Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC), which apparently shows a less severe cl...

OBJECTIVE To identify behavioral manifestations of executive dysfunction that are associated with cerebrovasculopathy, as measured by transcranial Doppler (TCD) ultrasonography, in children with sickle cell disease (HbSS). METHODS Participants were 62 children and adolescents with HbSS disease who had no documented history of cerebrovascular accident. Children were classified according to the...

We are reporting in vitro observations of sickle-unsickle transformation of freely suspended single cells (HbSS) induced by oxygenation-deoxygenation. The events were recorded by cinematography (phase optics, 16 mm cinematography). In addition, cells were fixed after progressive increments of oxygenation for subsequent scanning-beam electron microscopy. These studies seem to warrant the followi...

Abstract With global demand for SARS-CoV-2 testing ever rising, shortages in commercially available viral transport media pose a serious problem laboratories and health care providers. For reliable diagnosis of other respiratory viruses, executed by Real-time PCR, the quality specimens, predominantly determined storage conditions, is crucial. Therefore, our aim was to explore reliability minima...

Purpose To identify genetic, systemic, and biological factors associated with the occurrence of sickle cell maculopathy (SCM). evaluate microvascular macular alterations using optical coherence tomography angiography (OCTA) in disease (SCD). Design Cross-sectional study. Methods One hundred fifty-one eyes 78 adult SCD patients (43 HbSS, 30 HbSC, 4 S/β+, 1 HbS Lepore) 40 20 healthy controls unde...

Name Type Company Catalog Number Comments Dithizone reagent Dissolve 5mg Dithizone in 5 ml of DMSO in a 50 ml conical tube. Add 45 ml of normal saline and mix gently. Filter with a 60 cc syringe and a 0.22 "m syringe filter into a new 50 ml tube. Store at 4 OC, until use. Add 1 ml to each 35 mm counting Grid-plate, when needed. PBS Buffer HBSS Buffer Isolation soln. Buffer HBSS containing 10 mM...

Malignancies have been reported to occur in people with sickle cell disease. Renal medullary carcinoma (RMC), also tagged seventh sickle cell nephropathy, is an aggressive cancer seen almost exclusively in people with sickle cell disease with more than 160 cases reported worldwide, but only few cases were reported in patients with sickle cell anaemia (HBSS) and from Nigeria. Sarcomatoid renal c...

Risk factors for Buruli ulcer (BU) are poorly understood. We conducted a case-control study in southern Benin to investigate the association between haemoglobin variants S or C and BU, and particularly the association between haemoglobinopathies HbSS/SC and BU osteomyelitis. We compared the haemoglobin genotype of 179 patients with BU and 44 with BU osteomyelitis to that of 242 community contro...

Erythrocytes carrying a variant hemoglobin allele (HbS), which causes sickle cell disease and resists infection by the malaria parasite Plasmodium falciparum. The molecular basis of this resistance, which has long been recognized as multifactorial, remains incompletely understood. Here we show that the dysregulated microRNA (miRNA) composition, of either heterozygous HbAS or homozygous HbSS ery...