What are the subtypes of pediatric hemolytic uremic syndrome (HUS)?

Updated: Nov 12, 2018
  • Author: Robert S Gillespie, MD, MPH; Chief Editor: Craig B Langman, MD  more...
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The clinical course of hemolytic-uremic syndrome can vary from subclinical to life threatening. Studies have revealed distinct subgroups of hemolytic-uremic syndrome and have identified several etiologies for the disease. Nomenclature for the various types of hemolytic-uremic syndrome varies throughout the literature. For consistency, this article uses the following set of terms throughout this review:

  • STEC-HUS is used to describe hemolytic-uremic syndrome mediated by Shiga toxin (Stx)–producing Escherichia coli. This is also called classic, typical, Stx, diarrhea-positive, or D+ hemolytic-uremic syndrome.

  • Atypical HUS (aHUS) is used to describe hemolytic-uremic syndrome not mediated by Shiga toxin. This is also called diarrhea-negative, non–diarrhea-associated, or D- hemolytic-uremic syndrome. This disease is usually mediated by abnormalities of the complement system or other heritable factors.

  • Pneumococcal-associated HUS is a subtype of atypical hemolytic-uremic syndrome, mediated by neuraminidase in the presence of infection with Streptococcus pneumoniae. This is also called neuraminidase-associated hemolytic-uremic syndrome.

The distinction is important because the clinical courses, treatments, and prognoses differ for each category. The first reported cases were aHUS; however, STEC-HUS is now much more common. Classification based on the presence of diarrhea can be misleading, as a significant percentage of patients with aHUS may have diarrhea.

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