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 delayed sexual development. This is believed to be due, at least in part, to a direct effect of iron overload on the endocrine mechanisms of puberty. The role of iron overload remains speculative in relation to the disease severity and ethnic variations. This study determined the level of testosterone, cortisol, luteinizing hormone (LH), follicle stimulating hormone (FSH), free thyroxine (T4), tri-iodothyronine (T3), growth hormone (GH), iron, ferritin, and haematological parameters in 44 beta-thalassaemia patients (21 = beta-thal. major, 23 beta-thal minor), 25 Hb S/beta degrees-thalassaemia patients, and 50 normal controls with 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 beta-thalassaemia-major and the Hb S/beta degrees-thalassaemia patients had a significantly higher level of plasma ferritin (P < 0.01) while the mean level of total haemoglobin was significantly lower. The level of LH, FSH, testosterone, and plasma cortisol were lower in both beta-thalassaemia-major and Hb S/beta degrees-thalassaemia patients with a negative correlation with plasma ferritin level. Free T3 and T4 were raised, but the difference was not statistically significant. The data demonstrate the occurrence of impaired endocrine function in the beta-thalassaemia and Hb S/beta degrees-thalassaemia patients.