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01-02-2017 | Research Article

Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia

Authors: Ryan C. Dillon, Jose-Alberto Palma, Christy L. Spalink, Diana Altshuler, Lucy Norcliffe-Kaufmann, David Fridman, John Papadopoulos, Horacio Kaufmann

Published in: Clinical Autonomic Research | Issue 1/2017

Abstract

Objective

Adrenergic 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 α ₂-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.

Methods

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 h 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 h after dexmedetomidine cessation.

Results

Nine patients over 14 admissions were included in the final analysis. At 1 h 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 [interquartile range (IQR) 3–11 days] and median ICU length of stay was 7 days (IQR 3–11 days).

Conclusions

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.

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Title: Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia
Authors: Ryan C. Dillon
Jose-Alberto Palma
Christy L. Spalink
Diana Altshuler
Lucy Norcliffe-Kaufmann
David Fridman
John Papadopoulos
Horacio Kaufmann
Publication date: 01-02-2017
Publisher: Springer Berlin Heidelberg
Published in: Clinical Autonomic Research / Issue 1/2017
Print ISSN: 0959-9851
Electronic ISSN: 1619-1560
DOI: https://doi.org/10.1007/s10286-016-0383-5

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia

Abstract

Objective

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

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