nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1326Research ArticleШКОЛА НЕФРОЛОГАEDUCATIONAL MATERIALSДвусторонняя окклюзия почечных артерий с восстановлением функции почек после длительной терапии программным гемодиализом у пациента с генетической формой тромбофилииBilateral renal artery occlusion with recovery of renal function following long term hemodialysis in a patient with the genetic thrombophiliaКозловскаяН. Л.KozlovskayaN. L.noemail@neicon.ruКотляроваГ. В.KotlyarovaG. V.galopa@yandex.ruБоброваЛ. А.BobrovaL. A.MrLee2005@yandex.ruСафоновВ. В.SafonovV. V.noemail@neicon.ruКушнирВ. В.KushnirV. V.noemail@neicon.ruФоминыхЕ. В.FominikhE. V.noemail@neicon.ruКазаковС. В.KazakovS. V.noemail@neicon.ruМосковская медицинская академия им. И.М. СеченоваRussian FederationГКБ № 50; г. МоскваRussian Federation200819062025102151159Copyright © Козловская Н.Л., Котлярова Г.В., Боброва Л.А., Сафонов В.В., Кушнир В.В., Фоминых Е.В., Казаков С.В., 20252025Козловская Н.Л., Котлярова Г.В., Боброва Л.А., Сафонов В.В., Кушнир В.В., Фоминых Е.В., Казаков С.В.Kozlovskaya N.L., Kotlyarova G.V., Bobrova L.A., Safonov V.V., Kushnir V.V., Fominikh E.V., Kazakov S.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/1326

В последние годы получены данные, позволяющие предполагать возможность тромботического поражения сосудистого русла почек при наиболее частых генетических формах тромбофилии - Лейденской мутации V фактора свертывания крови, мутациях гена протромбина и метилентетрагидрофолатредуктазы (MTГФР). В отличие от других форм тромбофилии, для мутации гена MTГФР, приводящей к гипергомоцистеинемии (ГГц), характерны как венозные, так и артериальные тромбозы. Несмотря на многочисленные исследования, установившие важную роль различных генотипов МТГФР и ГГц в развитии коронарных и церебральных тромбозов, сообщения, свидетельствующие о возможности развития тромбозов иных локализаций у пациентов с ГГц, единичны. Приводимое собственное наблюдение демонстрирует тромботическое поражение обеих магистральных почечных артерий у больного с гетерозиготной мутацией МТГФР С677Т и умеренной ГГц, послужившее единственной причиной реноваскулярной гипертонии и тяжелой почечной недостаточности, потребовавшей лечения программным гемодиализом (ПГД). Особенностью представленного случая является также полное восстановление функции почек через 7, 5 лет лечения ПГД. Восстановлению почечного кровотока способствовали длительная антикоагулянтная терапия, молодой возраст больного и наличие добавочных почечных артерий с обеих сторон, обеспечивших адаптацию почек к длительной ишемии.

Last years we have obtained data allowing us to assume a possibility that the thrombotic complications of any location within the renal vasculature are caused by the most frequent genetic thrombophilias: Leiden mutation of the coagulation factor V, mutations of the prothrombin and methylentetrahydrofolate reductase (MTHFR). Unlike other thrombophilias, the mutation of gene MTHFR that leads to hyperhomocysteinemia is characterized by both arterial and venous thromboses. In spite of the numerous researches which have established the important role of various genotypes of MTHFR and hyperhomocysteinemia in development of coronary and cerebral thromboses, the studies of development of thromboses of other localizations in patients with hyperhomocysteinemia are rare. Our case illustrates bilateral renal artery trunk thrombosis in a hemodialysis patient with heterozygous MTHFR C677T mutation and mild hyperhomocysteinemia that was the only cause of renovascular hypertension and severe renal insufficiency. The peculiarity of this case is also the total recovery of renal function following 7.5 years of treatment by long term hemodialysis. As illustrated by this case, long term anticoagulant therapy, young age and additional renal arteries on both sides provided the best circumstances for adaptation to prolonged ischemia and reperfusion of the thrombosed vessels.

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