This is a single-site, open-label, phase II trial of C7, a food supplement or medical food, for the development of treatment outcome measures for glucose transporter type I deficiency (G1D). The primary outcome measures are: 1. Safety and tolerability of C7 as measured by changes in comprehensive blood work, including lipid and free fatty acid panels, self-reported side effects and clinical exam; 2. Changes in brain metabolic rate by MRI and EEG measurements during C7 treatment; and 3. Maintenance of ketosis in G1D patients on ketogenic diet, as measured by serial ketone levels during treatment initiation.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
BASIC_SCIENCE
Masking
NONE
Triheptanoin (C7 oil) is a 7-carbon medium chain triglyceride.
UT Southwestern Medical Center
Dallas, Texas, United States
Change in risk for Metabolic Syndrome
Triglycerides, lipid levels, and cholesterol are measured to evaluate change in risk of metabolic syndrome
Time frame: Baseline, 6 months, 9 months
Change on Biomarkers
EEG and brain metabolic rate will be measured at three time points. Changes in these biomarkers indicate the utilization of triheptanoin in brain metabolism
Time frame: Baseline, 6 months, 9 months
Change in Ketosis
Safety blood work (described in the first outcome measure) is measured along with ketone levels and EEG to confirm that triheptanoin is safe and does not break ketosis in patients on the ketogenic diet
Time frame: baseline, 6 months, 9 months
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