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.
|
Authors | Sufyan Shakir, Carlos Echevarria, Simon Doe, Malcolm Brodlie, Christopher Ward, Stephen J Bourke |
Journal | Journal 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 |
PMID | 35718668
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
|
Copyright | Copyright © 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
|