Abstract
There is considerable variability in the severity and progression of lung disease in primary ciliary dyskinesia (PCD).1-3 Few studies have related progression of lung function to the underlying ultrastructural defect.4,5 We investigated the age-related evolution of spirometry to identify associations between ultrastructural defects, gene mutations, BMI,6 and bacterial airway infections in a longitudinal study of adult and pediatric patients with PCD.
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