Abstract
The concept of the steroid resistance in children with nephrotic syndrome, who were initially sensitive to steroids, has been known for a long time. Despite several foreign studies, this problem remains largely unexplored. The results of the presented review demonstrate a lack of information on many important aspects of resistant nephrotic syndrome. In particular, the true causes and pathogenesis of glucocorticosteroid resistance remain unknown, despite the long history of this problem in pediatric nephrology. On the one hand, such patients are clinically similar to the patients with steroid-resistant nephrotic syndrome, but at the same time they have a relatively favorable outcome in general. On the other hand, the ambiguous response to the steroid and immunosuppressive therapy hinders the development of a unified concept of the disease and pathogenetic therapy. Thus, an important task for future research is to find the reasons for the development of late (secondary) steroid resistance and, to develop and implement a personalized therapeutic approach to the management of children into clinical practice.
Highlights
The concept of the steroid resistance in children with nephrotic syndrome, who were initially sensitive to steroids, has been known for a long time
On the one hand, such patients are clinically similar to the patients with steroid-resistant nephrotic syndrome, but at the same time they have a relatively favorable outcome in general
An important task for future research is to find the reasons for the development of late steroid resistance and, to develop and implement a personalized therapeutic approach to the management of children into clinical practice
Summary
– генетический нефротический синдром, обусловленный мутациями в генах, ответственных за синтез белков, участвующих в формировании щелевой диафрагмы (подоцин и др.);. Вторичная стероидная резистентность идиопатического нефротического синдрома у детей: состояние проблемы оценки истинной вторичной резистентности к глюкокортикостериодам [11]:. В дальнейшем ряд авторов установили, что период появления поздней резистентности к стероидной терапии сократился до 1–1,5 года от дебюта заболевания и в среднем составлял 4,5– 4,6 мес при средней частоте рецидивов нефротического синдрома 2,5 мес [3,4,5,6]. Что стероид-чувствительный нефротический синдром, как правило, дебютирует в возрасте 3–5 лет, как и у детей со вторичной стероидной резистентностью, в то время как для детей со стероид-резистентным нефротическим синдромом характерен более старший возраст дебюта, за исключением врожденных и инфантильных форм нефротического синдрома, которые в своей основе имеют генетические причины [2]. The incidence of late steroid resistance among children with steroid-sensitive nephrotic syndrome
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