Examining Bronchial Hyperresponsiveness in Primary Ciliary Dyskinesia

Summary

The purpose of this study is to look at children with PCD and see if they have another condition called "bronchial hyperresponsiveness".

Conditions

• Primary Ciliary Dyskinesia
• Healthy

Interventions

PROCEDURE

Lung 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.

PROCEDURE

Phlebotomy

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.

PROCEDURE

Allergy 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.

PROCEDURE

Methacholine Challenge

If the subject does not demonstrate a bronchodilator response in FEV1 of 10% or greater, and does not have a historical MCT on file, MCT will be performed. Following inhalation of saline, methacholine (MCh) will be inhaled in quadrupling concentrations starting with 0.0625 mg/ml and continuing until the MCh concentration required for FEV1 to decrease by 20% from baseline (PD20) is achieved or a maximum MCh concentration of 16 mg/ml is inhaled.

Sponsors & Collaborators

• Indiana University

  lead OTHER

Study Design

Allocation

NON_RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Model

PARALLEL

Eligibility

Min Age

6 Years

Sex

ALL

Healthy Volunteers

Yes

Timeline & Regulatory

Start

2023-05-04

Primary Completion

2028-04-30

Completion

2028-04-30

Countries

• United States

Study Locations