Safety and efficacy of the human neutrophil elastase inhibitor BAY 85-8501 for the treatment of non-cystic fibrosis bronchiectasis: a randomized controlled trial
Conclusions1 mg BAY 85-8501 OD had a favourable safety and tolerability profile when administered for 28 days to patients with non-CF BE. Further studies with a longer treatment duration are needed to evaluate the potential clinical efficacy in this study population.
CONCLUSION: Chronic sinusitis and its complications are common in patients with CF. Mucoceles are a rare complication of sinusitis that can be treated surgically. As seen in this case if left untreated mucoceles can lead to orbital pathologies such as proptosis, hypertelorism. To the best of our knowledge, we report the first case report of giant ethmoidal mucocele leading to proptosis and hypertelorism in a patient with cystic fibrosis. PMID: 32779423 [PubMed - in process]
CONCLUSION: ABPA is recognized as the most common Aspergillus associated disorder in CF patients and is related to deteriorated pulmonary function tests; however Aspergillus colonization and bronchitis may also be associated with worsening lung function. PMID: 32779408 [PubMed - in process]
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
Azole-resistant (azole-R) Aspergillus is an increasing challenge worldwide. Patients with cystic fibrosis (CF) are at risk of Aspergillus colonization and disease due to a favorable lung environment for microorganisms. We performed a nationwide study in 2018 of azole-non-susceptible Aspergillus in CF patients and compared with data from two prior studies. All airway samples with mold isolates from patients monitored at the two CF centers in Denmark (RH, Jan–Sept and AUH, Jan–Jun) were included. Classical species identification (morphology and thermo-tolerance) was performed and MALDI-TOF/β-tubulin sequenci...
ConclusionsOur results demonstrate that ivacaftor causes acute changes in blood monocyte transcriptional profiles and plasma chemokines, and suggest that increased monocyte inflammatory signals and changes in myeloid cell trafficking may contribute to changes in airway inflammation in people taking CFTR modulators. To our knowledge, this is the first report investigating the transcriptomic response of circulating blood monocytes in CF subjects treated with a CFTR modulator.
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.