Abstract: Primary ciliary dyskinesia syndrome (PCD) is a rare genetic disease with high heterogeneity, the diagnostic strategy of PCD is becoming more and more complete. However, the lack of prompt clinical managements for many patients resulted in severe consequences. This article will review on the recent advances of clinical managements of upper and lower respiratory tract and other systems to explore unified and prompt management strategy. In addition, azithromycin and gene editing are promising treatment approaches.

Key words: 　primary ciliary dyskinesia; treatment approaches; clinical managements; gene therapy