Is Shigatoxin 1 protective for the development of Shigatoxin 2-related hemolytic uremic syndrome in children? Data from the ItalKid-HUS Network

Pediatr Nephrol. 2020 Oct;35(10):1997-2001. doi: 10.1007/s00467-020-04697-y. Epub 2020 Jul 30.

Abstract

Background: Shigatoxin (Stx)-producing Escherichia coli (STEC) are the most common causes of hemolytic uremic syndrome (STEC-HUS). The aim of our study is to compare the risk of developing STEC-HUS in relation to the type of Stx genes (Stx1, Stx2, or both).

Methods: This is a prospective, observational, multicenter study involving 63 pediatric units in Northern Italy (ItalKid-HUS Network). STEC-infected children were identified within a screening program for bloody diarrhea during a 10-year period (2010-2019). Stx genes were detected by reverse dot blot or real-time PCR. After the identification of STEC infection, children were followed until diarrhea complete recovery for the possible development of STEC-HUS.

Results: Of the 214 Stx-positive patients, 34 (15.9%) developed STEC-HUS. The risk of HUS in STEC-infected children with Stx1 (n: 62; 29.0%) and Stx2 (n: 97; 45.3%) was respectively 0% and 23.7%, while in patients carrying both Stx1 and Stx2 (n: 55; 25.7%), the risk was 12.7% (p: 0.001).

Conclusions: Our data confirm that Stx1 is a very rare cause of STEC-HUS and demonstrate that the risk of STEC-HUS halves in the case of Stx1+2-producing Escherichia coli infection compared with infections where Stx2 is present alone. This observation is helpful in assessing the risk of individual STEC-infected patients for the development of HUS and suggests that Stx1, in the presence of Stx2, might exert a protective role possibly by receptor competition.

Keywords: Children; Diarrhea; Hemolytic uremic syndrome; Shiga toxin.

Publication types

  • Comparative Study
  • Multicenter Study
  • Observational Study

MeSH terms

  • Child
  • Child, Preschool
  • Escherichia coli Infections / complications
  • Escherichia coli Infections / microbiology*
  • Female
  • Hemolytic-Uremic Syndrome / epidemiology*
  • Hemolytic-Uremic Syndrome / microbiology
  • Humans
  • Infant
  • Molecular Typing
  • Prospective Studies
  • Protective Factors
  • Risk Assessment
  • Shiga Toxin 1 / genetics
  • Shiga Toxin 1 / isolation & purification
  • Shiga Toxin 1 / toxicity*
  • Shiga Toxin 2 / genetics
  • Shiga Toxin 2 / isolation & purification
  • Shiga Toxin 2 / toxicity*
  • Shiga-Toxigenic Escherichia coli / genetics*
  • Shiga-Toxigenic Escherichia coli / isolation & purification

Substances

  • Shiga Toxin 1
  • Shiga Toxin 2