Atypical aHUS: State of the art.

Abstract:

:Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade.

journal_name

Mol Immunol

journal_title

Molecular immunology

authors

Nester CM,Barbour T,de Cordoba SR,Dragon-Durey MA,Fremeaux-Bacchi V,Goodship TH,Kavanagh D,Noris M,Pickering M,Sanchez-Corral P,Skerka C,Zipfel P,Smith RJ

doi

10.1016/j.molimm.2015.03.246

subject

Has Abstract

pub_date

2015-09-01 00:00:00

pages

31-42

issue

1

eissn

0161-5890

issn

1872-9142

pii

S0161-5890(15)00350-8

journal_volume

67

pub_type

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