pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2025-35-3-332-339pulmo-4733Research ArticleОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯORIGINAL STUDIESПереносимость ингаляционного маннитола у детей с муковисцидозом: оценка первоначальной дозыTolerability of inhaled mannitol in children with cystic fibrosis: initiation dose assessmenthttps://orcid.org/0000-0002-8302-1207ГоряиноваА. В.GoryainovaA. V.

Горяинова Анастасия Васильевна – к. м. н., врач-педиатр, гастроэнтеролог, заместитель главного врача по работе с пациентами Клинического госпиталя «MD Group Мичуринский».

119607, Москва, Мичуринский пр-т, 31; тел.: (495) 292-49-64

Anastasia V. Goryainova - Candidate of Medicine, Pediatrician, Gastroenterologist, Deputy Chief Physician for Patient Care, “MD Group Michurinskiy” Clinical Hospital.

Michurinskiy pr. 31, Moscow, 119607; tel.: (495) 292-49-64

dr.goryainova@gmail.comhttps://orcid.org/0000-0003-1419-6756СемыкинС. Ю.SemykinS. Y.

Семыкин Сергей Юрьевич – к. м. н., заведующий педиатрическим отделением.

119571, Москва, Ленинский пр-т, 117, корп. 1; тел.: (495) 965-45-20

Sergey Yu. Semykin - Сandidate of Medicine, Head of the Pediatric Department.

tel.: (495) 965-45-20

dr.semykin@mail.ruhttps://orcid.org/0009-0005-8697-9196КазароваЛ. З.KazarovaL. S.

Казарова Лариса Замировна – врач-стажер, ординатор педиатрического отделения.

119571, Москва, Ленинский пр-т, 117, корп. 1; тел.: (495) 965-45-20

Larisa Z. Kazarova - Intern Physician, Resident Physician, Pediatric Department.

tel.: (495) 965-45-20

larisa123632@gmail.comhttps://orcid.org/0000-0003-0503-6371КаширскаяН. Ю.KashirskayaN. Yu.

Каширская Наталия Юрьевна – д. м. н., профессор, главный научный сотрудник лаборатории генетической эпидемиологии ФГБНУ Медико-генетический научный центр имени академика Н.П. Бочкова» Министерства науки и высшего образования РФ; профессор кафедры педиатрии ГБУ здравоохранения Московской области «Московский областной научно-исследовательский клинический институт имени М.Ф. Владимирского».

115522, Москва, ул. Москворечье, 1; 129110, Москва, ул. Щепкина, 61 / 2; тел.: (499) 320-60-90; Scopus ID: 6507308033; WoS Researcher ID: C-6404-2012; РИНЦ ID: 93625

Nataliya Yu. Kashirskaya - Doctor of Medicine, Professor, Chief Researcher, Laboratory of Genetic Epidemiology, FS Budgetary Scientific Institution “Research Centre for Medical Genetics”, Ministry of Science and Higher Education of the Russian Federation; Professor, Department of Pediatrics, State Budgetary Healthcare Institution of the Moscow Region “Moscow Regional Research and Clinical Institute (MONIKI)”.

Ul. Moskvorechye 1, Moscow, 115522; ul. Shchepkina 61/2, Moscow, 129110; tel.: (499) 320-60-90; Scopus ID: 6507308033; WoS Researcher ID: C-6404-2012; РИНЦ ID: 93625

kashirskayanj@mail.ruКлинический госпиталь «MD Group Мичуринский»Россия“MD Group Michurinskiy” Clinical HospitalRussian FederationРоссийская детская клиническая больница — филиал Федерального государственного автономного образовательного учреждения высшего образования «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияRussian Children’s Clinical Hospital, the Branch of the Russian National Research Medical University named after N.I. PirogovRussian FederationФедеральное государственное бюджетное научное учреждение «Медико-генетический научный центр имени академика Н.П. Бочкова» Министерства науки и высшего образования Российской Федерации; Государственное бюджетное учреждение здравоохранения Московской области «Московский областной научно-исследовательский клинический институт имени М.Ф. Владимирского»РоссияFederal State Budgetary Scientific Institution “Research Centre for Medical Genetics”, Ministry of Science and Higher Education of the Russian Federation; Moscow Regional Research and Clinical Institute (MONIKI)Russian Federation202525062025353332339Copyright © Горяинова А.В., Семыкин С.Ю., Казарова Л.З., Каширская Н.Ю., 20252025Горяинова А.В., Семыкин С.Ю., Казарова Л.З., Каширская Н.Ю.Goryainova A.V., Semykin S.Y., Kazarova L.S., Kashirskaya N.Y.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/4733

Несмотря на активное обсуждение в разных странах мира вопроса о возможном снижении объема базисной терапии у пациентов с муковисцидозом (МВ) на фоне применения таргетных препаратов, в частности муколитических, многие пациенты продолжают муколитическую ингаляционную терапию, включая маннитол. Целью исследования являлась ретроспективная оценка переносимости препарата – ингаляционного маннитола (Бронхитол Фармаксис, GEN Ilac, Турция) – у детей с МВ. Материалы и методы. Стационарное лечение в педиатрическом отделении Обособленного структурного подразделения «Российская детская клиническая больница» Федерального государственного бюджетного образовательного учреждения высшего образования «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской Федерации в 2021–2023 гг. получали дети с МВ (n = 142; из них 62 – мужского пола; возраст – 6–17 лет). Проводилась тестовая оценка переносимости первоначальной дозы ингаляционного маннитола (препарат Бронхитол) – Bronchitol Inhaled Dose Assessment (BIDA). Результаты. В подавляющем большинстве случаев продемонстрированы хорошая переносимость и выраженный муколитический эффект при использовании исследуемого препарата, а также единичные случаи (6,3 %) непереносимости в виде развития острого бронхообструктивного синдрома. Заключение. По результатам оценки эффекта первоначальной дозы ингаляционного маннитола на достаточно большой когорте детей разного возраста наглядно проиллюстрирован высокий профиль безопасности и эффективности исследуемого лекарственного средства.

Despite active discussion in different countries of the world about possible reduction of the amount of basic therapy of cystic fibrosis due to the use of CFTR-modulators, in particular mucolytics, many patients continue mucolytic inhalation therapy, including mannitol. The aim. A retrospective evaluation of the tolerability of the inhaled mannitol (Bronchitol Pharmaxis, GEN Ilac, Turkey) in children with cystic fibrosis (cwCF). Methods. 142 cwCF aged from 6 to 17 years (62 boys) who underwent inpatient treatment at the Separate Structural Unit Russian Children’s Clinical Hospital of the Federal State Autonomous Educational Institution of Higher Education “N.I. Pirogov Russian National Research Medical University” of the Ministry of Health of the Russian Federation from 2021 to 2023 were administered the initial dose assessment BIDA test. Results. Most cases demonstrated good tolerability and pronounced mucolytic effect when using the study drug. Isolated cases of intolerance (6.3%) in the form of acute bronchobstructive syndrome were observed. Conclusion. The presented evaluation of the effect of an initial dose of inhaled mannitol in a sufficiently large cohort of cwCF of different ages clearly illustrates the high safety and efficacy profile of the study drug.

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