Shiga Toxin-Producing Escherichia Coli Associated Hemolytic Uremic Syndrome

Abstract

Shiga toxin-producing Escherichia coli (STEC)-associated hemolytic uremic syndrome (STEC-HUS) is a clinical syndrome involving hemolytic anemia (with fragmented red blood cells), low levels of platelets in the blood (thrombocytopenia), and acute kidney injury (AKI). It is the major infectious cause of AKI in children. Severe forms can be associated with multiorgan involvement during the acute stage of the disease. Endothelial injury is the trigger event in the microangiopathic process. The host inflammatory response to toxin and E. coli lipopolysaccharide (LPS) is involved in disease pathophysiology. Early diagnosis and identification of underlying pathogenic mechanisms are of great significance for improving prognosis and reducing sequelae and mortality. Typical management of STEC-HUS patients relies on supportive care for electrolyte and water imbalance, anemia, hypertension, and renal failure. At present there is no specific therapy to ameliorate the prognosis. The Immediate outcome is most often favorable but long-term renal sequelae are frequent due to nephron loss. This review summarizes current knowledge regarding the epidemiological findings, the pathophysiological and clinical aspects, and its diagnosis and management.