BACKGROUND Iron-overload cardiomyopathy is a major cause of death in thalassemia patients due to the lack of an early detection strategy. Although cardiac magnetic resonance (CMR) T2* is used for early detection of cardiac iron accumulation, its availability is limited. Heart rate variability (HRV) has been used to evaluate cardiac autonomic function and found to be depressed in thalassemia. Ho...

Objectives: To evaluate left and right myocardial performance at rest and after maximal exercise by conventional and Tissue Doppler Imaging (TDI) echocardiography. Background: Iron Overload Cardiomyopathy (IOC) is the main cause of death in major (TM) patients but conventional Echocardiography fails to predict early cardiac dysfunction. As TDI is able to demonstrate regional myocardial dysfunct...

Background Transfusion-induced iron overload leads to many complications in patients with β-thalassemia major. This study aimed to compare the level of iron stored in the liver and heart measured by MRI T2 * with ferritin levels in these patients. Materials and Methods This study was done on 52 patients with β-thalassemia major aged 7 to 29 years. Serum ferritin level was checked and heart and ...

Regular blood transfusions as supportive care for patients with chronic anemia inevitably lead to iron overload as humans cannot actively remove excess iron. The cumulative effects of iron overload cause significant morbidity and mortality if not effectively treated with chelation therapy. Based on a comprehensive clinical development program, the once-daily, oral iron chelator deferasirox (Exj...

BACKGROUND Patients with chronic iron overload may develop a cardiomyopathy manifested by ventricular arrhythmias and heart failure. We hypothesized that iron-loaded cardiomyocytes may have abnormal excitability. METHODS AND RESULTS We examined a new model of human iron overload, the Mongolian gerbil given repeated injections of iron dextran. In ventricular myocytes, we measured iron concentr...

juvenile hemochromatosis is a rare autosomal recessive disorder that typically occurs in the first to third decades of life. its symptoms are more acute and severe than classic hemochromatosis. we describe a 27-year-old man who was referred to the gastrointestinal clinic with a probable diagnosis of fatty liver and was finally diagnosed as having juvenile hemochromatosis. a review of the scient...

hydroxypyridinone iron chelators are currently the main candidates for development of orally active iron chelating alternatives to desferrioxamine (dfo). in the present study, the relative efficacy and liver toxicity of a bidentate chelator, 2-methyl-3-hydroxypyridin-4-one (mhpo), was studied in iron overloaded rats and compared with those of dfo. for iron overloading, rats received i.p. inject...

Previous studies have demonstrated impaired ventricular repolarization in patients with β-TM. However, the effect of iron overload with cardiac T2* magnetic resonance imaging (MRI) on cardiac repolarization remains unclear yet. We aimed to examine relationship between repolarization parameters and iron loading using cardiac T2* MRI in asymptomatic β-TM patients. Twenty-two β-TM patients and 22 ...

Introduction: Cardiac complications secondary to iron overload are the leading cause of death in thalassemia major (TM). Cardiovascular magnetic resonance (CMR) can provide a noninvasive means of measuring the amount of tissue iron. With CMR, tissue iron is detected indirectly by the effect on relaxation times of hydrogen nuclei in the presence of ferritin and hemosiderin iron. The iron results...

Abstract Objective Thalassemia major is a severe transfusion-dependent anemia that needs iron chalation therapy to remove iron overload. The objectives of the present study were to assess the iron overload liver response to inulin of chicory supplementation by evaluating the serum ferritin and liver enzymes. Methods Among 70 beta thalassemia patients, 50 were selected for chelating therapy ...