Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia.
Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia.
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DOI：
10.1007/s10286-016-0383-5
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发表时间：
2017-02
期刊：
影响因子：
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通讯作者：
Kaufmann H
中科院分区：
文献类型：
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作者：
Dillon RC;Palma JA;Spalink CL;Altshuler D;Norcliffe-Kaufmann L;Fridman D;Papadopoulos J;Kaufmann H
肾上腺素能危机是家族性自主神经功能障碍（FD）的一个主要特征。传统上，肾上腺素能危象是用交感神经阻滞剂可乐定或苯二氮卓类药物治疗的，这可能会导致过度镇静和呼吸抑制。右美托咪定是一种 α2A 肾上腺素能激动剂，与可乐定相比具有更高的选择性和更短的半衰期。我们的目的是评估静脉注射右美托咪定治疗 FD 患者难治性肾上腺素能危象的初步有效性和安全性。对基因确诊的 FD 患者接受静脉右美托咪定治疗难治性肾上腺素能危象的回顾性图表审查。主要结局是右美托咪定的初步有效性，定义为开始使用右美托咪定后 1 小时血压 (BP) 和心率 (HR) 的变化。次要结局包括与右美托咪定相关的不良事件发生率、医院和重症监护病房 (ICU) 住院时间以及右美托咪定停用后 12 小时的血流动力学参数。入院14次以上的9名患者纳入最终分析。开始使用右美托咪定后 1 小时，收缩压从 160±7 降至 122±7 mmHg (p=0.0005)，舒张压从 103±6 降至 65±8 (p=0.0003)，心率从 112±4 降至 100±5 bpm (p=0.0047)。每次入院右美托咪定输注期间的中位总不良事件为 1 起。中位住院时间为 9 天（IQR，3 – 11 天），中位 ICU 住院时间为 7 天（IQR，3 – 11 天）。静脉注射右美托咪定对于 FD 患者是安全的，并且似乎可以有效治疗难治性肾上腺素能危象。对传统可乐定和苯二氮卓类药物治疗无反应的 FD 患者可考虑右美托咪定。
Adrenergic crises are a cardinal feature of familial dysautonomia (FD). Traditionally, adrenergic crisis have been treated with the sympatholytic agent clonidine or with benzodiazepines, which can cause excessive sedation and respiratory depression. Dexmedetomidine is an α2A-adrenergic agonist with greater selectivity and shorter half-life than clonidine. We aimed to evaluate the preliminary effectiveness and safety of intravenous dexmedetomidine in the treatment of refractory adrenergic crisis in patients with FD. Retrospective chart review of patients with genetically confirmed FD who received intravenous dexmedetomidine for refractory adrenergic crises. The primary outcome was preliminary effectiveness of dexmedetomidine defined as change in blood pressure (BP) and heart rate (HR) 1-hour after the initiation of dexmedetomidine. Secondary outcomes included incidence of adverse events related to dexmedetomidine, hospital and intensive care unit (ICU) length of stay, and hemodynamic parameters 12-hours after dexmedetomidine cessation. Nine patients over 14 admissions were included in the final analysis. At 1-hour after the initiation of dexmedetomidine, systolic BP decreased from 160±7 to 122±7 mmHg (p=0.0005), diastolic BP decreased from 103±6 to 65±8 (p=0.0003), and HR decreased from 112±4 to 100±5 bpm (p=0.0047). The median total adverse events during dexmedetomidine infusion was 1 per admission. Median hospital length of stay was 9 days (IQR, 3 – 11 days) and median ICU length of stay was 7 days (IQR, 3 – 11 days). Intravenous dexmedetomidine is safe in patients with FD and appears to be effective to treat refractory adrenergic crisis. Dexmedetomidine may be considered in FD patients who do not respond to conventional clonidine and benzodiazepine pharmacotherapy.