Universität zu Köln

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:	Creators Email ORCID ORCID Put Code Querfeld, U. UNSPECIFIED UNSPECIFIED UNSPECIFIED Doetsch, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Gellermann, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Hoyer, P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kemper, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Latta, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Toenshoff, B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Weber, L. T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Rascher, W. UNSPECIFIED UNSPECIFIED UNSPECIFIED
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:	Keywords Language Pediatrics Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/21293