Inflammation in cystic fibrosis: An update
EA Roesch, DP Nichols, JF Chmiel - Pediatric pulmonology, 2018 - Wiley Online Library
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease
progression making it an active area of research and important therapeutic target. In this …
progression making it an active area of research and important therapeutic target. In this …
[PDF][PDF] The impact of highly effective modulator therapy on cystic fibrosis microbiology and inflammation
LJ Caverly, SA Riquelme, KB Hisert - Clinics in chest medicine, 2022 - Elsevier
Highly-effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …
Impact of elexacaftor–tezacaftor–ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis
S Sheikh, RD Britt Jr, NA Ryan‐Wenger… - Pediatric …, 2023 - Wiley Online Library
Background Cystic fibrosis (CF) is a multisystem disease with progressive deterioration.
Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were …
Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were …
Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis
HH Jarosz-Griffiths, T Scambler, CH Wong… - Elife, 2020 - elifesciences.org
Previously, we showed that serum and monocytes from patients with CF exhibit an
enhanced NLRP3-inflammasome signature with increased IL-18, IL-1β, caspase-1 activity …
enhanced NLRP3-inflammasome signature with increased IL-18, IL-1β, caspase-1 activity …
Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor
S Zhang, CL Shrestha, F Robledo-Avila… - European …, 2023 - Eur Respiratory Soc
Background Abnormal macrophage function caused by dysfunctional cystic fibrosis
transmembrane conductance regulator (CFTR) is a critical contributor to chronic airway …
transmembrane conductance regulator (CFTR) is a critical contributor to chronic airway …
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function
Despite the addition of cystic fibrosis transmembrane conductance regulator (CFTR)
modulators to the cystic fibrosis (CF) treatment regimen, patients with CF continue to suffer …
modulators to the cystic fibrosis (CF) treatment regimen, patients with CF continue to suffer …
Elexacaftor/tezacaftor/ivacaftor corrects monocyte microbicidal deficiency in cystic fibrosis
L Cavinato, FR Luly, V Pastore… - European …, 2023 - Eur Respiratory Soc
Background Cystic fibrosis (CF), which is caused by mutations in the CF transmembrane
conductance regulator (CFTR), is characterised by chronic bacterial lung infection and …
conductance regulator (CFTR), is characterised by chronic bacterial lung infection and …
Comparative effects of CFTR modulators on phagocytic, metabolic and inflammatory profiles of CF and nonCF macrophages
DS Aridgides, DL Mellinger, LL Gwilt, TH Hampton… - Scientific Reports, 2023 - nature.com
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 bacterial persistence in the lung. Whether CF macrophage dysfunction is related directly …
Airway inflammation and host responses in the era of CFTR modulators
K Keown, R Brown, DF Doherty, C Houston… - International journal of …, 2020 - mdpi.com
The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a
new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in …
new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in …
SARS-CoV-2 (COVID-19) and cystic fibrosis
BA Stanton, TH Hampton… - American Journal of …, 2020 - journals.physiology.org
Cystic fibrosis (CF) is a genetic disease caused by mutations in the CFTR gene. Although
viral respiratory tract infections are, in general, more severe in patients with CF compared …
viral respiratory tract infections are, in general, more severe in patients with CF compared …