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J Cyst Fibros. Author manuscript; available in PMC 2017 November 01. Published in final edited form as: J Cyst Fibros. 2016 November ; 15(6): 794–801. doi:10.1016/j.jcf.2016.05.012.

Respiratory Pathogens Mediate the Association between Lung Function and Temperature in Cystic Fibrosis Joseph M. Collaco, MD, MPH, Karen S. Raraigh, MGC, Lawrence J. Appel, MD, MPH, and Garry R. Cutting, MD The Johns Hopkins Medical Institutions, Baltimore, MD

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Abstract Introduction—Mean annual ambient temperature is a replicated environmental modifier of cystic fibrosis (CF) lung disease with warmer temperatures being associated with lower lung function. The mechanism of this relationship is not completely understood. However, Pseudomonas aeruginosa, a pathogen that infects the lungs of CF individuals and decreases lung function, also has a higher prevalence in individuals living in warmer climates. We therefore investigated the extent to which respiratory pathogens mediated the association between temperature and lung function.

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Methods—Thirteen respiratory pathogens observed on CF respiratory cultures were assessed in multistep fashion using clustered linear and logistic regression to determine if any mediated the association between temperature and lung function. Analysis was performed in the CF TwinSibling Study (n=1730; primary population); key findings were then evaluated in the U.S. CF Foundation Data Registry (n=15,174; replication population). Results—In the primary population, three respiratory pathogens (Pseudomonas aeruginosa, mucoid P. aeruginosa, and methicillin-resistant Staphylococcus aureus) mediated the association between temperature and lung function. P. aeruginosa accounted for 19% of the association (p=0.003), mucoid P. aeruginosa for 31% (p=0.001), and MRSA for 13% (p=0.023). The same three pathogens mediated association in the replication population (7%, p

Respiratory pathogens mediate the association between lung function and temperature in cystic fibrosis.

Mean annual ambient temperature is a replicated environmental modifier of cystic fibrosis (CF) lung disease with warmer temperatures being associated ...
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