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Bacterial infections in patients with primary ciliary dyskinesia: Comparison with cystic fibrosis

Christiaan Dm Wijers1, James F Chmiel1, Benjamin M Gaston1

  • 11 Department of Pediatrics, Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

Chronic Respiratory Disease
|October 31, 2017
PubMed

Insights

Primary ciliary dyskinesia (PCD) involves impaired airway clearance due to ciliary defects. This review examines bacterial infections in PCD, comparing them to cystic fibrosis (CF) and discussing treatment strategies.

Area of Science:

  • Pulmonary Medicine
  • Genetics
  • Microbiology

Background:

  • Primary ciliary dyskinesia (PCD) is a genetic disorder affecting mucociliary clearance.
  • Recurrent respiratory infections are a hallmark of PCD, yet its specific airway microbiology is not well understood.
  • Current knowledge often relies on comparisons with cystic fibrosis (CF), despite differing disease mechanisms.

Purpose of the Study:

  • To review the current understanding of bacterial infections in primary ciliary dyskinesia (PCD).
  • To compare PCD airway microbiology with that of cystic fibrosis (CF).
  • To discuss current and future therapeutic strategies for managing recurrent bacterial infections in PCD patients.

Main Methods:

  • Literature review and synthesis of existing research on PCD and CF airway microbiology.
  • Analysis of common bacterial pathogens in PCD, including Pseudomonas aeruginosa, Staphylococcus aureus, and Moraxella catarrhalis.
  • Examination of treatment approaches for bacterial infections in PCD.

Main Results:

  • PCD is characterized by impaired mucociliary clearance due to ciliary dysfunction.
  • Specific bacterial profiles in PCD airways are understudied, with frequent extrapolation from CF data.
  • Key pathogens include Pseudomonas aeruginosa, Staphylococcus aureus, and Moraxella catarrhalis.

Conclusions:

  • Understanding PCD-specific airway microbiology is crucial for effective treatment.
  • Comparing PCD with CF provides insights but highlights the need for PCD-focused research.
  • Developing targeted treatment strategies is essential for improving patient care and outcomes.
Keywords:
CFPCDairwaysinfectionsmicrobiology

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