{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:09:44Z","timestamp":1786975784747,"version":"build-2736575974"},"reference-count":38,"publisher":"American Medical Association (AMA)","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Otolaryngol Head Neck Surg"],"published-print":{"date-parts":[[2023,12,1]]},"abstract":"<jats:sec id=\"ab-ooi230057-4\">\n                    <jats:title>Importance<\/jats:title>\n                    <jats:p>Cystic fibrosis (CF) is a multiorgan genetic disease with progressive upper and lower airway involvement. The effects of CF transmembrane conductance regulator (CFTR) modifier therapies on CF-related upper airway disease, specifically chronic rhinosinusitis (CRS), are not characterized.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-ooi230057-5\">\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To determine the outcome of elexacaftor-tezacaftor-ivacaftor (ETI) on CRS as measured by changes in sinus computed tomography (CT) metrics and on clinical parameters in individuals with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-ooi230057-6\">\n                    <jats:title>Design, Setting, and Participants<\/jats:title>\n                    <jats:p>This prospective longitudinal cohort study was conducted at the CF center of a tertiary care hospital between October 1, 2019, and July 31, 2021. A total of 64 participants with CF were included in the analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-ooi230057-7\">\n                    <jats:title>Intervention<\/jats:title>\n                    <jats:p>\n                      Sinus CT was obtained within 1 month of initiation of ETI therapy (baseline), and within 1 month of 1 year of ETI therapy. Images were independently analyzed by pulmonology, radiology, and otolaryngology physicians, using the Lund-Mackay and Sheikh-Lind scoring systems. Percent predicted forced expiratory volume in 1 second (ppFEV\n                      <jats:sub>1)<\/jats:sub>\n                      , body mass index (BMI), and microbiologic data collected at initiation of ETI therapy and 3-month intervals for 1 year were also measured.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-ooi230057-8\">\n                    <jats:title>Main Outcomes and Measures<\/jats:title>\n                    <jats:p>The study hypothesis was that ETI therapy will improve CRS as measured by changes in sinus CT at initiation and 1 year after ETI therapy and clinical parameters in individuals with CF.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-ooi230057-9\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among the 64 participants (39 [60.9%] female; median age, 18.5 [IQR, 16.0-28.5] years; 64 [100%] White), improvement in CRS was noted by improvements in sinus CT scans using both sinus CT scoring systems after 1 year of ETI therapy. The reduction in the median total score using the Lund-Mackay sinus CT scoring system (from 5.8 [IQR, 5.0-7.0] to 3.3 [IQR, 2.6-4.2]) and the Sheikh-Lind scoring system (from 3.8 [IQR, 3.0-5.0] to 2.2 [IQR, 2.0-2.5]) was noted. Increases in ppFEV\n                      <jats:sub>1<\/jats:sub>\n                      and BMI were also observed by 3 months of ETI therapy with persistent improvement through 1 year of treatment. Similarly, after 1 year of ETI therapy, participants with CF had reductions in positivity for\n                      <jats:italic>Pseudomonas aeruginosa<\/jats:italic>\n                      and\n                      <jats:italic>Staphylococcus aureus<\/jats:italic>\n                      in oropharyngeal cultures.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-ooi230057-10\">\n                    <jats:title>Conclusion and Relevance<\/jats:title>\n                    <jats:p>This cohort study found that use of ETI therapy was associated with improved CRS outcomes in participants with CF as quantified by improved sinus CT scans measured by 2 radiographic scoring systems and was also associated with improved clinical outcomes. Despite improvement in CT scan scores, most people with CF continue to have scores that indicate severe sinus disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1001\/jamaoto.2023.2701","type":"journal-article","created":{"date-parts":[[2023,9,7]],"date-time":"2023-09-07T11:33:08Z","timestamp":1694086388000},"page":"1075","source":"Crossref","is-referenced-by-count":21,"title":["Elexacaftor-Tezacaftor-Ivacaftor Therapy for Chronic Sinus Disease in Cystic Fibrosis"],"prefix":"10.1001","volume":"149","author":[{"given":"Shahid","family":"Sheikh","sequence":"first","affiliation":[{"name":"Department of Pediatrics, The Ohio State University College of Medicine, Columbus"},{"name":"Division of Pulmonary Medicine, Nationwide Children\u2019s Hospital, Columbus, Ohio"},{"name":"Nationwide Children\u2019s Hospital, Columbus, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mai-Lan","family":"Ho","sequence":"additional","affiliation":[{"name":"Nationwide 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