Diagnostik und Therapie des idiopathischen nephrotischen Syndroms im Kindesalter

• Verfasst von: Querfeld, Uwe [VerfasserIn]
• Verfasst von: Tönshoff, Burkhard [VerfasserIn]
• Titel: Diagnostik und Therapie des idiopathischen nephrotischen Syndroms im Kindesalter
• Verf.angabe: U. Querfeld, J. Dötsch, J. Gellermann, P. Hoyer, M. Kemper, K. Latta, B. Tönshoff, L.T. Weber, W. Rascher
• E-Jahr: 2017
• Jahr: November 2017
• Umfang: 8 S.
• Teil: volume:165
• Teil: year:2017
• Teil: number:11
• Teil: pages:997-1004
• Teil: extent:8
• Fussnoten: Online publiziert: 20. Juni 2017 ; Gesehen am 22.10.2018
• Titel Quelle: Enthalten in: Monatsschrift Kinderheilkunde
• Ort Quelle: Berlin : Springer, 1996
• Jahr Quelle: 2017
• Band/Heft Quelle: 165(2017), 11, Seite 997-1004
• ISSN Quelle: 1433-0474
• DOI: doi:10.1007/s00112-017-0309-x
• Datenträger: Online-Ressource
• Sprache: ger
• Sach-SW: Proteinuria
• Sach-SW: Proteinurie
• Sach-SW: Relapse
• Sach-SW: Remission
• Sach-SW: Rezidiv
• Sach-SW: Steroide
• Sach-SW: Steroids
• Sach-SW: Toxicity
• Sach-SW: Toxizität
• K10plus-PPN: 1582160708

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.