Results 31 to 40 of about 975,042 (316)

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

Mechanisms of parasite‐mediated disruption of brain vessels

FEBS Letters, EarlyView.
Parasites can affect the blood vessels of the brain, often causing serious neurological problems. This review explains how different parasites interact with and disrupt these vessels, what this means for brain health, and why these processes matter. Understanding these mechanisms may help us develop better ways to prevent or treat brain infections in ...
Leonor Loira, Sílvia Arroz‐Madeira, Cláudio A. Franco, Sara Silva Pereira   +3 more
wiley   +1 more source

Validating the Data Completeness and Accuracy of the Canadian Cystic Fibrosis Registry

Canadian Respiratory Journal
Conclusion: Our validation of the CCFR demonstrated high accuracy for clinical and demographic variables used in clinical research.
Ranjani Somayaji, Stephanie Y. Cheng, Noma Abdulrahem, Sanja Stanojevic, Paul Eckford, Bradley S. Quon, Elizabeth Cromwell, Albert Faro, Christopher H. Goss, Anne L. Stephenson   +9 more
doaj   +1 more source

Antibiofilm activity of a chionodracine‐derived peptide by NMR‐based metabolomics of cell‐free supernatant of Acinetobacter baumannii clinical strains

FEBS Open Bio, EarlyView.
KHS‐Cnd peptide is able to impair biofilm formation and disaggregate mature biofilms in Acinetobacter baumannii clinical isolates. Differences in extracellular metabolites reflect changes in biofilm metabolism due to KHS‐Cnd treatment. Among the differentially represented extracellular metabolites upon KHS‐Cnd treatment, the significantly altered ...
Fernando Porcelli, Enrico Landi, Francesco Maiurano, Irene Paris, Rosanna Papa, Marco Artini, Laura Selan, Stefano Borocci, Francesco Buonocore, Esther Imperlini   +9 more
wiley   +1 more source

Lipid Nanoparticles for the Delivery of CRISPR/Cas9 Machinery to Enable Site‐Specific Integration of CFTR and Mutation‐Agnostic Disease Rescue

Advanced Functional Materials, EarlyView.
Lipid nanoparticles (LNPs) are optimized to co‐deliver Cas9‐encoding messenger RNA (mRNA), a single guide RNA (sgRNA) targeting the endogenous cystic fibrosis transmembrane conductance regulator (CFTR) gene, and homologous linear double‐stranded donor DNA (ldsDNA) templates encoding CFTR.
Ruth A. Foley, Paul G. Ayoub, Vrishti Sinha, Colin Juett, Alicia Sanoyca, Emily C. Duggan, Lindsay E. Lathrop, Priyanka Bhatt, Kevin Coote, Beate Illek, Brigitte N. Gomperts, Donald B. Kohn, Steven J. Jonas   +12 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

Cystic Fibrosis Lung Disease: An Overview

Respiratory care, 2019
Although better insights into the natural course of cystic fibrosis (CF) have led to treatment approaches that have improved pulmonary health and increased the life expectancy of individuals with this disorder, lung disease remains the main cause of ...
N. Turcios
semanticscholar   +1 more source

A Human Kidney Tubuloid Model of Repeated Cisplatin‐Induced Cellular Senescence and Fibrosis for Drug Screening

Advanced Healthcare Materials, EarlyView.
Replicating aging and senescence‐related pathophysiological responses in kidney organoids remains a significant challenge. Human adult renal tubular organoid, tubuloids, are successfully developed recapitulating cellular senescence that is the central pathophysiological mechanism of chronic kidney disease (CKD).
Yuki Nakao, Makiko Mori, Yuta Sekiguchi, Iori Morita, Ryota Shindoh, Shintaro Mandai, Tamami Fujiki, Hiroaki Kikuchi, Fumiaki Ando, Koichiro Susa, Takayasu Mori, Ayumi Suzuki, Yuji Nashimoto, Hirokazu Kaji, Yuma Waseda, Soichiro Yoshida, Yasuhisa Fujii, Eisei Sohara, Shinichi Uchida, Kensuke Miyake, Yutaro Mori   +20 more
wiley   +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

Microbiology of Cystic Fibrosis Airway Disease

Seminars in Respiratory and Critical Care Medicine, 2019
Although survival of individuals with cystic fibrosis (CF) has been continuously improving for the past 40 years, respiratory failure secondary to recurrent pulmonary infections remains the leading cause of mortality in this patient population.
A. Blanchard, V. Waters
semanticscholar   +1 more source