Application of bronchoscopy in children with primary ciliary dyskinesia.
- 1Department of Respiratory Intervention, Jinan Children's Hospital, Jinan Children's Hospital, No.23796, Huaiyin District, Jinan City, Shangdong Province, 250000, China.
- 2Department of Respiratory Intervention, Jinan Children's Hospital, Jinan Children's Hospital, No.23796, Huaiyin District, Jinan City, Shangdong Province, 250000, China. [email protected].
Abstract
Background
To evaluate the value of bronchoscopy in the clinical diagnosis and management of primary ciliary dyskinesia (PCD).Methods
A retrospective analysis was conducted on six genetically confirmed PCD children admitted to the Department of Respiratory Intervention at Jinan Children's Hospital between October 2020 and August 2024. Bronchoscopic procedures were performed, including collection of specimens for microbiological testing, bronchoalveolar lavage (BAL) for cytological analysis, and interventional therapies such as local drug/saline lavage and forceps-assisted clearance. Mucosal biopsies were attempted in selected cases, but ultrastructural analysis was not consistently reliable.Results
Bronchoscopy revealed copious secretions and a characteristic fish-bone appearance (defined as tortuous, dilated segmental bronchi with prominent transverse ridging of the mucosa, resembling the spine of a fish) in the airways of all six PCD patients. Based on endoscopic assessment and microbiological results from BAL fluid, the anti-infective treatment regimen was adjusted for one patient. All patients underwent forceps-assisted clearance and lavage therapy, with two patients requiring two lavage sessions due to severe infection. BALF cytology revealed a predominantly neutrophilic and lymphocytic inflammation. No severe complications occurred during or after the procedures.Conclusion
In this small cohort of six pediatric PCD patients, bronchoscopy appeared to be a safe and potentially useful integrated approach, combining disease assessment, and therapeutic intervention (airway clearance). The procedure also provided valuable insights into the airway inflammatory and microbiological phenotype. However, given the preliminary nature of these findings and the limited sample size, larger prospective studies are needed to confirm the role of bronchoscopy in routine PCD management.
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BibTeX
@article{wang2026application,
title = {{Application of bronchoscopy in children with primary ciliary dyskinesia.}},
author = {Xinfeng Wang and Jinfeng Zhang},
journal = {BMC pediatrics},
year = {2026},
month = aug,
doi = {10.1186/s12887-026-07462-6},
url = {https://europepmc.org/article/MED/42972320},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Application of bronchoscopy in children with primary ciliary dyskinesia.
AU - Xinfeng Wang
AU - Jinfeng Zhang
PY - 2026
DA - 2026/08/05
JO - BMC pediatrics
DO - 10.1186/s12887-026-07462-6
UR - https://europepmc.org/article/MED/42972320
AB - Background To evaluate the value of bronchoscopy in the clinical diagnosis and management of primary ciliary dyskinesia (PCD).Methods A retrospective analysis was conducted on six genetically confirmed PCD children admitted to the Department of Respiratory Intervention at Jinan Children's Hospital between October 2020 and August 2024. Bronchoscopic procedures were performed, including collection of specimens for microbiological testing, bronchoalveolar lavage (BAL) for cytological analysis, and interventional therapies such as local drug/saline lavage and forceps-assisted clearance. Mucosal biopsies were attempted in selected cases, but ultrastructural analysis was not consistently reliable.Results Bronchoscopy revealed copious secretions and a characteristic fish-bone appearance (defined as tortuous, dilated segmental bronchi with prominent transverse ridging of the mucosa, resembling the spine of a fish) in the airways of all six PCD patients. Based on endoscopic assessment and microbiological results from BAL fluid, the anti-infective treatment regimen was adjusted for one patient. All patients underwent forceps-assisted clearance and lavage therapy, with two patients requiring two lavage sessions due to severe infection. BALF cytology revealed a predominantly neutrophilic and lymphocytic inflammation. No severe complications occurred during or after the procedures.Conclusion In this small cohort of six pediatric PCD patients, bronchoscopy appeared to be a safe and potentially useful integrated approach, combining disease assessment, and therapeutic intervention (airway clearance). The procedure also provided valuable insights into the airway inflammatory and microbiological phenotype. However, given the preliminary nature of these findings and the limited sample size, larger prospective studies are needed to confirm the role of bronchoscopy in routine PCD management.
SN - 1471-2431
ER -