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Optimal management of atypical hemolytic uremic disease: challenges and solutions

Authors Raina R, Grewal MK, Radhakrishnan Y, Tatineni V, DeCoy M, Burke LLG, Bagga A

Received 11 May 2019

Accepted for publication 23 July 2019

Published 4 September 2019 Volume 2019:12 Pages 183—204


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Pravin Singhal

Rupesh Raina1,2,*, Manpreet K Grewal2,*, Yeshwanter Radhakrishnan3, Vineeth Tatineni4, Meredith DeCoy5, Linda LG Burke5, Arvind Bagga6

1Department of Nephrology, Akron Children’s Hospital, Akron, OH, USA; 2Akron Nephrology Associates, Cleveland Clinic Akron General, Akron, OH, USA; 3Department of Internal Medicine, Cleveland Clinic Akron General Hospital, Akron, OH, USA; 4Department of Internal Medicine, Summa Health, Akron City Hospital, Akron, OH, USA; 5Atypical Hemolytic Uremic Syndrome Alliance, Cape Elizabeth, ME, USA; 6Division of Paediatric Nephrology, All India Institute of Medical Sciences, New Delhi, India

*These authors contributed equally to this work

Correspondence: Rupesh Raina
Department of Nephrology, Cleveland Clinic Akron General, 224 W. Exchange St., Akron, OH 44302, USA
Tel +1 330 436 3150

Abstract: Atypical hemolytic uremic syndrome (aHUS) is a chronic life threatening condition that arises from genetic abnormalities resulting in uncontrolled complement amplifying activity. The introduction of eculizumab, the humanized monoclonal antibody, has brought about a paradigm shift in the management of aHUS. However, there are many knowledge gaps, diagnostic issues, access and cost issues, and patient or physician challenges associated with the use of this agent. Limited data on the natural history of aHUS along with the underlying genetic mutations make it difficult to predict the relapses and thereby raising concerns about the appropriate duration and monitoring of treatment. In this review, we discuss the safety and efficacy of eculizumab in patients with aHUS and its associated challenges.

Keywords: atypical hemolytic uremic syndrome, eculizumab, challenges, thrombotic microangiopathy

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