Chapter 9 : Metabolism and Pathogenicity of Infections in the Lungs of Individuals with Cystic Fibrosis

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The lung is typically considered a sterile environment; however, this is rarely true in practice. Healthy individuals constantly inhale various microbes, and the innate immune system is responsible for their removal through a system of mucocilliary clearance. Some individuals experience altered mucocilliary clearance due to any of a number of diseases, and one of the best-studied examples of this occurs in individuals with the genetic disease cystic fibrosis (CF). A hallmark phenotype of CF is dehydration of the mucosal layer across all epithelial surfaces inside the body, resulting in a thickening of the mucus that cannot be adequately cleared despite frequent expectoration. This mucus is an excellent growth substrate for a range of bacteria, and the lung disease associated with these infections further alters the mucus, resulting in a complex mixture of host and microbe derived macromolecules called sputum that is not present in healthy lungs. The opportunistic pathogen is generally the most difficult of these invaders to eradicate, and infections with this organism are the leading cause of morbidity and mortality for individuals with CF ( ).

Citation: Palmer G, Whiteley M. 2015. Metabolism and Pathogenicity of Infections in the Lungs of Individuals with Cystic Fibrosis, p 185-213. In Conway T, Cohen P (ed), Metabolism and Bacterial Pathogenesis. ASM Press, Washington, DC. doi: 10.1128/microbiolspec.MBP-0003-2014
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