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DeCS
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Descriptor English:
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Spinal Muscular Atrophies of Childhood
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Descriptor Spanish:
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Atrofias Musculares Espinales de la Infancia
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Descriptor Portuguese:
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Atrofias Musculares Espinais da Infância
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Synonyms English:
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Infantile Spinal Muscular Atrophy
Muscular Atrophy, Spinal, Infantile
Spinal Muscular Atrophy, Infantile
HMN Proximal Type I
Werdnig-Hoffmann Disease
Juvenile Spinal Muscular Atrophy
Kugelberg-Welander Disease
Spinal Muscular Atrophy, Juvenile
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Tree Number:
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C10.228.854.468.800
C10.574.500.812
C10.574.562.500.750
C10.668.460.500.750
C10.668.475.500
C16.320.400.765
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Definition English:
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A group of recessively inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3) |
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Indexing Annotation English:
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infantile, childhood or adolescent: for other, MUSCULAR ATROPHY, SPINAL is available
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History Note English:
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2000(1988)
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Allowable Qualifiers English:
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Record Number:
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24374
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Unique Identifier:
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D014897
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Occurrence in VHL:
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Similar:
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DeCS SciELO LILACS LIS
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