Diagnostik und Therapie des idiopathischen nephrotischen Syndroms im Kindesalter
The German Society for Pediatric Nephrology (GPN) has issued a guideline for diagnosis and differential diagnosis of the idiopathic nephrotic syndrome and for therapy of the steroid-sensitive nephrotic syndrome (SSNS) in childhood. Children with SSNS usually remain steroid sensitive and have in gene...
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| Main Authors: | , |
|---|---|
| Format: | Article (Journal) |
| Language: | German |
| Published: |
November 2017
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| In: |
Monatsschrift Kinderheilkunde
Year: 2017, Volume: 165, Issue: 11, Pages: 997-1004 |
| ISSN: | 1433-0474 |
| DOI: | 10.1007/s00112-017-0309-x |
| Online Access: | Verlag, Volltext: http://dx.doi.org/10.1007/s00112-017-0309-x Verlag, Volltext: https://doi.org/10.1007/s00112-017-0309-x 
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| Author Notes: | U. Querfeld, J. Dötsch, J. Gellermann, P. Hoyer, M. Kemper, K. Latta, B. Tönshoff, L.T. Weber, W. Rascher |
MARC
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| 520 | |a The German Society for Pediatric Nephrology (GPN) has issued a guideline for diagnosis and differential diagnosis of the idiopathic nephrotic syndrome and for therapy of the steroid-sensitive nephrotic syndrome (SSNS) in childhood. Children with SSNS usually remain steroid sensitive and have in general a good prognosis even with frequent relapses. Initial manifestations and relapses should be treated with standard steroid therapy according to the current recommendations. Up to 80-90% of patients with SSNS experience relapses, either in the form of infrequent relapses (about 30%) or frequent relapses with or without steroid dependency (30-50%), and up to 15% of patients may become steroid resistant during follow-up. There is a high risk for steroid toxicity in patients with frequent relapses, which increases with duration of therapy and cumulative steroid exposure. Patients with steroid-induced side effects should therefore be treated with steroid-sparing medications. Therapeutic options include cyclosporin A, tacrolimus, mycophenolate mofetil, cyclophosphamide, levamisol, and rituximab. Patients with frequent relapses, steroid-dependent or steroid-resistant NS should be referred to specialized centers for pediatric nephrology. All medications may have serious side effects and complications. Treatment of these patients is demanding and often requires follow-up and immunosuppressive and supportive therapy over many years to prevent long-lasting damage to health. | ||
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