Treatment recommendations in Primary Ciliary Dyskinesia

Paediatr Respir Rev. 2016 Mar;18:39-45. doi: 10.1016/j.prrv.2015.10.002. Epub 2015 Oct 20.


Primary Ciliary Dyskinesia (PCD) is a rare heterogenic disorder leading to significant respiratory morbidity. Health-care providers who treat PCD must familiarize themselves with recommended treatment strategies. However, most of the treatments recommended in PCD have been extrapolated from cystic fibrosis (CF) and non-CF bronchiectasis literature. Mainstays of therapy are reviewed in detail, and should include at a minimum: regular airway clearance, routine microbiological surveillance, antibiotic treatment for pulmonary exacerbation, and health vaccinations. This review summarizes both medical and surgical pulmonary treatment considerations, as well as recommendations for the integration of non-pulmonary subspecialty care in the management of PCD.

Keywords: Bronchiectasis; Mucociliary clearance; Primary ciliary dyskinesia; Treatments.

Publication types

  • Review

MeSH terms

  • Disease Management*
  • Humans
  • Kartagener Syndrome / therapy*
  • Practice Guidelines as Topic*