Atypical aHUS: State of the art

作者:Nester Carla M; Barbour Thomas; Rodriquez de Cordoba Santiago; Dragon Durey Marie Agnes; Fremeaux Bacchi Veronique; Goodship Tim H J; Kavanagh David; Noris Marina; Pickering Matthew; Sanchez Corral Pilar; Skerka Christine; Zipfel Peter; Smith Richard J H*
来源:Molecular Immunology, 2015, 67(1): 31-42.
DOI:10.1016/j.molimm.2015.03.246

摘要

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.