Risk factors for progression of structural lung disease in school-age children with cystic fibrosis
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Non-cystic fibrosis bronchiectasis (NCFBE) is a rare, chronic lung disease characterized by bronchial inflammation and permanent airway dilation. Chronic infections with Pseudomonas aeruginosa (PA) have been linked to higher morbidity and mortality in NCFBE patients. We assessed treatment patterns for PA among NCFBE patients in a US commercially-insured database.
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Lung disease in cystic fibrosis is primarily due to a defect in the cystic fibrosis transmembrane regulating protein (CFTR). This results in abnormal chloride transfer across epithelial membranes causing an excessively viscid mucus lining of the airways. Bacterial invasion particularly with <i>Staphylococcus aureus, Haemophilus influenzae</i> and <i>Pseudomonas aeruginosa</i> stimulates a vigorous and excessive primarily neutrophil-driven inflammatory response throughout the lungs. Products of this inflammation not only damage incoming bacteria but also the host tissue itself. Over a period of years this chronic suppurative process results in permanent ongoing lung destruction principally manifested as bilateral bronchiectasis.
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To bead or not to bead: A review of Pseudomonas aeruginosa lung infection models for cystic fibrosis
Cystic fibrosis (CF) lung disease is characterised by recurring bacterial infections resulting in inflammation, lung damage and ultimately respiratory failure. Pseudomonas aeruginosa is considered one of the most important lung pathogens in those with cystic fibrosis. While multiple cystic fibrosis animal models have been developed, many fail to mirror the cystic fibrosis lung disease of humans, including the colonisation by opportunistic environmental pathogens. Delivering bacteria to the lungs of animals in different forms is a way to model cystic fibrosis bacterial lung infections and disease. This review presents an overview of previous models, and factors to consider when generating a new P. aeruginosa lung infection model. The future development and application of lung infection models that more accurately reflect human cystic fibrosis lung disease has the potential to assist in understanding the pathophysiology of cystic fibrosis lung disease and for developing treatments.
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