OBJECTIVES This study compared the subjective well-being of adults with homozygous sickle cell (SS) disease to a matched group of healthy adult peers. The differential influence of sociodemographic factors on the subjective well-being of Sickle Cell patients was also examined. METHODS The Ferran and Powers Quality of Life Index and the Positive and Negative Affect Schedule were used to assess...

CONTEXT Vascular occlusion in sickle cell disease causes increased levels of plasma cell-free DNA as a result of cell death and tissue damage. OBJECTIVES This study investigates plasma cell-free DNA concentrations in sickle cell disease patients, and aims at exploring the significance of plasma cell-free DNA as a potential biomarker in predicting its complications. DESIGN Plasma cell-free D...

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...

BACKGROUND AND PURPOSE SWI is a powerful tool for imaging of the cerebral venous system. The SWI venous contrast is affected by blood flow, which may be altered in sickle cell disease. In this study, we characterized SWI venous contrast in patients with sickle cell disease and healthy control participants and examined the relationships among SWI venous contrast, and hematologic variables in the...

In sickle cell disease transfusions improve blood flow by reducing the proportion of red cells capable of forming sickle hemoglobin polymer. This limits hemolysis and the endothelial damage that result from high proportions of sickle polymer-containing red cells. Additionally, transfusions are used to increase blood oxygen carrying capacity in sickle cell patients with severe chronic anemia or ...

Pain is the most common symptom in sickle cell disease (SCD) patients [1]. In acute setting, SCD may have painful crises that require hospitalization [2]. However, these also ...

Sickle cell haemoglobin (HbS) results from an autosomal recessively inherited mutation in which the 17th nucleotide of the beta globin gene is changed from thymine to adenine and the amino acid glutamic acid is replaced by valine at position 6 in the beta globin chain. [1, 2]Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chron...

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...

Our objective was to document the prevalence of sickle cell anemia among scheduled tribe (Garasia) of Sirohi district in Rajasthan state and study the clinical and hematological profile of the patients with sickle cell disease (Hb SS). In this prospective cross-sectional study, 1676 Garasia tribals attending the hospital or the mobile clinic were screened for sickle cell anemia by sickling test...

Increased leukocyte adhesion to vascular endothelium contributes to vaso-occlusion in sickle cell disease. Since nitric oxide bioavailability is decreased in sickle cell disease and nitric oxide may inhibit leukocyte adhesion, we investigated whether stimulation of NO-signaling pathways can reduce the adhesive properties of neutrophils from sickle cell disease individuals (sickle cell diseasene...