BACKGROUND: The thalassemic syndromes originate from mutations of the globin genes that cause, besides the characteristic clinical picture, also an increased Hb F amount. It is not yet clear if there are more factors, besides the beta globin genotype, determining the Hb F production. We have tried to find out if there are relations between total Hb and Hb F, between erythropoietin (Epo) and Hb ...

Phospholipid asymmetry in the red blood cell (RBC) lipid bilayer is well maintained during the life of the cell, with phosphatidylserine (PS) virtually exclusively located in the inner monolayer. Loss of phospholipid asymmetry, and consequently exposure of PS, is thought to play an important role in red cell pathology. The anemia in the human thalassemias is caused by a combination of ineffecti...

BACKGROUND In situ production of a secreted therapeutic protein is one of the major gene therapy applications. Nevertheless, the plasmatic secretion peak of transgenic protein may be deleterious in many gene therapy applications including Epo gene therapy. Epo gene transfer appears to be a promising alternative to recombinant Epo therapy for severe anaemia treatment despite polycythemia was rea...

OBJECTIVES The purpose of this study was to test the hypothesis that quantitative ultrasound properties of the calcaneus in Southeast Asian children treated for thalassemia have different characteristics than those of their healthy counterparts and thereby can be used for assessing the risk of osteoporosis. METHODS Broadband ultrasound attenuation and the speed of sound were measured from gro...

As initial human gene therapy trials for beta-thalassemia are contemplated, 2 critical questions important to trial design and planning have emerged. First, what proportion of genetically corrected hematopoietic stem cells (HSCs) will be needed to achieve a therapeutic benefit? Second, what level of expression of a transferred globin gene will be required to improve beta-thalassemic erythropoie...

BACKGROUND Most thalassemic patients with chronic hepatitis C virus (HCV) infection do not respond to therapy with pegylated interferon (Peg-IFN) plus ribavirin (RBV) due to hepatic siderosis and RBV dose reduction caused by RBV-induced anemia. OBJECTIVES In the present study, we recruited HCV genotype 1-infected thalassemic patients who had relapsed after a 48-week treatment with Peg-IFN plu...

Thalassemias are a geographically widespread group of genetic hemoglobinopathies characterized by defective globin production and hemolytic anemia [1]. Thalassemic patients are frequently transfusiondependent and this, in addition to heightened gastrointestinal avidity for iron, creates a propensity for iron overload [1]. Infection is a major cause of morbidity in thalassemic patients and is se...

this prospective case-control study was carried out to evaluate the Prevalence of positive cases of anti-HCV Ab in 107 thalassemic patients  and 107 children without any previous history of blood transfusion as a control group.ELISA GENERATION II was used for serologic investigation and then positive cases were confiemed  by RIBA generation I.the average age of thalassemic patients was 9.2+_4.3...