Results 11 to 20 of about 7,856 (243)

Epithelial responses to CFTR modulators are improved by inflammatory cytokines and impaired by antiinflammatory drugs [PDF]

JCI Insight
Cystic fibrosis (CF) is a genetic disorder that disrupts CF transmembrane conductance regulator (CFTR) anion channels and impairs airway host defenses. Airway inflammation is ubiquitous in CF, and suppressing it has generally been considered to improve ...
Tayyab Rehman, Alejandro A. Pezzulo, Andrew L. Thurman, Rachel L. Zemans, Michael J. Welsh   +4 more
doaj   +4 more sources

Medication adherence to CFTR modulators in patients with cystic fibrosis: a systematic review [PDF]

European Respiratory Review
Background In the last decade, a fundamental shift in the treatment of cystic fibrosis (CF) took place due to the introduction of CF transmembrane conductance regulator (CFTR) modulators.
Carina M.E. Hansen, Anna J. Breukelman, Patricia M.L.A. van den Bemt, Annelies M. Zwitserloot, Liset van Dijk, Job F.M. van Boven   +5 more
doaj   +5 more sources

Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators

Pediatric Pulmonology
AbstractIntroductionCystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non‐eligible patients for
Deniz Dogru, Sevgi Pekcan, Güzin Cinel   +2 more
exaly   +9 more sources

Mutual Effects of Single and Combined CFTR Modulators and Bacterial Infection in Cystic Fibrosis [PDF]

Microbiology Spectrum, 2023
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve clinical outcomes with varied efficacies in patients with CF. However, the mutual effects of CFTR modulators and bacterial adaptation, together with antibiotic regimens, can ...
Cristina Cigana, Ruggero Giannella, Alice Colavolpe, Beatriz Alcalá-Franco, Giulia Mancini, Francesca Colombi, Chiara Bigogno, Ulla Bastrup, Giovanni Bertoni, Alessandra Bragonzi   +9 more
doaj   +3 more sources

Comparative effects of CFTR modulators on phagocytic, metabolic and inflammatory profiles of CF and nonCF macrophages

Scientific Reports, 2023
Macrophage dysfunction has been well-described in Cystic Fibrosis (CF) and may contribute to bacterial persistence in the lung. Whether CF macrophage dysfunction is related directly to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in ...
Daniel S. Aridgides, Diane L. Mellinger, Lorraine L. Gwilt, Thomas H. Hampton, Dallas L. Mould, Deborah A. Hogan, Alix Ashare   +6 more
doaj   +3 more sources

Impact of CFTR modulators on exercise capacity in adolescents with cystic fibrosis [PDF]

ERJ Open Research
Background Exercise capacity is an independent predictor of clinical worsening in cystic fibrosis (CF). There is limited evidence of the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on exercise capacity in children with
Molla Imaduddin Ahmed, Naomi Dayman, Natalie Blyth, Joe Madge, Erol Gaillard   +4 more
doaj   +3 more sources

Effect of CFTR Modulators on Oxidative Stress and Autophagy in Non-CFTR-Expressing Cells. [PDF]

Int J Mol Sci
The triple combination therapy for cystic fibrosis (CF), including elexacaftor, tezacaftor and ivacaftor (ETI or Trikafta), has been shown to improve lung function and reduce pulmonary exacerbations, thereby enhancing the quality of life for most CF patients.
Scialò F, Cernera G, Polise L, Castaldo G, Amato F, Villella VR.   +5 more
europepmc   +5 more sources

Role of inhaled antibiotics in the era of highly effective CFTR modulators [PDF]

European Respiratory Review, 2023
Recurrent and chronic bacterial infections are common in people with cystic fibrosis (CF) and contribute to lung function decline. Antibiotics are the mainstay in the treatment of exacerbations and chronic bacterial infection in CF.
J. Elborn, F. Blasi, P. Burgel, D. Peckham   +3 more
semanticscholar   +2 more sources

CF Fungal Disease in the Age of CFTR Modulators [PDF]

Mycopathologia, 2021
AbstractFungi are increasingly recognised to have a significant role in the progression of lung disease in Cystic fibrosis with Aspergillus fumigatus the most common fungus isolated during respiratory sampling. The emergence of novel CFTR modulators has, however, significantly changed the outlook of disease progression in CF.
Amelia Bercusson, George Jarvis, Anand Shah   +2 more
openaire   +5 more sources

CFTR Modulators: From Mechanism to Targeted Therapeutics [PDF]

, 2022
People with cystic fibrosis (CF) suffer from a multi-organ disorder caused by loss-of-function variants in the gene encoding the epithelial anion channel cystic fibrosis transmembrane conductance regulator (CFTR). Tremendous progress has been made in both basic and clinical sciences over the past three decades since the identification of the CFTR gene.
Sheppard, David N, Yeh, Han-I, Sutcliffe, Katy, Hwang, Tzyh-Chang   +3 more
openaire   +4 more sources