To explore triheptanoin (C7 oil) compatibility with the ketogenic diet by evaluating EEG, and seizure rate, glycemia and ketosis in proven G1D patients receiving a ketogenic diet.
This is a single site, open label proof of principle exploratory trial to investigate the compatibility of C7 oil with the ketogenic diet in subjects diagnosed with G1D. The ketogenic diet will have been previously described by the patient's treating physician independently of this study and for clinical reasons. The ketogenic diet supplies over 50% of calories from fat, subjects, who are already tolerating over this much fat as part of their previously prescribed ketogenic diet, will replace 45% of their daily caloric intake with the triheptanoin for 24 hours, during a 48 hour inpatient stay. Subjects will have a continuous EEG to monitor for any potential C7 related changes in seizure before, during, and after triheptanoin oil ingestion.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
10
Dietary supplementation with triheptanoin
UTexasSouthwestern
Dallas, Texas, United States
Change in Ketosis (Beta-hydroxybutyrate Levels)
Change in Ketosis as measured by changes in blood beta-hydroxybutyric acid levels.
Time frame: Day 1, Day 4
Fraction of Subjects (of a Total of 10 Studied) Who Exhibit a Change in Observable Seizure Rate
The percent change in observable seizure rate is measured as the change in observable seizure numbers from baseline to Day 4. Seizure rate is defined as count of seizures per patient per day.
Time frame: Baseline (Day 1) - Day 4
Change in Glycemia
Change in Glycemia is measured as changes in blood glucose levels.
Time frame: Day 1 - Day 4
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