Universität zu Köln

Ehren, R., Brinkkoetter, P., Weber, L. T. and Querfeld, U. (2019). Nephrotic syndrome in childhood and adolescence. Summary of the AWMF guidelines considering special aspects of diagnostics and treatment in adults. Nephrologe, 14 (3). S. 184 - 192. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1862-0418

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Abstract

Background. Despite fulfilling the criteria for an orphan disease, idiopathic nephrotic syndrome (NS) in children is the most frequent glomerular disease in this age group. At the same time NS in children is aheterogeneous disease and in order to assess the individual facets of the disease aclassification using the following criteria is helpful: etiology, age at onset, histology and responsiveness to initial standard treatment with glucocorticoids. As for prognosis the differentiation between steroid-sensitive (SSNS) and steroid-resistant nephrotic syndromes (SRNS) is most important because SRNS is arisk factor for developing end-stage renal disease. High cumulative doses of glucocorticoids or prolonged administration in the initial treatment do not influence the risk of a relapse. Genetic testing is highly advisable in patients with SRNS to avoid unnecessary and ineffective immunosuppressive therapy. Methods. This manuscript gives an overview on the management of SSNS and SRNS and provides insights into the different diagnostic and therapeutic approaches in pediatric and adult medicine. Furthermore, associated risks and potential long-term side effects of treatment are described. Conclusion. Frequently relapsing NS and SRNS are still challenging to treat. The use of steroids and immunosuppressive maintenance therapy has severe long-term side effects. The optimal treatment of adult patients with childhood onset SSNS and infrequent relapses remains unclear.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Ehren, R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Brinkkoetter, P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Weber, L. T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Querfeld, U. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-149539
DOI:	10.1007/s11560-019-0313-3
Journal or Publication Title:	Nephrologe
Volume:	14
Number:	3
Page Range:	S. 184 - 192
Date:	2019
Publisher:	SPRINGER HEIDELBERG
Place of Publication:	HEIDELBERG
ISSN:	1862-0418
Language:	German
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SEGMENTAL GLOMERULAR SCLEROSIS; MYCOPHENOLATE-MOFETIL; CYCLOSPORINE-A; CHILDREN; THERAPY; TRIAL; GLOMERULOSCLEROSIS; REMISSION; PROGNOSIS; ABATACEPT Multiple languages Urology & Nephrology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/14953