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Nephrotisches Syndrom im Kindesalter

Nephrotic syndrome in childhood

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Zusammenfassung

Das nephrotische Syndrom im Kindesalter erfüllt zwar die Kriterien einer seltenen Erkrankung, ist aber gleichzeitig die häufigste Glomerulopathie in dieser Altersgruppe. Die Diagnose wird über die große Proteinurie und die Hypalbuminämie gestellt. Ödeme sind bei der Erstmanifestation meist vorhanden. Die Heterogenität der Erkrankung bezüglich Patientenalter bei Erstmanifestation, Ätiologie, histologischen Befunden und Ansprechen auf eine Standardtherapie mit Glukokortikoiden beeinflusst die Differenzialdiagnostik ebenso wie die Therapie. Da die Prognose des steroidsensiblen nephrotischen Syndroms hinsichtlich der Nierenfunktion generell gut ist, soll neben der Wirksamkeit auch das potenzielle Nebenwirkungsspektrum in die Planung der Behandlung einbezogen werden.

Abstract

Despite being an orphan disease, idiopathic nephrotic syndrome in children is the most frequent glomerular disease in this age group. Nephrotic syndrome is characterized by severe proteinuria and hypoalbuminemia. Edema is mostly present, especially at initial presentation. Nephrotic syndrome in children is a heterogeneous disease with respect to age of onset, etiology, histology and response to the standard treatment with glucocorticoids. This diversity strongly influences the diagnostic approach and treatment. As the prognosis of steroid-sensitive nephrotic syndrome with respect to renal function is generally good, not only the efficacy but also the toxicity of different treatment regimens should be kept in mind when planning individualized treatment.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. M.R. Benz und M.J. Kemper geben an, dass kein Interessenkonflikt besteht.

Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Fall von nichtmündigen Patienten liegt die Einwilligung eines Erziehungsberechtigten oder des gesetzlich bestellten Betreuers vor. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Pädiatrische Nephrologie, Klinik und Poliklinik für Kinder- und Jugendmedizin, Uniklinik Köln, Kerpener Str. 62, 50937, Köln, Deutschland

    M.R. Benz

  2. Pädiatrische Nephrologie, Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland

    M.J. Kemper

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Correspondence to M.R. Benz.

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Benz, M., Kemper, M. Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 163, 310–322 (2015). https://doi.org/10.1007/s00112-014-3224-4

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