Hereditary spherocytosis epidemiology and demographics
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
HS is seen in all populations but appears to be especially common in people of northern European ancestry.
Epidemiology and Demographics
Incidence
- In the United States, the incidence of the disorder is approximately one case in 5000 people.
- Given that approximately 25% of all HS is autosomal recessive, calculations indicate that 1.4% of the US population might be silent carriers of HS.
Prevalence
- In northern European, HS affects as many as 1 in 2000 to 1 in 5000 (prevalence, approximately 0.02 to 0.05 percent) [6,7,62,75].
- The frequency is thought to be lower in individuals from other parts of the world such as Africa and Southeast Asia, although comprehensive population survey data are unavailable.
Age
- HS can present at any age and with any severity, with case reports describing a range of presentations, from hydrops fetalis in utero through diagnosis in the ninth decade of life.[1][2]
- HS is most often diagnosed in childhood or early adulthood.
- Children diagnosed early in life usually have a severe form of HS that results in their early presentation. Jaundice is likely to be most prominent in newborns. The magnitude of hyperbilirubinemia may be such that exchange transfusion is required. Approximately 30-50% of adults with HS had a history of jaundice during the first week of life. Recognition of HS as a potential cause of neonatal anemia and hyperbilirubinemia and institution of prompt treatment may reduce the risk of bilirubin-induced neurologic dysfunction in these patients.[3]
Race
Gender
Region
- Hereditary spherocytosis occurs in 1 in 5,000 individuals of Northern European ancestry. This condition is the most common cause of inherited anemia in that population. The prevalence of hereditary spherocytosis in people of other ethnic backgrounds is unknown, but it is much less common.
Developed Countries
There is no particular relation of FA with developed countries.
Developing Countries
There is no particular relation of FA with developing countries.
References
- ↑ Perrotta S, Gallagher PG, Mohandas N (2008). "Hereditary spherocytosis". Lancet. 372 (9647): 1411–26. doi:10.1016/S0140-6736(08)61588-3. PMID 18940465.
- ↑ Whitfield CF, Follweiler JB, Lopresti-Morrow L, Miller BA (1991). "Deficiency of alpha-spectrin synthesis in burst-forming units-erythroid in lethal hereditary spherocytosis". Blood. 78 (11): 3043–51. PMID 1954389.
- ↑ Christensen RD, Yaish HM, Gallagher PG (2015). "A pediatrician's practical guide to diagnosing and treating hereditary spherocytosis in neonates". Pediatrics. 135 (6): 1107–14. doi:10.1542/peds.2014-3516. PMC 4444801. PMID 26009624.