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Brachyolmia-Amelogenesis Imperfecta Syndrome

Disease ID: disease_node_20255

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DbxrefGARD:5478, ICD10CM:Q76.3, MIM:601216, ORDO:2899
SubclassofDOID_225, DOID_0050737
Data SourceDOID
SynonymsDASS, STHAG6, dental anomalies and short stature, platyspondyly with amelogenesis imperfecta, selective tooth agenesis 5
Doid Labelbrachyolmia-amelogenesis imperfecta syndrome
Doid DescriptionA syndrome characterized by skeletal dysplasia (broad ilia, elongated femoral necks with coxa valga, scoliosis), mild platyspondyly, short trunked short stature, and amelogenesis imperfecta that has_material_basis_in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the latent transforming growth factor beta binding protein 3 gene (LTBP3) on chromosome 11q13.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20255
Doid IdDOID_0090143
LabelBrachyolmia-Amelogenesis Imperfecta Syndrome