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A study of the causes of anaemia in the south west Pacific archipelago of Vanuatu has identified one island, Maewo, where the carrier rate for beta thalassaemia exceeds 20%, one of the highest recorded incidences in the world. Homozygous beta thalassaemia is a major cause of infant mortality and a serious drain on health resources on this island. Interactions of beta thalassaemia with various forms of alpha + thalassaemia were common in this population. Coexistent alpha + thalassaemia leads to better haemoglobinised and larger red cells than are seen in simple beta thalassaemia heterozygotes and screening for the latter can only be reliably carried out by Hb A2 estimation.

Original publication

DOI

10.1136/jmg.24.6.357

Type

Journal article

Journal

J Med Genet

Publication Date

06/1987

Volume

24

Pages

357 - 361

Keywords

Female, Ferritins, Fetal Hemoglobin, Gene Frequency, Globins, Hemoglobin A, Heterozygote, Male, Melanesia, Pacific Islands, Pedigree, Thalassemia