perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2023-68-2-93-98perinatology-1807Research ArticleКЛИНИЧЕСКИЕ СЛУЧАИCLINICAL CASESИлеофеморальный тромбоз у пациента с рецидивирующим нефротическим синдромомIliofemoral thrombosis in a patient with recurrent nephrotic syndromehttps://orcid.org/0000-0002-9462-3000РаздолькинаТ. И.RazdolkinaT. I.

Раздолькина Татьяна Ивановна — кандидат медицинских наук, доцент кафедры педиатрии Медицинского института 

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

trazdolkina@mail.ruhttps://orcid.org/0000-0003-2927-3224ВерещагинаВ. С.VereshchaginaV. S.

Верещагина Вероника Сергеевна — кандидат медицинских наук, доцент кафедры педиатрии Медицинского института 

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

https://orcid.org/0000-0002-2290-0013БалыковаЛ. А.BalykovaL. A.

Балыкова Лариса Александровна — доктор медицинских наук, профессор, член-корреспондент РАН, директор Медицинского института 

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

larisabalykova@yandex.ruhttps://orcid.org/0000-0002-4504-7959МосковскаяЕ. Ф.MoskovskayaE. F.

Московская Елена Федоровна — заведующая нефрологическим отделением 

430032 Саранск, ул. Розы Люксембург, д. 15

Saransk

https://orcid.org/0000-0003-3990-9353КраснопольскаяА. В.KrasnopolskayaA. V.

Краснопольская Анна Валерьевна — кандидат медицинских наук, старший преподаватель кафедры педиатрии Медицинского института 

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

https://orcid.org/0000-0003-3730-7519ГорбатовВ. А.GorbatovV. A.

Горбатов Виктор Алексеевич — кандидат медицинских наук, доцент кафедры педиатрии Медицинского института 

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

https://orcid.org/0000-0001-8249-1795ШулепинаА. В.ShulepinaA. V.

Шулепина Алена Вячеславовна — студентка VI курса специальности «Педиатрия»  Медицинского  института  

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

https://orcid.org/0000-0002-1294-9411ИшуткинаС. С.IshutkinaS. S.

Ишуткина Светлана Сергеевна — студентка VI курса специальности «Педиатрия» Медицинского института 

430000 Саранск, ул. Большевистская, д. 68/1

Saransk

ФГБОУ ВО «Национальный исследовательский Мордовский государственный университет им. Н.П. Огарева»РоссияOgarev National Research Mordovia State UniversityRussian FederationГБУЗ РМ «Детская республиканская клиническая больница»РоссияRepublican Children’s Clinical HospitalRussian Federation2023270420236829398Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20232023Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/1807

Нефротический синдром — одна из самых распространенных гломерулопатий у детей, сопровождающаяся высоким риском тромбозов вследствие гипоальбуминемии, гиповолемии, гиперлипидемии, гиперфибриногенемии, дефицита антитромбина III. В статье описан клинический случай илеофеморального тромбоза у мальчика 16 лет с рецидивирующим нефротическим синдромом и первичной тромбофилией (гетерозигота F5 — лейденская мутация). Представленное клиническое наблюдение указывает на многофакторность тромботических осложнений при нефротическом синдроме, обусловленных как дисбалансом между прои антикоагулянтными звеньями свертывающей системы крови, так и наличием индивидуальных факторов риска (выраженность протеинурии, гипоальбуминемии, сопутствующая патология, наследственная тромбофилия), в связи с чем необходимо их дальнейшее изучение для определения тактики лечения и профилактики.

Nephrotic syndrome is one of the most common glomerulopathies in children, accompanied by a high risk of thrombosis due to hypoalbuminemia, hypovolemia, hyperlipidemia, hyperfibrinogenemia, and antithrombin III deficiency. The article describes a clinical case of iliofemoral thrombosis in a 16-year-old boy with recurrent nephrotic syndrome and primary thrombophilia (heterozygote F5 — Leiden mutation) was diagnosed. The presented clinical observation indicates the multifactorial origin of thrombotic complications in nephrotic syndrome, caused by a combination of an imbalance between the proand anticoagulant components of the blood coagulation system and individual risk factors (severity of proteinuria, hypoalbuminemia, comorbidity, hereditary thrombophilia), and therefore further study is needed to determine the tactics of prevention and treatment.

