Introduction. Bronchopulmonary dysplasia (BPD) is a complex disease with a significant genetic predisposition. The aim of the study was to determine genetic markers associated with the development of bronchopulmonary dysplasia in premature infants. Materials and methods. At Stage 1, whole exome sequencing followed by the bioinformatic analysis of one hundred samples was provided to evaluate the genetic variants. Sequencing data were compared with the data of the children without any congenital pulmonary diseases. At Stage 2, the obtained results were validated using real-time PCR. Further the genotyping of the control group (n = 70) was performed. The obtained frequencies of nucleotide variants were compared between the groups, as well as with general population data using the RUSeq database. Results. The prevalence of genetic variant rs12489516 in gene CPA3 was significantly higher in the control group of premature infants (p = 0.03; OR = 0.2; 95% CI: 0.02–0.94). Its presence in the genotype reduces the likelihood of developing BPD by 4.76 times. Moreover, statistically significant differences were also identified in the prevalence of rs45488997 in gene CCN2 (p = 0.023). This genetic variant was specific only for children with bronchopulmonary dysplasia. It was also identified that the prevalence of the nucleotide variant rs45488997 in the CCN2 gene was statistically more common among patients with bronchopulmonary dysplasia compared with the general population (p = 0.005). In addition, genetic variants rs5744174 in gene TLR5 and rs2476601 in gene PTPN22 were less frequently observed in the investigated group compared to the general population (p = 0.03 and p = 0.003, respectively). Conclusion. Identification of genetic markers together with clinical and laboratory data will contribute to the development of an effective predictive model for the calculation of the probability of BPD.

Aim: Genetic, clinical, laboratory-instrumental and morphological characteristics of genetic dysfunctions of the surfactant system in children, therapy and outcomes of the disease. Design: Multicentre, ambispective, open-label, descriptive pilot longitudinal study. Materials and methods. We observed 17 children from 16 families with identified mutations in the SFTPC, ABCA3, NKX2-1 genes. Methods used: genealogical, Sanger sequencing, clinical exome sequencing, computed tomography and histological examination of the lungs. Results. The study included 8 children with congenital deficiency of surfactant protein C, 8 children with brain-lung-thyroid syndrome and 1 patient with congenital deficiency of protein ABSA3. Based on the results of a genetic examination of patients, nucleotide variants c.218T>C were identified in 2 out of 8 patients with a mutation in the SFTPC gene, which is the most common according to the literature. In 5 children, the mutations were hereditary. Congenital deficiency of surfactant protein C, ABCA3 protein and brain-lung-thyroid syndrome were characterized by clinical, computed tomography, and morphological signs of interstitial lung disease. Despite complex respiratory, anti-inflammatory therapy, the frequency of deaths in congenital deficiency of surfactant protein C was 37.5%. Conclusion. Children with severe respiratory distress syndrome of newborns, interstitial lung disease with the development of severe chronic respiratory failure, burdened with a family history should undergo genetic testing to detect mutations in the genes SFTPB, SFTPC, ABCA3. The patient's combination of respiratory symptoms with congenital hypothyroidism and neurological pathology is the basis for genetic examination for NKX2-1 gene mutations to exclude the brain-lung-thyroid syndrome. Keywords: genetic dysfunctions of the surfactant system, congenital deficiency of surfactant protein C, congenital deficiency of ABCA3 protein, brain-lung-thyroid syndrome, NKX2-1 gene, children.

Early detection of such a formidable complication of bronchopulmonary dysplasia (BPD) as pulmonary hypertension (PH) is an urgent problem in pediatrics. Echocardiography is currently recognized as the most accessible and non-invasive method for determining pressure in the pulmonary artery. Disorders of alveogenesis and angiogenesis of the vessels of the small circulatory circle in premature infants forming BPD require careful echocardiographic control with using additional analysis of changes in the systolic eccentricity index (EI) as a reliable marker of PH in BPD infants. To increase the information content, it is also necessary to expand the use of EchoCG data in combination with analysis of the blood content of B type natriuretic peptide or the N-terminal pro-B-type natriuretic peptide.

