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Pediatric Nephrology
Published in: Pediatric Nephrology 10/2014

01-10-2014 | Review

Making sense of the spectrum of glomerular disease associated with complement dysregulation

Authors: Sally Ann Johnson, Edwin K. S. Wong, C. Mark Taylor

Published in: Pediatric Nephrology | Issue 10/2014

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Abstract

Over recent years, complement has emerged as a major player in the development of a number of glomerular diseases, including atypical haemolytic uraemic syndrome, membranoproliferative glomerulonephritis and the recently described C3 glomerulonephritis. Some patients and pedigrees show overlapping features of these conditions. Intriguingly, a few complement gene mutations are common to different disease phenotypes. In this review, we explore the evidence for complement dysregulation in these diseases and the clinical interface between them, and present a hypothesis to explain the variable phenotype associated with dysregulation of the alternative complement pathway.
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Metadata
Title
Making sense of the spectrum of glomerular disease associated with complement dysregulation
Authors
Sally Ann Johnson
Edwin K. S. Wong
C. Mark Taylor
Publication date
01-10-2014
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 10/2014
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-013-2559-8

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