Postnatal Airway Growth in Cystic Fibrosis Piglets

Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel cause cystic fibrosis (CF). The leading cause of death in the CF population is lung disease. Increasing evidence suggests that in utero airway development is CFTR-dependent and that developmental abnormalities may contribute to CF lung disease. However, relatively little is known of postnatal CF airway growth, largely because such studies are limited in people. Therefore, we examined airway growth and lung volume in a porcine model of CF. We hypothesized that CF pigs would have abnormal postnatal airway growth. To test this hypothesis, we performed CT-based airway and lung volume measurements in three-week-old non-CF and CF pigs. We found that three-week-old CF pigs had tracheas of reduced caliber and irregular shape. Their bronchial lumens were reduced in size proximally but not distally, were irregularly shaped, and had reduced distensibility. Our data suggest that lack of CFTR results in aberrant postnatal airway growth and development, which could contribute to CF lung disease pathogenesis.

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