A Multi-center Study Examining Bronchial Hyperresponsiveness in Primary Ciliary Dyskinesia
Indiana University
Summary
The purpose of this study is to look at children with PCD and see if they have another condition called "bronchial hyperresponsiveness".
Description
The purpose of this study is to look at children with PCD and see if they have another condition called "bronchial hyperresponsiveness". This is when some of the air tubes ("bronchi") are very sensitive ("hyperresponsive") to certain things in the environment such as pollen, mold, pet dander, changes in temperature, and viruses. Children with PCD are often prescribed daily inhaled steroids to help make their air tubes less sensitive, but there are few research studies showing that children with PCD really need this medication. In this study, the investigators will simulate the above triggers…
Eligibility
- Age range
- 6+ years
- Sex
- All
- Healthy volunteers
- Yes
Inclusion Criteria: * Confirmed diagnosis of PCD per standard diagnostic criteria4 and positive genetics * Age greater than or equal to 6 years (no upper age limit) * Any gender or race * Able to perform pulmonary function testing (historical documentation of reversibility will be accepted) Exclusion Criteria: * history of current pneumothorax * inability to perform pulmonary function testing
Interventions
- ProcedureLung Function Testing
will include baseline spirometry (pre- and post- max bronchodilator). All testing will be done according to American Thoracic Society/European Respiratory Society guidelines. Participants will be asked to refrain from taking any asthma medications, including inhaled corticosteroids, short- and long-acting bronchodilators, leukotriene receptor antagonists, and long-acting muscarinic antagonists, for 24 hours prior to any spirometry. This activity will take place at a clinical research center at the respective participating institution.
- ProcedurePhlebotomy
will be obtained at visit 2. Up to ten (10) ml of blood will be collected for measurement of serum biomarkers of atopy, based on whether participants prefer to receive an allergy skin prick test or have antigen-specific IgE levels tested. In the event a subject refuses phlebotomy, historical results up to one year old may be used in lieu of prospective results. Any remaining blood samples will be banked either for use in future studies or in the event that additional serum biomarkers are added to this study.
- ProcedureAllergy skin prick testing
may be completed at visit 2. Subjects will be instructed to withhold first-generation antihistamines for 3 days and second-generation antihistamines for 7 days prior to the test. If patients prefer to have serum antigen-specific IgE levels run with the required serum biomarkers of atopy, then skin prick testing will be omitted.
- ProcedureMethacholine Challenge
Location
- Riley Hospital for ChildrenIndianapolis, Indiana