nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное общество10.28996/2618-9801-2026-2-227-236nid-4010Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESРезультаты длительного наблюдения когорты пациентов с атипичным гемолитико-уремическим синдромомResults of long-term follow-up in patients cohort with atypical hemolytic-uremic syndromehttps://orcid.org/0009-0004-3475-9784БлиноваТ. В.BlinovaT. V.

Блинова Татьяна Валерьевна – врач-нефролог отделения нефрологии Свердловской Областной Клинической больницы № 1.

620102, Екатеринбург, ул. Волгоградская, д. 185

Blinova Tatyana V.

185, Volgogradskaya St., Yekaterinburg, 620102

tanikonova@yandex.ruhttps://orcid.org/0000-0002-2485-9464СтолярА. Г.StolyarA. G.

Столяр Алексей Геннадьевич – д-р мед. наук, заведующий отделением нефрологии Свердловской Областной Клинической больницы № 1.

620062, Екатеринбург, ул. Мира, д. 19

Stolyar Aleksey G.

19, Mira St., Yekaterinburg, 620062

ambr375@mail.ruhttps://orcid.org/0009-0003-2187-1710БлиновД. В.BlinovD. V.

Блинов Денис Владимирович – канд. эк. наук, доцент кафедры экономики и управления на металлургических и машиностроительных предприятиях института экономики и управления наук Уральского федерального университета им. первого Президента России Б.Н. Ельцина.

620062, Екатеринбург, ул. Мира, д. 19

Blinov Denis V.

19, Mira St., Yekaterinburg, 620062

d.v.blinov@urfu.ruhttps://orcid.org/0000-0002-1959-5222СолодушкинС. И.SoloduchkinS. I.

Солодушкин Святослав Игоревич – канд. физ-мат. наук, доцент кафедры вычислительной математики и компьютерных наук Уральского федерального университета им. первого Президента России Б.Н. Ельцина.

620102, Екатеринбург, ул. Волгоградская, д. 185

Solodushkin Svyatoslav I.

185, Volgogradskaya St., Yekaterinburg, 620102

s.i.solodushkin@urfu.ruГБУЗ СО «Свердловская областная клиническая больница № 1»Россия1 Regional Clinical Hospital, Sverdlovsk Regional Clinical Hospital No. 1Russian FederationУральский Федеральный УниверситетРоссияUral Federal UniversityRussian Federation202629062026282227236Copyright © Блинова Т.В., Столяр А.Г., Блинов Д.В., Солодушкин С.И., 20262026Блинова Т.В., Столяр А.Г., Блинов Д.В., Солодушкин С.И.Blinova T.V., Stolyar A.G., Blinov D.V., Soloduchkin S.I.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/4010Актуальность

Актуальность. Атипичный гемолитико-уремический синдром (аГУС) как разновидность тромботической микроангиопатии (ТМА), сопряженная с патологией системы комплемента, занимает важное место в структуре поражения почек и связана с высокой вероятностью потери функции почек и поражением других жизненно важных органов.

Цель исследования

Цель исследования. Оценить результаты наблюдения и лечения пациентов с аГУС, получающих комплемент-блокирующую терапию (КБТ), в плане общей и почечной выживаемости.

Материалы и методы

Материалы и методы. Проанализированы истории болезни и амбулаторные карты 25 пациентов с аГУС, наблюдавшихся в отделении нефрологии Областной клинической больницы № 1 г. Екатеринбурга с 2014 по 2024 год (10 лет). Применялись методы дифференциальной диагностики данной патологии, а также морфологические (биопсия почки) и генетические (генетическое исследование системы комплемента) методы исследования. В качестве методов лечения использовались: плазмотерапия (трансфузии свежезамороженной плазмы, СЗП и/или плазмообмен), и/или КБТ (экулизумаб).

Статистическая обработка материала, анализ общей и почечной выживаемости проводились с использованием пакета прикладных программ SPSS, версия 24. Для обработки были применены методы описательной статистики, оценка выживаемости по Каплану-Мейеру (с построением соответствующих графиков) и точный критерий Фишера для межгрупповых сравнений.

Результаты

Результаты. Были выявлены такие триггеры аГУС как: беременность и роды (32%), инфекции (16%), IgA-нефропатия (8%), прием оральных контрацептивов (8%), ингаляционное отравление краской (4%), трансплантация почки (4%). Поражение почек наблюдалось у всех пациентов. Частота экстраренальных поражений у пациентов с аГУС распределялась следующим образом: сердце и ЦНС – 48% пациентов, ЖКТ – 44%, легкие – 28% пациентов и другие. У 22 пациентов было выполнено генетическое исследование системы комплемента, при этом мутации генов, ассоциированных c аГУС были обнаружены у 12 (54,5%) обследованных пациентов. Среди них: CFH, CFHR1, CFHR3, CFHR5, CFI, CD46, ADAMTS13. Общая выживаемость пациентов составила: однолетняя – 96%, пяти и десятилетняя – 89%. При этом, однолетняя почечная выживаемость составила 100%, пятилетняя 84%. Плазмотерапия в виде трансфузий СЗП была применена у 10 (40%) пациентов, плазмообмен у 16 (64%) пациентов. Специфическая КБТ экулизумабом использовалась у 22 (88%) пациентов. Отмена экулизумаба была связана с высоким риском рецидива ТМА – 36%.

Выводы

Выводы. При длительном наблюдении когорты 25 пациентов с аГУС, получающих специфическую КБТ, отмечена удовлетворительная общая выживаемость пациентов: однолетняя – 96%, 5-летняя 89%, и почечная выживаемость: однолетняя 100%, 5-летняя 84%.

Introduction

Introduction. Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) associated with dysregulation of the complement system. aHUS take an important place in the spectrum of kidney disease because of the high risk of kidney loss and damage to other vital organs.

Objective

Objective. The evaluate the outcomes of follow-up and treatment of patients with aHUS receiving complement-blocking therapy (CBT), with assessment of overall and renal survival.

Materials and methods

Materials and methods. Medical records and outpatient charts of 25 patients with aHUS followed at the nephrology department of Yekaterinburg Regional Clinical Hospital No. 1 between 2014 and 2024 (10 years) were analyzed. Differential diagnostic methods, as well as morphological (kidney biopsy) and genetic studies (genetic testing of the complement system) studies, were performed. Treatment included plasma therapy (fresh frozen plasma (FFP) transfusions and/or plasma exchange) and/or CBT with eculizumab.

Statistical analysis was performed using SPSS version 24. Descriptive statistics, Kaplan-Meier survival estimates with corresponding graphs, and Fisher's exact test for intergroup comparisons were used.

Results

Results. The identified triggers of aHUS included pregnancy and childbirth (32%), infections (16%), IgA nephropathy (8%), oral contraceptive use (8%), paint inhalation poisoning (4%), and kidney transplantation (4%). Genetic testing of the complement system was performed in 22 patients, and pathogenic variants associated with aHUS were identified in 12 (54.5%) of the examined patients. Overall patient survival rates were 96% at as: 1 year, and 89% at both 5 and 10 years. Renal survival was 100% at 1 year and 84% at 5years. Plasma therapy with FFP transfusions was administrated to 10 (40%) patients, and plasma exchange was performed in 16 (64%) patients. Specific CBT with eculizumab was used in 22 (88%) patients. Discontinuation of eculizumab was associated with a high risk of TMA recurrence (36%).

Conclusion

Conclusion. Long-term follow-up of 25 patients with aHUS receiving specific CBT demonstrated satisfactory overall patient survival (96% at 1 year, 89% at 5 years) and renal survival (100% at 1year, and 84% 5 years).

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