Because Berkeley sickle cell mice are used as an animal model for human sickle cell disease, we investigated the progression of the histopathology in these animals over 6 months and compared these findings to those published in humans with sickle cell disease. The murine study groups were composed of wild-type mixed C57Bl/6-SV129 (control) mice and sickle cell (SS) mice (alpha-/-, beta-/-, tran...

The spectrum of sickle cell disease (SCD) encompasses a heterogeneous group of disorders that include: (I) homozygous SCD (HbSS), also referred to as sickle cell anaemia; (ii) heterozygous SCD (HbAS), also referred to as sickle cell trait; and (iii) compound heterozygous states such as HbSC disease, HbSβ thalassaemia, etc. Homozygous or compound heterozygous SCD patients manifest with clinical ...

background: sickle cell disease occurs due to a mutation in β chains and the substitution of valine instead of glutamate in the sixth position of the ß-chain that causes polymerization and vascular blockage. the aim of this study was to compare the serum c, s proteins and factor v leiden between sickle cell patients and the control group. materials and methods: in this case-control study, perfo...

Concentrations of two primary bile acids (cholic acid and chenodeoxycholic acid) were determined by radioimmunoassay in the serum of 15 African homozygous sickle cell patients, ages ranging from 4 to 22 years. The mean serum levels of the two primary bile acids studied were significantly elevated when compared with the normal mean values. About 67% of the patients had pathological elevation of ...

Sickle cell disease (SCD) is a known inherited hemoglobin disorder featured by the presence of sickle shaped erythrocytes in the blood. It can cause cerebrovascular accident (CVA) in adults and children and is responsible for the majority of the strokes in children. Repeated blood transfusion are often required in an attempt to dilute blood thus reducing the risk of vaso-occlusion and stroke in...

heterozygous and homozygous states to explain the symptomless sickle cell trait and the true sickle cell anaemia. An individual receiving the haemoglobin S gene from one parent only (the heterozygous state A/S) would have the sickle cell trait, whereas if the gene should be received from both parents (the homozygous state 5/5) he would have sickle cell anaemia (Fig. I). Sickle cell anaemia and ...

BACKGROUND Genes for haemoglobin S are found in high frequencies in Nigeria. However, there is little information on beta thalassemia in sickle cell anaemia in this population. The clinical presentation of HbS- β thalassemia is enormously variable, ranging from an asymptomatic state to a severe disorder similar to homozygous sickle cell disease. MATERIALS AND METHODS Haemoglobin A2 and HbF we...

Sickle cell anemia is a genetic disease that primarily affects the black population. This anemia is due to a homozygous state of the abnormal hemoglobin S. An alteration occurs on the DNA molecule involving the substitution of the amino acid valine for glutamic acid at the sixth position on the beta polypeptide chain. This biochemical variation on the DNA molecule creates a physiological change...