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Том 25 №4 2023 год - Нефрология и диализ
Клинические рекомендации по ведению взрослых пациентов с атипичным гемолитико-уремическим синдромом
Козловская Н.Л.
Добронравов В.А.
Боброва Л.А.
Демьянова К.А.
Драпкина О.М.
Карунная А.В.
Кирсанова Т.В.
Прокопенко Е.И.
DOI: 10.28996/2618-9801-2023-4-465-492
Аннотация: 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.
Для цитирования: Козловская Н.Л., Добронравов В.А., Боброва Л.А., Демьянова К.А., Драпкина О.М., Карунная А.В., Кирсанова Т.В., Прокопенко Е.И. Клинические рекомендации по ведению взрослых пациентов с атипичным гемолитико-уремическим синдромом. Нефрология и диализ. 2023. 25(4):465-492. doi: 10.28996/2618-9801-2023-4-465-492
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Ключевые слова: атипичный гемолитико-уремический синдром,
система комплемента,
тромботическая микроангиопатия,
молекулярно-генетическое исследование,
хроническая болезнь почек,
экулизумаб,
atypical hemolytic-uremic syndrome,
complement system,
thrombotic microangiopathy,
molecular genetic study,
chronic kidney disease,
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