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”10.21508/1027-4065-2016-61-6-21-31perinatology-411Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWSНЕФРОПАТИИ, СВЯЗАННЫЕ С ПАТОЛОГИЕЙ СИСТЕМЫ КОМПЛЕМЕНТАNEPHROPATHIES ASSOCIATED WITH COMPLEMENT SYSTEM PATHOLOGYДлинВ. В.DlinV. V.

д.м.н., профессор, руководитель отдела наследственных и приобретенных болезней почек

ИгнатоваМ. С.IgnatovaM. S.

заслуженный деятель науки РФ, д.м.н., профессор, научный консультант

ОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ГБОУ ВПО РНИМУ им. Н.И. Пирогова Минздрава России, Москва, РоссияРоссияAkademician Yu.E. Veltishcev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University Ministry of Health Russian Federation, MoscowRussian Federation2016040120176162131Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20172017Ltd. “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/411

Обобщен научный материал по нефропатиям, связанным с патологией системы комплемента у детей и взрослых. Представлены клинические, иммунологические и морфологические отличия нефропатий, обусловленных патологией системы комплемента, от других заболеваний почек, прежде всего от гломерулонефрита (в том числе мембранопролиферативного варианта) и нефротического синдрома другой природы. Представлен патогенез развития указанных нефропатий, где выделены формы, связанные с генетическими мутациями, и варианты, обусловленные образованием аутоантител к компонентам комплемента. Показаны варианты и эффективность лечения иммуносупрессивными препаратами и экулизумабом в зависимости от патогенетических и клинических особенностей нефропатий, связанных с патологией системы комплемента.

Summarized research material for nephropathy associated with the pathology of the complement system in children and adults. Presents clinical, immunological and morphological differences of the nephropathy associated with the pathology of the complement system with other renal diseases, especially glomerulonephritis, including membranoproliferative variants and nephrotic syndrome associated with disorders of complement. The pathogenesis of the development of nephropathy associated with the pathology of the complement system, where highlighted as forms, associated with genetic mutations or variants, due to the formation of autoantibodies to components of the complement. Shown the options and effectiveness of treatment immunosuppressive drugs and by eculizumab depending on pathogenetic and clinical features of nephropathy associated with the pathology of the complement system.

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