Perioperative use of dexmedetomidine in an infant with familial dysautonomia

Abulhasan, Yasser B.; Buu, Natalie; Frigon, Chantal

doi:10.1093/bja/aep178

Cited by 7 publications

(6 citation statements)

References 25 publications

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“…This selective agonist acts on α2 adrenoreceptors in the brain and spinal cord to inhibit sympathetic outflow, resulting in hypotension, bradycardia, sedation, analgesia, reduced salivation, and decreased bowel motility. 16 …”

Section: Cardiovascular Treatmentsmentioning

confidence: 99%

Current treatments in familial dysautonomia

Palma

Kaufmann

Fuente-Mora

et al. 2014

Expert Opinion on Pharmacotherapy

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Introduction Familial dysautonomia (FD) is a rare hereditary sensory and autonomic neuropathy (type III). The disease is caused by a point mutation in the IKBKAP gene that affects the splicing of the elongator-1 protein (also known as IKAP). Patients have dramatic blood pressure instability due to baroreflex failure, chronic kidney disease, and impaired swallowing leading to recurrent aspiration pneumonia, which results in chronic lung disease. Diminished pain and temperature perception results in neuropathic joints and thermal injuries. Impaired proprioception leads to gait ataxia. Optic neuropathy and corneal opacities lead to progressive visual loss. Areas covered This article reviews current therapeutic strategies for the symptomatic treatment of FD, as well as the potential of new gene modifying agents. Expert opinion Therapeutic focus on FD is centered on reducing the catecholamine surges caused by baroreflex failure. Managing neurogenic dysphagia with effective protection of the airway passages and prompt treatment of aspiration pneumonias is necessary to prevent respiratory failure. Sedative medications should be used cautiously due to risk of respiratory depression. Non-invasive ventilation during sleep effectively manages apneas and prevents hypercapnia. Clinical trials of compounds that increase levels of IKAP (ELP-1) are underway and will determine whether they can reverse or slow disease progression.

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Section: Cardiovascular Treatmentsmentioning

confidence: 99%

Current treatments in familial dysautonomia

Palma

Kaufmann

Fuente-Mora

et al. 2014

Expert Opinion on Pharmacotherapy

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“…Until our research, evidence for the use of dexmedetomidine in FD was limited to anecdotal cases for intraoperative administration and concurrently used with sedatives, muscle relaxants, and analgesic medications [1, 10, 13]. DiGiusto et al reported a 28-year-old woman with FD who, after a jejunostomy tube replacement, developed severe agitation, hypertension, tachycardia, and diaphoresis consistent with adrenergic crisis [3].…”

Section: Discussionmentioning

confidence: 99%

“…These are often referred to as “dysautonomic” or “adrenergic crises”. Adrenergic crises are a consequence of the inability to restrain sympathetic overflow caused by congenital damage to the afferent nerves carrying baroreceptor inputs to the central nervous system [1, 3, 7, 14, 23, 24]. Adrenergic crises can be triggered by a variety of situations ranging from surgery and severe illness to daily emotions and feeding [6, 21].…”

Section: Introductionmentioning

confidence: 99%

“…Dexmedetomidine, a centrally acting α 2 adrenergic agonist, has been anecdotally used in the perioperative management of FD patients [1, 3, 10, 13]. Intravenous administration combined with a short half-life (i.e., approximately 2 hours) allows an easy titration and makes it an attractive treatment for refractory adrenergic crises in FD [30].…”

Section: Introductionmentioning

confidence: 99%

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Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia

et al. 2016

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Objective Adrenergic crises are a cardinal feature of familial dysautonomia (FD). Traditionally, adrenergic crisis have been treated with the sympatholytic agent clonidine or with benzodiazepines, which can cause excessive sedation and respiratory depression. Dexmedetomidine is an α2A-adrenergic agonist with greater selectivity and shorter half-life than clonidine. We aimed to evaluate 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-hour 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-hours after dexmedetomidine cessation. Results Nine patients over 14 admissions were included in the final analysis. At 1-hour 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 (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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“…18 Olgularda entübasyon evresinde, ekstübasyon evresinde ve postoperatif dönemde istenmeyen bir hemodinamik dalgalanma yaşa-mamışlardır.…”

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