RT Book, Section A1 Ballas, Samir K. A1 Smith, Wally R. A2 Gladwin, Mark T. A2 Kato, Gregory J. A2 Novelli, Enrico M. SR Print(0) ID 1179344543 T1 Acute and Chronic Pain T2 Sickle Cell Disease YR 2021 FD 2021 PB McGraw-Hill Education PP New York, NY SN 9781260458596 LK hemonc.mhmedical.com/content.aspx?aid=1179344543 RD 2024/03/29 AB Sickle cell disease (SCD) is the most common monogenic disease worldwide. Currently, about 250 million people worldwide carry the gene responsible for the various types of sickle hemoglobinopathies. About 300,000 infants are born annually with SCD worldwide, although due to the lack of universal newborn screening in the most affected countries, this number is likely to underestimate the number of infants born with SCD. In the United States, about 1 in every 500 African Americans are born with the disease, and the affected population is expected to increase in the near future.1-3 The molecular lesion of the sickle hemoglobin (HbS) is a point mutation (GAG → GTG) in exon 1 of the β-globin gene, resulting in the substitution of glutamic acid by valine at position 6 of the β-globin polypeptide chain.4,5 This single point mutation renders the sickle gene pleiotropic in nature with multiple phenotypic expressions associated with complex genetic interactions and modifiers that are not well understood.4,5