The article presents current literature data from English-language (search made in PubMed) and Russian-language (search made in eLibrary) sources about the method for measurement of the lung diffusing capacity for carbon monoxide in children with cystic fibrosis (CF). This review presents the general characteristics of this test in children and adults with various diseases, and also describes the conflicting results of studies using this method in CF patients of various ages and disease severity. The expediency, the informativeness, and difficulties of evaluation and interpretation of the test’s results, depending on the age and course of the disease of patients, are discussed. The author concludes that further research of interpretation of indicators determined during the test of the lung diffusing capacity for carbon monoxide, such as the transfer factor, alveolar volume, and their ratio in CF children is necessary.
The article describes the features of mucolytic therapy in patients with cystic fibrosis, as the leading pathogenetic mechanism of the disease lies in the compromised mucociliary transport due to the presence of viscous secretions. Particular attention is paid to the key mucolytic drug (endonuclear mucolytic) dornase alfa, which has three clinical effects: powerful mucolytic, anti-inflammatory and antibacterial. The extracellular DNA cleavage in secretions results in a decrease of bronchial mucus viscosity, and a decrease in elastase and IL-8 concentrations in sputum results in the reduction of lung tissue destruction in chronic bacterial inflammation. The authors pay attention to the adherence to the drug policy, possible dosing regimens and drug use variants to achieve a clinical effect. Also, the article extensively covers the results of international clinical studies on the use of dornase alfa in cystic fibrosis. The authors presented their own clinical observation – the experience of clinical and functional control over the efficacy of the drug when comparing two groups of children with cystic fibrosis: those who received (2018–2021) and those who did not receive dornase alfa (1995–1998 – before it was introduced into clinical practice). The remarkable thing is that the results of FEV1, MEF 50, MEF 75, PEF were significantly higher in the group of modern children with cystic fibrosis, who receive dornase alfa continuously as part of the backbone therapy, than in children of the same age 25 years ago. It has been substantiated that dornase alfa is one of the main components of the backbone therapy for patients with cystic fibrosis, which should be prescribed on an ongoing basis after the diagnosis has been established, under the functional control of its efficacy
Introduction. Progression of pulmonary and heart failure often causes death in patients with cystic fibrosis (95%). Therefore, monitoring of lung condition is very important for patients with cystic fibrosis (CF). Structural changes are visualized by computed tomography of the chest (CT) and are scored using the Brody scale. For children older than 5 years, pulmonary function tests (PFTs) tests (which are evaluated in percent of predicted (%) values) are available, such as spirometry, body plethysmography (BP), diffusion capacity of the lungs (DL) test. The results of the single-breath (SB) DL for carbon monoxide test are DLCO-SB, alveolar volume (VA-SB) and their ratio (KCO-SB). In the presence of non-communicative zones for gas perfusion, VA-SB may not present true VA. For patients with CF, it is proposed to use VA determined by BP for calculating KCO-BP, or to adjust the predicted DLCO-SB and KCO-SB for VA.Aim. To assess the informativeness DLCO-SB, KCO-SB and KCO-BP in children with CF.Materials and methods. 28 children with CF (8–18 years old) were examined in the department of pulmonology of the National Research Center for Children’s Health. PFTs included DLCO-SB, spirometry and BP. Additionally, we evaluated the data of the blood gas and acid-base values, age, body mass index and CT scores.Results and discussion. We found that in most patients DLCO-SB and KCO-SB were within the normal range, and decreased in children older than 14 years with background of severe bronchiectasis.Conclusion. Thus, in children with CF the DL test is informative, and adjustment for VA is useful.
The progression of lung failure in children with cystic fibrosis (CF) is associated with chronic lung infection (Staphylococcus aureus, Pseudomonas aeruginosa, Achromobacter spp., etc.). Functional pulmonary tests (PFTs), spirometry and body plethysmography, computed tomography (CT) of the lungs and analysis of the lung microbiota are used for monitoring of lung condition of CF patients. Several studies have been devoted to assessing the correlation of structural changes in CT and pulmonary function tests (PFT), but at the moment there is not enough data on the relationship of these indicators and their differences depending on the respiratory microbiota in CF children in the Russian population. Materials and methods. Data was collected for CF 8–17.9 years children patients. We retrospectively analyzed genotype, body mass index, results of PFTs and CT scan of the chest (score by the Brody scale), deep throat cultures in all patients, and the capillary blood gas — in 56 children. Results. Significant correlations between functional tests and structural changes in the lungs were found. A trend towards impairment of PFTs and CT scores with age due to infectious pathogens was shown, and the most significant negative impact was exerted by the mucoid species Pseudomonas aeruginosa and, especially, Achromobacter spp. The last one was associated with the worst lung parameters in CF children. Conclusion. Pirometry, body plethysmography, and CT of the lungs are necessary for a comprehensive assessment of the lung condition, and a study of the lung microbiome due to its influence on structural and function changes in patients with CF.
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