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Postnatal airway growth in cystic fibrosis piglets

Authors :
Eric A. Hoffman
Daniel P. Cook
Ryan J. Adam
Linda S. Powers
Mallory R. Stroik
Peter J. Taft
Nicholas D. Gansemer
David A. Stoltz
David K. Meyerholz
Joseph Zabner
Mahmoud H. Abou Alaiwa
Mark J. Hoegger
James D. McMenimen
Michael J. Welsh
Drake C. Bouzek
Source :
Journal of Applied Physiology. 123:526-533
Publication Year :
2017
Publisher :
American Physiological Society, 2017.

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. NEW & NOTEWORTHY This CT scan-based study of airway morphometry in the cystic fibrosis (CF) postnatal period is unique, as analogous studies in humans are greatly limited for ethical and technical reasons. Findings such as reduced airway lumen area and irregular caliber suggest that airway growth and development are CF transmembrane conductance regulator-dependent and that airway growth defects may contribute to CF lung disease pathogenesis.

Details

ISSN :
15221601 and 87507587
Volume :
123
Database :
OpenAIRE
Journal :
Journal of Applied Physiology
Accession number :
edsair.doi.dedup.....43281f017f0f4b1e31039679efe0eb74
Full Text :
https://doi.org/10.1152/japplphysiol.00263.2017