Dysautonomia, Familial [familiární dysautonomie]

topical
7
Terms

dysautonomie familiární
hereditární senzitivní a autonomní neuropatie typ III
Rileyův-Dayův syndrom

 

Dominant Hereditary Sensory Neuropathy, Type III
Familial Dysautonomia
Hereditary Sensory and Autonomic Neuropathy 3
Hereditary Sensory Neuropathy Type 3
Hereditary Sensory Neuropathy, Dominant, Type 3
Hereditary Sensory Neuropathy, Dominant, Type III
Hereditary Sensory Neuropathy, Type 3, Dominant
Hereditary-Sensory and Autonomic Neuropathy Type III
HSAN (Hereditary Sensory and Autonomic Neuropathy) Type III
HSAN 3
HSAN III
HSAN Type III
HSAN3
HSN-III
Neuropathy, Hereditary and Autonomic, Type III
Neuropathy, Hereditary Sensory And Autonomic, Type III
Riley-Day Syndrome
Type 3 Hereditary Sensory Neuropathy, Dominant
Type III Hereditary Sensory Neuropathy, Dominant

Persistent link   https://www.medvik.cz/link/D004402
Definition

An autosomal disorder of the peripheral and autonomic nervous systems limited to individuals of Ashkenazic Jewish descent. Clinical manifestations are present at birth and include diminished lacrimation, defective thermoregulation, orthostatic hypotension (HYPOTENSION, ORTHOSTATIC), fixed pupils, excessive SWEATING, loss of pain and temperature sensation, and absent reflexes. Pathologic features include reduced numbers of small diameter peripheral nerve fibers and autonomic ganglion neurons. (From Adams et al., Principles of Neurology, 6th ed, p1348; Nat Genet 1993;4(2):160-4)

Annotation
PRIMARY DYSAUTONOMIAS is also available
DUI
D004402 MeSH Browser
CUI
M0006912
History note
1981; uss AUTONOMIC DYSFUNCTION 1963-1980
Public note
1981; see AUTONOMIC DYSFUNCTION 1963-1980