BACKGROUND Iron overload is a common complication of patients with β-thalassemia major (TM). Despite the availability of three iron chelators, deferoxamine (DFO), deferiprone (DFP), and deferasirox (DFX), some patients fail to respond adequately to monotherapy with any of them. We report a case of TM who had refractory severe iron overload and was successfully and safely chelated with the combi...

BACKGROUND Cardiac complications due to iron overload are the most common cause of death in patients with thalassemia major. The aim of this study was to compare iron chelation effects of deferoxamine, deferasirox, and combination of deferoxamine and deferiprone on cardiac and liver iron load measured by T2* MRI. METHODS In this study, 108 patients with thalassemia major aged over 10 years wh...

In the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. Without iron chelation therapy, heart disease is the major cause of death; however, hepatic and endocrine complications also occur. Currently there are three iron chelating agents available for continuous use in patients with thalassemia o...

transfusion-dependent patients such as those suffering from β-thalassaemia develop a fatal secondary haemosiderosis and consequently a selective iron chelator must be used to relieve such iron overload. 3- hydroxypyridin-4-ones are selective for iron(iii) under most biological conditions, but unlike desferrioxamine, are efficiently absorbed when administered orally. in this study, the synthesis...

iron overload is a serious clinical condition which can be largely prevented by the use of iron-specific chelating agents. in this study, the synthesis and determination of partition coefficients (kpart) of a range of n-alkyl hydroxypyridinones, as orally active iron chelators, are described. synthesis of n-aryl hydroxypyridinones was achieved via a single step synthetic pathway. in this method...

Thalassemia is anemia of variable severity, arising from mutations of genes encoding the hemoglobin alpha and beta chains. Severe thalassemia is associated with iron overload, tissue lesions, and high risk for cardiovascular complications, and iron-mediated cardiomyopathy is the main cause of death in this condition. Thalassemia major (TM) patients exhibit cardiovascular abnormalities consisten...

iron overload is known to occur in the west european and american population due to the consumption of iron-rich diets. on the other hand, genetic disorders leading to iron overload are also known. iron overload leads to increased peroxidation and disruptive disintegration of lipid-rich membranes, and predisposes humans for an enhanced risk of cancer induction. in experimental animals iron over...

abstract background: iron overload is a clinical consequence of repeated blood transfusions and causes significant organ damage, morbidity, and mortality in the absence of proper treatment. the primary targets of iron chelators used for treating transfusional iron overload are the prevention of iron ingress into tissues and its intracellular scavenging. the present study was aimed at elucidatin...

Magnetic resonance imaging (MRI) has played a key role in studies of iron overload in transfusion-dependent patients, providing insights into the relations among liver and cardiac iron loading, iron chelator dose, and morbidity. Currently, there is rapid uptake of these methods into routine clinical practice as part of the management strategy for iron overload in regularly transfused patients. ...

Background Chronic iron overload is prevalent and prognosis relates to the severity of cardiac siderosis. The black blood (BB) T2* technique is validated, with calibration against myocardial tissue iron at 1.5T. There are potential advantages of 3T over 1.5T in assessing T2*, but preliminary studies at 3T used white blood imaging, had small patient numbers, methodological differences, and poor ...