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Management of hemolytic-uremic syndrome in children

Authors Grisaru S

Received 13 February 2014

Accepted for publication 20 March 2014

Published 12 June 2014 Volume 2014:7 Pages 231—239


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3

Silviu Grisaru

University of Calgary, Alberta Children's Hospital, Calgary, Alberta, Canada

Abstract: Acute renal failure associated with a fulminant, life-threatening systemic disease is rare in previously healthy young children; however, when it occurs, the most common cause is hemolytic-uremic syndrome (HUS). In most cases (90%), this abrupt and devastating illness is a result of ingestion of food or drink contaminated with pathogens that produce very potent toxins. Currently, there are no proven treatment options that can directly inactivate the toxin or effectively interfere with the cascade of destructive events triggered by the toxin once it gains access to the bloodstream and binds its receptor. However, HUS is self-limited, and effective supportive management during the acute phase is proven to be a life saver for children affected by HUS. A minority of childhood HUS cases, approximately 5%, are caused by various genetic mutations causing uncontrolled activation of the complement system. These children, who used to have a poor prognosis leading to end-stage renal disease, now have access to exciting new treatment options that can preserve kidney function and avoid disease recurrences. This review provides a summary of the current knowledge on the epidemiology, pathophysiology, and clinical presentation of childhood HUS, focusing on a practical approach to best management measures.

Keywords: hemolytic, uremic, E.coli O157:H7, thrombotic, microangiopathy, complement system

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