Six minute walk test in Italian children with cystic fibrosis aged 6 and 11
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
Early respiratory viral infections in infants with cystic fibrosis [PDF]
This article is made available for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source.
A Peshkin +41 more
core +1 more source
MRSA eradication of newly acquired lower respiratory tract infection in cystic fibrosis [PDF]
UK cystic fibrosis (CF) guidelines recommend eradication of methicillin-resistant Staphylococcus aureus (MRSA) when cultured from respiratory samples.
Downey, DG +7 more
core +3 more sources
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
core +1 more source
How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old
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
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation [PDF]
Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures
Amin R +39 more
core +4 more sources
Background Increasing awareness of milder presentations of cystic fibrosis (CF) and greater interest in non-CF bronchiectasis are likely to lead to more CF screening by respiratory clinicians. As a result, adults who may not strictly fulfil CF diagnostic
Alessandro N. Franciosi +8 more
doaj +1 more source
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
core +1 more source
VX‐445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles
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
Interventions for the eradication of meticillin-resistant Staphylococcus aureus (MRSA) in people with cystic fibrosis [PDF]
Background: Cystic fibrosis is an inherited recessive disorder of chloride transport that is characterised by recurrent and persistent pulmonary infections from resistant organisms that result in lung function deterioration and early mortality in ...
Adeboyeku +70 more
core +4 more sources

