Trautmann, Agnes, Vivarelli, Marina, Samuel, Susan, Gipson, Debbie, Sinha, Aditi, Schaefer, Franz, Hui, Ng Kar, Boyer, Olivia ORCID: 0000-0002-3957-1359, Saleem, Moin A., Feltran, Luciana, Mueller-Deile, Janina, Becker, Jan Ulrich ORCID: 0000-0003-2929-8085, Cano, Francisco, Xu, Hong, Lim, Yam Ngo, Smoyer, William, Anochie, Ifeoma, Nakanishi, Koichi, Hodson, Elisabeth and Haffner, Dieter (2020). IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome. Pediatr. Nephrol., 35 (8). S. 1529 - 1562. NEW YORK: SPRINGER. ISSN 1432-198X

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Abstract

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Trautmann, AgnesUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vivarelli, MarinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Samuel, SusanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gipson, DebbieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sinha, AditiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schaefer, FranzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hui, Ng KarUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Boyer, OliviaUNSPECIFIEDorcid.org/0000-0002-3957-1359UNSPECIFIED
Saleem, Moin A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Feltran, LucianaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mueller-Deile, JaninaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Becker, Jan UlrichUNSPECIFIEDorcid.org/0000-0003-2929-8085UNSPECIFIED
Cano, FranciscoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Xu, HongUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lim, Yam NgoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Smoyer, WilliamUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Anochie, IfeomaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nakanishi, KoichiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hodson, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haffner, DieterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-334266
DOI: 10.1007/s00467-020-04519-1
Journal or Publication Title: Pediatr. Nephrol.
Volume: 35
Number: 8
Page Range: S. 1529 - 1562
Date: 2020
Publisher: SPRINGER
Place of Publication: NEW YORK
ISSN: 1432-198X
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
FOCAL SEGMENTAL GLOMERULOSCLEROSIS; INTRAVENOUS PULSE CYCLOPHOSPHAMIDE; RENAL-TRANSPLANT RECIPIENTS; MINIMAL-CHANGE DISEASE; HIGH BLOOD-PRESSURE; MYCOPHENOLATE-MOFETIL; KIDNEY-TRANSPLANTATION; CYCLOSPORINE-A; RISK-FACTORS; RITUXIMAB THERAPYMultiple languages
Pediatrics; Urology & NephrologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/33426

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