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Том 9 №4 2007 год - Нефрология и диализ

Гемолитико-уремический синдром, не ассоциированный с шига-токсином: эпидемиология, классификация, клиника, диагностика, лечение (Обзор литературы. Часть 1)


Байко С.В.

Аннотация: Гемолитико-уремический синдром (ГУС) - это заболевание, включающее неиммунную (Кумбс-отрицательную) гемолитическую анемию, тромбоцитопению и поражение почек [70]. У детей пусковым фактором развития заболевания чаще всего служит Escherichia coli , продуцирующая шига-подобный токсин (Stx), а типичным проявлением заболевания является диарея («Д+» ГУС), часто кровянистого характера. Острая почечная недостаточность наблюдается в 55-70% случаев [79], однако в большинстве случаев (до 70% по различным данным) функция почек восстанавливается [35, 70]. ГУС, не ассоциированный с шига-токсином (non-Stx-HUS), включает гетерогенную группу пациентов, у которых этиологическое значение инфекции, вызванной бактериями, образующими шига-токсин и шига-подобные токсины, было исключено. Эта форма ГУС может носить спорадический или семейный (т. е. заболевание отмечается более чем у одного члена семьи и воздействие Stx исключено) характер. В целом, исход при non-Stx-HUS хуже. До 50% случаев протекает с развитием терминальной почечной недостаточности или необратимого повреждения головного мозга, а смертность в острой фазе заболевания может достигать 25% [11]. Генетические исследования показали, что семейная форма связана с нарушениями в системе регуляторных белков комплемента. Появляются также данные о том, что аналогичные генетические нарушения могут предрасполагать к развитию спорадических случаев non-Stx-HUS.

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Ключевые слова: атипичный гемолитико-уремический синдром, система комплемента, острая почечная недостаточность

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