title: Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia.
abstract: 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 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.
  
  
  
  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.
  
  
  
  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).
  
  
  
  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.
authors.affiliation: Department of Pharmacy, NYU Langone Medical Center, New York, NY, USA.
authors.forename: Ryan C
authors.initials: RC
authors.lastname: Dillon
authors.affiliation: Department of Neurology, Dysautonomia Center, New York University School of Medicine, 530 First Avenue Suite 9Q, New York, NY, 10016, USA.
authors.forename: Jose-Alberto
authors.initials: JA
authors.lastname: Palma
authors.affiliation: Department of Neurology, Dysautonomia Center, New York University School of Medicine, 530 First Avenue Suite 9Q, New York, NY, 10016, USA.
authors.forename: Christy L
authors.initials: CL
authors.lastname: Spalink
authors.affiliation: Department of Pharmacy, NYU Langone Medical Center, New York, NY, USA.
authors.forename: Diana
authors.initials: D
authors.lastname: Altshuler
authors.affiliation: Department of Neurology, Dysautonomia Center, New York University School of Medicine, 530 First Avenue Suite 9Q, New York, NY, 10016, USA.
authors.forename: Lucy
authors.initials: L
authors.lastname: Norcliffe-Kaufmann
authors.affiliation: Department of Medicine, NYU Langone Medical Center, New York, NY, USA.
authors.forename: David
authors.initials: D
authors.lastname: Fridman
authors.affiliation: Department of Pharmacy, NYU Langone Medical Center, New York, NY, USA.|Department of Medicine, NYU Langone Medical Center, New York, NY, USA.
authors.forename: John
authors.initials: J
authors.lastname: Papadopoulos
authors.affiliation: Department of Neurology, Dysautonomia Center, New York University School of Medicine, 530 First Avenue Suite 9Q, New York, NY, 10016, USA. Horacio.Kaufmann@nyumc.org.
authors.forename: Horacio
authors.identifier: 0000-0002-1345-6774
authors.initials: H
authors.lastname: Kaufmann
mesh_terms: D061605:Administration, Intravenous; D000293:Adolescent; D058647:Adrenergic alpha-2 Receptor Agonists / Q000008:administration & dosage*; D000328:Adult; D001794:Blood Pressure / Q000187:drug effects* / Q000502:physiology; D020927:Dexmedetomidine / Q000008:administration & dosage*; D004402:Dysautonomia, Familial / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology; D005260:Female; D006339:Heart Rate / Q000187:drug effects* / Q000502:physiology; D006801:Humans; D006973:Hypertension / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D007902:Length of Stay / Q000639:trends; D008297:Male; D012189:Retrospective Studies; D013610:Tachycardia / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D055815:Young Adult
chemical_list: D058647:Adrenergic alpha-2 Receptor Agonists; D020927:Dexmedetomidine
keywords: Alpha-2 agonist; Dexmedetomidine; Familial dysautonomia; Hereditary sensory and autonomic neuropathies; Imidazoline receptors; Riley–Day syndrome