Management des steroidsensiblen nephrotischen Syndroms im Kindes- und Jugendalter

R. Ehren; M. R. Benz; A. Hackl; L. T. Weber

doi:10.1055/s-0037-1616337

Kinder- und Jugendmedizin, Inhaltsverzeichnis

Kinder- und Jugendmedizin 2016; 16(05): 359-365
DOI: 10.1055/s-0037-1616337

Nephrologie

Schattauer GmbH

Management des steroidsensiblen nephrotischen Syndroms im Kindes- und Jugendalter

Management of childhood nephrotic syndrome

R. Ehren

¹Klinik und Poliklinik für Kinder- und Jugendmedizin der Uniklinik Köln, Abteilung für Pädiatrische Nephrologie, Immunologie und Hypertensiologie

,

M. R. Benz

¹Klinik und Poliklinik für Kinder- und Jugendmedizin der Uniklinik Köln, Abteilung für Pädiatrische Nephrologie, Immunologie und Hypertensiologie

,

A. Hackl

¹Klinik und Poliklinik für Kinder- und Jugendmedizin der Uniklinik Köln, Abteilung für Pädiatrische Nephrologie, Immunologie und Hypertensiologie

,

L. T. Weber

¹Klinik und Poliklinik für Kinder- und Jugendmedizin der Uniklinik Köln, Abteilung für Pädiatrische Nephrologie, Immunologie und Hypertensiologie

› Institutsangaben

Abstract

Zusammenfassung

Das nephrotische Syndrom im Kindesalter erfüllt die Kriterien einer seltenen Erkrankung und doch ist es die häufigste Glomerulopathie in dieser Altersgruppe. Gleichzeitig ist es eine heterogene Erkrankung, die durch die Kategorien Alter bei Erstmanifestation, Ätiologie, Histologie und Ansprechen auf eine Standardtherapie mit Glukokortikoiden eingeteilt werden kann. Wichtigster Prognosefaktor ist das primäre Ansprechen auf eine Standardtherapie mit Glukokortikoiden und differenziert zwischen steroidsensiblem (SSNS) und steroidresistentem nephrotischem Syndrom (SRNS). Eine hohe kumulative Dosis an Glukokortikoiden oder die verlängerte Gabe bei Behandlung der Erstmanifestation scheinen das Rezidivrisiko nicht zu beeinflussen. Trotz der kurz- und langfristigen Nebenwirkungen der Glukokortikoide, die die primäre Therapiesäule des nephrotischen Syndroms darstellen, ist die Bewertung der medikamentösen Alternativen bei Patienten mit häufigen Rezidiven eines SSNS eine ständige differenzialtherapeutische Herausforderung. Dies gilt ebenso für die Therapie des SRNS. Beim SRNS ist eine molekulargenetische Abklärung dringend zu empfehlen. Der vorliegende Text gibt eine Übersicht zur Therapie des SSNS.

Summary

Despite being an orphan disease, idiopathic nephrotic syndrome in children is the most frequent glomerular disease in this age group. Nephrotic syndrome in children is a heterogeneous disease and in order to assess the individual facets of the disease a classification 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 syndrome (SRNS) is most important, because SRNS is a risk factor for developing end-stage renal disease. High cumulative dosages of glucocorticoids or prolonged initial therapy do not seem to influence risk of relapse. In order to avoid short- and long-term glucocorticoid-associated side effects alternative treatment options should be kept in mind planning individual treatment of patients with frequently relapsing disease. Genetic diagnostic is highly advisable in patients with SRNS. The following manuscript gives an overview on the management of SSNS.

Schlüsselwörter

Steroidresistentes nephrotisches Syndrom - Therapie - Glukokortikoide - Komplikationen - Alternativtherapie

Keywords

Steroid-sensitive nephrotic syndrome - steroid-resistant nephrotic syndrome - treatment - glucocorticoids

Volltext

Referenzen

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