Changes in sputum viscoelastic properties and airway inflammation in primary ciliary dyskinesia are comparable to cystic fibrosis on elexacaftor/tezacaftor/ivacaftor therapy
Abstract
ivacaftor (ETI) therapy. Methods: We measured and compared sputum rheology, inflammation markers and the proteome in 42 clinically stable adolescent and adult patients with PCD, 40 patients with CF with at least one F508del allele before and 3 months after initiation of ETI and 15 age-matched healthy controls. Results: Patients with PCD showed increased viscoelastic properties (G’ and G’’ P
Fields of Study & Subjects
Cystic Fibrosis Research Advances
Genetic and Kidney Cyst Diseases
Immunodeficiency and Autoimmune Disorders
Publication details
- Journal / source
- MDC Repository (Max-Delbrueck-Center for Molecular Medicine)
- Publication date
- 12 January 2026
- Document type
- Dataset