References

nefr

Нефрология

Nephrology (Saint-Petersburg)

1561-62742541-9439

Pavlov First Saint-Petersburg State Medical University

10.24884/1561-6274-2018-22-4-18-39

nefr-489

Research Article

ОБЗОРЫ И ЛЕКЦИИ

REVIEWS AND LECTURES

АТИПИЧНЫЙ ГЕМОЛИТИКО-УРЕМИЧЕСКИЙ СИНДРОМ И С3-ГЛОМЕРУЛОПАТИЯ: ИТОГИ КОНФЕРЕНЦИИ «KIDNEY DISEASE: IMPROVING GLOBAL OUTCOMES» (KDIGO) ПО СПОРНЫМ ВОПРОСАМ

ATYPICAL HEMOLYTIC UREMIC SYNDROME AND C3 GLOMERULOPATHY: CONCLUSIONS FROM A «KIDNEY DISEASE: IMPROVING GLOBAL OUTCOMES» (KDIGO) CONTROVERSIES CONFERENCE

Г. Дж. Гудшип

Тимоти

H.J. Goodship

Timothy

Центал Парквэй, Ньюкасл-на-Тайне, NE1 3BZ, Великобритания

tim.goodship@ncl.ac.uk

Кук

Г. Теренс

Cook

H. Terence

Центр изучения комплемента и воспаления, Отделение медицины

Department of Medicine

Фахури

Фади

Fakhouri

Fadi

INSERM, UMR-S 1064 (Центр исследований в трансплантации и иммунологии) и Отделение Нефрологии и Иммунологии

INSERM, UMR-S 1064, and Department of Nephrology and Immunology

Фервенца

Фернандо К.

Fervenza

Fernando C.

Отделение нефрологии и гипертензии

Department of Nephrology and Hypertension

Фремо-Бакши

Вероник

Fremeaux-Bacchi

Veronique

Каванаг

Давид

Kavanagh

David

Нестер

Карла М.

Nester

Carla M.

Лаборатория молекулярной отоларингологии и исследований в нефрологии

Подразделение Нефрологии, Отделение внутренних болезней

Molecular Otolaryngology and Renal Research Laboratories

Division of Nephrology, Department of Internal Medicine

Норис

Марина

Noris

Marina

Пикеринг

Мэтью К.

Pickering

Matthew C.

Центр изучения комплемента и воспаления, Отделение медицины

Department of Medicine

де Кордоба

Сантьяго Родригес

De Co´rdoba

Santiago Rodrı´guez

Руменина

Лубка Т.

Roumenina

Lubka T.

Сети

Санджив

Sethi

Sanjeev

Отделение лабораторной медицины и патологии

Department of Laboratory Medicine and Pathology

Дж. Н. Смит

Ричард

J.H. Smith

Richard

Лаборатория молекулярной отоларингологии и исследований в нефрологии

Подразделение Нефрологии, Отделение внутренних болезней

200 Хоукинс Драйв – 21151 PFP, Айова Сити, Айова 52242, США

Molecular Otolaryngology and Renal Research Laboratories

Division of Nephrology, Department of Internal Medicine

richard-smith@uiowa.edu

Институт Медицинской Генетики Университет НьюкаслаВеликобританияInstitute of Genetic Medicine, Newcastle University, Newcastle upon TyneUnited Kingdom

Кампус Императорского КолледжаВеликобританияCentre for Complement and Inflammation Research, Imperial College Hammersmith CampusUnited Kingdom

Университетский госпитальный центр НантаФранцияCHU de NantesFrance

Клиника MayoСоединённые Штаты АмерикиMayo ClinicUnited States

Государственный госпиталь Парижа Европейский госпиталь Жоржа ПомпидуФранцияAssistance Publique-Hôpitaux de Paris, Hôpital Européen Georges PompidouFrance

Медицинский колледж Карвера, Университет АйовыСоединённые Штаты АмерикиCarver College of Medicine, University of IowaUnited States

IRCCS – Институт фармакологических исследований «Марио Негри» Клинический исследовательский Институт редких заболеваний «Aldo e Cele Dacco»ИталияIRCCS–Istituto di Ricerche Farmacologiche “Mario Negri,” Clinical Research Center for Rare Diseases “Aldo e Cele Daccò,”Italy

Кампус Императорского Колледжа Хэммерсмита,ВеликобританияCentre for Complement and Inflammation Research, Imperial College Hammersmith CampusUnited Kingdom

Центр Биологических исследований, Национальный совет научных исследований Центр Биомедицинских исследований редких заболеванийИспанияCentro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas Centro de Investigación Biomédica en Enfermedades RarasSpain

Национальный институт здоровья и медицинских исследований, объединенное исследовательское отделение S 1138, комплемент и болезни, францисканский исследовательский центр Парижский Университет Декарта в составе Сорбонны Университет Пьера и Мари Кюри (UPMC)ФранцияInstitut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche S1138, Complément et Maladies, Centre de Recherche des Cordeliers Université Paris Descartes Sorbonne Paris-Cité Université Pierre et Marie Curie (UPMC-Paris-6)France

2018

21072018

2241839

2018

Г. Дж. Гудшип Т., Кук Г., Фахури Ф., Фервенца Ф., Фремо-Бакши В., Каванаг Д., Нестер К.М., Норис М., Пикеринг М.К., де Кордоба С., Руменина Л.Т., Сети С., Дж. Н. Смит Р.

H.J. Goodship T., Cook H., Fakhouri F., Fervenza F.C., Fremeaux-Bacchi V., Kavanagh D., Nester C.M., Noris M., Pickering M.C., de Co´rdoba S., Roumenina L.T., Sethi S., J.H. Smith R.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

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

https://journal.nephrolog.ru/jour/article/view/489

Система комплемента играет ключевую роль в развитии таких заболеваний, как атипичный гемолитико-уремический синдром (аГУС) и С3-гломерулопатия (С3ГП). В данной публикации мы обобщаем итоги конференции «Kidney Disease: Improving Global Outcomes» (KDIGO) 2015 по спорным вопросам, в ходе которой экспертами были коллегиально приняты ключевые подходы к ведению этих двух заболеваний. Были рассмотрены такие области, как патоморфология почек, клинические фенотипы и клиническая оценка, генетические аспекты болезни, варианты приобретенной болезни и терапевтические стратегии. С целью помощи клиницистам, которые наблюдают таких пациентов, подробно обсуждены и обоснованы рекомендации по выбору оптимальной лечебной стратегии. Были выделены пробелы в знании и обозначена стратегия приоритетных исследований с целью разрешения основных противоречий.

In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.

антикомплементная терапияатипичный гемолитико-уремический синдромС3-гломерулопатиякомплементгломерулонефритболезни почек

anti-complement therapiesatypical hemolytic uremic syndromeC3 glomerulopathycomplementglomerulonephritiskidney disease

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