scholarly journals Case report: Perioperative management of a patient with familial dysautonomia

2021 ◽  
Vol 6 (2) ◽  
pp. 029-032
Author(s):  
Sarebanha Melodie ◽  
Valente Laura ◽  
Kalra Minnea ◽  
Joseph Layon A ◽  
Crimi Ettore
Keyword(s):  
Intensive Care Unit ◽  
Nervous System ◽  
Autonomic Nervous System ◽  
Crisis Management ◽  
Autosomal Recessive ◽  
Perioperative Management ◽  
Hemodynamic Instability ◽  
Familial Dysautonomia ◽  
Autonomic Nervous ◽  
Noxious Stimuli

Familial dysautonomia is a rare autosomal recessive neurodegenerative disease affecting cells of the autonomic nervous system. Patients with this disease are insensitive to pain but their autonomic nervous system is still activated with noxious stimuli. This report details a case of a patient with familial dysautonomia who underwent right ankle open reduction and internal fixation for a bimalleolar right ankle fracture. The patients preoperative and intraoperative course were uneventful but shortly after handoff to the intensive care unit, the patient experienced an autonomic crisis. Management of these patients is complex, requiring maintenance of physiologic homeostasis as well as preventing hemodynamic instability caused by noxious stimuli. Any deviations from baseline may cause an autonomic crisis, as happened in our patient. Herein, we detail the perioperative management of a patient with familial dysautonomia in further detail.

Practising through the pandemic

2021 ◽  
Vol 15 (1) ◽  
pp. 119-120
Author(s):  
Gülcan Sutton Purser
Keyword(s):  
Mental Health ◽  
Nervous System ◽  
Autonomic Nervous System ◽  
Crisis Management ◽  
After Effects ◽  
Autonomic Nervous

Practising psychotherapy online during Covid-19; uncertainties and crisis management; after effects of lockdown; trauma and its effects on autonomic nervous system; how we regulate. This article consists of my work during the global Covid-19 crisis and to address the consequences of it on mental health.

scholarly journals Development of the Autonomic Nervous System: Clinical Implications

2020 ◽  
Vol 40 (05) ◽  
pp. 473-484
Author(s):  
Frances Lefcort
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Developmental Disorders ◽  
Molecular Mechanisms ◽  
Hirschsprung Disease ◽  
Familial Dysautonomia ◽  
Clinical Implications ◽  
Autonomic Nervous ◽  
Central Hypoventilation ◽  
Hypoventilation Syndrome

AbstractInvestigations of the cellular and molecular mechanisms that mediate the development of the autonomic nervous system have identified critical genes and signaling pathways that, when disrupted, cause disorders of the autonomic nervous system. This review summarizes our current understanding of how the autonomic nervous system emerges from the organized spatial and temporal patterning of precursor cell migration, proliferation, communication, and differentiation, and discusses potential clinical implications for developmental disorders of the autonomic nervous system, including familial dysautonomia, Hirschsprung disease, Rett syndrome, and congenital central hypoventilation syndrome.

Respiratory and cardiovascular indicators of autonomic nervous system dysregulation in familial dysautonomia

2011 ◽  
Vol 47 (7) ◽  
pp. 682-691 ◽  
Author(s):  
Michael S. Carroll ◽  
Anna S. Kenny ◽  
Pallavi P. Patwari ◽  
Jan-Marino Ramirez ◽  
Debra E. Weese-Mayer
Keyword(s):  
Nervous System ◽  
Autonomic Nervous System ◽  
Familial Dysautonomia ◽  
Autonomic Nervous

scholarly journals The Cardiac R-R Variation and Sympathetic Skin Response in the Intensive Care Unit

2007 ◽  
Vol 34 (3) ◽  
pp. 313-315 ◽  
Author(s):  
Charles Bolton ◽  
Jillian Thompson ◽  
Linda Bernardi ◽  
Christopher Voll ◽  
Bryan Young
Keyword(s):  
Intensive Care Unit ◽  
Nervous System ◽  
Intensive Care ◽  
Autonomic Nervous System ◽  
Neurological Disorders ◽  
Sympathetic Skin Response ◽  
Assisted Ventilation ◽  
Icu Patients ◽  
Autonomic Nervous ◽  
Skin Response

Background and Purpose:The central and peripheral nervous systems are often affected in intensive care unit (ICU) patients, especially those with prolonged assisted ventilation and sepsis or systemic inflammatory response syndrome (SIRS). The autonomic nervous system, however, has been under-investigated in such patients. We evaluated autonomic nervous system (ANS) function in 29 ICU patients with various neurological disorders.Methods:Testing involved cardiac R-R variation (CRRV) as an index of parasympathetic function and the sympathetic skin response (SSR) for sympathetic assessment.Results:Only those 8 patients with sepsis-related neuropathy or encephalopathy had abnormal CRRV, while the SSR was absent in all but 2 patients.Conclusions:Our preliminary study revealed a high incidence of autonomic dysfunction in ICU patients with various neurological disorders.

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