The future of cystic fibrosis treatment: from disease mechanisms to novel therapeutic approaches

SY Graeber, MA Mall - The Lancet, 2023 - thelancet.com
With the 2019 breakthrough in the development of highly effective modulator therapy
providing unprecedented clinical benefits for over 90% of patients with cystic fibrosis who …

[HTML][HTML] Eradication treatment for Pseudomonas aeruginosa infection in adults with bronchiectasis: a systematic review and meta-analysis

M Conceição, M Shteinberg… - European …, 2024 - Eur Respiratory Soc
Introduction: Pseudomonas aeruginosa is the most commonly isolated pathogen in
bronchiectasis and is associated with worse outcomes. Eradication treatment is …

[HTML][HTML] Longitudinal microbial and molecular dynamics in the cystic fibrosis lung after Elexacaftor–Tezacaftor–Ivacaftor therapy

C Martin, DV Guzior, CT Gonzalez, M Okros… - Respiratory …, 2023 - Springer
Background Cystic fibrosis (CF) is a genetic disorder causing poor mucociliary clearance in
the airways and subsequent respiratory infection. The recently approved triple therapy …

Adjunctive systemic corticosteroids for pulmonary exacerbations of cystic fibrosis

OJ McElvaney, SL Heltshe, K Odem-Davis… - Annals of the …, 2024 - atsjournals.org
Rationale: Pulmonary exacerbations (PEx) remain the most common cause of morbidity,
recurrent hospitalization, and diminished survival in people with cystic fibrosis (PWCF) and …

[HTML][HTML] Ion mobility-tandem mass spectrometry of mucin-type O-glycans

L Bechtella, J Chunsheng, K Fentker, GR Ertürk… - Nature …, 2024 - nature.com
The dense O-glycosylation of mucins plays an important role in the defensive properties of
the mucus hydrogel. Aberrant glycosylation is often correlated with inflammation and …

[HTML][HTML] Mucus structure, viscoelastic properties, and composition in chronic respiratory diseases

M Abrami, A Biasin, F Tescione, D Tierno… - International Journal of …, 2024 - mdpi.com
The respiratory mucus, a viscoelastic gel, effectuates a primary line of the airway defense
when operated by the mucociliary clearance. In chronic respiratory diseases (CRDs), such …

Pharmacological improvement of CFTR function rescues airway epithelial homeostasis and host defense in children with cystic fibrosis

J Loske, M Völler, S Lukassen, M Stahl… - American Journal of …, 2024 - atsjournals.org
Rationale: Pharmacological improvement of CFTR function with elexacaftor/tezacaftor/
ivacaftor (ETI) provides unprecedented improvements in lung function and other clinical …

Long-term therapy with elexacaftor/tezacaftor/ivacaftor (ETI) in cystic fibrosis: improved clinical outcomes but infection and inflammation persist

MM Tunney, P Wark - European Respiratory Journal, 2023 - Eur Respiratory Soc
Extract In people with cystic fibrosis (CF), CF transmembrane conductance regulator (CFTR)
protein dysfunction in the lower airways results in dehydrated airway secretions and …

Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor

P Cazier, G Chassagnon, T Dhote… - European …, 2024 - Eur Respiratory Soc
Background This study sought to evaluate the impact of elexacaftor/tezacaftor/ivacaftor (ETI)
on lung structural abnormalities in adults with cystic fibrosis (awCF) with a specific focus on …

[HTML][HTML] Ceragenins exhibit bactericidal properties that are independent of the ionic strength in the environment mimicking cystic fibrosis sputum

K Skłodowski, Ł Suprewicz… - Frontiers in …, 2023 - frontiersin.org
The purpose of the work was to investigate the impact of sodium chloride (NaCl) on the
antimicrobial efficacy of ceragenins (CSAs) and antimicrobial peptides (AMPs) against …