детинефротический синдромилеофеморальный тромбозгемостазchildrennephrotic syndromeiliofemoral thrombosishemostasisReferencesКлинические рекомендации. Нефротический синдром у детей. 2016; 31 с. https://www.pediatr-russia.ru/information/klin-rek/deystvuyushchie-klinicheskie-rekomendatsii/index.php / Ссылка активна на 12.02.2023.Clinical guidelines. Nephrotic syndrome in children, 2016; 31 (in Russ) https://www.pediatr-russia.ru/information/klin-rek/deystvuyushchie-klinicheskie-rekomendatsii/index.php / Ссылка активна на 12.02.2023.Wang C.S., Greenbaum L.A. Nephrotic syndrome. Pediatr Clin North Am 2019; 66(1): 73–85. DOI: 10.1016/j.pcl.2018.08.006Wang C.S., Greenbaum L.A. Nephrotic syndrome. Pediatr Clin North Am 2019; 66(1): 73–85. DOI: 10.1016/j.pcl.2018.08.006Noone D.G., Iijima K., Parekh R. Idiopathic nephrotic syndrome in children. Lancet 2018; 392(10141): 61–74. DOI: 10.1016/S0140–6736(18)30536–1Noone D.G., Iijima K., Parekh R. Idiopathic nephrotic syndrome in children. Lancet 2018; 392(10141): 61–74. DOI: 10.1016/S0140–6736(18)30536–1Tapia C., Bashir K. Nephrotic Syndrome. https://www.ncbi.nlm.nih.gov/books/NBK470444 / Ссылка активна на 12.02.2023.Tapia C., Bashir K. Nephrotic Syndrome. https://www.ncbi.nlm.nih.gov/books/NBK470444 / Ссылка активна на 12.02.2023.Носов В.П., Соловьянова Е.Н., Королева Л.Ю. Нефротический синдром как фактор риска тромботических и тромбоэмболических осложнений. Нефрология и диализ 2021; 23(3): 366–378. DOI: 10.28996/2618–9801–2021–3–366–378Nosov V.P., Solovyanova E.N., Koroleva L.Yu. Nephrotic syndrome as a risk factor for thrombotic and thromboembolic complications. Nephrologiya i dialys 2021; 23(3): 366–378. (in Russ.) DOI: 10.28996/2618–9801–2021–3–366–378Lv YL., Guan N., Ding J., Yao Y., Xiao H-J., Zhong X-H. et al. Spectrum of thrombotic complications and their outcomes in Chinese children with primary nephrotic syndrome. Ital J Pediatr 2020; 46: 182. DOI: 10.1186/S13052–020–00942–0Lv YL., Guan N., Ding J., Yao Y., Xiao H-J., Zhong X-H. et al. Spectrum of thrombotic complications and their outcomes in Chinese children with primary nephrotic syndrome. Ital J Pediatr 2020; 46: 182. DOI: 10.1186/S13052–020–00942–0Busuioc R.M., Mircescu G. Nephrotic Syndrome Complications — New and Old. Part 1. Maedica (Bucur) 2022; 17(1): 153–168. DOI: 10.26574/maedica.2022.17.1.153Busuioc R.M., Mircescu G. Nephrotic Syndrome Complications — New and Old. Part 1. Maedica (Bucur) 2022; 17(1): 153–168. DOI: 10.26574/maedica.2022.17.1.153Tavil B., Kara F., Topaloglu R., Aytac S., Unal S., Kuskonmaz B. et al. Case series of thromboembolic complications in childhood nephrotic syndrome: Hacettepe experience. Clin Exp Nephrol 2015; 19(3): 506–513. DOI: 10.1007/s10157–014–1005-yTavil B., Kara F., Topaloglu R., Aytac S., Unal S., Kuskonmaz B. et al. Case series of thromboembolic complications in childhood nephrotic syndrome: Hacettepe experience. Clin Exp Nephrol 2015; 19(3): 506–513. DOI: 10.1007/s10157–014–1005-yIshikawa T., Nakajima Y., Omae T., Ogiwara K., Nogami K. Comprehensive coagulation and fibrinolytic potential in the acute phase of pediatric patients with idiopathic nephrotic syndrome evaluated by whole blood-based rotational thromboelastometry. Pediatr Nephrol 2022; 37(7): 1605– 1614. DOI: 10.1007/s00467–021-05366–4Ishikawa T., Nakajima Y., Omae T., Ogiwara K., Nogami K. Comprehensive coagulation and fibrinolytic potential in the acute phase of pediatric patients with