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 |Favorite Table|Download (.pdf)
Table 9-1. Population-Based Estimates of Kernicterus Incidence
|Author(s)||Country||Years||Ascertainment Case Definition||No. of Cases||Denominator||Rate|
|Bjerre and Ebbesen2||Denmark||1994–2002||Registry; voluntary reports; ≥35 weeks gestation; TSB ≥31.1 mg/dL; symptoms of chronic bilirubin encephalopathy||8||576,000||1.4/100,000|
|Bjerre et al.3||Denmark||1994–2002||National laboratory information system linked to medical reports; ≥35 weeks gestation and ≤28 days of age; TSB ≥26.5 mg/dL and advanced phase symptoms of bilirubin encephalopathy||1||249,308a||0.4/100,000a|
|Manning et al.4||United Kingdom||2003–2005||Voluntary reports; ≥35 weeks gestation and <1 month of age; TSB ≥30 mg/dL; death or postmortem examination or typical sequelae at 12-month follow-up||7||1,500,052||0.46/100,000|
|Sgro et al.5||Canada||2002–2004||Surveillance program; voluntary reports; ≥35 weeks gestation and ≤60 days of age TSB ≥25 mg/dL and/or exchange transfusion and clinically important neurologic abnormalities at final discharge||13||640,000||2/100,000|
|Sgro et al.6||Canada||2007–2008||Surveillance program; voluntary reports; any child up to 6 years old; ≥35 weeks gestation at birth; TSB ≥25 mg/dL or exchange transfusion and two or more signs/symptoms of kernicterus; or abnormal MRI with history of hyperbilirubinemia||22||900,000||2.4/100,000|
|Burke et al.7||United States||1988–2005||Hospital discharge abstracts; ≤30 days of age; ICD-9 for kernicterus and CPT for phototherapy or exchange transfusion||436||Not stated||2.7/100,000|
|Brooks et al.8||California||1988–1997||State registry for developmental services; ICD-9 for kernicterus||25||5,697,147||0.49/100,000|
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