Hemoglobin Bart's hydrops syndrome in Greece.

A case of haemoglobin Bart's hydrops syndrome was characterised in a Greek family with a history of three other fetuses with hydrops. Family studies showed that both the mother and father carried α-thalassaemia genes, and globin-chain synthesis analysis of the present fetus showed a total absence of α-chain production. The haemoglobin composition of the fetus was similar to that seen in cases in south-east Asia, and analysis of DNA from the Greek case confirmed the total deletion of the α-chain genes. The extent of the deletion, however, differed from that seen in south-east Asian cases and included the loss of one of the embryonic ζ-chain genes. Thus the severe form of α-thalassaemia occurs in Greece but has arisen independently from, the similar condition in south-east Asia. The condition must be considered in any woman of this racial background who gives a history of unexplained stillbirths. [ABSTRACT FROM AUTHOR]