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Atypical aHUS: State of the art
Journal article   Peer reviewed

Atypical aHUS: State of the art

Carla M Nester, Thomas Barbour, Santiago Rodriquez de Cordoba, Marie Agnes Dragon-Durey, Veronique Fremeaux-Bacchi, Tim H J Goodship, David Kavanagh, Marina Noris, Matthew Pickering, Pilar Sanchez-Corral, …
Molecular immunology, Vol.67(1), pp.31-42
09/2015
DOI: 10.1016/j.molimm.2015.03.246
PMID: 25843230

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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.
Communicable Diseases - pathology Kidney Neoplasms - genetics Complement Activation Kidney - pathology Humans Kidney - immunology Kidney Neoplasms - immunology Complement C3b Inactivator Proteins - genetics Atypical Hemolytic Uremic Syndrome - etiology Communicable Diseases - genetics Atypical Hemolytic Uremic Syndrome - immunology Autoantibodies - biosynthesis Adult Kidney Neoplasms - complications Atypical Hemolytic Uremic Syndrome - genetics Child Complement Factor H - immunology Genetic Predisposition to Disease Gene Expression Regulation - immunology Kidney Transplantation Atypical Hemolytic Uremic Syndrome - pathology Communicable Diseases - immunology Complement C3b - genetics Kidney Neoplasms - pathology Complement Factor H - genetics Communicable Diseases - complications Complement C3b - immunology Complement C3b Inactivator Proteins - immunology

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