Results 51 to 60 of about 1,105,613 (399)

Diagnostic agreement among experts assessing adults presenting with possible cystic fibrosis: need for improvement and implications for patient care

ERJ Open Research, 2022
Background Increasing awareness of milder presentations of cystic fibrosis (CF) and greater interest in non-CF bronchiectasis are likely to lead to more CF screening by respiratory clinicians. As a result, adults who may not strictly fulfil CF diagnostic
Alessandro N. Franciosi, April Tanzler, Jodi Goodwin, Pearce G. Wilcox, George M. Solomon, Albert Faro, Noel G. McElvaney, Damian G. Downey, Bradley S. Quon   +8 more
doaj   +1 more source

Cystic Fibrosis and Pseudomonas aeruginosa: the Host-Microbe Interface

Clinical Microbiology Reviews, 2019
In human pathophysiology, the clash between microbial infection and host immunity contributes to multiple diseases. Cystic fibrosis (CF) is a classical example of this phenomenon, wherein a dysfunctional, hyperinflammatory immune response combined with ...
Sankalp Malhotra, D. Hayes, D. Wozniak
semanticscholar   +1 more source

Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation [PDF]

, 2014
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures
Amin R, Andrea Venerando, Angela Sepe, Anil Mehta, Anna Bossi, Anna Zolin, Antonella Tosco, Boyle MP BS, Carlo A Leone, Daniela De Stefano, Donaldson S., Fabiola De Gregorio, Fischer H, Gianni Bona, Gibson LE, Giuseppe De Rosa, Guido Kroemer, Illek B, Jayaraman S, Kerem B, Kerem Eithan HMO, Laura Salvadori, Legssyer R, Lorenzo A Pinna, Luigi Maiuri, Lukacs G, Maria C Maiuri, Massimo Pettoello-Mantovani, Masvidal L, Pharmaceuticals Cylene, Pharmaceuticals Cylene, Rosa Grassia, Speranza Esposito, Standardization of Spirometry, Stefano Guido, Stern M, Valeria R Villella, Valeria Raia, van Doorninck JH, Villella VR   +39 more
core   +3 more sources

Acceptance and well-being in adolescents and young adults with cystic fibrosis: a prospective study [PDF]

, 2011
Objective To prospectively investigate the role of acceptance in well-being in adolescents and young adults with cystic fibrosis (CF). Method A total of 40 adolescents and young adults with CF (ages 14-22 years) completed questionnaires assessing ...
Casier, Annabelle, Crombez, Geert, De Baets, Frans, Goubert, Liesbet, Huse, Danielle, Matthys, Dirk, Theunis, Marleen   +6 more
core   +2 more sources

Sensing of Chloride Ions in Sweat by Means of Printed Extended‐Gate Organic Electrochemical Transistors

Advanced Functional Materials, EarlyView.
In this work, a platform based on an Extended‐Gate OECT is successfully developed and tailored for the detection of physiologically relevant chloride ion concentrations in artificial eccrine sweat. Leveraging the extended‐gate architecture, a high‐performance OECT based on inkjet‐printed pg2T‐TT is coupled with an inkjet‐printed Ag/AgCl electrode ...
Cristiano Bortolotti, Andrea Ottomaniello, Paola Parlanti, Virgilio Mattoli, Dario Natali, Andrea Aliverti, Adrica Kyndiah, Mario Caironi   +7 more
wiley   +1 more source

Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis [PDF]

, 2015
Background: Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in ...
Smyth, Alan R.
core   +4 more sources

Recent Applications of Mesoporous Silica Nanoparticles in Gene Therapy

Advanced Healthcare Materials, EarlyView.
The review summarizes the synthesis of mesoporous silica nanoparticles (MSNs) with modifiable surface properties, functionalization strategies, mechanism of therapeutic payload release, and current applications in gene therapy, focusing on their capabilities in the targeted delivery of therapeutic nucleic acids, CRISPR‐Cas systems, and other genetic ...
Tamanna Binte Huq, Punnya Anil Kumar Jeeja, Sudip Kumar Dam, Sabya Sachi Das, Juan L. Vivero‐Escoto   +4 more
wiley   +1 more source

Elexacaftor/Tezacaftor/Ivacaftor Efficacy in a Cohort of Italian Patients with CFTR Rare Mutations

International Journal of Translational Medicine
Background: Cystic Fibrosis is an inherited disorder caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene, encoding a chloride and bicarbonate channel widely expressed in epithelia.
Francesca Lucca, Sonia Volpi, Mirco Ros, Benedetta Fabrizzi, Ilaria Meneghelli, Marica Bordicchia, Francesca Buniotto, Alessia Lancini, Cecilia Brignole, Francesca Pauro, Valentino Bezzerri, Marco Cipolli   +11 more
doaj   +1 more source

CFTR trafficking mutations disrupt cotranslational protein folding by targeting biosynthetic intermediates

Nature Communications, 2020
Cystic fibrosis (CF) is a lethal genetic disease that is primarily caused by misfolding of the cystic fibrosis transmembrane conductance regulator (CFTR). Here authors show that disease-causing mutations located within the first nucleotide binding domain
Hideki Shishido, Jae Seok Yoon, Zhongying Yang, William R. Skach   +3 more
doaj   +1 more source

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

Science Translational Medicine, 2019
Increased airway mucus, occurs before infection or structural lung disease in early cystic fibrosis and promotes inflammation. Early changes in cystic fibrosis Cystic fibrosis (CF) is a genetic disorder caused by mutations in the CFTR gene.
C. Esther, M. Muhlebach, C. Ehre, D. Hill, M. Wolfgang, M. Kesimer, K. Ramsey, K. Ramsey, M. Markovetz, Ian C. Garbarine, M. Forest, Ian Seim, Bryan T Zorn, Cameron B. Morrison, M. Delion, William R. Thelin, D. Villalon, J. Sabater, L. Turkovic, S. Ranganathan, S. Stick, S. Stick, R. Boucher   +22 more
semanticscholar   +1 more source