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

• Published in: Pediatric nephrology (Berlin, West) Vol. 32; no. 7; pp. 1263 - 1268
• Main Authors: Wijnsma, Kioa L., Schijvens, Anne M., Rossen, John W. A., Kooistra-Smid, A. M. D. (Mirjam), Schreuder, Michiel F., van de Kar, Nicole C. A. J.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.07.2017; Springer; Springer Nature B.V
• Subjects: Analysis; Anti-Bacterial Agents - therapeutic use; Antibodies, Monoclonal, Humanized - therapeutic use; Bacterial toxins; Biopsy; Blood Culture; Brief Report; Care and treatment; Causes of; Ceftriaxone - therapeutic use; Central nervous system; Children; Complications; Diagnosis; Dosage and administration; Escherichia coli; Escherichia coli Infections - blood; Escherichia coli Infections - complications; Escherichia coli Infections - drug therapy; Escherichia coli Infections - microbiology; Hemolytic uremic syndrome; Hemolytic-Uremic Syndrome - blood; Hemolytic-Uremic Syndrome - complications; Hemolytic-Uremic Syndrome - drug therapy; Hemolytic-Uremic Syndrome - microbiology; Humans; Infant; Kidneys; Liver - pathology; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Midazolam - therapeutic use; Multiple Organ Failure - blood; Multiple Organ Failure - complications; Multiple Organ Failure - drug therapy; Multiple Organ Failure - microbiology; Nephrology; Pediatrics; Real-Time Polymerase Chain Reaction; Renal failure; Resuscitation; Serotypes; Serotyping; Serotyping - methods; Shiga toxin; 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; Thrombotic microangiopathy; Toxins; Urology; Virulence; Virulence (Microbiology); Serotype O80:H2; Hemolytic uremic syndrome; STEC-HUS
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-017-3642-3

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.