nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical Universitynefr-164Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESБолезнь минимальных изменений и стероид-чувствительный нефротический синдром у детей: одна или две болезни?Minimal change disease and steroids sensitive nephrotic syndrome in children: one or two diseases?ПетросянЭдита КонстантиновнаPetrosyanEdita K.Ed3565@yandex.ruРоссийский Национальный исследовательский медицинский университетРоссияThe Russian National Research Medical UniversityRussian Federation2016010320162023338Copyright © Петросян Э.К., 20162016Петросян Э.К.Petrosyan E.K.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/164

Более трех десятилетий прошло после глобального исследования ISKDC (1981) нефротического синдрома у детей, позволившего сделать определенные рекомендации, такие как не проводить нефробиопсию у детей в дебюте заболевания и в случае положительного ответа на стероидную терапию признавать у больного наличие болезни минимальных изменений. Однако в последние годы отмечается прирост ФСГС у детей с первичным НС, в дебюте которого возможна стероидная чувствительность. В связи с чем следует пересмотреть восприятие первичного нефротического синдрома в детском возрасте как доброкачественное с благоприятным исходом. Более того, болезнь минимальных изменений, в патогенезе которой традиционно рассматривали только клеточно-опосредованный механизм, может развиваться и при других нарушениях, таких как дисрегуляция экспрессии CD80 на подоцитах или деструкция подоцита в связи с мутацией генов структурных белков последнего.

More than three decades have passed since the global study ISKDC (1981) of nephrotic syndrome in children allowed to make certain recommendations such as not to biopsy children at the onset of the disease and in the case of a sensitive to steroid therapy in a patient to recognize the presence of minimal change disease. However, in recent years there has been an increase FSGS in children with primary NS, in the onset of which steroid sensitivity is possible. Therefore, perception of primary nephrotic syndrome in children as a benign, with a favorable outcome should be revised. Moreover, minimal change disease, the pathogenesis of which is traditionally considered the only cell-mediated mechanism can be developed for other violations such as the dysregulation of CD80 expression in podocytes or podocyte destruction due to its structural proteins genes mutation.

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