Cover Image

Atypical hemolytic uremic syndrome: a brief review

Kuixing Zhang, Yuxin Lu, Kevin T. Harley, Minh-Ha Tran
  • Kuixing Zhang
    Department of Pathology and Laboratory Medicine, Irvine School of Medicine, University of California, Orange, CA, United States | kzhang9@uci.edu
  • Yuxin Lu
    Department of Pathology and Laboratory Medicine, Irvine School of Medicine, University of California, Orange, CA, United States
  • Kevin T. Harley
    Department of Internal Medicine, Division of Nephrology and Hypertension, Irvine School of Medicine, University of California, Orange, CA, United States
  • Minh-Ha Tran
    Department of Pathology and Laboratory Medicine, Irvine School of Medicine, University of California, Orange, CA, United States

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury. Pathologic mutations include those resulting in loss-of-function in a complement regulatory gene (CFH, CFI, CD46 or THBD) or gain-of-function in an effector gene (CFB or C3). Treatment with the late complement inhibitor, eculizumab – a monoclonal antibody directed against C5 – is effective.

Keywords

Atypical hemolytic uremic syndrome; genetics

Full Text:

PDF
HTML
Submitted: 2017-01-17 18:52:09
Published: 2017-06-01 11:46:54
Search for citations in Google Scholar
Related articles: Google Scholar
Abstract views:
885

Views:
PDF
641
HTML
90

Article Metrics

Metrics Loading ...

Metrics powered by PLOS ALM


Copyright (c) 2017 Kuixing Zhang, Yuxin Lu, Kevin T. Harley, Minh-Ha Tran

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
 
© PAGEPress 2008-2017     -     PAGEPress is a registered trademark property of PAGEPress srl, Italy.     -     VAT: IT02125780185