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BACKGROUND Homozygous sickle-cell anemia and beta-thalassemia are characterized by impaired endothelial function, while data on arterial stiffness have hitherto been conflicting. We sought to investigate aortic elastic properties and endothelial function in sickle-thalassemia, which combines molecular and clinical features of the above conditions. METHODS(More)
BACKGROUND Heart disease represents the main determinant of survival in beta-thalassemia, but its particular features in the two clinical forms of the disease, thalassemia major (TM) and thalassemia intermedia (TI), are not completely clarified. METHODS We compared clinical and echocardiographic global parameters in 131 TM patients who received regular(More)
OBJECTIVE To assess cardiac status in a large group of patients with thalassemia major who had been treated in a standard way since their early infancy with intensive transfusions and deferoxamine chelation therapy and who had good compliance with this regimen. METHODS AND RESULTS We assessed clinically and echocardiographically 202 thalassemia major(More)
OBJECTIVES To apply magnetic resonance imaging (MRI) for the assessment of myocardial iron deposition in patients with beta-thalassemia and compare the results with cardiac biopsy data. BACKGROUND Myocardial iron accumulation is the main cause for cardiac complications in beta-thalassemia. METHODS Twenty-five consecutive thalassemic patients were(More)
BACKGROUND Plasma B-type natriuretic peptide (BNP) concentration has significant diagnostic accuracy and prognostic value in various forms of heart disease. Whether BNP is also useful in the evaluation and management of thalassaemia heart disease remains to be determined. METHODS AND RESULTS Eighty three thalassaemia major patients; 8 with acutely(More)
To address how low titer, variable expression, and gene silencing affect gene therapy vectors for hemoglobinopathies, in a previous study we successfully used the HPFH (hereditary persistence of fetal hemoglobin)-2 enhancer in a series of oncoretroviral vectors. On the basis of these data, we generated a novel insulated self-inactivating (SIN) lentiviral(More)
BACKGROUND Beta-thalassaemia major (b-TM) has been defined as a combination of chronic hemolytic anemia, iron storage disease and myocarditis, and it has been associated with premature death especially due to heart failure. To the best of our knowledge the status of blood lipids in these patients has rarely been investigated. Thus, we assessed the levels of(More)
Haemoglobinopathies are the most common hereditary disorders in Greece. Although there is a successful national prevention program, established 35 years ago, there is lack of an official registry and collection of epidemiological data for haemoglobinopathies. This paper reports the results of the first National Registry for Haemoglobinopathies in Greece(More)
Cardiovascular involvement is a leading cause of mortality and morbidity in patients with inherited hemoglobinopathies, but it has not been adequately assessed in sickle beta-thalassemia. We evaluated 115 sickle beta-thalassemia patients, aged 34 +/- 14 years, along with 50 healthy controls, by resting echocardiography. Patients with systolic left(More)
BACKGROUND HCV infection and transfusional iron overload in Thalassemic patients may result in liver disease. HCV treatment in Thalassemia has raised safety concerns. AIM Estimate effectiveness and tolerability of interferon-based therapy in HCV-infected Thalassemic patients. MATERIAL AND METHODS Over a 12-year period, consecutive patients with β(More)