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Ellis-Van Creveld Syndrome

Disease ID: disease_node_2761

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DbxrefGARD:1301, ICD10CM:Q77.6, ICD9CM:756.55, MESH:D004613, MIM:225500, NCI:C84684, SNOMEDCT_US_2023_03_01:62501005, UMLS_CUI:C0013903
SubclassofDOID_225, DOID_0050737
Data SourceDOID, MESH
SynonymsChondroectodermal dysplasia, mesoectodermal dysplasia
Mesh IdD004613
Mesh LabelEllis-Van Creveld Syndrome
Mesh SubclassofD010009, D004476
Doid LabelEllis-Van Creveld syndrome
Doid DescriptionA syndrome characterized by short limbs, short ribs, postaxial polydactyly, dysplastic nails and teeth, and in many patients congenital cardiac defects that has_material_basis_in homozygous or compound heterozygous mutation in either the EVC or EVC2 gene on chromosome 4p16.2. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_2761
Doid IdDOID_12714
LabelEllis-Van Creveld Syndrome