Academic Journal
Impact of elexacaftor/tezacaftor/ivacaftor on the presence of bacterial and fungal pathogens in the lower respiratory tract of children with cystic fibrosis.
| Τίτλος: | Impact of elexacaftor/tezacaftor/ivacaftor on the presence of bacterial and fungal pathogens in the lower respiratory tract of children with cystic fibrosis. |
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| Συγγραφείς: | Setnikar Kimovec G; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia., Seme K; Institute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Matos T; Institute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Cvitković Špik V; Institute of Microbiology and Immunology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia., Krivec U; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia; University of Ljubljana, Faculty of Medicine, Department of Paediatrics, Ljubljana, Slovenia., Praprotnik M; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia., Rodman Berlot J; Department of Paediatric Pulmonology, University Children's Hospital Ljubljana, University Medical Centre Ljubljana, Ljubljana, Slovenia; University of Ljubljana, Faculty of Medicine, Department of Paediatrics, Ljubljana, Slovenia. Electronic address: jasna.rodman@kclj.si. |
| Πηγή: | Respiratory medicine [Respir Med] 2026 Jun; Vol. 257, pp. 108846. Date of Electronic Publication: 2026 Apr 17. |
| Τύπος έκδοσης: | Journal Article; Observational Study |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: Elsevier Country of Publication: England NLM ID: 8908438 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-3064 (Electronic) Linking ISSN: 09546111 NLM ISO Abbreviation: Respir Med Subsets: MEDLINE |
| Imprint Name(s): | Publication: 2003- : Oxford : Elsevier Original Publication: London : Baillière Tindall, in association with the British Thoracic Society, [c1989- |
| Ιατρικοί όροι (MeSH): | Cystic Fibrosis*/drug therapy , Cystic Fibrosis*/microbiology , Cystic Fibrosis*/physiopathology , Indoles*/therapeutic use , Indoles*/pharmacology , Aminophenols*/therapeutic use , Aminophenols*/pharmacology , Quinolones*/therapeutic use , Quinolones*/pharmacology , Benzodioxoles*/therapeutic use , Benzodioxoles*/pharmacology , Pyridines*/therapeutic use , Pyrrolidines*/therapeutic use , Pyrroles*/therapeutic use , Pyrazoles*/therapeutic use, Sputum/microbiology ; Respiratory Tract Infections/microbiology ; Humans ; Child ; Male ; Female ; Prospective Studies ; Drug Combinations |
| Περίληψη: | Background: Highly effective CFTR modulator therapy, such as elexacaftor/tezacaftor/ivacaftor (ETI), has been linked to significant clinical improvements in people with cystic fibrosis (pwCF). However, its effect on the presence of respiratory pathogens in the lower respiratory tract of younger, mainly non-expectorant children with CF remains poorly understood, especially using lower airway sampling methods. We aimed to investigate changes in lower airway microbiology and clinical outcomes in children with CF aged 6-12 years before and after starting ETI. Methods: We conducted a prospective observational single-centre study including children with CF commencing ETI who had no prior exposure to CFTR modulators, inhaled antibiotics, or prophylactic antibiotics. Lower airway microbiology was assessed longitudinally using sputum or induced sputum (IS). Pathogen prevalence, sweat chloride concentration, pulmonary function, nutritional status, and rate of exacerbations were evaluated before and up to 12 months after ETI initiation. Results: Sixteen pwCF were included (median age 8.4 years, 69 % boys, 75% were F508del homozygous). None of the children was expectorant at any point during the study. Following ETI initiation, we observed decreased growth of methicillin-sensitive Staphylococcus aureus and Aspergillus fumigatus, reduced variability in fungal populations, and an increase in negative bacterial and fungal culture results. Conclusions: In this exploratory study of young, modulator-naïve children with CF, ETI initiation was associated with changes in the lower airway microbiology composition assessed by IS. These findings highlight potential shifts in lower airway microbiology and the importance of age-appropriate lower airway sampling in future paediatric studies. (Copyright © 2026 The Authors. Published by Elsevier Ltd.. All rights reserved.) |
| Competing Interests: | Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. |
| Contributed Indexing: | Keywords: Children; Cystic fibrosis; Elexacaftor/tezacafot/ivacaftor; Respiratory pathogens |
| Substance Nomenclature: | 0 (Indoles) 0 (Aminophenols) 0 (Quinolones) 0 (Drug Combinations) 0 (Benzodioxoles) 0 (Pyridines) 0 (Pyrrolidines) 0 (Pyrroles) 0 (Pyrazoles) 0 (tezacaftor, ivacaftor drug combination) RRN67GMB0V (elexacaftor) |
| Entry Date(s): | Date Created: 20260419 Date Completed: 20260715 Latest Revision: 20260715 |
| Update Code: | 20260715 |
| DOI: | 10.1016/j.rmed.2026.108846 |
| PMID: | 42001970 |
| Βάση Δεδομένων: | MEDLINE |
| ISSN: | 1532-3064 |
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| DOI: | 10.1016/j.rmed.2026.108846 |