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Congenital Disorder Of Glycosylation Iu

Disease ID: disease_node_20402

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DbxrefGARD:12416, MIM:615042, ORDO:329178
SubclassofDOID_0050737, DOID_0050570
Data SourceDOID
Synonymscongenital disorder of glycosylation 1u
Doid Labelcongenital disorder of glycosylation Iu
Doid DescriptionA congenital disorder of glycosylation I that is characterized by respiratory distress and severe hypotonia at birth, severe global developmental delay, early-onset intractable seizures, myopathic fascies with craniofacial dysmorphism (trigonocephaly/progressive microcephaly, low anterior hairline, arched eyebrows, hypotelorism, strabismus, small nose, prominent philtrum, thin upper lip, high-arched palate, micrognathia, malocclusion), severe, congenital flexion joint contractures and elevated serum creatine kinase levels and has_material_basis_in homozygous or compound heterozygous mutation in the DPM2 gene on chromosome 9q34.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20402
Doid IdDOID_0080571
Disease Has Basis InHP_0001197
LabelCongenital Disorder Of Glycosylation Iu