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杜氏肌营养不良症患者的脂肪栓塞综合征:早期识别与积极治疗

Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy.

作者信息

Murphy Lee D, Yabrodi Mouhammad, Lutfi Riad

机构信息

Division of Pediatric Critical Care Medicine, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.

Division of Pediatric Cardiology, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.

出版信息

Case Rep Crit Care. 2018 Jun 4;2018:3686470. doi: 10.1155/2018/3686470. eCollection 2018.

Abstract

We describe two pediatric patients with Duchenne muscular dystrophy that presented with acute neurologic deterioration and hypoxic respiratory failure requiring mechanical ventilation. These cases fulfill the clinical criteria for Fat Embolism Syndrome. Early recognition and aggressive supportive therapy with mechanical ventilation, right ventricular afterload reduction, and blood transfusion led to survival without any residual effects from the event. Fat Embolism Syndrome needs to be considered early in the course of patients with Duchenne muscular dystrophy who present with respiratory and neurological symptoms.

摘要

我们描述了两名患有杜氏肌营养不良症的儿科患者,他们出现了急性神经功能恶化和低氧性呼吸衰竭,需要机械通气。这些病例符合脂肪栓塞综合征的临床标准。早期识别并采用机械通气、降低右心室后负荷和输血等积极的支持性治疗,使患者得以存活,且此次事件未留下任何后遗症。对于出现呼吸和神经症状的杜氏肌营养不良症患者,在病程早期就需要考虑脂肪栓塞综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b45/6008769/1cf67acc3d54/CRICC2018-3686470.001.jpg

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