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Treatment and prognosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) 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]. The most common cause of HUS is due to Shiga toxin-producing Escherichia coli (STEC), and it is one of the main causes of acute kidney injury in children under the age of three years.

The treatment and prognosis of STEC-HUS is reviewed here. The clinical manifestations and diagnosis of STEC-HUS are discussed separately. (See "Clinical manifestations and diagnosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) in children".)


Traditionally, HUS had been divided into diarrhea-positive and diarrhea-negative HUS. The former, also referred to as typical HUS, primarily resulted from 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.

However, the following classification is used based on a better understanding of the various causes of HUS (see "Overview of hemolytic uremic syndrome in children"):

Primary causes without coexisting disease; such as cases due to complement dysregulation (also referred to as atypical HUS) [2]:

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