Postnatal airway growth in cystic fibrosis piglets

Ryan J. Adam, Mahmoud H.Abou Alaiwa, Drake C. Bouzek, Daniel P. Cook, Nicholas D. Gansemer, Peter J. Taft, Linda S. Powers, Mallory R. Stroik, Mark J. Hoegger, James D. McMenimen, Eric A. Hoffman, Joseph Zabner, Michael J. Welsh, David K. Meyerholz, David A. Stoltz

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Mutations in the gene encoding the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) anion channel cause 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 about postnatal CF airway growth, largely because such studies are limited in humans. 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 3-wk-old non-CF and CF pigs. We found that 3-wk-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.

Original languageEnglish
Pages (from-to)526-533
Number of pages8
JournalJournal of Applied Physiology
Volume123
Issue number3
DOIs
StatePublished - Sep 2017

Keywords

  • Airway distensibility
  • Computed tomography
  • Cystic fibrosis
  • Porcine

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