OSTEOPENIA IN BETA-THALASSEMIA MAJOR

Regular blood transfusions in patients with beta-thalassaemia major le ad to secondary hemo chromatosis in the majority of cases. As a conseq uence of chronic iron overload, many endocrinopathies may occur. The m ost frequent endocrine dysfunction is hypogonadotropic hypogonadism, w hich is mainly responsible for osteopenia in as much as 80% of thalass emic patients. The frequencies of other endocrine disorders (hypothyro idism, diabetes mellitus and hypoparathyroidism) are lower. We investi gated 5 female patients aged 22-25 years for endocrine dysfunction and bone density. All presented with hypogonadotropic hypogonadism and am enorrhea (four primary and one secondary). 4 patients showed absent or delayed pubertal development and short stature (below 10th percentile ). In all five, hypogonadism is the most relevant cause of osteopenia as demonstrated by osteodensitometry. Endocrine disorders, especially absent pubertal development, should be detected in good time and treat ed with hormonal replacement. Established osteopenia is treated hormon ally and with vitamin D-3 and calcium.