Abstract
Being one of the main causes of renal failure in children, hemolytic uremic syndrome (HUS) is a significant therapeutic challenge for pediatricians. Limited epidemiological data on the incidence and prevalence of HUS in the pediatric population suggests the need for studies in this area. Objective. To assess specific clinical characteristics of HUS in children residing in Tyumen region, analyze diagnostic approach used in this region, and identify the main effective therapeutic regimens. Materials and methods. We performed retrospective analysis of 33 HUS cases. In the majority of them, symptoms of acute renal failure were preceded by an episode of diarrhea with hemocolitis, while their disease progression manifested with extrarenal complications involving the cardiovascular system, nervous systems, and gastrointestinal tract. Laboratory testing demonstrated signs typical of thrombotic microangiopathy, which is considered as an underlying pathological mechanism of HUS: Coombs-negative hemolytic anemia, thrombocytopenia. Acute kidney damage was confirmed by hyperazotemia and proteinuria. Differential diagnosis of typical and atypical HUS was based on the measurement of ADAMTS-13 level and testing for Shiga toxin. Results. Instrumental examinations confirmed renal failure and extrarenal complications. In the majority of patients, first-line therapy included renal replacement therapy, plasma transfusion, and hemotransfusion. Patients with verified atypical HUS received the complement inhibitor Eculizumab. Since new diagnostic tests are not available everywhere, it is necessary to consider an organizational decision regarding testing according to indications activity ADAMTS-13 and the administration of complement-inhibiting drugs as first-line therapy for atypical HUS. Key words: hemolytic uremic syndrome, dialysis, monoclonal antibodies, renal failure, ADAMTS-13
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