The natural history of sickle cell disease.

Cold Spring Harb Perspect Med

Sickle Cell Trust (Jamaica), Kingston 6, Jamaica.

Published: October 2013

The term sickle cell disease embraces a group of genetic conditions in which pathology results from the inheritance of the sickle cell gene either homozygously or as a double heterozygote with another interacting gene. The spectrum of resulting conditions is therefore influenced by the geography of individual hemoglobin genes, but in most populations, the commonest genotype at birth is homozygous sickle cell (SS) disease. Because this genotype generally manifests a greater mortality, the relative proportion of sickle cell genotypes is influenced by age as well as the geographical distribution of individual genes.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3784812PMC
http://dx.doi.org/10.1101/cshperspect.a011783DOI Listing

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