Objectives

To determine the clinical features of bilirubin encephalopathy in preterm infants (pBE) in Japan.

Methods

We performed a retrospective, nationwide questionnaire-based survey. The initial survey determined the number of children with pBE who were born after 2000. Using a structured questionnaire, the second survey clarified the clinical manifestations and characteristics of children with pBE, including demographic data, neurological symptoms, and MRI and auditory brainstem response (ABR) findings.

Results

The initial survey identified 41 pBE infants from 18 institutions. After exclusion of patients included in previous studies, clinical information was collected from 30 patients (21 boys and 9 girls) during the secondary survey. The median gestational age was 26 weeks and the median birthweight was 846 g. Chronic lung disease and symptomatic patent ductus arteriosus were common neonatal complications. Head control was observed in 63% and functional gait in 17% of patients. Purposeful hand use was seen in 57% and verbal communication in 50% of patients. MRI showed T2 hyperintensities in the globus pallidus of 29 of 30 patients. ABR abnormalities were present in 11 of 15 patients. None of the variables were significantly different between the 2017 and 2021 surveys.

Conclusions

The pBE infants had severely impaired gross motor function and relatively preserved manual function and verbal communication. MRI and ABR findings aid in the diagnosis of pBE.

中文翻译：

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日本第二次全国早产儿胆红素脑病调查

 目标

旨在确定日本早产儿胆红素脑病（pBE）的临床特征。

 方法

我们进行了一项回顾性、全国性的问卷调查。初次调查确定了 2000 年以后出生的 pBE 儿童数量。第二次调查采用结构化问卷，明确了 pBE 儿童的临床表现和特征，包括人口统计数据、神经系统症状、MRI 和听觉脑干反应 (ABR) ） 发现。

 结果

初步调查确定了来自 18 个机构的 41 名 pBE 婴儿。排除先前研究中纳入的患者后，在二次调查期间收集了 30 名患者（21 名男孩和 9 名女孩）的临床信息。中位胎龄为 26 周，中位出生体重为 846 克。慢性肺病和有症状的动脉导管未闭是常见的新生儿并发症。 63% 的患者观察到头部控制，17% 的患者观察到功能性步态。 57% 的患者有目的地使用手，50% 的患者有目的地进行口头交流。 MRI 显示 30 名患者中有 29 名苍白球出现 T2 高信号。 15 名患者中有 11 名存在 ABR 异常。 2017 年和 2021 年的调查之间没有任何变量存在显着差异。

 结论

pBE 婴儿的粗大运动功能严重受损，但手部功能和言语交流相对保留。 MRI 和 ABR 结果有助于 pBE 的诊断。