{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T00:38:47Z","timestamp":1791419927821,"version":"4.3.3"},"reference-count":39,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,3,2]],"date-time":"2025-03-02T00:00:00Z","timestamp":1740873600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","award":["ZEMKE19A0"],"award-info":[{"award-number":["ZEMKE19A0"]}],"id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007921","name":"University of Pittsburgh","doi-asserted-by":"publisher","award":["UL1\u2010TR\u2010001857"],"award-info":[{"award-number":["UL1\u2010TR\u2010001857"]}],"id":[{"id":"10.13039\/100007921","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int Forum Allergy Rhinol"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Severe chronic rhinosinusitis (CRS) is a near universal manifestation of cystic fibrosis. Elexacaftor\/tezacaftor\/ivacaftor (ETI) is an oral, small molecule, highly effective Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) corrector\u2013potentiator drug. In people with cystic fibrosis age &gt; 12 years, ETI improves sinonasal symptoms, endoscopy findings, polyp size, and radiologic findings. This study evaluates changes in CRS in children ages 6\u201312 years newly started on ETI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a prospective, three center, pre\u2013post study of 11 children age 6\u201311 years newly started on ETI. Study endpoints included the SN\u20105 sinonasal health survey, Sniffin\u2019 Kids olfaction test, a sinus computerized tomography (CT) scan, and nasal endoscopy with mucus sampling for full\u2010length 16S rRNA sequencing microbiome analysis. Study visits were conducted before ETI and at a median of 9 months after treatment initiation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>ETI lead to improvement in symptoms, endoscopy scores and radiologic findings of CRS. Olfaction was below normal at baseline and did not improve. The sinonasal microbiome was dominated by typically commensal organisms before and after treatment for most participants. Additionally, <jats:italic>Staphylococcus aureus<\/jats:italic> was found in five participants at baseline and six participants on treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>ETI improves sinonasal symptoms and endoscopy findings in children 6\u201311 years of age. Olfaction did not improve with ETI treatment in this age group, suggesting that olfactory dysfunction associated with CF is established early in life. This younger cohort of pediatric patients presented with abundant <jats:italic>Staphylococcus aureus<\/jats:italic> and only very rare <jats:italic>Pseudomonas aeruginosa<\/jats:italic> at baseline or after treatment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/alr.23555","type":"journal-article","created":{"date-parts":[[2025,3,12]],"date-time":"2025-03-12T06:38:46Z","timestamp":1741761526000},"page":"706-714","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Elexacaftor\u2013Tezacaftor\u2013Ivacaftor Improves Sinonasal Outcomes in Young Children With Cystic Fibrosis"],"prefix":"10.1002","volume":"15","author":[{"given":"Amanda L.","family":"Stapleton","sequence":"first","affiliation":[{"name":"Department of Otolaryngology \u2013 Head &amp; Neck Surgery University of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Kimple","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology \u2013 Head &amp; Neck Surgery University of North Carolina  Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer L.","family":"Goralski","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology University of North Carolina  Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8612-5442","authenticated-orcid":false,"given":"Daniel M.","family":"Beswick","sequence":"additional","affiliation":[{"name":"Department of Head and Neck Surgery University of California Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anshika","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Microbiology and Molecular Genetics University of Pittsburgh  Pittsburgh Pennsylvania USA"},{"name":"Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine University of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas A.","family":"Li","sequence":"additional","affiliation":[{"name":"Division of Pediatric Pulmonology University of California Los Angeles  Los Angeles California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barton F.","family":"Branstetter","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology \u2013 Head &amp; Neck Surgery University of Pittsburgh  Pittsburgh Pennsylvania USA"},{"name":"Department of Radiology University of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Mehdi","family":"Nouraie","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine University of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5783-9198","authenticated-orcid":false,"given":"Amber D.","family":"Shaffer","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology \u2013 Head &amp; Neck Surgery University of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1503-3150","authenticated-orcid":false,"given":"Brent","family":"Senior","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology \u2013 Head &amp; Neck Surgery University of North Carolina  Chapel Hill North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7722-6708","authenticated-orcid":false,"given":"Anna C.","family":"Zemke","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy, Critical Care, and Sleep Medicine University of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,3,2]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.08.007"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.27454"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.03.022"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ismej.2011.83"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.2500\/AJRA.2015.29.4196"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/J.JCF.2022.03.002"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202101-057OC"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/J.JCF.2020.07.002"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ALR.22716"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.09.014"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.22891"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ALR.23288"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1128\/spectrum.00787\u201024"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/PPUL.26898"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202209\u2010763OC"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1177\/0194599817702332"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0101086"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1067\/mhn.2003.41"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1177\/000348949510410s02"},{"key":"e_1_2_7_21_1","first-page":"183","article-title":"Staging in Rhinosinusitis","volume":"31","author":"Lund V.","year":"1993","journal-title":"Rhinology"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/CPZ1.978"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1093\/NAR\/GKU1201"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1093\/NAR\/GKV1189"},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/apm.13427"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005537\u2010199608000\u201000018"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.03.011"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.31438"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.23016"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202101\u2010057OC"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2021.109829"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1128\/spectrum.01251\u201022"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.22700"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.03.002"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.01.004"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0101086"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/alr.22891"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.adn5611"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.20396"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1128\/spectrum.00787\u201024"}],"container-title":["International Forum of Allergy &amp; 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