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Symposium : Hematology/Oncology-II Indian J Pediatr 1998; 65 : 825-828 Nearly thirty million people are carriers of beta thalassemia alone in India and 8-10 thousand babies with thalassemia major are born every year in IndiaL All these children with thalassemia major are potential candidates to develop iron overload and toxicity from blood transfusions. Iron, a constituent Of many enzymes is so essential for life that during evolution, the mechanisms for its absorption, preservation and storage are well developed. Unfortunately nature has not developed any mechanisms to excrete iron even in state of iron excess. Iron overload results in the oxidative denaturation of lipids, sugars, aminoacids, DNA and various regulatory mechanisms resulting in tissue damage. Excessive iron deposition causes liver damage and various endocrinopathies resulting in diabetes mellitus, growth failure, delayed puberty, joint pains, and cardiac damage leading to congestive cardiac failure and arrhythmias. Cardiac and liver complications are the major causes of death in thalassemic patients, who do not receive regular chelation therapy. In the last 30 years over 400 iron chelators have been evaluated in vitro and in animals studies ~. Among the large number of iron chelators, only 15 have been clinically evaluated. Among these, 1-2
Indian Journal of Pediatrics – Springer Journals
Published: Nov 1, 1998
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