Detection of Deficient Erythrocyte Regeneration of Reduced Triphosphopyridine Nucleotide from Glucose-6-Phosphate

Abstract
A simple, rapid test is described for the detection of glucose-6-phosphate dehydrogenase deficiency of red blood cells by employing the principle of reduced triphosphopyridine nucleotide (TPNH) regeneration. This test, the brilliant cresyl blue (BCB) reduction test, was compared with the glutathione (GSH) stability test in 629 patients and, in addition, with the TPNH regeneration test in 179 individuals. Agreement between these tests was good. The BCB reduction test was thus established as a reliable method for detection of this hereditary defect. The GSH levels as tested by the nitroprusside method compared well with those obtained by the glyoxalase technique, indicating that both tests measure primarily the sulfhydryl groups of GSH. Deficient TPNH regeneration was found in 12% of the Negro population (14% males and 6% females) of the Memphis area. In 125 Negroes who had various hemoglobinopathies, no correlation of the two hereditary defects was observed. Despite good agreement between the GSH stability and the TPNH regeneration tests, a straight-line correlation could not be demonstrated. This finding, coupled with the discovery of occasional "intermediate" males and of defective TPNH regeneration in some Caucasians and in some patients with hereditary non-spherocytic anemia, is interpreted to suggest that deficient TPNH regeneration may be the result of several enzymatic factors, not necessarily related.