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

Kioa L Wijnsma,Michiel F Schreuder,Nicole C A J Van De Kar,John W A Rossen,Anne M Schijvens,A M D Kooistra-Smid

doi:10.1007/s00467-017-3642-3

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.

Highlights

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children and is diagnosed when the features of hemolytic anemia and thrombocytopenia are present simultaneously [1]
This report underlines the importance of Shiga toxinproducing Escherichia coli (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
In over 90% of pediatric cases, HUS follows a gastro-intestinal infection with Shiga toxinproducing Escherichia coli (STEC), previously known by the name of post-diarrheal or typical HUS, because of its main characteristic of diarrhea [1]

Summary

Introduction

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children and is diagnosed when the features of hemolytic anemia and thrombocytopenia are present simultaneously [1]. A previously healthy 16-month-old boy presented at the emergency department of a general hospital with convulsions and impaired consciousness. He had a 2-day history of coughing, vomiting, and lethargy, without signs of fever. A detailed medical history of the patient and family revealed no ingestion of unpasteurized milk or cheese nor a visit to a foreign country On physical examination he exhibited fluctuating levels of alertness. Minimal signs of neurological improvement were observed and over time the patient developed spastic dystonia He exhibited persistently decreased levels of consciousness with frequent periods of severe agitation. Nine months after initial presentation, the patient died due to peritoneal and hemodialysis failure combined with progressive liver failure and further neurological impairment

Discussion

Findings

Compliance with ethical standards