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Congenital Disorder Of Glycosylation Ih

Disease ID: disease_node_20413

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DbxrefGARD:9834, MIM:608104, ORDO:79325
SubclassofDOID_0050737, DOID_0050570
Data SourceDOID
Synonymscongenital disorder of glycosylation 1h
Doid Labelcongenital disorder of glycosylation Ih
Doid DescriptionA congenital disorder of glycosylation I that is characterized by gastrointestinal symptoms (diarrhea, vomiting, feeding problems with failure to thrive, protein-losing enteropathy), edema and ascites (including hydrops fetalis), hepatomegaly, renal tubulopathy, coagulation anomalies due to thrombocytopenia, brain involvement (psychomotor delay, seizures, ataxia), facial dysmorphism (low-set ears and retrognathia), pes equinovarus, and muscular hypotonia and has_material_basis_in heterozygous mutation in the gene encoding dolichyl-P-glucose:Glc-1-Man-9-GlcNAc-2-PP-dolichyl-alpha-3-glucosyltransferase on chromosome 11q14.
Has SymptomSYMP_0000465, SYMP_0020046, SYMP_0000526, SYMP_0000460, SYMP_0000538, SYMP_0000124, SYMP_0019145, SYMP_0000570, SYMP_0000470, SYMP_0000005
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_20413
Doid IdDOID_0080560
Disease Has Basis InHP_0001197
LabelCongenital Disorder Of Glycosylation Ih