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ppharm

Педиатрическая фармакология

Pediatric pharmacology

1727-57762500-3089

Издательство «ПедиатрЪ»

10.15690/pf.v19i2.2400

ppharm-2147

Research Article

КЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИ

CLINICAL RECOMMENDATIONS

Современные подходы к ведению детей с атипичным гемолитико-уремическим синдромом

Modern Approaches to the Management of Children with Atypical Hemolytic Uremic Syndrome

https://orcid.org/0000-0002-7523-5889

Эмирова

Х. М.

Emirova

Khadizha M.

Москва.

Moscow.

https://orcid.org/0000-0002-7175-5633

Абасеева

Т. Ю.

Abaseeva

Tatiana Yu.

Москва.

Moscow.

https://orcid.org/0000-0003-3987-8112

Баранов

А. А.

Baranov

Alexander A.

Москва.

Moscow.

https://orcid.org/0000-0001-8320-2027

Вашакмадзе

Н. Д.

Vashakmadze

Nato D.

Москва.

Moscow.

https://orcid.org/0000-0001-7398-0562

Вишнева

Е. А.

Vishneva

Elena A.

Москва.

Moscow.

https://orcid.org/0000-0002-0184-4163

Генералова

Г. А.

Generalova

Galina A.

Москва.

Moscow.

https://orcid.org/0000-0002-5020-1180

Захарова

Е. Ю.

Zakharova

Ekaterina Yu.

Москва.

Moscow.

https://orcid.org/0000-0003-1453-4671

Калюжная

Т. А.

Kaluzhnaya

Tatiana A.

Москва.

Moscow.

https://orcid.org/0000-0002-3133-8018

Куцев

С. И.

Kutsev

Sergey I.

Москва.

Moscow.

https://orcid.org/0000-0002-2209-7531

Намазова-Баранова

Л. С.

Namazova-Baranova

Leyla S.

Намазова-Баранова Лейла Сеймуровна - доктор медицинских наук, профессор, академик РАН, заведующая кафедрой факультетской педиатрии педиатрического факультета РНИМУ им. Н.И. Пирогова Минздрава России; руководитель НИИ педиатрии и охраны здоровья детей НКЦ №2 РНЦХ им. акад. Б.В. Петровского; президент Союза педиатров России.

119333, Москва, ул. Фотиевой, д. 10.

тел.: +7 (499) 400-47-33

Moscow; Belgorod.

lsnamazova@yandex.ru

https://orcid.org/0000-0002-5722-8490

Макарова

Т. П.

Makarova

Tamara P.

Казань.

Kazan.

https://orcid.org/0000-0002-2395-1322

Маргиева

Т. В.

Margieva

Tea V.

Москва.

Moscow.

Мстиславская

С. А.

Mstislavskaya

Sofya A.

Москва.

Moscow.

https://orcid.org/0000-0003-4131-9440

Музуров

А. Л.

Muzurov

Aleksandr L.

Москва.

Moscow.

https://orcid.org/0000-0002-1791-7486

Панкратенко

Т. Е.

Pankratenko

Tatiana E.

Москва.

Moscow.

https://orcid.org/0000-0002-6921-4483

Шилова

М. М.

Shilova

Marina M.

Москва.

Moscow.

https://orcid.org/0000-0003-0797-5612

Федосеенко

М. В.

Fedoseenko

Marina V.

Москва.

Moscow.

Московский государственный медико-стоматологический университет им. А.И. Евдокимова; Центр гравитационной хирургии крови и гемодиализа ГБУЗ «Детская городская клиническая больница св. Владимира ДЗМ»РоссияA.I. Yevdokimov Moscow State University of Medicine and Dentistry; Center of Gravitational Blood Surgery and Hemodialysis of Saint Vladimir Children’s Clinical HospitalRussian Federation

НИИ педиатрии и охраны здоровья детей НКЦ №2 РНЦХ им. акад. Б.В. Петровского; Первый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет)РоссияResearch Institute of Pediatrics and Children's Health in Petrovsky National Research Centre of Surgery; Sechenov First Moscow State Medical UniversityRussian Federation

НИИ педиатрии и охраны здоровья детей НКЦ №2 РНЦХ им. акад. Б.В. Петровского; Российский национальный исследовательский медицинский университет им. Н.И. ПироговаРоссияResearch Institute of Pediatrics and Children's Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical UniversityRussian Federation

Центр гравитационной хирургии крови и гемодиализа ГБУЗ «Детская городская клиническая больница св. Владимира ДЗМ»; Российская медицинская академия непрерывного последипломного образования МЗ РФРоссияCenter of Gravitational Blood Surgery and Hemodialysis of Saint Vladimir Children’s Clinical Hospital; Russian Medical Academy of Continuing Professional EducationRussian Federation

Медико-генетический научный центр им. академика Н.П. БочковаРоссияResearch Centre for Medical GeneticsRussian Federation

НИИ педиатрии и охраны здоровья детей НКЦ №2 РНЦХ им. акад. Б.В. Петровского; Российский национальный исследовательский медицинский университет им. Н.И. Пирогова; Белгородский государственный национальный исследовательский университетРоссияResearch Institute of Pediatrics and Children's Health in Petrovsky National Research Centre of Surgery; Pirogov Russian National Research Medical University; Belgorod National Research UniversityRussian Federation

Казанский Государственный Медицинский УниверситетРоссияKazan State Medical UniversityRussian Federation

Первый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет)РоссияSechenov First Moscow State Medical UniversityRussian Federation

Центр гравитационной хирургии крови и гемодиализа ГБУЗ «Детская городская клиническая больница св. Владимира ДЗМ»РоссияCenter of Gravitational Blood Surgery and Hemodialysis of Saint Vladimir Children’s Clinical HospitalRussian Federation

Российский национальный исследовательский медицинский университет им. Н.И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian Federation

2022

21042022

192127152

Copyright © Эмирова Х.М., Абасеева Т.Ю., Баранов А.А., Вашакмадзе Н.Д., Вишнева Е.А., Генералова Г.А., Захарова Е.Ю., Калюжная Т.А., Куцев С.И., Намазова-Баранова Л.С., Макарова Т.П., Маргиева Т.В., Мстиславская С.А., Музуров А.Л., Панкратенко Т.Е., Шилова М.М., Федосеенко М.В., 2022

2022

Эмирова Х.М., Абасеева Т.Ю., Баранов А.А., Вашакмадзе Н.Д., Вишнева Е.А., Генералова Г.А., Захарова Е.Ю., Калюжная Т.А., Куцев С.И., Намазова-Баранова Л.С., Макарова Т.П., Маргиева Т.В., Мстиславская С.А., Музуров А.Л., Панкратенко Т.Е., Шилова М.М., Федосеенко М.В.

Emirova K.M., Abaseeva T.Y., Baranov A.A., Vashakmadze N.D., Vishneva E.A., Generalova G.A., Zakharova E.Y., Kaluzhnaya T.A., Kutsev S.I., Namazova-Baranova L.S., Makarova T.P., Margieva T.V., Mstislavskaya S.A., Muzurov A.L., Pankratenko T.E., Shilova M.M., Fedoseenko M.V.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.pedpharma.ru/jour/article/view/2147

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.

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.

гемолитико-уремический синдроматипичный гемолитико-уремический синдромхроническая болезнь почекдети

hemolytic uremic syndromeatypical hemolytic uremic syndromechronic kidney diseasechildren

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