BACKGROUND Thalassemia intermedia is a variety of beta thalassemia which shows clinical symptoms somewhere between asymptomatic carriers and thalassemia major. Cardiac dysfunctions due to chronic anemia and hemosiderosis are the major causes of death in these patients. The purpose of this study is to evaluate cardiac function in these patients by echocardiography. MATERIALS AND METHODS This c...

An estimation of the incidence and demographic picture of the major hemoglobinopathies in Belgium has been approached through a confidential inquiry sent to 228 pediatric and adult hematological departments. Forty-two percent of responses showed that 417 patients are known in Belgium: 83% with sickle cell disease, 13% with beta-thalassemia (beta-thal) major, 2% with beta-thal intermedia, and 1%...

Thalassemia intermedia is a genetically diverse group of diseases that is the result of an imbalance in the production of the alpha and beta chains with ensuing chronic hemolysis, ineffective erythropoiesis, and iron overload. Resulting complications include bone changes, hypercoagulability, and end-organ damage due to iron overload. This decade has witnessed major breakthroughs in the manageme...

Even the life span has prolonged for the last 40 years, increase in frequently seen complications with increasing age negatively affect the life quality of thalassemia patients. In our study, complications encountered in 67 ß-thalassemia patients who were followedup at our hospital between 1 January 2004 and 31 May 2009 were retrospectively analyzed. Fifty-six patients were followed up with dia...

this study presented a mini review on folic acid deficiency and recommendations for its supplementation in thalassemia intermedia (ti). ti is a clinical condition which lies between thalassemia major and thalassemia minor. although ti patients may not need regular blood transfusion, precise diagnosis and management are critical for the prevention of clinical complications and quality of life im...

background: cardiac dysfunction due to chronic anemia and hemosiderosis are the major causes of death among patients with thalassemia intermedia. this study was performed to compare the cardiac function in thalassemia intermedia patients with normal subjects by means of echocardiography. materials and methods: this was a case-control study performed on 22 patients affected by thalassemia interm...

In patients with thalassemia intermedia in whom hyperabsorption of iron may result in serious organ dysfunction, an orally effective iron-chelating drug would have major therapeutic advantages, especially for the many patients with thalassemia intermedia in the Third World. We report reduction in tissue iron stores and normalization of serum ferritin concentration after 9-month therapy with the...

BACKGROUND Patients with β thalassemia intermedia can have substantial iron overload, irrespectively of their transfusion status, secondary to increased intestinal iron absorption. This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity. DESIGN AND METHODS This was a cross-sectional study of 168 patients with β thalassemia intermedia t...

BACKGROUND The molecular defects resulting in a β-thalassemia phenotype, in the Egyptian population, show a clear heterogenic mutations pattern. PCR-based techniques, including direct DNA sequencing are effective on the molecular detection and characterization of these mutations. The molecular characterization of β-thalassemia is necessary for carrier screening, genetic counseling, and to offer...