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Lung function from school age to adulthood in primary ciliary dyskinesia
Florian S Halbeisen1,2,3, Eva S L Pedersen1,3, Myrofora Goutaki1,4
1Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
The European Respiratory Journal
|March 18, 2022
Summary
Lung function in primary ciliary dyskinesia (PCD) generally declines from childhood to young adulthood, even with specialized care. Low BMI is linked to poorer lung function and further decline in PCD patients.
Area of Science:
- Pulmonology
- Genetics
- Pediatric Medicine
Background:
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder affecting cilia function, leading to chronic respiratory issues.
- Longitudinal data on lung function trajectories in individuals with PCD are limited, hindering understanding of disease progression.
- Early-onset symptoms and potential disease progression highlight the need for detailed lung function analysis in PCD patients.
Purpose of the Study:
- To describe lung function trajectories in children, adolescents, and young adults with PCD.
- To identify factors associated with lung function decline in PCD.
- To provide insights for improving the prognosis of PCD.
Main Methods:
- Multinational cohort study analyzing data from 486 patients with PCD.
- Repeated lung function measurements (FEV1, FVC, FEV1/FVC) from age 6 to 24 years.
- Mixed-effects linear regression models used to assess lung function changes and determinants.
Main Results:
- Average annual decline of -0.07 z-scores for FEV1 was observed across the cohort.
- Lung function improved in 21%, remained stable in 40%, and declined in 39% of patients.
- Low body mass index (BMI) was associated with poorer baseline lung function and accelerated decline.
Conclusions:
- Lung function in PCD patients declines on average throughout lung growth, from childhood to young adulthood.
- Lung function trajectories vary among individuals, with some showing improvement or stability.
- Developing strategies to reverse lung function decline is crucial for improving PCD prognosis.
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