A Moderate Decrease in ADAMTS13 Activity Correlates with the Severity of STEC-HUS

Atypical hemolytic uremic syndrome (HUS) develops as a result of damage to the endothelium of microvasculature vessels by Shiga toxin produced by enterohemorrhagic Escherichia coli (STEC-HUS). STEC-HUS remains the leading cause of acute kidney injury (AKI) in children aged 6 months to 5 years. The pathomorphological essence of the disease is the development of thrombotic microangiopathy (TMA). One of the key causes of TMA is an imbalance in the ADAMTS13–von Willebrand factor (vWF)–platelet system. The goal of the work was to clarify the role of a moderate decrease in ADAMTS13 activity in the pathogenesis of STEC-HUS. The activity of ADAMTS13 was determined in 138 children (4 months–14.7 years) in the acute period of STEC-HUS and the features of the course of the disease in these patients were analyzed. The study revealed a decrease in the activity and concentration of ADAMTS13 in 79.8% and 90.6% of patients, respectively. Measurements of von Willebrand factor antigen content and the activity of von Willebrand factor in the blood plasma of part of these patients were carried out. In 48.6% and 34.4% of cases, there was an increase in the antigen concentration and the activity of the Willebrand factor, respectively. Thrombocytopenia was diagnosed in 97.8% of children. We have demonstrated that moderately reduced ADAMTS13 activity correlates with the risk of severe manifestations of STEC-HUS in children; the rate of developing multiple organ failure, cerebral disorders, pulmonary edema, and acute kidney injury with the need for dialysis increases. It is assumed that reduction in ADAMTS13 activity may serve as a predictor of disease severity

Современные подходы к ведению детей с атипичным гемолитико-уремическим синдромом

Experts of the Union of Pediatricians of Russia have developed modern guidelines on management of children with atypical hemolytic uremic syndrome (aHUS). aHUS is ultra-rare (orphan) disease with progressive and relapsing course during the life with potentially life-threatening state. This article covers issues of disease etiology, pathogenesis, and classification. Specific attention is paid to the diagnosis, differential diagnosis, and therapy of aHUS according to the evidence-based approaches.Экспертами Союза педиатров России разработаны актуальные клинические рекомендации по оказанию медицинской помощи детям с атипичным гемолитико-уремическим синдромом (аГУС). аГУС — ультраредкое (орфанное) заболевание прогрессирующего течения, характеризующееся рецидивированием на протяжении всей жизни и представляющее собой потенциально жизнеугрожающее состояние. В статье подробно рассмотрены вопросы этиологии, патогенеза, классификации заболевания. Особое внимание уделено диагностике, дифференциальной диагностике и терапии аГУС, основанным на принципах доказательности