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Immunodeficiency 23

Disease ID: disease_node_13300

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DbxrefGARD:4331, MIM:615816, NCI:C126339, ORDO:443811, SNOMEDCT_US_2023_03_01:1187623009, UMLS_CUI:C4014371
SubclassofDOID_628, DOID_0050737
Data SourceDOID
SynonymsCID due to PGM3 deficiency, IMD23, PGM3-CDG, PGM3-related congenital disorder of glycosylation, combined immunodeficiency due to PGM3 deficiency
Doid Labelimmunodeficiency 23
Doid DescriptionA combined T cell and B cells immunodeficiency characterized by marked atopy and autoimmunity caused by increased T(H)2 and T(H)17 cytokine production by CD4(+) T cells, T-cell lymphopenia, reduced memory B-cell numbers, recurrent respiratory and skin infections beginning in early childhood, increased serum IgE, and variable developmental delay or intellectual impairment that has_material_basis_in homozygous or compound heterozygous mutation in the PGM3 gene on chromosome 6q14.1.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13300
Doid IdDOID_0111953
LabelImmunodeficiency 23