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Pediatric Nephrology

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01.10.2014 | Review

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

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

Erschienen in: Pediatric Nephrology | Ausgabe 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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Titel: Making sense of the spectrum of glomerular disease associated with complement dysregulation
verfasst von: Sally Ann Johnson
Edwin K. S. Wong
C. Mark Taylor
Publikationsdatum: 01.10.2014
Verlag: Springer Berlin Heidelberg
Erschienen in: Pediatric Nephrology / Ausgabe 10/2014
Print ISSN: 0931-041X
Elektronische ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-013-2559-8

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