the ingested nitrates sourced from tap water, food, chemicals and pharmaceuticals areconverted to nitrites in the body surfaces by bacteria and then, the nitrite ions can lead thestructural changing in hemoglobin. in the present work, aggregation of the purified hemoglobinin adult (hba) and in fetus or newborn (hbf) in the presence of nitrite ions were studied.hemoglobin aggregation was perform...

hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. it is usually a multifactorial process. a rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. we report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. sickle cell disease is often mild in the iranian populati...

background: efficient induction of fetal hemoglobin (hbf) is considered as an effective therapeutic approach in beta thalassemia. hbf inducer agents can induce the expression of γ-globin gene and produce high levels of hbf via different epigenetic and molecular mechanisms. thalidomide and sodium butyrate are known as hbf inducer drugs. material and methods : cd133 + stem cells were isolated fro...

background: &beta-thalassemia; is the most common monogenic disorder in human. the (ct) polymorphism at -158 upstream region of the &gammag-globin; gene and pharmacological factors such as hydroxyurea have been reported to influence &gamma-globin; gene expression and the severity of clinical symptoms of &beta-thalassemia.; methods: in the present study, 51 &beta-thalassemia; intermediate patie...

potent induction of fetal hemoglobin (hbf) production results in alleviating the complications of β-thalassemia and sickle cell disease (scd). hbf inducer agents can trigger several molecular signaling pathways critical for erythropoiesis. janus kinase/signal transducer and activator of transcription (jak/stat), mitogen activated protein kinas (mapk) and phosphoinositide 3-kinase (pi3k) are con...

our previous studies on thalassemia patients, homozygous or compound heterozygous for severe beta thalassemia mutations, has suggested that 5’hs4-lcr and/or xmn1-hbg2 backgrounds, which were linked in our thalassemia patients, are important in determining the blood transfusion dependency of these individuals. while the majority of patients with aa/-- 5’hs4/xmn1 background, were severely depende...

objective: the use of fetal hemoglobin (hbf) inducer drugs is considered as a novel approach in treatment of β-hemoglobinopathies, especially β- thalassemia and sickle cell disease. hbf inducers including hydroxyurea, histone deacetylase (hdac) inhibitor agents such as sodium butyrate, azacitidine, decitabine and new immunomodulator drugs like pomalidomide, lenalidomide and thalidomide can redu...

background: there are many parameters that modulate the severity of sickle cell anemia. fetal hemoglobin (hb f) is one of these major variables. however, its effect is clinically inconsistent. we conducted a descriptive study to assess the influence of hb f on clinical events and hematological variables in patients with sickle cell anemia. methods: 151 patients with sickle cell anemia with a st...

increased fetal hemoglobin (hbf) in b-globin gene disorders ameliorates the clinical symptoms of the underlying disease. 5-azacytidine, butyrate and hydroxyurea, have been shown to activate g-globin gene expression. it has also been found that hematopoietic growth factors can influence expression of g-globin in erythroid cultures and in animal models. this study was designed to evaluate the in ...

abstract background and objective: diabetes mellitus is one of complications that thalassemia major patients face with. hence, blood glucose monitoring is of vital importance to these patients. because of high level of fetal hemoglobin in these patients, the measurement of hemoglobin a1c is not reliable and should be displaced by fructosamine test. material and methods: the current descriptive ...