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Immunodeficiency 58

Disease ID: disease_node_13312

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DbxrefMIM:618131, ORDO:542301, UMLS_CUI:C4748304
SubclassofDOID_628, DOID_0050737
Data SourceDOID
SynonymsIMD58, severe combined immunodeficiency due to CARMIL2 deficiency
Doid Labelimmunodeficiency 58
Doid DescriptionA combined T cell and B cell immunodeficiency characterized by defective T-cell function with decreased Treg cells and deficient CD3/CD28 costimulation responses in both CD4+ and CD8+ T cells, variable impairment in B-cell function, early-onset skin lesions, recurrent respiratory infections or allergies, and chronic persistent infections that has_material_basis_in homozygous or compound heterozygous mutation in the CARMIL2 gene on chromosome 16q22.1.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13312
Doid IdDOID_0111984
LabelImmunodeficiency 58