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Introduction: Elexacaftor/tezacaftor/ivacaftor (ETI) has shown significant improvements in pulmonary and nutritional status in persons with cystic fibrosis (pwCF). Less is known about the extrapulmonary impact of ETI and effects on airway microbiology, lung clearance index (LCI) and fraction of exhaled nitric oxide (FeNO).
Methods: A multicentre prospective observational trial, including 79 pwCF ≥ 18 years eligible for ETI.
The role and therapeutic targeting of the CCL2/CCR2 signaling axis in inflammatory and fibrotic diseases.
Front Immunol
January 2025
Department of Gastroenterology, The Affiliated Hospital of Qingdao University, Qingdao, China.
CCL2, a pivotal cytokine within the chemokine family, functions by binding to its receptor CCR2. The CCL2/CCR2 signaling pathway plays a crucial role in the development of fibrosis across multiple organ systems by modulating the recruitment and activation of immune cells, which in turn influences the progression of fibrotic diseases in the liver, intestines, pancreas, heart, lungs, kidneys, and other organs. This paper introduces the biological functions of CCL2 and CCR2, highlighting their similarities and differences concerning fibrotic disorders in various organ systems, and reviews recent progress in the diagnosis and treatment of clinical fibrotic diseases linked to the CCL2/CCR2 signaling pathway.
View Article and Find Full Text PDFThe Pharmacokinetic Changes in Cystic Fibrosis Patients Population: Narrative Review.
Medicines (Basel)
December 2024
Pharmacy School, West Coast University, Los Angeles, CA 90004, USA.
Cystic fibrosis (CF) is a rare genetic disorder commonly affecting multiple organs such as the lungs, pancreas, liver, kidney, and intestine. Our search focuses on the pathophysiological changes that affect the drugs' absorption, distribution, metabolism, and excretion (ADME). This review aims to identify the ADME data that compares the pharmacokinetics (PK) of different drugs in CF and healthy subjects.
View Article and Find Full Text PDFIdentification of a vimentin-expressing α-cell phenotype in CF and normal pancreas.
J Endocrinol
January 2025
J Shaw, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom of Great Britain and Northern Ireland.
Endocrine dysfunction and diabetes can develop secondary to fibrotic diseases within the pancreas including cystic fibrosis (CF). Phenotypic shift within epithelial cells has been recognised in association with pro-fibrotic signalling. We sought evidence of endocrine cell epithelial-to-mesenchymal transition in CF and non-CF pancreas.
View Article and Find Full Text PDFUnraveling the Connection Between Ion Channels and Pancreatic Stellate Cell Activation.
Cell Physiol Biochem
January 2025
UR-UPJV 4667, UFR Sciences, Université de Picardie Jules Verne, Amiens, France,
Quiescent pancreatic stellate cells (PSCs) represent only a very low proportion of the pancreatic tissue, but their activation leads to stroma remodeling and fibrosis associated with pathologies such as chronic pancreatitis and pancreatic ductal adenocarcinoma (PDAC). PSC activation can be induced by various stresses, including acidosis, growth factors (PDGF, TGFβ), hypoxia, high pressure, or intercellular communication with pancreatic cancer cells. Activated PSC targeting represents a promising therapeutic strategy, but little is known regarding the molecular mechanisms underlying the activation of PSCs.
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