Rates of Extreme Neonatal Hyperbilirubinemia and Kernicterus in Children and Adherence to National Guidelines for Screening, Diagnosis, and Treatment in Sweden
Rates of Extreme Neonatal Hyperbilirubinemia and Kernicterus in Children and Adherence to National Guidelines for Screening, Diagnosis, and Treatment in Sweden
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DOI：
10.1001/jamanetworkopen.2019.0858
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发表时间：
2019-03-01
影响因子：
13.8
通讯作者：
Norman, Mikael
中科院分区：
文献类型：
--
作者：
Alken, Jenny;Hakansson, Stellan;Norman, Mikael
新生儿高胆红素血症可导致终身神经发育障碍（核黄疸），即使在高资源环境中。更好地了解导致核黄疸的发病率和过程可能有助于预防措施的设计。目的：确定危险的高胆红素血症和核黄疸在近足月新生儿中的发病率，并评估卫生保健专业人员对最佳实践的坚持。这些人口-一项基于全国范围的队列研究，前瞻性收集了所有妊娠35周或更长时间存活并接受新生儿护理的婴儿的最高血清胆红素水平数据2008年至2016年，瑞典共有46个分娩单位和37个新生儿单位。对患有危险性高胆红素血症的新生儿的病历进行了评价，以获得最佳新生儿实践和2岁以下核黄疸的诊断。数据分析在2017年9月至2018年2月期间进行。（血清胆红素水平，25.0-29.9mg/dL [425-509 μ mol/L]）和危险（血清胆红素水平，>30.0mg/dL [>510 μ mol/L]）新生儿高胆红素血症。主要结果和测量主要结果是核黄疸，定义为危险的新生儿高胆红素血症，随后为脑瘫、感音神经性听力损失、凝视麻痹或神经发育迟缓。次要结果是卫生保健专业人员遵守国家指南，使用预先定义的10个关键性能指标进行诊断和治疗，以及评估是否可以避免与胆红素相关的脑损伤。（958 051例足月分娩和34 327例近期分娩），494（320名男孩;平均[SD]出生体重，3505 [527] g）发展为极度高胆红素血症（50/100 000婴儿），6.8/100 000婴儿发生危险的高胆红素血症，1.3/100 000婴儿发生核黄疸。在13名发生核黄疸的儿童中，基于以下一种或几种可能的原因，11名儿童的脑损伤被评估为可能可以避免：出院前胆红素筛查不及时或缺乏（n = 6），胆红素值错误解读（n = 2），不及时或延迟开始强化光疗治疗（n = 1），不及时或不进行换血治疗（n = 6），或尽管有指征但缺乏重复换血（n = 1）。结论和相关性危险的高胆红素血症在近足月或足月新生儿中仍然发生在瑞典，并且与每百万新生儿中13例的致残性脑损伤相关。对于大多数这些情况下，医疗保健专业人员不遵守最佳做法被确定，这表明这些情况下，很大一部分可能是可以避免的。
IMPORTANCE Neonatal hyperbilirubinemia can cause lifelong neurodevelopmental impairment (kernicterus) even in high-resource settings. A better understanding of the incidence and processes leading to kernicterus may help in the design of preventive measures.OBJECTIVES To determine incidence rates of hazardous hyperbilirubinemia and kernicterus among near-term to term newborns and to evaluate health care professional adherence to best practices.DESIGN, SETTING, AND PARTICIPANTS This population-based nationwide cohort study used prospectively collected data on the highest serum bilirubin level for all infants born alive at 35 weeks' gestation or longer and admitted to neonatal care at all 46 delivery and 37 neonatal units in Sweden from 2008 to 2016. Medical records for newborns with hazardous hyperbilirubinemia were evaluated for best neonatal practices and for a diagnosis of kernicterus up to 2 years of age. Data analyses were performed between September 2017 and February 2018.EXPOSURES Extreme (serum bilirubin levels, 25.0-29.9mg/dL [425-509 mu mol/L]) and hazardous (serum bilirubin levels, >30.0mg/dL [>510 mu mol/L]) neonatal hyperbilirubinemia.MAIN OUTCOMES AND MEASURES The primary outcome was kernicterus, defined as hazardous neonatal hyperbilirubinemia followed by cerebral palsy, sensorineural hearing loss, gaze paralysis, or neurodevelopmental retardation. Secondary outcomeswere health care professional adherence to national guidelines using a predefined protocol with 10 key performance indicators for diagnosis and treatment as well as assessment of whether bilirubin-associated brain damage might have been avoidable.RESULTS Among 992 378 live-born infants (958 051 term births and 34 327 near-term births), 494 (320 boys; mean [SD] birth weight, 3505 [527] g) developed extreme hyperbilirubinemia (50 per 100 000 infants), 6.8 per 100 000 infants developed hazardous hyperbilirubinemia, and 1.3 per 100 000 infants developed kernicterus. Among 13 children developing kernicterus, brain injury was assessed as potentially avoidable for 11 children based on the presence of 1 or several of the following possible causes: untimely or lack of predischarge bilirubin screening (n = 6), misinterpretation of bilirubin values (n = 2), untimely or delayed initiation of treatment with intensive phototherapy (n = 1), untimely or no treatment with exchange transfusion (n = 6), or lack of repeated exchange transfusions despite indication (n = 1).CONCLUSIONS AND RELEVANCE Hazardous hyperbilirubinemia in near-term or term newborns still occurs in Sweden and was associated with disabling brain damage in 13 per million births. For most of these cases, health care professional noncompliance with best practices was identified, suggesting that a substantial proportion of these cases might have been avoided.