Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: An Off-Label Use.
| Autor: | Subedi A; Internal Medicine, Avera McKennan Hospital & University Health Center, Sioux Falls, USA., Sharma R; Internal Medicine, Avera McKennan Hospital & University Health Center, Sioux Falls, USA., Lalani I; Pulmonary and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, USA. |
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| Jazyk: | angličtina |
| Zdroj: | Cureus [Cureus] 2022 Oct 06; Vol. 14 (10), pp. e29988. Date of Electronic Publication: 2022 Oct 06 (Print Publication: 2022). |
| DOI: | 10.7759/cureus.29988 |
| Abstrakt: | Familial dysautonomia is a rare genetic neurodevelopmental disorder characterized by episodes of hyperautonomic state known as dysautonomic crises. The features of dysautonomic crises are hypertension, tachycardia, vomiting, sweating, flushing, and behavioral changes. The etiology of such crises is supposed to be a consequence of the inability to control sympathetic overflow due to damage to the afferent neurons carrying baroreceptor inputs to the central nervous system. A 19-year-old male with a known history of familial dysautonomia and frequent dysautonomic crises presented to the Emergency Department with intractable nausea and vomiting for six hours. He was hypertensive and tachycardic on presentation. The patient had tried oral labetalol and clonidine at home with no improvement. In the emergency room, the patient received intravenous labetalol, diazepam, and clonidine which were ineffective. He was then treated with intravenous dexmedetomidine, and his symptoms resolved within a few hours. The patient was discharged home on the same day. The mainstay of treatment for dysautonomic crises is benzodiazepines and clonidine. The use of these treatment modalities has its challenges. Here, we present a case of a dysautonomic crisis that was resistant to the conventional treatment, treated safely and successfully with dexmedetomidine. Competing Interests: The authors have declared that no competing interests exist. (Copyright © 2022, Subedi et al.) |
| Databáze: | MEDLINE |
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