Querfeld, U., Doetsch, J., Gellermann, J., Hoyer, P., Kemper, M., Latta, K., Toenshoff, B., Weber, L. T. and Rascher, W. (2017). Diagnosis and therapy of idiopathic nephrotic syndrome in childhood. Summary of the S2e guideline AWMF registry number 166-001, headed by the German Society for Pediatric Nephrology. Mon.schr. Kinderheilkd., 165 (11). S. 997 - 1005. NEW YORK: SPRINGER. ISSN 1433-0474

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Abstract

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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Querfeld, U.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doetsch, J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gellermann, J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoyer, P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kemper, M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Latta, K.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Toenshoff, B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weber, L. T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rascher, W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-212932
DOI: 10.1007/s00112-017-0309-x
Journal or Publication Title: Mon.schr. Kinderheilkd.
Volume: 165
Number: 11
Page Range: S. 997 - 1005
Date: 2017
Publisher: SPRINGER
Place of Publication: NEW YORK
ISSN: 1433-0474
Language: German
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
PediatricsMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/21293

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