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Abstract Multiple cell types have been implicated in pathogenesis of pulmonary fibrosis with pericytes emerging as a new focus due to their role in promoting fibrotic remodeling. Fibrotic environment changes normal pericyte functions, but transcriptional programs regulating pericyte transition towards fibrotic state remain unclear.
1. Introduction Primary ciliary dyskinesia (PCD) is a rare genetic disorder affecting upper and lower airway motile cilia, causing recurrent chronic respiratory infections, starting in infancy [1]. So far, the incidence rate of PCD in children is between 1/20,000 and 1/10,000 [2]. The prevalence of PCD in children with recurrent respiratory infections is up to 5%, and in children with bronchiectasis, it is estimated to be 26% [3,4].
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