The hemizygous males (n = 2) and homozygous female (n = 1) for the G6PD Aures mutation presented with a severe G6PD deficiency, whereas the phenotype of G6PD Aures heterozygous females varied from normal to moderately G6PD deficient (Table 1). The gene discussed is G6PD; the disease is hyperinsulinemic hypoglycemia, familial, 4.