jcf.2014.07.013
DO - https://doi.org/10.1016/j.jcf.2014.07.013
M3 - Article
C2 - 25169789
SN - 1569-1993
VL - 14
SP - 412
EP - 413
JO - Journal of cystic fibrosis
JF - Journal of cystic fibrosis
IS - 3
ER -