• Ryan J Adam, Andrew S Michalski, Christian Bauer, Mahmoud H Abou Alaiwa, Thomas J Gross, Maged S Awadalla, Drake C Bouzek, Nicholas D Gansemer, Peter J Taft, Mark J Hoegger, Amit Diwakar, Matthias Ochs, Joseph M Reinhardt, Eric A Hoffman, Reinhard R Beichel, David K Meyerholz, and David A Stoltz.
• 1 Department of Internal Medicine.
• Am. J. Respir. Crit. Care Med. 2013 Dec 15; 188 (12): 1434-41.

RationaleAir trapping and airflow obstruction are being increasingly identified in infants with cystic fibrosis. These findings are commonly attributed to airway infection, inflammation, and mucus buildup.ObjectivesTo learn if air trapping and airflow obstruction are present before the onset of airway infection and inflammation in cystic fibrosis.MethodsOn 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 ResultsOn 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.ConclusionsThe 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.

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