Laboratory Medicine Program

Glucose-6-phosphate dehydrogenase, neonatal
(GPD, G-6-PD)

Clinical Decription:
G6PD oxidizes glucose-6-phosphate and produces reduced nicotinamide adenine dinucleotide phosphate (NADP), which provides reducing power for detoxification of oxygen radicals (Desforges JF. N Engl J Med 1991; 324: 169). In the absence of G6PD, erythrocytes are susceptible to oxidative damage. G6PD deficiency is an X-linked genetic disorder; males may be either normal or deficient hemizygotes, females may be normal homozygotes, deficient homozygotes, or heterozygotes. Due to varying X-inactivation pattern, heterozygous females can show a spectrum of G6PD activity (Algur N et al. J Pediatr 2012; 161: 197). In daily life, deficiency in G6PD activity poses very few problems. However, if the body encounters any oxidant stress, severe hemolysis may occur. The drugs listed below, infections, and fava beans are all known to be causes of hemolysis in G6PD-deficient individuals. It is therefore important to diagnose individuals suspected of the deficiency and screen their relatives. Deficient individuals should be instructed to avoid substances that may induce hemolysis. Drugs that may cause hemolysis in G6PD deficiency: Antimalarials: primaquin, chloroquin, paraquin, dapsone; Sulfonamides: septra, sulfaxosazole, etc; Nitrofurans; Others: methylene blue, probenecid, nalidixic acid, PAS, naphthalene.
<br/><em>Lohr GW, Waller HD. In methods of enzymatic analysis. Ed.H.U Bergmeyer, Academic Press, N.Y., 1964:636. Ellers RJ. Notification of final adoption of an international method and standard solution for hemoglobinometry specifications for preparations of standard solution. Am J Clin Path, 1967;47:212</em>

Method: Turbidometry, c8000; c8000

Component Tests Used: n/a

Reference Ranges Used:
6.0 - 16.0 U/g Hb

Specimen Type: EDTA microtainer, no gel
Collected In: EDTA
Volume: 10 uL (minimum: 10 uL)

Shipping: 4C

Special Instructions: Do not centrifuge.

Testing Schedule(s): Weekly.

Turnaround Time: 5 days

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