perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2017-62-6-29-44perinatology-588Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWSНЕФРОТИЧЕСКИЙ СИНДРОМ: ПРОШЛОЕ, НАСТОЯЩЕЕ И БУДУЩЕЕNEPHROTIC SYNDROME: PAST, PRESENT AND FUTUREИгнатоваМ. С.IgnatovaM. S.

Игнатова Майя Сергеевна – доктор медицинских наук, профессор, главный научный сотрудник отдела наследственных и приобретенных болезней почек.

Moscow

https://orcid.org/0000-0002-3050-7748ДлинВ. В.DlinV. V.

Длин Владимир Викторович – доктор медицинских наук, профессор, руководитель отдела наследственных и приобретенных болезней почек.

125412 Москва, ул. Талдомская, д. 2

Moscow

ОСП «Научно-исследовательский клинический институт педиатрии им. академика Ю.Е. Вельтищева» ФГБОУ ВО РНИМУ им. Н.И. ПироговаРоссияYu. Е. Veltischev Clinical Research Institute of Pediatrics, Pirogov Russian National Research Medical UniversityRussian Federation2017110120186262944Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20182018Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/588

Настоящий обзор литературы посвящен изменению наших представлений об этиологии, патогенезе и тактике лечения  нефротического синдрома за последние десятилетия. Показано изменение исходов лечения первичного нефротического  синдрома в связи с появлением новых технологий терапии. Представлены особенности течения, обследования и терапии  врожденного и инфантильного нефротического синдрома и возможность дебюта нефротического синдрома, связанного  с различными генными мутациями, и в более старшем возрасте. Подчеркнуты принципиальные различия диагностических  и терапевтических подходов в зависимости от причины развития заболевания. Представлены современные синдромологические и патогенетические методы терапии первичного нефротического синдрома и показаны ближайшие перспективы  внедрения новых технологий лечения, основанные на использовании моноклональных антител.

This literature review is focused to change our ideas about the etiology, pathogenesis and treatment tactics of the nephrotic syndrome  in recent decades. The change in the treatment outcomes of the primary nephrotic syndrome in connection with the emergence of new  therapy technologies, is shown. Features of the course, examination and therapy of congenital and infantile nephrotic syndrome and  the possibility of the debut of a nephrotic syndrome associated with various gene mutations and at an older age are presented. Principal differences in diagnostic and therapeutic approaches are accentuated depending on the cause of the development of the disease.  Modern syndromological and pathogenetic methods of therapy of primary nephrotic syndrome are presented, and the immediate opportunities for the introduction of new treatment technologies based on the use of monoclonal antibodies, are shown.

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