ABSTRACT
Glucose-6-phosphate dehydrogenase deficiency, the most worldwide common enzyme deficiency, causes a spectrum of diseases including neonatal hyperbilirubinemia, acute and chronic hemolysis. Persons with this condition also may be asymptomatic. This X-linked inherited disorder most commonly affects persons of African, Asian, Mediterranean, or middle-Eastern descent. Approximately 400 million people are affected in the world. Prevalence of G6PD deficiency in Tehran was 2.1% [3.6% boys- 0.6% girls] in Babol [12.5% boys, 4.1% girls] in Sari [14.3% boys, 3% girls]. This condition usually is diagnosed by fluorescent spot test. Different gene mutation different levels of enzyme deficiency, with classes assigned to various degrees of deficiency and disease manifestation. Acute hemolysis is usually caused by exposure to an oxidative stressor in the form of oxidative drug or fava beans and is self-limited, but in rare instances it can be severe enough to warrant a blood transfusion. Neonatal hyperbilirubinemia may require treatment with phototherapy or exchange transfusion to prevent kernicterus. Variant that causes chronic hemolysis is uncommon and is related to sporadic gene mutations. The prevalence of molecular mutation varied in north of Iran, Mazandaran [66.25% Mediterranean, 27% Cantham and 6.75% Cosensa]. Gilan [86.4% Mediterranean, 9.7% Cantham], Golestan [69% Mediterranean, 2.67% Cantham]. The most important way for prevention is screening of neonate for G6PD deficiency and avoidance of exposure to oxidant agent, Fava bean and also early treatment of neonatal jaundice