Results 41 to 50 of about 1,208,918 (256)

Enhanced Expression of Human Epididymis Protein 4 (HE4) Reflecting Pro-Inflammatory Status Is Regulated by CFTR in Cystic Fibrosis Bronchial Epithelial Cells

Frontiers in Pharmacology, 2021
Decreased human epididymis protein 4 (HE4) plasma levels were reported in cystic fibrosis (CF) patients under CFTR potentiator ivacaftor therapy, which inversely correlated with lung function improvement.
Zsolt Bene, Zsolt Bene, Zsolt Bene, Zsolt Fejes, Zsolt Fejes, Tibor Gabor Szanto, Ferenc Fenyvesi, Judit Váradi, Luka A. Clarke, Gyorgy Panyi, Milan Macek, Margarida D. Amaral, István Balogh, István Balogh, Béla Nagy, Béla Nagy   +15 more
doaj   +1 more source

Cystic fibrosis patients of minority race and ethnicity less likely eligible for CFTR modulators based on CFTR genotype

Pediatric Pulmonology, 2021
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are disease‐modifying medications for cystic fibrosis (CF) and are shown to be efficacious for only specific CFTR mutations.
M. McGarry, S. McColley
semanticscholar   +1 more source

Nutritional Status and Circulating Levels of Fat-Soluble Vitamins in Cystic Fibrosis Patients: A Cohort Study and Evaluation of the Effect of CFTR Modulators

Children, 2023
Background: Improved therapy in CF has led to an overall improvement in nutritional status. The objectives of our study are: to cross-sectionally assess nutritional status and serum levels of fat-soluble vitamins; to retrospectively evaluate the efficacy
M. Francalanci, V. Terlizzi, C. Fevola, Giulia Di Rosa, V. Pierattini, E. Roselli, P. Bonomi, M. Cavicchi, V. Galici, A. Neri, C. Bianchimani, S. Campana, D. Dolce, N. Ravenni, E. Camera, T. Orioli, G. Taccetti   +16 more
semanticscholar   +1 more source

The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis

International Journal of Molecular Sciences, 2022
The advent of Cystic fibrosis transmembrane receptor (CFTR) modulators in 2012 was a critical event in the history of cystic fibrosis (CF) treatment. Unlike traditional therapies that target downstream effects of CFTR dysfunction, CFTR modulators aim to ...
Caitlyn Harvey, S. Weldon, S. Elborn, D. Downey, C. Taggart   +4 more
semanticscholar   +1 more source

Prospectively evaluating maternal and fetal outcomes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design

BMJ Open Respiratory Research, 2022
Introduction Therapeutic advances have markedly increased life expectancy for those with cystic fibrosis (CF), resulting in a median predicted survival over 50 years. Consequently, people with CF (pwCF) are living through their reproductive years and the
R. Jain, A. Magaret, Phuong T. Vu, J. Vandalfsen, A. Keller, A. Wilson, M. Putman, N. Mayer-Hamblett, C. Esther, J. Taylor-Cousar   +9 more
semanticscholar   +1 more source

Characterization of Defects in Ion Transport and Tissue Development in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR)-Knockout Rats [PDF]

, 2014
Animal models for cystic fibrosis (CF) have contributed significantly to our understanding of disease pathogenesis. Here we describe development and characterization of the first cystic fibrosis rat, in which the cystic fibrosis transmembrane conductance
Bedwell, David M., Birket, Susan E., Cadillac, Joan M., Chambers, Andre, Chu, Kengyeh K., Cui, Xiaoxia, Du, Ming, Fanucchi, Michelle V., Fortenberry, James A., Gamber, Kevin, Hong, Jeong, Ji, Diana, Reid, Lara, Rowe, Steven M., Sorscher, Eric J., Tang, Li Ping, Tearney, Guillermo, Tuggle, Katherine L., Warren, Joe   +18 more
core   +21 more sources

Current prices versus minimum costs of production for CFTR modulators

medRxiv, 2022
Background: While the clinical benefits of CFTR modulators are clear, their high prices render them inaccessible outside of a select few countries. Despite this, there is currently limited evidence regarding access to these transformative therapies ...
Jonathan Guo, Junzheng Wang, Jingchun Zhang, J. Fortunak, A. Hill   +4 more
semanticscholar   +1 more source

Chloride channels regulate differentiation and barrier functions of the mammalian airway. [PDF]

, 2020
The conducting airway forms a protective mucosal barrier and is the primary target of airway disorders. The molecular events required for the formation and function of the airway mucosal barrier, as well as the mechanisms by which barrier dysfunction ...
Caplan, Michael J, Chen, Yuzhang, Darmanis, Spyros, He, Mu, Jan, Lily Yeh, Jan, Yuh Nung, Le, Daniel D, Li, Ke-Xin, Neff, Norma, Padovano, Valeria, Sinclair, Adriane W, Sit, Rene, Tan, Michelle, Wu, Bing, Ye, Wenlei   +14 more
core   +1 more source

Mechanisms of endothelial cell dysfunction in cystic fibrosis [PDF]

, 2017
Although cystic fibrosis (CF) patients exhibit signs of endothelial perturbation, the functions of the cystic fibrosis conductance regulator (CFTR) in vascular endothelial cells (EC) are poorly defined.
Anile, Marco, Cianci, Eleonora, Del Porto, Paola, Dell'Elba, Giuseppe, Di Silvestre, Sara, Evangelista, Virgilio, Guarnieri, Simone, Lanuti, Paola, Marchisio, Marco, Mari, Veronica C., Mariggiã², Maria A., Moretti, Paolo, Mucilli, Felice, Pandolfi, Assunta, Patruno, Sara, Piccoli, Antonio, Plebani, Roberto, Prioletta, Marco, Recchiuti, Antonio, Romano, Mario, Sacchetti, Silvio, Totani, Licia, Venuta, Federico   +22 more
core   +1 more source

Cognitive Outcome After Deep Brain Stimulation for Refractory Obsessive–Compulsive Disorder: A Systematic Review

Neuromodulation: Technology at the Neural Interface, EarlyView., 2021
Abstract Introduction Deep brain stimulation (DBS) is an effective treatment for refractory obsessive–compulsive disorder (OCD). Neuropsychological assessment contributes to DBS treatment in several ways: it monitors the cognitive safety of the treatment, identifies beneficial or detrimental cognitive side effects and it could aid to explain ...
Tim A. M. Bouwens van der Vlis, Annelien Duits, Mégan M. G. H. van de Veerdonk, Anne E. P. Mulders, Koen R. J. Schruers, Yasin Temel, Linda Ackermans, Albert F. G. Leentjens   +7 more
wiley   +1 more source