The outlook for those with cystic fibrosis (CF) has never been brighter with ever increasing life expectancy and the approval of the highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor. With that being said, the progressive pulmonary decline and importance of lung health, infection, and inflammation in CF remains. This review is the second part in a three-part CF Year in Review 2020. Part one focused on the literature related to CFTR modulators while part three will feature the multisystem effects related to CF. This review focuses on articles from Pediatric Pulmonology, including articles from other journals that are of particular interest to clinicians. Herein, we highlight studies published during 2020 related to CF pulmonary disease, infection, treatment, and diagnostics.
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[Elexacaftor, Tezacaftor and Ivacaftor: immediate efficacy evaluation using home spirometry].
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.
Lung transplantation and bone health: A narrative review.
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 PDFFlow cytometric measurement of CFTR-mediated chloride transport in human neutrophils.
J 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 PDFIL-17 family members exert an autocrine pro-inflammatory loop in CF respiratory epithelial cells ex vivo.
Cell 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 PDFReply to Martin and Grasemann: Is There a Role for Lumacaftor/Ivacaftor in Young Children with Cystic Fibrosis?
Ann 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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