Emerging and Established Pulmonary Function Measurements of Primary Ciliary Dyskinesia: One of These Things Is Not Like the Others
Document Type
Article
Publication Date
12-15-2018
Journal
Annals of the American Thoracic Society
Volume
15
Issue
12
First Page
1396
Last Page
1398
URL with Digital Object Identifier
https://doi.org/10.1513/AnnalsATS.201809-642ED
Abstract
Primary ciliary dyskinesia (PCD) is an inherited genetic disorder that causes progressive lung disease manifesting early in childhood (1), with impaired bronchial mucus transport causing frequent infection and mucus plugging, bronchiectasis, bronchial wall thickening, and ventilation abnormalities (2). PCD is often underdiagnosed or diagnosed late during the disease course (3), because currently used diagnostic tests are relatively insensitive to subclinical impairment. This knowledge gap is the driver of studies aimed at earlier PCD detection and diagnosis, when the opportunity to change overall outcomes is heightened.
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Notes
This is an author-accepted version of the manuscript initially published in the Annals of the American Thoracic Society. The final published version is available at: https://doi.org/10.1513/AnnalsATS.201809-642ED