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Immunodeficiency 49

Disease ID: disease_node_20001

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DbxrefMIM:617237, UMLS_CUI:C4310656
SubclassofDOID_0050736, DOID_11200
Data SourceDOID
SynonymsIMD49, SCID, T-cell negative, B-cell positive, NK cell positive, with intellectual disability, spasticity, and craniofacial abnormalities, severe combined immunodeficiency, T-cell negative, B-cell positive, NK cell positive, with intellectual disability, spasticity, and craniofacial abnormalities
Doid Labelimmunodeficiency 49
Doid DescriptionA T cell deficiency characterized by T cell lymphopenia, low T-cell receptor excision circles, impaired T-cell proliferative responses, dysmorphic facial features, hypotonia and severe global developmental delay that has_material_basis_in heterozygous mutation in the BCL11B gene on chromosome 14q32.2.
Has Material Basis InGENO_0000147
Disease Node Iddisease_node_20001
Doid IdDOID_0111979
LabelImmunodeficiency 49