[The role of vitamin E in the therapy of thalassemia].
Serum vitamin E values are depressed in thalassaemia owing to increased consumption because of the oxidative stress imposed both to red cells and other tissues by haemochromatosis. A study of vitamin E deficiency was carried over a period of about 2 years in 161 transfusion dependent thalassaemic patients aged 4 months to 18 years (including 74 splenectomized subjects) all following the same transfusion and chelation protocol (pretransfusion Hb = 11 gr/dl and daily chelation with subcutaneous infusion of desferrioxamine 12 hr a day). Serum vitamin E levels were determined by Martinek's method. The mean value for the entire series was 0.45 +/- 0.21 mg/dl (normal value 0.76 +/- 0.22), with no differences between splenectomized (0.43 +/- 0.19) and not--splenectomized (0.45 +/- 0.21) subjects. Values of less than 0.32 mg/dl (mean-2SD) were found in 50 patients (31,1%). Below--normal values were noted in 5/11 patients at the time of diagnosis. 124 subjects with less than 0.54 mg/dl received 5-10 mg/Kg/day vitamin E per os. In 38 cases it has been possible to control vitamin E level after one year of therapy. Mean values before treatment were 0.36 +/- 0.13 mg/dl and 1.19 +/- 0.35 mg/dl after therapy. No patient failed to respond and no adverse effect was recorded. These results show that by no means all thalassaemic patients are vitamin E deficient to the point where replacement therapy is necessary, and oral administration can easily correct low serum levels, contrary to what has been found by other workers.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006720:Homozygote; D006801:Humans; D007223:Infant; D013789:Thalassemia / Q000097:blood* / Q000188:drug therapy / Q000235:genetics; D014810:Vitamin E / Q000627:therapeutic use; D014811:Vitamin E Deficiency / Q000097:blood*
D014810:Vitamin E
Ruolo della vitamina E nella terapia della talassemia.