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 +6 more
core +2 more sources
Does cystic fibrosis constitute an advantage in COVID-19 infection?
Italian Journal of Pediatrics, 2020 The Veneto region is one of the most affected Italian regions by COVID-19. Chronic lung diseases, such as chronic obstructive pulmonary disease (COPD), may constitute a risk factor in COVID-19. Moreover, respiratory viruses were generally associated with
Valentino Bezzerri +3 more
doaj +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
Six minute walk test in Italian children with cystic fibrosis aged 6 and 11
Monaldi Archives for Chest Disease, 2021 Six Minute Walk Test (6MWT) is a field exercise test widely used in clinical practice, both in adults and in pediatric patients. The primary aim of the study is to evaluate the physical performance of the subjects and compare them with the predicted ...
Diletta Innocenti +11 more
doaj +1 more source
MRSA eradication of newly acquired lower respiratory tract infection in cystic fibrosis [PDF]
, 2016 UK cystic fibrosis (CF) guidelines recommend eradication of methicillin-resistant Staphylococcus aureus (MRSA) when cultured from respiratory samples.
Downey, DG +7 more
core +3 more sources
How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old
Respiratory Medicine Case Reports, 2017 Background and aims: To specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics. Patients and methods: Observational study of CF patients which were diagnosed at age ≥60 year-old.
C. Prados +10 more
doaj +1 more source
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 +8 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
core +1 more source
TAS2R38 is a novel modifer gene in patients with cystic fbrosis [PDF]
, 2020 The clinical manifestation of cystic fbrosis (CF) is heterogeneous also in patients with the same cystic fbrosis transmembrane regulator (CFTR) genotype and in afected sibling pairs.
Amato, F. +10 more
core +1 more source
VX‐445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles
New England Journal of Medicine, 2018 BACKGROUND VX‐445 is a next‐generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore Phe508del CFTR protein function in patients with cystic fibrosis when administered with tezacaftor and ivacaftor (VX‐445 ...
D. Keating +19 more
semanticscholar +1 more source