G6PD Enzyme Deficiency in Neonatal Hyperbilirubinemia

Abstract

aundice is the most common condition requiring medical attention in newborns. The yellow colouration of the skin and sclera in newborns with jaundice is the result of accumulation of unconjugated bilirubin. In most infants, unconjugated hyperbilirubinemia reflects a normal transitional phenomenon. However, in some infants, serum bilirubin levels may rise exclusively, which can be a cause for concern because unconjugated bilirubin is neurotoxic and can cause death in newborns and lifelong neurologic sequelae in infants who survive (kernicterus). For these reasons, the presence of neonataljaundice frequently results in diagnostic evaluation. A common complication of G-6-PD deficiency is that of severe neonatal hyperbilirubinemia with the potential of bilirubin encephalopathy or kernicterus. Because of the association of G-6-PD deficiency with severe, acute hemolysis, this hyperbilirubinemia has traditionally been related to as hemolytic in origin. In many cases, this may indeed be the case, and substances which has been applied to the umbilicus for antisepsis, have been implicated as triggers of hemolysis. Additional triggers may include metabolites of fava, transmitted via breast milk of mothers who had ingested the bean, and henna, whic