Sickle cell disease is one of the commonest single gene disorders in man and has a widespread distribution in different parts of the world with variable clinical manifestations. An important clinical issue requiring further clarification is the effect of this abnormal hemoglobin on the physical growth and development of children with sickle cell disease. Earlier reports have shown that American...

The experiences of young people living with a sickle cell disorder in schools in England are reported through a thematic analysis of forty interviews, using Bourdieu's notions of field, capital and habitus. Young people with sickle cell are found to be habitually dys-positioned between the demands of the clinic for health maintenance through self-care and the field of the school, with its empha...

BACKGROUND Deoxygenation of sickle erythrocytes activates a cation permeability of unknown molecular identity (Psickle), leading to elevated intracellular [Ca(2+)] ([Ca(2+)](i)) and subsequent activation of K(Ca) 3.1. The resulting erythrocyte volume decrease elevates intracellular hemoglobin S (HbSS) concentration, accelerates deoxygenation-induced HbSS polymerization, and increases the likeli...

Sickle red blood cell (RBC) adhesion to the blood vessel wall is hypothesized to be the initiating event in the periodic vaso-occlusive episodes that characterize sickle cell disease (SCD). Thrombospondin-1 (TSP) and von Willebrand factor (vWF) have each been implicated in the adhesion of sickle RBC to vascular endothelial cells (EC) and subendothelial matrices. To better understand the contrib...

This work aimed to characterize mortality by sickle cell disease in Brazil. The MEDLINE electronic database was searched using the terms 'mortality' and 'sickle cell disease' and 'Brazil' for articles published in the last five years aiming to provide a current analysis of the subject in question. Eight studies on mortality by sickle cell disease were carried out in the Brazilian states of Mara...

RBCs from individuals with sickle cell disease are more susceptible to oxidant damage. Because key antioxidant defense reactions are linked to the pyridine nucleotides nicotinamide adenine dinucleotide (NAD) and nicotinamide adenine dinucleotide phosphate (NADP), we tested the hypothesis that the RBC redox potential as manifested by the NADH/[NAD+ + NADH] and NADPH/[NADP+ + NADPH] ratios is dec...

Blood microparticles (MPs) in sickle cell disease (SCD) are reportedly derived only from erythrocytes and platelets. Yet in SCD, endothelial cells and monocytes are activated and abnormally express tissue factor (TF). Thus, sickle blood might contain TF-positive MPs derived from these cells. With the use of flow cytometry to enumerate and characterize MPs, we found total MPs to be elevated in c...

Haematuria is a well-known complication of sickle cell disease. A South African coloured patient with repeated episodes of gross haematuria is described in whom the diagnosis of sickle cell disease was suggested after the finding of sickled erythrocytes in the urine sediment. The diagnosis was then confirmed by haemoglobin electrophoresis, which revealed sickle cell trait (Hb-AS). It is conclud...

BACKGROUND Women with sickle cell disease have an increased risk of pregnancy-related complications and need safe, effective contraceptive methods to prevent unintended pregnancy. STUDY DESIGN We conducted a systematic review to examine the safety of hormonal and intrauterine contraceptive use among women with sickle cell disease. RESULTS Eight articles met the inclusion criteria. The evide...

Sickle cell disease is an inherited, structural hemoglobin defect with multisystemic sequelae including renal failure. Patients with sickle cell disease are thought to benefit from renal transplant, but the long-term outcomes in such patients are unclear and have not been supported by any large prospective studies. Similarly, the renal morbidity and outcome after transplant in patients with sic...