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Systemic Biomarkers of Brain Injury From Hyperammonemia
Sponsor: Children's National Research Institute
Summary
Ammonia is a waste product of protein and amino acid catabolism and is also a potent neurotoxin. High blood ammonia levels on the brain can manifest as cytotoxic brain edema and vascular compromise leading to intellectual and developmental disabilities. The following aims are proposed: Aim 1 of this study will be to determine the chronology of biomarkers of brain injury in response to a hyperammonemic (HA) brain insult in patients with an inherited hyperammonemic disorder. Aim 2 will be to determine if S100B, NSE, and UCHL1 are altered in patients with two other inborn errors of metabolism, Maple Syrup Urine Disease (MSUD) and Glutaric Acidemia (GA1).
Key Details
Gender
All
Age Range
7 Years - 18 Years
Study Type
OBSERVATIONAL
Enrollment
24
Start Date
2020-07-09
Completion Date
2027-05
Last Updated
2024-02-07
Healthy Volunteers
No
Conditions
Locations (1)
Children's National Research Institute
Washington D.C., District of Columbia, United States