Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review

Adam J. Shapiro, Maimoona A. Zariwala, Thomas Ferkol, Stephanie D. Davis, Scott D. Sagel, Sharon D. Dell, Margaret Rosenfeld, Kenneth N. Olivier, Carlos Milla, Sam J. Daniel, Adam J. Kimple, Michele Manion, Michael R. Knowles, Margaret W. Leigh

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Abstract

Summary Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, rare lung disease resulting in chronic oto-sino-pulmonary disease in both children and adults. Many physicians incorrectly diagnose PCD or eliminate PCD from their differential diagnosis due to inexperience with diagnostic testing methods. Thus far, all therapies used for PCD are unproven through large clinical trials. This review article outlines consensus recommendations from PCD physicians in North America who have been engaged in a PCD centered research consortium for the last 10 years. These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long-term therapeutics in PCD patients.

Original languageEnglish
Pages (from-to)115-132
Number of pages18
JournalPediatric Pulmonology
Volume51
Issue number2
DOIs
StatePublished - Feb 1 2016

Keywords

  • PCD Foundation
  • PCD, kartagener
  • consensus statement
  • primary ciliary dyskinesia

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    Shapiro, A. J., Zariwala, M. A., Ferkol, T., Davis, S. D., Sagel, S. D., Dell, S. D., Rosenfeld, M., Olivier, K. N., Milla, C., Daniel, S. J., Kimple, A. J., Manion, M., Knowles, M. R., & Leigh, M. W. (2016). Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatric Pulmonology, 51(2), 115-132. https://doi.org/10.1002/ppul.23304