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丙泊酚输注综合征还是肾上腺脑白质营养不良?

Propofol infusion syndrome or adrenoleukodystrophy?

作者信息

Karaman Yucel, Goktay Aylin, Agin Hasan, Karaarslan Utku

机构信息

Anesthesiology and Reanimation Clinic, Tepecik Research and Training Hospital, Izmir, Turkey.

出版信息

Paediatr Anaesth. 2013 Apr;23(4):368-70. doi: 10.1111/pan.12113. Epub 2013 Jan 10.

Abstract

Following a propofol anesthetic, a 5-year-old girl with lower extremity spasticity seized and developed hypertriglyceridemia, hyperkalemia, and metabolic acidosis. A presumed diagnosis of propofol infusion syndrome (PRIS) was made, but further investigation revealed neonatal adrenoleukodystrophy. PRIS should be considered with this constellation of symptoms, but other neurometabolic disorders must always be ruled out.

摘要

在接受丙泊酚麻醉后,一名患有下肢痉挛的5岁女孩出现惊厥,并发展为高甘油三酯血症、高钾血症和代谢性酸中毒。初步诊断为丙泊酚输注综合征(PRIS),但进一步检查发现是新生儿肾上腺脑白质营养不良。出现这一系列症状时应考虑PRIS,但必须始终排除其他神经代谢疾病。

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