G6PD deficiency

- G6PD is an enzyme required for maintaining normal life span of RBCs.

(Rate limiting enzyme in pentose phosphate pathway----> maintaining glutathione levels ----->preventing oxidative damage to RBCs)



- X-linked recessive disorder.
- 400 million people affected worldwide
- Common in africa, southeast asia
- Most are asymptomatic. May cause acute or chronic hemolysis, neonatal hyperbilirubinemia
 - Avoid oxidative stress - infection, oxidative drugs, fava beans

- Aspirin up to 1 gm daily dose is usually harmless (e.g. - for prophylaxis of pre-eclampsia)

- Neonatal screening should be done if family history, ethnic origin suggests.