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High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
Published on: January 19, 2022
Respiratory Bacterial Epidemiology at Three Primary Ciliary Dyskinesia Clinical Centers
Amanda Ziminski1, Madsen Zimbric1, Harlan McCaffery1
1Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI, United States.
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 3 pediatric PCD Centers and 1 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. Pseudomonas aeruginosa period prevalence at pediatric Centers A, B, and C was 37%, 32%, and 47%, respectively (P = .55). In contrast, pediatric Haemophilus influenzae prevalence (P = .001) and chronic H. influenzae infection rates (P < .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. Pseudomonas 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.
Insights
In primary ciliary dyskinesia (PCD), enhanced infection prevention and control (IPC) strategies did not significantly alter Pseudomonas aeruginosa prevalence. However, these measures were associated with lower Haemophilus influenzae rates in pediatric patients.
Area of Science:
- Respiratory Medicine
- Infectious Diseases
- Rare Genetic Disorders
Background:
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder causing chronic oto-sino-pulmonary infections.
- Pseudomonas aeruginosa (P. aeruginosa) is a common pathogen in PCD, similar to cystic fibrosis (CF).
- Enhanced infection prevention and control (IPC) strategies, successful in CF, have not been evaluated in PCD.
Purpose of the Study:
- To characterize the bacterial epidemiology, specifically P. aeruginosa, across different PCD Clinical Centers.
- To investigate the impact of varying IPC strategies on bacterial prevalence in PCD.
- To test the hypothesis that P. aeruginosa prevalence is higher in PCD centers with less stringent IPC measures.
Main Methods:
- Retrospective observational study analyzing period prevalence and chronic infection rates of P. aeruginosa and other respiratory microorganisms.
- Data collected from three pediatric PCD Centers and one adult PCD Center.
- Statistical analysis included Fisher's exact test for between-center comparisons and Bayesian mixed-effects logistic regression for age-adjusted analyses.
Main Results:
- P. aeruginosa period prevalence was similar across pediatric centers (37%-47%), regardless of IPC strategy.
- Pediatric centers with enhanced IPC showed significantly lower Haemophilus influenzae prevalence and chronic infection rates compared to standard precautions.
- P. aeruginosa prevalence increased around age 10 in pediatric patients, while H. influenzae prevalence decreased with age; nearly 85% of adults had a history of P. aeruginosa infection.
Conclusions:
- CF-IPC protocols may not influence P. aeruginosa epidemiology in PCD as they do in CF.
- P. aeruginosa prevalence in PCD might be higher than previously recognized.
- Variations in IPC strategies were not significantly associated with differences in P. aeruginosa rates across pediatric PCD centers, but showed an association with H. influenzae.
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