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In vivo Evaluation of Mucociliary Clearance in Mice
Published on: December 18, 2020
Bronchiectasis in children with recurrent pneumonia: an immunopathological damage associated with secondary ciliary
M Pifferi1, D Caramella, V Ragazzo
1Department of Pediatrics, University of Pisa, Pisa, Italy. m.pifferi@med.unipi.it
Insights
Ciliary dysmotility is common in children with bronchiectasis, correlating with disease severity. This suggests a link between impaired ciliary function and the development of this lung condition.
Area of Science:
- Pediatric Pulmonology
- Respiratory Cell Biology
Background:
- Recurrent pneumonia in children can indicate underlying respiratory issues.
- Bronchiectasis, a chronic lung condition, affects airway structure and function.
- Ciliary function is crucial for clearing respiratory secretions.
Purpose of the Study:
- To evaluate ciliary motion patterns in children with bronchiectasis.
- To compare ciliary function in affected children versus healthy controls.
- To determine the relationship between ciliary dysmotility and bronchiectasis severity.
Main Methods:
- High-resolution computed tomography (HRCT) to diagnose and score bronchiectasis.
- Assessment of ciliary ultrastructure, beat frequency, and motion patterns.
- Comparison of findings in 51 children with bronchiectasis and 30 healthy children.
Main Results:
- Bronchiectasis was identified in 31 of 51 children.
- Ciliary dysmotility was present in 64.5% of children with bronchiectasis and 76.5% overall.
- Ciliary dysmotility significantly correlated with HRCT scores (p=0.02) and absent ciliary motion (p=0.005).
Conclusions:
- Ciliary dysmotility is associated with the presence and severity of bronchiectasis in children.
- A potential self-maintaining mechanism between ciliary dysfunction and airway lesions exists.
- Further research is needed to clarify the causal relationship between ciliary dysmotility and bronchiectasis.
Abstract:
The aim of this study is to assess ciliary motion patterns in children with bronchiectasis unrelated to cystic fibrosis or primary ciliary dyskinesia. In 51 children with recurrent pneumonia, high resolution computed tomography (HRCT) was carried out to detect and score bronchiectasis. Moreover, ciliary ultrastructure, beat frequency and motion pattern were evaluated and compared to those observed in 30 healthy children. Bronchiectasis at HRCT was found in 31/51 children. Ciliary dysmotility was found in 20/31 children with bronchiectasis (64.5%). Overall, ciliary dysmotility was found in 39/51 patients (76.5%). Ciliary dysmotility showed a significant correlation with the HRCT score (p=0.02). Absent motion in some fields was found in 44/51 patients (86.3%) and this also showed significant correlation with the HRCT score (p=0.005). The specificity and sensitivity of ciliary dysmotility as an indicator of bronchiectasis was 74.3% and 83.3% respectively. The positive predictive value was 93.5%, and negative predictive value was 50%. Ciliary dysmotility, in children with recurrent airways infections, correlates with the presence and severity of bronchiectasis. Whether ciliary dysmotility is a cause or a consequence of anatomical lesion is a matter of speculation. Very likely there is an amplification and self-maintaining mechanism between the two events which may lead to more serious disease.
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