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Information Journal Paper

Title

EVALUATION OF ENDOCRINE DISORDERS IN PATIENTS WITH THALASSEMIA MAJOR

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 Start Page 35 | End Page 43

Abstract

 Introduction: THALASSEMIA MAJOR is a genetic disorder, in which blood transfusion is critical for the survival of patients. Over the course of the past two and three decades, hypertransfusion therapy in these patients has significantly increased life expectancy and quality of life.Unfortunately however this type of therapy has also increased the frequency of complications due to iron overload. Today endocrine abnormalities are far more common than before in betathalassemia patients. The aim of this study was to evaluate the prevalence of endocrine disturbances in patients with THALASSEMIA MAJOR, aged over 10 years. Materials and methods: Fifty six patients, aged over 10 years, with THALASSEMIA MAJOR were enrolled. Physicians collected demographic data and history of therapies as well as menstrual history in females. Patients were examined to determine their pubertal status and SDS of height for evaluation of SHORT STATURE. For evaluation of glucose tolerance, fasting blood glucose and oral glucose tolerance tests were performed. Serum levels of calcium, phosphorous, thyroid stimulating hormone, free thyroxin, luteinizing hormone, and follicular stimulating hormone, estradiol in girls and testosterone in boys were measured. Results: Fifty-six patients 10 to 27 years with THALASSEMIA MAJOR old were evaluated. In this study, the prevalences of DIABETES MELLITUS IMPAIRED FASTING GLUCOSE and IMPAIRED GLUCOSE TOLERANCE TEST were 8.9%, 28.6% and 7.1% respectively. SHORT STATURE (SDS £ -2) was seen in 70% of boys and 73% of girls; HYPOCALCAEMIA and primary overt HYPOTHYROIDISM were present in 41% and 16% respectively; 14.3% did not have any endocrine abnormalities. Conclusion: Despite recent therapy with Desferal in the management of beta-THALASSEMIA MAJOR, the risk of secondary endocrine dysfunction remains high, with HYPOGONADISM being one of the most frequent endocrine complications. Endocrine evaluation in patients with THALASSEMIA MAJOR must be carried out regularly especially in those patients over the age of 10 years in Tabriz.

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