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Open Access 01-10-2020 | Nephrectomy | Pro/Con Debate

Congenital nephrotic syndrome: is early aggressive treatment needed? Yes

Authors: Tuula Hölttä, Hannu Jalanko

Published in: Pediatric Nephrology | Issue 10/2020

Abstract

Congenital nephrotic syndrome (CNS) was primarily considered one disease entity. Hence, one treatment protocol was proposed in the beginning to all CNS patients. Today, with the help of gene diagnostics, we know that CNS is a heterogeneous group of disorders and therefore, different treatment protocols are needed. The most important gene defects causing CNS are NPHS1, NPHS2, WT1, LAMB2, and PLCE1. Before active treatment, all infants with CNS died. It was stated already in the mid-1980s that intensive medical therapy followed by kidney transplantation (KTx) should be the choice of treatment for infants with severe CNS. In Finland, early aggressive treatment protocol was adopted from the USA and further developed for treatment of children with the Finnish type of CNS. The aim of this review is to state reasons for “early aggressive treatment” including daily albumin infusions, intensified nutrition, and timely bilateral nephrectomy followed by KTx at the age of 1–2 years.

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Title: Congenital nephrotic syndrome: is early aggressive treatment needed? Yes
Authors: Tuula Hölttä
Hannu Jalanko
Publication date: 01-10-2020
Publisher: Springer Berlin Heidelberg
Keywords: Nephrectomy
Nephrectomy
Nephrectomy
Nephrotic Syndrome
Published in: Pediatric Nephrology / Issue 10/2020
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-020-04578-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Congenital nephrotic syndrome: is early aggressive treatment needed? Yes

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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