Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya3500010.26442/00403660.2020.06.000649ConsensunsКонсенсусResearch ArticleThe experience of using the Russian biosimilar of the original drug eculizumab for the treatment of patients with atypical hemolytic-uremic syndromeОпыт применения российского биоаналога оригинального препарата экулизумаба для лечения больных атипичным гемолитико-уремическим синдромомhttps://orcid.org/0000-0002-3073-2785LavrishchevaYulia V.ЛаврищеваЮлия Владимировна
Russian Federation

врач-нефролог

lavrischeva@gmail.comhttps://orcid.org/0000-0003-1045-9336YakovenkoAlexander A.ЯковенкоАлександр Александрович
Russian Federation

канд. мед. наук, доц. каф. нефрологии и диализа

lavrischeva@gmail.comKudlaiDmitrii A.КудлайДмитрий Анатольевич
Russian Federation

д-р мед. наук, ген. дир.

lavrischeva@gmail.comAlmazov National Medical Research CentreФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава РоссииPavlov First Saint Petersburg State Medical UniversityФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Минздрава РоссииJSC GENERIUMАО «ГЕНЕРИУМ»09072020926768008072020Copyright ©; 2020, Consilium MedicumCopyright ©; 2020, ООО "Консилиум Медикум"2020Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/35000

Atypical hemolytic-uremic syndrome (aHUS) is a chronic systemic disease of a genetic nature, which is based on uncontrolled activation of the alternative complement pathway, leading to generalized thrombosis in the vessels of the microvasculature (complement-mediated thrombotic microangiopathy). To date, therapy with eculizumab is the most effective and pathogenetically substantiated method of treating patients with ASH. Using the example of three clinical cases of patients with a verified diagnosis of aHUS, the high efficiency and safety of the world’s first bioanalogue of eculizumab in the treatment of adult patients with aHUS (complement-mediated thrombotic microangiopathy) was demonstrated.

Атипичный гемолитико-уремический синдром (аГУС) – хроническое системное заболевание генетической природы, в основе которого лежит неконтролируемая активация альтернативного пути комплемента, ведущая к генерализованному тромбообразованию в сосудах микроциркуляторного русла (комплемент-опосредованная тромботическая микроангиопатия). На сегодняшний день наиболее эффективным и патогенетически обоснованным методом лечения больных аГУС является терапия препаратом экулизумаб. На примере трех клинических случаев пациентов с верифицированным диагнозом аГУС продемонстрированы высокая эффективность и безопасность первого в мире биоаналога экулизумаба в лечении взрослых пациентов с аГУС (комплемент-опосредованной тромботической микроангиопатией).

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