Unusual severe case of hemolytic uremic syndrome due to Shiga toxin 2d-producing E. coli O80:H2

Abstract

BackgroundHemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children, with the majority of cases caused by an infection with Shiga toxin-producing Escherichia coli (STEC). Whereas O157 is still the predominant STEC serotype, non-O157 serotypes are increasingly associated with STEC-HUS. However, little is known about this emerging and highly diverse group of non-O157 serotypes. With supportive therapy, STEC-HUS is often self-limiting, with occurrence of chronic sequelae in just a small proportion of patients.Case diagnosis/treatmentIn this case report, we describe a 16-month-old boy with a highly severe and atypical presentation of STEC-HUS. Despite the presentation with multi-organ failure and extensive involvement of central nervous system due to extensive thrombotic microangiopathy (suggestive of atypical HUS), fecal diagnostics revealed an infection with the rare serotype: shiga toxin 2d-producing STEC O80:H2.ConclusionsThis report underlines the importance of STEC diagnostic tests in all children with HUS, including those with an atypical presentation, and emphasizes the importance of molecular and serotyping assays to estimate the virulence of an STEC strain.

Characteristics of Shiga toxin producing- and enteropathogenic Escherichia coli of the emerging serotype O80:H2 isolated from humans and diarrhoeic calves in Belgium

“…Human STEC O80:H2 infections are uncommon in Belgium (only nine isolates since 2008, without increasing frequency) and their clinical outcome is mostly mild, in contrast to the severe French and Dutch cases [2,3]. No STEC O80:H2 were identified in healthy cattle at slaughterhouses in 2014 and only two stx1a-positive STEC strains were found in one diarrhoeic calf in Belgium going back to 1987 [11].…”

Shiga Toxin-Associated Hemolytic Uremic Syndrome: A Narrative Review

“…In northern America and Europe, non-O157 serogroups are increasingly associated with post-STEC-HUS and since the 2010s have even exceeded the number of O157:H7 infections [4,41,43]. Moreover, an unusual serogroup, O80, is currently emerging in France [44][45][46] and Europe [47][48][49]. Several epidemiological and clinical features differentiate O157 and non-O157 serogroups.…”

Emerging Multidrug-Resistant Hybrid Pathotype Shiga Toxin–Producing Escherichia coli O80 and Related Strains of Clonal Complex 165, Europe

“…In 2016, O80 represented the second most frequent serogroup isolated in France, after serogroup O26 ( 4 ). This phenomenon is no longer restricted to France; strains of serotype O80:H2, all belonging to sequence type (ST) 301, have been identified in Spain ( 5 ), the Netherlands ( 6 ), and Switzerland ( 7 ).…”

“…Furthermore, unusual extraintestinal infections have recently been described for this serogroup ( 9 , 10 ), such as bacteremia, whereas EHEC is generally known to be a strictly intestinal pathogen. A recent case in the Netherlands illustrates the potential extreme pathogenicity of this serogroup; a 16-month-old boy died from multiorgan failure and extensive cerebral thrombotic microangiopathy attributable to an O80 Stx2d-producing E. coli strain ( 6 ). Finally, O80 EHEC appears to be a hybrid pathotype that combines intestinal VFs (Shiga toxin [ stx ], intimin [ eae ], enterohemolysin [ ehxA ]) and extraintestinal VFs (aerobactin [ iucC ], salmochelin [ iroN ], an iron uptake protein encoded by sitABCD ; serum resistance protein [ iss p ], a putative secretion system I [ etsC ], omptin [ ompTp ], hemolysin [ hlyF ], and 2 bacteriocins [ cia and cva ]), suggesting the presence of a pS88-like plasmid ( 11 ).…”