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Том 22 №4 2020 год - Нефрология и диализ

Клинические практические рекомендации IPNA по диагностике и лечению детей со стероид-резистентным нефротическим синдромом


Агнес Траутманн Марина Виварелли Сьюзан Самуэль Дебби Джипсон Адити Синха Франц Шафер Нг Кар Хуи Оливия Бойер Мойн А Салим Луциана Фельтран Янина Мюллер-Дайле Ян Ульрих Беккер Франциско Кано Хонг Сю Ям Нго Лим Уильям Смойер Ифеома Аночи Коичи Наканиси Элизабет Ходсон Дитер Хаффнер

DOI: 10.28996/2618-9801-2020-4-435-473

Аннотация: Идиопатический нефротический синдром ежегодно выявляется у 1-3 детей на 100 000 детской популяции. Примерно в 85% случаев наблюдается полная ремиссия протеинурии после лечения глюкокортикостероидами. У пациентов, не достигших полной ремиссии в течение 4-6 недель стероидной терапии, имеет место стероид-резистентный нефротический синдром (СРНС). У 10-30% пациентов со СРНС могут быть обнаружены мутации в генах, ассоциированных с подоцитами, в то время как у остальных детей в генезе заболевания предполагаются циркулирующие факторы иммунного происхождения. Диагностика и лечение СРНС представляет собой сложную задачу в связи с гетерогенной этиологией заболевания, частым отсутствием эффекта при дальнейшей иммуносупрессивной терапии, а также с тяжелыми осложнениями и развитием хронической болезни почек (ХБП) 5 стадии и возвратом заболевания после трансплантации почек. Группа экспертов, включающая детских нефрологов и генетиков из Международной ассоциации педиатрической нефрологии (IPNA), ренального патоморфолога и взрослого нефролога, разработала клинические практические рекомендации по диагностике и лечению СРНС у детей. Экспертная группа провела систематический обзор литературы по 9 клинически значимым вопросам PICO (Patient or Population covered, Intervention, Comparator, Outcome/Пациент или Популяция, Вмешательство, Сравнение, Исход), сформулировала рекомендации и присвоила им степень на консенсусном совещании с участием представителей пациентов и диетолога, выступавшего в качестве внешнего консультанта, а также группы детских нефрологов, участвующих в голосовании.

Весь текст

Ключевые слова: стероид-резистентный нефротический синдром, дети, хронические заболевания почек, генетика, исход, педиатрия, иммуносупрессивное лечение, steroid-resistant nephrotic syndrome, children, chronic kidney disease, genetics, outcome, pediatrics, immunosuppressive treatment

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