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Nephronophthisis

Disease ID: disease_node_19859

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DbxrefGARD:206, ICD10CM:Q61.5, MIM:PS256100, NCI:C123200, ORDO:655, SNOMEDCT_US_2023_03_01:204958008, UMLS_CUI:C0687120
SubclassofDOID_557, DOID_0050737
Data SourceDOID
Synonymsmedullary cystic disease, medullary cystic kidney
Doid Labelnephronophthisis
Doid DescriptionA kidney disease that is characterized by a chronic tubulointerstitial nephritis that progress to terminal renal failure during the second decade (juvenile form) or before the age of 5 years (infantile form) resulting from dysfunction of ciliary proteins (ciliopathy). Xref MGI. OMIM mapping confirmed by DO. [SN].
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_19859
Doid IdDOID_12712
LabelNephronophthisis