condition

Sickle cell disease

Sickle cell disease refers to a group of disorders resulting from mutations in the hemoglobin gene that can lead to deformation of the red blood cell (RBC) into a crescent, or sickle, shape. Hemoglobin S is formed when valine replaces glutamine at the sixth amino acid position of the beta chain of hemoglobin. Patients who inherit hemoglobin S in a homozygous autosomal recessive fashion (Hb SS) have sickle cell anemia, while those who are heterozygous have sickle trait (Hb AS).1 Sickle cell disease primarily affects persons of African, Mediterranean, Indian, and Middle Eastern descent. In the United States, African Americans are most affected, with approximately 0.15% having sickle cell anemia (Hb SS) and approximately 8% having sickle trait (Hb AS).1,2 There is also a high incidence of sickle cell disease in Hispanic Americans from the Caribbean, Central America, and South America.3,4 Children born in many hospitals in the United States are routinely screened in the neonatal period, and antenatal diagnosis is becoming more prevalent in at-risk populations. However, an index of suspicion is necessary when treating patients born outside U.S. hospitals or when results of the neonatal screen are unavailable.

Ref:
Timothy G. Givens, Chapter 127 - Sickle Cell Disease, Editor(s): Jill M. Baren, Steven G. Rothrock, John A. Brennan, Lance Brown, Pediatric Emergency Medicine, W.B. Saunders, 2008, Pages 898-904, ISBN 9781416000877, https://doi.org/10.1016/B978-141600087-7.50130-6. (https://www.sciencedirect.com/science/article/pii/B9781416000877501306)

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Added on Feb 5, 2022 by Barbara Van De Keer
Edited on Aug 29, 2022 by Barbara Van De Keer

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