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Sickle cell disorder, β-globin gene cluster haplotypes and α-thalassemia in neonates and adults from Guadeloupe

✍ Scribed by Kéclard, L.; Romana, M.; Lavocat, E.; Saint-Martin, C.; Berchel, C.; Mérault, G.

Publisher
John Wiley and Sons
Year
1997
Tongue
English
Weight
47 KB
Volume
55
Category
Article
ISSN
0361-8609

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✦ Synopsis

We have studied haplotype of ␤ S chromosome and ␣-globin gene status in 534 patients (255 adults and 279 children of whom 159 neonates) from Guadeloupe with various sickle cell-related conditions, namely SS (n = 298), SC (n = 170), S-␤-thal (n = 56), and other rare forms (n = 10). Haplotype data on ␤ S chromosomes confirm our previous observation that Benin type is the most prevalent (75%) ␤ S chromosome in Guadeloupe, in disagreement with the historical records. Comparison of the frequency of distribution of various ␤ S haplotypes between neonates and adults on the one hand and between SS and SC cases on the other shows that the current ␤ S haplotype distribution in this island is not distorted by haplotype-related differential survival. We also show that the frequency of ␣-thalassemia (-3.7 kb) in Guadeloupe is one of the highest recorded in this region involved in Atlantic slave trade and also failed to reveal any age-dependent increase in frequency. We conclude that the African component of Guadeloupe is distinct from that of Brazil and Cuba but is close to that of Jamaica. Am.

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