scholarly journals 406: Impact of highly-effective CFTR modulation on the microbial environment in cystic fibrosis

2021 ◽  
Vol 20 ◽  
pp. S191-S192
Author(s):  
G. Hong ◽  
C. Lipscombe ◽  
S. Daniel ◽  
A. O’Dea ◽  
K. Bittinger ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Highly Effective ◽  
Microbial Environment

Effect of highly effective modulator therapy on quality of life in adults with cystic fibrosis

2020 ◽  
Author(s):  
Emily DiMango ◽  
Daniel B. Spielman ◽  
Jonathan Overdevest ◽  
Claire Keating ◽  
Sarah Fracasso Francis ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Highly Effective

scholarly journals Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis

2020 ◽  
Author(s):  
Emily DiMango ◽  
Jonathan Overdevest ◽  
Claire Keating ◽  
Sarah Fracasso Francis ◽  
David Dansky ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Highly Effective ◽  
Sinonasal Symptoms

scholarly journals 94: Assessing symptom burden and quality of life in patients with cystic fibrosis in the era of highly effective modulator therapy

2021 ◽  
Vol 20 ◽  
pp. S47-S48
Author(s):  
A. Perez ◽  
K. Bischoff ◽  
D. Zhang ◽  
D. Dawson ◽  
M. Israni ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Symptom Burden ◽  
Highly Effective

scholarly journals Microbial interaction: Prevotella spp. reduce P.aeruginosa induced inflammation in Cystic Fibrosis bronchial epithelial cells

2020 ◽  
Author(s):  
Anne Bertelsen ◽  
Stuart J Elborn ◽  
Bettina Schock
Keyword(s):  
Cystic Fibrosis ◽  
Epithelial Cells ◽  
Inflammatory Response ◽  
Bacterial Species ◽  
Airway Epithelial Cells ◽  
Lung Damage ◽  
Airway Epithelial ◽  
Lung Function Decline ◽  
Mapk Signalling ◽  
Microbial Environment

Abstract Background: In Cystic Fibrosis (CF) airways, mutations in the Cystic Fibrosis Transmembrane Regulator (CFTR) lead to dehydrated, thick mucus which promotes the establishment of persistent polymicrobial infections and drives chronic airways inflammation. This also predisposes the airways to further infections, a vicious, self-perpetuating cycle causing lung damage and progressive lung function decline. The airways are a poly-microbial environment, containing both aerobic and anaerobic bacterial species. Pseudomonas aeruginosa (P.aeruginosa) infections contribute to the excessive inflammatory response in CF, but the role of anaerobic Prevotella spp., frequently found in CF airways, is not known.Materials: We assessed innate immune signalling in CF airway epithelial cells in response to clinical strains of P.histicola, P.nigresens and P.aeruginosa. CFBE41o- cells were infected with P.aeruginosa (MOI 100, 2h) followed by infection with P.histicola or P.nigrescens (MOI 100, 2h). Cells were incubated under anaerobic conditions for the duration of the experiments.Results: Our study shows that P.histicola and P.nigresens can reduce the growth of P.aeruginosa and dampen the inflammatory response in airway epithelial cells. We specifically illustrate that the presence of Prevotella spp. reduces Toll-like-receptor (TLR)-4, MAPK, NF-kB(p65) signalling and cytokine release (Interleukin (IL)-6, IL-8) in mixed infections. Conclusion: Our work, for the first time, strongly indicates a relationship between P. aeruginosa and anaerobe Prevotella spp. The observed modified NF-kB and MAPK signalling provides some mechanisms of this interaction that could offer a novel therapeutic approach to combat chronic P.aeruginosa infection in people with CF.

Current evidence on the effect of highly effective CFTR modulation on interleukin-8 in cystic fibrosis

2021 ◽  
Author(s):  
Michael Williamson ◽  
Michelle Casey ◽  
Claudie Gabillard-Lefort ◽  
Aram Alharbi ◽  
Yu Qing Jolene Teo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Interleukin 8 ◽  
Current Evidence ◽  
Highly Effective

scholarly journals P279 Withdrawing existing chronic therapies in people with cystic fibrosis who benefit from highly effective CFTR modulator drugs

2019 ◽  
Vol 18 ◽  
pp. S136 ◽  
Author(s):  
A. Gifford ◽  
K. Pearson ◽  
E. Aliaj ◽  
S. Heltshe ◽  
C. Goss ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Highly Effective
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