Abstract
Genetic ion transport defects, like cystic fibrosis (CF), are known to cause chronic obstructive lung disease. However, it is not known whether those defects could affect early lung development. To answer this question, we estimated quantitatively the 3D-lung structure and studied the airway architecture of neonatal mice with CF‐like lung disease. Lungs of βENaC‐Tg mice and control littermates were obtained at day 3, inflated at 20 cm water column, critical point dried, and scanned by synchrotron radiation-based X-ray tomographic microscopy. After segmentation of air versus tissue, the diameter of the surrounding airspace was determined for each air-voxel using a local thickness map algorithm. In the resulting 3D airspace diameter map, the number of voxels with the same diameter value yields the total volume of airspace of the same diameter. In neonatal βENaC‐Tg lungs we observed a larger volume-weighted mean airspace diameter (60.5±1.5 vs 51.0±4.4 mm, p=0.01) than in controls. The peaks of the airspace diameter histograms appeared at a larger diameter in βENaC‐Tg than in control lungs (54.5 vs 42.8 mm, p<0.001, Fig.1). The larger airspace size in neonatal βENaC‐Tg mice may be caused by prenatal mucus obstruction and pulmonary overextension influencing fetal branching morphogenesis. A possible developmental defect could shed further light on the pathophysiology and clinical management in ion transport disorders.
Footnotes
Cite this article as: European Respiratory Journal 2021; 58: Suppl. 65, OA1611.
This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2021