Red cell hexose monophosphate shunt activity measurement for rapid determination of severity of G6PD deficiency

Persons having an inborn deficiency of erythrocytic glucose-6-phosphate dehydrogenase (G6PD) may be resistent to malaria infection and susceptible to acute intravascular hemolysis following administration of a therapeutic regimen of one of several known hemolytic antimalarial agents. Measurement of red cell hexose monophosphate shunt (HMS) activity provides a direct measure of G6PD activity because this enzyme limits overall HMS activity. In the erythrocyte, respiration is achieved through glycolysis, and therefore CO2 release from these cells may be attributed to glucose passage through the HMS. Incubation of intact red cells in the presence of D(1-14C) glucose with subsequent recovery and quantitation of 14CO2 provides a direct measure of HMS activity. The procedure described in this report provides a measure of relative G6PD activity which may putatively identify a G6PD deficient individual while at the same time providing a reliable estimate of the severity of G6PD deficiency