nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-3207Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESКлинико-морфологическая характеристика фокального сегментарного гломерулосклероза/гиалинозаClinico-morphological characteristics of focal segmental glomerulosclerosis/hyalinosisДжаналиевБ. Р.DjanalievB. R.noemail@neicon.ruВаршавскийВ. А.VarshavskyV. A.noemail@neicon.ruПроскурневаЕ. П.ProskurnevaE. P.noemail@neicon.ruКафедра патологической анатомии ММА имени И.М. СеченоваRussian Federation200228062025413338Copyright © Джаналиев Б.Р., Варшавский В.А., Проскурнева Е.П., 20252025Джаналиев Б.Р., Варшавский В.А., Проскурнева Е.П.Djanaliev B.R., Varshavsky V.A., Proskurneva E.P.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/3207

Целью исследования явилось изучение клинико-морфологической характеристики фокального сегментарного гломерулосклероза/гиалиноза (ФСГГ). Изучено 67 больных первичным ФСГГ. При исследовании нефробиоптатов использованы гистологический, иммуногистохимический и электронно-микроскопический методы. На основании комплексного морфологического исследования выделены две стадии ФСГГ. I стадия характеризуется поражением в основном юкстамедуллярных клубочков со слабо выраженной фибропластической трансформацией (ФТ) клубочков без или с незначительным тубулоинтерстициальным компонентом (ТИК). Иммуногистохимически 53,2% наблюдений были иммунонегативными, в 21,8% отмечена фиксация IgM. Индекс активности составил 1,6 ± 0,6 балла, индекс склероза - 3,7 ± 0,8 балла. Для данной стадии более (71,4%) характерна нефротическая форма. II стадия характеризуется поражением как юкстамедуллярных, так и медуллярных клубочков с резко выраженной ФТ клубочков и ТИК. Иммунонегативными были всего 7,7%, фиксация IgM отмечена в 73,1% наблюдений. Индекс активности составил 3,3 ± 0,8 балла, индекс склероза - 10,6 ± 2,2 балла. Для данной стадии, кроме нефротической (34,3%), характерны гипертоническая (21,8%) и смешанная (34,3%) формы. В этой стадии отмечено снижение показателей клубочковой фильтрации и концентрационной функции почек.

Clinico-morphological characteristic of idiopathic focal segmental glomerulosclerosis/hyalinosis (FSGH) was investigated by histological, immunohystochemical and electron microscopy methods. Two stage of FSGH were devided. The first stage characterized by the predominantly lesion of juxtamedullar glomeruli with mild fibroplastic transformation (FT). Tubulointerstitial component (TC) was weakly expressed or absent. 53,2% cases were immunonegative, in 21,8% fixation IgM was detected. Activity index (AI) was 1,6 ± 0,6, sclerosis index (IS) - 3,7 ± 0,8. In 71,4% cases nephrotic syndrome was present. Second stage of FSGH was characterized by the lesion of juxtomedullar as well as medullar with high intensity of FT and TC. Immunonegative were only 7,7% of cases, fixation of IgM revealed in 73,1%. AI was 3,3 ± 0,8; IS - 10,6 ± 2,2. The nephrotic syndrome (34,3%), hypertonic (21,8%) and mixed form (34,3%) were detected. Decrease of glomerular filtration rate and concentrative function of kidney was found.

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