BACKGROUND Cells respond to stress stimuli through a number of response pathways, of which one of the most important and well characterized is the unfolded protein response. Despite a large body of work which suggests that stress in erythroblasts may play a pivotal role in the pathogenesis of beta-thalassemia/Hb E disease, this pathway remains uninvestigated. DESIGN AND METHODS Day 10 erythro...

This paper describes a comparison of two analytical techniques, one employing bathophenanthrolinedisulfonate (BPT), a most commonly-used reagent for Fe (II) determination, as chromogen and an electrothermal atomic absorption spectroscopy (ETAAS) for the quantification of non-transferrin bound iron (NTBI) in sera from thalassemic patients. Nitrilotriacetic acid (NTA) was employed as the ligand f...

The a-thalassemic mouse has a hereditary microcytic anemia. almost certainly has a shortened RBC life span. and is a potential candidate for cell replacement therapy. In a routine study of bone marrow repopulating capacity using hemoglobin as a cell marker. normal donor marrow cells. but not a-thalassemic donor marrow cells. completely replaced the host cells. Further analysis showed that at le...

beta- thalassemia major is a common hemoglobinopathy in humans. In some journals, numerous studies have reported different prevalence of hepatitis C among beta- thalassemia major because thalassemic patients need multiple blood transfusions and blood transfusion is a common transmission pathway for hepatitis C virus. Thus this study was performed for detection of anti-HCV between beta- thalasse...

Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

Up to 30% of Iranian adult multi-transfused thalassemic patients are infected with hepatitis C virus (HCV) which can intensify the progression of liver disease caused by iron overload in this group of patients. Our aim was to assess the biochemical and virological response of interferon alfa (INF-α) and its safety in thalassemic patients with chronic HCV infection. This trial was a single c...

Patients with higher liver iron stores are likely to have a worse cardiac outcome following noncompliance with chelation. Cardiovascular magnetic resonance identifies myocardial siderosis allowing optimization of iron chelation regimes. Diabetes puts thalassemic patients at increased risk of myocardial fibrosis. Dual chelation therapy with deferoxamine and deferiprone offers improved cardiac ou...

PURPOSE To compare contrast sensitivity (CS) in multi-transfused β-thalassemia patients who received deferoxamine with those who received Osveral. METHODS In this cross sectional study a total of 60 β-thalassemia patients (30 used deferoxamine and 30 used deferasirox) were regarded as case group and 30 age and sex matched healthy subjects were selected as control group. All subjects had a set...