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DeCS
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Descriptor English:
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Neurofibromatosis 2
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Descriptor Spanish:
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Neurofibromatosis 2
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Descriptor Portuguese:
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Neurofibromatose 2
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Synonyms English:
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Neurofibromatosis, Acoustic, Bilateral
Neurofibromatosis, Central, NF2
Neuroma, Acoustic, Bilateral
Schwannoma, Acoustic, Bilateral
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Tree Number:
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C04.557.465.625.650.595.610.500
C04.557.580.600.580.590.655
C04.557.580.600.610.595.610.500
C04.700.645.655
C09.218.807.800.675.500
C09.647.675.500
C10.292.225.750.500
C10.292.910.600.500
C10.562.600.750
C10.574.500.549.700
C16.320.400.560.700
C16.320.700.645.655
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Definition English:
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An autosomal dominant disorder characterized by a high incidence of bilateral acoustic neuromas as well as schwannomas (NEURILEMMOMA) of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal neurofibromas, and gliomas. The disease has been linked to mutations of the NF2 gene (GENES, NEUROFIBROMATOSIS 2) on chromosome 22 (22q12) and usually presents clinically in the first or second decade of life. |
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Indexing Annotation English:
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do not confuse with NEUROFIBROMATOSIS 1; coord IM with precoord/neopl term (IM) if relevant
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See Related English:
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Genes, Neurofibromatosis 2
Neurofibromin 2
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History Note English:
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1992; NEUROFIBROMATOSIS, CENTRAL, NF2 use NEUROFIBROMATOSIS, CENTRAL, NF-2 use NEUROMA, ACOUSTIC 1989-1991; NEUROMA, ACOUSTIC, BILATERAL use NEUROMA, ACOUSTIC 1989-91
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Allowable Qualifiers English:
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Record Number:
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29932
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Unique Identifier:
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D016518
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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