Ten years of chest MRI for patients with cystic fibrosis : Translation from the bench to clinical routine.
CONCLUSION: After more than a decade, lung MRI has become a valuable tool for monitoring CF in clinical routine application and as an endpoint for clinical studies. PMID: 31172247 [PubMed - as supplied by publisher]
ABSTRACT Objective: To evaluate factors associated with the performance of children and adolescents with cystic fibrosis (CF) in the Modified Shuttle Test (MST) and compare it with healthy children and adolescents. Methods: This is a cross-sectional study, with children and adolescents divided into two groups: cystic fibrosis (CFG) and control (CG). Variables evaluated in the MST: walking distance, test level, heart rate variation ( ∆ Hr), post-test mean arterial pressure (MAP Pt) and peripheral oxygen saturation variation ( ∆ SPO2). Statistical analysis included Mann Whitney and Spearman coefficient tests, being significant p
Publication date: Available online 8 August 2020Source: Nutrition, Metabolism and Cardiovascular DiseasesAuthor(s): Cécile Nguyen, Marie-Hélène Denis, Miguel Chagnon, Rémi Rabasa-Lhoret, Geneviève Mailhot
Chronic oral azithromycin therapy improves clinical outcomes in people with cystic fibrosis (CF) , and is recommended for treatment of CF lung disease . Azithromycin is categorized as pregnancy class B; no evidence of impaired fertility or fetal harm was observed in mice and rats at maternally toxic doses . However, no adequate/well-controlled studies are likely to be performed in pregnant women.
Abstract Two Burkholderia gladioli strains isolated from the lungs of cystic fibrosis patients were found to produce unusual lipodepsipeptides containing a unique citrate-derived fatty acid and a rare dehydro-β-alanine residue. The gene cluster responsible for their biosynthesis was identified by bioinformatics and insertional mutagenesis. In-frame deletions and enzyme activity assays were used to investigate the functions of several proteins encoded by the biosynthetic gene cluster, which was found in the genomes of about 50% of B. gladioli isolates, suggesting that its metabolic products play an important r...
Unified airway disease where upper respiratory tract inflammation including chronic rhinosinusitis (CRS) affects lower airway disease is known from asthma, bronchiectasis, cystic fibrosis and primary ciliary dyskinesia but little is known about CRS and health related quality of life in COPD. We investigate firstly, the prevalence of CRS in COPD. Secondly the impact of CRS on HRQoL. Thirdly, risk factors for CRS in COPD.
In Belgium, COVID-19 epidemy began on February 4, 2020 with a peak on April 10, 2020. Patients with cystic fibrosis (CF) followed in the Cliniques universitaires Saint-Luc were rapidly isolated before the government lockdown.
It is well established that many patients with cystic fibrosis (CF) have pain, including those with stable lung disease. Hayes et al. reported that 82% of adult patients with CF reported pain within the past 30 days and 27% reported pain ≥ 6 months during the past year . Additionally, in a meta-analysis of 1,665 pooled patients with stable disease, 77% of adults and 42% of children reported pain . Pain correlated to worsening quality of life, decreased physical function, and greater risk for respiratory exacerbations [1,2].
Heel-prick tests are already used in Britain to check for nine rare but serious conditions, including cystic fibrosis and sickle cell disease. Scientists are pushing for diabetes to be added.
In this edition of the Journal, the outputs from two workshops provide an important framework for the future development of a pipeline for new CF therapies. The workshops considered drug development, clinical trial design, and regulatory pathways, in the evolving and challenging research environment that has developed with clinical use of CFTR modulation.
ConclusionsBone involvement can present with diverse severity in the same pedigree, ranging from low BMD to multiple fragility fractures. Antiresorptive therapy improves BMD, but its anti-fracture efficacy remains to be shown. The presence of CD might indicate the significant role of NOTCH2 signaling in different tissues.