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G6PD deficiency

posted Nov 24, 2009, 12:51 AM by DERC 藥物教育資源中心

(Originally posted on 2004-10-20)

Drugs and chemicals to be avoided in Glucose-6-phosphate dehydrogenase deficiency

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest enzyme disorder of human beings and a globally important cause of neonatal jaundice, which can lead to kernicterus and death or spastic cerebral palsy. It can also lead to life-threatening haemolytic crises in childhood and at later ages, by interacting with specific drugs and with fava beans in the diet.

The best known morbid effect of G6PD deficiency is hemolysis induced by oxidative drugs. When prescribing drugs for G6PD-deficient subjects, two points should be kept in mind: (1) different genetic variants of G6PD deficiency entail different susceptibility to the hemolytic risk from drugs; thus a drug found to be safe in some G6PD-deficient subjects may not be equally safe in others; (2) the risk and severity of hemolysis is almost always dose-related.

Drugs and chemicals may be separated into those that must be avoided by G6PD-deficient subjects (such as sulphonamides, quinolones, nitrofurantoin), and those that do not readily precipitate hemolysis but must nevertheless be prescribed with caution.

Very few G6PD-deficient individuals with chronic non-spherocytic haemolytic anaemia have haemolysis even in the absence of an exogenous trigger. These patients must be regarded as being at high risk of severe exacerbation of haemolysis following administration of ANY of the drugs listed in the following tables1:

Drugs & chemicals with definite risk of haemolysis in most G6PD-deficient individuals:

Acetanilid2Primaquined,1
Dapsone & sulphonesa,1Quinolonese,1
Furazolidone2Sulphonamidesf,1
Isobutyl nitrate2Sulfacetamide2
Methylthioninium chloride1 (Methylene blue)Sulfamethoxazole2 (e.g. Septrin)
Naphthaleneb,c,2Sulfanilamide2
Nitrofurantoin1Sulfapyridine2
Niridazole1Toluidine bluec,2
Pamaquin1Trinitrotoluenec,2 (TNT)
Phenazopyridine2 (Pyridium) 

Drugs & chemicals with possible risk of haemolysis in most G6PD-deficient individualsg

Aspirinh,1Menadione1
Ascorbic acidi,2,3 (Vitamin C)Probenecid1
Chloramphenicol2,3 (Kemicetine)Quinidinej,1
Chloroquinej,1Quininej,1

Footnotes:

  1. Higher doses for dermatitis herpetiformis more likely to cause problems1;
  2. In mothball;
  3. Chemicals;
  4. 30mg weekly for 8 weeks has been found to be without undue harmful effects in African and Asian people1;
  5. Quinolones include: ciprofloxacin1, levofloxacin3, moxifloxacin3, nalidixic acid1, norfloxacin1 & ofloxacin1;
  6. Including co-trimoxazole; some sulphonamides, e.g. sulfadiazine, have been tested and found not to be haemolytic in many G6PD-deficient individuals1;
  7. Drugs in this table can probably be given in normal therapeutic doses to G6PD deficiency without non-spherocytic haemolytic anaemia2
  8. Acceptable up to a dose at least 1g daily in most G6PD-deficiency individuals1
  9. Very high therapeutic doses (~80g administered intravenously) have precipitated severe, even fatal, haemolysis2
  10. Acceptable in acute malaria1.

The tables in this bulletin are compiled based on the most updated reference materials available at the time of publication, and are therefore not intended to be comprehensive. Other drugs and chemicals might later found to be incompatible with G-6PD deficiency, the reader is advised to exercise caution and clinical judgment when using these tables.

Reference

  1. British National Formulary 46; September 2003, p447-448.
  2. Beutler E. "G6PD deficiency." Blood 1994; 84: p3613-3636.
  3. Micromedex Healthcare Series, Volume 119, 2004.
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