Abstract
Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) is a form of thrombotic microangiopathy secondary to an infection by an enterohemorrhagic E. coli. Historically considered a pediatric disease, its presentation has been described as typical, with bloody diarrhea at the forefront. However, in adults, the clinical presentation is more diverse and makes the early diagnosis hazardous. In this review, we review the epidemiology, most important outbreaks, physiopathology, clinical presentation and prognosis of STEC-HUS, focusing on the differential features between pediatric and adult disease. We show that the clinical presentation of STEC-HUS in adults is far from typical and marked by the prevalence of neurological symptoms and a poorer prognosis. Of note, we highlight knowledge gaps and the need for studies dedicated to adult patients. The differences between pediatric and adult patients have implications for the treatment of this disease, which remains a public health threat and lack a specific treatment.
Highlights
Overview of Thrombotic Microangiopathies in AdultsThrombotic microangiopathy (TMA) syndromes share a common pathologic description of arterial, intra-renal or systemic micro-vascular occlusion, resulting from endothelial aggression accompanied by the formation of platelet aggregates
The reduced pool of nephrons induces hyperfiltration which may elicit the development of focal segmental glomerulosclerosis and hyalinosis, further aggravating the renal prognosis [145,146]. This finding is common to all Acute kidney injury (AKI) regardless of the cause [147], and it is reasonable to assume that the risk of Chronic kidney disease (CKD) after a Shiga toxinproducing Escherichia coli (STEC)-hemolytic uremic syndrome (HUS) episode in adults is not specific to this disease
We recently reported 12 sporadic STEC-HUS adult patients (median age 64 (IQR 50–72) years) amongst 236 Thrombotic Microangiopathies in AdultsThrombotic microangiopathy (TMA) admitted to the intensive care unit (ICU) [6]
Summary
Thrombotic microangiopathy (TMA) syndromes share a common pathologic description of arterial, intra-renal or systemic micro-vascular occlusion, resulting from endothelial aggression accompanied by the formation of platelet aggregates. Major advances in the understanding of the pathophysiological mechanisms underpinning these different forms of TMA over the past thirty years have been a real turning point, permitting comprehensive nosologic delineation Most of all, they have paved the way for targeted therapies, such as eculizumab in atypical HUS [7]. It was eventually traced to an atypical hybrid pathotype O104:H4 strain combining enteroaggregative and enterohemorrhagic virulence and producing an extended spectrum beta-lactamase This outbreak, related to organic fenugreek sprout consumption, mostly affected adults (88%, median age 42 years) and contributed to raised awareness of the risk and the severity of STEC-HUS in adults. We will summarize the current evidence on the epidemiology, pathophysiology, clinical presentation, diagnosis and prognosis of STEC-HUS patients, focusing on the specific features of adult cases and their implication for clinical practice
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