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Chondrodysplasia Punctata, Rhizomelic

Disease ID: disease_node_9832

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DbxrefGARD:13160, ICD10CM:E71.540, MESH:D018902, MIM:PS215100, NCI:C85047, ORDO:177, SNOMEDCT_US_2023_03_01:56692003, UMLS_CUI:C0282529
SubclassofDOID_0050737, DOID_2581
Data SourceDOID, MESH
SynonymsChondrodysplasia Punctata, Rhizomelic Form
Mesh IdD018902
Mesh LabelChondrodysplasia Punctata, Rhizomelic
Mesh SubclassofD002806, D018901
Doid Labelrhizomelic chondrodysplasia punctata
Doid DescriptionA chondrodysplasia punctata that is characterized by disproportionately short stature primarily affecting the proximal parts of the extremities, a typical facial appearance including a broad nasal bridge, epicanthus, high-arched palate, dysplastic external ears, and micrognathia, congenital contractures, characteristic ocular involvement, dwarfism, and severe mental retardation with spasticity. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_9832
Doid IdDOID_2580
LabelChondrodysplasia Punctata, Rhizomelic