PREVALENCE OF BRONCHIAL HYPERRESPONSIVENESS IN PRIMARY CILIARY DYSKINESIA

E
Evans Machogu, MD

Primary Investigator

Overview

All people have tiny hairs that help move mucus through their air tubes. When these hairs do not form or do not work properly it can cause the air tubes to get clogged with mucus, which can cause PCD. 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 a medicine to breathe in every day called steroids to help make their air tubes less sensitive, but there are not very many research studies showing that children with PCD really need this medication.

Description

Participants will be asked to complete two visits and be given the option to complete a third visit. During these visits, researchers will take participants' vitals, give them an overall physical examination, and give participants a breathing test called spirometry.

Eligibility

You may be eligible for this study if you meet the following criteria:

  • Conditions:
    primary ciliary dyskinesia
  • Age: Between 6 Years - 100 Years
  • Gender: All

Inclusion Criteria:
  • Confirmed diagnosis of PCD per standard diagnostic criteria4 and positive genetics, OR probable diagnosis of PCD based on clinical symptoms, nasal nitric oxide values and/or ciliary biopsy but with negative genetics
  • 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

Updated on 06 May 2024. Study ID: 16122, PPUL-IIR-BHR-PCD
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