Современные подходы к консервативной терапии поликистозной болезни почек


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Аннотация

Поликистозная болезнь почек (ПБП) - генетически обусловленный патологический процесс, связанный с образованием и ростом кист, происходящих из эпителиоцитов канальцев и/или собирательных трубочек. ПБП представлена двумя основными типами - аутосомно - доминантным (АДПБП) и аутосомно - рецессивным (АРПБП). Основной причиной АДПКП являются мутации в генах PKD1 и PKD2, которые кодируют образование белков полицистина-1 и полицистина-2. АРПБП связана с мутацией гена PKHD1, приводящей к полному отсутствию или синтезу неполноценного рецепторного белка первичных ресничек (цилий) - фиброцистина. Имеется связь структурных и функциональных дефектов в цилиях с ПБП. Образованию кист и накоплению в них жидкости способствуют: а) мутации генов полицистинов, расположенных на первичных ресничках; б) повышение активности внутриклеточного цАМФ в почке; в) активация V2-рецепторов вазопрессина; г) нарушение полярности эпителия канальцев (транслокация Na,K-АТФазы с базолатеральной мембраны на апикальную); д) повышение активности mTOR в эпителиальных клетках, выстилающих почечные кисты. Наиболее перспективными направлениями терапии АДПБП на сегодняшний день являются блокада активации V2-рецепторов вазопрессина, ингибирование mTOR сигнальных путей и снижение уровня внутриклеточного цАМФ. В обзоре представлены клинические исследования по оценке эффективности лекарственных средств с указанными механизмами действия при АДПБП.

Об авторах

Татьяна Евгеньевна Руденко

ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)

Email: atatianaer@yandex.ru
к.м.н., ассистент каф. внутренних, профессиональных болезней и ревматологии медико-профилактического факультета; тел.: +7(499)246-61-76; e-mail: atatianaer@yandex.ru; ORCID: 0000-0002-1296-4494 Москва, Россия

Ирина Николаевна Бобкова

ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)

д.м.н., проф. каф. внутренних, профессиональных болезней и ревматологии медико-профилактического факультета Москва, Россия

Екатерина Викторовна Ставровская

ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)

к.м.н., доц. каф. внутренних, профессиональных болезней и ревматологии медико-профилактического факультета Москва, Россия

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