TY - JOUR
T1 - Atypical aHUS
T2 - State of the art
AU - Nester, Carla M.
AU - Barbour, Thomas
AU - de Cordoba, Santiago Rodriquez
AU - Dragon-Durey, Marie Agnes
AU - Fremeaux-Bacchi, Veronique
AU - Goodship, Tim H J
AU - Kavanagh, David
AU - Noris, Marina
AU - Pickering, Matthew
AU - Sanchez-Corral, Pilar
AU - Skerka, Christine
AU - Zipfel, Peter
AU - Smith, Richard J H
PY - 2015/9/1
Y1 - 2015/9/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=84936985706&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84936985706&partnerID=8YFLogxK
U2 - 10.1016/j.molimm.2015.03.246
DO - 10.1016/j.molimm.2015.03.246
M3 - Article
C2 - 25843230
AN - SCOPUS:84936985706
VL - 67
SP - 31
EP - 42
JO - Molecular Immunology
JF - Molecular Immunology
SN - 0161-5890
IS - 1
ER -