Difficulties in providing respiratory support to children with neuromuscular diseases
- Authors: Rastegina S.E.1, Vlodavets D.V.1, Mizernitsky Y.L.1
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Affiliations:
- Pirogov Russian National Research Medical University
- Issue: Vol 36, No 4 (2025)
- Pages: 31-34
- Section: Problem
- URL: https://journals.eco-vector.com/0236-3054/article/view/679099
- DOI: https://doi.org/10.29296/25877305-2025-04-06
- ID: 679099
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Abstract
Neuromuscular diseases (NMD) are a heterogeneous group of diseases. They are characterized by damage of different structures in patients, including motor neurons, peripheral nerves, neuromuscular junctions, or the muscles. As the disease progresses, the respiratory muscles are usually involved in the pathological process, which puts patients at risk of developing alveolar hypoventilation and respiratory failure. If patients have respiratory system disorders or there is a high risk of developing these disorders, such patients need therapy aimed at correcting such conditions. Difficulties in providing medical care to such children can lead to the development of life-threatening conditions.
This article reviews clinical cases of patients with NMD and respiratory system disorders.
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About the authors
S. E. Rastegina
Pirogov Russian National Research Medical University
Author for correspondence.
Email: svtrjbv@yandex.ru
ORCID iD: 0000-0002-3646-7062
Russian Federation, Moscow
D. V. Vlodavets
Pirogov Russian National Research Medical University
Email: svtrjbv@yandex.ru
ORCID iD: 0000-0003-2635-2752
SPIN-code: 2657-4710
Candidate of Medical Sciences
Russian Federation, MoscowYu. L. Mizernitsky
Pirogov Russian National Research Medical University
Email: svtrjbv@yandex.ru
ORCID iD: 0000-0002-0740-1718
SPIN-code: 6135-5260
MD, Professor
Russian Federation, MoscowReferences
- Рябых С.О., Филатов Е.Ю., Очирова П.В., и др. Нервно-мышечный сколиоз. Клинические рекомендации, 2023. [Ryabykh S.O., Filatov E.Yu., Ochirova P.V. et al. Neuromuscular scoliosis. Clinical guidelines, 2023 (in Russ.)].
- Рушкевич Ю.Н., Галиевская О.В., Чечик Н.М. и др. Значение исследования функции внешнего дыхания у пациентов с нервно-мышечными заболеваниями. Медицинские новости. 2020; 2: 22–5 [Rushkevich Yu.N., Galievskaya O.V., Chechik N.M. et al. Importance of the study of respiratory function in patients with neuromuscular diseases. Meditsinskie novosti. 2020; 2: 22–5 (in Russ.)].
- Fauroux B., Amaddeo A., Quijano-Roy S. et al. Respiratory insight to congenital muscular dystrophies and congenital myopathies and its relation to clinical trial. Neuromuscul Disord. 2018; 28 (9): 731–40. doi: 10.1016/j.nmd.2018.06.013
- Растегина С.Е., Влодавец Д.В., Мизерницкий Ю.Л. Функциональное состояние респираторной системы при проксимальной спинальной мышечной атрофии 5q: от естественной истории течения заболевания к эре патогенетической терапии. Проблемы и ожидания. Российский вестник перинатологии и педиатрии. 2024; 69 (4): 16–30 [Rastegina S.E., Vlodavets D.V., Mizernitskiy Yu.L. The functional state of the respiratory system of the patients with proximal spinal muscular atrophy 5q (SMA 5q): from the natural history of the disease to the era of pathogenetic therapy. Problems and expectations. Russian Bulletin of Perinatology and Pediatrics. 2024; 69 (4): 16–30 (in Russ.)]. doi: 10.21508/1027-4065-2024-69-4-16-30
- Рушкевич Ю.Н., Галиевская О.В., Куликова Ю.С. и др. Неинвазивная вентиляция легких при нервно-мышечных болезнях. Журнал неврологии и психиатрии им. С.С. Корсакова. 2021; 121 (8): 77–82 [Rushkevich Yu.N., Haliyeuskaya O.V., Kulikova Yu.S. et al. Non-invasive ventilation of the lungs in neuromuscular diseases. S.S. Korsakov Journal of Neurology and Psychiatry. 2021; 121 (8): 77–82 (in Russ.)]. doi: 10.17116/jnevro202112108177
- Халл. Д., Аниаправан Р., Чан Э., и др. Руководство по респираторной поддержке детей с нервномышечными заболеваниями, 2015; с. 207 [Hull D., Aniapravan R., Chan E. Chetwin M. et al. Guide to Respiratory Support for Children with Neuromuscular Diseases, 2015; р. 207 (in Russ.)].
- De Vito EL. When should and when should not use oxygen in neuromuscular diseases? Medicina (B Aires). 2022; 82 (2): 244–8.
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