家族性自主神经异常症的肾上腺素危象经鼻给予右美托咪定。
Intranasal dexmedetomidine for adrenergic crisis in familial dysautonomia.
机构信息
Department of Neurology, Dysautonomia Center, New York University School of Medicine, New York, NY, USA.
出版信息
Clin Auton Res. 2017 Aug;27(4):279-282. doi: 10.1007/s10286-017-0442-6. Epub 2017 Jul 3.
PURPOSE
To report the use of intranasal dexmedetomidine, an α-adrenergic agonist for the acute treatment of refractory adrenergic crisis in patients with familial dysautonomia.
METHODS
Case series.
RESULTS
Three patients with genetically confirmed familial dysautonomia (case 1: 20-year-old male; case 2: 43-year-old male; case 3: 26-year-old female) received intranasal dexmedetomidine 2 mcg/kg, half of the dose in each nostril, for the acute treatment of adrenergic crisis. Within 8-17 min of administering the intranasal dose, the adrenergic crisis symptoms abated, and blood pressure and heart rate returned to pre-crises values. Adrenergic crises eventually resumed, and all three patients required hospitalization for investigation of the cause of the crises.
CONCLUSIONS
Intranasal dexmedetomidine is a feasible and safe acute treatment for adrenergic crisis in patients with familial dysautonomia. Further controlled studies are required to confirm the safety and efficacy in this population.
目的
报告使用鼻腔内给予右美托咪定（一种 α-肾上腺素能激动剂）治疗家族性自主神经功能异常患者难治性肾上腺素能危象的情况。
方法
病例系列研究。
结果
3 名经基因确诊的家族性自主神经功能异常患者（病例 1：20 岁男性；病例 2：43 岁男性；病例 3：26 岁女性）接受了鼻腔内给予右美托咪定 2 mcg/kg，每侧鼻腔给予半剂量，用于治疗急性肾上腺素能危象。在给予鼻腔内剂量后 8-17 分钟内，肾上腺素能危象症状缓解，血压和心率恢复到危象前的水平。肾上腺素能危象最终再次出现，所有 3 名患者均需要住院以调查危象的原因。
结论
鼻腔内给予右美托咪定是治疗家族性自主神经功能异常患者肾上腺素能危象的一种可行且安全的急性治疗方法。需要进一步的对照研究来证实该方法在该人群中的安全性和疗效。
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