Endothelial Dysfunction in Adult Patients of Sickle Cell Disease

Abstract

Sickle cell disease is one of the most common hemoglobinopathy worldwide. It is an autosomal recessive genetic disorder caused by replacement of adenine to thymine nucleotide in the beta chain of hemoglobin results in replacement of valine for glutamic acid at 6th position. This substitution results in formation of sickle hemoglobin (HbS) which in turn leads to a reduced lifespan of red blood cells (RBC). In hypoxic conditions, HbS has a tendency to aggregate and form fibrillar structure called tactoid within the red cells