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Complement-mediated hemolytic uremic syndrome

Author
Patrick Niaudet, MD
Section Editor
Tej K Mattoo, MD, DCH, FRCP
Deputy Editor
Melanie S Kim, MD

INTRODUCTION

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). Research over the last 20 years has shown that complement dysregulation accounts for most of the non-STEC cases of HUS. This discovery has had a major impact on identifying the underlying cause of familial HUS, and on the management of these patients, who historically have had a poor prognosis.

The clinical manifestation, diagnosis, and management of complement-mediated HUS will be reviewed here. An overview of HUS and topics on the clinical manifestations, diagnosis, and management of STEC-HUS are found separately. (See "Overview of hemolytic uremic syndrome in children" and "Clinical manifestations and diagnosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) in children" and "Treatment and prognosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) in children".)

CLASSIFICATION

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 E. 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-HUS also presented with diarrhea.

The classification system used to describe the different etiologies of HUS has evolved as ongoing research has provided a better understanding of the underlying causes of HUS.

Currently, HUS is divided into [2]:

                                     

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Literature review current through: Nov 2016. | This topic last updated: Tue Sep 27 00:00:00 GMT+00:00 2016.
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