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”10.21508/1027-4065-2021-66-5-67-74perinatology-1484Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESРоль катионных каналов потенциального рецептора TRPC в патогенезе идиопатического нефротического синдрома детейThe role of cationic channels of the potential TRPC receptor in the pathogenesis of idiopathic nephrotic syndrome in childrenhttps://orcid.org/0000-0002-0942-0103МорозовС. Л.MorozovS. L.

Морозов Сергей Леонидович – к.м.н., ст. науч. сотр. отдела наследственных и приобретенных болезней почек им. профессора М.С. Игнатовой; доц. кафедры госпитальной педиатрии №2 педиатрического факультета 

Москва

Moscow

mser@list.ruhttps://orcid.org/0000-0002-0942-0103ДлинВ. В.DlinV. V.

Длин Владимир Викторович – д.м.н., проф., рук. отдела наследственных и приобретенных болезней почек им. профессора М.С. Игнатовой, дир.

125412 Москва, ул. Талдомская, д. 2

Moscow

ОСП «Научно-исследовательский клинический институт педиатрии им. академика Ю.Е. Вельтищева» ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России; ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииРоссияVeltischev Research and Clinical Institute for Pediatrics at the Pirogov Russian National Research Medical University; Pirogov Russian National Research Medical UniversityRussian FederationОСП «Научно-исследовательский клинический институт педиатрии им. академика Ю.Е. Вельтищева» ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииРоссияVeltischev Research and Clinical Institute for Pediatrics at the Pirogov Russian National Research Medical UniversityRussian Federation2021081220216656774Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20212021Ltd. “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/1484

Идиопатический нефротический синдром – наиболее частая гломерулопатия в детском возрасте, распространенность которой составляет примерно 16 на 100 тыс. детского населения во всем мире. Любое хроническое гломерулярное заболевание имеет в своем роде однотипный механизм развития. Независимо от повреждающего фактора после гибели значительной части нефронов происходит устойчивое снижение скорости клубочковой фильтрации, при этом морфологически чаще всего определяется фокальный сегментарный гломерулосклероз. Изучение причин формирования фокального сегментарного гломерулосклероза составляет актуальную проблему в педиатрической нефрологии. В последнее время обсуждается роль катионных каналов потенциального рецептора TRPC подоцитов в развитии протеинурии и фокального сегментарного гломерулосклероза. В статье приводятся данные о роли рецепторов TRPC в патогенезе фокального сегментарного гломерулосклероза. Приводятся собственные данные, которые демонстрируют экспрессию генов семейства катионных каналов потенциального рецептора TRPC1, TRPC3, TRPC4, TRPC5 и TRPC6 у детей с идиопатическим нефротическим синдромом в зависимости от морфологической картины заболевания и чувствительности к стероидной терапии.

Idiopathic nephrotic syndrome is the most common glomerulopathy in children, with a prevalence of approximately 16 per 100,000 of child population worldwide. Any chronic glomerular disease has the same type of development mechanism. Regardless of the damaging factor, after the death of a significant part of the nephrons, there occurs a steady decrease in the glomerular filtration rate, while morphologically we most often determine focal segmental glomerulosclerosis. Studying the causes of focal segmental glomerulosclerosis is an urgent problem in pediatric nephrology. Recently, there has been discussed the role of the cation channels of the potential receptor TRPC of podocytes in the development of proteinuria and focal segmental glomerulosclerosis. The article provides data on the role of TRPC receptors in the pathogenesis of focal segmental glomerulosclerosis. The authors present their our own data demonstrating gene expression of the cationic channels family of the potential receptor TRPC1, TRPC3, TRPC4, TRPC5 and TRPC6 in children with idiopathic nephrotic syndrome, depending on the morphological picture of the disease and sensitivity to steroid therapy.

детинефротический синдромфокальный сегментарный гломерулосклерозэкспрессиярецептор TRPCchildrennephrotic syndromefocal segmental glomerulosclerosisexpressionTRPC receptorработа выполнена в рамках финансирования Госзадания «Клинические и молекулярно-генетический критерии прогнозирования эффективности стероидной и иммуносупрессивной терапии первичного нефротического синдрома у детей» № 200080056the work was carried out within the framework of funding from the State Assignment “Clinical and molecular genetic criteria for predicting the effectiveness of steroid and immunosuppressive therapy for primary nephrotic syndrome in children” No. 200080056ReferencesEddy A.A., Symons J.M. Nephrotic syndrome in childhood. Lancet 2003; 362(9384): 629–639. DOI: 10.1016/S0140-6736(03)14184-0Eddy A.A., Symons J.M. Nephrotic syndrome in childhood. Lancet 2003; 362(9384): 629–639. DOI: 10.1016/S0140-6736(03)14184-0Becherucci F., Roperto R.M., Materassi M., Romagnani P. Chronic kidney disease in children. 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The authors declare that there are no conflicts of interest present.