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Overview of hemolytic uremic syndrome in children

Patrick Niaudet, MD
Olivia Gillion Boyer, MD, PhD
Section Editor
Tej K Mattoo, MD, DCH, FRCP
Deputy Editor
Melanie S Kim, MD


The hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury [1]. It is one of the main causes of acute kidney injury in children. Although all pediatric cases exhibit the classic triad of findings that define HUS, there are a number of various etiologies of HUS that result in differences in presentation, management, and outcome.

This topic will provide an overview of the different causes, evaluation, and initial management of HUS in children.


The classification system used to describe the different etiologies of hemolytic uremic syndrome (HUS) has evolved as ongoing research has provided a better understanding of the underlying causes of HUS, especially those due to genetic mutations in the alternative pathway of complement [2].

Traditionally, HUS had been divided into diarrhea-positive and diarrhea-negative HUS. The former, also referred to as typical HUS, primarily resulted from Shiga toxin-producing Escherichia coli (STEC) infections, and less frequently from Shigella dysenteriae type 1 infection. All other causes of HUS were referred to as atypical HUS or assigned to the diarrhea-negative HUS, even though some patients with non-STEC-associated HUS also presented with diarrhea.

Primary causes without coexisting disease; such as cases due to complement dysregulation (also referred to as atypical HUS) [3]

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Literature review current through: Oct 2017. | This topic last updated: Sep 26, 2017.
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