Results 41 to 50 of about 163,456 (151)

Identification of BPIFA1/SPLUNC1 as an epithelium-derived smooth muscle relaxing factor

Nature Communications, 2017
Asthma is characterized by abnormal airway hyperresponsiveness. Here the authors identify BPIFA1 as a factor secreted by airway epithelial cells, and show that it regulates contractility of airway smooth muscle cells by binding to and regulating the ...
Tongde Wu, Julianne Huang, Patrick J. Moore, Michael S. Little, William G. Walton, Robert C. Fellner, Neil E. Alexis, Y. Peter Di, Matthew R. Redinbo, Stephen L. Tilley, Robert Tarran   +10 more
doaj   +1 more source

The complexities of elexacaftor/tezacaftor/ivacaftor therapeutic drug monitoring in a person with cystic fibrosis and Mycobacterium abscessus pulmonary disease

European Clinical Respiratory Journal
Therapeutic drug monitoring (TDM) of elexacaftor/tezacaftor/ivacaftor (ETI) remains challenging due to a lack of clarity around the parameters that govern ETI plasma concentrations, whilst the use of concomitant CYP3A inducers rifabutin and rifampicin is
Claire Y Mou, Daniel J Henderson, Angela G Matson, Karen M Herd, David W Reid, Timothy Riddles, Ellie Johnson, Brett McWhinney, Rebecca Swenson, Andrew Burke, Ieuan E S Evans   +10 more
doaj   +1 more source

Pseudomonas aeruginosa Acquisition in Cystic Fibrosis Patients in Context of Otorhinolaryngological Surgery or Dentist Attendance: Case Series and Discussion of Preventive Concepts

Case Reports in Infectious Diseases, 2015
Introduction. P. aeruginosa is the primary cause for pulmonary destruction and premature death in cystic fibrosis (CF). Therefore, prevention of airway colonization with the pathogen, ubiquitously present in water, is essential.
Jochen G. Mainz, Andrea Gerber, Michael Lorenz, Ruth Michl, Julia Hentschel, Anika Nader, James F. Beck, Mathias W. Pletz, Andreas H. Mueller   +8 more
doaj   +1 more source

Eradication of Burkholderia cepacia Using Inhaled Aztreonam Lysine in Two Patients with Bronchiectasis

Case Reports in Pulmonology, 2014
There are not many articles about the chronic bronchial infection/colonization in patients with underlying lung disease other than cystic fibrosis (CF), especially with non-CF bronchiectasis (NCFBQ). The prevalence of B. cepacia complex is not well known
A. Iglesias, I. Artiles, J. J. Cabanillas, R. Álvarez-Sala, C. Prados   +4 more
doaj   +1 more source

Modulation of immune responses by elexacaftor/tezacaftor/ivacaftor therapy in cystic fibrosis: data from a compassionate use program

Respiratory Research
Background Elexacaftor-tezacaftor-ivacaftor (ETI) improves clinical outcomes in people with Cystic Fibrosis (pwCF), with possible anti-inflammatory properties. However, the molecular mechanisms underlying these effects remain unclear.
Francesca Lucca, Anna Pianazzola, Ilaria Meneghelli, Giuseppina Catanzaro, Giuseppe Sabbioni, Gloria Tridello, Anca Manuela Hristodor, Erik De Luca, Giacomo Menichetti, Giulia Breveglieri, Maria Teresa Altieri, Marica Bordicchia, Benedetta Fabrizzi, Elisabetta Ferretti, Giuseppe Lippi, Mirco Ros, Monica Borgatti, Marco Cipolli, Valentino Bezzerri   +18 more
doaj   +1 more source

Lung clearance index short-term variability in cystic fibrosis: a pre-post pulmonary exacerbation study

Italian Journal of Pediatrics
Background Multiple Breath washout (MBW) represents an important tool to detect early a possible pulmonary exacerbation especially in Cystic Fibrosis (CF) disease.
Matteo De Marchis, Enza Montemitro, Alessandra Boni, Alessandra Federici, Daniele Di Giovanni, Luca Cristiani, Renato Cutrera, Alessandro G. Fiocchi   +7 more
doaj   +1 more source

Exploring the utilisation and effectiveness of implementation science strategies by cystic fibrosis registries for healthcare improvement: a systematic review

European Respiratory Review
Background Cystic fibrosis (CF) registries capture important information in high-burden health domains to support improvement in health outcomes, although a number of unanswered questions persist, as follows.
Rob G. Stirling, Bhumika Sood, Macken J.L. Stirling, Tom Kotsimbos, Dominic T. Keating, Catherine E. Rang, James M. Trauer, Alan C. Young, Christiaan Yu, Julianna Bailey, Peter Wark, Angela Melder, Paul Dawkins   +12 more
doaj   +1 more source

Improving the Rate of Sufficient Sweat Collected in Infants Referred for Sweat Testing in Michigan

Global Pediatric Health, 2014
Objective . Sweat collected for testing should have quantity not sufficient (QNS) rate of ≤10% in babies ≤3 months of age. Michigan (MI) cystic fibrosis (CF) centers’ QNS rates were 12% to 25% in 2009. This project was initiated to reduce sweat QNS rates
Ibrahim Abdulhamid MD, Mary Kleyn MSC, Carrie Langbo MS, Myrtha Gregoire-Bottex MD, John Schuen MD, Krithika Shanmugasundaram BS, Samya Z Nasr MD   +6 more
doaj   +1 more source

CFTR modulators response of S737F and T465N CFTR variants on patient-derived rectal organoids

Orphanet Journal of Rare Diseases
Background Predictions based on patient-derived materials of CFTR modulators efficacy have been performed lately in patient-derived cells, extending FDA-approved drugs for CF patients harboring rare variants.
Karina Kleinfelder, Paola Melotti, Anca Manuela Hristodor, Cristina Fevola, Giovanni Taccetti, Vito Terlizzi, Claudio Sorio   +6 more
doaj   +1 more source

Sodium Glucose‐Like Co‐Transporter 2 Inhibitor (SGLT2i) Use in Adults With Cystic Fibrosis

Respirology Case Reports
Sodium‐glucose cotransporter 2 inhibitors (SGLT2i) also called flozins or gliflozins have been demonstrated to have multi‐system benefits across the endocrine, metabolic, renal and cardiovascular systems.
Ellie Johnson, Angela Matson, Robyn Cobb, Shanal Kumar   +3 more
doaj   +1 more source