ABSTRACT
Sickle cell disease (SCD) is one of the most common monogenetic disorders in the world. The autosomal recessive transmission of a single-point mutation in the b-globin gene, namely the substitution of valine for glutamic acid at position 6 in the b-globin chain, results in the production of a mutant hemoglobin called hemoglobin S (1-4). While 8% of the African American population are heterozygotes, approximately 1 out of 600 have homozygous SCD at birth. In sub-Saharan Africa, an estimated 40% to 60% of individuals are heterozygotes suggesting that 1% to 4% of babies born in this region have the disease (5). Hemoglobin mutations producing SCD are also widespread in the southeastern United States, Central and South America, India, and the Arabian Peninsula.
