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 …

[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) …

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 …

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 …

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 …

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function

S Zhang, CL Shrestha, BT Kopp - Scientific reports, 2018 - nature.com
Despite the addition of cystic fibrosis transmembrane conductance regulator (CFTR)
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 …

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 …

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 …

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 …