Tomioka Kazumi, Nishiyama Masahiro, Nagase Hiroaki, Ishida Yusuke, Tanaka Tsukasa, Tokumoto Shoichi, Yamaguchi Hiroshi, Toyoshima Daisaku, Maruyama Azusa, Fujita Kyoko, Aoki Kazunori, Seino Yusuke, Nozu Kandai, Nishimura Noriyuki, Kurosawa Hiroshi, Iijima Kazumoto
Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.
Brain Dev. 2019 Sep;41(8):691-698. doi: 10.1016/j.braindev.2019.04.012. Epub 2019 Jul 20.
Although the mortality among previously healthy children with acute encephalopathy (AE) is approximately 5%, their detailed clinical course has not been clarified. The objective of the present study was to describe the detailed clinical course, in minutes, of fatal AE.
We retrospectively reviewed the medical records of five patients (from 6 months to 14 years of age) who previously had no neurological disorders and were diagnosed with brain death due to AE between 2002 and 2018 at Kobe Children's Hospital.
The initial clinical symptoms were convulsion in three cases and impaired consciousness in two. The earliest noted brain imaging abnormality was 7.5 h after neurological symptom detection. Liver enzymes and creatinine levels increased at initial examination, and sodium elevated gradually. All patients met the criteria of systemic inflammatory response syndrome, disseminated intravascular coagulation, and shock within 14 h of symptom detection. High dose steroids and targeted temperature management were initiated 3.5-14 h after onset. Despite these therapies, patients were diagnosed with brain death from 16 h to 4 days after initial neurological symptoms. AE diagnoses were made between 4 h 29 min and 4 days after initial neurological symptoms and included hemorrhagic shock and encephalopathy syndromes, Reye-like syndrome, and acute necrotizing encephalopathy in two, two, and one patient(s), respectively.
We revealed the time series' of clinical events (e.g. SIRS, shock, DIC, AE diagnosis, brain death, and treatments) and laboratory findings relative to initial neurological symptom in fatal AE.
尽管既往健康的急性脑病（AE）患儿死亡率约为5%，但其详细的临床病程尚未明确。本研究的目的是描述致命性AE以分钟计的详细临床病程。
我们回顾性分析了2002年至2018年期间在神户儿童医院就诊的5例（年龄6个月至14岁）既往无神经系统疾病且因AE被诊断为脑死亡患儿的病历。
初始临床症状为惊厥3例，意识障碍2例。最早发现的脑成像异常是在神经症状检测后7.5小时。初诊时肝酶和肌酐水平升高，血钠逐渐升高。所有患者在症状检测后14小时内均符合全身炎症反应综合征、弥散性血管内凝血和休克的标准。发病后3.5 - 14小时开始使用大剂量类固醇和目标温度管理。尽管采取了这些治疗措施，患者在初始神经症状出现后16小时至4天被诊断为脑死亡。AE诊断在初始神经症状出现后4小时29分钟至4天之间做出，分别包括出血性休克和脑病综合征2例、瑞氏样综合征2例、急性坏死性脑病1例。
我们揭示了致命性AE中与初始神经症状相关的临床事件（如全身炎症反应综合征、休克、弥散性血管内凝血、AE诊断、脑死亡和治疗）的时间序列以及实验室检查结果。
