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Background: Newborn screening is an area with potential for immense impact in lifelong morbidity and mortality. Objective: To determine cost effective for glucose-6-phosphate dehydrogenase (G-6-PD) enzyme screening and prevalence of deficiency in newborns and children in Tehran, Iran. Materials and Methods: All full term newborns, neonates with icterus and children with acute hemolysis were evaluated to determine prevalence of G-6-PD enzyme deficiency and cost effectiveness of a screening test for G-6-PD enzyme deficiency. The qualitative color reduction test performed on healthy newborns and quantitative test on newborns with icterus and children with acute hemolysis. Results: Three (2%) of 146 screened newborns were G-6-PD deficient at a cost of $ 18 for three positive screening tests and $ 856 for 143 unaffected newborns Thirty-one (11.4%) of 272 newborns readmitted with icterus were G-6-PD deficient with a cost of 31 tests were $186 and $1446 for unaffected newborns. The only significant difference between G-6-PD deficient and normal newborns was bilirubin level (p < 0.001). Eleven (0.2%) of 5054 hospitalized children were found to be G-6-PD deficient at a screening cost of $ 66 and $ 3.258 for remainder children. The prevalence of G-6-PD is estimated to be approximately 2.4% and 2.2% in males and females respectively. Conclusion: Glucose-6- phosphate dehydrogenase screening in newborns is not cost effective and for prevention of hemolysis during the next years all newborns admitted with icterus should be evaluated for G-6-PD enzyme deficiency

Prevalence of Glucose-6-Phosphate Dhydrogenase Deficient Neonates and Cost Effective of Enzyme Screening in Tehran, Iran