Definition of "Familial dysautonomia"

Last modified: 6 days



Familial dysautonomia (aka Riley-Day syndromehereditary sensory and autonomic neuropathy type III) is a disorder of which the autonomic nervous system which affects the development and survival of sensory, sympathetic and some parasympathetic neurons in the autonomic and sensory nervous system.

Sx
  • Variable Sx including:
    • Insensitivity to pain
    • Inability to produce tears
    • Poor growth
    • Labile BP (episodic hypertension and postural hypotension)
  • Frequent vomiting crises
  • Pneumonia problems with speech and movment
  • Difficulty swallowing
  • Inappropriate perception of heat, pain, and taste
  • Unstable BP
  • GI dysmotility
Epidemiology
  • It doesn't affect intelligence
See also
  • Hereditary sensory and autonomic neuropathies (HSAN), its category, all of which involves widespreaed sensory dysfunction and variable autonomic dysfunction caused by incomplete development of sensory and autonomic neurons. The disorders are believed to be genetically distinct from each other

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Definition of Familial dysautonomia | Autoprac


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