АТИПИЧНЫЙ ГЕМОЛИТИКО-УРЕМИЧЕСКИЙ СИНДРОМ У ДЕТЕЙ: ПРАКТИЧЕСКИЕ АСПЕКТЫ ДИФФЕРЕНЦИАЛЬНОЙ ДИАГНОСТИКИ И ЛЕЧЕНИЕ

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare progressive form of systemic thrombotic microangiopathy (TMA) that develops as a result of uncontrolled activation of the alternative pathway of the complement system. In the case of late diagnosis and inadequate treatment, aHUS has an unfavorable outcome with death rates as high as 25% during the acute phase and up to 50% of cases progressing to end-stage renal disease. Clinically, aHUS is very similar to disseminated intravascular coagulation, other TMAs: HUS associated with enterohemorrhagic E. coli, thrombotic thrombocytopenic purpura, etc. The article presents the sequence and scope of studies for the differential diagnosis of aHUS. Eculizumab and its biosimilars, blocking the terminal complement complex, have changed the future of patients with aHUS, so timely diagnosis and early treatment are crucial in the outcome of the disease.