Abstract
Bronchiectasis is a chronic pulmonary disease affecting the conducting air ways of the lung, which may result from congenital disorders that affect ciliary motility. The disease is being recognised with increasing frequency around the world. Patients with bronchiectasis show permanent enlargement of peripheral bronchi accompanied by repeated respiratory infections, disabling productive cough and shortness of breath, resulting in loss of lung function. Mouse models of reduced mucociliary clearance have failed to display signs of bronchiectasis in multiple studies, hindering the development of targeted therapies. In this brief report we present the detection and quantification of bronchiectasis in TAp73 knockout mice using synchrotron radiation-based free-propagation phase contrast CT imaging, allowing the study of bronchiectasis in a pre-clinical and translational setting for the assessment of novel treatment strategies.
Clinical Relevance: Murine models of reduced mucociliary clearance have been established to investigate their pathobiology and develop novel treatment approaches but failed to develop visible airway enlargements. Synchrotron free-propagation phase contrast CT imaging is an innovative, sensitive, non-destructive in-situ technique, that allows for three-dimensional ultra-high-resolution detection of bronchiectasis in murine models of impaired mucociliary clearance.
Footnotes
This manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.
Conflict of interest: Dr. Wagner has nothing to disclose.
Conflict of interest: Dr. Dullin has nothing to disclose.
Conflict of interest: Dr. Andreas reports personal fees from Boehringer Ingelheim, personal fees from LaxoSmithKline, other from AstraZeneca, other from Berlin Chemie, other from Chiesi, other from Novartis, outside the submitted work;.
Conflict of interest: Dr. Lize reports to be an employee at Bayer AG.
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- Received September 1, 2020.
- Accepted December 7, 2020.
- Copyright ©ERS 2021
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