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Updated: Jul 5, 2025
High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
Published on: January 19, 2022
Bronchodilator responsiveness in children with primary ciliary dyskinesia
Elias Seidl1,2, Dvir Gatt1, Wallace B Wee1
1Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada.
Insights
Bronchodilator response testing in children with primary ciliary dyskinesia may identify those at risk for faster lung disease progression. This finding aids in early intervention for pediatric respiratory conditions.
Area of Science:
- Pediatric Pulmonology
- Rare Genetic Diseases
- Respiratory Medicine
Background:
- Primary ciliary dyskinesia (PCD) commonly presents with reversible airway obstruction in children.
- The diagnostic utility of bronchodilator (BD) response testing in routine spirometry for PCD is not well-established.
Purpose of the Study:
- To evaluate the diagnostic value of bronchodilator response testing in children with primary ciliary dyskinesia.
- To determine if BD response testing can predict lung disease progression in pediatric PCD patients.
Main Methods:
- Retrospective analysis of pulmonary function test results from children with PCD.
- Inclusion of spirometry with BD response testing (pre- and post-BD measurements) from outpatient visits.
- Comparison of BD response data with baseline and follow-up spirometry to assess lung function changes.
Main Results:
- 18.1% of pulmonary function tests in children with PCD showed a positive BD response.
- Positive BD response correlated with a significant absolute change in % predicted FEV1 from Baseline to Visit pre-BD.
- Children with positive BD responses exhibited a greater annual decline in FEV1 % predicted and were more likely to initiate antimicrobial therapy.
Conclusions:
- Positive bronchodilator response in children with PCD may indicate a higher risk of accelerated lung disease progression.
- BD testing could be a valuable tool for identifying at-risk pediatric patients with PCD.
- Findings suggest potential for earlier therapeutic interventions in PCD management.
Background:
Reversible airway obstruction is common in children with primary ciliary dyskinesia. However, the diagnostic value of adding bronchodilator (BD) response testing to routine spirometry is unclear.
Methods:
This is a retrospective analysis of pulmonary function test results obtained from children with primary ciliary dyskinesia seen as outpatients at the Hospital for Sick Children, Toronto. Spirometry results were collected for every appointment with BD response testing ("Visit", with pre-BD and post-BD measurements) as well as for the previous ("Baseline") and following ("Follow-up") encounters.
Results:
A positive BD response was seen in 86 out of 474 (18.1%) of the pulmonary function tests from 82 children with primary ciliary dyskinesia. BD responsiveness was associated with a significant absolute change (±sd) in % predicted forced expiratory volume in 1 s (FEV1) from Baseline to Visit pre-BD (-6.5±10.3%, p<0.001), but not from Baseline to Follow-up (0.4±10.8, p=0.757). Antimicrobial therapy was initiated more commonly following a Visit with a positive BD response (OR 3.8, 95% CI 2.2-6.6) compared to no BD response. Children with a positive BD response had a greater annual decline in FEV1 % predicted compared to those with no BD response (-0.9% per year versus -0.5% per year, p<0.001). The annual decline in FEV1 % predicted was greater in children with multiple compared to one measured positive BD responses (-1.3% per year versus -0.6% per year, p<0.001) and in those not treated with antibiotic therapy following a positive BD response compared to those treated with antibiotics (-1.1% versus -0.6%, p<0.001).
Conclusion:
A positive BD response in children with primary ciliary dyskinesia may help identify those at risk for accelerated lung disease progression.
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