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This lecture is
based strictly on the review of Antonio Cao, Renzo Galanello and M. Cristina
Rosatelli that appeared in 1998 in Bailliere’s Clinical Haematology Vol. 11,
215-238. These genetic diseases are evolutionary results of selection by
malaria. Therefore as the selector is known and also in action as in
tropical Africa, the globin genes are untrivaled in the study of evolution.
The immense question: What happens when the selection pressure is removed?,
is answered by the distributions of these hemoglobinopathies. This group of
blood diseases has high incidences in the Mediterranean, the Middle East,
India, the Far East and tropical Africa. Today these diseases are also found
in northern Europe, North and South America and the Caribbean. About 250
million people or 4.5 % of the world population carry defective globin
genes. Each year about 300,000 affected homozygotes are born equally divided
between a) sickle cell anemia and b) a and
b
thalassemias. |