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February 1964

Erythrocyte Glucose-6-Phosphate Dehydrogenase: A Study of the Enzyme Activity in Children

Am J Dis Child. 1964;107(2):180-184. doi:10.1001/archpedi.1964.02080060182013

Hemolytic conditions following the administration of primaquine, naphthalene, sulfonamides, nitrofurantoin (Furadantin), sulfisoxazole (Gantrisin), acetophenetidin (phenacetin), vitamin K analogues, fava beans, and other drugs or chemicals have been intensively investigated.1-6 Biochemical studies have revealed a new type of intrinsic red cell abnormality. Erythrocytes from individuals susceptible to these hemolytic conditions have revealed a deficiency in reduced glutathione (GSH), a decreased activity of glucose-6-phosphate dehydrogenase (G6PD), an abnormal glutathione stability test,6-9 and an increased tendency to form Heinz bodies.2,10 These biochemical defects are genetically transmitted and are probably sex-linked, the responsible gene being located on the X-chromosome.2,6,11

The basic abnormality in sensitive red cells seems to be a deficiency of G6PD,12 since the changes in glutathione metabolism have been attributed to a reduction of G6PD activity. In addition to the above abnormalities, there is a decreased glycine incorporation rate into GSH in vitro, an increase in glutathione reductase,