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Abstract Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is the most common human enzyme defect, affects more than 400 million people worldwide, most commonly males due to X-Linked heritability (Luzzatto et al., 2020) . Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is an X-Linked recessive disorder with varied clinical presentations including neonatal jaundice, hemolysis, acute icterus after exposure to chemicals and drugs, anemia, acute jaundice following consumption of fava beans (favism), and also congenital chronic non-spherocytic hemolytic anemia (Richardson and O’Malley, 2020) . Neonatal jaundice is the earliest manifestation of G6PD deficiency, and the most critical sign for early diagnosis of this genetic disorder (Roper et al., 2020) . The incidences of G6PD deficiency in icteric newborns in numerous studies were found to be from 3.5% to 40% (Kasemy et al., 2020) . Two Egyptian studies, one at Cairo University and another at Menoufia University found that the prevalence of G6PD deficiency among jaundiced neonates was 14.9% and Introduction 12 8.9% respectively (Elella et al., 2017; M Abo El Fotoh and Rizk, 2016) . The American Academy of Pediatrics (AAP) recognizes G6PD deficiency as a major risk factor for the development of sever neonatal hyperbilirubinemia, and recommends that treatments such as phototherapy and exchange transfusion, occur at lower total bilirubin levels in infants with G6PD deficiency than in those without (Sinha et al., 2017) . Around 5% of neonates with G6PD deficiency will develop jaundice after the first 24 hours of life (in contrast to fetal erythroblastosis), and their serum indirect bilirubin reaches a peak at days 3 to 5, often more than 20mg/dl. When jaundice becomes apparent from the end of first week, its peak may be delayed up to the 2 nd week. Early diagnosis of deficiency of G6PD is quit important, because this disorder may cause sever hemolysis and anemia in the newborn, if undiagnosed (Elella et al., 2017) . World Health Organization (WHO) data suggest that the global infant mortality due to G6PD- associated neonatal jaundice is in the range of 0.7ــ 1.6 per 1000 of all birth. Infants, who survive the acute hyperbilirubinemia, may develop sever athetoid cerebral palsy, auditory dysfunction, Introduction 13 intellectual and other handicaps associated with chronic bilirubin encephalopathy (M Abo El Fotoh and Rizk, 2016) . Neonatal screening programs coupled with parental and medical caretaker education may be successful in limiting the severity of disease (Luzzatto et al., 2020) |