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.

Objective. To analyze clinical and instrumental characteristics of sleep disorders in children with cardiomyopathies (CMPs). Patients and methods. We performed retrospective analysis of clinical, laboratory, and instrumental parameters in 107 children with CMPs aged 2 to 17 years treated in the National Medical Research Center of Children's Health in 2018–2019. The study sample was formed in accordance with inclusion criteria (confirmed diagnosis of CMP with functional class I or II, NYHA or Ross R.D.) and exclusion criteria (age <2 years, other heart and vascular diseases). We enrolled 26 children with hypertrophic CMP, 63 children with dilated CMP, and 18 children with unclassified CMP. According to the signs of sleep disorders (from sleep questionnaires filled in by parents), we formed 3 groups: patients with no sleep disorders (n = 40), patients with symptoms of insomnia/parasomnia (n = 26), and patients with indirect and/or direct signs of sleep apnea syndrome (SAS). We analyzed patients’ complaints, as well as clinical, instrumental (liver ultrasound, echocardiography, Holter ECG), and laboratory (glucose, cholesterol, alanine aminotransferase, and aspartate aminotransferase in serum) parameters. Results. Sleep disorders were identified in 63% of children: 58% had signs of insomnia/parasomnia and 38% had signs of SAS. In contrast to the questionnaires, medical records had information about sleep disorders only in two cases. Medical records primarily contained complaints of fatigue and reduced tolerance to physical activity (73%), excessive sweating (23%), and shortness of breath (17%). Patients with SAS usually had more complaints (according to their medical records), and their complaints were more diverse, including abnormal blood pressure, cephalgia, palpitations, and syncope. Body mass index (BMI) (p = 0.001) and serum glucose (p = 0.001) were higher in children with SAS than in children with normal sleep. Even after the exclusion of BMI, glucose levels (although being within the reference range) were still significantly higher in the SAS group (p = 0.020). The QTc interval at the maximum heart rate (HR) (p = 0.018) in children with sleep disorders was longer and had a positive correlation with serum glucose level (r = 0.195, p = 0.052). The analysis of echocardiography parameters (excluding the diagnosis factor) showed a smaller diameter of the pulmonary artery (p = 0.058) in children with SAS and correlation between right atrial remodeling and the factor of sleep disorder in children with various forms of CMP (p = 0.040). Conclusion. The analysis of sleep questionnaires revealed sleep disorders in 63% of children with CMP, including insomnia/parasomnia (24%) and/or SAS (38%). The presence of SAS was associated with a substantial number and variety of subjective complaints. The signs of myocardial electrical instability (longer QTc interval at maximum heart rate), association between QTc and serum glucose level, specific features of remodeling of the heart and blood vessels in patients with sleep disorders, and, most importantly, SAS in children indicate the need for early detection and correction of sleep disorders (insomnia, parasomnia) and main causes of SAS, such as chronic diseases of the ENT organs, overweight, and obesity. Treatment of sleep disorders is very important in terms of prevention of complications, treatment and prognosis of cardiomyopathy in children, which will help to increase therapeutic efficacy. Key words: children, cardiomyopathy, comorbidity, sleep disorders, sleep apnea, sleep questionnaires

Introduction. Currently, there is an increase in the incidence and an increase in the severity of the course of COVID-19 in children. The tropism of the SARS-CoV-2 virus to the cardiovascular system has been established, while post-COVID syndrome with various manifestations is recorded in 25% of recovered adolescents. The purpose of the work was to identify the features of the electrocardiogram (ECG) pattern in children hospitalized with a diagnosis of COVID-19. Results. Significant changes in the conductivity and activity of the left heart myocardium were found in COVID-19 patients with pneumonia and respiratory failure. Pronounced changes in ECG indices were found in children of senior school age who were admitted for treatment 2.4 times more often than other children. Proarrhythmogenic ECG indices in children were detected in severe COVID-19 - with community-acquired pneumonia (ΔQTc, QTcmin) and respiratory failure (TpTe/QTmax). These ECG changes, combined with the trend in inflammation markers (an increase in the C-reactive protein level and a decrease in the number of lymphocytes) in children with a moderate course of COVID-19 may be a sign of the involvement of the myocardium in an infectious inflammatory process. This suggests that the effect on the myocardium is exerted by systemic inflammation and not by the hemodynamic overload of the right heart, which is expected in pulmonary pathology. Conclusion. The obtained data indicate the need for dynamic ECG monitoring during the acute stage of the disease and rehabilitation of children who suffered from COVID-19.