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Primary Ciliary Dyskinesia and Cystic FibrosisPrimary Ciliary Dyskinesia and Cystic Fibrosis: Different Diseases Require Different Treatment

Jane S. Lucas, BM, PhD; Mary Carroll, MD
Author and Funding Information

From the Southampton Primary Ciliary Dyskinesia and Cystic Fibrosis Centers, National Institute for Health Research Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton, National Health Service Foundation Trust.

Correspondence to: Jane S. Lucas, BM, PhD, University Hospital Southampton Foundation Trust, Tremona Rd, Mailpoint 803, Southampton, SO16 6YD, England; e-mail: jlucas1@soton.ac.uk


Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;145(4):674-676. doi:10.1378/chest.13-2590
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Extract

Orphan diseases are often managed according to evidence from similar, more common conditions. This should be avoided, since differences in pathophysiology, morbidity, and prognosis will likely lead to problems of treatment failure and lack of adherence. The prevalence of primary ciliary dyskinesia (PCD)1 is approximately one-fourth that of cystic fibrosis (CF) (one in 10,000 and one in 2,500, respectively), and it is, therefore, not particularly rare. Despite recent advances in PCD research and clinical infrastructure,2 there have been no randomized controlled trials, and current management guidelines are mostly limited to expert consensus extrapolated from evidence from CF.3

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