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Background and Objective: Regarding to probable high frequency of Glucose - 6 - phosphate dehydrogenase (G6PD) deficiency in Iran, screening of all neonates by cord blood is under consideration. This study was conducted to determine the prevalence of G6PD deficiency in newborns and the relation between gender, jaundice, hemolysis, anemia and the G6PD deficiency in neonated born in in Tehran, Iran.

Materials and Methods: In this descriptive study, cord blood of 450 neonates born in Akbarabady hospital in Tehran, Iran during 2008-09 were screened. Demographic information was recorded by questionnaires and the newborns were examined for detection of jaundice till discharge. G6PD level was determined by Fluorescent Spot Test (FST). G6PD deficient neonate were put under close observation for detection of jaundice. Enzyme activity was rechecked by spectrophotometry.

Results: Nine neonates out of 450 were G6PD deficient (8 boys and one girl). Prevalence of G6PD deficiency was 2% (3.3% for boys and 0.5% for girls). Six neonates of nine G6PD deficient neonates (66%) developed pathologic jaundice. Four neonates were managed by phototherapy and two by exchange transfusion.

Conclusion: This study showed that G6PD deficiency is more prevalate among male neonates, therefore, G6PD determination is recommended to prevent the possible neonatal jaundice.

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Background and Objective: The national screening program for G6PD enzyme deficiency is not able to detect all affected neonates. This study was done to compare the fluorescent spot test (FST), decolorization test, and quantitative enzyme assay (QEA) for detecting G6PD enzyme deficiency in neonates. Methods: In this descriptive study, cord blood samples of 365 neonates were collected. Decolorization test, QEA and DNA test was done for each sample. All of the neonates were tested by FST as a part of national screening program on heel-prick blood sample collected on day 3–5 after birth. QEA was considered as the gold standard. According to QEA test results, neonates with <20% and 20–60% of mean normal enzyme activity were considered as total deficient and partial deficient, respectively. Results: Fluorescent spot test detected 13 male neonates with G6PD enzyme deficiency while decolorization test identified 18 male and 1 female neonates. Using QEA, 19 of male and 28 of female neonates with G6PD enzyme deficiency (26 cases with partial and 2 cases with total deficiency) were diagnosed. DNA analysis detected 34 female case as heterozygote and 14 male neonates as hemizygote for the disease. Conclusion: Fluorescent spot test do not have required sensitivity for screening of neonates with G6PD enzyme deficiency. QEA test is recommended to replace the fluorescent spot test in national screening program.