Glucose-6-Phosphate Dehydrogenase Deficiency

作者：Mak GK, Shah M · 年份：2025

Glucose-6-phosphate dehydrogenase (G6PD) is a cytosolic "housekeeping" enzyme present in all cells that helps prevent damage from reactive oxygen species by generating substrates needed for antioxidative defenses. Erythrocytes are especially vulnerable to reactive oxygen species because they transport oxygen and, once mature, cannot replace damaged proteins. Inherited G6PD deficiency can therefore lead to acute hemolytic anemia during periods of increased oxidative stress. Triggers include infections, fava beans, and several medications. Antimalarials, in particular, are well known to precipitate hemolysis in affected patients. The Clinical Pharmacogenetics Implementation Consortium has provided a comprehensive, periodically updated list. High- and medium-risk medications that have been associated with hemolytic crises in G6PD deficiency include: High-risk medications: Dapsone. Methylene blue. Pegloticase. Phenazopyridine. Primaquine (standard dosage). Rasburicase. Tafenoquine. Toluidine blue. Medium-risk medications: Nitrofurantoin. Primaquine (medium dosage) .