Concept information
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Neuromuscular Diseases
Peripheral Nervous System Diseases
Polyneuropathies
Hereditary Sensory and Autonomic Neuropathies
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Genetic Diseases, Inborn
Heredodegenerative Disorders, Nervous System
Hereditary Sensory and Autonomic Neuropathies
Nervous System Diseases
Neurodegenerative Diseases
Heredodegenerative Disorders, Nervous System
Hereditary Sensory and Autonomic Neuropathies
Preferred term
Dysautonomia, Familial
Type
-
Topical Descriptor
Broader concept
Entry terms
- Dominant Hereditary Sensory Neuropathy, Type III
- Familial Dysautonomia
- Hereditary Sensory and Autonomic Neuropathy 3
- Hereditary Sensory and Autonomic Neuropathy Type III
- Hereditary-Sensory and Autonomic Neuropathy Type III
- Hereditary Sensory Neuropathy, Dominant, Type 3
- Hereditary Sensory Neuropathy, Dominant, Type III
- Hereditary Sensory Neuropathy Type 3
- Hereditary Sensory Neuropathy, Type 3, Dominant
- HSAN 3
- HSAN3
- HSAN (Hereditary Sensory and Autonomic Neuropathy) Type III
- HSAN III
- HSAN Type III
- HSN-III
- Neuropathy, Hereditary and Autonomic, Type III
- Neuropathy, Hereditary Sensory And Autonomic, Type III
- Riley Day Syndrome
- Riley-Day Syndrome
- Type 3 Hereditary Sensory Neuropathy, Dominant
- Type III Hereditary Sensory Neuropathy, Dominant
Note
- PRIMARY DYSAUTONOMIAS is also available
Scope note
- 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)
History note
- 1981; uss AUTONOMIC DYSFUNCTION 1963-1980
In other languages
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Finnish
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dysautonomia familialis
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familiaalinen dysautonomia
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familiaarinen dysautonomia
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familjär dysautonomi (Riley-Day)
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Riley-Day
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Riley-Dayin syndrooma
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Riley-Dayin tauti
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Riley-Dayn tauti
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suvuittainen dysautonomia
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Swedish
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Dysautonomi, familjär
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Riley-Days syndrom
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Ärftlig dysautonomi
URI
http://www.yso.fi/onto/mesh/D004402
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