Measuring and reporting quality of life outcomes in clinical trials in cystic fibrosis: a critical review [PDF]
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
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
A retrospective analysis of longitudinal changes in bone mineral content in cystic fibrosis [PDF]
Background: We aimed to describe the longitudinal changes in bone mineral content and influencing factors, in children with cystic fibrosis (CF). Methods: One hundred children (50 females) had dual X-ray absorptiometry (DXA) performed.
Ahmed, Syed Faisal+7 more
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Does cystic fibrosis constitute an advantage in COVID-19 infection?
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
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A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion
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
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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
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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
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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
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
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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
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