DOI: 10.1111/j.1365-2141.1970.tb01449.x. The Haemoglobin Constitution of Infants with the Haemoglobin Bart's Hydrops Foetalis Syndrome

The Haemoglobin Constitution of Infants with the Haemoglobin Bart's Hydrops Foetalis Syndrome

10.1111/j.1365-2141.1970.tb01449.x

Crossref journal-article

Wiley

British Journal of Haematology (311)

Abstract

Summary. The haemoglobin constitution of 14 infants with the haemoglobin Bart's hydrops syndrome has been examined. Thirteen of the infants were stillborn or lived only a few minutes after delivery, but in one case an exchange transfusion was performed and the pattern of globin chain synthesis examined by an [H3]leucine incorporation experiment utilizing umbilical cord blood. These studies indicate that the syndrome results from a total deficiency of α‐chain production. Furthermore, it has been possible to exclude the presence of fragments of α‐chain longer than II residues in the cells of these infants.In each infant a small quantity of haemoglobin was found which consisted of normal γ‐chains in association with chains of unique constitution. A similar component was found in trace amounts in normal umbilical cord blood. This haemoglobin may be identical with haemoglobin Portland. It is suggested that the non‐γ‐chain of this haemoglobin is the product of a normal foetal haemoglobin locus which produces very small amounts of gene product in normal infants but which is capable of increased activity in the presence of a partial or total deficiency of α‐chains.

Bibliography

Weatherall, D. J., Clegg, J. B., & Boon, W. H. (1970). The Haemoglobin Constitution of Infants with the Haemoglobin Bartâs Hydrops Foetalis Syndrome. British Journal of Haematology, 18(3), 357â368. Portico.

Authors 3

D. J. Weatherall (first)
J. B. Clegg (additional)
Wong Hock Boon (additional)

References 24 Referenced 107

10.1136/bmj.1.5076.929
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{'key': 'e_1_2_1_21_1', 'volume-title': 'The Thalassaemia Syndromes', 'author': 'Weatherall D. J.', 'year': '1965'} / The Thalassaemia Syndromes by Weatherall D. J. (1965)
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10.1111/j.1365-2141.1969.tb00400.x
{'key': 'e_1_2_1_24_1', 'first-page': '8', 'article-title': 'Hydrops foetalis in Singapore', 'volume': '1', 'author': 'Wong Hock Boon', 'year': '1965', 'journal-title': 'Far East Medical Journal'} / Far East Medical Journal / Hydrops foetalis in Singapore by Wong Hock Boon (1965)
10.1056/NEJM196701052760103

Dates

Type	When
Created	17 years, 5 months ago (March 12, 2008, 9:48 a.m.)
Deposited	1 year, 9 months ago (Nov. 11, 2023, 5:56 a.m.)
Indexed	1 year ago (Aug. 22, 2024, 2:17 p.m.)
Issued	55 years, 6 months ago (March 1, 1970)
Published	55 years, 6 months ago (March 1, 1970)
Published Online	17 years, 5 months ago (March 12, 2008)
Published Print	55 years, 6 months ago (March 1, 1970)

Funders 0

None

BibTeX

@article{Weatherall_1970, title={The Haemoglobin Constitution of Infants with the Haemoglobin Bart’s Hydrops Foetalis Syndrome}, volume={18}, ISSN={1365-2141}, url={http://dx.doi.org/10.1111/j.1365-2141.1970.tb01449.x}, DOI={10.1111/j.1365-2141.1970.tb01449.x}, number={3}, journal={British Journal of Haematology}, publisher={Wiley}, author={Weatherall, D. J. and Clegg, J. B. and Boon, Wong Hock}, year={1970}, month=mar, pages={357–368} }

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