The accepted importance of circulatory impairment to sickle cell anemia remains to be verified by in vivo experimentation. Intravital microscopy studies of blood flow in patients are limited to circulations that can be viewed noninvasively and are restricted from deliberate perturbations of the circulation. Further knowledge of sickle blood flow abnormalities has awaited an animal model of huma...

The pattern of membrane abnormalities in sickle red blood cells suggests that sickle hemoglobin damages membrane proteins. We have previously shown a functional defect in sickle ankyrin, poor spectrin-binding ability. Here we examine the other major binding interactions of sickle membrane proteins including spectrin self-association, binding of ankyrin and protein 4.1 to protein 3, and the form...

Background: Molecular genetic factors regulating hemoglobin F (Hb F) expression are important modifiers of the severity of sickle cell anemia (SS). Methods: The prevalence of XmnI polymorphic site, the Gg:Ag ratio and the Hb F level were determined using PCR-RFLP procedure, HPLC and alkaline denaturation method, respectively, in various haplotypes of 52 patients with SS, 18 patients with sickle...

A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not previously known to cause retinopathy and surprisingly was observed in a non-Asian individual. The ...

Sickle cell disorder is the most important genetic haematological disease that affects the people of black African descent. About 2% of all babies born to Nigerian parents have sickle cell anaemia (1). The distribution of indigenous sickle cell disorder coincides with the distribution of falciparum malaria. Possession of sickle cell trait confers a natural protection against death from malaria ...

OBJECTIVE To determine whether sickle cell carriers ('sickle cell trait') have an increased risk of venous thromboembolism (VTE). DESIGN Cohort study with nested case-control analysis. SETTING General population with data from 609 UK general practices in the Clinical Practice Research Datalink (CPRD). PARTICIPANTS All individuals registered with a CPRD general practice between 1998 and 20...

Erythrocyte magnesium (Mg2+) deficiency has been demonstrated in sickle cell disease to contribute to erythrocyte dehydration, K loss, and thus sickling. No studies have assessed the functional properties of the Na/Mg exchanger in sickle cell disease. Using Mg(2+)-loaded erythrocytes, we measured Mg2+ efflux induced by extracellular Na+. We estimated that the Na/Mg exchanger had higher maximal ...

Transgenic sickle mice expressing betaS hemoglobin have activated vascular endothelium that exhibits enhanced expression of NF-kappaB and adhesion molecules that promote vascular stasis in sickle, but not in normal, mice in response to hypoxia/reoxygenation. Sickle mice hemolyze rbcs in vivo as demonstrated by increased reticulocyte counts, plasma hemoglobin and bilirubin, and reduced plasma ha...

Sickle cell trait is not usually regarded as a disease state because it has complications that are either uncommon or mild. Nevertheless, under unusual circumstances, serious morbidity or mortality can result from complications related to polymerization of deoxy-hemoglobin S. A previous study was earlier conducted to study Sickle cell traits and it revealed that there was enhanced lipid per oxi...

BACKGROUND Renal failure is common among older patients with sickle cell disease; this is preceded by subclinical glomerular hyperfiltration. Data about renal function of adults with sickle cell disease have been reported, but data on children is scarce, especially when comparing heterozygotic and homozygotic patients. OBJECTIVE The goal of this study was to investigate the glomerular filtrat...