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

Kioa L Wijnsma; Anne M Schijvens; John W A Rossen; A M D Mirjam Kooistra-Smid; Michiel F Schreuder; Nicole C A J van de Kar

doi:10.1007/s00467-017-3642-3

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

Pediatr Nephrol. 2017 Jul;32(7):1263-1268. doi: 10.1007/s00467-017-3642-3. Epub 2017 Mar 25.

Authors

Kioa L Wijnsma¹, Anne M Schijvens², John W A Rossen³, A M D Mirjam Kooistra-Smid⁴, Michiel F Schreuder², Nicole C A J van de Kar²

Affiliations

¹ Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands. [email protected].
² Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands.
³ Department of Medical Microbiology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands.
⁴ Department of Medical Microbiology, Certe Laboratory for Infectious Diseases, PO Box 909, 9700 AX, Groningen, The Netherlands.

Abstract

Background: Hemolytic 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/treatment: In 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.

Conclusions: This 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.

Keywords: Hemolytic uremic syndrome; STEC-HUS; Serotype O80:H2.

Publication types

Case Reports

MeSH terms

Anti-Bacterial Agents / therapeutic use
Antibodies, Monoclonal, Humanized / therapeutic use
Biopsy
Blood Culture
Ceftriaxone / therapeutic use
Escherichia coli Infections / blood
Escherichia coli Infections / complications
Escherichia coli Infections / drug therapy
Escherichia coli Infections / microbiology*
Hemolytic-Uremic Syndrome / blood
Hemolytic-Uremic Syndrome / complications
Hemolytic-Uremic Syndrome / drug therapy
Hemolytic-Uremic Syndrome / microbiology*
Humans
Infant
Liver / pathology
Magnetic Resonance Imaging
Male
Midazolam / therapeutic use
Multiple Organ Failure / blood
Multiple Organ Failure / complications
Multiple Organ Failure / drug therapy
Multiple Organ Failure / microbiology*
Real-Time Polymerase Chain Reaction
Resuscitation
Serotyping / methods
Shiga Toxin 2 / isolation & purification
Shiga Toxin 2 / toxicity*
Shiga-Toxigenic Escherichia coli / isolation & purification
Shiga-Toxigenic Escherichia coli / metabolism
Shiga-Toxigenic Escherichia coli / pathogenicity*
Thrombotic Microangiopathies / blood
Thrombotic Microangiopathies / complications
Thrombotic Microangiopathies / drug therapy
Thrombotic Microangiopathies / microbiology*
Virulence

Substances

Anti-Bacterial Agents
Antibodies, Monoclonal, Humanized
Shiga Toxin 2
Ceftriaxone
eculizumab
Midazolam