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Text
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<a href="http://doi.org/10.1111/1744-9987.12763" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/1744-9987.12763</a>
Pages
4–21
Issue
1
Volume
23
Dublin Core
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Title
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Atypical Hemolytic-Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment.
Publisher
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Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy
Date
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2019
2019-02
Subject
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Atypical hemolytic uremic syndrome; Complement regulatory protein mutation; Eculizumab; Renal impairment; Thrombotic microangiopathy
Creator
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Raina Rupesh; Krishnappa Vinod; Blaha Taryn; Kann Taylor; Hein William; Burke Linda; Bagga Arvind
Description
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Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over-activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy-associated aHUS, and aHUS in renal transplant patients.
Identifier
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<a href="http://doi.org/10.1111/1744-9987.12763" target="_blank" rel="noreferrer noopener">10.1111/1744-9987.12763</a>
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Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
2019
Atypical hemolytic uremic syndrome
Bagga Arvind
Blaha Taryn
Burke Linda
Complement regulatory protein mutation
Department of Internal Medicine
Eculizumab
Hein William
Kann Taylor
Krishnappa Vinod
NEOMED College of Graduate Studies Student
NEOMED College of Medicine
Raina Rupesh
Renal impairment
Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy
Thrombotic microangiopathy