Air trapping and airflow obstruction in newborn cystic fibrosis piglets

Ryan Adam, Andrew Michalski, Christian Bauer, MH Abou Alaiwa, Thomas Gross, Maged Awadalla, Drake Bouzek, Nicholas Gansemer, Peter Taft, Mark Hoegger, Amit Diwakar, Matthias Ochs, Joseph Reinhardt, Eric Hoffman, Reinhard Beichel, David Meyerholz, David Stoltz

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43 Citations (Scopus)

Abstract

Rationale: Air trappingandairflow obstruction are being increasingly identifiedin infants with cystic fibrosis. Thesefindings arecommonly attributed to airway infection, inflammation, and mucus buildup. Objectives: To learn if air trappingandairflow obstruction are present before the onset of airway infection and inflammation in cystic fibrosis. Methods: On the day they are born, piglets with cystic fibrosis lack airway infection and inflammation. Therefore, we used newborn wild-type piglets and piglets with cystic fibrosis to assess air trapping, airway size, and lung volume with inspiratory and expiratory X-ray computed tomography scans. Micro-computed tomography scanning was used to assess more distal airway sizes. Airway resistance was determined with a mechanical ventilator. Mean linear intercept and alveolar surface area were determined using stereologic methods. Measurements and Main Results: On the day they were born, piglets with cystic fibrosis exhibited air trapping more frequently than wild-type piglets (75% vs. 12.5%, respectively). Moreover, newborn piglets with cystic fibrosis had increased airway resistance that was accompanied by luminal size reduction in the trachea, mainstem bronchi, and proximal airways. In contrast, mean linear intercept length, alveolar surface area, and lung volume were similar between both genotypes. Conclusions: The presence of air trapping, airflow obstruction, and airway size reduction in newborn piglets with cystic fibrosis before the onset of airway infection, inflammation, and mucus accumulation indicates that cystic fibrosis impacts airway development. Our findings suggest that early airflow obstruction and air trapping in infants with cystic fibrosis might, in part, be caused by congenital airway abnormalities.

Original languageEnglish
Pages (from-to)1434-1441
Number of pages8
JournalAmerican journal of respiratory and critical care medicine
Volume188
Issue number12
DOIs
Publication statusPublished - 15 Dec 2013

Keywords

  • CFTR
  • Computed tomography
  • Congenital
  • Infant
  • Porcine

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    Adam, R., Michalski, A., Bauer, C., Abou Alaiwa, MH., Gross, T., Awadalla, M., Bouzek, D., Gansemer, N., Taft, P., Hoegger, M., Diwakar, A., Ochs, M., Reinhardt, J., Hoffman, E., Beichel, R., Meyerholz, D., & Stoltz, D. (2013). Air trapping and airflow obstruction in newborn cystic fibrosis piglets. American journal of respiratory and critical care medicine, 188(12), 1434-1441. https://doi.org/10.1164/rccm.201307-1268OC