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Updated: Aug 9, 2025

High-speed Video Microscopy Analysis for First-line Diagnosis of Primary Ciliary Dyskinesia
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Lung Function in Children with Primary Ciliary Dyskinesia

Valentina Agnese Ferraro1, Raimondo Junior Castaldo1, Valentina Tonazzo1

  • 1Pediatric Respiratory Medicine and Allergy Unit, Women's and Children's Health Department, University of Padova, 35128 Padova, Italy.

Children (Basel, Switzerland)
|February 25, 2023
PubMed

Insights

Primary ciliary dyskinesia (PCD) lung function is often normal in children, but some show impairment. Lung Clearance Index may aid early detection of airway disease in PCD patients.

Area of Science:

  • Pediatric Pulmonology
  • Respiratory Medicine

Background:

  • Primary ciliary dyskinesia (PCD) impairs mucociliary clearance, leading to mucus and bacterial accumulation in airways.
  • Recurrent lower respiratory tract infections in PCD cause airway remodeling and lung function decline.
  • Understanding lung function in children with PCD is crucial for managing disease progression.

Purpose of the Study:

  • To review current data on lung function in children diagnosed with Primary Ciliary Dyskinesia.
  • To identify key risk factors associated with lung function impairment in pediatric PCD patients.

Main Methods:

  • A narrative review of published studies was conducted.
  • Searches were performed on MEDLINE/PubMed using terms related to PCD and pulmonary function tests.
  • Studies included were in English and focused on subjects aged 0-18 years.

Main Results:

  • Most recent studies indicate normal spirometry values in children with PCD.
  • Some studies report pulmonary impairment, highlighting the need for comprehensive assessment.
  • Lung Clearance Index shows promise in detecting peripheral airway disease and early-stage lung issues.

Conclusions:

  • Lung function trajectories in pediatric PCD patients exhibit significant heterogeneity.
  • Further prospective research is needed to track lung function from childhood to adulthood.
  • Investigating the impact of PCD phenotype, ciliary defect, and genetics on lung function is essential.
Abstract

Keywords:
lung functionprimary ciliary dyskinesiapulmonary function testspirometry

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