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

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 test...

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Published inPediatric pulmonology Vol. 51; no. 2; pp. 115 - 132
Main Authors 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.
Format Journal Article
LanguageEnglish
Published United States Blackwell Publishing Ltd 01.02.2016
Wiley Subscription Services, Inc
John Wiley and Sons Inc
Subjects
Biopsy
Breath Tests
Chronic Disease
consensus statement
Disease Management
Genetic Testing
Humans
Influenza Vaccines - therapeutic use
kartagener
Kartagener Syndrome - diagnosis
Kartagener Syndrome - therapy
Microscopy, Electron
Microscopy, Video
North America
PCD
PCD Foundation
PCD, kartagener
Pneumococcal Vaccines - therapeutic use
primary ciliary dyskinesia
Respiratory Syncytial Virus Vaccines - therapeutic use
Respiratory Therapy - methods
State of the Art
consensus statement
PCD, kartagener
PCD Foundation
primary ciliary dyskinesia
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Summary: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. Pediatr Pulmonol. 2016;51:115–132. © 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc.
Bibliography:ark:/67375/WNG-35P1Z8TD-D
National Institutes of Health (NIH) - No. U54HL096458; No. 5R01HL071798
CTSA NIH/NCATS Colorado UL1TR000154
Intramural Research Program of NIH/NIAID
CTSA NIH/NCATS UNC ULTR000083
The Genetic Disorders of Mucociliary Clearance (U54HL096458) is a part of the NCATS Rare Diseases Clinical Research Network (RDCRN). RDCRN is an initiative of the Office of Rare Diseases Research (ORDR), NCATS, funded through a collaboration between NCATS and NHLBI
istex:4CA03C01E7206A7214AE5DFDFA46C3054FB5D6F5
ArticleID:PPUL23304
ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 14
ObjectType-News-1
ObjectType-Review-6
ObjectType-Feature-4
ObjectType-Conference-2
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ObjectType-Instructional Material/Guideline-5
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Michael R. Knowles and Margaret W. Leigh are co‐senior who contributed equally to this statement as senior authors.
Supported in part by the intramural research program of the NHLBI, NIH.
ISSN:8755-6863
1099-0496
1099-0496
DOI:10.1002/ppul.23304