Study of the Absorption of Vitamin E Water-soluble Form (Pegylated) in the Familial Hypocholesterolemia With Chylomicron Retention

Study Overview
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Brief Summary
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To evaluate the kinetics of intestinal absorption of vitamin E water-soluble form from the classical lipid-soluble form in a population of patients with intestinal malabsorption (hypocholesterolemias family by retention of chylomicrons).

Official Title
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Study of the Absorption of Vitamin E Water-soluble Form (Pegylated) in the Familial Hypocholesterolemia With Chylomicron Retention

Conditions
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Hypobetalipoproteinemias

Intervention / Treatment
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* Drug: Tocofersolan
* Drug: tocopherol alpha
* Drug: Tocofersolan and tocopherol alpha


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patient suffering from familial hypocholesterolemia by retention of chylomicrons Systematically followed in the department of Gastroenterology and Paediatric Nutrition in Women Mother Child's Hospital or in the endocrinology department of GHE (Louis Pradel Hospital); During treatment with oral vitamin E; over the age of 3 years and weighing over 16 kg at the time of inclusion For which there is a signed informed consent from the patient or parents / guardians in the case of a minor patient, and benefit from social security coverage. Exclusion Criteria: Patient with encephalopathy Hypersensitivity to the active substance or the excipients of Vedrop A suspected allergy to local anaesthetics (including xylocaine) Patients who may not be compliant to treatment (psychiatric); In case of refusal to participate in the study from the patient and / or parents or legal guardian; Patients unable to consent (if patients with encephalopathy)

Ages Eligible for Study
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Minimum Age: 6 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Crossover Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Tocofersolan: Vitamin E water-soluble<br>2 months off the conventional treatment before the study. Administration of a daily dose of vitamin E for 4 months | Drug: Tocofersolan<br>* Administration of a daily dose of vitamin E for 4 months:50 IU/kg<br>|
| Active Comparator: Tocopherol alpha: Vitamin E conventional fat-soluble form<br>2 months off the conventional treatment before the study. Administration of a daily dose of vitamin E for 4 months | Drug: tocopherol alpha<br>* Administration of a daily dose of vitamin E for 4 months:50 IU/kg<br>|
| Active Comparator: volunteers<br> | Drug: Tocofersolan and tocopherol alpha<br>* Administration of a single dose of vitamin E (Vitamin E water-soluble then Vitamin E conventional fat-soluble form)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement the area under the curve of absorption of vitamin E according to its dosage form (fat-soluble versus water-soluble) in the beginning of each treatment period. versus water-soluble) in the beginning of each treatment period. |  | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement of serum (membranes erythrocytic) of vitamin E at the beginning and after each treatment. | Measurement of serum (membranes erythrocytic) of vitamin E at the beginning and after each treatment. Search for a correlation between the rates in adipose tissue and those in the membrane of erythrocytes. | 12 months | 
| Measurement of tissue (subcutaneous adipose tissue) of vitamin E at the beginning and after each treatment. | Measurement of tissue (subcutaneous adipose tissue) of vitamin E at the beginning and after each treatment. Search for a correlation between the rates in adipose tissue and those in the membrane of erythrocytes. | 12 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Vitamin E pegylated, chylomicron retention, Familial Hypocholesterolemia, Hypobetalipoproteinemias