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Lung Function in Children with Primary Ciliary Dyskinesia. | LitMetric

Lung Function in Children with Primary Ciliary Dyskinesia.

Children (Basel)

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

Published: February 2023

AI Article Synopsis

  • Primary ciliary dyskinesia (PCD) leads to poor mucociliary clearance, causing mucus and bacteria buildup that can result in lung infections and respiratory issues in children.
  • A narrative review of recent research focused on lung function in PCD children, utilizing studies that examined pulmonary function tests and relevant keywords through MEDLINE/Pubmed.
  • Although most studies found normal spirometric values in PCD children, there is variability in lung function over time, highlighting a need for further research on the long-term effects of PCD and its influencing factors.

Article Abstract

Background: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance that results in accumulation of mucus and bacteria in the airways. Lower respiratory tract infections lead to airway remodeling and lung function impairment. The aim of our narrative review is to discuss available data on lung function in PCD children, focusing on risk factors for lung function impairment.

Methods: Relevant published studies searching MEDLINE/Pubmed are included in this narrative review, using these terms: "primary ciliary dyskinesia" and "pulmonary function test" or "spirometry" or "lung function". Filters were language (English) and age of study subjects (0-18 years).

Results And Conclusions: The majority of recent published studies showed normal spirometric values in PCD children, even if some authors described a pulmonary impairment. Together with spirometry, Lung Clearance Index has been applied for detecting peripheral airway disease, and it might have a role in early mild lung disease assessment. Studies on lung function trajectories after PCD diagnosis showed a significant heterogeneity, with some patients maintaining reasonably good lung function, whereas others showing a decline. Further studies are needed to analyze lung function prospectively from childhood into adulthood, and to evaluate whether lung function trajectories are affected by PCD clinical phenotype, ultrastructural ciliary defect or genetic background.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9955480PMC
http://dx.doi.org/10.3390/children10020290DOI Listing

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