تأثیر درمانی هیدروکسی اوره در فرم های خفیف تالاسمی

Background and purpose: β thalassemia major is one of the most prevalent progressive and severe genetical disorder in our country. The homozygote patients (major) sometimes show the milder form of the disease, known as intermedia. Some chemicals like hidroxy urea has been tried in thalassemic patients. Ïn this study the therapeutic effect of the drug on the patients referred to Boali Sina thalassemic center was considered in 1377-78. Materials and Methods: Ôur study was a clinical trial done on the onset diagnosed thalassemic patients whose start of blood receiving or else was from the age of 4 years more, they did have severe blood transfusion dependency. Âfter clarification of the issue to the parents of the patients and their agreement the hydroxy urea capsules (made of medac, germany) were given orally with the primary sigle doses of 10-30 mg/kg daily cell blood count and monthly reficulocyle count indices and F-hemoglubin measurement before and treatment and in the third and sixth month was done. White blood cells count , measurement of urea and creatinine were done monthly, in order to study the probable side effects of the drug. The mutations were determined by ÂRMS method and PÇR technique in Pasteur institute, Tehran. Descriptive and analytic statistical methods (Turkey Kranmer) ÂNÔVÂ were used for the repeated measurements. Results : 21 girls and 9 boys took part in this study, with mean age 7 ± 19 the follow up term was 6 months, and the mean dose was 18 mg/kg. The effect of hydroxy urea caused no need of blood transfusion in 73%. The Hb, Hct, MÇV, Retic variation the rate and the percentage of HbF were calculated during the term of therapy, which were significant statistically (P<0.05 to <0.0001) ÏVSÏÏ-Ï was the predominant mutation, recorded in 46 chromosome under studies from 39 cases. Çonclusion : Hydroxy urea was very effective with less side effects, and has less cost compare to transfusion so, more study on molecular biology and on probable mechanism of effect is recommended and also supervision on the domestic production of hydroxy urea is suggested.