What is G6PD deficiency?

More common in MENA, since if heterozygous provide protection against malaria, Xlinked (avoid oxidants), other examples Pyruvate kinase. Lack of sufficient G6PD leads to oxidation damaging RBC due to inadequate NADPH (prevents oxidative stress by reducing glutathione by glutathione reductase). HMP shunt is the way of producing NADPH, leading to reduced glutathione, detoxify peroxidase and free radicals w/in RBC preserving RBC membrane integrity. So in G6PD deficiency we see hemolysis, and reticulocytes appear w/in 5-10 days, and have G6PD activity to compensate the loss, so ends the anemia w/in a week (self limited) Tx (remove inciting agent of hemolysis, maybe aggressive hydration for acute intravascular hemolysis/transfusion for severe anemia), chronic hemolysis give folic acid Test (first resolve hemolysis then test, used for people in need for oxidant drug