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Refsum Disease

Disease ID: disease_node_6552

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DbxrefGARD:5691, ICD10CM:G60.1, ICD9CM:356.3, MESH:D012035, MIM:266500, NCI:C85043, ORDO:773, SNOMEDCT_US_2023_03_01:25362006, UMLS_CUI:C0034960
SubclassofDOID_3146
Data SourceDOID, MESH
SynonymsHMSN type IV, HSMN IV, Heredopathia atactica polyneuritiformis, Refsum's disease, adult Refsum disease, classic Refsum disease, phytanic acid oxidase deficiency
Mesh IdD012035
Mesh LabelRefsum Disease
Mesh SubclassofD020739, D015417, D018901
Doid LabelRefsum disease
Doid DescriptionA lipid metabolic disorder that is characterized by a tetrad of clinical abnormalities: retinitis pigmentosa, peripheral neuropathy, cerebellar ataxia, and accumulation of an unusual branched-chain fatty acid, phytanic acid, in blood and tissues. OMIM mapping confirmed by DO. [SN].
Disease Node Iddisease_node_6552
Doid IdDOID_10582
LabelRefsum Disease