Abstract
THE occurrence of erythrocyte glucose-6-phosphate dehydrogenase deficiency in patients with haemoglobin H disease was first reported by Ramot, Sheba, Fisher, Ager and Lehmann1 and Vella2. The former investigators considered the association in their patient to be coincidental, but Vella2 reported that three of his six patients with haemoglobin H disease were deficient in this enzyme. Using the assay method of Kornberg and Horecker8 we have determined the erythrocyte glucose-6-phosphate dehydrogenase levels in twenty consecutive, but unrelated, Chinese patients (eight males and twelve females) with haemoglobin H disease and found only one female to be enzyme deficient. The association of these two defects is therefore not so common as would appear from the report by Vella2. Although the number of patients investigated is not large, the incidence of erythrocyte glucose-6-phosphate dehydrogenase deficiency in haemoglobin H disease is probably not greater than that in the population —5.5 per cent in males4 and 8.4 per cent in females (using calculations described by Allison5 and assuming that approximately only 80 per cent of female heterozygotes are identified by the assay procedure6).
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References
Ramot, B., Sheba, C., Fisher, S., Ager, J. A. M., and Lehmann, H., Brit. Med. J., 2, 1228 (1959).
Vella, F., Experientia (Basle), 16, 284 (1960).
Kornberg, A., and Horecker, B. L., in Methods of Enzymology, 1 (Academic Press Inc., New York, 1955).
Chan, T. K., Todd, D., and Wong, C. C., Brit. Med. J., 2, 102 (1964).
Allison, A. C., Nature, 186, 531 (1960).
Tarlov, A. R., Brewer, G. J., Carson, P. E., and Alving, A. S., Arch. Intern. Med., 109, 209 (1962).
Rigas, D. A., and Koler, R. D., J. Lab. and Clin. Med., 58, 417 (1961).
Marks, P. A., Science, 127, 1338 (1958).
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CHAN, T., TODD, D. & WONG, C. Erythrocyte Glucose-6-phosphate Dehydrogenase Activity in Haemoglobin H Disease. Nature 209, 1147–1148 (1966). https://doi.org/10.1038/2091147a0
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DOI: https://doi.org/10.1038/2091147a0
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