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Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance

Disease definition

A rare, idiopathic nephrotic syndrome characterized by pediatric onset of proteinuria, hypoalbuminemia and edema. Patients respond successfully to the initial standard course of corticosteroids, but are resistant to standard therapy for a subsequent relapse and following this relapse remain steroid-resistant.


Classification level: Disorder
  • Synonym(s):
    • Idiopathic SSNS with secondary steroid resistance
    • Secondary SRNS
    • Secondary steroid-resistant nephrotic syndrome
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: All ages
  • ICD-10: N04.8
  • OMIM: -
  • UMLS: C5680131
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
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