nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1029Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESКлинико-морфологические особенности и эффективность лечения фокально-сегментарного гломерулосклероза у детей в КазахстанеClinical and morphological peculiarities and efficacy of treatment of children with focal segmental glomerulosclerosis in KazakhstanНаушабаеваА. Е.NaushabayevaA. Ye.noemail@neicon.ruНаучный центр педиатрии и детской хирургии МЗ РК; г. Алматы, КазахстанRussian Federation200919062025112103108Copyright © Наушабаева А.Е., 20252025Наушабаева А.Е.Naushabayeva A.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/1029

В последние десятилетия отмечается рост частоты фокально-сегментарного гломерулосклероза (ФСГС) по всему миру. Установлено, что ответ на лечение и прогноз ФСГС зависят от его морфологического варианта. Целью исследования явилось изучение частоты морфологических вариантов ФСГС и эффективности лечения среди детей казахской национальности. Мы наблюдали 16 детей казахской национальности с ФСГС, который проявлялся нефротическим синдромом (НС) у большинства детей - 11, нефритическим синдромом (НиС) - у 4 и сочетанием НС и НиС у 1. Наиболее частым морфологическим вариантом ФСГС у наших пациентов был верхушечный вариант у 7 (43,8%) детей. Типичный вариант отмечен у 6 (37,5%) детей и коллабирующий у 1 (6,3%). У 2 детей ФСГС вероятно ассоциирован с мутацией генов подоцитов у 1 и коллагена IV типа у 1, в связи с чем не был отнесен к вариантам первичного ФСГС. 9 детей с НС получали терапию циклоспорином А, пульсами метилпреднизолона и пероральным ПЗ. Полная ремиссия достигнута у 8 детей. Всем детям с НиС назначена терапия иАПФ, у 1 в сочетании с ММФ и пульсами метилпреднизолона. Отмечено снижение протеинурии. Таким образом, ФСГС у наших детей чаще всего проявляется клинически нефротическим, реже - нефритическим синдромами, морфологически - верхушечным («tip-lesion») и типичным вариантами. Циклоспорин А в сочетании с пульсами метилпреднизолона и пероральным преднизолоном показал высокую эффективность в достижении полной ремиссии при нефротическом синдроме, мофетила микофенолат в сочетании с иАПФ - при нефритическом синдроме (частичная ремиссия).

In the last decades the frequency of the focal segmental glomerulosclerosis (FSGS) increases. It is established that the dependence of the response on therapy and prognosis of the FSGS depend on morphological type. The aim of our study was to assess the frequency of types of FSGS and the response on therapy in Kazakh children. We have observed 16 Kazakh children with FSGS that was manifested with the nephrotic syndrome (NS) in 11 children, nephritic syndrome (NiS) in 4 children and combination of the NS and NiS in 1 child. The most frequent morphological type of FSGS was tip-lesion - 7 (43,8%). Typical type was observed in 6 (37,5%) children and collapsing glomerulopathy in 1 (6,3%) child. In 2 children FSGS was probably associated with podocytes’ and collagen type IV gene mutations and was not assessed as a primary disease. 9 children with the NS have received cyclosporine A, methylprednisolone pulses and oral prednisolone. Complete remission was achieved in 8 children. All children with NiS have received ACEi and one of them received mofetil mycophenolate with methylprednisolone pulses. A decrease in proteinuria was found in some cases. Thus, the FSGS in Kazakh children more is more often manifested clinically with the nephrotic syndrome, more rarely with the nephritic syndrome, and morphologically - with tip-lesion and typical type. Cyclosporine in combination with pulses of methylprednisolone and oral prednisolone are effective in achievement of complete remission of the nephrotic syndrome, and mofetil myciphenolate with ACEi - in nephritic syndrome (partial remission).

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