Respiratory bacterial epidemiology at three primary ciliary dyskinesia clinical centers

Abstract Background Primary ciliary dyskinesia (PCD) is a rare congenital disorder marked by impaired motile ciliary function, resulting in chronic oto-sino-pulmonary infections, including Pseudomonas aeruginosa . While enhanced infection prevention and control (IPC) practices have successfully reduced P. aeruginosa prevalence and transmission in cystic fibrosis (CF), comparable IPC strategies have not been evaluated in PCD. This study aimed to characterize bacterial epidemiology across PCD Clinical Centers, hypothesizing that P. aeruginosa prevalence would be higher at centers lacking enhanced IPC measures. Methods We performed a retrospective observational study assessing the period prevalence and chronic infection rates of P. aeruginosa and other respiratory microorganisms at three pediatric PCD Centers and one adult Center. Between-center comparisons of patients ever infected status used Fisher’s exact test, with age-adjusted repeated-measures analyses conducted via Bayesian mixed-effects logistic regression. Results The cohort included 41 pediatric patients from Center A (Standard Precautions), 25 from Center B (clinic cohorting), and 30 from Center C (enhanced IPC based on CF Foundation Guidelines, CF-IPC). An additional 13 adult patients from Center A were included for descriptive analysis only. P. aeruginosa period prevalence at pediatric Centers A, B, and C was 37%, 32%, and 47%, respectively (p = 0.55). In contrast, pediatric Haemophilus influenzae prevalence (p=0.001) and chronic H. influenzae infection rates (p0.0001) were significantly higher at Center A compared with Centers B and C. Age-stratified analysis across all pediatric Centers demonstrated increasing P. aeruginosa prevalence beginning around age 10, while H. influenzae prevalence declined with age. Nearly 85% of adults had a history of P. aeruginosa infection. Conclusions Our findings suggest that CF- IPC protocols may not exert the same influence on bacterial epidemiology in PCD as they do in CF. P. aeruginosa may be more prevalent in PCD than previously recognized, and variations in IPC strategies were not significantly associated with differences in P. aeruginosa rates across pediatric PCD Centers.