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.We have established a consortium of 4 geographically-dispersed clinical sites that are designed to study rare diseases of the airways. The purpose of this longitudinal study protocol is to define age-related prevalence of phenotypic characteristics and the progression of key features of lung diseases in participants with one of these disorders, Primary Ciliary Dyskinsesia (PCD). In PCD, the abnormal structure and function of cilia results in impaired clearance of secretions and consequent obstruction and chronic recurrent infections in the airways, sinuses and middle ears.The clinical course of PCD lung disease is not well defined.This longitudinal study is designed to define a rate of progression of PCD lung function in participants between 5 and 18 year of age using spirometry. We will also systematically track other outcomes, including pathogens of the airways(assessed by respiratory cultures), age at onset and progression of airway damage and bronchiectasis (assessed by high-resolution tomography (HRCT) of the chest.
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