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Hyper-Igm Immunodeficiency Syndrome, Type 1

Disease ID: disease_node_11682

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DbxrefGARD:73, MESH:D053307, MIM:308230, NCI:C158531, ORDO:101088, SNOMEDCT_US_2023_03_01:403835002, UMLS_CUI:C0398689
SubclassofDOID_0080012, DOID_0080544
Data SourceDOID, MESH
SynonymsHIGM1, X-linked hyper-IgM immunodeficiency, XHIM, hyper-IgM immunodeficiency syndrome type 1, hyper-IgM syndrome 1, hyper-IgM syndrome type 1, immunodeficiency with hyper-IgM type 1
Mesh IdD053307
Mesh LabelHyper-IgM Immunodeficiency Syndrome, Type 1
Mesh SubclassofD040181, D053306
Doid LabelX-linked hyper IgM syndrome
Doid DescriptionA hyper IgM syndrome that is characterized by neutropenia and a high rate of gastrointestinal and central nervous system infections and that has_material_basis_in a mutation in the CD40LG gene on chromosome Xq26.3.
Has Material Basis InGENO_0000149
Disease Node Iddisease_node_11682
Doid IdDOID_6620
LabelHyper-Igm Immunodeficiency Syndrome, Type 1