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Lookup NU author(s): Professor Tim Goodship


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A microangiopathy is seen in a wide range of conditions, including haemolytic uraemic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), antiphospholipid syndrome (APS), malignant hypertension, pre-eclampsia, the HELLP syndrome, scleroderma and acute allograft vascular rejection. Underlying all these conditions is endothelial cell activation, which is associated with a procoagulant phenotype. The molecular mechanisms responsible for many of these conditions have recently been elucidated. In the atypical form of HUS, mutations have been found in three regulators of the alternative complement pathway. In TTP, antibodies against, and mutations of, the metalloproteinase ADAMTS13 have been described. In APS, antibodies against β2-glycoprotein 1 have been reported. Hopefully these advances in our understanding of these diseases will translate into improved management. © 2007 Elsevier Ltd. All rights reserved.

Publication metadata

Author(s): Goodship T

Publication type: Review

Publication status: Published

Journal: Medicine

Year: 2007

Volume: 35

Issue: 9

Pages: 510-511

ISSN (print): 1357-3039

ISSN (electronic): 1365-4357


DOI: 10.1016/j.mpmed.2007.06.003