Aspergillus fumigatus enhances elastase production in pseudomonas aeruginosaco-cultures [PDF]
, 2015 In the cystic fibrosis (CF) lung the presence of bacteria and fungi in the airways promotes an inflammatory response causing progressive lung damage, ultimately leading to high rates of morbidity and mortality.
Kerr, Stephen +6 more
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Italian Journal of PediatricsBackground During the pandemic, the pneumology team at Bambino Gesù Children’s Hospital highlighted that telemedicine was a valuable tool for remotely managing the medical needs of children with medical complexity (CMC).
Alessandro Onofri +5 more
doaj +1 more source
Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression [PDF]
, 2005 Abnormalities in mucus properties and clearance make a major contribution to the pathology of cystic fibrosis (CF). Our aim was to test the hypothesis that the defects in CF mucus are a direct result of mutations in the CF transmembrane conductance ...
Dell, Anne +6 more
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Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused by a defined subset of minimal function CFTR mutants. [PDF]
, 2019 Available CFTR modulators provide no therapeutic benefit for cystic fibrosis (CF) caused by many loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel, including N1303K. We previously introduced the
Finkbeiner, Walter E +7 more
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A Genotypic-oriented View of CFTR Genetics Highlights Specific Mutational Patterns Underlying Clinical Macro-categories of Cystic Fibrosis. [PDF]
, 2015 Cystic Fibrosis (CF) is a monogenic disease caused by mutations of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene. The genotype-phenotype relationship in this disease is still unclear, and diagnostic, prognostic and therapeutic ...
Amato, Annalisa +10 more
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Worldwide rates of diagnosis and effective treatment for cystic fibrosis.
Journal of Cystic Fibrosis, 2022 Jonathan Guo, A. Garratt, A. Hill
semanticscholar +1 more source
A laminar flow model of aerosol survival of epidemic and non-epidemic strains of Pseudomonas aeruginosa isolated from people with cystic fibrosis [PDF]
, 2008 Cystic fibrosis (CF) is an inherited multi-system disorder characterised by chronic airway infection with pathogens such as Pseudomonas aeruginosa. Acquisition of P. aeruginosa by patients with CF is usually from the environment, but recent studies have
Beggs, Clive B. +4 more
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European Clinical Respiratory JournalTherapeutic 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 +10 more
doaj +1 more source
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 +10 more
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Disruption of Interleukin-1β Autocrine Signaling Rescues Complex I Activity and Improves ROS Levels in Immortalized Epithelial Cells with Impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Function [PDF]
, 2014 Patients with cystic fibrosis (CF) have elevated concentration of cytokines in sputum and a general inflammatory condition. In addition, CF cells in culture produce diverse cytokines in excess, including IL-1B. We have previously shown that IL-1B, at low
Clauzure, Mariangeles +5 more
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