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Effects of Elexacaftor-Tezacaftor-Ivacaftor on Nasal and Sinus Symptoms in Children With Cystic Fibrosis.
Pediatr Pulmonol
January 2025
Hôpital Femme Mère Enfant, Hospices Civils de Lyon, 59 Boulevard Pinel, Lyon, France.
Background: New CFTR Modulator triple therapy Elexacaftor-Ivacaftor-Tezacaftor (ETI) prove efficacy in pulmonary outcomes. However, its impact on nasal sinus symptoms in children has not been specifically studied. The aim of this study is to evaluate the impact of this therapy on nasal sinus symptomatology in children aged 6-12 years.
View Article and Find Full Text PDFIntroduction: Elexacaftor/tezacaftor/ivacaftor (ETI) has shown significant improvements in pulmonary and nutritional status in persons with cystic fibrosis (pwCF). Less is known about the extrapulmonary impact of ETI and effects on airway microbiology, lung clearance index (LCI) and fraction of exhaled nitric oxide (FeNO).
Methods: A multicentre prospective observational trial, including 79 pwCF ≥ 18 years eligible for ETI.
VX-770, C-A1, and Increased Intracellular cAMP Have Distinct Acute Impacts upon CFTR Activity.
Int J Mol Sci
January 2025
Department of Pediatrics, National Jewish Health, Denver, CO 80206, USA.
The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel that is dysfunctional in individuals with cystic fibrosis (CF). The permeability of CFTR can be experimentally manipulated though different mechanisms, including activation via inducing the phosphorylation of residues in the regulatory domain as well as altering the gating/open probability of the channel. Phosphorylation/activation of the channel is achieved by exposure to compounds that increase intracellular cAMP, with forskolin and IBMX commonly used for this purpose.
View Article and Find Full Text PDFShort-term modification of breathprint by Elexacaftor/Tezacaftor/Ivacaftor in a paediatric cohort.
J Cyst Fibros
January 2025
INSERM U1151, Institut Necker Enfants Malades, Paris, France; Hôpital Necker Enfants Malades, Centre de Référence Maladies Rares Mucoviscidose et Maladies apparentées, Paris, France; Université Paris-Cité, Paris, France; European Reference Network-Lung. Frankfurt, Germany. Electronic address:
Background: The triple combination Elexacaftor/Tezacaftor/Ivacaftor (ETI) translates into major respiratory improvements in adults; yet current clinical endpoints may prove insufficiently sensitive in young children. We hypothesised that ETI rapidly modifies the lungs' metabolism, resulting in changes in breath composition.
Methods: Eleven children with CF were enrolled in a longitudinal pilot study at the paediatric Necker hospital.
Secondhand vape exposure regulation of CFTR and immune function in cystic fibrosis.
Am J Physiol Lung Cell Mol Physiol
January 2025
Division of Pulmonology, Asthma, Cystic Fibrosis, and Sleep, Emory University School of Medicine, Atlanta, GA, USA.
Secondhand smoke exposure (SHSe) is a public health threat for people with cystic fibrosis (CF) and other lung diseases. Primary smoking reduces CFTR channel function, the causative defect in CF. We reported that SHSe worsens respiratory and nutritional outcomes in CF by disrupting immune responses and metabolic signaling.
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