The erythrocytes of certain human individuals undergo a reversible change in shape known as sickling if deprived of oxygen (1, 2). Taliaferro and Huck (3) postulated that this characteristic is transmitted by a single dominant gene, but failed to account genetically for the wide divergence in clinical signs and symptoms among individuals who possess sickling red cells. A small fraction, about 1...

Microvascular occlusion in sickle cell disease can be initiated by adhesion of sickle red blood cells (RBCs) to the endothelium. Our objective in this study was to verify the relevance in vivo of our discovery that sickle RBCs adhere abnormally to endothelial P-selectin in vitro. We used computer-assisted intravital microscopy to characterize RBC flow velocity (V(RBC)) in mice. We found faster ...

Background: The sickle gene confers an increased vulnerability to infection, particularly to certain bacterial pathogens, and this has significantly contributed to morbidity and mortality in individuals with sickle cell disease. Immunization plays an important role in the prevention of infection in children with sickle cell disease; we sought to assess the pneumococcal vaccination rate of child...

Sickle cell anemia was first described by Herrick in 1910. Pioneering studies by Pauling et al. established that Sickle Cell Disease (SCD) results from a defect in the hemoglobin molecule. 1 The sickle mutation was characterized several years later by Ingram et al. as a glutamine-to valine substitution at the sixth residue of the beta globin polypeptide. Homozygosity for the sickle mutation (i....

The purpose of this communication is to describe the necropsy findings in three patients dying of sickle cell haemoglobin C disease and two of sickle cell anaemia. In southern Ghana there is a high incidence of abnormal haemoglobins in the population, the incidence of haemoglobin S being 18% and of haemoglobin C 12% (Edington and Lehmann, 1954a). Haemoglobin G has also been described (Edington ...

Previous studies have shown that the sickle environment is highly enriched for reactive oxygen species (ROS). We examined the oxidative effects of sickle cell disease on hematopoietic stem cell function in a sickle mouse model. In vitro colony-forming assays showed a significant decrease in progenitor colony formation derived from sickle compared to control bone marrow (BM). Sickle BM possessed...

We have previously reported that sickle erythrocytes sedimenting at high specific density after gradient centrifugation exhibit increased IgG binding in vivo as compared with low-density paired samples. We have performed the present study to determine whether the opsonization of dense sickle cells in vivo could also involve autologous IgM, IgA, and complement. IgA, IgM, and complement binding i...

OBJECTIVE Evaluate the Neonatal Screening Program of the Health Secretariat of the State of Santa Catarina for sickle-cell disease, from January 2003 to December 2012, regarding program coverage and disease frequency. METHODS Descriptive, observational, cross-sectional study with retrospective data collection. The variables analyzed were: number of live births in the State of Santa Catarina; ...

background: approximately 180 mutations have been described in β-thalassemia worldwide with specific spectrum in each ethnic population. this study determines the spectrum and the frequency of β-thalassemia mutations in patients with β-thalassemia trait and sickle cell-β-thalassemia.   methods: fifteen compound heterozygous sickle cell thalassemia (sct) and 23 β-thalassemia trait patients were ...