Results 41 to 50 of about 2,385 (134)

hERG1 channels and potential therapeutics for long QT syndrome

The Journal of Physiology, EarlyView.
Abstract figure legend Prolonged QT results from hERG1 channel dysfunction. (A) Physiological anterograde trafficking of hERG1 channels to the plasma membrane, leading to a normal electrocardiogram. (B) Prolonged QT results from the presence of fewer hERG1 channels on the plasma membrane due to decreased anterograde trafficking or reduced function due ...
Elizabeth H. Schneider, Kelly A. Aromolaran, Michael C. Sanguinetti, Ademuyiwa S. Aromolaran   +3 more
wiley   +1 more source

Elexacaftor–Tezacaftor–Ivacaftor Therapy for Cystic Fibrosis Patients with The F508del/Unknown Genotype

Antibiotics, 2021
The new CFTR modulator combination, elexacaftor/tezacaftor/ivacaftor (Trikafta) was approved by the FDA in October 2019 for treatment of Cystic Fibrosis in patients 6 years of age or older who have at least one F508del mutation in one allele and a ...
Marika Comegna, Vito Terlizzi, Donatello Salvatore, Carmela Colangelo, Antonella Miriam Di Lullo, Immacolata Zollo, Giovanni Taccetti, Giuseppe Castaldo, Felice Amato   +8 more
doaj   +1 more source

Uncovering Cystic Fibrosis Carrier: Insights From a Heterozygous CFTR‐F508del Rabbit Model

International Forum of Allergy &Rhinology, Volume 16, Issue 8, Page 788-796, August 2026.
ABSTRACT Background Chronic rhinosinusitis (CRS) is a heterogeneous inflammatory disorder frequently associated with impaired mucociliary clearance and bacterial infection. Individuals carrying a single cystic fibrosis transmembrane conductance regulator (CFTR) mutation exhibit partial CFTR dysfunction and are increasingly recognized as being at risk ...
Do‐Yeon Cho, Alexis E. McFeely, Daniel Skinner, Shaoyan Zhang, Justin Hedvat, Weslee Ping‐ay, Daniel M. Beswick, Justin H. Turner, Bradford A. Woodworth, Jie Xu   +9 more
wiley   +1 more source

Successful Rapid Desensitization to Ceftazidime/Avibactam in a Patient With Cystic Fibrosis and Multidrug‐Resistant Pseudomonas aeruginosa Pneumonia: A Case Report

Clinical Case Reports, Volume 14, Issue 8, August 2026.
ABSTRACT Ceftazidime/avibactam (CAZ‐AVI) is an important therapeutic option for multidrug‐resistant (MDR) Pseudomonas aeruginosa infections; however, hypersensitivity reactions may preclude its use and create significant therapeutic challenges. We report a 21‐year‐old woman with cystic fibrosis and recurrent MDR P.
Zuoren Zhou, Jinqi Zhu, Ge Wu, Xi Kang, Qing Wang, Jie Liu, Yan Jiang   +6 more
wiley   +1 more source

Polymicrobial Extracellular Vesicles Reduce the Innate Immune Response of Human Cystic Fibrosis Bronchial Epithelial Cells

Journal of Extracellular Biology, Volume 5, Issue 8, August 2026.
ABSTRACT Chronic antibiotic‐resistant cystic fibrosis (CF) lung infections are the leading cause of death in adults with CF. Despite advances in highly effective modulator therapies, microbial communities persist in the CF lung. The pathogenesis of CF airway infections can be exacerbated by pathogens such as Pseudomonas aeruginosa, which communicates ...
Lily A. Charpentier, Roxanna L. Barnaby, Carolyn T. Roche, Byoung‐Kyu Cho, Prashant Kaushal, Young Ah Goo, Brad Vietje, Douglas J. Taatjes, Alix Ashare, Fabrice Jean‐Pierre, Bruce A. Stanton   +10 more
wiley   +1 more source

Obstructive Sleep Apnea: Epidemiology, Pathophysiology, Complications, Diagnosis, Management, and Emerging Fibrosis‐Linked Remodeling

MedComm, Volume 7, Issue 8, August 2026.
Obstructive sleep apnea (OSA) is characterized by recurrent upper‐airway collapse, which generates key nocturnal stressors including intermittent hypoxia, sleep fragmentation, intrathoracic pressure stress, and sympathetic activation. These physiological disturbances converge on shared biological mechanisms, including oxidative stress, inflammation ...
Nhi Ho Thi Thuy, Thị Hằng Giang Phan, Ali Hussein Eid, Paola Maccioccu, Stefania Camboni, Gavino Casu, Anna Maria Posadino, Roberta Giordo, Gianfranco Pintus   +8 more
wiley   +1 more source

The relationship between the pharmacogenetics and pharmacokinetics of CFTR modulators in a population of children with cystic fibrosis

Фармация и фармакология (Пятигорск)
The triple combination of elexacaftor / tezacaftor / ivacaftor is one of the key tools for targeted pharmacotherapy of cystic fibrosis. In the pediatric population, its use may be accompanied by a variable therapeutic response, which may be due to age ...
S. K. Zyryanov, E. I. Kondratyeva, E. K. Zhekaite, O. I. Butranova, P. O. Bochkov   +4 more
doaj   +1 more source

Utilization of Des‐Gamma‐Carboxy‐Prothrombin in Estimating Vitamin K Status in People With Cystic Fibrosis: A Single Center Retrospective Review

Pediatric Pulmonology, Volume 61, Issue 8, August 2026.
ABSTRACT Background People with cystic fibrosis (CF) are at increased risk of fat‐soluble vitamin (A, D, E, and K) deficiencies secondary to exocrine pancreatic insufficiency (EPI). Estimation of vitamin K deficiency in routine clinical practice is challenging, and no uniform consensus exists due to lack of an accurate clinical test for the evaluation ...
Senthilkumar Sankararaman, Terri Schindler, Aravind Thavamani, Catherine Pamer, Alison Marie Rodatt, Kimberly A. McBennett, Erica Roesch, Alex H. Gifford   +7 more
wiley   +1 more source

Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort

ERJ Open Research
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor.
Christian Leo-Hansen, Daniel Faurholt-Jepsen, Tavs Qvist, Christine Højte, Bibi U. Nielsen, Thomas Bryrup, Esben H. Henriksen, Terese Katzenstein, Marianne Skov, Inger H.M. Mathiesen, Majbritt Jeppesen, Søren Jensen-Fangel, Hanne V. Olesen, Frederik Fouirnaies Buchvald, Kim Gjerum Nielsen, Espen Jimenez-Solem, Christian Ritz, Tacjana Pressler, Mette F. Olsen, the TransformCF Study Group   +19 more
doaj   +1 more source

Triple combination cystic fibrosis transmembrane receptor modulator effects on glycaemia and insulin kinetics in cystic fibrosis with and without diabetes

Internal Medicine Journal, Volume 56, Issue 8, Page 1356-1360, August 2026.
Abstract Background Greater insight into the effects of cystic fibrosis (CF) transmembrane modulators such as elexacaftor‐tezacaftor‐ivacaftor (ETI) on glucose metabolism can support a more dynamic and individualised approach to CF‐related dysglycaemia.
Yi W. Chen, Rachel Prasad, Peter G. Middleton, D. Jane Holmes‐Walker   +3 more
wiley   +1 more source