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High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
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Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study
Florian S Halbeisen1, Myrofora Goutaki1,2, Ben D Spycher1,2
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
The European Respiratory Journal
|July 28, 2018
Summary
Primary ciliary dyskinesia (PCD) impairs lung function from childhood. While similar to cystic fibrosis (CF) in young children, PCD shows less severe impairment in young adults compared to CF.
Area of Science:
- Pulmonary Medicine
- Genetics
- Pediatrics
Background:
- Primary ciliary dyskinesia (PCD) is often considered milder than cystic fibrosis (CF).
- Limited research exists on lung function in PCD patients.
- Previous studies on PCD lung function have been small and few.
Purpose of the Study:
- To compare lung function in PCD patients to normal reference values.
- To compare lung function in PCD patients to cystic fibrosis (CF) patients.
- To assess factors associated with lung function in PCD.
Main Methods:
- Spirometry data from 991 international PCD Cohort patients were analyzed.
- Z-scores and % predicted values for FEV1 and FVC were calculated using GLI 2012.
- Linear regression models assessed associations with patient characteristics.
Main Results:
- PCD patients exhibited reduced lung function compared to reference values across all ages and sexes.
- Lung function impairment was least pronounced in children aged 6-9 years.
- PCD patients showed similar FEV1 reduction as CF patients in children (6-9 years), but less reduction in young adults (18-21 years).
Conclusions:
- PCD impacts lung function early in life.
- Early, standardized care is crucial for PCD patients.
- PCD lung function is less severe than CF in young adults.
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