A Multi-center Study Examining Bronchial Hyperresponsiveness in Primary Ciliary Dyskinesia

What this Trial Is About

This research aims to investigate children with Primary Ciliary Dyskinesia (PCD) to determine if they also have bronchial hyperresponsiveness, a condition where the air tubes in the lungs react strongly to environmental triggers like pollen, mold, pet dander, temperature changes, and viruses. Children with PCD often receive daily inhaled steroids to reduce airway sensitivity, but there is limited research confirming the need for this treatment. Participants will undergo a methacholine challenge test, where increasing doses of methacholine are inhaled to simulate airway triggers and measure breathing response through spirometry. If symptoms like shortness of breath or wheezing occur, albuterol will be given to open airways. The study includes at least two visits to the hospital, each lasting 2 to 2.5 hours, where breathing tests, medication inhalations, skin allergy testing, blood draws, and questionnaires will be completed. Visits one and two may be scheduled on the same day, and a third visit may be offered depending on eligibility. During the study, participants’ medical records will be reviewed for demographic and disease history. Researchers will measure bronchial hyperresponsiveness by comparing spirometry results before and after methacholine and bronchodilator use. The study may last up to one month for two visits or up to five months if a third visit is included. Safety is monitored throughout, and any discomfort from methacholine is temporary with no long-term effects expected.

Examining Bronchial Hyperresponsiveness in Primary Ciliary Dyskinesia