medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2026-215medsovet-10261Research ArticleМУКОВИСЦИДОЗCYSTIC FIBROSISНеинвазивная вентиляция легких при муковисцидозеNon-invasive ventilation in cystic fibrosishttps://orcid.org/0000-0002-5804-8643МукатоваИ. Ю.MukatovaI. Yu.

Мукатова Ирина Юрьевна - д.м.н., профессор, профессор кафедры внутренних болезней с курсом нефрологии, гематологии, аллергологии, иммунологии.

010000, Астана, ул. Бейбитшилик, д. 49а

Irina Yu. Mukatova - Dr. Sci. (Med.), Professor, Professor of Department of Internal Illnesses with a Course of Nephrology, Hematology, Allergology, Immunology.

49a, Beybitshilik St., Astana, 010000

irinamukatova24@gmail.comhttps://orcid.org/0009-0002-7864-2399СериковаА. С.SerikovaA. S.

Серикова Аурини Сериковна - докторант кафедры внутренних болезней с курсом нефрологии, гематологии, аллергологии, иммунологии.

010000, Астана, ул. Бейбитшилик, д. 49а

Aurini S. Serikova - Student of Department of Internal Illnesses with a Course of Nephrology, Hematology, Allergology, Immunology.

49a, Beybitshilik St., Astana, 010000

auriniserikova@gmail.comhttps://orcid.org/0000-0002-5356-9415АмелинаЕ. Л.AmelinaE. L.

Амелина Елена Львовна - к.м.н., ведущий научный сотрудник лаборатории муковисцидоза, Научно-исследовательский институт пульмонологии Федерального медико-биологического агентства; Медицинский университет Астана.

115682, Москва, Ореховый бульвар, д. 28, стр. 10; 010000, Астана, ул. Бейбитшилик, д. 49а

Elena A. Amelina - Cand. Sci. (Med.), Leading Researcher, Cystic Fibrosis Laboratory, Research Institute for Pulmonology of the Federal Medical Biological Agency; AMU.

28, Bldg. 10, Orekhovy Boulevard, Moscow, 115682; 49a, Beybitshilik St., Astana, 010000, Kazakhstan

eamelina@mail.ruhttps://orcid.org/0000-0002-4726-4906НуралиеваГ. С.NuralievaG. S.

Нуралиева Галия Сериковна - к.м.н., доцент кафедры пульмонологии.

119048, Москва, ул. Трубецкая, д. 8, стр. 2

Galiya S. Nuralieva - Cand. Sci. (Med.), Associate Professor, Department of Pulmonology.

8, Bldg. 2, Trubetskaya St., Moscow, 119048

galia32@yandex.ruМедицинский университет АстанаРоссияAstana Medical UniversityRussian FederationМедицинский университет Астана; Научно-исследовательский институт пульмонологии Федерального медико-биологического агентстваРоссияAstana Medical University; Research Institute for Pulmonology of the Federal Medical Biological AgencyRussian FederationПервый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)РоссияSechenov First Moscow State Medical University (Sechenov University)Russian Federation20261407202609174178Copyright © Мукатова И.Ю., Серикова А.С., Амелина Е.Л., Нуралиева Г.С., 20262026Мукатова И.Ю., Серикова А.С., Амелина Е.Л., Нуралиева Г.С.Mukatova I.Y., Serikova A.S., Amelina E.L., Nuralieva G.S.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/10261

Муковисцидоз (МВ, кистозный фиброз) – аутосомно-рецессивное моногенное наследственное заболевание, характеризующееся дисфункцией экзокринных желез с поражением жизненно важных органов и систем, в первую очередь – респираторной системы и системы пищеварения. МВ ассоциируется с сокращением продолжительности жизни, наиболее распространенной причиной смерти является прогрессирующее поражение легких с развитием дыхательной недостаточности (ДН). Именно ДН чаще всего определяет прогноз МВ, поэтому необходимы своевременная диагностика и коррекция этого осложнения. По изменению газового состава артериальной крови различают 2 типа ДН: гипоксемическая и гиперкапническая. С учетом типов ДН определяется вид респираторной поддержки: кислородотерапия и/или неинвазивная вентиляция легких (НВЛ). НВЛ оказывает положительное влияние на функцию легких, показатели газового состава артериальной крови, массу тела, длительность и качество жизни пациентов с тяжелым течением МВ. К позитивным эффектам НВЛ также можно отнести эффект улучшения дренажа мокроты за счет значительного улучшения коллатеральной вентиляции. В последние годы в связи с введением таргетной терапии клиническая картина пациентов с МВ изменилась за счет уменьшения частоты обострений хронического бронхита, улучшения функциональных показателей, замедления деградации легочной функции. Однако применение НВЛ у пациентов с МВ остается актуальным, в тех случаях, где имеет место хроническая гиперкапническая ДН, сформировались выраженные бронхолегочные изменения в виде распространенных бронхоэктазов, хронического, часто рецидивирующего инфекционного процесса и выраженного нарушения дренажа мокроты. НВЛ также показана, когда таргетная терапия не может быть назначена в связи с отсутствием соответствующих мутаций в гене CFTR или если у пациентов с МВ нет доступа к таргетной терапии. В связи с этим накопление опыта проведения НВЛ у пациентов с МВ является актуальной задачей.

Cystic fibrosis (CF) is an autosomal recessive monogenic hereditary disease characterized by dysfunction of the exocrine glands with damage to vital organs and systems, primarily the respiratory and digestive systems. Cystic fibrosis is associated with a reduction in life expectancy, the most common cause of death is progressive lung damage with the development of respiratory failure (RF). It is RF that most often determines the prognosis of CF, so timely diagnosis and correction of this complication is necessary. According to changes in the gas composition of arterial blood, there are 2 types of respiratory failure: hypoxemic and hypercapnic. Taking into account the types of RF, the type of respiratory support is determined: oxygen therapy and / or non-invasive ventilation (NIV). NIV has a positive effect on lung function, arterial blood gas parameters, body weight, duration and quality of life of patients with severe CF. Positive effects of NIV also include the effect of improving sputum drainage due to significant improvement of collateral ventilation. In recent years, due to the introduction of targeted therapy, the clinical picture of patients with CF has changed due to a decrease in the frequency of exacerbations of chronic bronchitis, improvement of functional indicators, and slowing down of the degradation of pulmonary function. However, the use of NIV in patients with CF remains relevant in cases where there is chronic hypercapnic respiratory failure, pronounced bronchopulmonary changes have formed in the form of widespread bronchiectasis, chronic, frequently recurring infectious process and pronounced impairment of sputum drainage. NIV is also indicated when targeted therapy cannot be prescribed due to the absence of corresponding mutations in the CFTR gene or if patients with CF do not have access to targeted therapy. In this regard, the accumulation of experience in NIV in patients with CF is an urgent task.

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