Plasma steroid levels in thalassemic girls

Abstract

Thalassemia Major, a severe disease principally characterized by chronic hemolitic anemia is frequently associated with delayed sexual maturation.12 thalassemic girls aged from 18 to 22 years at Tanner's first stage of puberty and 12 healty girls at the same age but at the fifth stage of puberty, were studied.After informed consent of parents,blood samples were taken at 8-9 a.m.,after overnight fasting.Specific RIA was measured FSH,LH,Prolactin (Prl),Cortisol (F) and Dehydroepiandrosterone sulphate (DHA-S)directly in the plasma.Plasma ether extraction and celite column chromatograpty were performed to measure by RIA the following hormones:Dehydroepiandrosterone (DHA),Pregnenolone (Δ5P),Progesterone (P),170HProgesterone (17P),Androstenedione (A),Testosterone (T),Dihydrotestosterone (DHT) and Estradiol (E2).Results from healty to thalassemic girls:FSH (from 9 to 4 mU/ml),LH(from 9 to 2,5 mU/ml),Prl(from 10 to 6 ng/ml),F(from 120 to 80 ng/ml),DHAS(from 1,8 to 0,08 μg/ml), Δ₅P(from 1,6 to 0,5 ng/ml),P(from 270 to 110 pg/ml),17P(from 0,43 to 0,15 ng/ml),A(from 10 to 2,5 ng/ml),T(from 0,4 to 0,1 ng/ml), DHT(from 120 to 25 pg/ml) and E2(from 85 to 50 pg/ml). These data suggest that thalassemic girls show a delayed adrenarche,according to ours previous studies due to the abnormal iron deposits.

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