IRON-ENDOCRINE PATTERN IN PATIENTS WITH BETA-THALASSEMIA

Patients,vith beta-thalassaemia (major, minor, and in association with sickle cell gene) frequently suffer from growth retardation and delay ed sexual development. This is believed to be due, at least in part, t o a direct effect of iron overload on the endocrine mechanisms of pube rty. The role of iron overload remains speculative in relation to the disease severity and ethnic variations. This study determined the leve l of testosterone, cortisol, luteinizing hormone (LH), follicle stimul ating hormone (FSH), free thyroxine (T4), tri-iodothyronine (T3), grow th hormone (GH), iron, ferritin, and haematological parameters in 44 b eta-thalassaemia patients (21 = beta-thal. major, 23 beta-thal minor), 25 Hb S/beta degrees-thalassaemia patients, and 50 normal controls wi th age range 2-15 years. The iron endocrine status and haematological findings were evaluated, and the results were correlated and compared with age-and sex-matched controls. In comparison with controls the bet a-thalassaemia-major and the Hb S/beta degrees-thalassaemia patients h ad a significantly higher level of plasma ferritin (P < 0.01) while th e mean level of total haemoglobin was significantly lower. The level o f LH, FSH, testosterone, and plasma cortisol were lower in both beta-t halassaemia-major and Hb S/beta degrees-thalassaemia patients with a n egative correlation with plasma ferritin level. Free T3 and T4 were ra ised, but the difference was not statistically significant. The data d emonstrate the occurrence of impaired endocrine function in the beta-t halassaemia and Hb S/beta degrees-thalassaemia patients.