ABSTRACT:
Aim: To review the inheritance, manifestation and management of glucose-6-phosphate dehydrogenase deficiency Objective: The article reviews the inheritance, management and manifestation of glucose-6- phosphate dehydrogenase (G6PD) deficiency. Background: Glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) is an X-linked recessive genetic condition that predisposes to hemolysis and jaundice. Very severe crises can cause acute renal failure. The G6PD/NADPH pathway is the only source of reduced glutathione in RBC. The role of haemoglobin as oxygen carrier exposes it to the risk of damage fromoxidising free radicals except for the protective effect of G6PD/NADPH/glutathione. Hemolysis in G6PD patients may be due to Antimalarial drugs like primaquine and pamaquine, Sulphonamides such as sulphanilamide, sulfamethoxazole, thiazolesulfone, methylene blue and naphthalene , certain analgesics like aspirin, phenazopyridine and acetanilide and non-sulfa antibiotics like nalidixic acid, nitrofurantoin, isoniazid, dapsone and furazolidone. These drugs should be avoided in G6PD patients. Reason: The reason is to increase the awareness of potential risks and their prevention in patients with glucose-6-phosphate dehydrogenase deficiency.