Introduction. To date, Рalivizumab is the only approved monoclonal antibody preparation used to prevent the development of respiratory syncytial virus (RSV) infection. The aim of the work is to evaluate the benefits of immunization with Palivizumab in premature infants with bronchopulmonary dysplasia in a day hospital of the Federal Center. Materials and methods. One hundred seven infants of different gestational age at birth were immunized with Palivizumab in the day patient department of hospital-replacing technologies. The preparation was administered to infants at risk for the formation of severe consequences of an RSV infection. Results. Of the 107 children hospitalized in the day hospital, 74 premature babies were diagnosed with bronchopulmonary dysplasia (69%). Before immunization all infants were consulted by pediatrician, pulmonologist, and if necessary, other specialists. Discussion. The length of stay of children in a day hospital ranged from 2 to 3.5 hours on average. Each child stayed with their parents in a separate room. During this time period, the patient received the full range of the services associated with immunization. Conclusion. Thus, immunization with Palivizumab in a day hospital of the Federal Center allows implementing a comprehensive multidisciplinary and individual approach to each infant, without exposing him to an undesirable risk of infection, in compliance with the principle of staged nursing of premature infants for patients with a new form of bronchopulmonary dysplasia who need a long follow-up observation.

Objective of the Review: To determine the mechanisms of development of pulmonary hypertension in children suffering from bronchopulmonary dysplasia. Key points. The pathogenesis of bronchopulmonary dysplasia is currently not fully understood. Changes in the transmission of intracellular signals affecting the regulation of angiogenesis play an important role. Hypoxia, hyperoxia, and exposure to mechanical ventilation lead to oxidative and inflammatory stress, causing damage to the lung alveoli and vasculature with the development of pulmonary hypertension. It is necessary to understand the interaction of growth factors, transcription factors and inflammatory processes that regulate the normal development of the parenchyma and microvascular bed of the lungs to develop preventive methods. Conclusion. Further study of the role of significant biomarkers of the formation of bronchopulmonary dysplasia can help in the early diagnosis and prevention of the development of this disease, as well as such a threatening complication as pulmonary hypertension. Keywords: prematurity, bronchopulmonary dysplasia, pulmonary hypertension, angiogenesis biomarkers.

Vaccination is one of the essential areas of preventive medicine for protecting the population from diseases and infections. They have helped reduce the incidence of severe childhood diseases and, in some cases, have even eradicated some of the world’s infectious diseases. However, since the first available vaccine against smallpox, antivaccine prophylaxis has always accompanied an antivaccine movement based on various myths. More recently, the development of this movement is connected for many reasons. First of all, it is associated with forgetting the world’s population of the severity of many infectious diseases, the consequences of epidemics, and the availability of any information on the Internet. Leading to myths, parents refuse to vaccinate their children, resulting in reduced vaccination coverage, reduced collective immunity and outbreaks of diseases that have already been considered conquered. The article reviews the literature on the results of anti-vaccination research conducted in the Russian Federation and abroad concerning the causes, main postulates of this movement, trends and directions. Scientific evidence is presented that disproves anti-vaccination myths, and the primary arguments for vaccination are presented. The article describes the anti-vaccination movement’s worldwide trend and the Russian features. The position of WHO is presented about overcoming barriers to the adoption and use of vaccines.

In current conditions, the most effective method of preventing pneumococcal infections (PI) is vaccination, which can significantly reduce the incidence and mortality from pneumococcus and reduce the level of antibiotic resistance. The use of pneumococcal conjugate vaccines has reduced the incidence of invasive PIs in vaccinated children and unvaccinated populations. This is especially true for children with severe somatic pathology, including those with various forms of heart failure (HF). The data show that vaccination of sick HF children can be a clinically effective intervention to improve the treatment results of circulatory failure and improve patients’ quality of life. However, questions regarding the optimal timing of vaccination of sick children with heart failure, dose, frequency and strategies of vaccine introduction still need to be resolved. Data on the pathophysiology of cardioprotection provision during effective vaccination against PI, influenza and COVID-19 in cardiac pathology are summarized. The authors recommend providing conditions for effective vaccine prevention of PI in children with heart failure.

