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Background  On 20–21 February 2006, six cases of diarrhoea-associated haemolytic uraemic syndrome (HUS) were reported by paediatricians to the Norwegian Institute of Public Health. We initiated an investigation to identify the etiologic agent and determine the source of the outbreak in order to implement control measures.    Methods  A case was defined as a child with diarrhoea-associated HUS or any person with an infection with the outbreak strain of  E. coli  O103 (defined by the multi-locus variable number tandem repeats analysis (MLVA) profile) both with illness onset after January 1st 2006 in Norway. After initial hypotheses-generating interviews, we performed a case-control study with the first fifteen cases and three controls for each case matched by age, sex and municipality. Suspected food items were sampled, and any  E. coli  O103 strains were typed by MLVA.    Results  Between 20 February and 6 April 2006, 17 cases were identified, of which 10 children developed HUS, including one fatal case. After pilot interviews, a matched case-control study was performed indicating an association between a traditional cured sausage (odds ratio 19.4 (95% CI: 2.4–156)) and STEC infection.  E. coli  O103:H25 identical to the outbreak strain defined by MLVA profile was found in the product and traced back to contaminated mutton.    Conclusion  We report an outbreak caused by a rare STEC variant (O103:H25,  stx  2 -positive). More than half of the diagnosed patients developed HUS, indicating that the causative organism is particularly virulent. Small ruminants continue to be important reservoirs for human-pathogen STEC. Improved slaughtering hygiene and good manufacturing practices for cured sausage products are needed to minimise the possibility of STEC surviving through the entire sausage production process.

Outbreak of haemolytic uraemic syndrome in Norway caused by stx 2-positive Escherichia coliO103:H25 traced to cured mutton sausages