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DeCS
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Descriptor English:
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Cryopyrin-Associated Periodic Syndromes
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Descriptor Spanish:
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Síndromes Periódicos Asociados a Criopirina
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Descriptor Portuguese:
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Síndromes Periódicas Associadas ŕ Criopirina
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Synonyms English:
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CINCA Syndrome
Chronic Infantile Neurological, Cutaneous, and Articular Syndrome
Cryopyrinopathy
Familial Cold Autoinflammatory Syndrome
Familial Cold Urticaria
IOMID
Infantile Onset Multisystem Inflammatory Disease
Muckle-Wells Syndrome
Multisystem Inflammatory Disease, Neonatal-Onset
NOMID
Neonatal Onset Multisystem Inflammatory Disease
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Tree Number:
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C16.320.382.500
C17.800.827.368.500
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Definition English:
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A group of rare autosomal dominant diseases, commonly characterized by atypical URTICARIA (hives) with systemic symptoms that develop into end-organ damage. The atypical hives do not involve T-cell or autoantibody. Cryopyrin-associated periodic syndrome includes three previously distinct disorders: Familial cold autoinflammatory syndrome; Muckle-Wells Syndrome; and CINCA Syndrome, that are now considered to represent a disease continuum, all caused by NLRP3 protein mutations. |
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History Note English:
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2010; use Cryopyrin-associated Periodic Syndromes, 2010
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Allowable Qualifiers English:
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Record Number:
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53556
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Unique Identifier:
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D056587
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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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