Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis
EH Chang, AA Pezzulo, DK Meyerholz… - The …, 2012 - Wiley Online Library
The Laryngoscope, 2012•Wiley Online Library
Abstract Objectives/Hypothesis: Chronic sinusitis is nearly universal in humans with cystic
fibrosis (CF) and is accompanied by sinus hypoplasia (small sinuses). However, whether
impaired sinus development is a primary feature of loss of the cystic fibrosis transmembrane
conductance regulator (CFTR) or a secondary consequence of chronic infection remains
unknown. Our objective was to study the early pathogenesis of sinus disease in CF. Study
Design: Animal/basic science research. Methods: Sinus development was studied in a …
fibrosis (CF) and is accompanied by sinus hypoplasia (small sinuses). However, whether
impaired sinus development is a primary feature of loss of the cystic fibrosis transmembrane
conductance regulator (CFTR) or a secondary consequence of chronic infection remains
unknown. Our objective was to study the early pathogenesis of sinus disease in CF. Study
Design: Animal/basic science research. Methods: Sinus development was studied in a …
Objectives/Hypothesis
Chronic sinusitis is nearly universal in humans with cystic fibrosis (CF) and is accompanied by sinus hypoplasia (small sinuses). However, whether impaired sinus development is a primary feature of loss of the cystic fibrosis transmembrane conductance regulator (CFTR) or a secondary consequence of chronic infection remains unknown. Our objective was to study the early pathogenesis of sinus disease in CF.
Study Design
Animal/basic science research.
Methods
Sinus development was studied in a porcine CF model.
Results
Porcine sinus epithelia expressed CFTR and exhibited transepithelial anion transport. Disruption of the CFTR gene eliminated both. Sinuses of newborn CF pigs were not infected and showed no evidence of inflammation, yet were hypoplastic at birth. Older CF pigs spontaneously developed sinus disease similar to that seen in humans with CF.
Conclusions
These results define a role for CFTR in sinus development and suggest the potential of the CF pig as a genetic model of CF‐sinus disease in which to test therapeutic strategies to minimize sinus‐related CF morbidity.
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