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Clinical care of children with primary ciliary dyskinesia

Jane S Lucas1, Mikkel Christian Alanin2, Samuel Collins1

  • 1a Primary Ciliary Dyskinesia Centre, NIHR Biomedical Research Centre , University of Southampton and University Hospital Southampton , Southampton , United Kingdom.

Expert Review of Respiratory Medicine
|July 27, 2017
PubMed

Insights

Primary ciliary dyskinesia (PCD) management lacks robust evidence, often relying on cystic fibrosis data. Future research emphasizes well-designed trials for effective PCD treatment strategies.

Area of Science:

  • Pediatric Pulmonology
  • Rare Genetic Disorders

Background:

  • Primary ciliary dyskinesia (PCD) is a rare, heterogeneous genetic disorder affecting cilia function.
  • Symptoms include neonatal respiratory distress, chronic cough, bronchiectasis, rhinosinusitis, and hearing loss; 50% exhibit situs inversus.

Purpose of the Study:

  • To review current evidence for managing pediatric Primary ciliary dyskinesia (PCD).
  • To highlight the limitations of extrapolating treatment guidelines from other diseases like cystic fibrosis (CF).
  • To emphasize the critical need for PCD-specific clinical trials.

Main Methods:

  • Searched PubMed and Cochrane databases for publications on pediatric PCD management.
  • Analyzed existing literature to identify evidence gaps.

Main Results:

  • A significant paucity of evidence exists for effective Primary ciliary dyskinesia (PCD) treatments.
  • Current management guidelines are often based on data from cystic fibrosis (CF), which may be inappropriate due to differing pathophysiology.
  • Reliance on non-PCD data risks treatment failure and poor adherence.

Conclusions:

  • The evidence base for treating Primary ciliary dyskinesia (PCD) is currently poor.
  • Management strategies often inappropriately extrapolate from cystic fibrosis (CF) or chronic rhinosinusitis.
  • Ongoing international research efforts aim to develop evidence-based guidelines through well-designed clinical trials for children with PCD.
Abstract

Keywords:
PCDPrimary ciliary dyskinesiakartagenermanagementmicrobiologyrare diseasesreviewtherapytransition

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