Status:
COMPLETED
Utilizing Hyperpolarized 129Xe Magnetic Resonance Imaging in Children With Primary Ciliary Dyskinesia
Lead Sponsor:
The Hospital for Sick Children
Collaborating Sponsors:
Provincial Health Services Authority British Columbia
Conditions:
Primary Ciliary Dyskinesia
Eligibility:
All Genders
6-18 years
Brief Summary
This study investigates the use of hyperpolarized 129Xe magnetic resonance imaging (MRI) in children with primary ciliary dyskinesia (PCD) in detecting ventilation defects. The investigators will esta...
Detailed Description
Primary Ciliary Dyskinesia (PCD) is an autosomal recessive inherited disorder caused by defects in ciliary structure and/or function. Prevention or delaying disease progression requires medical therap...
Eligibility Criteria
Inclusion
- Inclusion Criteria
- Diagnosis of PCD as having either (i) biallelic mutations in known PCD genes or (ii) classic transmission electron microscopy structural ciliary defect
- Informed consent and verbal assent (as appropriate) provided by the participant's parent or legal guardian and the participant
- Ages 6-18 years and able to perform reproducible spirometry and achieve a breath hold duration sufficient for MRI acquisition
- Exclusion Criteria
- Any other cardiac or respiratory disease
- Inability to perform a breath-hold of adequate duration for MRI acquisition
- Medical instability that would preclude the ability to undergo the required investigations
- FEV1 % predicted \<40% on any PFT within last 2 months at time of consent
- Use of supplementary oxygen
- Severe claustrophobia
- Pregnancy or lactation
- Presence of metal implants or other MRI contraindications
Exclusion
Key Trial Info
Start Date :
September 1 2021
Trial Type :
OBSERVATIONAL
Allocation :
ACTUAL
End Date :
June 30 2023
Estimated Enrollment :
16 Patients enrolled
Trial Details
Trial ID
NCT04858191
Start Date
September 1 2021
End Date
June 30 2023
Last Update
October 23 2023
Active Locations (1)
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1
Hospital for Sick Children
Toronto, Ontario, Canada, M5G1X8