nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное общество10.28996/2618-9801-2023-4-465-492nid-127Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL GUIDELINESКлинические рекомендации по ведению взрослых пациентов с атипичным гемолитико-уремическим синдромомClinical guidelines for the management of adult patients with atypical hemolytic-uremic syndromeКозловскаяН. Л.KozlovskayaN. L.nkozlovskaya@yandex.ruДобронравовВ. А.DobronravovV. A.noemail@neicon.ruБоброваЛ. А.BobrovaL. A.noemail@neicon.ruДемьяноваК. А.DemyanovaK. A.noemail@neicon.ruДрапкинаО. М.DrapkinaO. M.noemail@neicon.ruКаруннаяА. В.KarunnayaA. V.noemail@neicon.ruКирсановаТ. В.KirsanovaT. V.noemail@neicon.ruПрокопенкоЕ. И.ProkopenkoE. I.noemail@neicon.ruГБУЗ ГКБ им. А.К. Ерамишанцева ДЗМ; ФГАОУ ВО Российский университет дружбы народовРоссияA.K. Eramishantsev City Clinical Hospital; RUDN University of RussiaRussian FederationПервый Санкт-Петербургский государственный медицинский университета им. акад. И.П. ПавловаРоссияAcademician I.P. Pavlov First St Petersburg State Medical UniversityRussian FederationПервый МГМУ им. И.М. Сеченова Минздрава России (Сеченовский Университет)РоссияI.M. Sechenov First Moscow State Medical University (Sechenov University)Russian FederationФГБУ Национальный медицинский исследовательский центр терапии и профилактической медицины Минздрава РоссииРоссияNational Medical Research Center for Therapy and Preventive Medicine of the Russian Ministry of HealthRussian FederationНациональный медицинский исследовательский центр акушерства, гинекологии и перинатологии имени академика В.И. КулаковаРоссияNational Medical Research Center for Obstetrics, Gynecology and PerinatologyRussian FederationГБУЗ МО Московский областной научно-исследовательский клинический институт имени М.Ф. ВладимирскогоРоссияM.F. Vladimirsky Moscow Regional Research Clinical InstituteRussian Federation202321062024254465492Copyright © Козловская Н.Л., Добронравов В.А., Боброва Л.А., Демьянова К.А., Драпкина О.М., Карунная А.В., Кирсанова Т.В., Прокопенко Е.И., 20242024Козловская Н.Л., Добронравов В.А., Боброва Л.А., Демьянова К.А., Драпкина О.М., Карунная А.В., Кирсанова Т.В., Прокопенко Е.И.Kozlovskaya N.L., Dobronravov V.A., Bobrova L.A., Demyanova K.A., Drapkina O.M., Karunnaya A.V., Kirsanova T.V., Prokopenko E.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/127

Введение. Атипичный гемолитико-уремический синдром (аГУС) - системное заболевание из группы тромботических микроангиопатий с прогрессирующим течением и неблагоприятным прогнозом, в основе которого лежит неконтролируемая активация альтернативного пути комплемента наследственной или приобретённой природы, приводящая к генерализованному тромбообразованию в сосудах микроциркуляторного русла. Цель - предоставить актуальные клинические рекомендации по оказанию медицинской помощи взрослым пациентам с аГУС. Основные сведения. Экспертами Национальной ассоциации нефрологов разработаны актуальные клинические рекомендации по оказанию медицинской помощи взрослым пациентам с аГУС. В рекомендациях подробно рассмотрены вопросы этиологии, патогенеза, эпидемиологии и клинических проявлений заболевания. Особое внимание уделено диагностике, дифференциальной диагностике и лечению аГУС, основанным на принципах доказательной медицины.

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.

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The authors declare that there are no conflicts of interest present.