INTRODUCTION There are limited reports about selenium status in major thalassemia patients. The aim of this study is evaluation of selenium status in patients with major thalassemia south east of Iran with large sample size and wide range of age. This study compared selenium status with other sites of the world. METHODS In this study 369 cases that had major thalassemia for more than 5 years ...

Transfusional hemosiderosis is a frequent complication in patients with transfusion dependent chronic diseases such as thalassemias and severe type of sickle cell diseases. As there are no physiological mechanisms to excrete the iron contained in transfused red cells (1 unit of blood contains approximately 200 mg of iron) the excess of iron is stored in various organs. Cardiomyopathy is the mos...

BACKGROUND Excessive iron accumulation leads to iron toxicity in the brain; however the underlying mechanism is unclear. We investigated the effects of iron overload induced by high iron-diet consumption on brain mitochondrial function, brain synaptic plasticity and learning and memory. Iron chelator (deferiprone) and antioxidant (n-acetyl cysteine) effects on iron-overload brains were also stu...

Here, we report the case of a 36-year-old patient with a diagnosis of de novo mutation of the WDR45 gene, responsible for beta-propeller protein-associated neurodegeneration, a phenotypically distinct, X-linked dominant form of Neurodegeneration with Brain Iron Accumulation. The clinical history is characterized by a relatively stable intellectual disability and a hypo-bradykinetic and hyperton...

PURPOSE Cardiac failure due to myocardial iron overload is the most common cause of death in beta-thalassemia patients. Multi/ two echo times-turbo field echo (TE-TFE) magnetic resonance imaging (MRI) is considered the gold standard technique in the evaluation of myocardial iron accumulation. However, multi TE-TFE technique is not available in all scanners. The aim of our study was to show the ...

Over the past four decades, there have been dramatic improvements in survival for patients with thalassemia major due in large measure to improved iron chelators. Two chelators are approved for use in the United States and Canada, parenteral deferoxamine and oral deferasirox. Three are available in much of the rest of the world, where oral deferiprone is also approved (in the United States, def...

BACKGROUND AND OBJECTIVE Excessive hemosiderosis is the main reason for the multi-organ failure observed in multitransfused patients. Deferiprone (1,2-dimethyl-3-hydroxy-pyridine-4-one, L1) is an orally active iron chelator mainly excreted via urine. We conducted a study in order to determine the efficacy and safety of L1 in Greek thalassemic patients. DESIGN AND METHODS A group of 11 thalass...

This study was conducted to assess the effect of age, ferritin level, hemoglobin level and chelating agents on the physical growth in thalassemic children and to determine the prevalence of dental caries in thalassemic children. Weight, standing height, sitting height and subischial leg length were measured in 65 children attending the Thalassemia day care center at a tertiary hospital in Delhi...