pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2023-33-6-781-791pulmo-4396Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL STUDIESОпыт применения генерического препарата элексакафтор / тезакафтор / ивакафтор + ивакафтор у пациентовUsing generic drug elexacaftor/tezacaftor/ivacaftor+ ivacaftor in patients with cystic fidosis in routine clinical practicehttps://orcid.org/0000-0001-9642-0947КрасовскийС. А.KrasovskiyS. A.

Красовский Станислав Александрович – кандидат медицинских наук., старший научный сотрудник лаборатории муковисцидоза Научно-исследовательский институт пульмонологии; ведущий научный сотрудник научно-клинического отдела Медико-генетический научный центр имени академика Н.П. Бочкова; Author ID: 688178.

115682, Москва, Ореховый бульвар, 28; 115522, Москва, ул. Москворечье, 1; тел: (495) 111-03-03

Stanislav А. Krasovskiy - Candidate of Medicine, Senior Researcher, Cystic Fibrosis Laboratory, Federal Pulmonology Research Institute, FMBAR; Leading Researcher, Scientific and Clinical Department, FSBSI “RCMG”; Author ID: 688178

Orekhovyy bul’var 28, Moscow, 115682; Ul. Moskvorechye 1, Moscow, 115522; tel.: (495) 111-03-03

sa_krasovsky@mail.ruhttps://orcid.org/0009-0009-2500-663XКагазежевР. У.KagazezhevR. U.

Кагазежев Резуан Уматиевич – аспирант лаборатории муковисцидоза

115682, Москва, Ореховый бульвар, 28; тел. (495) 965-23-24

Rezuan U. Kagazezhev - Postgraduate student, Cystic Fibrosis Laboratory.

Orekhovyy bul’var 28, Moscow, 115682; tel. (495) 965-23-24

kagazezhevru.64@gmail.comФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства России; Федеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н.П. Бочкова» Министерства науки и высшего образования Российской ФедерацииРоссияFederal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation; Federal State Budgetary Scientific Institution “Research Centre for Medical Genetics”, Ministry of Science and Higher Education of the Russian FederationRussian FederationФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства РоссииРоссияFederal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian FederationRussian Federation202312122023336781791Copyright © Красовский С.А., Кагазежев Р.У., 20232023Красовский С.А., Кагазежев Р.У.Krasovskiy S.A., Kagazezhev R.U.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/4396

Муковисцидоз (МВ) – системная экзокринопатия с прогрессирующим течением. Кроме симптоматической терапии в последние годы на первый план выступает таргетное (патогенетическое) лечение, направленное на коррекцию функции хлорного канала. Наибольшая эффективность продемонстрирована при применении тройной комбинации элексакафтор (ELX) / тезакафтор (TEZ) / ивакафтор (IVA) + IVA (препарат Трикафта® (Вертекс Фармасьютикалс, США), международное непатентованное наименование (МНН) – ELX / TEZ / IVA + IVA), который стал «золотым стандартом» таргетной терапии. В настоящее время практически отсутствует информация об эффективности и безопасности генерических форм данного препарата.

Целью исследования явилась оценка эффективности и безопасности генерического препарата Трилекса® (Тютор С.А.С.И.Ф.И.А., Буэнос-Айрес, Аргентина) (МНН – ELX / TEZ / IVA + IVA), применяемого у взрослых пациентов с МВ в реальной клинической практике.

Материалы и методы

Материалы и методы. В 6-месячное исследование включены пациенты (n = 11) в возрасте от 18 до 46 лет с диагнозом МВ, которым назначено лечение генерическим препаратом патогенетической терапии ELX / TEZ / IVA + IVA. Анализировались показатели функции внешнего дыхания (форсированная жизненная емкость легких, объем форсированного выдоха за 1-ю секунду), проводимость электролитов потовой жидкости (потовый тест), антропометрические и другие клинико-функциональные данные.

Результаты

Результаты. По результатам исследования установлены выраженные положительные эффекты в отношении показателей дыхательной функции, потового теста, нутритивного статуса. Генерический лекарственный препарат ELX / TEZ / IVA + IVA хорошо переносится, отмечается клиническое улучшение в виде уменьшения интенсивности кашля, объема мокроты, улучшения переносимости ежедневной физической нагрузки, увеличения массы тела. Серьезных нежелательных явлений (НЯ) не зарегистрировано, так же, как и случаев прерывания лечения из-за развития НЯ.

Заключение

Заключение. В течение 6 мес. терапии комбинацией ELX / TEZ / IVA + IVA продемонстрированы положительная динамика и клинико-функциональная безопасность генерического препарата.

Cystic fibrosis (CF) is a multisystem disease of exocrine glands with a progressive course. In recent years, targeted (pathogenetic) treatment aimed at correcting the function of the chloride channel has come to the fore in addition to the symptomatic therapy. The greatest effectiveness was demonstrated with the use of the triple combination of the drug elexacaftor (ELX) / tezacaftor (TEZ) / ivacaftor (IVA) + IVA – Trikafta® (Vertex Pharmaceuticals, USA) that has become the “gold standard” of targeted therapy. Currently, there is virtually no information about the effectiveness and safety of the generic products. The aim of the study was to evaluate the efficacy and safety of the generic drug ELX/TEZ/IVA+IVA (Trilexa®) (Tutor S.A.S.I.F.I.A., Buenos Aires, Argentina) in adult patients with CF in real clinical practice.

Methods

Methods. The 6-month study included patients (n = 11) aged 18 to 46 years with a diagnosis of CF who were prescribed pathogenetic treatment with ELX/TEZ/IVA+IVA. Their external respiration function (forced vital capacity, forced expiratory volume in 1 second), conductivity of sweat fluid electrolytes (sweat test), anthropometric and other clinical and functional data were analyzed. Results. This study demonstrated pronounced positive effects in relation to indicators of respiratory function, sweat test, and nutritional status. The ELX/TEZ/IVA+IVA treatment was well tolerated, with clinical improvement in the form of a decrease in cough intensity, sputum volume, improvement in daily exercise tolerance, height, and body weight. No serious adverse events were recorded and none of the patients discontinued treatment due to adverse reactions.

Conclusion

Conclusion. Obvious clinical and functional positive dynamics and safety over 6 months were demonstrated with ELX/TEZ/IVA+IVA combination.

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