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-thalassemias are among the most common autosomal recessive disorders. They have a remarkably high frequency in the Mediterranean region, the Middle East, the Indian subcontinent, and East Asia. The
-thalassemias are not uncommon among persons of African descent,1 and the migration of populations has carried them to Northern Europe, North and South America, and Australia.
In the
-thalassemias there is reduced (
+) or no (
0) production of the
-globin chains of hemoglobin, which normally consist of two
-globin and two
-globin chains (
2
2). In homozygotes, a shortage of
chains results in an excess of
chains, which
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