Background <p>Shiga toxin-producing <i>Escherichia coli</i> hemolytic and uremic syndrome (STEC-HUS) is predominantly a pediatric disease, but adult presentations can be highly atypical and severe. This case-based review addresses the clinical question of how to diagnose and manage adult STEC-HUS when classic peripheral thrombotic microangiopathy (TMA) markers are absent, evaluating its unique clinical features, diagnostic pitfalls, and outcomes.</p> Methods <p>We describe an atypical, life-threatening case of adult STEC-HUS managed in our intensive care unit (ICU). To contextualize this case, we performed a narrative review of the literature using data sources from international surveillance networks, the French national registry, and major adult outbreak cohorts. We appraised studies focusing on adult clinical characteristics, the diagnostic yield of non-enteric samples, antibiotic implications, and long-term renal outcomes.</p> Results <p>A 47-year-old male developed severe anuric acute kidney injury (AKI) and coma following deer pâté ingestion. Notably, classic peripheral TMA hallmarks were absent, and stool testing was negative. Diagnosis was established via urine culture and PCR, which identified an enterohemorrhagic E. coli O171 strain producing Stx1 and Stx2. A renal biopsy diagnosed cortical necrosis with glomerular capillary thrombosis. With aggressive ICU supportive care, the patient achieved complete neurological recovery and ceased hemodialysis after one month. Literature assessment corroborated that adult STEC-HUS exhibits high heterogeneity, frequently presenting as an “organ-failure first” phenotype. Furthermore, up to 7% of adult cases are positive only via urine samples, and robust supportive care can lead to unexpected, favorable renal recovery despite cortical necrosis.</p> Conclusions <p>Adult STEC-HUS can present with severe multi-organ failure and renal cortical necrosis entirely masking as non-TMA, representing a dangerous diagnostic trap. Clinicians should implement systematic STEC PCR screening on alternative biological samples like urine for unexplained severe AKI. Finally, aggressive intensive care support is strongly indicated, as it can achieve excellent functional and renal recovery despite traditional poor prognostic indicators.</p>

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Atypical presentation of Shiga toxin-producing Escherichia coli hemolytic and uremic syndrome (STEC-HUS): case-based discussion and literature review

  • Baptiste Hirsinger,
  • Clément Deltombe,
  • Emmanuel Canet

摘要

Background

Shiga toxin-producing Escherichia coli hemolytic and uremic syndrome (STEC-HUS) is predominantly a pediatric disease, but adult presentations can be highly atypical and severe. This case-based review addresses the clinical question of how to diagnose and manage adult STEC-HUS when classic peripheral thrombotic microangiopathy (TMA) markers are absent, evaluating its unique clinical features, diagnostic pitfalls, and outcomes.

Methods

We describe an atypical, life-threatening case of adult STEC-HUS managed in our intensive care unit (ICU). To contextualize this case, we performed a narrative review of the literature using data sources from international surveillance networks, the French national registry, and major adult outbreak cohorts. We appraised studies focusing on adult clinical characteristics, the diagnostic yield of non-enteric samples, antibiotic implications, and long-term renal outcomes.

Results

A 47-year-old male developed severe anuric acute kidney injury (AKI) and coma following deer pâté ingestion. Notably, classic peripheral TMA hallmarks were absent, and stool testing was negative. Diagnosis was established via urine culture and PCR, which identified an enterohemorrhagic E. coli O171 strain producing Stx1 and Stx2. A renal biopsy diagnosed cortical necrosis with glomerular capillary thrombosis. With aggressive ICU supportive care, the patient achieved complete neurological recovery and ceased hemodialysis after one month. Literature assessment corroborated that adult STEC-HUS exhibits high heterogeneity, frequently presenting as an “organ-failure first” phenotype. Furthermore, up to 7% of adult cases are positive only via urine samples, and robust supportive care can lead to unexpected, favorable renal recovery despite cortical necrosis.

Conclusions

Adult STEC-HUS can present with severe multi-organ failure and renal cortical necrosis entirely masking as non-TMA, representing a dangerous diagnostic trap. Clinicians should implement systematic STEC PCR screening on alternative biological samples like urine for unexplained severe AKI. Finally, aggressive intensive care support is strongly indicated, as it can achieve excellent functional and renal recovery despite traditional poor prognostic indicators.