Educational Aims: To recognise the clinical and radiological presentation of the spectrum of diseases associated with bronchiectasis.To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems.
Unlabelled: Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF), an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different "bronchiectasis syndromes". The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF.
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http://dx.doi.org/10.1183/20734735.007516 | DOI Listing |
Rev Mal Respir
January 2025
Unité de pneumologie adulte, centre de mucoviscidose liégeois, CHC MontLégia, Liège, Belgique.
Introduction: Following two weeks of application of the triple combination therapy of Elexacaftor (E), Tezacaftor (T), and Ivacaftor (I) known as ETI, substantial pulmonary improvement in patients with cystic fibrosis is well-documented. However, few detailed data are available on the action of this treatment over the course of these first 14 days.
Methods: In this prospective study (NCT05599230), 20 patients aged≥12 years, all of them eligible for ETI, were recruited at the initiation of treatment.
J Heart Lung Transplant
January 2025
Department of Medicine, University Health Network and Sinai Health System, University of Toronto, Toronto, ON, Canada; Toronto General Hospital Research Institute, University Health Network, Toronto, ON, Canada; Joint Department of Medical Imaging, University of Toronto, Toronto, ON, Canada. Electronic address:
Bone health after lung transplantation has not been comprehensively reviewed in over two decades. This narrative review summarizes available literature on bone health in the context of lung transplantation, including epidemiology, presentation and post-operative management. Osteoporosis is reported in approximately 30-50% of lung transplant candidates, largely due to disease-related impact on bone and lifestyle, and corticosteroid-related effects during end-stage lung disease (interstitial lung diseases, chronic obstructive pulmonary disease, and historically cystic fibrosis).
View Article and Find Full Text PDFJ Leukoc Biol
January 2025
Center for Microbial Pathogenesis, The Abigail Wexner Research Institute at Nationwide Children's Hospital.
Immune cells express a variety of ion channels and transporters in the plasma membrane and intracellular organelles, responsible of the transference of charged ions across hydrophobic lipid membrane barriers. The correct regulation of ion transport ensures proper immune cell function, activation, proliferation, and cell death. Cystic fibrosis (CF) is a genetic disease in which the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) chloride channel gene is defective, consequently, the CFTR protein is dysfunctional, and the chloride efflux in CF cells is markedly impaired.
View Article and Find Full Text PDFCell Immunol
January 2025
Department of Clinical and Experimental Medicine, University of Foggia, Foggia, Italy. Electronic address:
Background: Lungs of people with Cystic Fibrosis (pwCF) are characterized by chronic inflammation and infection with P. aeruginosa. High levels of IL-17 A and F have been observed in sputum of pwCF and the interleukin-17(IL-17) family (A-to-F) has been suggested to play a key role in CF pulmonary disease.
View Article and Find Full Text PDFAnn Am Thorac Soc
January 2025
Charité Universitätsmedizin Berlin, Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Berlin, Germany.
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