Medical and Health Project

1. DEFINITIONS:
• Sickle cell disease: an inherited blood disorder characterized from an abnormality of hemoglobin that is altered structural b-globin chain due to the substitution of a single amino acid (glutamic acid to valine) resulting in hemoglobin S
(HbS).
• facilforme Feature: The child is carrying the defective gene, HbS, but also has some normal hemoglobin, HbA. This is called HBAs. Children who have sickle cell trait usually have no symptoms at of the disease.
• Sickle cell anemia: Most or all of the normal hemoglobin (HbA) the child is changed by sickle hemoglobin (HbS). This is
referred to as HbSS. It is the most common and more severe varieties cell anemia.
2. PATHOPHYSIOLOGY
The pathophysiology of sickle cell anemia lies in changing the characteristics of hemoglobin when Hb soluble monomers are transformed gelled polymer to environmental variations in pH, temperature and ion concentrations. The result is the production of rigid red cells, which together to abnormal interactions established with the vascular endothelium determines the sickling phenomenon and vascular occlusion. Among the factors that determine the tendency to form polymer shows values of HbF.
(more…)