Abstract Rationale Primary ciliary dyskinesia (PCD) is a rare congenital disorder of motile ciliary dysfunction leading to chronic oto-sino-pulmonary infections including Pseudomonas aeruginosa (PA). Standardized enhanced infection prevention and control (IPC) measures have significantly reduced the prevalence and transmission of PA and other bacterial pathogens among patients with cystic fibrosis (CF) but practices vary widely across PCD clinical centers. No studies have adequately described bacterial epidemiology at PCD clinical centers with different IPC practices. Our objective was to investigate the bacterial epidemiology at three pediatric PCD centers employing different IPC strategies. Our hypothesis was that there would be greater prevalence of PA at centers without enhanced IPC measures. Methods This was a retrospective observational study exploring the prevalence of microorganisms between 2016 and 2024 at three pediatric PCD centers and one adult center. Prevalence rates were defined as the proportion of patients at a center who ever tested positive for each microorganism. Deidentified demographic and microbiological data were entered into a shared REDCap database. Proportions of patients ever infected were compared using Fischer’s exact test. Longitudinal analysis of infection status was assessed using mixed effects logistic regression models. Results There were 41 pediatric patients included from center A (no enhanced IPC), 25 from center B (clinic cohorting), and 14 from center C (CF-type enhanced IPC). An additional 15 adults were included from center A but not included in the comparative analysis. Bacterial prevalence of PA was 37%, 32% and 47% at centers A, B and C, respectively. Prevalence of Staphylococcus aureus was significantly lowest at center B and prevalence of HI was significantly highest at center A. No significant differences were observed for Stenotrophomonas and Streptotoccus pneumoniae. Mixed effects logistic regression modeling, adjusting for age and repeated measures, predicted a greater odds of HI at center A. When cultures across all centers, including the adult center, were assessed by age, the age-based prevalence of PA increased with age starting at age 10, whereas the age-based prevalence of (HI) and other organisms remained mostly unchanged. Conclusions Bacterial prevalence of PA was similar across centers with different IPC practices but the prevalence of HI was significantly highest at a center with no enhanced IPC. These preliminary findings at small centers suggest that IPC measures might not have the expected effect on bacterial epidemiology in PCD. Larger, multi-centers studies and the developing PCD Foundation national registry are required to investigate these preliminary findings. This abstract is funded by: Not funded
Ziminski et al. (Fri,) studied this question.