nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1556Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESЦиклоспорин А в лечении детей с фокально-сегментарным гломерулосклерозомCyclosporine A treatment of focal and segmentary glomerulosclerosis in childrenКомароваО. В.KomarovaO. V.noemail@neicon.ruЦыгинА. Н.TsyginA. N.tsygin@nczd.ruСухановА. В.SukhanovA. V.noemail@neicon.ruНаучный центр здоровья детей РАМНRussian Federation20062306202583249253Copyright © Комарова О.В., Цыгин А.Н., Суханов А.В., 20252025Комарова О.В., Цыгин А.Н., Суханов А.В.Komarova O.V., Tsygin A.N., Sukhanov A.V.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/1556

21 ребенок с фокально-сегментарным гломерулосклерозом (ФСГС) в возрасте от 1,5 до 16 лет получал терапию циклоспорином А (ЦсА) в средней дозе 4-5 мг/кг в сочетании с преднизолоном 1-1,5 мг/кг/48 ч. Для индукции ремиссии у 14 больных проводилась пульс-терапия метилпреднизолоном 30 мг/кг № 3-9. Через 6 мес. терапии циклоспорином А у 38% больных (8 детей) была констатирована полная клинико-лабораторная ремиссия стероид-резистентного нефротического синдрома (СРНС), частичная ремиссия - у 24% (5 больных), уменьшение активности отмечено в 10% случаев (2 больных), сохранение активности СРНС - в 28% (6 пациентов). У одного больного в связи с нарастанием гиперазотемии ЦсА был отменен через 6 мес. терапии. Через год терапии ЦсА полная ремиссия СРНС была отмечена в 55% случаев (11 детей), частичная - в 20% (4 больных), уменьшение активности констатировано в 5% случаев (1 ребенок), отсутствие эффекта от терапии - в 20% (4 больных). В 59% случаев при достижении ремиссии СРНС на фоне терапии ЦсА была снижена доза или полностью отменена терапия стероидами. У 5 из 8 больных, находящихся под наблюдением, через 1,5 года от начала терапии ЦсА проведена повторная нефробиопсия. Признаки тубулоинтерстициальной токсичности выявлены в одном случае, в связи с чем доза ЦсА была снижена вдвое. Пятеро больных находились на терапии ЦсА в терапевтической дозе в течение 2 лет. В одном случае отмечено нарастание уровня креатинина крови, что потребовало снижения дозы ЦсА с 4 до 2 мг/кг. Таким образом, в 75% случаев ФСГС отмечено достижение полной или частичной клинико-лабораторной ремиссии СРНС через год терапии ЦсА.

Twenty one children aged 1,5-16 years with steroid-resistant GN and biopsy-proven focal and segmentary glomerulosclerosis (FSGS) were treated with Cyclosporine A (CsA) 4-5 mg/kg as initial dosage and oral prednisolone 1-1,5 mg/kg every other day tapered to the 12-th month. Methylprednisolone pulses (MP) 30 mg/kg every other day for the first 2-4 weeks were used in 14 patients. The duration of the disease before treatment varied from 6 months to 7 years. After 6 months of treatment complete absence of proteinuria was achieved in 8 (38%) children, partial remission was found in 5 (24%) patients, more than 2-fold decrease in proteinuria was observed in 2 (10%) children. In 6 (28%) children including one patient who developed the ESRD the treatment had no effect. After one year of treatment complete remission was observed in 11 (55%) patients, partial remission was observed in 4 (20%) children, a decrease in proteinuria was found in 1 (5%) patient and no effect was achieved in 5 (25%) children. In all non-responders, the CsA-treatment was terminated. The MP-pulses were not used in 7 patients because of less severe disease or contraindications. However, 6 of them demonstrated a good response to treatment. None of responders had any severe side effects of CsA. In 8 patients the CsA-treatment was continued for 18 months. Only in 1 case an additional biopsy showed the tubular interstitial toxicity because the dose of CsA was tapered from 4 to 2 mg/kg. Five children were treated with CsA for 2 years, 4 of them had no any side effects. In one case an increase in serum creatinine level demanded tapering the CsA-dose. We conclude that early and prolonged treatment with CsA can provide complete or partial remission of GN in about 75% of patients with idiopathic FSGS.

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