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Encyclopedia of Medical Immunology pp 1–9Cite as

Complement Deficiencies Associated with Atypical Hemolytic Uremic Syndrome

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Synonyms

Kidney disease; Microangiopathy

Definition

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy characterized by a triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure in the absence of an infection with Shiga toxin-producing bacteria.

Introduction

The complement system is a major component of innate immunity and an effector arm and instructor of the adaptive immune response (Walport 2001a, b). It plays a key role in immune complex handling and clearance of apoptotic cells. Because of the potency of the system, strict regulation is required in time and space. Dysregulation of complement is increasingly recognized as a predisposing factor to human disease such as in aHUS (Holers 2008). Inherited defects in complement genes and acquired autoantibodies against a regulatory protein have been linked to development of aHUS. Until recently, the prognosis for aHUS patients was poor, but a novel complement inhibitory monoclonal...

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References

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Authors and Affiliations

  1. Sevion Inc, Anthro Bio Inc, St. Louis, 63108, MO, USA

    Elizabeth C. Schramm

  2. Division of Nephrology, Department of Medicine, Washington University School of Medicine, St. Louis, 63110, MO, USA

    Anuja Java

  3. Division of Rheumatology, Department of Medicine, Washington University School of Medicine, St. Louis, 63110, MO, USA

    M. Kathryn Liszewski & John P. Atkinson

Authors
  1. Elizabeth C. Schramm
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  2. Anuja Java
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  3. M. Kathryn Liszewski
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  4. John P. Atkinson
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Corresponding author

Correspondence to Elizabeth C. Schramm .

Editor information

Editors and Affiliations

  1. Molecular Biology, Monash University Dept. Biochemistry &, Clayton, Victoria, Australia

    Ian MacKay

  2. Autoimmune Disease Research Center, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

    Noel R. Rose

Section Editor information

  1. University of Pennsylvania, PA 19104, Philadelphia, USA

    Kathleen Sullivan

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Schramm, E.C., Java, A., Liszewski, M.K., Atkinson, J.P. (2016). Complement Deficiencies Associated with Atypical Hemolytic Uremic Syndrome. In: MacKay, I., Rose, N. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9209-2_5-1

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  • DOI: https://doi.org/10.1007/978-1-4614-9209-2_5-1

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9209-2

  • Online ISBN: 978-1-4614-9209-2

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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