Emerging and Established Pulmonary Function Measurements of Primary Ciliary Dyskinesia: One of These Things Is Not Like the Others
Annals of the American Thoracic Society
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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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