Department of Pediatrics, Aichi Medical University, Japan.
Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan.
Brain Dev. 2020 Nov;42(10):730-737. doi: 10.1016/j.braindev.2020.06.010. Epub 2020 Jul 10.
To examine the clinical characteristics of bilirubin encephalopathy in preterm infants (pBE) in Japan.
We performed a two-step nationwide questionnaire survey. The initial survey determined the number of children with pBE. Using a structured questionnaire, the second survey clarified the clinical manifestations and characteristics of children with pBE, including the perinatal history, neonatal complications, neurological features, verbal communication, diet, and magnetic resonance imaging (MRI) and auditory brainstem response (ABR) findings.
The initial survey included 190 pBE infants, indicating an incidence of approximately 10 per year. Clinical information was available for 142 of them. The median gestational age was 26 weeks and the median birthweight was 883 g. As to neonatal complications, 20% had none, 25% had one complication, 54% had two or more. Head control was observed in 45% and functional gait in 8%. Purposeful hand use was seen in 41% of patients and verbal communication in 40%. MRI showed T2 hyperintensities in the globi pallidi in 111 of 136 patients, especially between 7 and 18 months of corrected age. ABR abnormalities were present in 88 of 117 patients.
pBE was infrequent but constantly observed during the study period, especially in very preterm infants, even in those with no severe neonatal complications. Severely impaired gross motor function and relatively preserved manual function and verbal communication were characteristic. MRI and ABR abnormalities will facilitate diagnosis.
研究日本早产儿胆红素脑病（pBE）的临床特征。
我们进行了两步全国性问卷调查。初步调查确定了患有 pBE 的儿童人数。使用结构化问卷，第二次调查阐明了患有 pBE 的儿童的临床表现和特征，包括围产期史、新生儿并发症、神经特征、言语交流、饮食以及磁共振成像（MRI）和听性脑干反应（ABR）结果。
初步调查包括 190 例 pBE 婴儿，每年发病率约为 10 例。其中 142 例有临床信息。中位胎龄为 26 周，中位出生体重为 883 克。至于新生儿并发症，20%无并发症，25%有 1 种并发症，54%有 2 种或更多并发症。45%的患儿能够控制头部，8%能够功能性行走。41%的患儿能够有意使用手，40%的患儿能够言语交流。在 136 例患者中，111 例 MRI 显示苍白球 T2 高信号，尤其是在矫正年龄 7-18 个月时。117 例患者中有 88 例 ABR 异常。
pBE 在研究期间虽然不常见，但一直在观察到，尤其是在极早产儿中，即使在没有严重新生儿并发症的情况下也是如此。严重受损的粗大运动功能和相对保留的手动功能和言语交流是其特征。MRI 和 ABR 异常将有助于诊断。
