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Clinical manifestations and diagnosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) in children

Patrick Niaudet, MD
Section Editors
Tej K Mattoo, MD, DCH, FRCP
Sheldon L Kaplan, MD
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 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 clinical manifestations and diagnosis of STEC HUS in children are presented in this topic review. The treatment and prognosis of this disorder and other causes of HUS are presented separately. (See "Treatment and prognosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) in children" and "Overview of hemolytic uremic syndrome in children" and "Complement-mediated hemolytic uremic syndrome".)


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.

The following classification is now preferred, 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: Jan 24, 2017.
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