Daily oral supplementation with Vitamin E (alpha-tocopherol) for 2 weeks.

Hyperinsulinism-Hyperammonemia Syndrome

Subjects will take an oral Vitamin E (alpha-tocopherol) supplement once daily with a fat-containing meal for 2 weeks. The dose will be based on subject age (150 IU if 1-3 years old, 300 IU if 4-8 years old, 450 IU if 9-17 years old, 600 IU if >17 years old). Formulations include 50 IU/mL liquid and 200 IU capsules. The liquid formulation will be used for subjects who will receive <600 IU daily, or for any subjects who prefer liquid medication to capsules.

Vitamin E

Children's Hospital of Philadelphia

Vitamin E Supplementation in Hyperinsulinism/Hyperammonemia Syndrome

The following symptoms will be scored as either "none" (did not occur), "mild" (minimal symptoms, no treatment needed), "moderate" (symptoms requiring treatment at home or as an outpatient, or "severe" (symptoms requiring hospitalization or emergency room visit, or life-threatening or potentially life-threatening symptoms):
Seizure, Headache, Vision change/blurred vision, Weakness, Fatigue, Nausea, Vomiting, Diarrhea, Stomach pain, Constipation, Bruising, Bleeding, Rash, Itching, Other

change in fasting plasma alpha-tocopherol concentration before versus after Vitamin E supplementation

change in delta-glucose concentration (fasting plasma glucose - nadir plasma glucose during oral protein tolerance test) before versus after Vitamin E supplementation

change in fasting plasma glucose concentration before versus after Vitamin E supplementation

change in nadir plasma glucose concentration during oral protein tolerance test before versus after Vitamin E supplementation

change in fasting plasma insulin concentration before versus after Vitamin E supplementation

change in peak plasma insulin concentration during oral protein tolerance test before versus after Vitamin E supplementation

change in delta-plasma insulin concentration (peak plasma insulin - fasting plasma insulin during oral protein tolerance test) before versus after Vitamin E supplementation

change in fasting plasma ammonia concentration before versus after Vitamin E supplementation

change in delta-plasma ammonia concentration (plasma ammonia at 60 minutes - fasting plasma ammonia during oral protein tolerance test) before versus after Vitamin E supplementation

frequency of hypoglycemia (plasma glucose <70 mg/dL) detected on home glucose meter

Amanda M Ackermann

Attending Physician, Assistant Professor

University of Pennsylvania

Lawson Wilkins Pediatric Endocrine Society

This open-label tolerability and feasibility pilot clinical study will use a before-and-after design, with blood tests and fasting oral protein tolerance test performed prior to and after 2 weeks of daily oral Vitamin E supplementation in individuals with HI/HA syndrome.

Subjects will complete 45 minutes of aerobic exercise twice during a 7 day period. Exercise will be graded based on the participant's relative capacity determined at the screening visit. Each exercise session will be followed by 60 minutes of monitored resting recovery.

Subjects will complete 45 minutes of anaerobic exercise twice during a 7 day period. Each exercise session will be followed by 60 minutes of monitored resting recovery.

Subjects will perform no exercise during this week.

Hypoglycemia

Subjects will exercise on a treadmill for 45 minutes twice during the intervention week. Subjects will exercise at a fixed intensity level to a target heart rate (±10%) based on the heart rate achieved at 60% of their VO2max determined at screening. The speed and grade of the treadmill will be adjusted by trained research personnel with a goal of keeping participants within their target heart rate range for the entire 45 minutes.

Aerobic Exercise

Subjects will participate in resistance exercises for 45 minutes twice during the intervention week. Subjects will perform multiple-joint exercises with slow to moderate lifting velocity, for 1-3 sets per exercise at a weight that can be lifted for 8-12 repetitions (~60-80% of 1-repetition max). The exercises may include leg press, bench press, leg extension, leg flexion and seated row. Subjects will perform the exercises through the full range of motion. Between each set of repetitions, there would be a 2 minute rest period. The duration of the exercise testing would be approximately 45 minutes.

Resistance Exercise

Oregon Health and Science University

Effect of Hypoglycemia on Sleep

A Randomized, Three-way, Cross-over Study to Assess the Impact of Nocturnal Hypoglycemia on Sleep in Patients With Type 1 Diabetes

The mean duration of time in hypoglycemia in minutes (defined as sensor glucose less than 70 mg/dl) will be calculated using sensor glucose data acquired by the Dexcom G4 for 16 hours after the beginning of exercise.

The mean duration of time in euglycemia in minutes (defined as sensor glucose between 70-180 mg/dl) will be calculated using sensor glucose data acquired by the Dexcom G4 for 16 hours after the beginning of exercise.

The mean duration of time in euglycemia in minutes (defined as capillary blood glucose between 70-180 mg/dl) will be calculated using blood glucose data acquired by the Contour Next blood glucose (BG meter.

Using data acquired from an Actigraph activity monitor, mean duration of time in minutes of wake after sleep onset for the 16 hours after the beginning of exercise.

The mean amplitude of glucose excursions will be calculated using sensor glucose data acquired by the Dexcom G4.

Using data acquired from an Actigraph activity monitor, mean duration of time in minutes that subject is asleep.

Using data acquired from an Actigraph activity monitor, mean duration of time in minutes that subject is in bed.

Using data acquired from an Actigraph activity monitor, mean duration of time in minutes associated with the first epoch of the first 10 minute period of immobility starting from lights out time.

Using data acquired from an Actigraph activity monitor, a ratio of total sleep time to assumed sleep time multiplied by 100.

Using data acquired from an Actigraph activity monitor, mean of continuous blocks of length greater than or equal to 1 epoch in which each epoch is scored as wake in the assumed sleep period.

Using data acquired from an Actigraph activity monitor, mean time in minutes of wake after sleep onset divided by the number of wake bouts.

Using data acquired from an Actigraph activity monitor, mean of continuous blocks of length greater than or equal to 1 epoch in which each epoch is scored as sleep in the assumed sleep period.

Using data acquired from an Actigraph activity monitor, mean time in minutes of total sleep time divided by the number of sleep bouts.

Number of rescue carbohydrate treatments administered by the subject through study completion.

Measuring the number of grams of carbohydrates consumed by the subjects through study completion.

Measuring the number of units of insulin administered to the subject by his/her insulin pump through study completion.

Using data acquired from an Actigraph activity monitor, mean duration of time in minutes of activity by the subject through study completion.

The mean duration of time in hypoglycemia in minutes (defined as sensor glucose below 50 mg/dl) will be calculated using sensor glucose data acquired by the Dexcom G4.

The mean duration of time in hypoglycemia in minutes (defined as capillary blood glucose below 50 mg/dl) will be calculated using capillary blood glucose data acquired by the Contour Next BG meter.

The mean duration of time in hypoglycemia in minutes (defined as capillary blood glucose below 70 mg/dl) will be calculated using capillary blood glucose data acquired by the Contour Next BG meter.

The mean duration of time in hyperglycemia in minutes (defined as sensor glucose greater than 180 mg/dl) will be calculated using sensor glucose data acquired by the Dexcom G4.

The mean duration of time in hyperglycemia in minutes (defined as blood glucose greater than 180 mg/dl) will be calculated using glucose data acquired by the Contour Next BG meter.

hyperinsulinism/seizures

Vitamin E Supplementation in Hyperinsulinism/Hyperammonemia Syndrome

Effect of Hypoglycemia on Sleep

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