Hemoglobin Barts

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(bärts)
n. (Abbr. Hb Bart's)

An abnormal hemoglobin that is not effective in oxygen transport, found in alpha-thalassemia.

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hemoglobin made up of homotetramers of gamma globin chains. In alpha thalassemia, the underproduction of alpha globin chains stimulates the expression of gamma globin genes. As a result, gamma globin tetramers become predominant. In fetuses homozygous for deletions in alpha hemoglobin genes, the level of Bart's hemoglobin is 80%–100%, and hydrops fetalis (q.v.) results. The hemoglobin was identified at St. Bartholomew's Hospital in London. This venerable hospital, founded in 1123, is nicknamed “Bart's.” .

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Hemoglobin Barts

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Hemoglobin Barts consists of four gamma chains. It is moderately insoluble, and therefore accumulates in the red blood cells. It has an extremely high affinity for oxygen, resulting in almost no oxygen delivery to the tissues. It is produced in the disease alpha-thalassemia and in the most severe of cases, it is the only form of haemoglobin in circulation. In this situation, a fetus will develop hydrops fetalis and normally die before or shortly after birth, unless intrauterine blood transfusion is performed.

Since Hemoglobin Barts is elevated in alpha thalassaemia, it can be measured, providing a useful screening test for this disease in some populations.[1]

This variant of hemoglobin is so called as it was discovered at St. Bartholomew's Hospital in London. The hospital has the fond sobriquet, St. Barts, and the hemoglobin was named hemoglobin Barts.

Notes

  1. ^ F. P. van der Dijs, M. Volmer, D. G. van Gijssel-Wiersma, J. W. Smit, R. van Veen & F. A. Muskiet (September 1999). "Predictive value of cord blood hematological indices and hemoglobin Barts for the detection of heterozygous alpha-thalassemia-2 in an African-Caribbean population". Clinical chemistry 45 (9): 1495–1500. PMID 10471652. 

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