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


METHODS
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.
Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands. kioa.wijnsma@radboudumc.nl.
Kioa L
KL
Wijnsma
Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands.
Anne M
AM
Schijvens
Department of Medical Microbiology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands.
John W A
JWA
Rossen
Department of Medical Microbiology, Certe Laboratory for Infectious Diseases, PO Box 909, 9700 AX, Groningen, The Netherlands.
A M D Mirjam
AMDM
Kooistra-Smid
Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands.
Michiel F
MF
Schreuder
Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands.
Nicole C A J
NCAJ
van de Kar
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001706:Biopsy; D000071997:Blood Culture; D002443:Ceftriaxone / Q000627:therapeutic use; D004927:Escherichia coli Infections / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000382:microbiology*; D006463:Hemolytic-Uremic Syndrome / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000382:microbiology*; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008874:Midazolam / Q000627:therapeutic use; D009102:Multiple Organ Failure / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000382:microbiology*; D060888:Real-Time Polymerase Chain Reaction; D012151:Resuscitation; D012703:Serotyping / Q000379:methods; D022641:Shiga Toxin 2 / Q000302:isolation & purification / Q000633:toxicity*; D054323:Shiga-Toxigenic Escherichia coli / Q000302:isolation & purification / Q000378:metabolism / Q000472:pathogenicity*; D057049:Thrombotic Microangiopathies / Q000097:blood / Q000150:complications / Q000188:drug therapy / Q000382:microbiology*; D014774:Virulence
D000900:Anti-Bacterial Agents; D061067:Antibodies, Monoclonal, Humanized; D022641:Shiga Toxin 2; D002443:Ceftriaxone; C481642:eculizumab; D008874:Midazolam
Hemolytic uremic syndrome; STEC-HUS; Serotype O80:H2