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Immunodeficiency 57

Disease ID: disease_node_20446

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DbxrefMIM:618108, ORDO:529977
SubclassofDOID_0050737, DOID_612
Data SourceDOID
SynonymsIMD57, immune dysregulation-inflammatory bowel disease-arthritis-recurrent infections-lymphopenia syndrome, immunodeficiency 57 with autoinflammation
Doid Labelimmunodeficiency 57
Doid DescriptionA primary immunodeficiency disease characterized by recurrent infections starting in the first year of life, lymphopenia, altered production of various cytokines, inflammatory polyarthritis, and chronic active inflammation of the digestive tract that has_material_basis_in homozygous or compound heterozygous mutation in the RIPK1 gene on chromosome 6p25.2.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20446
Doid IdDOID_0111952
LabelImmunodeficiency 57