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

Treatment recommendations in Primary Ciliary Dyskinesia.

Author information

1
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, Kansas.
2
Section of Pediatric Pulmonology, Allergy and Sleep Medicine, Riley Hospital for Children at Indiana University Health, Indiana University School of Medicine, Indianapolis, Indiana.
3
Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada, University of Toronto, Toronto, Ontario, Canada. Electronic address: sharon.dell@sickkids.ca.

Abstract

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

PMID:
26586601
DOI:
10.1016/j.prrv.2015.10.002
[Indexed for MEDLINE]

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