isolated rat hepatocytes in culture were incubated with different concentrations of iron-sorbitol (50, 100, 150, and 200 µm) to assess the changes in reactive oxygen species (ros) and lipid peroxidation leading to apoptotic hepatocyte cell death. the viability of hepatocytes was declined depending on the iron concentration. one hour incubation of the cells with 100 µm iron resulted in decreased...

background: sickle-cell anemia is characterized by defective hemoglobin synthesis with production of sickle hemoglobin. sickle red blood cells become deformed and rigid with difficulty to pass through narrow capillaries and frequent clotting and thrombosis leading to repetitive vascular occlusions and progressive organ damage. we conducted this work to study some adrenal functions using acth st...

Iron overload cardiomyopathy (IOC), defined as the presence of systolic or diastolic cardiac dysfunction secondary to increased deposition of iron, is emerging as an important cause of heart failure due to the increased incidence of this disorder seen in thalassemic patients and in patients of primary hemochromatosis. At present, although palliative treatment by regular iron chelation was recom...

background: patients with transfusional iron overload have depended on iron chelation therapy and improving chelation regimens have been of the highest priority. the aim of this study was to compare effect of combined versus monotherapy with deferoxamine (dfo) and deferiprone (dfp) in iron overloaded beta thalassemia (bt) major patients materials and methods we studied 36 bt major patients (mea...

BACKGROUND Patients with transfusion-dependent beta-thalassemia major (TM) are at risk for myocardial iron overload and cardiac complications. Spatial repolarization heterogeneity is known to be elevated in patients with certain cardiac diseases, but little is known in TM patients. The purpose of this study was to evaluate spatial repolarization heterogeneity in patients with TM, and to investi...

Hepcidin is the master regulator of systemic iron homeostasis. Derived primarily from the liver, it inhibits the iron exporter ferroportin in the gut and spleen, the sites of iron absorption and recycling respectively. Recently, we demonstrated that ferroportin is also found in cardiomyocytes, and that its cardiac-specific deletion leads to fatal cardiac iron overload. Hepcidin is also expresse...

PURPOSE Cardiac involvement in thalassemia major (TM) is mainly characterized by left ventricular dysfunction caused by iron overload. Cardiovascular magnetic resonance imaging (MRI) including myocardial T2* measurement is becoming increasingly popular for quantitatively evaluating myocardial iron overload. The aim of this study was to evaluate the relationship between the myocardial T2* value ...

BACKGROUND Cardiac iron toxicity is the leading cause of death among β-halassaemia major (TM) patients. Once heart failure becomes overt, it is difficult to reverse. OBJECTIVES To investigate non-overt cardiac dysfunctions in TM patients using pulsed wave Tissue Doppler Imaging (TD I) and its relation to iron overload and brain natriuretic peptide (BNP). METHODS Thorough clinical, conventio...

BACKGROUND Patients with transfusional iron overload may develop a life-limiting cardiomyopathy. The sensitivity of lipid-metabolizing enzymes to peroxidative injury, as well as the reported effects of arachidonic acid (AA) and metabolites on cardiac rhythm, led us to hypothesize that iron-overloaded cardiomyocytes display alterations in the release of AA and prostaglandins. METHODS AND RESUL...

BACKGROUND Iron overload cardiomyopathy remains the major cause of death in patients with transfusion-dependent thalassemia. Cardiac T2* magnetic resonance imaging is costly yet effective in detecting cardiac iron accumulation in the heart. Heart rate variability (HRV) has been used to evaluate cardiac autonomic function and is depressed in cases of thalassemia. We evaluated whether HRV could b...