Pathogenesis of lung disease in cystic fibrosis

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 Staphylo coccus aureus, Haemophilus influenzae and Pseudomonas aeruginosa stimulates a vigorous and excessive primarily neutrophil-driven inflammatory response throughout the lungs. Products of this inflammation not only damage incomi ng bacteria but also the host tissue itself. Over a period of years this ch ronic suppurative process;results in permanent ongoing lung destruction pri ncipally manifested as bilateral bronchiectasis. Copyright (C) 2000 S. Karg er AG, Basel.