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

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 meth...

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Autores principales: Shapiro, Adam J., Zariwala, Maimoona A., Ferkol, Thomas, Davis, Stephanie D., Sagel, Scott D., Dell, Sharon D., Rosenfeld, Margaret, Olivier, Kenneth N., Milla, Carlos, Daniel, Sam J., Kimple, Adam J., Manion, Michele, Knowles, Michael R., Leigh, Margaret W.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4912005/
https://www.ncbi.nlm.nih.gov/pubmed/26418604
http://dx.doi.org/10.1002/ppul.23304
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author Shapiro, Adam J.
Zariwala, Maimoona A.
Ferkol, Thomas
Davis, Stephanie D.
Sagel, Scott D.
Dell, Sharon D.
Rosenfeld, Margaret
Olivier, Kenneth N.
Milla, Carlos
Daniel, Sam J.
Kimple, Adam J.
Manion, Michele
Knowles, Michael R.
Leigh, Margaret W.
author_facet Shapiro, Adam J.
Zariwala, Maimoona A.
Ferkol, Thomas
Davis, Stephanie D.
Sagel, Scott D.
Dell, Sharon D.
Rosenfeld, Margaret
Olivier, Kenneth N.
Milla, Carlos
Daniel, Sam J.
Kimple, Adam J.
Manion, Michele
Knowles, Michael R.
Leigh, Margaret W.
author_sort Shapiro, Adam J.
collection PubMed
description 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. Pediatr Pulmonol. 2016;51:115–132. © 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc.
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spelling pubmed-49120052016-09-23 Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review Shapiro, Adam J. Zariwala, Maimoona A. Ferkol, Thomas Davis, Stephanie D. Sagel, Scott D. Dell, Sharon D. Rosenfeld, Margaret Olivier, Kenneth N. Milla, Carlos Daniel, Sam J. Kimple, Adam J. Manion, Michele Knowles, Michael R. Leigh, Margaret W. Pediatr Pulmonol State of the Art 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. Pediatr Pulmonol. 2016;51:115–132. © 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc. John Wiley and Sons Inc. 2015-09-29 2016-02 /pmc/articles/PMC4912005/ /pubmed/26418604 http://dx.doi.org/10.1002/ppul.23304 Text en © 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc. This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs (http://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle State of the Art
Shapiro, Adam J.
Zariwala, Maimoona A.
Ferkol, Thomas
Davis, Stephanie D.
Sagel, Scott D.
Dell, Sharon D.
Rosenfeld, Margaret
Olivier, Kenneth N.
Milla, Carlos
Daniel, Sam J.
Kimple, Adam J.
Manion, Michele
Knowles, Michael R.
Leigh, Margaret W.
Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review
title Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review
title_full Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review
title_fullStr Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review
title_full_unstemmed Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review
title_short Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review
title_sort diagnosis, monitoring, and treatment of primary ciliary dyskinesia: pcd foundation consensus recommendations based on state of the art review
topic State of the Art
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4912005/
https://www.ncbi.nlm.nih.gov/pubmed/26418604
http://dx.doi.org/10.1002/ppul.23304
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