Effect of iron overload on endocrinopathies in patients with beta-thalassaemia major and intermedia.

INTRODUCTION: Iron overload is a major problem in patients with b-thalassemia major, and it has many structural and metabolic consequences. In this study, we aimed to consider the prevalence of endocrine abnormalities in patients with ß-thalassemia major and thalassemia intermedia. MATERIALS AND METHODS: We ordered following tests for consideration endocrine abnormalities: fasting plasma glucose, oral glucose tolerance, iron, total iron binding capacity, ferritin, thyroid-stimulating hormone (TSH), free thyroxin (fT4), free triiodothyronine (fT3), parathyroid hormone levels. RESULTS: According to our study including 70 patients with thalassemia major, 7 (10%) had diabetes, 5 (7.1%) had impaired glucose tolerance, 9 (12.8%) had hypothyroidism, 2 (2.8%) had hypoparathyroidism, 2 (2.8%) had hyperparathyroidism. Of 22 patients with thalassemia intermedia, 1 (4.5%) had diabetes. CONCLUSIONS: These findings reinforce the importance of regular follow-up of patients with b-thalassemia major and thalassemia intermedia for early detection and management of associated complications. In this way, the prevalence of endocrine abnormalities can be decreased in future.

Outcome of kidney transplantation for renal amyloidosis:a single-center experience.

The aim of this retrospective study was to investigate the results of kidney transplantation in patients with renal amyloidosis. We analyzed the results of renal transplantation in 13 amyloidotic transplant recipients compared with those in a control group of 13 nonamyloidotic patients. While the etiology of amyloidosis was rheumatoid arthritis in one patient, in all of the others it was secondary to familial Mediterranean fever. Acute rejection episodes developed once in six and twice in one patient. The renal function in these patients was improved by antirejection treatment. Chronic rejection did not develop in any patient. However six patients (46%) died due to various complications despite functional grafts. The others are still being followed with well-functioning grafts. Among the control group, acute and chronic rejection were diagnosed in three and two patients, respectively: one patient returned to hemodialysis after 26 months of transplantation, while the others are still alive with functional grafts. There was no death in the control group. The 5- and 10-year actuarial patient survival rates of the amyloidosis and control groups were 52.2%, 26.6%, and 100%, 100%, respectively (P = .002). However, the graft survivals of the amyloidosis versus control groups were 100%, 100%, versus 87.5%, 87.5, respectively (P = .47). In conclusion, we observed a high rate of early mortality among recipients with amyloidosis associated with infectious complications. Moreover, patient survivals were lower among amyloidotic renal recipients.