Novel Use of Combined High-Speed Video Microscopy and Nasal Nitric Oxide Screening for Identification of Primary Ciliary Dyskinesia in Adult Bronchiectasis
Indiana University
Summary
The purpose of this study is to help determine how often primary ciliary dyskinesia (PCD) is present but undiagnosed in adults with bronchiectasis.
Description
Bronchiectasis is a chronic respiratory disease characterized by irreversible bronchial dilatation, impaired mucociliary clearance, and recurrent infection. Despite comprehensive evaluation, 40-80% of adults with non-cystic fibrosis (CF) bronchiectasis have no identifiable cause. This diagnostic absence limits opportunities for targeted therapy, individualized prognostication, and potential genetic counseling. Primary ciliary dyskinesia (PCD) is an inherited disorder of motile cilia that leads to chronic otosinopulmonary disease. Nearly 100% of affected individuals develop bronchiectasis by a…
Eligibility
- Age range
- 18+ years
- Sex
- All
- Healthy volunteers
- No
Inclusion Criteria: * Adults (≥18) with CT-confirmed bronchiectasis Exclusion Criteria: * Pre-existing diagnosis of cystic fibrosis * Pre-existing diagnosis of primary ciliary dyskinesia * Inability to perform testing * Refusal of consent
Interventions
- ProcedureNasal Nitric Oxide Measurement
Subjects will have their nasal nitric oxide measured using commercially available chemiluminescence analyzer in accordance with American Thoracic Society/European Respiratory Society guidelines
- ProcedureNasopharyngeal (Nasal) Samples
Human nasal epithelial cells will be collected via nasal swab, nasal curettage or nasal brush from each nostril.
Location
- Riley Hospital for ChildrenIndianapolis, Indiana