Adrenarche in Egyptian children with beta-thalassemia major

ABSTRACT

In this study 40 children with Beta-thalassaemia major were studied for the changing levels of dehydroepiandrosterone sulphate [DHEAS] with age starting from 5 to 13 years in order to detect the abrupt rise in DHEAS characteristic of the onset of adrenarche, i.e., the maturation of the adrenal gland. The children were studied for anthropometric measurements including weight, height, sitting height, biacromial and biiliac diameters. The hormones studied were DHEAS, cortisol, growth hormone and testosterone. The study showed that the maturation of the adrenal gland was incomplete and delayed to age 9 to 11 in children with Beta-thalassaemia major compared to 6 years as reported in healthy Egyptian children. The delay was less severe in females. No significant differences were observed with GH, testosterone and cortisol levels in prepubertal thalassaemic children. Splenectomized patients had significantly higher DHEAS levels, i.e. adrenal gland maturation was more developed than those with huge spleen. A significant negative correlation, was observed between DHEAS and GH [r -0.39] [p <0.05]. The weight, height and sitting height were > -2 S.D. in 45.16%, 65.71% and 78.26% respectively. Highly significant positive correlation were observed between DHEAS levels and weight [r 0.75 p < 0.01], height [r 0.66 p < 0.01], sitting height [r 0.062 p < 0.01], biacromial diameter [r 0.63 p < 0.01] and biiliac diameter [r 0.37 p < 0.05]. It is concluded that the severe growth retardation in thalassaemic children is definitely attributed partly if not wholly to the delayed and incomplete maturation of the adrenal gland in these children