in recent years of experience fetal hemoglobin (hbf) induction considers as novel therapeutic approach for β-thalassemia and sickle cell in therapeutic approaches. several pharmacologic agents such as hydroxyurea, azacytidine, butyrate deriviates and immunomodulator drugs like pomalidomide and lnalidomide could able to up-regulate hbf level with different molecular and epigenetic paterns (1-4)....

Hereditary persistence of fetal hemoglobin (HPFH) has typically been ascribed to mutations in the beta-globin gene cluster. Pharmacologic agents, including the short-chain fatty acid butyrate, have been shown to upregulate fetal and embryonic globin gene expression. In this report we investigate the possibility that metabolic derangements characterized by an inability to metabolize another shor...

Cytotoxic drugs increase circulating fetal hemoglobin levels. We examined the mechanism by measuring the fetal hemoglobin produced per BFU-E-derived erythroblast following hydroxyurea treatment in vivo and in vitro. Treatment of four sickle cell patients increased the percentage of circulating F reticulocytes. The frequencies of bone marrow or peripheral blood BFU-E or CFU-E-derived colonies an...

OBJECTIVES To assess the value of fetal aortic time-domain measurement of volume flow (using color velocity imaging quantification (CVI-Q)) in predicting the severity of fetal anemia. METHODS This was a prospective observational study, in which 24 pregnant women with suspected fetal anemia due to rising anti-red blood cell antibody titers underwent cordocentesis. The fetal aortic time-domain ...

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OBJECTIVE In red blood cell (RBC) isoimmunized pregnancies fetal anemia is associated with a hyperdynamic circulation. The aim of the present study was to examine further the possible value of fetal middle cerebral artery peak systolic velocity (MCA-PSV) in the management of affected pregnancies. METHODS A reference range of fetal MCA-PSV with gestation was constructed from the study of 813 n...

BACKGROUND Although erythroid cells prepared from fetal liver, cord blood, or blood from β-thalassemia patients are known to express fetal hemoglobin at high levels, the underlying mechanisms remain elusive. We previously showed that cyclic nucleotides such as cAMP and cGMP induce fetal hemoglobin expression in primary erythroid cells. Here we report that cAMP signaling contributes to high-leve...

BACKGROUND An improved understanding of the regulation of the fetal hemoglobin genes holds promise for the development of targeted therapeutic approaches for fetal hemoglobin induction in the β-hemoglobinopathies. Although recent studies have uncovered trans-acting factors necessary for this regulation, limited insight has been gained into the cis-regulatory elements involved. METHODS We iden...