Cystic Fibrosis Foundation and European Cystic Fibrosis Society Survey of cystic fibrosis mental health care delivery [PDF]
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
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The impact of 12 months treatment with ivacaftor on Scottish paediatric patients with cystic fibrosis with the G551D mutation : a review [PDF]
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)
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Lack of Correlation Between Pulmonary Disease and Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Cystic Fibrosis: A Case Report [PDF]
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
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Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis [PDF]
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
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TAS2R38 is a novel modifer gene in patients with cystic fbrosis [PDF]
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
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Elevated sweat chloride test: is it always cystic fibrosis?
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
Social interactions in the Burkholderia cepacia complex : biofilms and quorum sensing [PDF]
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
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New era of cystic fibrosis: full mutational analysis and personalized therapy [PDF]
Despite its apparently simple genetics, cystic fibrosis (CF) is a rather complex genetic disease. A lot of variability in the steps of the path from the cystic fibrosis transmembrane conductance regulator (CFTR ) gene to the clinical manifestations ...
Lucarelli, Marco
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Relationship between pulmonary exacerbations and daily physical activity in adults with cystic fibrosis [PDF]
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
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Pancreatic cystosis in cystic fibrosis. Sometimes a bike ride can help you decide [PDF]
Pancreatic cystosis (PC) is an uncommon manifestation of pancreas involvement in cystic fibrosis (CF), characterized by the presence of multiple macrocysts partially or completely replacing pancreas.
Di Gaeta, A.+4 more
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