Interaction of hemoglobin S polymers with the red blood cell (RBC) membrane induces a reversible increase in permeability ("P(sickle)") to (at least) Na(+), K(+), Ca(2+), and Mg(2+). Resulting changes in [Ca(2+)] and [H(+)] in susceptible cells activate 2 transporters involved in sickle cell dehydration, the Ca(2+)-sensitive K(+) ("Gardos") channel (K(Ca)) and the acid- and volume-sensitive K:C...

Previous studies of the Hpa I cleavage site-sickle cell hemoglobin gene linkage in various African populations suggested that the sickle gene arose independently more than once. In the present study we have performed restriction endonuclease haplotype analysis for the beta-globin-like gene cluster from four separate geographic areas in Africa, all of which possess the sickle gene. In Benin (Cen...

There are about 50,000 people in the United States who are homozygous for the sickle hemoglobin gene and thus have sickle cell anemia. Sickle cell anemia is primarily seen in persons of African heritage, about 1 in 14 of whom is an asymptomatic carrier (a heterozygote). One in 700 newborns of African heritage is affected.1 Although it is the most severe of the common sickle cell diseases (which...

The interactions of normal erythrocytes and erythrocytes from patients having hemoglobin S hemoglobinopathies with normal human endothelial cells (EC) were investigated under flow conditions. When EC supernatant, containing 2.8-11.0 U/dl of von Willebrand factor (vWF) antigen and vWF multimeric forms larger than those present in normal plasma, was the red blood cell (RBC)-suspending medium inst...

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts...

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; ...

Sickle cell anemia is characterized by painful vaso-occlusive crises. It is hypothesized that monocytes are activated in sickle cell disease and can enhance vaso-occlusion by activating endothelium. To test this hypothesis, human umbilical vein endothelial cells (HUVEC) and human microvascular endothelial cells (MVEC) with sickle and normal mononuclear leukocytes were incubated, and endothelial...

Rheumatoid arthritis (RA) is a chronic, systemic, autoimmune disease that primarily affects the synovial joints. Sickle cell anemia chronic inherited with vaso-occlusive attacks associated hemoglobin structure defect. The coexistence of rheumatoid and sickle rarely reported in literature. In this case report, diagnosis treatment plan 26-year-old female patient who was diagnosed applied to our o...

Sickle cell disease is one of the most common hemoglobinopathy worldwide. It an autosomal recessive genetic disorder caused by replacement adenine to thymine nucleotide in beta chain hemoglobin results valine for glutamic acid at 6th position. This substitution formation sickle (HbS) which turn leads a reduced lifespan red blood cells (RBC). In hypoxic conditions, HbS has tendency aggregate and...

factor-a, which increase endothelial expression of E-selectin. These molecules tether rolling neutrophils by binding to granulocyte Lewis x sialyated carbohydrate (CD15). This allows neutrophil integrins such as complement receptor 3 (CD11b) to adhere to the endothelium via interaction with its ligand intercellular adhesion molecule-1.7 The importance of CD64 (Fc-gamma receptor I) as a marker o...