Abstract

BackgroundHemolytic uremic syndrome (HUS) is a multisystemic disease. In a nationwide study, we characterized the incidence, clinical course, and prognosis of HUS caused by Shiga toxin (Stx)–producing Escherichia coli (STEC) strains with emphasis on risk factors, disease severity, and long-term outcome.MethodsThe data on pediatric HUS patients from 2000 to 2016 were collected from the medical records. STEC isolates from fecal cultures of HUS and non-HUS patients were collected from the same time period and characterized by whole genome sequencing analysis.ResultsFifty-eight out of 262 culture-positive cases developed verified (n = 58, 22%) STEC-HUS. Another 29 cases had probable STEC-HUS, the annual incidence of STEC-HUS being 0.5 per 100,000 children. Eleven different serogroups were detected, O157 being the most common (n = 37, 66%). Age under 3 years (OR 2.4), stx2 (OR 9.7), and stx2a (OR 16.6) were found to be risk factors for HUS. Fifty-five patients (63%) needed dialysis. Twenty-nine patients (33%) developed major neurological symptoms. Complete renal recovery was observed in 57 patients after a median 4.0 years of follow-up. Age under 3 years, leukocyte count over 20 × 109/L, and need for dialysis were predictive factors for poor renal outcome.ConclusionsAge under 3 years, stx2, and stx2a were risk factors for HUS in STEC-positive children. However, serogroup or stx types did not predict the renal outcome or major CNS symptoms.

Highlights

  • Hemolytic uremic syndrome (HUS) is a complex, multisystemic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and impaired kidney function, and it is one of the most common causes of acute kidney injury in children

  • Fifty-eight out of the 262 (22%) children with Shiga toxin–producing E.coli (STEC) isolated from the stool had verified STEC-HUS (Table 1)

  • 56 isolates were available for serotyping and determination of stx genotype by Whole genome sequencing (WGS)

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Summary

Introduction

Hemolytic uremic syndrome (HUS) is a complex, multisystemic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and impaired kidney function, and it is one of the most common causes of acute kidney injury in children. It is most often caused, especially among children, by Shiga toxin (Stx)–producing Escherichia coli (STEC). The serotype O157:H7 has been previously recognized as the predominant cause of HUS worldwide [1] This may arise from the diagnostic bias for this serotype. We characterized the incidence, clinical course, and prognosis of HUS caused by Shiga toxin (Stx)–producing Escherichia coli (STEC) strains with emphasis on risk factors, disease severity, and long-term outcome

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