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Start free with EleplanIdiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance
ORPHA:567546Clinical syndrome
Also called Idiopathic SSNS with secondary steroid resistance · Secondary SRNS · Secondary steroid-resistant nephrotic syndrome
What it is
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.
Key facts
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Clinical syndrome
Signs and symptoms
Common30–79%
9Sometimes5–29%
9- Abdominal pain
- Allergy
- Dyspnea
- Hydrocele testis
- Hypertension
- Microscopic hematuria
- Minimal change glomerulonephritis
- Respiratory tract infection
and 1 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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