期刊
PEDIATRIC PULMONOLOGY
卷 51, 期 2, 页码 115-132出版社
WILEY
DOI: 10.1002/ppul.23304
关键词
primary ciliary dyskinesia; PCD; kartagener; consensus statement; PCD Foundation
资金
- National Institutes of Health (NIH) [U54HL096458, 5R01HL071798]
- Genetic Disorders of Mucociliary Clearance is a part of the NCATS Rare Diseases Clinical Research Network (RDCRN) [U54HL096458]
- RDCRN is an initiative of the Office of Rare Diseases Research (ORDR), NCATS
- CTSA NIH/NCATS UNC [ULTR000083]
- CTSA NIH/NCATS Colorado [UL1TR000154]
- Intramural Research Program of NIH/NIAID
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. (C) 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc.
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