GROWTH AND ENDOCRINE DISORDERS IN THALASSEMIA

Scientific publications 01 January 2013

De Sanctis V. - Soliman A.T. - Elsedfy H. - Skordis N. - Kattamis C. - Angastiniotis M. - Karimi M. - Yassin M.A. - El Awwa A. - Stoeva I. - Raiola G. - Galati M.C. - Bedair E.M. - Fiscina B. - El Kholy M.

Abstract

The current management of thalassemia includes regular transfusion programs and chelation therapy. It is important that physicians be aware that endocrine abnormalities frequently develop mainly in those patients with significant iron overload due to poor compliance to treatment, particularly after the age of 10 years. Since the quality of life of thalassemia patients is a fundamental aim, it is vital to monitor carefully their growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment.

Abnormalities should be identified and treatment initiated in consultation with a pediatric or an adult endocrinologist and managed accordingly. Appropriate management shall put in consideration many factors such as age, severity of iron overload, presence of chronic liver disease, thrombophilia status, and the presence of psychological problems. All these issues must be discussed by the physician in charge of the patient's care, the endocrinologist and the patient himself.

Because any progress in research in the field of early diagnosis and management of growth disorders and endocrine complications in thalassemia should be passed on to and applied adequately to all those suffering from the disease, on the 8 May 2009 in Ferrara, the International Network on Endocrine Complications inThalassemia (I-CET) was founded in order to transmit the latest information on these disorders to the treating physicians. The I-CET position statement outlined in this document applies to patients with transfusion-dependent thalassemia major to help physicians to anticipate, diagnose, and manage these complications properly.

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GROWTH AND ENDOCRINE DISORDERS IN THALASSEMIA

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