Journal article
Postnatal airway growth in cystic fibrosis piglets
Journal of applied physiology (1985), Vol.123(3), pp.526-533
09/01/2017
DOI: 10.1152/japplphysiol.00263.2017
PMCID: PMC5625074
PMID: 28620056
Abstract
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.
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
- Title: Subtitle
- Postnatal airway growth in cystic fibrosis piglets
- Creators
- Ryan J Adam - Department of Internal MedicineMahmoud H Abou Alaiwa - Department of Internal MedicineDrake C Bouzek - Department of Internal MedicineDaniel P Cook - Department of Internal MedicineNicholas D Gansemer - Department of Internal MedicinePeter J Taft - Department of Internal MedicineLinda S Powers - Department of Internal MedicineMallory R Stroik - Department of Internal MedicineMark J Hoegger - Department of Internal MedicineJames D McMenimen - Department of Internal MedicineEric A Hoffman - Department of Internal MedicineJoseph Zabner - Department of Internal MedicineMichael J Welsh - Department of Internal MedicineDavid K Meyerholz - Department of PathologyDavid A Stoltz - Department of Internal Medicine
- Resource Type
- Journal article
- Publication Details
- Journal of applied physiology (1985), Vol.123(3), pp.526-533
- Publisher
- American Physiological Society; Bethesda, MD
- DOI
- 10.1152/japplphysiol.00263.2017
- PMID
- 28620056
- PMCID
- PMC5625074
- ISSN
- 8750-7587
- eISSN
- 1522-1601
- Grant note
- STOLTZ14XX0 / CFF DP2 HL-117744; T32 HL-007638; P01 HL-051670; P01 HL-091842 / HHS | NIH | National Heart, Lung, and Blood Institute (NHBLI) T32 GM007337 / NIH
- Alternative title
- Airway Growth in CF Piglets Adam RJ et al
- Language
- English
- Date published
- 09/01/2017
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Stead Family Department of Pediatrics; Pathology; Neurosurgery; Internal Medicine
- Record Identifier
- 9984025369702771
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