Thalassemia major is a genetic disorder and blood transfusion is critical for survival in these patients. Over the course of the past three dec-ades, hyper transfusion therapy in these patients has shown significant increase in life expectancy and quality of life. Unfortunately this type of therapy also increased the frequency of compli-cations due to iron overload. The aim of this study was to evaluate the prevalence of endo-crine disturbances in patients, aged over 10 years, with thalassemia major. Materials and Methods: Fifty six patients, aged over 10 years, with thalassemia major were en-rolled. Physicians collected demographic data and history of therapies as well as menstrual his-tories in females. Patients were examined to de-termine 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 were measured for calcium, phosphorous, thyroid stimulating hormone, free thyroxin, lu-teinizing hormone, follicular stimulating hor-mone, and estradiol and testosterone in girls and boys respectively. Results: Fifty-six patients with thalassemia ma-jor, aged between 10-27 years, were evaluated. In this study prevalences of diabetes mellitus, im-paired fasting glucose and impaired glucose tol-erance test were 8.9%, 28.6% and 7.1% respec-tively. Short stature (SDS ≤-2) was seen in 70% of boys and in 73% of girls. Hypocalcaemia and primary overt hypothyroidism were present in 41% and 16% respectively; 14.3% of our patients had no endocrine abnormalities. Conclusion: Despite recent therapy with Desferal in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Hypogonadism is 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.

"/> Thalassemia major is a genetic disorder and blood transfusion is critical for survival in these patients. Over the course of the past three dec-ades, hyper transfusion therapy in these patients has shown significant increase in life expectancy and quality of life. Unfortunately this type of therapy also increased the frequency of compli-cations due to iron overload. The aim of this study was to evaluate the prevalence of endo-crine disturbances in patients, aged over 10 years, with thalassemia major. Materials and Methods: Fifty six patients, aged over 10 years, with thalassemia major were en-rolled. Physicians collected demographic data and history of therapies as well as menstrual his-tories in females. Patients were examined to de-termine 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 were measured for calcium, phosphorous, thyroid stimulating hormone, free thyroxin, lu-teinizing hormone, follicular stimulating hor-mone, and estradiol and testosterone in girls and boys respectively. Results: Fifty-six patients with thalassemia ma-jor, aged between 10-27 years, were evaluated. In this study prevalences of diabetes mellitus, im-paired fasting glucose and impaired glucose tol-erance test were 8.9%, 28.6% and 7.1% respec-tively. Short stature (SDS ≤-2) was seen in 70% of boys and in 73% of girls. Hypocalcaemia and primary overt hypothyroidism were present in 41% and 16% respectively; 14.3% of our patients had no endocrine abnormalities. Conclusion: Despite recent therapy with Desferal in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Hypogonadism is 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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Evaluation of Endocrine Disorders in Patients with Thalassemia Major

AUTHORS

F Najafipour 1 , *

1 Department of Endocrinology and Metabolism, Emam Reza Hospital, Tabriz University of Medical sciences, [email protected], IR.Iran

How to Cite: Najafipour F. Evaluation of Endocrine Disorders in Patients with Thalassemia Major, Int J Endocrinol Metab. Online ahead of Print ; 6(2):-.

ARTICLE INFORMATION

International Journal of Endocrinology and Metabolism: 6 (2)
Article Type: Original Article
Received: November 22, 2007
Accepted: April 23, 2008
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Abstract

Thalassemia major is a genetic disorder and blood transfusion is critical for survival in these patients. Over the course of the past three dec-ades, hyper transfusion therapy in these patients has shown significant increase in life expectancy and quality of life. Unfortunately this type of therapy also increased the frequency of compli-cations due to iron overload. The aim of this study was to evaluate the prevalence of endo-crine disturbances in patients, aged over 10 years, with thalassemia major. Materials and Methods: Fifty six patients, aged over 10 years, with thalassemia major were en-rolled. Physicians collected demographic data and history of therapies as well as menstrual his-tories in females. Patients were examined to de-termine 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 were measured for calcium, phosphorous, thyroid stimulating hormone, free thyroxin, lu-teinizing hormone, follicular stimulating hor-mone, and estradiol and testosterone in girls and boys respectively. Results: Fifty-six patients with thalassemia ma-jor, aged between 10-27 years, were evaluated. In this study prevalences of diabetes mellitus, im-paired fasting glucose and impaired glucose tol-erance test were 8.9%, 28.6% and 7.1% respec-tively. Short stature (SDS ≤-2) was seen in 70% of boys and in 73% of girls. Hypocalcaemia and primary overt hypothyroidism were present in 41% and 16% respectively; 14.3% of our patients had no endocrine abnormalities. Conclusion: Despite recent therapy with Desferal in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Hypogonadism is 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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