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Journal

Pathophysiology of cystic fibrosis lung disease.

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Published:1st Jun 2014
Author: <p>Mall MA, Boucher RC.</p>
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Ref.:Eur Respir Monogr. 2014 June;64:1-13.
DOI:10.1183/1025448x.10008513

Chronic obstructive lung disease starting in the first months of life remains the major cause of morbidity and mortality in patients with cystic fibrosis (CF). The discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago paved the way for investigation of the molecular and cellular basis of CF lung disease, and the generation of animal models forin vivostudies of pathogenesis. In this chapter, we focus on major advances in the understanding of the link between mutations in CFTR and the predictable development of chronic mucus obstruction, inflammation and infection of CF airways. We discuss evidence from translational studies supporting the concept that increased mucus concentration (i.e. dehydration) and reduced pH of the airway surface layer are key abnormalities underlying impaired innate defence of the CF lung. Novel therapeutic strategies targeting these defects may be successful for the prevention and treatment of CF lung disease independent of the patient's CF genotype.

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