An outbreak of Shiga toxin-producing Escherichia coli O104:H4 hemolytic uremic syndrome in Germany: presentation and short-term outcome in children

Clin Infect Dis. 2012 Sep;55(6):753-9. doi: 10.1093/cid/cis531. Epub 2012 Jun 5.


Background: In May and June 2011 the largest known outbreak of hemolytic uremic syndrome (HUS) occurred in northern Germany. Because, quite unusually, a large number of adults was affected and the causative Escherichia coli strain, serotype O104:H4, showed an atypical virulence factor pattern, it was speculated that this outbreak was associated with an aggressive course and an unfavorable prognosis also in children.

Methods: Retrospective analysis of medical records of 90 children and comparison to previous outbreak and sporadic case series.

Results: Median age was unusually high (11.5 years) compared with that in historical series. Only 1 patient (1.1%) died in the acute phase. Most patients (67/90 [74%]) received supportive care only. Renal replacement therapy was required in 64 of 90 (71%) of the children. Neurological complications, mainly seizures and altered mental stage, were present in 23 of 90 (26%) patients. Ten patients received plasmapheresis, 6 eculizumab, and 7 a combination of both. After a median follow-up of 4 months, renal function normalized in 85 of 90 (94%) patients, whereas 3 patients had chronic kidney disease stage 3 or 4, and 1 patient (1.1%) still requires dialysis. Complete neurological recovery occurred in 18 of 23 patients. Mild to moderate and major residual neurological changes were present in 3 patients and 1 patient, respectively, although all patients were still improving.

Conclusions: E. coli O104:H4 caused the largest HUS outbreak in children reported in detail to date and most patients received supportive treatment only. Initial morbidity, as well as short-term outcome, due to this pathogen, is comparable to previous pediatric series of Shiga toxin-producing E. coli HUS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease Outbreaks*
  • Female
  • Germany / epidemiology
  • Hemolytic-Uremic Syndrome / epidemiology*
  • Humans
  • Infant
  • Male
  • Renal Replacement Therapy / methods
  • Retrospective Studies
  • Shiga-Toxigenic Escherichia coli / classification*
  • Shiga-Toxigenic Escherichia coli / genetics
  • Shiga-Toxigenic Escherichia coli / isolation & purification*
  • Treatment Outcome