Glucose-6-Phosphate Dehydrogenase Deficiency

Excerpt

Glucose-6-phosphate dehydrogenase (G6PD) is an enzyme found in the cytoplasm of all cells in the body. It is a housekeeping enzyme that plays a vital role in the prevention of cellular damage from reactive oxygen species (ROS). It does this by providing substrates to prevent oxidative damage. Erythrocytes are particularly vulnerable to ROS due to their role in oxygen transport and inability to replace cellular proteins as mature cells. Inherited deficiencies of G6PD can result in acute hemolytic anemia during times of increased ROS production. This may be caused by stress or exposure to certain foods that contain high amounts of oxidative substances, for example, fava beans, or certain medications. In particular, anti-malarial agents have a strong association with inducing hemolytic anemia in patients with G6PD deficiency. Below are medications more commonly used in the United States that have been shown to trigger a hemolytic crisis in those with G6PD deficiency; however, a more comprehensive list of medications to be avoided has been published by the Italian G6PD Deficiency Association and can be found at www.g6pd.org.

Common medications to be avoided or used with caution in G6PD-deficient patients include:

1. Acetaminophen

2. Acetylsalicylic acid

3. Chloramphenicol

4. Chloroquine

5. Colchicine

6. Diaminodiphenyl sulfone

7. Diphenhydramine

8. Glyburide

9. Isoniazid

10. L-Dopa

11. Methylene blue

12. Nitrofurantoin

13. Phenazopyridine

14. Primaquine

15. Rasburicase

16. Streptomycin

17. Sulfacetamide

18. Sulfanilamide

19. Sulfapyridine

20. Sulfacytine

21. Sulfadiazine

22. Sulfaguanidine

23. Sulfamethoxazole

24. Sulfisoxazole

25. Trimethoprim

26. Tripelennamine

27. Vitamin K