β-thalassemias result from diminished β-globin synthesis and are associated with ineffective erythropoiesis and secondary iron overload caused by inappropriately low levels of the iron regulatory hormone hepcidin. The serine protease TMPRSS6 attenuates hepcidin production in response to iron stores. Hepcidin induction reduces iron overload and mitigates anemia in murine models of β-thalassemia ...

Clinical data suggest that in beta-thalassemia-intermedia patients, higher levels of circulating fetal hemoglobin (HbF) are associated with greater disease severity at comparable degrees of anemia. We assessed the influence of the amount of circulating HbF on serum erythropoietin (s-Epo) levels and on serum transferrin receptor, a measure of erythropoiesis, in 30 beta-thalassemia-intermedia pat...

BACKGROUND Using two logistic regression models, we determined the associates of poor physical and mental health related quality of life (HRQoL) among beta thalassemia patients. METHODS In this cross-sectional study which was conducted during 2006 and 2007 in outpatient adult thalassemia clinic, Blood Transfusion Organization, Tehran, Iran, Short Form 36 (SF-36) was used for measuring HRQoL i...

Moyamoya disease is a cerebrovascular disorder with unknown cause characterized by the occlusion of the bilateral internal carotid arteries (ICA) and proximal segments of ICA.1,2 On the other hand, moyamoya syndrome (MMS) is a rare form of this condition with underlying several pathologies including hematologic disorders, congenital syndromes, vascular malformations or vasculitis after irradiat...

Patients with thalassemia major are considered to have high erythropoietin (Epo) activity.' However, Hammond et a1 reported that thalassemic patients had much less serum Epo activity compared with patients with hypoplastic anemia.* Determination of serum Epo levels in 32 patients from our clinic with homozygous p-thalassemia major and intermedia showed that Epo activity was not significantly hi...

background: beta-thalassemia is considered to be the most frequent hereditary blood disorder worldwide. lipid abnormalities have been detected in different types of beta-thalassemia . the aim of this study is to assess the lipid profiles in beta-thalassemia major (btm) and beta-thalassemia intermedia (bti) patients in southern iran. methods: the study group consisted of 55 btm patients and 50 b...

conclusions the etiology of pah in thalassemia is multifactorial such as inflammatory mediators. also, the absence of the spleen plays an important role in developing a high trv and pah. results average age of the patients was 21.15 ± 6.68 years. no significant difference was observed in the pap between the 2 groups of thalassemia major and intermedia and also the 2 sex groups. indeed, 6.6% of ...

Patients with thalassemia major are considered to have high erythropoietin (Epo) activity.' However, Hammond et a1 reported that thalassemic patients had much less serum Epo activity compared with patients with hypoplastic anemia.* Determination of serum Epo levels in 32 patients from our clinic with homozygous p-thalassemia major and intermedia showed that Epo activity was not significantly hi...