Hemolytic-uremic syndrome (HUS) is characterized by the triad of hemolytic anemia, thrombocytopenia and acute renal failure. There are different etiologies leading to HUS, with the Shiga toxin-producing Escherichia coli (STEC) HUS and complement mediated atypical HUS (atypical, aHUS) as the most prominent ones. The most prevalent serotype associated with STEC-HUS is O157. Even though, other serotypes are increasingly associated with HUS, also known as the non-O157 STEC. Since 1999 STEC is a mandatory reportable pathogen. However, there is no notification requirement for HUS. Therefore, no incidence rates of STEC-HUS in the Netherlands are known. Consequently, until now incidence rates of HUS and serotypes causing STEC-HUS are only available by estimation. As a result, insufficient data are available to describe the epidemiology of STEC-HUS in the Netherlands. Furthermore, the epidemiology of STEC-HUS varies highly between countries. Hereby underlining the need for adequate epidemiological data on STEC-HUS in the Netherlands.

Discrimination between both types of HUS is challenging due to overlapping clinical presentation. However, differentiation is highly important regarding treatment options. Nowadays the standard treatment of aHUS comprises one of world’s most expensive drugs: eculizumab. Since aHUS is a diagnosis per exclusionem, providing proof of the presence of STEC forms the basis for differentiation between aHUS and STEC-HUS.

To diagnose STEC-HUS remains a clinical conundrum. There are several diagnostic tools available for detecting STEC infection: examination of fecal material for the presence of STEC and/or shiga toxin (Stx) or by the use of serological assays to establish antibodies directed against the lipopolysaccharides (LPS) of different STEC serotypes. Fecal diagnostics, with either feces culture and/or polymerase chain reaction (PCR) to detect Stx encoding genes, is the gold standard of current daily practice. However, our own data, in addition to several other studies show great advantage of combining both serological and fecal diagnostics to detect STEC-HUS.

Therefore, we want to set up a national observational study by creating a national STEC-HUS online web based database, to gain more insight in the epidemiology and diagnostics of STEC-HUS in the Netherlands.

Study design

Observational nation-wide single center study, both prospective and in retrospect.

Study population

All patients diagnosed with STEC-HUS since 2007, in any Dutch university medical center will be included.

Main study parameters/endpoints

A national STEC-HUS online web based database will be set up in Castor. Clinical, biochemical, diagnostic and follow up data will be gathered in this database. After two years the data will be evaluated within the Dutch HUS working group. This way we will gain more insight in the epidemiology of STEC-HUS, the incidence of STEC-HUS, which serotypes of STEC are correlated with STEC-HUS in the Netherlands and the relation with disease severity. Furthermore, with new techniques we hope to improve diagnostics of STEC-HUS.