nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2018-22-6-9-22nefr-1622Research ArticleПЕРЕДОВАЯ СТАТЬЯLEADING ARTICLEАУТОСОМНО-ДОМИНАНТНАЯ ТУБУЛОИНТЕРСТИЦИАЛЬНАЯ БОЛЕЗНЬ ПОЧЕКAUTOSOMAL DOMINANT TUBULOINTERSTITIAL KIDNEY DISEASEКаюковИ. Г.KayukovI. G.

197022, Россия, Санкт-Петербург, ул. Л. Толстого, д. 17, корп. 54.

Проф. Каюков Иван Глебович, лаборатория клинической физиологии почек, заведующий.

Тел.: (812)346-39-26.

197022, Russia, Saint Petersburg, L. Tolstoy st. 17, build. 54.

Prof. Ivan G. Kayukov MD, PhD, DMedSci, Laboratory of Clinical Physiology of the Kidney, head.

Phone: (812)346-39-26.

kvaka55@mail.ruДобронравовВ. А.DobronravovV. A.

197022, Россия, Санкт-Петербург, ул. Л. Толстого, д. 17, корп. 54.

Проф. Добронравов Владимир Александрович, заместитель директора по научной работе.

197022, Russia, Saint Petersburg, L. Tolstoy st. 17, build. 54.

Prof. Vladimir A. Dobronravov, MD, PhD, DSci, vice director.

dobronpavov@nephrolog.ruБересневаО. Н.BeresnevaO. N.

197022, Россия, Санкт-Петербург, ул. Л. Толстого, д. 17, корп. 54.

Береснева Ольга Николаевна, канд. биол. наук, лаборатория клинической физиологии почек, ст. науч. сотр.

Тел.: (812)346-39-26

197022, Russia, Saint Petersburg, L. Tolstoy st. 17, build. 54.

Olga N. Beresneva, PhD, senior researcher, Laboratory of Clinical Physiology of the Kidney.

Phone: (812)346-39-26.

 

beresnevaolga@list.ruСмирновА. В.SmirnovA. V.

197022, Россия, Санкт-Петербург, ул. Л. Толстого, д. 17, корп. 54.

Проф. Смирнов Алексей Владимирович, директор.

Тел.: (812)338-69-01.

197022, Russia, Saint Petersburg, L. Tolstoy st. 17, build. 54.

Prof. Alexey V. Smirnov MD, PhD, DMedSci, director.

Phone: (812)338-69-01.

smirnov@nephrolog.ruНаучно-исследовательский институт нефрологии, Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова.РоссияResearch Institute of Nephrology, First Pavlov Saint-Petersburg State Medical University.Russian Federation201823122018226922Copyright © Каюков И.Г., Добронравов В.А., Береснева О.Н., Смирнов А.В., 20182018Каюков И.Г., Добронравов В.А., Береснева О.Н., Смирнов А.В.Kayukov I.G., Dobronravov V.A., Beresneva O.N., Smirnov A.V.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/1622

В последние годы существенно пересматриваются определения и классификации врожденных тубулоинтерстициальных заболеваний почек. Это связано, прежде всего, с достижениями в области молекулярной биологии и генетики, позволившими существенно уточнить роль ряда генов, кодирующих те или иные белки, тесно вовлеченные в деятельность почек. В 2015 г. KDIGO предложено унифицировать терминологию, диагностические критерии и подходы к мониторингу состояний, ассоциированных с мутациями генов MUC1, UMOD, HNF1B и REN, что привело к разработке представлений о новой нозологии – аутосомно-доминантной тубулоинтерстициальной болезни почек (АДТБП). Краткое описание основных сведений об АДТБП составило содержание настоящего сообщения.

In recent years, the definitions and classifications of congenital tubulo-interstitial kidney diseases have been significantly revised. This is primarily due to the achievements in the molecular biology and genetics, which made it possible to significantly clarify the role of a number of genes encoding certain proteins that are closely involved in kidney functions. In 2015, KDIGO proposed to unify the terminology, diagnostic criteria and approaches to monitoring conditions associated with mutations of the MUC1, UMOD, HNF1B and REN genes, which led to the development of ideas about the new nosology - autosomal dominant tubulointerstitial kidney disease (ADTKD). A brief description of the basic information about the ADTKD compiled the content of this message.

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