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    Thrombotic microangiopathy and associated renal disorders

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    Author
    Barbour, T; Johnson, S; Cohney, S; Hughes, P
    Date
    2012-07-01
    Source Title
    Nephrology Dialysis Transplantation
    Publisher
    OXFORD UNIV PRESS
    University of Melbourne Author/s
    Hughes, Peter
    Affiliation
    Medicine and Radiology
    Metadata
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    Document Type
    Journal Article
    Citations
    Barbour, T., Johnson, S., Cohney, S. & Hughes, P. (2012). Thrombotic microangiopathy and associated renal disorders. NEPHROLOGY DIALYSIS TRANSPLANTATION, 27 (7), pp.2673-2685. https://doi.org/10.1093/ndt/gfs279.
    Access Status
    Open Access
    URI
    http://hdl.handle.net/11343/257308
    DOI
    10.1093/ndt/gfs279
    Abstract
    Thrombotic microangiopathy (TMA) is a pathological process involving thrombocytopenia, microangiopathic haemolytic anaemia and microvascular occlusion. TMA is common to haemolytic uraemic syndrome (HUS) associated with shiga toxin or invasive pneumococcal infection, atypical HUS (aHUS), thrombotic thrombocytopenic purpura (TTP) and other disorders including malignant hypertension. HUS complicating infection with shiga toxin-producing Escherichia coli (STEC) is a significant cause of acute renal failure in children worldwide, occurring sporadically or in epidemics. Studies in aHUS have revealed genetic and acquired factors leading to dysregulation of the alternative complement pathway. TTP has been linked to reduced activity of the ADAMTS13 cleaving protease (typically with an autoantibody to ADAMTS13) with consequent disruption of von Willebrand factor multimer processing. However, the convergence of pathogenic pathways and clinical overlap create diagnostic uncertainty, especially at initial presentation. Furthermore, recent developments are challenging established management protocols. This review addresses the current understanding of molecular mechanisms underlying TMA, relating these to clinical presentation with an emphasis on renal manifestations. A diagnostic and therapeutic approach is presented, based on international guidelines, disease registries and published trials. Early treatment remains largely empirical, consisting of plasma replacement/exchange with the exception of childhood STEC-HUS or pneumococcal sepsis. Emerging therapies such as the complement C5 inhibitor eculizumab for aHUS and rituximab for TTP are discussed, as is renal transplantation for those patients who become dialysis-dependent as a result of aHUS.

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