Lung Ultrasound safe and effective tool for evaluating Pediatric Primary Ciliary Dyskinesia: Study

Primary ciliary dyskinesia (PCD) is a chronic disorder of mucociliary clearance that leads to recurrent respiratory infections and progressive structural lung disease. Lung ultrasound (LUS) is a radiation-free bedside imaging modality; however, its use in Primary ciliary dyskinesia is limited, and no validated scoring system is available. This study aimed to evaluate lung ultrasound findings in children with Primary ciliary dyskinesia; assess the relationship between lung ultrasound aeration score and clinical characteristics, pulmonary function parameters, and chest radiograph severity; and to describe ultrasound-detected atelectatic findings.

This prospective cross-sectional study included children with Primary ciliary dyskinesia evaluated at a tertiary center between December 2025 and February 2026. Lung ultrasound was performed using a standardized 12-zone scanning protocol and assessed using a conventional lung ultrasound aeration score (0–36). Atelectasis was recorded as a separate sonographic finding and was not incorporated into the scoring system. Chest radiographs were evaluated using the modified Chrispin–Norman score (CNS), and spirometric measurements were obtained in eligible patients. Associations between lung ultrasound aeration score, radiographic severity, and clinical parameters were analyzed. A total of 42 children with primary ciliary dyskinesia were included, of whom 24 (57.1%) were male. The median lung ultrasound aeration score was 1.0 (0.0–2.0), and the median modified CNS was 4.0 (2.0–6.0). A moderate positive correlation was observed between the lung ultrasound aeration score and CNS (r = 0.430, p = 0.005). The lung ultrasound aeration score also showed a moderate positive correlation with the number of respiratory exacerbations in the previous year (r = 0.342, p = 0.026) and with the number of bronchiectatic lobes (r = 0.490, p = 0.001). Atelectatic findings detected by LUS were associated with significantly higher CNS scores (p = 0.031), although they were not associated with higher lung ultrasound aeration scores.

Lung ultrasound is a valuable, radiation-free, bedside, and reproducible imaging modality for assessing structural lung involvement in children with Primary ciliary dyskinesia. The lung ultrasound aeration score correlates with radiographic severity and clinical disease burden, supporting its potential role as a complementary tool in clinical follow-up. Atelectatic findings detected by lung ultrasound appear clinically significant and are associated with greater radiographic severity. However, according to current guidelines, atelectasis should be documented as an individual sonographic finding rather than included in the scoring system. Taken together, these findings suggest that lung ultrasound may help assess both structural lung involvement and overall disease burden in children with primary ciliary dyskinesia.

Reference

Bilgiç, I., Çakar, M.K., Dinç, G.A. et al. Lung ultrasound in children with primary ciliary dyskinesia: is it able to detect structural and functional impairments?. Eur J Pediatr 185, 265 (2026). https://doi.org/10.1007/s00431-026-06923-5

Keywords:

Lung, Ultrasound, safe, effective tool, evaluating, Pediatric, Primary, Ciliary, Dyskinesia,Study, Bilgiç, I., Çakar, M.K., Dinç,