The severity of the course of bronchopulmonary dysplasia (BPD) in the population changes due to the increase in the number of children born with very low and extremely low body weight, the introduction into the practice of new standards of respiratory support for premature infants, drug prevention and treatment of this pathology. The aim of the work is to determine the influence of BPD form, gestational age, birth weight, concomitant diseases on the severity of its course in children. Materials and methods. In 72 BPD children aged from 1 month to 3 years, the severity of the course of the disease was determined according to the computed tomography of the chest organs, using an assessment of the severity in points on a special scale as follows: 0-5 points for mild BPD, 6-10 points - moderate, 11-15 points - severe. Results. In children born after 2012, a moderate course of BPD prevails (8.1 ± 0.32 points), being typical for both the classical (55.1%) and new (69.7%) forms of the disease. The classical form is more severe than the new one (9.0 ± 0.1 and 7.53 ± 0.38 points; p = 0.009). BPD was more severe in children with gestational age less than 28 weeks than in children with gestational age of 29-32 weeks (9.0 ± 0.4 and 7.4 ± 0.6 points; p = 0.000). BPD was more severe in children with birth weight less than 1000 g than in children with birth weight 1000-1500 g (8.79 ± 0.49 and 7.18 ± 0.61 points; p = 0.000). When BPD was combined with patent ductus arteriosus or pulmonary hypertension, the disease progressed more severely than in the absence of these forms of pathology (9.6 ± 0.8, 10.0 ± 0.7 and 7.22 ± 0.34 points; p = 0.031). The severity of changes detected by computed tomography of the chest organs decreases by the age of 3 years, but recovery was not complete in any of the examined patients: 8.6 ± 1.0 points at the age of 6 months and 6.2 ± 0.44 points by 3 years (p = 0.009). Conclusion. In children aged 1 month to 3 years, the severity of BPD is influenced by the form of the disease, gestational age, birth weight, concomitant patent ductus arteriosus or pulmonary hypertension, and the age at which the examination is performed.

Objective of the study: assessment of the nutritional status of infants with bronchopulmonary dysplasia. Materials and methods of research: a retrospective uncontrolled non-randomized cross-sectional comparative study was carried out. It involved 40 premature babies (boys – 19, girls – 21) at the corrected age of 3,3 [2,3–4,0] months and 16 age matched full-term babies (comparison group). Children born prematurely were divided into 2 groups depending on their body mass (BM) at birth: 1st – 25 children with BM less than 1500 g, 2nd – 15 children with BM from 1500 to 2500 g. The first group included subgroups 1A and 1B, depending on the presence or absence of bronchopulmonary dysplasia in children. Physical development was assessed using INTERGROWTH-21st and WHO Anthro, 2009 anthropometric calculators, body composition was determined by air plethysmography using a PEA POD apparatus. Results: the assessment of anthropometric indices (WAZ and HAZ) calculated for postnatal and corrected age revealed the most severe manifestations of nutritional deficiency in children with very low BM at birth who developed bronchopulmonary dysplasia. The percentage of fatty BM was statistically significantly lower in children of subgroups 1A and 1B compared to full-term infants (p<0,006), as well as fatfree BM (p<0,012). Conclusion: the slow development of anthropometric indicators of premature babies with very low BM, especially those with bronchopulmonary dysplasia, indicates the need for timely correction of their nutrition.

