STEC:O111-HUS complicated by acute encephalopathy in a young girl was successfully treated with a set of hemodiafiltration, steroid pulse, and soluble thrombomodulin under plasma exchange

Hemolytic uremic syndrome (HUS) is a life-threatening disease, characterized by microangiopathic hemolytic anemia, destructive thrombocytopenia, and renal failure 1. Most HUS occurs in association with Shiga toxin-producing Escherichia coli (STEC) infection 2. Patients with STEC-HUS generally recover with fluid therapy and hemodialysis. Mortality is high among STEC-HUS patients with encephalopathy, despite treatments including plasma exchange, steroid pulse, and more recently eculizumab 3. In recent STEC outbreaks in the United States (STEC-O111) and Germany (STEC-O104) in 2008 and 2011, respectively 4,5, STEC-HUS incidence and mortality were 16.7% and 3.8% and 22% and 3.7%, respectively. In 2011, an outbreak of STEC-O111 and/or -O157 infection in Toyama, Japan occurred following raw meat ingestion in a barbecue restaurant chain. Overall, 181 patients were infected, of whom 34 developed STEC-HUS (18.8%) including 21 with encephalopathy (61.8%) and five deaths (14.7%; all with encephalopathy) 6–8. Ten STEC-HUS patients were aged 1–14 years, including eight with encephalopathy 7. Seven children including five with encephalopathy recovered and three died 7. We report clinical and laboratory findings for a 14-year-old girl in the Toyama series with STEC-HUS and encephalopathy.