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Glycogen Storage Disease Type I

Disease ID: disease_node_3508

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DbxrefICD10CM:E74.01, MESH:D005953, NCI:C84733, ORDO:364, SNOMEDCT_US_2023_03_01:7265005, UMLS_CUI:C0017920
SubclassofDOID_2747
Data SourceDOID, MESH
SynonymsGlycogen storage disease 1, deficiency of glucose-6-phosphatase, glycogen storage disease type I, glycogenosis type I, von Gierke disease, von Gierke's disease
Mesh IdD005953
Mesh LabelGlycogen Storage Disease Type I
Mesh SubclassofD006008
Doid Labelglycogen storage disease I
Doid DescriptionA glycogen storage disease that is characterized by severe hypoglycemia and hepatomegaly caused by the accumulation of glycogen. Affected individuals exhibit growth retardation, delayed puberty, lactic acidemia, hyperlipidemia, hyperuricemia, and in adults a high incidence of hepatic adenomas.
Existence Starts DuringHP_0003593
Disease Node Iddisease_node_3508
Doid IdDOID_0081329
LabelGlycogen Storage Disease Type I