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Published in: Pediatric Nephrology 12/2013

01-12-2013 | Brief Report

Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

Authors: Daniel Landau, Evgenia Gurevich, Joseph Kapelushnik, Hannah Tamary, Ilan Shelef, Isaac Lazar

Published in: Pediatric Nephrology | Issue 12/2013

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Abstract

Background

Hemophagocytic lymphohistiocytosis (HLH) is caused by an excessive activation of nonmalignant macrophages. Renal lesions have been described in association with, but always after, HLH diagnosis.

Case-diagnosis

We describe a previously healthy 26-month-old girl who presented originally with steroid-responsive nephrotic syndrome (NS), but after 4 months, on the first NS relapse, experienced numerous complications (many of them reported to accompany NS as single events). Clinical and laboratory signs of HLH evolved with time and led to deterioration of her condition and death, within 5 months of her original presentation.

Conclusions

To our knowledge, this is the first report of NS antedating the presentation of HLH.
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Metadata
Title
Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis
Authors
Daniel Landau
Evgenia Gurevich
Joseph Kapelushnik
Hannah Tamary
Ilan Shelef
Isaac Lazar
Publication date
01-12-2013
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 12/2013
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-013-2583-8

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