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.

Group case: modified ketogenic diet to reduce at least 20% saturated fat, up> 50% of the acid supply monounsaturated, increasing> 50% polyunsaturated fatty acid content and a lower ratio w6 / w3 at least 50% compared to classic diet used by the control group

Epilepsy

Cardiovascular Disease

Non-alcoholic Fatty Liver Disease

Quality of Life

ketogenic diet

Nagila Raquel Teixeira Damasceno

Impact of Ketogenic Diet on Lipoproteins in Refractory Epilepsy

Classic Ketogenic Diet and Modified: Evaluation of the Therapeutic Potential and Impact on the Oxidative Profile, Lipidomic, Inflammatory and Size of Lipoproteins in Children and Adolescents With Refractory Epilepsy

age, income, disease, parent´s education, use of drugs and supplements, type of seizures, questions about quality of life

phase angle mass by impedance bioeletrical

Food record applied during 3 days to each time

Concentration of B-hydroxybutirate in plasma. Determination with kit Ranbut (Randox Laboratories Limited, Reino Unido).

Concentration in plasma. Determination with kit NEFAS(Randox Laboratories Limited, Reino Unido)

Determination of antioxidant concentration in plasma by HPLC

Determination in plasma by mass spectrometry

Patricia Azevedo de Lima

Ph.D. Student

University of Sao Paulo

A single intragastric dose of dl-α-tocopheryl acetate (Aquasol E®) 50 IU/kg.

Sterile water in volume equal to that of the comparator drug.

Infant, Newborn

Infant, Small for Gestational Age

Infant, Very Low Birth Weight

Infant, Premature

Intracranial Hemorrhages

A single intragastric dose of dl-α-tocopheryl acetate 50 IU/kg.

Sterile water in volume equal to that of the comparator drug

Placebo

University of Alabama at Birmingham

Stanford University

Yale University

Emory University

Indiana University

University of Iowa

Tufts Medical Center

Wayne State University

University of New Mexico

RTI International

Duke University

Case Western Reserve University, Rainbow Babies and Children's Hospital

Brown University, Women & Infants Hospital of Rhode Island

University of Texas Southwestern Medical Center at Dallas

University of Texas Health Science Center at Houston

University of Utah

Vitamin E for Extremely Preterm Infants

Single-Dose Vitamin E for Prevention of Mortality and Morbidity in Extremely Preterm Infants: Pilot Study

Blood samples will be tested for tocopherol levels at baseline, 24 hours, and 7 days.

tocopherol/seizures

Vitamin E Supplementation in Hyperinsulinism/Hyperammonemia Syndrome

Impact of Ketogenic Diet on Lipoproteins in Refractory Epilepsy

Vitamin E for Extremely Preterm Infants

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