Lack of Correlation Between Pulmonary Disease and Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Cystic Fibrosis: A Case Report [PDF]
, 2013 Introduction: Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride ...
Asher, Daniel +7 more
core +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
Relationship between pulmonary exacerbations and daily physical activity in adults with cystic fibrosis [PDF]
, 2015 The aim of this study was to examine the relationship between pulmonary exacerbations and physical activity (PA) in adults with cystic fibrosis (CF)
Banya, Winston +7 more
core +2 more sources
Social interactions in the Burkholderia cepacia complex : biofilms and quorum sensing [PDF]
, 2010 Burkholderia cepacia complex bacteria are opportunistic pathogens that cause respiratory tract infections in susceptible patients, mainly people with cystic fibrosis. There is convincing evidence that B.
Coenye, Tom
core +2 more sources
Measuring and reporting quality of life outcomes in clinical trials in cystic fibrosis: a critical review [PDF]
, 2005 Good quality clinical trials are essential to inform the best cystic fibrosis (CF) management and care, by determining and comparing the effectiveness of new and existing therapies and drug delivery systems.
Abbott, Janice, Hart, Anna
core +2 more sources
Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis [PDF]
, 2018 BACKGROUND: In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis (Responders) is associated with better growth and less lung disease at age 6 years.
Farrell, Philip M. +3 more
core +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
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