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Sickle Cell Disease

Sickle Cell Disease is a topic covered in the Select 5-Minute Pediatrics Topics.

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Sickle cell disease (SCD) is a hemoglobinopathy caused by a one base pair change leading to an amino acid change in the beta globin gene at the sixth position: glutamic acid to valine. The mutation allows the polymerization of hemoglobin in the red cell.


  • Disruption of the red cell membrane: leading to increased adhesion to vascular endothelium, activation of cytokines leading to activation of platelets and leukocytes, activation of the coagulation system leading to a hypercoagulable state and ultimately to vaso-occlusion. Red cells release red cell membrane as microparticles.
  • Red cell hemolysis: release of free hemoglobin, methemoglobin production, increased plasma ferric iron leading to oxidative stress, decreased nitric oxide leading to decreased production of cyclic GMP causing vasoconstriction, inflammation, and platelet activation


  • The incidence of SCD is 1 in 500 African Americans, 1 in 36,000 Hispanics, with a lesser frequency in other ethnic groups. The incidence of sickle cell trait in African Americans is 1 in 14.
  • There are 70,000–100,000 affected individuals with SCD in the United States.

Risk Factors


  • SCD is autosomal recessive.
  • Combinations leading to the disease state when inherited with hemoglobin S: SS, SC, S beta zero thalassemia, S beta plus thalassemia, S DLos Angeles, and S OArab are examples.

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Cabana, Michael D., editor. "Sickle Cell Disease." Select 5-Minute Pediatrics Topics, 7th ed., Wolters Kluwer Health, 2015. Medicine Central, im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14112/all/Sickle_Cell_Disease.
Sickle Cell Disease. In: Cabana MD, ed. Select 5-Minute Pediatrics Topics. 7th ed. Wolters Kluwer Health; 2015. https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14112/all/Sickle_Cell_Disease. Accessed August 21, 2019.
Sickle Cell Disease. (2015). In Cabana, M. D. (Ed.), Select 5-Minute Pediatrics Topics. Available from https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14112/all/Sickle_Cell_Disease
Sickle Cell Disease [Internet]. In: Cabana MD, editors. Select 5-Minute Pediatrics Topics. Wolters Kluwer Health; 2015. [cited 2019 August 21]. Available from: https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14112/all/Sickle_Cell_Disease.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Sickle Cell Disease ID - 14112 ED - Cabana,Michael D, BT - Select 5-Minute Pediatrics Topics UR - https://im.unboundmedicine.com/medicine/view/Select-5-Minute-Pediatric-Consult/14112/all/Sickle_Cell_Disease PB - Wolters Kluwer Health ET - 7 DB - Medicine Central DP - Unbound Medicine ER -