Clinical aspects of a nationwide epidemic of severe haemolytic uremic syndrome (HUS) in children

Lars Krogvold,Thore Henrichsen,Damien Brackman,Gaute Syversen,Hans Bangstad,Helga Gudmundsdottir,Pål Næss,Anna Bjerre,Henrik Dollner

doi:10.1186/1757-7241-19-44

Abstract

BackgroundReport a nationwide epidemic of Shiga toxin-producing E. coli (STEC) O103:H25 causing hemolytic uremic syndrome (D+HUS) in children.MethodsDescription of clinical presentation, complications and outcome in a nationwide outbreak.ResultsTen children (median age 4.3 years) developed HUS during the outbreak. One of these was presumed to be a part of the outbreak without microbiological proof. Eight of the patients were oligoanuric and in need of dialysis. Median need for dialysis was 15 days; one girl did not regain renal function and received a kidney transplant. Four patients had seizures and/or reduced consciousness. Cerebral oedema and herniation caused the death of a 4-year-old boy. Two patients developed necrosis of colon with perforation and one of them developed non-autoimmune diabetes.ConclusionThis outbreak of STEC was characterized by a high incidence of HUS among the infected children, and many developed severe renal disease and extrarenal complications. A likely explanation is that the O103:H25 (eae and stx2-positive) strain was highly pathogen, and we suggest that this serotype should be looked for in patients with HUS caused by STEC, especially in severe forms or outbreaks.

Highlights

Report a nationwide epidemic of Shiga toxin-producing E. coli (STEC) O103:H25 causing hemolytic uremic syndrome (D+Haemolytic uremic syndrome (HUS)) in children
More than 90% of the cases are due to Shiga toxin-producing E. coli (STEC) infections; termed typical HUS or diarrhoea associated HUS (D+HUS)
Sixteen children were infected by E. coli O103:H25 during the outbreak

Summary

Introduction

Report a nationwide epidemic of Shiga toxin-producing E. coli (STEC) O103:H25 causing hemolytic uremic syndrome (D+HUS) in children. Haemolytic uremic syndrome (HUS) is a severe, acute and dramatic disease affecting previously healthy children. HUS is defined as a triad of acute kidney injury, microangiopatic haemolytic anaemia and thrombocytopenia in patients with no other explanation for coagulopathy [1] e.g. thrombotic thrombocytopenic purpura. More than 90% of the cases are due to Shiga toxin-producing E. coli (STEC) infections; termed typical HUS or diarrhoea associated HUS (D+HUS). Many different serotypes can cause HUS, the most prevalent in Europe and USA being O157:H7 [2,3]. A broad spectrum of extrarenal complications may occur in HUS, the most common are gastrointestinal and cerebral. Extrarenal involvement at an early stage is associated with increased morbidity

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