Modern Approaches in Management of Children with Cystic fibrosis

Баранов, А. А.; Namazova-Baranova, Leyla; Куцев, С. И.; Avdeev, S. N.; Полевиченко, Е. В.; Белевский, А. С.; Kondratyeva, E.I.; Симонова, О.И.; Kashirskaya, N.; Sherman, V.D.; Voronkova, A.; Амелина, Е. Л.; Gembitskaya, T. E.; Krasovsky, S.; Chermensky, A. G.; Stepanenko, Tatyana A.; Selimzyanova, L.R.; Вишнева, Е.А.; Gorinova, Yu.V.; Roslavtseva, Е.А.; Asherova, I.; Ильенкова, Н. А.; Zyryanov, S. К.; Odinaeva, N. D.; Maksimycheva, T.Yu.; Орлов, А. В.; Semykin, S. Yu.; Chernukha, M.; Shaginyan, I.A.; Avetisyan, L.; Шумкова, Г. Л.; Крылова, Н. А.; Dronov, I.A.; Костылева, М. Н.; Желенина, Л. А.; Климко, Н Н; Borzova, Yu. V.; Vasilyeva, N. V.; Bogomolova, T. S.; Speranskaya, A.S.; Баранова, И. А.; Furman, E. G.; Shadrina, V.; Shchapov, Nikolay F.; Petrova, N.V.; Пашков, И. В.; Цирульникова, О. М.; Polyakov, D. S.; Свистушкин, В. М.; Синьков, Э. В.; Черных, В. Б.; Репина, С. А.; Благовидов, Д. А.; Kostinov, M.P.; Kondratenko, O.; Lyamin, A. V.; Поликарпова, С. В.; Polyakov, A. V.; Adyan, T.; Goldshtein, D. V.; Бухарова, Т. Б.; Ефремова, А.; Ovsyankina, E.S.; Panova, Ludmila V.; Черкашина, И. И.

doi:10.15690/pf.v19i2.2417

Cited by 7 publications

(1 citation statement)

References 193 publications

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“…Основными методами визуализации легких в традиционной практике являются рентгенография (РГ) и компьютерная томография (КТ) [2], по данным которых возможно выявить признаки поражения легочной ткани в виде деформации легочного рисунка, пневмофиброза, перибронхиальной инфильтрации, консолидации и ателектазов, бронхоэктазов и булл, а также определить признаки бронхиальной обструкции -локальные участки вздутия легочной ткани, увеличение ретростернального пространства, уплощение диафрагмы. Метод КТ с учетом возможности послойной визуализации в значительной степени превосходит рутинную РГ в локализации и детализации спектра поражений легочной ткани [3].…”

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The first experience of using magnetic resonance tomography for lung imaging in children with cystic fibrosis: opportunities and prospects

Mustafin,

Kustova,

Simonova

et al. 2024

Pulʹmonologiâ (Mosk.)

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Cystic fibrosis (CF) is a hereditary disease associated with systemic failure of the exocrine glands. In 95% of the cases, progressive damage to the lung tissue occurs that requires follow-up. The main imaging methods are radiography and computed tomography (CT). Both methods involve ionizing radiation. According to clinical guidelines, a chest X-ray is performed no more than once every 2 years. However, given the high risk of developing inflammatory processes in children with CF, the frequency of radiological examinations may increase significantly. The search for alternative methods without ionizing radiation is in great demand among children with CF. One of these methods is magnetic resonance imaging (MRI).The aim was to explore the capabilities of MRI for imaging lung tissue in children with CF.Methods. The study included 12 patients aged 7 to 18 years with newly diagnosed and previously diagnosed CF. All children underwent CT and MRI of the chest.Results. In all children (100%), chest CT revealed a complex of changes typical of CF: signs of chronic bronchitis, widespread saccular and cylindrical bronchiectasis of various localizations, areas of pulmonary fibrosis, infiltrative changes in the lung tissue. Chest MRI is most effective (100%) in identifying areas of pulmonary fibrosis (thickening of the lung tissue), accumulation of exudate in the lumen of bronchiectasis, and infiltrative changes. In contrast to CT, MRI is less effective in the assessment of bronchitis changes with thickening of the bronchial walls without exudate (83%). A low efficiency of MRI is observed in the visualization of bronchiectasis with a free lumen (without signs of mucostasis).Conclusion. Radiography and computed tomography remain the main methods for lung imaging in children with CF. However, taking into account the absence of ionizing radiation, the development of the method and the emergence of new sequences, chest MRI can be used effectively for follow-up instead of radiography and CT, reducing the radiation dose received.

