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Том 14 №2 2012 год - Нефрология и диализ

Наследственные митохондриальные цитопатии: коэнзим Q нефропатии (Обзор литературы)


Вашурина Т.В. Зробок О.И. Маргиева Т.В. Цыгин А.Н.

Аннотация: В обзоре представлены современные данные о крайне редких СoQ -дефицитных гломерулопатиях, как изолированных, так и в рамках мультисистемных инфантильных форм. Установление вторичности стероид-резистентного нефротического синдрома по отношению к дефициту коэнзима Q не представляется возможным без установления генетических дефектов его биосинтеза. Тем не менее, объединяющим ультраструктурным признаком гломерулярного повреждения, вторичного к наследственной митохондриальной дисфункции, является обнаружение распространенной пролиферации дисморфных митохондрий в подоцитах, эндотелиальных и мезангиальных клетках. Ранняя постановка диагноза имеет решающее значение, вследствие возможной чувствительности СoQ-дефицитных форм стероид-резистентного нефротического синдрома к введению коэнзима Q .

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Ключевые слова: CoQ -нефропатия, CoQ -нефропатия, стероид-резистентный нефротический синдром, CoQ nephropathy, CoQ nephropathy, steroid-resistant nephrotic syndrome

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