miR-17 overexpression in cystic fibrosis airway epithelial cells decreases interleukin-8 production. [PDF]
, 2015 Interleukin (IL)-8 levels are higher than normal in cystic fibrosis (CF) airways, causing neutrophil infiltration and non-resolving inflammation.
Agrawal, Raman +9 more
core +2 more sources
Staphylococcus aureus in cystic fibrosis: pivotal role or bit part actor? [PDF]
, 2018 Purpose of review: describe why this review is timely and relevant. The cystic fibrosis lung has long been appreciated as a competitive niche for complex interactions between bacterial species.
Hurley, Matthew N., Smyth, Alan R.
core +2 more sources
Elevated sweat chloride test: is it always cystic fibrosis?
Italian Journal of Pediatrics, 2021 Background The sweat chloride test (ST) is the gold standard for cystic fibrosis (CF) diagnosis in symptomatic patients, within the newborn screening and in the follow-up of CF patients during molecular therapies.
C. Cimbalo +6 more
doaj +1 more source
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype phenotype correlations [PDF]
, 1996 We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure.
Alton, Ewfw +11 more
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The Lancet, 1998 OBJECTIVE: Due to the great advances recently achieved in the treatment of Cystic Fibrosis as well as to the fact that pediatricians need to have a better understanding of this disease, the authors propose an extensive review of the subject. METHODS: We selected the most outstanding publications on Cystic Fibrosis in the international literature of the
B J, Rosenstein, P L, Zeitlin
openaire +4 more sources
Deutsches Ärzteblatt international, 2017 Universal screening of newborn babies for cystic fibrosis was launched in Germany on 1 September 2016. Here we present up-to-date information on the diagnosis, treatment, and prognosis of this disease.This article is based on relevant publications retrieved by a selective search in PubMed, along with guidelines from Germany and abroad and systematic ...
Radtke, Thomas +2 more
openaire +5 more sources
The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation : a review [PDF]
, 2016 We reviewed the impact of ivacaftor on Scottish paediatric cystic fibrosis (CF) patients ≥ 6 years of age after 12 months of treatment. Statistically significant improvements in FEV1 and BMI and a reduction in sweat chloride, all comparable with ...
Scottish Paediatric Cystic Fibrosis Managed Clinical Network (SPCFMCN)
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Longitudinal impact of demographic and clinical variables on Health-Related Quality of Life in Cystic Fibrosis [PDF]
, 2015 Objectives: The insights that people with cystic fibrosis have concerning their health are important given that aspects of health-related quality of life (HRQoL) are independent predictors of survival and a decrease in lung function is associated with a ...
A-Rahman +33 more
core +2 more sources
Cystic Fibrosis Foundation and European Cystic Fibrosis Society Survey of cystic fibrosis mental health care delivery [PDF]
, 2015 Background: Psychological morbidity in individuals with cystic fibrosis (CF) and their caregivers is common. The Cystic Fibrosis Foundation (CFF) and European Cystic Fibrosis Society (ECFS) Guidelines Committee on Mental Health sought the views of CF ...
A.L. Quittner +33 more
core +1 more source
A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion
Nature Communications, 2021 Premature termination codons can cause early translation termination and lead to disease. Here the authors perform a screen to identify compounds with readthrough activity and show that these reduce eRF1 levels to suppress premature termination ...
Jyoti Sharma +29 more
doaj +1 more source