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As noted in several other chapters in this book, bilirubin encephalopathy and kernicterus are still occurring throughout the world with population-based estimates of incidence in North America and Europe ranging from 0.5 to 2.4 cases per 100,000 live births1 (Table 9-1). In contrast to the early clinical case descriptions, most of the infants who now develop kernicterus are not those with Rh disease and they often have no documented evidence of hemolytic disease.9 Many are term and late preterm infants who have been discharged from the nursery as “healthy newborns,” yet have returned to a pediatrician's office, a clinic, or an emergency department with total serum bilirubin (TSB) levels often exceeding 30 mg/dL9—and have gone on to develop the classic neurodevelopmental findings associated with kernicterus.10 There is also a smaller group of infants, more difficult to identify, who suffer an unanticipated precipitous increase in the TSB while still in the hospital or soon after discharge and present with acute bilirubin encephalopathy.9,11,12 Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an important cause of the hyperbilirubinemia in some of these infants.9,12

Table 9-1. Population-Based Estimates of Kernicterus Incidence

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