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...

BACKGROUND Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported. METHODS We administered the Medical Outcomes Study 36-item Short-Form to 308 patients in the Pain in Sickle Cell Epidemiology Study (PiSCES) to assess HRQOL. Scale...

BACKGROUND In patients with sickle cell trait or disease, reduced life expectancy and a tendency for complications are believed to negatively affect likelihood of survival after open heart surgery. The aim of this study was to review retrospectively the perioperative results of patients undergoing cardiac surgery at our institution. METHODS AND RESULTS Between January 1995 and December 2006, ...

‘Sickle hepatopathy’ is a condition which seen in only 10% cases of sickle cell disease (SCD). Sickle hepatopathy could be due to acute crisis, sequestration, cholangiopathy, severe intrahepatic cholestasis or as result chronic blood transfusion related infection and hemosiderosis. Differentiating the cause for SCD challenging. Hepatopathy manifestation can range from mild liver function test a...

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...

A 17 year old boy with sickle cell anaemia presented with acute myocardial infarction associated with severe hypoxia and reticulocytopenia. Ischaemic heart disease is rare in sickle cell anemia and in this case it is possible that the acute episode of hypoxia led to myocardial infarction.

In Yemen, the prevalence of sickle cell trait and β-thalassemia trait are high. The aim of this premarital program is to identify sickle cell and thalassemia carrier couples in Yemen before completing marriages proposal, in order to prevent affected birth. This can be achieved by applying a low-cost premarital screening program using simple blood tests compatible with the limited health resourc...

background & objectives: the researcher clarified that β/globin gene cluster haplotypes in patients with sickle cell anemia provide useful population data as predictors of the disease severity, gene flow, and the origins of sickle cell mutation in this region. materials and methods: a total of 150 subjects was investigated in two different groups for five polymorphism restriction sites of t...

Background & objectives: The researcher clarified that β/Globin gene cluster haplotypes in patients with sickle cell anemia provide useful population data as predictors of the disease severity, gene flow, and the origins of sickle cell mutation in this region. Materials and methods: A total of 150 subjects was investigated in two different groups for five polymorphism restriction site...

Patients with sickle cell anemia experience severe vascular occlusive phenomena including acute pain crisis and cerebral infarction. Obstruction occurs at both the microvascular and the arterial level, and the clinical presentation of vascular events is heterogeneous, suggesting a complex etiology. Interaction between sickle erythrocytes and the endothelium may contribute to vascular occlusion ...