Le Pichon Jean-Baptiste, Riordan Sean M, Watchko Jon, Shapiro Steven M
Division of Child Neurology, Department of Pediatrics, Children's Mercy Hospital, Kansas City, MO. United States.
Department of Neurology, University of Kansas Medical Center, Kansas City, KS. United States.
Curr Pediatr Rev. 2017;13(3):199-209. doi: 10.2174/1573396313666170815100214.
Despite its lengthy history, the study of jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. For example, the term Bilirubin-induced Neurological Dysfunction (BIND) has been used to refer to all neurological sequelae caused by exposure to high levels of bilirubin, to only mild neurological sequelae, or to scoring systems that quantitate the progressive stages of Acute Bilirubin Encephalopathy (ABE).
We seek to clarify and simplify terminology by introducing, defining, and proposing new terms and diagnostic criteria for kernicterus.
We propose a systematic nomenclature based on pathophysiological and clinical criteria, presenting a logical argument for each term. Acknowledging observations that kernicterus is symptomatically broad and diverse, we propose the use of the overarching term Kernicterus Spectrum Disorders (KSDs) to encompass all the neurological sequelae of bilirubin neurotoxicity including Acute Bilirubin Neurotoxicity (ABE). We further suggest subclassification of KSDs based on the principal disabling features of kernicterus (motor, auditory). Finally, we suggest the term subtle KSD to designate a child with a history of significant bilirubin neurotoxicity with mild or subtle developmental delays.
We conclude with a brief description of the limited treatments currently available for KSD, thereby underscoring the importance of further research. We believe that adopting a systematic nomenclature for the spectrum of clinical consequences of hyperbilirubinemia will help unify the field and promote more effective research in both prevention and treatment of KSDs.
尽管黄疸、高胆红素血症和核黄疸的研究历史悠久，但用于描述这些病症临床和病理生理本质的关键术语缺乏清晰度和一致性。例如，胆红素诱导的神经功能障碍（BIND）这一术语被用于指代因暴露于高水平胆红素而导致的所有神经后遗症、仅轻度神经后遗症，或用于量化急性胆红素脑病（ABE）进展阶段的评分系统。
我们试图通过引入、定义和提出核黄疸的新术语及诊断标准来澄清和简化术语。
我们基于病理生理和临床标准提出一种系统的命名法，为每个术语提供合理依据。鉴于认识到核黄疸在症状上广泛且多样，我们提议使用总括性术语核黄疸谱系障碍（KSDs）来涵盖胆红素神经毒性的所有神经后遗症，包括急性胆红素神经毒性（ABE）。我们还建议根据核黄疸的主要致残特征（运动、听觉）对KSDs进行亚分类。最后，我们建议使用术语轻微KSD来指代有显著胆红素神经毒性病史且伴有轻度或轻微发育迟缓的儿童。
我们以对目前KSDs有限治疗方法的简要描述作为结论，从而强调进一步研究的重要性。我们认为，采用一种系统的命名法来描述高胆红素血症临床后果的谱系将有助于统一该领域，并促进在KSDs预防和治疗方面更有效的研究。
