Results 21 to 30 of about 975,042 (316)
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
JAMA Network Open, 2020 This cross-sectional study compares disease-specific federal and foundation funding for sickle cell disease and cystic fibrosis research and assesses the factors associated with research productivity.
Faheem Farooq +4 more
semanticscholar +1 more source
Frontiers in Cellular and Infection Microbiology, 2011 IntroductionOrganisms of the Burkholderia cepacia complex (BCC) are important pathogens in cystic fibrosis (CF). The majority of those who acquire BCC develop chronic infection but it can also result in rapid decline in a significant minority.
Alex eHorsley +7 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
Molecular Therapy: Nucleic AcidsNonsense or premature termination codon (PTC) variants of the CFTR gene are pathogenic and found in ∼10% of North American people with cystic fibrosis.
Normand E. Allaire +15 more
doaj +1 more source
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
Cystic Fibrosis and Pseudomonas aeruginosa: the Host-Microbe Interface
Clinical Microbiology Reviews, 2019 In human pathophysiology, the clash between microbial infection and host immunity contributes to multiple diseases. Cystic fibrosis (CF) is a classical example of this phenomenon, wherein a dysfunctional, hyperinflammatory immune response combined with ...
Sankalp Malhotra, D. Hayes, D. Wozniak
semanticscholar +1 more source
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
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