Modulator-refractory cystic fibrosis: Defining the scope and challenges of an emerging at-risk population
L Somerville, L Borish, I Noth… - Therapeutic Advances in …, 2024 - journals.sagepub.com
Cystic fibrosis (CF) causes life-shortening respiratory and systemic disease due to
dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride …
dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride …
Nasal epithelium transcriptomics predict clinical response to Elexacaftor/Tezacaftor/Ivacaftor
Elexacaftor/tezacaftor/ivacaftor (ETI) has had a substantial positive impact for people living
with cystic fibrosis (pwCF). However, there can be substantial variability in efficacy, and we …
with cystic fibrosis (pwCF). However, there can be substantial variability in efficacy, and we …
[HTML][HTML] Allergic Bronchopulmonary Aspergillosis (ABPA) in the Era of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators
P Chatterjee, CT Moss, S Omar, E Dhillon… - Journal of Fungi, 2024 - mdpi.com
Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disease caused by
Aspergillus fumigatus (Af), prevalent in persons with cystic fibrosis (CF) or asthma. In ABPA …
Aspergillus fumigatus (Af), prevalent in persons with cystic fibrosis (CF) or asthma. In ABPA …
How many functioning ciliated airway epithelial cells are necessary for effective mucociliary clearance?
RM Urbantat, MA Mall - European Respiratory Journal, 2024 - publications.ersnet.org
Extract Primary ciliary dyskinesia (PCD), the second most common genetic muco-obstructive
lung disease, is caused by mutations in over 50 different genes that encode proteins that are …
lung disease, is caused by mutations in over 50 different genes that encode proteins that are …
Gene expression responses of CF airway epithelial cells exposed to elexacaftor/tezacaftor/ivacaftor suggest benefits beyond improved CFTR channel function
TH Hampton, R Barnaby, C Roche… - … of Physiology-Lung …, 2024 - journals.physiology.org
The combination of elexacaftor/tezacaftor/ivacaftor (ETI, Trikafta) reverses the primary defect
in cystic fibrosis (CF) by improving CFTR-mediated Cl− and HCO3− secretion by airway …
in cystic fibrosis (CF) by improving CFTR-mediated Cl− and HCO3− secretion by airway …
Anti-inflammatory effects of elexacaftor/tezacaftor/ivacaftor in adults with cystic fibrosis heterozygous for F508del
HH Jarosz-Griffiths, L Gillgrass, LR Caley, G Spoletini… - Plos one, 2024 - journals.plos.org
Inflammation is a key driver in the pathogenesis of cystic fibrosis (CF). We assessed the
effectiveness of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on downregulating systemic …
effectiveness of elexacaftor/tezacaftor/ivacaftor (ETI) therapy on downregulating systemic …
Gene expression profile of intestinal organoids from people with cystic fibrosis upon exposure to elexacaftor/tezacaftor/ivacaftor
O Cinek, E Furstova, S Novotna, K Hubackova… - Journal of Cystic …, 2024 - Elsevier
The forskolin-induced swelling assay (FIS) in patient-derived intestinal organoids (PDIOs),
used to determine in vitro responsiveness to elexacaftor/tezacaftor/ivacaftor (ETI), showed …
used to determine in vitro responsiveness to elexacaftor/tezacaftor/ivacaftor (ETI), showed …
[HTML][HTML] Sex Differences in Sleep Profiles and the Effect of Elexacaftor/Tezacaftor/Ivacaftor on Sleep Quality in Adult People with Cystic Fibrosis: A Prospective …
S Dietz-Terjung, S Straßburg, T Schulte, P Dietz… - Diagnostics, 2024 - mdpi.com
Background/Objectives: Recent studies indicate that sleep and sleep disorders differ
between men and women, but corresponding data in people with chronic lung diseases are …
between men and women, but corresponding data in people with chronic lung diseases are …
[HTML][HTML] Elexacaftor/tezacaftor/ivacaftor improves nasal nitric oxide in patients with cystic fibrosis
CO Pioch, N Ziegahn, C Allomba, LM Busack… - Journal of Cystic …, 2024 - Elsevier
Background In health, nitric oxide (NO) shows high concentrations in the upper airways,
while nasal NO (nNO) is significantly lower in patients with sinonasal inflammation, such as …
while nasal NO (nNO) is significantly lower in patients with sinonasal inflammation, such as …
Depths of Dysfunctional Epithelial and Immune Crosstalk in Cystic Fibrosis Revealed
LM Yonker, TB Kinane - … Journal of Respiratory and Critical Care …, 2024 - atsjournals.org
Highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulatory
therapy has revolutionized the lives of people with cystic fibrosis (CF) with at least one …
therapy has revolutionized the lives of people with cystic fibrosis (CF) with at least one …