Chronic blood transfusion is the only treatment for severe anemia in patients with β-thalassemia major. However, red blood cell transfusions lead to iron overload and subsequent organ damage because of the toxic effects of iron. The heart is particularly vulnerable to iron toxicity, and heart failure is the leading cause of death among these patients. Iron chelation therapy prevents or reverses...

The term hemochromatosis encompasses at least four types of genetic iron overload conditions, most of them recently distinguished from one another as a result of the identification of a series of genes related to iron metabolism. At least three of these entities (HFE hemochromatosis, juvenile hemochromatosis and transferrin receptor 2 hemochromatosis) involve systemic hepcidin deficiency as a k...

TREATMENT OF LEAD TOXICITY with chelation was first reported with EDTA in the early 1950s. Apparent success in reducing metastatic calcium deposits led Clarke et al in 1956 to treat angina patients with EDTA, and others to use chelation for various forms of atherosclerotic disease. Chelation therapy evolved to constitute infusions of vitamins and disodium EDTA, a drug that binds divalent and so...

BACKGROUND Thalassemia is a hereditary disorder requiring regular blood transfusion. PURPOSE To determine hearing sensitivity in transfusion-dependent thalassemics who were receiving desferioximine and on oral iron chelation (desferioximine) therapy. METHODS 26 patients with B-thelassemia in the age range of 5-22 years were enrolled in the thalassemia transfusion unit in the Advanced Pediat...