What question did this study set out to answer?

This study aims to identify risk factors for bronchiectasis in children with primary ciliary dyskinesia.

May 20, 2026American Journal of Respiratory and Critical Care Medicine0 citations

D91-07 Excessive Immune and Inflammatory Activation in Primary Ciliary Dyskinesia: Association With Bronchiectasis in Children

DLD LuSichuan University WYW YangSichuan University LCL ChenSichuan University

Key Points

This study aims to identify risk factors for bronchiectasis in children with primary ciliary dyskinesia.
Retrospective analysis of 73 children with primary ciliary dyskinesia divided into bronchiectasis (n=24) and non-bronchiectasis (n=49) groups.
Logistic regression to identify independent factors associated with bronchiectasis.
Cytokine levels in bronchoalveolar lavage fluid compared between the two groups, and transcriptome sequencing performed on airway organoids from both groups.
PCD+BE children had a higher incidence of premature birth (P = 0.022).
Age identified as an independent risk factor for bronchiectasis with an OR of 1.49 (95% CI 1.02 to 2.17, P = 0.040).
Cytokine analysis showed significant elevations in G-CSF, IL-6, and other markers in PCD+BE children with all P-values < 0.05.

Abstract

Abstract Background and Objectives Primary ciliary dyskinesia (PCD) frequently leads to bronchiectasis in children, significantly impacting prognosis, yet the underlying mechanisms remain unclear. This study investigates risk factors for bronchiectasis in PCD children. Methods 73 children diagnosed with PCD were retrospectively enrolled and divided into two groups according to whether they had bronchiectasis (PCD+BE, n = 24) or not (PCD-BE, n = 49). Logistic regression analysis was performed to identify independent factors associated with bronchiectasis. We analyzed cytokine level in bronchoalveolar lavage fluid (BALF) between two groups. We constructed airway organoids from PCD+BE and PCD-BE children and completed the transcriptome sequencing to evaluate the molecular expression patterns. Results PCD+BE children had a higher incidence of premature birth (P = 0.022). The age was identified as an independent risk factor for bronchiectasis in PCD children (OR, 1.49; 95% CI 1.02 to 2.17, P = 0.040). Cytokine analysis revealed significant elevations in G-CSF (P = 0.004), ICAM-1 (P = 0.010), IL-6 (P = 0.006), IL-6R (P = 0.037), MCP-1 (P = 0.025), MIP-1a (P = 0.025), MIP-1d (P = 0.037), and TIMP-2 (P = 0.037) in the BALF of PCD+BE children. Transcriptome sequencing of airway organoids revealed significant upregulation of gene sets related to immune response, inflammation, and airway repair in PCD+BE children. Conclusions Age as well as excessive immune and inflammatory activation are associated with the presence of bronchiectasis in PCD children. Early diagnosis, prompt intervention, and appropriate immune regulation may delay the presence of bronchiectasis in PCD children. This abstract is funded by: the National Natural Science Foundation of China

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