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Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis.

Abstract
Upper gastrointestinal and upper airway disease are common in cystic fibrosis (CF) and may contribute to lower airway infection and inflammation. In a longitudinal cohort study of 32 patients (23 men; median age 32.5 years) with advanced CF lung disease (median FEV1 24.8% predicted) starting elexacaftor-tezacaftor-ivacaftor, the reflux symptom index score fell from a pre-treatment median (IQR) of 15 (11-23) to 5 (2.8-7.3) (p<0.001), the Hull airway reflux score fell from a median of 26.5 (16.3-39) to 7.5 (4-12) (p<0.001), and the sinonasal outcome score from a median of 36.5 (22-24) to 20 (10-32) (p<0.001) at 6 months on treatment. Mean FEV1% predicted rose by 9.2 points, the median respiratory domain score of the CF Questionnaire-Revised rose by 27.8 points and mean body mass index rose by 2.6 kg/m2. In addition to improving lung function and weight, CFTR modulators improve upper airway and gastro-oesophageal reflux symptoms in advanced CF.
AuthorsSufyan Shakir, Carlos Echevarria, Simon Doe, Malcolm Brodlie, Christopher Ward, Stephen J Bourke
JournalJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (J Cyst Fibros) Vol. 21 Issue 5 Pg. 807-810 (09 2022) ISSN: 1873-5010 [Electronic] Netherlands
PMID35718668 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2022. Published by Elsevier B.V.
Chemical References
  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists
  • Indoles
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones
  • tezacaftor
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor
  • elexacaftor
Topics
  • Adult
  • Aminophenols (therapeutic use)
  • Benzodioxoles
  • Chloride Channel Agonists
  • Cystic Fibrosis (complications, diagnosis, drug therapy)
  • Cystic Fibrosis Transmembrane Conductance Regulator (genetics)
  • Gastroesophageal Reflux (complications, diagnosis, drug therapy)
  • Humans
  • Indoles
  • Longitudinal Studies
  • Male
  • Mutation
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones

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