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Elexacaftor-Tezacaftor- Ivacaftor improves sinonasal outcomes in cystic fibrosis.
Stapleton, Amanda L; Kimple, Adam J; Goralski, Jennifer L; Nouraie, S Mehdi; Branstetter, Barton F; Shaffer, Amber D; Pilewski, Joseph M; Senior, Brent A; Lee, Stella E; Zemke, Anna C.
Afiliación
  • Stapleton AL; Department of Otolaryngology - Head and Neck Surgery, University of Pittsburgh, United States.
  • Kimple AJ; Department of Otolaryngology - Head & Neck Surgery, University of North Carolina, United States.
  • Goralski JL; Division of Pulmonary Diseases & Critical Care Medicine, University of North Carolina, United States.
  • Nouraie SM; Department of Medicine, Division of Pulmonary, Allergy & Critical Care Medicine, University of Pittsburgh, United States.
  • Branstetter BF; Department of Otolaryngology - Head and Neck Surgery, University of Pittsburgh, United States; Department of Radiology, University of Pittsburgh, United States; Department of Biomedical Informatics, University of Pittsburgh, United States.
  • Shaffer AD; Department of Otolaryngology - Head and Neck Surgery, University of Pittsburgh, United States.
  • Pilewski JM; Department of Medicine, Division of Pulmonary, Allergy & Critical Care Medicine, University of Pittsburgh, United States.
  • Senior BA; Department of Otolaryngology - Head & Neck Surgery, University of North Carolina, United States.
  • Lee SE; Department of Otolaryngology - Head and Neck Surgery, University of Pittsburgh, United States.
  • Zemke AC; Department of Medicine, Division of Pulmonary, Allergy & Critical Care Medicine, University of Pittsburgh, United States.
J Cyst Fibros ; 21(5): 792-799, 2022 09.
Article en En | MEDLINE | ID: mdl-35300931
BACKGROUND: Many 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. METHODS: The 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. RESULTS: The 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. CONCLUSIONS: Sinonasal 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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Sinusitis / Fibrosis Quística Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Humans / Middle aged Idioma: En Revista: J Cyst Fibros Año: 2022 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Países Bajos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Sinusitis / Fibrosis Quística Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Humans / Middle aged Idioma: En Revista: J Cyst Fibros Año: 2022 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Países Bajos