The progressive deterioration in lung function seen in cystic fibrosis (CF) has always been thought to be due to the primary effects of the gene mutation on respiratory mucosal secretions, compounded by bacterial infections. Although we knew that inflammation per se plays a part, exuberant inflammatory processes were thought to be a result of chronic infection rather than a primary effect of the mutation on inflammatory cells: until now. Researchers from Edinburgh conducted a series of experiments that implicate neutrophils, and more specifically neutrophil extracellular traps (NETs), as being a primary rather than secondary cause of damage (Gray R et al. Thorax 2017. http://ift.tt/2yLEbwA). NETs form when neutrophils survive too long and can further exaggerate inflammatory responses through macrophage activation. They are abundant in CF airways
They extracted neutrophils from the blood of adult CF patients with established lung inflammation, healthy controls, and transgenic pig models, bred...
from Archives of Disease in Childhood current issue http://ift.tt/2AhxZPd
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