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Nijmegen Breakage Syndrome

Disease ID: disease_node_11410

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DbxrefGARD:3904, MESH:D049932, MIM:251260, NCI:C4692, ORDO:647, SNOMEDCT_US_2023_03_01:234638009, UMLS_CUI:C0398791
SubclassofDOID_225, DOID_0050737
Data SourceDOID, MESH
SynonymsBerlin breakage syndrome, Microcephaly, normal intelligence and immunodeficiency, NBS, Seemanova syndrome II, Seemanova syndrome type 2, ataxia-telangiectasia variant, immunodeficiency-microcephaly-chromosomal instability syndrome, microcephaly-immunodeficiency-lymphoreticuloma syndrome
Mesh IdD049932
Mesh LabelNijmegen Breakage Syndrome
Mesh SubclassofD049914
Doid LabelNijmegen breakage syndrome
Doid DescriptionA syndrome characterized by chromosomal instability, microcephaly, growth retardation, immunodeficiency, cellular hypersensitivity to X-rays, and predisposition to cancer that has_material_basis_in homozygous or compound heterozygous mutation in NBN on chromosome 8q21.3. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_11410
Doid IdDOID_7400
LabelNijmegen Breakage Syndrome