Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia.

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 cha

SUBMITTER: Dillon RC 

PROVIDER: S-EPMC5292083 | biostudies-literature | 2017 Feb

REPOSITORIES: biostudies-literature