Shiga toxin-producing Escherichia coli hemolytic uremic syndrome

The hemolytic–uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). The major cause of HUS in childhood (>90%) is infection with verocytotoxin (Shiga-like toxin – “Stx”)-producing bacteria, usually enterohemorrhagic Escherichia coli (VTEC/STEC). The infection may be transmitted by the consumption of undercooked meat, pasteurized dairy products, contaminated vegetables, fruits and water, or by contact with STEC diarrhea. After an incubation period of three to eight days, patients commonly develop bloody diarrhea followed in 5–22% by HUS that may be complicated by central nervous system, pancreatic, skeletal, and myocardial involvement. HUS is one of the main causes of AKI in children in Europe. The management of HUS includes the usual treatment of children with AKI. Transfusion with packed red blood cells is needed in case of a severe anemia, while platelet transfusions are limited to the need for a surgical procedure or in active bleeding. Currently, there is no consensus on the use of antibiotic therapy. Treatment with plasma and/or plasma exchange has not been proven beneficial in STEC-HUS. Eculizumab has been used for the treatment of STEC-HUS, but the value of this treatment remains to be determined. The mortality of HUS is reported to be 3–5%. About 12% of patients will progress to end-stage renal failure within four years and about 25% will have long-term complications, including hypertension, proteinuria, renal insufficiency, and insulin-dependent diabetes mellitus. Transplantation can be performed without increased risk for the recurrence of the disease.