Clinical guidelines for the management of adult patients with atypical hemolytic-uremic syndrome; [Клинические рекомендации по ведению взрослых пациентов с атипичным гемолитико-уремическим синдромом]

Introduction. Atypical hemolytic-uremic syndrome (aHUS) is a systemic disease from the group of thrombotic microangiopathies with a progressive course and unfavorable prognosis, which is based on the uncontrolled activation of the alternative complement pathway of hereditary or acquired nature, leading to generalized thrombosis in the microvasculature. The goal is to provide current clinical guidelines for the care of adult patients with aHUS. Basic information. Experts from the National Association of Nephrologists have developed current clinical guidelines for the provision of medical care to adult patients with aHUS. The recommendations discuss in detail the issues of etiology, pathogenesis, epidemiology and clinical manifestations of the disease. Particular attention is paid to the diagnosis, differential diagnosis and treatment of aHUS, based on the principles of evidence-based medicine. © 2023 JSC Vidal Rus. All rights reserved.