Glucose-6-phosphate dehydrogenase deficiency

An inherited deficiency of glucose-6-phosphate dehydrogenase that reduces red blood cells’ ability to withstand oxidative stress, sometimes causing hemolysis.

Glucose-6-phosphate dehydrogenase: An enzyme that generates NADPH in the pentose phosphate pathway, supporting antioxidant defenses in cells. Its reduced activity is the defining biochemical defect.
Fava bean: The seed of Vicia faba, which can provoke hemolysis in some people with G6PD deficiency. Its oxidant compounds explain the condition’s traditional name, favism.
X-linked recessive inheritance: A pattern in which variants on the X chromosome usually cause disease more often in males than females. The G6PD gene lies on the X chromosome, shaping who is affected and how.
Pyruvate kinase deficiency: An inherited red-cell enzyme deficiency that causes chronic hemolytic anemia through impaired glycolysis. It causes hemolysis through energy failure, not impaired oxidative defense.
Pentose phosphate pathway: A metabolic pathway that produces NADPH and ribose-5-phosphate from glucose-6-phosphate. It supplies the NADPH that deficient red cells cannot readily replace.
Primaquine: An antimalarial medicine that can cause hemolysis in people with G6PD deficiency. Testing can guide its safe use, especially before radical cure of malaria.
G6PD gene: The X-chromosomal gene that encodes glucose-6-phosphate dehydrogenase. Inherited variants in this gene produce the enzyme deficiency.
Hereditary spherocytosis: An inherited red-cell membrane disorder that produces spherical cells and chronic hemolysis. Its membrane defect contrasts with G6PD-related vulnerability to oxidants.
NADPH: A cellular electron carrier that provides reducing power for biosynthesis and antioxidant reactions. Low NADPH limits the red cell’s capacity to restore reduced glutathione.
Dapsone: An antimicrobial and anti-inflammatory drug that can cause oxidative hemolysis in susceptible patients. It is a clinically relevant oxidant exposure for people with this deficiency.

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