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The impact of Primary Ciliary Dyskinesia on the upper respiratory tract

Lucy C Morgan1, Catherine S Birman2

  • 1Sydney Medical School, University of Sydney, NSW; Department of Thoracic Medicine, Concord Hospital, Sydney, NSW; Australian School of Advanced Medicine, Macquarie University, Sydney, NSW.

Paediatric Respiratory Reviews
|February 23, 2016
PubMed

Insights

Primary Ciliary Dyskinesia (PCD) causes persistent ear and sinus infections due to impaired cilia function. This review highlights diagnostic challenges and management strategies for these common PCD complications.

Area of Science:

  • Otolaryngology
  • Genetics
  • Respiratory Medicine

Background:

  • Primary Ciliary Dyskinesia (PCD) is a genetic disorder affecting motile cilia function.
  • Ciliated epithelium lines the respiratory tract, making it susceptible to PCD-related complications.
  • Neonatal onset of persistent secretion retention and infection in the ears, nose, and sinuses is a hallmark of PCD.

Purpose of the Study:

  • To review the complexities in diagnosing Chronic Rhinosinusitis (CRS) and Chronic Otitis Media with Effusion (ChOME) in PCD patients.
  • To discuss the significant morbidity associated with CRS and ChOME in the context of PCD.
  • To present current evidence-based best practices for managing CRS and ChOME in individuals with PCD.

Main Methods:

  • Literature review focusing on diagnostic criteria for CRS and ChOME in PCD.
  • Analysis of studies detailing the impact of CRS and ChOME on PCD patient morbidity.
  • Synthesis of current clinical guidelines and research on PCD management.

Main Results:

  • Diagnosing CRS and ChOME in PCD presents unique challenges due to overlapping symptoms.
  • Untreated CRS and ChOME contribute substantially to the overall morbidity of PCD.
  • Evidence-based management strategies are crucial for improving outcomes in PCD patients with these conditions.

Conclusions:

  • Accurate diagnosis and effective management of CRS and ChOME are vital for PCD patient care.
  • Clinicians need to be aware of the complexities and best practices for managing these common PCD complications.
  • Improved management of otolaryngologic issues can mitigate the burden of disease in Primary Ciliary Dyskinesia.

Keywords:
Chronic otitis media with effusionChronic rhinosinusitisPrimary ciliary dyskinesia

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