Beta-Thalassemia
Conditions
Keywords
thalasssemia, vitamin E, Oxidative stress
Brief summary
repeated transfusions are the mainstay of disease management in most patients with transfusion dependent beta thalassemia.iron overload predispose to oxidative stress and tissue injury. oxidative stress play important role in pathogenesis of anemia in beta thalassemia. vitamin E is often depleted in thalassemia patients.
Detailed description
oxidative stress status is very important in thalassemic patients and explains the different manifestations in thalassemic patients. vitamin E is fat soluble vitamin shown to reduce the oxidative stress in thalassemia and to reduce lipid peroxidation of red cell membranes. therefore, this study shows the safety of oral vitamin E as adjuvant therapy to three iron chelators : desferoxamine,deferiprone and deferasirox in moderately iron overloaded children and adolescents with transfusion dependent beta thalassemia and its relation to iron overload over one year.
Interventions
DIETARY_SUPPLEMENTvitamin E
Vitamin E will be taken for 12 months for beta thalassemia patiens on regular packed red blood cell transfusion and chelators(desferoxamine,deferiprone and deferasirox )
OTHERPLACEBO
placebo will be taken for 12 months for beta thalassemia patiens on regular packed red blood cell transfusion and chelators(desferoxamine,deferiprone and deferasirox )
Sponsors
Ain Shams University
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
No
Inclusion criteria
* Beta thalassemia patients on regular packed red blood cells transfusion,with vitamin E deficiency * serum ferritin 1000-2500 ug/l * cardiacT2\*\>10ms and ejection fraction \>55%
Exclusion criteria
* Diabetes,active hepatitis(serum transaminases \>2 times ULN) * renal impairment (serum creatinine\>2times ULN) * participation in previous investigational drug study with 3 months preceding screening supplementation with antioxidants or vitamins within 3 months prior to the study and patients with known allergy to vitamin
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| effect of vitamin E on Liver iron concentration (LIC) | 12 months | measuring the LIC using MRI (magnetic resonance imaging) in mg/g |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| effect of vitamin E on antioxidants enzymes | 12 months | measuring antioxidants enzymes * antioxidant enzymes, |
| effect of vit E supplementation on SF | 12 months | measuring serum ferritin(SF)in ng/ml |
Countries
Egypt
Outcome results
None listed