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慢性胆红素脑病:诊断与预后。

Chronic bilirubin encephalopathy: diagnosis and outcome.

机构信息

Divisions of Child Neurology and Clinical Neurophysiology, Department of Neurology, Virginia Commonwealth University, Medical College of Virginia Campus, Richmond, VA 23298-0211, USA.

出版信息

Semin Fetal Neonatal Med. 2010 Jun;15(3):157-63. doi: 10.1016/j.siny.2009.12.004. Epub 2010 Jan 29.

DOI:10.1016/j.siny.2009.12.004
PMID:20116355
Abstract

Chronic bilirubin encephalopathy (kernicterus) can be diagnosed using semi-objective criteria based on history, physical and neurological examination and laboratory findings including auditory brainstem responses and magnetic resonance imaging. Classical kernicterus is a well-described clinical tetrad of (i) abnormal motor control, movements and muscle tone, (ii) an auditory processing disturbance with or without hearing loss, (iii) oculomotor impairments, especially impairment of upward vertical gaze, and (iv) dysplasia of the enamel of deciduous teeth. Subtle kernicterus or bilirubin-induced neurologic dysfunction (BIND) refers to individuals with subtle neurodevelopmental disabilities without classical findings of kernicterus that, after careful evaluation and consideration, appear to be due to bilirubin neurotoxicity. Kernicterus can be further classified as auditory predominant or motor predominant and characterized based on the severity of clinical sequelae. Proposed research definitions for kernicterus diagnosis in infants from 3 to 18 months are reviewed, as are treatments of auditory and motor deficits and other complications of bilirubin encephalopathy.

摘要

慢性胆红素脑病(核黄疸)可根据病史、体格检查和神经学检查以及包括听脑干反应和磁共振成像在内的实验室发现,使用半客观标准进行诊断。典型的核黄疸是一种描述明确的临床四联征,包括(i)运动控制、运动和肌肉张力异常,(ii)听觉处理障碍伴或不伴听力损失,(iii)眼球运动障碍,特别是上垂直凝视障碍,以及(iv)乳牙牙釉质发育不良。轻微核黄疸或胆红素诱导的神经功能障碍(BIND)是指存在轻微神经发育障碍但无典型核黄疸发现的个体,经过仔细评估和考虑,这些障碍似乎是由于胆红素神经毒性引起的。核黄疸可进一步分为以听觉为主或以运动为主,并根据临床后遗症的严重程度进行特征描述。本文回顾了 3 至 18 个月婴儿核黄疸的诊断研究定义,以及听觉和运动缺陷以及胆红素脑病其他并发症的治疗方法。

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