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Schinzel Type Phocomelia

Disease ID: disease_node_20232

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DbxrefGARD:9212, MIM:276820, ORDO:2879
SubclassofDOID_225, DOID_0050737
Data SourceDOID
SynonymsAARRS, Al Awadi-Raas-Rothschild syndrome, Al-Awadi/Raas-Rothschild/Schinzel phocomelia syndrome, LPHAS, Schinzel phocomelia syndrome, absence of ulna and fibula with severe limb deficiency, aplasia/hypoplasia of limbs and pelvis, congenital absence of ulna and fibula, limb/pelvis-hypoplasia/aplasia syndrome, severe limb deficit
Doid LabelSchinzel type phocomelia
Doid DescriptionA syndrome characterized by severe malformations of upper and lower limbs,severely hypoplastic pelvis, and abnormal genitalia that has_material_basis_in homozygous or compound heterozygous mutation in the WNT7A gene on chromosome 3p25.1.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20232
Doid IdDOID_0112181
LabelSchinzel Type Phocomelia