[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) …

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 …

Use of elexacaftor/tezacaftor/ivacaftor among cystic fibrosis lung transplant recipients

KJ Ramos, JS Guimbellot, M Valapour, LE Bartlett… - Journal of Cystic …, 2022 - Elsevier
Background Cystic fibrosis (CF) lung transplant (LT) recipients may warrant treatment with
elexacaftor/tezacaftor/ivacaftor (ETI) to improve extrapulmonary manifestations of CF. Our …

[PDF][PDF] Update on innate and adaptive immunity in cystic fibrosis

EM Bruscia, TL Bonfield - Clinics in Chest Medicine, 2022 - Elsevier
Cystic fibrosis (CF) pathophysiology is hallmarked by excessive inflammation and the
inability to efficiently resolve lung infections, contributing to morbidly and eventually the …

Revisiting the role of leukocytes in cystic fibrosis

M Averna, P Melotti, C Sorio - Cells, 2021 - mdpi.com
Cystic fibrosis in characterized by pulmonary bacterial colonization and hyperinflammation.
Lymphocytes, monocytes/macrophages, neutrophils, and dendritic cells of patients with CF …

CFTR modulator therapy enhances peripheral blood monocyte contributions to immune responses in people with cystic fibrosis

KB Hisert, TP Birkland, KQ Schoenfelt… - Frontiers in …, 2020 - frontiersin.org
Background CFTR modulators decrease some etiologies of CF airway inflammation;
however, data indicate that non-resolving airway infection and inflammation persist in …

Anti-inflammatory influences of cystic fibrosis transmembrane conductance regulator drugs on lung inflammation in cystic fibrosis

KH Harwood, RM McQuade, A Jarnicki… - International Journal of …, 2021 - mdpi.com
Cystic fibrosis (CF) is caused by a defect in the cystic fibrosis transmembrane conductance
regulator protein (CFTR) which instigates a myriad of respiratory complications including …

[HTML][HTML] Ivacaftor therapy post myocardial infarction augments systemic inflammation and evokes contrasting effects with respect to tissue inflammation in brain and …

L Vanherle, F Matthes, FE Uhl, A Meissner - Biomedicine & …, 2023 - Elsevier
Acquired cystic fibrosis transmembrane regulator (CFTR) dysfunctions have been
associated with several conditions, including myocardial infarction (MI). Here, CFTR is …

Ivacaftor inhibits glioblastoma stem cell maintenance and tumor progression

K Liu, J Pu, Z Nie, Y Shi, L Jiang, Q Wu… - Frontiers in Cell and …, 2021 - frontiersin.org
Glioblastoma (GBM) is the most common and malignant primary brain tumor. Glioblastoma
stem cells (GSCs) not only initiate and sustain uncontrolled cell proliferation but also …

Cystic fibrosis in the era of highly effective CFTR modulators

CJ Britto, JL Taylor-Cousar - Clinics in Chest Medicine, 2022 - chestmed.theclinics.com
communities with associated opportunities for developing novel antimicrobial therapies,
evolving knowledge regarding the surveillance and management of long-term endocrine …