Perspective: A novel prognostic for sickle cell disease
Sickle hemoglobin (α2βS2) polymerization drives disease pathophysiology in sickle cell anemia. Fetal hemoglobin (α2γ2) restricts disease severity by inhibiting the polymerization of sickle hemoglobin in a concentration-dependent manner. Clinical decision-
Brian M Mozeleski+3 more
doaj +1 more source
Breaking Free from Your Fetal Chains: A Case-Based Review of the Literature on Gamma Chain Variant Hemoglobinopathies [PDF]
Methemoglobinemia is a dyshemoglobinemia characterized by cyanosis and reduced oxygen saturation with increased methemoglobin values. The etiology may be congenital or acquired, with the latter being more common.
Pearl Mary Varughese+3 more
doaj +1 more source
β-hemoglobinopathies are caused by abnormal or absent production of hemoglobin in the blood due to mutations in the β-globin gene (HBB). Imbalanced expression of adult hemoglobin (HbA) induces strong anemia in patients suffering from the disease. However,
Andrés Lamsfus-Calle+12 more
semanticscholar +1 more source
Fetal Hemoglobin in Sickle Hemoglobinopathies: High HbF Genotypes and Phenotypes
Fetal hemoglobin (HbF) usually consists of 4 to 10% of total hemoglobin in adults of African descent with sickle cell anemia. Rarely, their HbF levels reach more than 30%.
M. Steinberg
semanticscholar +1 more source
MR imaging–derived oxygen-hemoglobin dissociation curves and fetal-placental oxygen-hemoglobin affinities [PDF]
PURPOSE: To generate magnetic resonance (MR) imaging–derived, oxygen-hemoglobin dissociation curves and to map fetal-placental oxygen-hemoglobin affinity in pregnant mice noninvasively by combining blood oxygen level–dependent (BOLD) T2* and oxygen ...
Akselrod-Ballin, Ayelet+6 more
core +2 more sources
Hydroxyurea ameliorates mouse hypoxia through inducing embryonic hemoglobin
Objective To investigate the ameliorative effect and mechanism of hydroxyurea (HU) on hypoxia mice in a closed environment. Methods Sixty male C57BL/6J mice (6~8 weeks old, weighing 18~22 g) were randomly divided into normoxia control group (NC, n=10 ...
ZHOU Xiaoying+4 more
doaj +1 more source
Fetal hemoglobin rescues ineffective erythropoiesis in sickle cell disease
While ineffective erythropoiesis has long been recognized as a key contributor to anemia in thalassemia, its role in anemia of sickle cell disease (SCD) has not been critically explored.
Sara El Hoss+13 more
semanticscholar +1 more source
Effect of hydroxyurea on G gamma chain fetal hemoglobin synthesis by sickle-cell disease patients
Hydroxyurea is used for sickle-cell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes.
S.M. Teixeira+2 more
doaj +1 more source
Whole transcriptome analysis of human erythropoietic cells during ontogenesis suggests a role of VEGFA gene as modulator of fetal hemoglobin and pharmacogenomic biomarker of treatment response to hydroxyurea in β-type hemoglobinopathy patients [PDF]
Background: Human erythropoiesis is characterized by distinct gene expression profiles at various developmental stages. Previous studies suggest that fetal-to-adult hemoglobin switch is regulated by a complex mechanism, in which many key players still ...
Chondrou, Vasiliki+11 more
core +4 more sources
In vitro and in vivo induction of fetal hemoglobin with a reversible and selective DNMT1 inhibitor
Pharmacological induction of fetal hemoglobin (HbF) expression is an effective therapeutic strategy for the management of β-hemoglobinopathies such as sickle cell disease.
A. Gilmartin+22 more
semanticscholar +1 more source