RARE DISEASES CLINICAL RESEARCH NETWORK
Rare Bronchiectatic Diseases Consortium |  |
Rare disease insights driving progress in bronchiectasis, a progressive pulmonary condition marked by chronic infection and airway inflammation. | |
Principal Investigators
University of North Carolina at Chapel Hill
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TOP PUBLICATION
JOURNAL: American Journal of Respiratory and Critical Care Medicine
Bronchiolar disease is a group of conditions that damages small air tubes in the lungs called bronchioles. In people with asthma, bronchiolar dysfunction can exacerbate symptoms and make them difficult to control. However, not much is known about how asthmatic bronchiolar disease starts and spreads at the molecular level.
In this study, researchers explored the epithelial biology of asthmatic bronchioles. The team evaluated peripheral lung tissues from severe asthmatics, fatal asthmatics, and controls.
Results reveal that asthmatic bronchioles exhibit a type-2 inflammation (T2)-driven proximalization associated with mucus plugging. Findings also suggest that asthmatic bronchioles contain cellular niches which perpetuate T2-initiated epithelial remodeling.
What was your consortium's most significant achievement from the past year?
Joseph Hatch, MPH, Project Manager, RBDC: Key start-up activities for our new consortium have been accomplished in the previous year such that we should be poised to initiate our primary natural history study relatively soon into year 2 of the award.
EARLY CAREER INVESTIGATOR SPOTLIGHT
What are your research goals?
Dr. Michaels: To better understand and support development of treatments for primary ciliary dyskinesia.