EVALUATION OF GROWTH HORMONE SECRETION AND ADRENAL RESERVE, IN PARALLEL WITH CARDIAC AND OSSEOUS PARAMETRES, IN ADULT PATIENTS WITH THALASSEMIA

Thalassemia is the most severe hereditary hemolitic anemia, as yet lacking an appropriate genetic cure. The cure of chronic anemia is therefore the mainstay of therapy, through appropriate transfusions and iron chelation. These two approaches have markedly modified both the quality of life and the life expectancy of these patients, enabling them to reach adulthood in the majority of cases. However, patients with thalassemia are burdened by several problems, such as heart dysfunction, hepatic insufficiency and several defects in endocrine secretions (e.g. failure to undergo puberty, short stature, diabetes, insufficient secretion of thyroid and parathyroid hormones, osteoporosis). The study of the growth hormone (GH) and adrenal cortisol secretion in adult patients with thalassemia appears extremely interesting for most studies have been performed in children or teenagers. Defective GH secretion due is known to induce important derangements in heart and bone and both these two organs are also affected in the thalassemia. Should a defect in GH secretion exist also in adult patients with thalassemia then therapy with GH could aid in the correction of heart and bone disorders which so heavily impair their quality of life. On the other hand, normal adrenal cortisol secretion is essential for everyday living and for an adequate response to stress. In patients with thalassemia, the physician is frequently faced with a progressive enfeeblement of the patient, which does not seem to depend on transfusion regimen, and often also an unexplained fatal course due to infectious or cardiac disease. These clinical observations underline the need for an assessment of adrenal cortisol secretion, because, if cortisol secretion is insufficient in adult patients with thalassemia, then this can easily be corrected by daily replacement therapy and/or treatment with steroids during stressful events. This may well prove to be a life-saving therapeutic measure in patients with thalassemia.