19 hasil untuk "Nicholas J. Simmonds"
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Artikel Jurnal
Cystic fibrosis modulator therapy can reverse cystic bronchiectasis
Abstract Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms. Cystic fibrosis, (CF) caused by pathogenic variants in the cystic fibrosis transmembrane conductance…
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Artikel Jurnal
Beyond Cystic Fibrosis: Recognising Shwachman‐Diamond Syndrome in the Respiratory Clinic
ABSTRACT Cystic fibrosis (CF) and Shwachman‐Diamond Syndrome (SDS) share overlapping features, including recurrent respiratory infections and pancreatic insufficiency, which can complicate diagnosis. We report a case of a six‐year‐old girl who presented with productive cough, steatorrhea and…
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Artikel Jurnal
Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil
Abstract Background Advanced lung disease in adult cystic fibrosis (CF) drives most clinical care requirements. The aim was to evaluate outcome (time to death while in the study) in a cohort of adult CF patients with severe lung disease, and to determine the association among baseline patient…
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Artikel Jurnal
Assessing the health risk of living near composting facilities on lung health, fungal and bacterial disease in cystic fibrosis: a UK CF Registry study
Abstract Aim To explore the health risk of living near permitted composting sites (PCSs) on disease severity in children and adults with cystic fibrosis (CF) across the UK. Methods A semi-individual cross-sectional study was used to examine the risk of disease severity in people with CF (pwCF)…
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Artikel Jurnal
Is daily physical activity affected by dynamic hyperinflation in adults with cystic fibrosis?
Abstract Background The aim of this study was to investigate the relationship between dynamic hyperinflation and daily physical activity (DPA) in adults with cystic fibrosis (CF). Methods Thirty-four clinically stable CF were studied. All patients undertook incremental cardiopulmonary exercise…
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Artikel Jurnal Teks lengkap
Yoga Outcomes Get Assessed in Cystic Fibrosis (YOGA-CF): protocol of a multicentre interventional randomised controlled clinical trial—investigating effects of a 12-week yoga intervention for adults with cystic fibrosis
Introduction Yoga is an emerging exercise choice for people with cystic fibrosis (CF), but evidence of its effect in this population is scarce, with a recent systematic review advocating for further research. Yoga Outcomes Get Assessed in CF (YOGA-CF) is a real-world multicentre randomised…
Juga tersedia di: DOAJ
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Artikel Jurnal
Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial
Introduction Airway clearance techniques (ACTs) are a gold standard of cystic fibrosis management; however, the majority of research evidence for their efficacy is of low standard; often attributed to the lack of sensitivity from outcome measures (OMs) used historically. This randomised controlled…
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Artikel Jurnal
Clinimetric analysis of outcome measures for airway clearance in people with cystic fibrosis: a systematic review
Background: Airway clearance techniques (ACTs) are integral to cystic fibrosis (CF) management. However, there is no consensus as to which outcome measures (OMs) are best for assessing ACT efficacy. Objectives: To summarise OMs that have been assessed for their clinimetric properties (including…
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complex and limited cutaneous vasculitis in patients with cystic fibrosis: a case series
Lesson There is a high association of reactive skin presentations, mainly limited cutaneous vasculitis in patients with cystic fibrosis and Burkholderia cepcia complex chronic infection. This may be due to raised levels of circulating inflammatory mediators.
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Artikel Jurnal Teks lengkap
Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to Aspergillus fumigatus in a mouse model of cystic fibrosis.
Aspergillus fumigatus (Af) is a major mould pathogen found ubiquitously in the air. It commonly infects the airways of people with cystic fibrosis (CF) leading to Aspergillus bronchitis or allergic bronchopulmonary aspergillosis. Resident alveolar macrophages and recruited neutrophils are important…
Juga tersedia di: PLOS
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Artikel Jurnal
New strategies of physical activity assessment in cystic fibrosis: a pilot study
Abstract Background Regular physical activity (PA) is a valued part of cystic fibrosis (CF) care. Although the accelerometer, SenseWear Armband (SWA), accurately measures habitual PA in CF, it is mostly used for research purposes. For the first time, we analyzed different methods of measuring PA in…
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Artikel Jurnal
Protecting clinical trials in cystic fibrosis during the SARS-CoV-2 pandemic: risks and mitigation measures
Abstract The SARS-CoV-2 pandemic has disrupted clinical trials worldwide. The European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN) has tracked clinical trial disruption by surveying its 58 trial sites across 17 European countries and collated information on measures to mitigate the…
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Artikel Jurnal
Health State Utilities Associated With Treatment Burden in Cystic FibrosisTake-home Points
Background: Although recent advancements in the treatment of cystic fibrosis (CF) have improved survival, reducing high levels of treatment burden remains a priority issue for many people with cystic fibrosis (pwCF). However, economic evaluations of novel interventions may fail to capture their…
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Artikel Jurnal
Improving ototoxicity monitoring in patients receiving aminoglycosides using a novel digital approach: a quality improvement project
Aminoglycoside antibiotics cause ototoxicity for which baseline audiometric testing is recommended but often not done. Barriers to successful implementation include limited availability of sound-booths and audiologists. An ototoxicity monitoring programme (OMP) was implemented using tablet-based…
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Artikel Jurnal
Managing an ageing cystic fibrosis population: challenges and priorities
The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, is likely to herald a shift in the focus of…
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Artikel Jurnal
Genotype and transcript processing of the tumour necrosis factor receptor TNFRSF1A in epithelial cells: implications for survival in cystic fibrosisResearch in context
Summary: Background: Cystic fibrosis is caused by mutations of the cystic fibrosis transmembrane conductance regulator, CFTR, an epithelial anion transport protein, responsible for, inter alia, sputum viscoelasticity in the lung. We previously identified the TNF receptor superfamily 1A TNFRSF1A…
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Artikel Jurnal
A randomised, double-blind, placebo-controlled trial of repeated nebulisation of non-viral cystic fibrosis transmembrane conductance regulator (CFTR) gene therapy in patients with cystic fibrosis
Background: Cystic fibrosis (CF) is a chronic, life-limiting disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene leading to abnormal airway surface ion transport, chronic lung infections, inflammation and eventual respiratory failure. With the exception of the…