Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis.

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 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 hyp

SUBMITTER: Chang EH 

PROVIDER: S-EPMC3449319 | biostudies-literature | 2012 Sep

REPOSITORIES: biostudies-literature