This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.The PI's have established a consortium of 4 geographically-dispersed clinical research sites that are designed to study rare diseases of the airways. In this protocol one of these disorders Primary Ciliary Dyskinesia (PCD) will be studied. In Primary Ciliary Dyskinesia (PCD) the abnormal structure and function of cilia results in impaired clearance of secretions and consequent obstruction and chronic recurrent infection in the airways, sinuses and middle ears. The clinical manifestations include chronic/recurrent bronchitis andpneumonia, chronic/recurrent sinusitis and chronic otitis media. Chronic airway infection ultimately results in structural damage to the airways, known as bronchiectasis, and loss of lung function. Although it seems likely that the onset of PCD airway disease occurs early in childhood, as has been reported for cystic fibrosis (CF), the clinical course of PCD lung disease is not well-defined, nor, is the time course of emergence of specific microbial pathogens, or the age of onset and rate of progression of airway diseaseand bronchiectasis.This longitudinal study is designed to define the rate of progression of PCD lung function in subjects between 5-18 years of age, track pathogens infecting the airways, and age at onset and progression of airway damage and bronchiectasis.
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