BACKGROUND Patients with Sickle cell disease (SCD) who receive regular transfusions are at risk for developing cardiac toxicity from iron overload. The aim of this study was to assess right and left cardiac volumes and function, late gadolinium enhancement (LGE) and iron deposits in patients with SCD using CMR, correlating these values with transfusion burden, ferritin and hemoglobin levels. ...

BACKGROUND Transfusional therapy for thalassemia major and sickle cell disease can lead to iron deposition and damage to the heart, liver, and endocrine organs. Iron causes the MRI parameters T1, T2, and T2* to shorten in these organs, which creates a potential mechanism for iron quantification. However, because of the danger and variability of cardiac biopsy, tissue validation of cardiac iron ...

background: iron-mediated cardiomyopathy is the main complication of thalassemia major (tm) patients. therefore, there is an important clinical need in the early diagnosis and risk stratification of patients. the aim of this study was to evaluate the efficacy of tissue doppler imaging (tdi) to study cardiac iron overload in patients with tm using t2* magnetic resonance (mr) as the gold-standard...

Human β-thalassemia major is one of the most prevalent genetic diseases characterized by decrease/absence of β-globin chain production with reduction of erythrocyte number. The main cause of death of treated β-thalassemia major patients with chronic blood transfusion is early cardiac complications that have been attributed to secondary iron overload despite optimal chelation. Herein, we investi...

Briefly, they report that a cardiac T2* value 10 ms had a sensitivity of 98% and a specificity of 86% for prediction of symptomatic heart failure in 1 year. Risk was graded with respect to T2*, with 47% of patients having T2* 6 ms developing cardiac failure in the same interval. Similar, but less striking, risk stratification was also observed for prospective arrhythmia risk. Metrics of total b...

BACKGROUND The diagnosis of genetic haemochromatosis (GH) before iron overload has developed is difficult. However a convincing candidate gene for GH, HFE (previously HLA-H), has been described recently. AIMS To determine the prevalence of the haemochromatosis associated HFE mutations C282Y and H63D in United Kingdom affected and control populations. METHODS The prevalence of the HFE C282Y ...

Iron overload has not been studied extensively and prospectively in pediatric survivors of allogeneic hematopoietic stem cell transplantation (HSCT); therefore, we conducted a prospective long-term study of 133 survivors of childhood leukemia to assess the incidence of and risk factors for iron overload and to investigate its association with organ dysfunction. One yr after HSCT, the mean serum...

Background: Iron is an essential mineral for normal cellular physiology but its ‎overload can lead to cell injury. For many years, deferoxamine injection has been used ‎as an iron chelator for treatment of iron overload. The aim of this study is to compare ‎oral deferoxamine, activated charcoal, and vitamin C, as an absorbent factor of Fe, in ‎changing the serum level of iron in iron overload r...

For nearly 30 years, patients with transfusional iron overload have depended on nightly deferoxamine infusions for iron chelation. Despite dramatic gains in life expectancy in the deferoxamine era for patients with transfusion-dependent anemias, the leading cause of death for young adults with thalassemia major and related disorders has been cardiac disease from myocardial iron deposition. Stra...

For nearly 30 years, patients with transfusional iron overload have depended on nightly deferoxamine infusions for iron chelation. Despite dramatic gains in life expectancy in the deferoxamine era for patients with transfusion-dependent anemias, the leading cause of death for young adults with thalassemia major and related disorders has been cardiac disease from myocardial iron deposition. Stra...