Observational studyJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2022
Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis.
Observational study in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
25 citing papers in PubMed, 2 syntheses or guidelines pooled it, 82 citations in OpenAlex.
- Systematic Review of Nasal Endoscopy Scores in Cystic Fibrosis Patients Treated With Cystic Fibrosis Transmembrane Conductance Regulator Modulators.Pediatric pulmonology · 2026Pooled it
- Efficacy and safety profile of elexacaftor-tezacaftor-ivacaftor triple therapy on cystic fibrosis: a systematic review and single arm meta-analysis.Frontiers in pharmacology · 2023Pooled it
- Sinus Surgery Improves Maxillary Sinus Deformation in Cystic Fibrosis Detected by Magnetic Resonance Imaging.American journal of rhinology & allergy · 2026Article
- Elexacaftor-Tezacaftor-Ivacaftor Reduces Revision Sinus Surgery in People With Cystic Fibrosis.The Laryngoscope · 2026Article
- The Upper Airway Microbiome in Bronchiectasis: Expanding the Landscape of Airway Dysbiosis.American journal of respiratory and critical care medicine · 2025Article
- Clinical Outcomes in Patients with Cystic Fibrosis-Related Chronic Rhinosinusitis Treated with Functional Endoscopic Sinus Surgery or Triple Highly Effective Modulator Therapy: A Monocentric Retrospective Experience.Journal of clinical medicine · 2025Article
- Elexacaftor-Tezacaftor-Ivacaftor Improves Sinonasal Outcomes in Young Children With Cystic Fibrosis.International forum of allergy & rhinology · 2025Article
- Beyond the Lung. Impact of Elexacaftor/Tezacaftor/Ivacaftor on Sinonasal Disease in Children With Cystic Fibrosis.International forum of allergy & rhinology · 2025Observational
- Olfactory loss in people with cystic fibrosis: Community perceptions and impact.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- Impact of sociodemographic status and sex on chronic rhinosinusitis and olfaction in people with cystic fibrosis.International forum of allergy & rhinology · 2024Article
- Review
- Predictors of Sinonasal Improvement After Highly Effective Modulator Therapy in Adults with Cystic Fibrosis.The Laryngoscope · 2024Observational
- Elexacaftor-tezacaftor-ivacaftor decreases pseudomonas abundance in the sinonasal microbiome in cystic fibrosis.International forum of allergy & rhinology · 2024Article
- Effects of highly effective modulator therapy on the dynamics of the respiratory mucosal environment and inflammatory response in cystic fibrosis.Pediatric pulmonology · 2024Article
- Patient perspectives on elexacaftor/tezacaftor/ivacaftor after lung transplant.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- Scoring Abdominal Symptoms in People with Cystic Fibrosis.Journal of clinical medicine · 2024Review
- Observational
- Sinonasal quality of life in primary ciliary dyskinesia.International forum of allergy & rhinology · 2023Article
- Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis.Pharmaceuticals (Basel, Switzerland) · 2023Review
- Revisiting Host-Pathogen Interactions in Cystic Fibrosis Lungs in the Era of CFTR Modulators.International journal of molecular sciences · 2023Review
Corrections and comments
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
Abstract
backgroundMany individuals with cystic fibrosis (CF) have chronic rhinosinusitis resulting in nasal obstruction, sinus infections, and repeated surgeries. Elexacaftor-tezacaftor-ivacaftor is a highly effective modulator therapy approved for individuals aged 6 years or older with CF who have at least one F508del allele or other responsive mutation. The current study tests the hypothesis that ELX/TEZ/IVA improves sinonasal disease in CF.
methodsThe study was a pre/post, observational cohort study conducted at two sites. Participants underwent a study visit prior to starting ELX/TEZ/IVA and a second visit at a median of 9 months on therapy. Each visit included sinus CT scan, rigid nasal endoscopy, and sweat chloride measurement. Symptoms were measured with the 22 item Sinonasal Outcome Test at scheduled intervals during the study. Regression models were used to test for improvement in symptoms, endoscopy, and CT scales.
resultsThe study enrolled 34 individuals, with a median age of 27 years (range 12-60). Symptoms improved within 7 days of therapy and plateaued by day 28. Endoscopic crusting resolved and nasal polyposis improved, with a decrease in size or resolution of polyps. Sinus opacification and mucosal thickening improved on CT radiographs with treatment.
conclusionsSinonasal symptoms improved rapidly and durably for at least 180 days on ELX/TEZ/IVA therapy. Objective measures of disease including endoscopic and CT findings improved with ELX/TEZ/IVA.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.