Results 21 to 30 of about 925,943 (256)
Children, 2023 Background: Cystic fibrosis (CF) patients require regular airway clearance therapy (ACT). The aim of this study was to evaluate homecare therapeutic effects of a new ACT (Simeox®) added to the optimal standard of care, including home chest physiotherapy,
Dorota Sands +8 more
doaj +1 more source
Clinical Case Reports, 2021 Elexacaftor/tezacaftor/ivacaftor (ETI) is a cystic fibrosis (CF) transmembrane regulator (CFTR) modulator. It is known to be efficacious in stable patients with severe pneumopathy, but there are few data concerning its effectiveness during acute ...
Donatello Salvatore +4 more
doaj +1 more source
Paraquat ingestion in an adult with cystic fibrosis (CF): Diagnostic and management dilemmas
Respirology Case Reports, 2023 N,N'‐dimethyl‐4,4'bipyridinium dichloride (Paraquat) is a potent herbicide used widely in agriculture. We report the effects of an ingestion of paraquat by a 28 year old male with cystic fibrosis and the diagnostic and management challenges this posed in
Ieuan E. S. Evans +8 more
doaj +1 more source
Malignancies in patients with cystic fibrosis: a case series
Journal of Medical Case Reports, 2022 Background Previous reports have shown an increased number of colorectal cancers in patients with cystic fibrosis. We assessed the database of our cystic fibrosis center to identify patients with all kinds of cancer retrospectively. All patients visiting
Dorothea Appelt +3 more
doaj +1 more source
Virtual Peer Support for People With Cystic Fibrosis and Their Family Members: A Program Evaluation
Journal of Patient Experience, 2020 We sought to evaluate the feasibility, acceptability, and benefits of a virtual one-to-one peer support program for people with cystic fibrosis and their family members through a retrospective program evaluation.
Aimee Jeffrey MSN +5 more
doaj +1 more source
Cystic fibrosis in the year 2020: A disease with a new face
Acta paediatrica, 2020 The autosomal recessive disease cystic fibrosis (CF) was once untreatable and deadly in childhood, but now most patients survive to adulthood. Many countries have instituted CF newborn screening because early diagnosis improves outcome.
K. De Boeck
semanticscholar +1 more source
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
New England Journal of Medicine, 2019 BACKGROUND Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, and nearly 90% of patients have at least one copy of the Phe508del CFTR mutation.
P. Middleton +20 more
semanticscholar +1 more source
VX-659–Tezacaftor–Ivacaftor in patients with cystic fibrosis and one or two Phe508del alleles
Canadian Journal of Respiratory Critical Care and Sleep Medicine, 2020 Cystic fibrosis is an autosomal-recessive, multisystem disease that affects ∼80,000 patients worldwide.1,2 It is caused by mutations in the CFTR gene, an epithelial chloride ion channel with varyin...
E. Merman, M. Stanbrook, A. Anand
semanticscholar +1 more source
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
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