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...

Sickle cell disease affects 70,000 Americans who experience an average of 0.8 painful episodes each year. The pathophysiology of sickle cell pain is not completely understood. The disease is characterized by both acute and chronic pain syndromes. Patients with sickle cell pain often encounter barriers to receiving appropriate care, including lack of continuity of care and perceived opiate addic...

Proliferative sickle cell retinopathy is an uncommon complication in individuals with sickle cell trait (AS). However, the risk for proliferative retinopathy development is increased in patients with AS hemoglobinopathy associated with systemic conditions or ocular trauma. A case of a patient with AS hemoglobinopathy who developed proliferative sickle cell retinopathy after the occurrence of ge...

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 ...

The aim of the study was to determine the factors associated with resting and exercise-induced hemoglobin oxygen desaturation. The well-established six-minute walk test was conducted in 107 sickle cell children (50 with sickle hemoglobin C disease and 57 with sickle cell anemia) at steady state. Hemoglobin oxygen saturation was measured before and immediately after the six-minute walk test. Blo...

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...

Activation of vascular endothelium plays an essential role in vasoocclusion in sickle cell disease. The anti-inflammatory agents dexamethasone and adhesion molecule-blocking antibodies were used to inhibit endothelial cell activation and hypoxia-induced vasoocclusion. Transgenic sickle mice, expressing human alpha-, beta(S)-, and beta(S-Antilles)-globins, had an activated vascular endothelium i...

Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the β-globin gene on chromosome 11, which leads to a single amino acid substitution (glutamine to valine). Sickle-(δβ)0-thalassemia is a rare variant of sickle cell disease (delta-beta thalassemia occurring in association with sickle hemoglobin, HbS), sparsely reported in literature, and has been associated with symptomatol...

BACKGROUND There are a paucity of data on epistaxis as it pertains to sickle cell anaemia. Some case studies suggest epistaxis to be a significant complication in patients with sickle cell anaemia in sub-Saharan Africa; however, no robust studies have sought to establish the epidemiology or pathophysiology of this phenomenon. METHODS We conducted a case-control study with the aim of investiga...

An automated technique is described which is capable of detecting sickle-cell haemoglobin and differentiating the sickle-cell trait from sickle-cell anaemia. The method is based upon the Itano solubility test and utilizes Technicon equipment.