Observations on the epidemiology of sickle cell disease

Transactions of the Royal Society of Tropical Medicine and Hygiene
G R Serjeant

Abstract

The four common genotypes of sickle cell disease in Jamaica are homozygous sickle cell (SS) disease, sickle cell-haemoglobin C (SC) disease, sickle cell-B+ thalassemia, and sickle cell-BO thalassaemia with respective incidence at birth of 3.2, 2.0. 0.34, and 0.16 per 1000 live births. Haematological indices, clinical features, and over-all prognosis vary between these genotypes and also between patients within individual genotypes. Although symptomatic selection has tended to emphasize more severely affected patients, this wide variation of clinical and haematological severity is especially apparent in SS disease. Factors contributing to this variability in SS disease include the persistence of foetal haemoglobin, the association with alpha thalassemia, and the interaction with environmental factors of which socioeconomic status is the most obvious. Further elucidation of factors determining the severity of SS disease will increase understanding of the pathogenetic mechanisms in the disease and may also identify new possibilities for therapeutic intervention.

References

Jan 1, 1979·British Journal of Haematology·G R SerjeantB E Serjeant
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Citations

Mar 16, 2011·Nature Reviews. Urology·Belinda F Morrison, Arthur L Burnett
Dec 1, 1989·Archives of Disease in Childhood·J P Evans
May 9, 1987·British Medical Journal·M BrozovićA I Brownell
Dec 1, 1984·Journal of Clinical Pathology·M Brozović, E Anionwu
May 11, 2012·British Journal of Haematology·Ryan RodgersMhairi Copland
Aug 1, 1987·British Journal of Haematology·M O KehindeG W Marsh
Oct 1, 1985·American Journal of Hematology·R L Nagel, M E Fabry

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