Наследственные митохондриальные цитопатии: коэнзим Q нефропатии (Обзор литературы)
Аннотация
В обзоре представлены современные данные о крайне редких СoQ10-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 Q10.
Об авторах
Т. В. Вашурина
НИИ педиатрии ФБГУ «НЦЗД» РАМН, Москва
Россия
Россия
О. И. Зробок
НИИ педиатрии ФБГУ «НЦЗД» РАМН, Москва
Россия
Россия
Т. В. Маргиева
НИИ педиатрии ФБГУ «НЦЗД» РАМН, Москва
Россия
Россия
А. Н. Цыгин
НИИ педиатрии ФБГУ «НЦЗД» РАМН, Москва
Россия
Россия
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Рецензия
Для цитирования:
Вашурина Т.В., Зробок О.И., Маргиева Т.В., Цыгин А.Н. Наследственные митохондриальные цитопатии: коэнзим Q нефропатии (Обзор литературы). Нефрология и диализ. 2012;14(2):95-101.
For citation:
Vashurina T.V., Zrobok O.I., Margieva T.V., Tsygin A.N. Inherited Mitochondriopathy: CoQ nephropathy Review. Nephrology and Dialysis. 2012;14(2):95-101. (In Russ.)
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