الفهرس 
Sickle cell anemia.Only 14 pages are availabe for public view
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Abstract
Sickle cell anemia (SCA) is the most common form of sickle cell disease and worldwide, it is one of the commonest inherited disorders. The prevalence of sickle cell disease is highest in sub-Saharan Africa. Current studies demonstrate that over 230,000 affected children are born in this region annually which is an estimated 80% of the global total. Globally, Nigeria has the highest prevalence of the disease with reported prevalence values of between 2% and 3%.
The manifestations seen in SCA are as a result of two major pathophysiological processes: vaso-occlusion with ischemia-perfusion injury and hemolytic anemia.
Hypercoagulability in SCA may be responsible for the increased development of vascular occlusion in certain organs and acute pain episodes. The causes of hypercoagulability in SCA are multifactorial. Factors such as increased plasma levels of homocysteine, reduced levels of natural anticoagulants like Protein C and S.
Homocysteine is a highly reactive sulfur-containing amino acid which is known to cause endothelial injury, endothelial dysfunction, and thrombin formation. Studies have revealed that elevated plasma total homocysteine may result from a deficiency of folate and vitamin B12 and could, therefore, be used as a surrogate marker of folate and cobalamin (vitamin B12) deficiency. Elevated plasma homocysteine levels are also associated with increased risk of cardiovascular diseases such as atherosclerosis, coronary artery disease, and ischemic stroke. Homocysteine has also been proposed as a hemolytic toxin. Although the precise mechanism of the hemolytic effect of homocysteine is not clear, its pro- oxidant attributes have been suggested as a cause.
The aim of this study was to assess serum homocysteine concentration in patients with sickle cell disease. This study was conducted in Hematology unit, pediatric department, Menoufia university hospital. It included 60 subjects; 30 children patients below 18 years old age both sex with SCD randomly selected from the inpatient wards and outpatient clinic of pediatric Department, Faculty of Medicine, Menoufia university. In addition to 30 apparently healthy children matching age and gender served as control group.