ABSTRACT
Adrenal endocrine function was assessed in a cohort of 20 patients, between 10 and 20 years of age, with transfusion dependent beta thalassemia. Cortisol levels were assayed before and after ACTH stimulation with 1 micrograms and 250 micrograms. Adrenal dysfunction was defined as a basal cortisol of greater than 400 nmol/L and/or peak cortisol levels of greater than 500 nmol/L. Overall, 9 patients (45 %) had in vitro evidence of adrenal dysfunction. A statistical significant correlation (r=0.4308; P < 0.05), between wasting and the basal cortisol level, was observed. Similarly, there was correlation between the number of transfusions received and growth failure (r=0.4774;P < 0.05). In comparison to the involvement of other endocrine axes in polytransfused thalassemics, the adrenal endocrine function abnormalities are minor and clinically of little consequence. The observations, albeit, in a small cohort of thalassemics, stress the need for an annual estimation of basal cortisol level, especially in patients with wasting.