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Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance

ORPHA:567546· ICD-10 N04.8

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.

Prevalence
Unknown
Age of onset
All ages