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DeCS
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Descriptor English:
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Hereditary Sensory and Autonomic Neuropathies
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Descriptor Spanish:
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Neuropatías Hereditarias Sensoriales y Autónomas
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Descriptor Portuguese:
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Neuropatias Hereditárias Sensoriais e Autônomas
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Synonyms English:
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Neuropathies, Hereditary Sensory and Autonomic
HSAN
HSAN Type I
HSAN Type II
HSAN Type IV
HSAN Type V
HSN Type I
HSN Type II
Sensory and Autonomic Neuropathies, Hereditary
Sensory Neuropathy, Hereditary
Insensitivity to Pain with Anhidrosis, Congenital
Pain Insensitivity with Anhidrosis, Congenital
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Tree Number:
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C10.114.750.137
C10.314.750.600
C10.500.310
C10.574.500.496
C10.668.829.800.625
C10.668.829.800.750.450
C16.131.666.310
C16.320.400.415
C20.111.258.750.600
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Definition English:
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A group of inherited disorders characterized by degeneration of dorsal root and autonomic ganglion cells, and clinically by loss of sensation and autonomic dysfunction. There are five subtypes. Type I features autosomal dominant inheritance and distal sensory involvement. Type II is characterized by autosomal inheritance and distal and proximal sensory loss. Type III is DYSAUTONOMIA, FAMILIAL. Type IV features insensitivity to pain, heat intolerance, and mental deficiency. Type V is characterized by a selective loss of pain with intact light touch and vibratory sensation. (From Joynt, Clinical Neurology, 1995, Ch51, pp142-4) |
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Indexing Annotation English:
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do not confuse with HEREDITARY MOTOR AND SENSORY NEUROPATHIES; for X refs to HSAN types: HSAN TYPE III is see DYSAUTONOMIA, FAMILIAL
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See Related English:
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Pain Insensitivity, Congenital
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History Note English:
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2000(1989); use NEUROPATHY, HEREDITARY SENSORY 1979-1988
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Allowable Qualifiers English:
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Record Number:
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23938
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Unique Identifier:
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D009477
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Occurrence in VHL:
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Similar:
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DeCS SciELO LILACS LIS
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