Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

Daniel Landau; Evgenia Gurevich; Joseph Kapelushnik; Hannah Tamary; Ilan Shelef; Isaac Lazar

doi:10.1007/s00467-013-2583-8

Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

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

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

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.

Original language	English
Pages (from-to)	2389-2392
Number of pages	4
Journal	Pediatric Nephrology
Volume	28
Issue number	12
DOIs	https://doi.org/10.1007/s00467-013-2583-8
State	Published - 1 Dec 2013

Keywords

Acute kidney injury
Hemophagocytic lymphohistiocytosis
Macrophage activation syndrome
Nephrotic syndrome

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Nephrology

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10.1007/s00467-013-2583-8

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title = "Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis",

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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AU - Landau, Daniel

AU - Gurevich, Evgenia

AU - Kapelushnik, Joseph

AU - Tamary, Hannah

AU - Shelef, Ilan

AU - Lazar, Isaac

PY - 2013/12/1

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N2 - 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.

AB - 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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KW - Hemophagocytic lymphohistiocytosis

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Association between childhood nephrotic syndrome and hemophagocytic lymphohistiocytosis

Abstract

Keywords

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