Bone Health in Young Individuals with Primary Ciliary Dyskinesia: Insights from a Comparison with Cystic Fibrosis and Healthy Controls
An erratum to this article was published (Horm Res Paediatr, 2026 ↗)
Authors' conclusion
Skeletal health appears compromised in young people with PCD. Despite mostly normal densitometry, tibial bone microarchitecture was altered. This study positions bone health as a research priority in PCD and supports the need for larger confirmatory studies. </p>.
Study context
- Population
- Individuals with primary ciliary dyskinesia (PCD) or cystic fibrosis (CF) aged 12–20 years
- Exposure
- PCD vs healthy controls and CF
- PCDn = 15
- CFn = 45
- Outcome
- Bone phenotype: BMD (whole body, lumbar spine) by DXA and tibial bone microarchitecture by HR-pQCT
Methodological detailsexploratory, matched healthy controls (one-to-one by pubertal stage and sex)
- Exploratory
- Matched healthy controls (one-to-one by pubertal stage and sex)
Main finding
As reported in the abstract
Bone phenotype in PCD vs controls
- No differences in DXA areal bone mineral density (BMD) or high-resolution peripheral quantitative computed tomography (HR-pQCT) total volumetric BMD between PCD and controls
- Reduced cortical thickness, area and BMD at ultra-distal tibia in PCD vs controls
Observational data: associations, not effects.
Further findings
PCD vs CF
- PCD and CF did not differ; PCD bone phenotype more closely resembled pancreatic-insufficient than pancreatic-sufficient CF
Adjustment for body mass index (BMI) or lean mass
- Dimensional cortical deficits largely explained by BMI or lean mass, especially in CF, but remained reduced in PCD after adjustment
Lung function and bone outcomes in PCD
- Lung function did not clearly correlate with bone outcomes in PCD
Original publication
Bone Health in Young Individuals with Primary Ciliary Dyskinesia: Insights from a Comparison with Cystic Fibrosis and Healthy Controls. Horm Res Paediatr. Published 17 November 2025. doi:10. 1159/ 000549604
Composed by AI from the published abstract and checked against it. How Briefs are made →