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Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: An Off-Label Use.
Cureus. 2022 Oct; 14(10):e29988.C

Abstract

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.

Authors+Show Affiliations

Internal Medicine, Avera McKennan Hospital & University Health Center, Sioux Falls, USA.Internal Medicine, Avera McKennan Hospital & University Health Center, Sioux Falls, USA.Pulmonary and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, USA.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

36381719

Citation

Subedi, Ananta, et al. "Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: an Off-Label Use." Cureus, vol. 14, no. 10, 2022, pp. e29988.
Subedi A, Sharma R, Lalani I. Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: An Off-Label Use. Cureus. 2022;14(10):e29988.
Subedi, A., Sharma, R., & Lalani, I. (2022). Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: An Off-Label Use. Cureus, 14(10), e29988. https://doi.org/10.7759/cureus.29988
Subedi A, Sharma R, Lalani I. Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: an Off-Label Use. Cureus. 2022;14(10):e29988. PubMed PMID: 36381719.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Experience With Dexmedetomidine Use in the Treatment of Dysautonomic Crisis in Familial Dysautonomia: An Off-Label Use. AU - Subedi,Ananta, AU - Sharma,Rakshya, AU - Lalani,Ishan, Y1 - 2022/10/06/ PY - 2022/10/05/accepted PY - 2022/11/16/entrez PY - 2022/11/17/pubmed PY - 2022/11/17/medline KW - autonomic crisis KW - dexmedetomidine KW - dysautonomic crisis KW - familial dysautonomia KW - off-label drug use SP - e29988 EP - e29988 JF - Cureus JO - Cureus VL - 14 IS - 10 N2 - 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. SN - 2168-8184 UR - https://www.unboundmedicine.com/medline/citation/36381719/Experience_With_Dexmedetomidine_Use_in_the_Treatment_of_Dysautonomic_Crisis_in_Familial_Dysautonomia:_An_Off-Label_Use. DB - PRIME DP - Unbound Medicine ER -
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