TY - JOUR
T1 - The criteria for chronic rhinosinusitis in children with cystic fibrosis are rarely fulfilled after initiation of CFTR modulator treatment
AU - Bech, Magne
AU - Skov, Marianne
AU - Andersen, Ida S B
AU - von Buchwald, Christian
AU - Aanæs, Kasper
N1 - © 2024 The Authors. APMIS published by John Wiley & Sons Ltd on behalf of Scandinavian Societies for Pathology, Medical Microbiology and Immunology.
PY - 2024
Y1 - 2024
N2 - The vast majority of people with cystic fibrosis (pwCF) have untreated secondary chronic rhinosinusitis (CRS). Whereas the introduction of the cystic fibrosis transmembrane conductance regulator modulator (CFTRm) treatment regime has improved the lung function of pwCF, few studies have been published examining the effect on sinonasal symptoms in children. Our aim was to explore the effect of double CFTRm treatment on CRS and olfaction in children with CF. pwCF were included in this non-randomized cross-sectional study, where an otolaryngologist performed a complete ENT examination before initiating treatment with elaxacaftor/tezacaftor/ivacaftor (ETI). Twenty-three pwCF aged 6-12 years were included. Eighteen of 23 patients were on a double CFTRm treatment, and 5 patients were CFTRm naive, respectively. Altogether, 19 had normal olfaction, 20 had none or mild CRS symptoms according to SNOT-22, and 14 had a normal endoscopy. None of the patients had symptoms of chronic rhinosinusitis lasting for more than 12 weeks, thus none of the patients fulfilled the criteria for CRS. Children with CF treated with double CFTRm have few to no symptoms of CRS and normal olfaction, which is an improvement compared with children following treatment modalities prior to CFTRm.
AB - The vast majority of people with cystic fibrosis (pwCF) have untreated secondary chronic rhinosinusitis (CRS). Whereas the introduction of the cystic fibrosis transmembrane conductance regulator modulator (CFTRm) treatment regime has improved the lung function of pwCF, few studies have been published examining the effect on sinonasal symptoms in children. Our aim was to explore the effect of double CFTRm treatment on CRS and olfaction in children with CF. pwCF were included in this non-randomized cross-sectional study, where an otolaryngologist performed a complete ENT examination before initiating treatment with elaxacaftor/tezacaftor/ivacaftor (ETI). Twenty-three pwCF aged 6-12 years were included. Eighteen of 23 patients were on a double CFTRm treatment, and 5 patients were CFTRm naive, respectively. Altogether, 19 had normal olfaction, 20 had none or mild CRS symptoms according to SNOT-22, and 14 had a normal endoscopy. None of the patients had symptoms of chronic rhinosinusitis lasting for more than 12 weeks, thus none of the patients fulfilled the criteria for CRS. Children with CF treated with double CFTRm have few to no symptoms of CRS and normal olfaction, which is an improvement compared with children following treatment modalities prior to CFTRm.
KW - Aminophenols/therapeutic use
KW - Child
KW - Chronic Disease
KW - Cross-Sectional Studies
KW - Cystic Fibrosis Transmembrane Conductance Regulator/genetics
KW - Cystic Fibrosis/drug therapy
KW - Drug Combinations
KW - Female
KW - Humans
KW - Male
KW - Quinolones/therapeutic use
KW - Rhinitis/drug therapy
KW - Rhinosinusitis
KW - Sinusitis/drug therapy
UR - http://www.scopus.com/inward/record.url?scp=85193741183&partnerID=8YFLogxK
U2 - 10.1111/apm.13427
DO - 10.1111/apm.13427
M3 - Journal article
C2 - 38773823
SN - 0903-4641
VL - 132
SP - 625
EP - 631
JO - APMIS - Journal of Pathology, Microbiology and Immunology
JF - APMIS - Journal of Pathology, Microbiology and Immunology
IS - 9
ER -