pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2026-36-2-262-271pulmo-4928Research ArticleОБЗОРЫREVIEWСостав тела больных муковисцидозом: его влияние на показатели заболеваемости и выживаемости, динамика на фоне таргетной терапииBody composition in patients with cystic fibrosis, its impact on morbidity and survival rates, and its changes during targeted therapyhttps://orcid.org/0000-0002-3788-0887КрыловаН. А.KrylovaN. A.

Крылова Наталья Анатольевна — врач-диетолог, научный сотрудник лаборатории муковисцидоза 

115682, Москва, Ореховый бульвар, 28, стр. 10

Natalia A. Krylova, Nutritionist, Researcher, Cystic Fibrosis Laboratory

Orekhovyy bul’var 28, build. 10, Moscow, 115682

nataliya_2007_78@mail.ruhttps://orcid.org/0000-0002-5356-9415АмелинаЕ. Л.AmelinaE. L.

Амелина Елена Львовна — к. м. н., ведущий научный сотрудник лабора­тории муковисцидоза 

115682, Москва, Ореховый бульвар, 28, стр. 10

Author ID: 7003985681

Elena L. Amelina, Candidate of Medicine, Leading Researcher, Cystic Fibro­sis Laboratory

Orekhovyy bul’var 28, build. 10, Moscow, 115682

Scopus Author ID: 7003985681

eamelina@mail.ruhttps://orcid.org/0000-0001-9642-0947КрасовскийС. А.KrasovskiyS. A.

Красовский Станислав Александрович — к. м. н., старший научный со­трудник, исполняющий обязанности заведующего лабораторией му­ковисцидоза Федерального государственного бюджетного учреждения «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства; ведущий научный сотрудник науч­но-клинического отдела Федерального государственного бюджетного научного учреждения «Медико-генетический научный центр имени академика Н.П. Бочкова»

115682, Москва, Ореховый бульвар, 28, стр. 10,

115522, Москва, ул. Москворечье, 1

Author ID: 688178

Stanislav А. Krasovskiy, Candidate of Medicine, Senior Researcher, Acting Head of the Cystic Fibrosis Laboratory, Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Bio­logical Agency of Russian Federation; Leading Researcher, Scientific and Clini­cal Department, Research Centre for Medical Genetics

Orekhovyy bul’var 28, build. 10, Moscow, 115682, 

ul. Moskvorechye 1, Moscow, 115522

Scopus Author ID: 688178

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

Кагазежев Резуан Уматиевич — аспирант лаборатории муковисцидоза 

115682, Москва, Ореховый бульвар, 28, стр. 10

Rezuan U. Kagazezhev, Postgraduate Student, Cystic Fibrosis Laboratory

Orekhovyy bul’var 28, build. 10, Moscow, 115682

kagazezhevru.64@gmail.comhttps://orcid.org/0000-0002-8260-7126АфанасьеваМ. В.AfanasyevaM. V.

Афанасьева Мария Валерьевна — научный сотрудник лаборатории му­ковисцидоза Федерального государственного бюджетного учреждения «Научно-исследовательский институт пульмонологии» Федерального ме­дико-биологического агентства России; врач-пульмонолог центра диагно­стики и лечения муковисцидоза у взрослого населения Государственного бюджетного учреждения здравоохранения Московской области «Москов­ский областной научно-исследовательский клинический институт имени М.Ф. Владимирского»

115682, Москва, Ореховый бульвар, 28, стр. 10,

129110, Москва, ул. Щепкина, 61/2

Maria V. Afanaseva, Researcher, Cystic Fibrosis Department, Federal State Budgetary Institution «Pulmonology Scientific Research Institute» under Fed­eral Medical and Biological Agency of Russian Federation; Pulmonologist, Center for Diagnosis and Treatment of Cystic Fibrosis in Adults, Moscow Regional Research and Clinical Institute (“MONIKI”)

Orekhovyy bul’var 28, build. 10, Moscow, 115682, 

ul. Shchepkina 61/2, Moscow, 129110

usa-mariya@yandex.ruФедеральное 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 FederationФедеральное 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; Research Centre for Medical GeneticsRussian FederationФедеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии» Федерального медико-биологического агентства России; Государственное бюджетное учреждение здравоохранения Московской области «Московский областной научно-исследовательский клинический институт имени М.Ф. Владимирского»РоссияFederal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation; Moscow Regional Research and Clinical Institute (“MONIKI”)Russian Federation202617042026362262271Copyright © Крылова Н.А., Амелина Е.Л., Красовский С.А., Кагазежев Р.У., Афанасьева М.В., 20262026Крылова Н.А., Амелина Е.Л., Красовский С.А., Кагазежев Р.У., Афанасьева М.В.Krylova N.A., Amelina E.L., Krasovskiy S.A., Kagazezhev R.U., Afanasyeva M.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/4928

По результатам исследований последних лет показано, что при оценке состояния питания людей с муковисцидозом (МВ) необходимо оценивать не только индекс массы тела (ИМТ), но и состав тела. Расчет жировой (ЖМТ) и безжировой (БЖМТ) массы тела (МТ) не менее важен, поскольку связь этих характеристик с показателями респираторной функции сильнее, чем с традиционными антропомет­рическими параметрами. Низкий или нормальный ИМТ может маскировать высокую ЖМТ или низкую безжировую (мышечную) МТ. Сообщается о положительном влиянии таргетной терапии на ИМТ, но влияние патогенетической терапии МВ на состав тела изучен недостаточно.

Целью обзора являлось ознакомление с результатами исследований по оценке состава тела у пациентов с МВ. В обзоре изложены данные о методах и преимуществах оценки состава тела перед традиционными антропометрическими измерениями, особен­ностях состава тела у пациентов с МВ, влиянии компонентов тела, в частности ЖМТ и БЖМТ, на респираторную функцию, особенно­стях изменения состава тела на фоне таргетной терапии и возникновения кардиометаболических рисков.

Материалы и методы

Материалы и методы. Изучены доступные публикации базы данных PubMed, отобранные в результате поиска по следующим ключевым словам: «состав тела у пациен­тов с муковисцидозом», «состав тела у пациентов с муковисцидозом на фоне таргетной терапии».

Заключение

Заключение. Оценка состава тела может стать важным инструментом мониторинга нутритивного статуса пациента, оценки эффективности патогенетической терапии, а также руководством в коррекции диеты и подбора препаратов лечебного питания для пациентов с МВ.

Recent research findings indicate that assessment of the nutritional status of people with cystic fibrosis (CF) should account for not only BMI but also body composition. Estimating fat and lean mass is equally important because these characteristics are associated with respiratory function indicators stronger than traditional anthropometric parameters. A low or normal BMI may mask high fat mass or low lean (muscle) mass. Researchers report a positive effect of targeted therapy on BMI, but the impact on body composition has not been adequately studied.

The aim of this paper was to review the results of studies assessing body composition in patients with cystic fibrosis. The review presents data on methods for assessing body composition, the advantages of body composition assessment over traditional anthropometric measurements, body composition characteristics in patients with cystic fibrosis, the impact of body components, in particular fat and lean mass, on respiratory function, changes in body composition during targeted therapy, and the emergence of cardiometabolic risks.

Methods

Methods. We selected publications in the PubMed database using the following keywords: “body composition in patients with cystic fibrosis” and “body composition in patients with cystic fibrosis during targeted therapy”.

Conclusion

Conclusion. Body composition assessment can be an important tool for monitoring patient’s nutritional status, assessing effectiveness of targeted therapy, and guiding dietary modifications and selection of therapeutic nutritional supplements for patients with cystic fibrosis.

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