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The first experience of using magnetic resonance tomography for lung imaging in children with cystic fibrosis: opportunities and prospects

Mustafin,

Kustova,

Simonova

et al. 2024

Pulʹmonologiâ (Mosk.)

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The Risk of Developing of Malnutrition and the Principles of Correction of Nutritional Status Disorders in Children with Cystic Fibrosis

Kleshchenko,

Shimchenko,

Komarov

et al. 2023

Pediatr. farmakol.

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Cystic fibrosis is a severe hereditary disease with polysystemic manifestations and progressive course. Malnutrition in cystic fibrosis occurs as a result of exocrine insufficiency of the pancreas, an increase in energy losses in chronic inflammation in the bronchopulmonary system, manifested by increased stress on the respiratory system. The presented literature review highlights the modern principles of prevention and correction of malnutrition in children with cystic fibrosis, identifies the most promising methods for further development that correct nutritional status disorders. The review has shown that an active approach to nutrition at any age, the use of aggressive methods of nutritional support against the background of enzyme replacement therapy, timely and adequate therapy of respiratory tract pathology lead to an improvement in the indicators of nutritional status in cystic fibrosis. The most promising is the further development of targeted therapy, which allows, as a result of exposure to the etiopathogenetic mechanisms of the disease, to reduce the frequency and severity of bronchopulmonary exacerbations, partially restore the exocrine function of the pancreas, which is manifested in patients with cystic fibrosis by an increase in body weight and mass-growth index.

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Method for the prevention of hospital infection in Saint Petersburg

Орлов

Антонович²,

Matveev³

et al. 2022

Medical academic journal

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BACKGROUND: Cystic fibrosis is one of the most frequent monogenic diseases, in which, in addition to medical measures, measures for the prevention of the infectious process in the lungs are of great importance, which is provided by a clear organization of the work of any medical institution where patients are observed and where medical and rehabilitation measures are carried out. AIM: The article describes the experience of the childrens Center in St. Petersburg to prevent cross-infection of patients with cystic fibrosis who have the different luge microbiome. MATERIALS AND METHODS: The measures taken included a system for monitoring and dynamic control of the seeded pathogenic microflora with a strict separation of patients with cystic fibrosis, who had the isolation and carriage of various pathogens. The system provided for the separation of flows of patients with cystic fibrosis and the prevention of cross-infection, as well as a number of activities related to the disinfection of equipment. RESULTS: The methods adopted and put into practice have ensured the prevention of cross-infection of patients with cystic fibrosis, excreting various pathogenic bacteria from sputum. The patient management system made it possible to halve the number of hospitalizations of patients with Pseudomonas aeruginosa infection per CF patient per year: from 1.3 in 2001 to 0.6 in 2021, reduce the risk of cross-infection, reduce the number of relapses, hospitalizations and the average length of stay of a patient in a hospital. CONCLUSIONS: The developed system of assistance has shown its effectiveness and can be implemented in one form or another in the work of cystic fibrosis treatment centers in the Russian Federation.

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Modern Approaches in Management of Children with Cystic fibrosis

Cited by 7 publications

References 193 publications

The first experience of using magnetic resonance tomography for lung imaging in children with cystic fibrosis: opportunities and prospects

The first experience of using magnetic resonance tomography for lung imaging in children with cystic fibrosis: opportunities and prospects

The Risk of Developing of Malnutrition and the Principles of Correction of Nutritional Status Disorders in Children with Cystic Fibrosis

Method for the prevention of hospital infection in Saint Petersburg

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