Abe Yuriko, Ayusawa Mamoru, Kawamura Kengo, Yonezawa Ryuta, Kato Masataka, Komori Akiko, Kohira Ryutaro, Morioka Ichiro
Department of Pediatrics and Child Health, Nihon University School of Medicine, 30-1, Oyaguchi, Kami-cho, Itabashi-ku, Tokyo 173-8610, Japan.
Department of Pediatrics, Kobari General Hospital, Noda, Chiba 278-8501, Japan.
Open Med (Wars). 2019 Dec 26;15:8-13. doi: 10.1515/med-2020-0002. eCollection 2018.
Kawasaki disease (KD) is a form of acute multisystem vasculitis that presents with various complications, including coronary artery aneurysm. Heart failure and brain damage are rare, but life-threatening complications are associated with KD. Here, we describe a 4-year-old girl who developed intravenous immunoglobulin-resistant KD with both left ventricular failure and acute encephalopathy. On day 8 of the illness, the low left ventricular ejection fraction, mitral regurgitation, and low blood pressure, which required continuous administration of dobutamine, were observed during the treatments for KD, including intravenous immunoglobulin. She also appeared unconscious, where the electroencephalogram showed slow waves of activity in all regions of the brain. The cardiac performance improved after she received plasma exchange for three days. However, her unconsciousness with slow waves of activity on electroencephalogram and fever continued after the plasma exchange. Therefore, she was treated with methylprednisolone pulse, followed by prednisolone, as well as intravenous immunoglobulin. Finally, she recovered without any cardiac or neurological sequelae not only at the time she was discharged, but also throughout the follow-up period. The combination therapy using plasma exchange and methylprednisolone pulse may be a treatment option for severe KD with left ventricular failure and acute encephalopathy complications.
川崎病（KD）是一种急性多系统血管炎，可出现多种并发症，包括冠状动脉瘤。心力衰竭和脑损伤较为罕见，但却是与KD相关的危及生命的并发症。在此，我们描述一名4岁女孩，她患了对静脉注射免疫球蛋白耐药的KD，并伴有左心室衰竭和急性脑病。在患病第8天，在包括静脉注射免疫球蛋白在内的KD治疗过程中，观察到左心室射血分数降低、二尖瓣反流和低血压，需要持续静脉输注多巴酚丁胺。她还出现昏迷，脑电图显示大脑所有区域均有慢波活动。在接受三天的血浆置换后，心脏功能有所改善。然而，血浆置换后，她仍昏迷，脑电图有慢波活动且发热持续。因此，她接受了甲泼尼龙冲击治疗，随后口服泼尼松龙，同时静脉注射免疫球蛋白。最后，她不仅在出院时，而且在整个随访期间均未留下任何心脏或神经后遗症而康复。血浆置换和甲泼尼龙冲击联合治疗可能是伴有左心室衰竭和急性脑病并发症的重症KD的一种治疗选择。
