Cystic fibrosis triple therapy

Thousands of cystic fibrosis patients in England have had their health drastically improved by a “miracle” treatment, allowing one to give birth to a baby, since the NHS made it available one year ago.
Source: NHS Networks - Category: UK Health Source Type: news

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Biochem J. 2021 Nov 25:BCJ20210644. doi: 10.1042/BCJ20210644. Online ahead of print.ABSTRACTSLC26A9, a member of the solute carrier protein family, transports chloride ions across various epithelia. SLC26A9 also associates with other ion channels and transporters linked to human health, and in some cases these heterotypic interactions are essential to support the biogenesis of both proteins. Therefore, understanding how this complex membrane protein is initially folded might provide new therapeutic strategies to overcome deficits in the function of SLC26A9 partners, one of which is associated with Cystic Fibrosis. To this ...
Source: The Biochemical Journal - Category: Biochemistry Authors: Source Type: research
Discussion: In this small group of evaluated patients, we can’t show evidence of a significant short-term influence of chest physiotherapy on FEV1 nor LCI in PCD and CF, although the variability is large. The study group will be extended in the next few months (aim 15 patients in each group), with additional statistical evaluations.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric bronchology Source Type: research
Conclusion: In our small patient group with CF, it can be said that  the regular health checks of children distrupted, the duration of pulmonary rehabilitation decreased and the CF symptoms and immobilization time increased during the COVID-19 pandemic.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Physiotherapists Source Type: research
Conclusions: We found reduced moderate-intensity physical activity level and impaired quality of life in patients with non-CF bronchiectasis. Patients with non-CF bronchiectasis and healthy individuals had a similar level of COVID-19 phobia. Physical activity counseling and telerehabilitation can gain importance to improve affected outcomes during the COVID-19 pandemic.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Physiotherapists Source Type: research
Conclusion: This study demonstrates comprehensive lung MRI assessment in CF. UTE provides structural imaging without ionizing radiation. PREFUL MRI provides sensitive and regionally localized functional data, similar to 129Xe MRI, but is easier to perform. Together, these techniques could be used to assess response to therapeutics and potentially disease progression in pwCF in the future.Fig 1.UTE &PREFUL. Bronchiectatic (arrow) and cystic (arrowhead) changes are seen with UTE. Normal (green) and abnormal (purple) PREFUL ventilation is seen.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Imaging Source Type: research
Secretory leukocyte proteinase inhibitor (SLPI) is a major airways antiprotease. Its primary function is considered to be the inhibition of damaging neutrophil elastase activity, however, it also exerts antimicrobial and immunomodulatory activity. The presence of a protease/antiprotease imbalance and associated dysregulation of the levels and/or activity of host defence proteins like SLPI are features of chronic lung diseases such as cystic fibrosis and chronic obstructive pulmonary disease. The aim of this study was to elucidate the effects of sustained SLPI inactivation on the development and progression of chronic muco-...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Airway cell biology and immunopathology Source Type: research
Chronic airway inflammation is the main driver of pathogenesis in respiratory diseases, such as severe asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), and bronchiectasis. While the role of common pathogens in airway inflammation is widely recognized, the influence of other microbiota members is still poorly understood. Here, we show that Rothia mucilaginosa, a common resident of the oral cavity that is also often detectable in the lower airways in chronic disease, has an inhibitory effect on pathogen- and LPS-induced pro-inflammatory responses, both in vitro (3-D cell culture model) and in vivo ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Airway cell biology and immunopathology Source Type: research
Conclusions CFTR expression is strongly decreased in pulmonary artery smooth muscle and endothelial cells in human and animal models of PH. CFTR inhibition increases vascular cell proliferation and strongly reduces pulmonary artery relaxation.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular disease Source Type: research
Conclusions: Expression of lncRNAs profile changes during cystic fibrosis pulmonary exacerbation, suggesting their possible role in regulating disease course.The study was supported by the Polish National Science Centre, grant no. 2016/22/E/NZ5/00383 (A. Szczepankiewicz).
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Molecular pathology and functional genomics Source Type: research
Conclusion: In COPD and CF, no variables outperformed sea-level SpO2 to predict HCT outcome, while in ILD and EPR, sea-level PaCO2 was the best predictor. This should be taken into account when evaluating the need for an HCT in these populations.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Clinical respiratory physiology, exercise and functional imaging Source Type: research
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