Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia

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• 作者：Ryan C. Dillon ; Jose-Alberto Palma ; Christy L. Spalink…
• 关键词：Familial dysautonomia ; Dexmedetomidine ; Alpha ; 2 agonist ; Imidazoline receptors ; Hereditary sensory and autonomic neuropathies ; Riley–Day syndrome
• 刊名：Clinical Autonomic Research
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：27
• 期：1
• 页码：7-15
• 全文大小：
• 刊物类别：Medicine
• 刊物主题：Neurology; Cardiology; Endocrinology; Diabetes; Gastroenterology; Ophthalmology;
• 出版者：Springer Berlin Heidelberg
• ISSN：1619-1560
• 卷排序：27

文摘

ObjectiveAdrenergic crises are a cardinal feature of familial dysautonomia (FD). Traditionally, adrenergic crises have been treated with the sympatholytic agent clonidine or with benzodiazepines, which can cause excessive sedation and respiratory depression. Dexmedetomidine is a centrally-acting α₂-adrenergic agonist with greater selectivity and shorter half-life than clonidine. We evaluated the preliminary effectiveness and safety of intravenous dexmedetomidine in the treatment of refractory adrenergic crisis in patients with FD.