Members of 7 large families, containing 20 patients with sickle cell anemia (SS) characterized by high levels of fetal hemoglobin (HbF), were studied using immunofluorescence to count F cells and a radioimmunoassay to measure small amounts of HbF. In five of these families, one of the sickle cell trait (AS) parents had a much higher HbF and F-cell count than the other; in one family, both paren...

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 reduc...

Increased levels of fetal hemoglobin (HbF, alpha(2)gamma(2)) are of no consequence in healthy adults, but confer major clinical benefits in patients with sickle cell anemia (SCA) and beta thalassemia, diseases that represent major public health problems. Inter-individual HbF variation is largely genetically controlled, with one extreme caused by mutations involving the beta globin gene (HBB) co...

The implications of lncRNAs regarding fetal hemoglobin (HbF) induction in hemoglobin disorders remain poorly understood. In this study, microarray analysis was performed to profile lncRNAs, miRNAs and mRNAs in individuals with hereditary persistence of fetal hemoglobin (HPFH), β-thalassemia carriers with high HbF levels and healthy controls. The results show aberrant expression of 862 lncRNAs, ...

BACKGROUND Vasa previa is a rare but potentially dangerous fetal condition that may occur during pregnancy. Ideally, all cases such cases are detected antenatally, but many present as late vaginal hemorrhaging. At the current time, there is no test for fetal hemoglobin (HbF) in general use. METHODS A modified method of identifying HbF is presented. Five milliliters of 0.14 M NaOH was combined...

The persistence of high fetal hemoglobin level in adults may ameliorate the clinical phenotype of beta-thalassemia and sickle cell anemia. Several genetic variants responsible for hereditary persistence of fetal hemoglobin, linked and not linked to the beta globin gene cluster, have been identified in patients and in normal individuals. Monoallelic loss of KLF1, a gene with a key role in erythr...

For the treatment of β-thalassemia and sickle cell disease (SCD), pharmacological induction of fetal hemoglobin (HbF) production may be a promising approach. To date, numerous studies have been done on identifying the novel HbF-inducing agents and understanding the underlying mechanism for stimulating the HbF production. In this review, we have summarized the identified HbF-inducing agents by f...

Hemoglobin-based oxygen carriers have long been pursued to meet clinical needs by using native hemoglobin (Hb) from human or animal blood, recombinantly produced Hb, but the development has impeded safety and toxicity issues. Herewith we report successful production of fetal (HbF) in Nicotiana benthamiana through Agrobacterium tumefaciens-mediated transient expression. HbF is a heterotetrameric...

This study investigated the potential contribution of nitric oxide (NOx) production to enhanced fetal hemoglobin (HbF) synthesis in patients with diabetes. Glycated hemoglobin (HbA1c), HbF, high sensitivity C-reactive protein (hsCRP), plasma glucose levels, and serum NOx concentrations were measured in 350 diabetics and 125 healthy subjects. There were no significant correlations between HbF an...

Differences in the amount of fetal hemoglobin (HbF) that persists into adulthood affect the severity of sickle cell disease and the beta-thalassemia syndromes. Genetic association studies have identified sequence variants in the gene BCL11A that influence HbF levels. Here, we examine BCL11A as a potential regulator of HbF expression. The high-HbF BCL11A genotype is associated with reduced BCL11...