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Postnatal airway growth in cystic fibrosis piglets
- 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.
- Subjects :
- 0301 basic medicine
Pathology
medicine.medical_specialty
Cystic Fibrosis
Swine
Physiology
Population
Cystic Fibrosis Transmembrane Conductance Regulator
Bronchi
Computed tomography
Cystic fibrosis
Animals, Genetically Modified
Pathogenesis
03 medical and health sciences
0302 clinical medicine
Physiology (medical)
medicine
Animals
Lung volumes
education
Lung
Cause of death
education.field_of_study
medicine.diagnostic_test
business.industry
respiratory system
medicine.disease
respiratory tract diseases
Trachea
030104 developmental biology
Animals, Newborn
030228 respiratory system
In utero
Airway
business
Research Article
Subjects
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