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Nephrotic syndrome, idiopathic, steroid-resistant MeSH Supplementary Concept Data 2025


MeSH Supplementary
Nephrotic syndrome, idiopathic, steroid-resistant
Unique ID
C536404
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/C536404
Entry Term(s)
Nephrotic syndrome, steroid-resistant, autosomal recessive
Registry Numbers
0
Heading Mapped to
Nephrotic Syndrome / congenital
Frequency
147
Note
An autosomal recessive disorder characterized by childhood onset of PROTEINURIA; HYPOALBUMINEMIA; HYPERLIPIDEMIA; and EDEMA. Kidney biopsies show nonspecific histologic changes such as minimal change, FOCAL SEGMENTAL GLOMERULOSCLEROSIS (FSGS), and diffuse mesangial proliferation. The disorder is resistant to steroid treatment and progresses to END-STAGE RENAL FAILURE in the first or second decades. Germline mutations in the PDCN (NPHS2) gene have been identified. OMIM: 600995
Date of Entry
2010/08/25
Revision Date
2015/08/18
Nephrotic syndrome, idiopathic, steroid-resistant Preferred
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