Haemoglobin Bart's hydrops syndrome in Greece.
- 26 July 1980
- Vol. 281 (6235) , 268-270
- https://doi.org/10.1136/bmj.281.6235.268
Abstract
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 alpha-thalassaemia genes, and globin-chain synthesis analysis of the present fetus showed a total absence of alpha-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 alpha-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 zeta-chain genes. Thus the severe form of alpha-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.Keywords
This publication has 12 references indexed in Scilit:
- The chromosomal arrangement of human α-like globin genes: Sequence homology and α-globin gene deletionsCell, 1980
- Haemoglobin Bart's hydrops fetalis syndrome in an infant of Greek origin and prenatal diagnosis of alpha-thalassaemia.1979
- K562 human leukaemic cells synthesise embryonic haemoglobin in response to haeminNature, 1979
- The duplicated human alpha globin genes lie close together in cellular DNA.Proceedings of the National Academy of Sciences, 1978
- Gene deletion as the cause of α thalassaemia: Genetic lesion in homozygous α thalassaemia (hydrops fetalis)Nature, 1974
- Gene deletion as the cause of α thalassaemia: The severe form of α thalassaemia is caused by a haemoglobin gene deletionNature, 1974
- Hemoglobins in Human Fetuses: Evidence for Adult Hemoglobin Production After the 11th Gestational WeekBlood, 1972
- The Genetical Interpretation of Haemoglobin H DiseaseHuman Heredity, 1970
- The Pattern of Disordered Haemoglobin Synthesis in Homozygous and Heterozygous β-ThalassaemiaBritish Journal of Haematology, 1969
- Case of Intrauterine Death Due to -ThalassaemiaBMJ, 1965