idiopathic nephrotic syndrome evaluated by whole blood-based rotational thromboelastometry. Pediatr Nephrol 2022; 37(7): 1605– 1614. DOI: 10.1007/s00467–021-05366–4Eneman B., Levtchenko E., van den Heuvel B., Van Geet C., Freson K. Platelet abnormalities in nephrotic syndrome. Pediatr Nephrol 2016; 31:1267–1279. DOI: 10.1007/s00467–015–3173–8Eneman B., Levtchenko E., van den Heuvel B., Van Geet C., Freson K. Platelet abnormalities in nephrotic syndrome. Pediatr Nephrol 2016; 31:1267–1279. DOI: 10.1007/s00467–015–3173–8Boussetta A., Jaber C., Jellouli M., Gargah T. Thromboembolic complications in children with primary nephrotic syndrome: A Tunisian series. Tunis Med 2022; 100(1): 33–36. https://www.researchgate.net/publication/359972764 / Ссылка активна на 12.02.2023.Boussetta A., Jaber C., Jellouli M., Gargah T. Thromboembolic complications in children with primary nephrotic syndrome: A Tunisian series. Tunis Med 2022; 100(1): 33–36. https://www.researchgate.net/publication/359972764 / Ссылка активна на 12.02.2023.Kumar M., Malhotra A., Gupta S., Singh R. Thromboembolic complications at the onset of nephrotic syndrome. Sudan J Paediatr 2017; 17(2): 60–63. DOI: 10.24911/SJP.2017.2.8Kumar M., Malhotra A., Gupta S., Singh R. Thromboembolic complications at the onset of nephrotic syndrome. Sudan J Paediatr 2017; 17(2): 60–63. DOI: 10.24911/SJP.2017.2.8Lv Y.L., Guan N., Ding J., Yao Y., Xiao H.J., Zhong X.H. et al. Spectrum of thrombotic complications and their out-comes in Chinese children with primary nephrotic syndrome. Ital J Pediatr 2020; 46(1): 182. DOI: 10.1186/s13052–020–00942–0Lv Y.L., Guan N., Ding J., Yao Y., Xiao H.J., Zhong X.H. et al. Spectrum of thrombotic complications and their out-comes in Chinese children with primary nephrotic syndrome. Ital J Pediatr 2020; 46(1): 182. DOI: 10.1186/s13052–020–00942–0Martinez Ara J., Gomez Rioja R., Rinon C., Garcia Munoz M.S., Ruiz Caravaca M.L., Miguel J.L. Prevalence of genetic prothrombotic factors (factor V Leiden and II20210 prothrombin mutation) in glomerular nephropathies with or without thrombosis. Nefrologiya 2000; 20: 139–144Martinez Ara J., Gomez Rioja R., Rinon C., Garcia Munoz M.S., Ruiz Caravaca M.L., Miguel J.L. Prevalence of genetic prothrombotic factors (factor V Leiden and II20210 prothrombin mutation) in glomerular nephropathies with or without thrombosis. Nefrologiya 2000; 20: 139–144Pasini A., Benetti E., Conti G., Ghio L., Lepore M., Massella L. et al. The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I — Diagnosis and treatment of the first episode and the first relapse. Ital J Pediatr 2017; 43(1): 41. DOI: 10.1186/s13052–017–0356-xPasini A., Benetti E., Conti G., Ghio L., Lepore M., Massella L. et al. The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I — Diagnosis and treatment of the first episode and the first relapse. Ital J Pediatr 2017; 43(1): 41. DOI: 10.1186/s13052–017–0356-xMcCaffrey J., Lennon R., Webb N.J. The non-immunosuppressive management of childhood nephrotic syndrome. Pediatr Nephrol 2016; 31(9): 1383–1402. DOI: 10.1007/s00467–015–3241–0McCaffrey J., Lennon R., Webb N.J. The non-immunosuppressive management of childhood nephrotic syndrome. Pediatr Nephrol 2016; 31(9): 1383–1402. DOI: 10.1007/s00467–015–3241–0Derebail V.K., Rheault M.N., Kerlin B.A. Role of direct oral anticoagulants in patients with kidney disease. Kidney Int 2020; 97(4): 664–675. DOI: 10.1016/j.kint.2019.11.027Derebail V.K., Rheault M.N., Kerlin B.A. Role of direct oral anticoagulants in patients with kidney disease. Kidney Int 2020; 97(4): 664–675. DOI: 10.1016/j.kint.2019.11.027Singhal R., Brimble K.S. Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management. Thromb Res 2006; 118: 397–407. DOI: 10.1016/j.thromres.2005.03.030Singhal R., Brimble K.S. Thromboembolic complications in the nephrotic syndrome: pathophysiology and clinical management. Thromb Res 2006; 118: 397–407. DOI: 10.1016/j.thromres.2005.03.030Artoni A., Passamonti S.M., Edefonti A., Gianniello F., Civelli V., Martinelli I. Antithrombotic prophylaxis in a patient with nephrotic syndrome and congenital protein S deficiency. Ital J Pediatr 2016; 42: 22. DOI: 10.1186/s13052–016–0227-xArtoni A., Passamonti S.M., Edefonti A., Gianniello F., Civelli V., Martinelli I. Antithrombotic prophylaxis in a patient with nephrotic syndrome and congenital protein S deficiency. Ital J Pediatr 2016; 42: 22. DOI: 10.1186/s13052–016–0227-xGigante A., Barbano B., Sardo L., Martina P., Gasperini M.L., Labbadia R. et al. Hypercoagulability and nephrotic syndrome. Curr Vasc Pharmacol 2014; 12: 512–517. DOI: 10.2174/157016111203140518172048Gigante A., Barbano B., Sardo L., Martina P., Gasperini M.L., Labbadia R. et al. Hypercoagulability and nephrotic syndrome. Curr Vasc Pharmacol 2014; 12: 512–517. DOI: 10.2174/157016111203140518172048Egerman M.A., Wong J.S., Runxia T. Mosoyan G., Chauhan K., Reyes-Bahamonde J. et al. Plasminogenuria is assosiated with podocyte injury, edema, and kidney dysfunction glomerular disease. FASEB J 2020; 34: 16191–16204. DOI: 10.1096/fj.202000413REgerman M.A., Wong J.S., Runxia T. Mosoyan G., Chauhan K., Reyes-Bahamonde J. et al. Plasminogenuria is assosiated with podocyte injury, edema, and kidney dysfunction glomerular disease. FASEB J 2020; 34: 16191–16204. DOI: 10.1096/fj.202000413ROzkayin N., Mir S., Kavakli K. Hypercoagulability risk factors in children with minimal change disease and the protective role of protein-C activity. Int Urol Nephrol 2004; 36: 599– 603. DOI: 10.1007/s11255–004–0868–3Ozkayin N., Mir S., Kavakli K. Hypercoagulability risk factors in children with minimal change disease and the protective role of protein-C activity. Int Urol Nephrol 2004; 36: 599– 603. DOI: 10.1007/s11255–004–0868–3Боброва Л.А., Козловская Н.Л. Тромбоэмболические осложнения нефротического синдрома. Терапевтический архив 2020; 92(6): 105–116. DOI: 10.26442/00403660.2020.06.000667Боброва Л.А., Козловская Н.Л. Тромбоэмболические осложнения нефротического синдрома. Терапевтический архив 2020; 92(6): 105–116. (in Russ.)] DOI: 10.26442/00403660.2020.06.000667Eneman B.L., Levtchenko E., van den Heuvel B., Van Geet C., Freson K. Platelet abnormalities in nephrotic syndrome. Pediatric Neph-rology. 2015; 31:1267–1279. DOI: 10.1007/s00467-015-3173-8Eneman B.L., Levtchenko E., van den Heuvel B., Van Geet C., Freson K. Platelet abnormalities in nephrotic syndrome. Pediatric Neph-rology. 2015; 31:1267–1279. DOI: 10.1007/s00467-015-3173-8Rovin B.H., Adler S.G., Barratt J. KDIGO 2021 Clinical Prac-tice Guideline for the Management of Glomerular Diseases. Kidney Inter-national. 2021; 100:1–276. DOI: 10.1016/j.kint.2021.05.021Rovin B.H., Adler S.G., Barratt J. KDIGO 2021 Clinical Prac-tice Guideline for the Management of Glomerular Diseases. Kidney Inter-national. 2021; 100:1–276. DOI: 10.1016/j.kint.2021.05.021

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