Original Paper

European Journal of Pediatrics

, Volume 169, Issue 5, pp 591-598

Hemolytic-uremic syndrome in Switzerland: a nationwide surveillance 1997–2003

  • Alexandra SchifferliAffiliated withUniversity Children’s Hospital UKBB
  • , Rodo O. von VigierAffiliated withUniversity Children’s Hospital
  • , Matteo FontanaAffiliated withUniversity Children’s Hospital UKBB
  • , Giuseppina SpartàAffiliated withUniversity Children’s Hospital
  • , Hans SchmidAffiliated withFederal Office of Public Health (FOPH)
  • , Mario G. BianchettiAffiliated withDivision of Pediatrics, Ospedale San Giovanni
  • , Christoph RudinAffiliated withUniversity Children’s Hospital UKBB Email author 
  • , The Swiss Pediatric Surveillance Unit (SPSU)

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Abstract

Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in childhood. In its typical presentation, it is preceded by an episode of diarrhea mostly due to Shiga-toxin-producing Escherichia coli. There is important geographical variation of many aspects of this syndrome. Nationwide data on childhood HUS in Switzerland have not been available so far. In a prospective national study through the Swiss Pediatric Surveillance Unit 114 cases (median age 21 months, 50% boys) were reported between April 1997 and March 2003 by 38 pediatric units (annual incidence 1.42 per 105 children ≤16 years). Shiga-toxin-producing E. coli were isolated in 32 (60%) of tested stool samples, serotype O157:H7 in eight. Sixteen children presented with only minimal renal involvement, including three with underlying urinary tract infection. Six patients presented with atypical hemolytic-uremic syndrome, and six with HUS due to invasive Streptococcus pneumoniae infection. Mortality was 5.3%, including two out of six children with S. pneumoniae infection. The severity of thrombocytopenia and the presence of central nervous system involvement significantly correlated with mortality. In conclusion, childhood HUS is not rare in Switzerland. Contrasting other countries, E. coli O157:H7 play only a minor role in the etiology. Incomplete manifestation is not uncommon.

Keywords

Hemolytic-uremic syndrome Children Enterohemorrhagic E. coli (EHEC) Shiga toxin Renal failure Clinical spectrum