Glucose tolerance disorders in patients suffering from thalassemia major

Background. In patients suffering from thalassemia major, hemosiderosis fre quently causes endocrine disorders. We investigated the development of gluc ose tolerance disorders and the influence of therapeutical interventions su ch as intensified chelation therapy and diet. Methods and patients. 60 patients with thalassemia major, ages ranging from 4 to 36 years,treated regularly with both subcutaneous desferrioxamine inf usions and erythrocyte transfusions, were investigated for endocrine disord ers. Oral and intravenous glucose tolerance tests as well as intravenous gl ucagon challenge were used to investigate the insulin secretion. In patient s with impaired glucose tolerance the influence of intensified (intravenous ) chelation therapy and dietary interventions on blood glucose and serum in sulin levels were studied. Results. In patients with thalassemia major, disturbed glucose tolerance ap pears to be one of the four most common endocrine disorders in the second d ecade of life and later. In the early stages of the disease, glucose tolera nce disorders were associated with hyperinsulinemia; in later stages insuli nopenic diabetes develops due to p-cell exhaustion. In some patients in the early stages of the disease, intensified chelation therapy or dietary trea tment only improved their glucose tolerance. Conclusion. Especially for thalassemia patients in the early stages of gluc ose tolerance disorders, intensified desferrioxamine treatment and diet can be of benefit and in some cases retard the manifestation of diabetes melli tus. These treatments do not, however, change the requirement for insulin s ubstitution at an advanced stage of disturbed glucose tolerance.