OBJECTIVE To determine the incidence and etiology of extreme neonatal hyperbilirubinemia, defined as total serum bilirubin (TSB) ≥450 µmol/L, and kernicterus spectrum disorder (KSD) in Denmark between 2000 and 2015. STUDY DESIGN We identified all infants born between 01.01.2000 and 31.12.2015 with TSB ≥450 µmol/L, ratio of conjugated to TSB <0.30, gestational age ≥35 weeks, and postnatal age ≤4 weeks, using Danish hospitals' laboratory databases. RESULT We included 408 infants. The incidence of extreme neonatal hyperbilirubinemia among infants with gestational age ≥35 weeks was 42/100,000 during the study period with a seemingly decreasing incidence between 2005 and 2015. Twelve of the 408 infants developed KSD, (incidence 1.2/100,000) Blood type ABO isohemolytic disease was the most common explanatory etiology. CONCLUSIONS Our study stresses the importance of a systematic approach to neonatal jaundice and ongoing surveillance of extreme neonatal hyperbilirubinemia and KSD.

中文翻译：

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2000-2015年间丹麦的极端新生儿高胆红素血症和角核谱疾病。

目的确定2000至2015年间丹麦的极端新生儿高胆红素血症的发生率和病因，其定义为总血清胆红素（TSB）≥450µmol / L，以及kerkerterterus谱障碍（KSD）。研究设计我们确定了所有出生于01.01之间的婴儿。使用丹麦医院的实验室数据库，在2000年和2015年12月31日，TSB≥450µmol / L，TSB的结合比率<0.30，胎龄≥35周，出生后≤4周。结果我们纳入了408名婴儿。在研究期间，胎龄≥35周的婴儿中的极端新生儿高胆红素血症的发生率为42 / 100,000，在2005年至2015年之间似乎有所下降。408名婴儿中有十二名发生了KSD，（发生率1.2 / 100,000）疾病是最常见的解释性病因。