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
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
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
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)
core +1 more source
Allergic bronchopulmonary aspergillosis: diagnostic and treatment challenges [PDF]
, 2016 Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder, occurring mostly in asthmatic and cystic fibrosis patients, caused by an abnormal T-helper 2 lymphocyte response of the host to Aspergillus fumigatus antigens.
CINICOLA, BIANCA LAURA +3 more
core +1 more source
A new targeted CFTR mutation panel based on next-generation sequencing technology [PDF]
, 2017 Searching for mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR) is a key step in the diagnosis of and neonatal and carrier screening for cystic fibrosis (CF), and it has implications for prognosis and personalized therapy ...
Alberti, Luisella +10 more
core +2 more sources
Mechanisms of endothelial cell dysfunction in cystic fibrosis [PDF]
, 2017 Although cystic fibrosis (CF) patients exhibit signs of endothelial perturbation, the functions of the cystic fibrosis conductance regulator (CFTR) in vascular endothelial cells (EC) are poorly defined.
Anile, Marco +22 more
core +1 more source
Impact of social complexity on outcomes in cystic fibrosis after transfer to adult care [PDF]
, 2018 Objective This study evaluates the roles of medical and social complexity in health care use outcomes in cystic fibrosis (CF) after transfer from pediatric to adult care.
Bosslet, Gabriel Timothy +4 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