The aim of the work is to analyze the data of Echo-CG examination of premature infants who have formed and have not formed bronchopulmonary dysplasia (BPD) to determine the frequency of the formation of pulmonary hypertension (PH). Materials and methods. A total of 199 preterm infants treated in the Department of Pathology of Newborns were examined. The first group included moderate and severe BPD children (n = 117; 59%). The second group consisted of children without BPD within clearly decreed terms (n = 82; 41%). In each group, patients were divided into four subgroups by the gestational age at birth and the timing of the Echo-CG. Results. Only two (1.1%) patients out of 117 BPD children of the first group were diagnosed with pulmonary hypertension (PH). In 3 (2.5%) of 117 infants of the same group, enlargement of the right heart without PH was revealed. Out of 82 children without BPD, two patients had signs of right heart enlargement. PH was not diagnosed in any of the patients in this group. Discussion. To aggravate the efficiency of PH diagnosis, a number of indices of screening echocardiography seem to be increased by additional analysis of changes in the systolic eccentricity index (EI), as a reliable marker of PH in BPD children. Systolic IE should be integrated into screening in preterm infants for the diagnosis of PH. The use of Echo-CG data along with analysis of blood BNP or NT-proBNP blood content be also expanded. Optimizing the diagnosis of PH at the early stages of BPD is necessary to increase the efficacy of targeted therapy and reduce the risk of severe complications of BPD.

The experts of the Union of Pediatricians of Russia have developed up-to-date clinical guidelines on management of children with acute bronchiolitis. Around 150 million cases of bronchiolitis (11 cases per 100 infants) are registered annually, 7–13 % of all cases require hospital treatment and 1–3 % require management in intensive care unit. Almost all children of the age under 2 years old (90 %) undergone respiratory syncytial viral infection. In 20 % of them they have bronchiolitis due to various factors. This article covers the issues of epidemiology, pathogenesis, differential diagnostics, treatment based on the principles of evidence in detail.

Study Objective: to analyse the dynamics in morbidity of the classic and New forms of bronchopulmonary dysplasia (BPD) in children hospitalised to the National Medical Research Centre of Children Health during previous 8 years. Study Design: retrospective analysis of medical records. Materials and Methods. In 2012–2019, we analysed 369 cases of children with the new BPD and 231 cases of children with the classic disease. Study Results. The article contains the information on the 8-year observation demonstrating prevalence of the new BPD and describes disease characteristics and course in premature infants. The retrospective analysis of 2012–2019 data shows that the number of paediatric patients with the new BPD is gradually increasing. Whereas in 2012 the classic BPD prevailed (156 (68.4%) out of 228 children with confirmed diagnosis), later the new form accounted for more BPD cases (p < 0.05). According to information available, an equal ratio (50% for the classic form and 50% for the new form) is demonstrated in 2013, the period when the Russian Federation started using the live birth and dead birth criteria proposed by the World Health Organisation (WHO). Conclusion. Improved neonatal intensive care methods and transition of the Russian Federation to the WHO live birth and dead birth criteria in 2012 were pre-conditions for BPD pathomorphism. The of rate of the classic form in the Russian Federation has been decreasing year after year. It is essential to identify the long-term complications from the new BPD, especially functional capabilities of the respiratory tract in older patients with the history of disease. Study of the features and long-term complications from this new form is a burning issue of paediatric pulmonology and requires careful attention. Keywords: bronchopulmonary dysplasia, new form, classic form, premature infants.

The paper presents a clinical case of congenital cleft palate as a manifestation of 22q11.2 deletion syndrome accompanied by other systemic disorders having direct impact on functional indicators and perioperative period during cleft surgery. Specific for 22q11.2 deletion syndrome endocrine disorders affect the facial development. Multidisciplinary approach contributes to the early optimal treatment outcome and prevents further postoperative disturbances in maxillofacial development.

Prevention of exacerbations of bronchopulmonary dysplasia (BPD) directly affects the outcome of the disease and belongs to the priority areas of pulmonology of early childhood age. Seasonal immunoprophylaxis of the severe course of respiratory syncytial viral infection (RSVI) with palivizumab and vaccine prophylaxis of pneumococcal and hemophilic infections in children who have formed BPD has been established to allow to reduce the frequency of hospitalization, resuscitation and death. The authors present their own data on results of passive and active immunization of BPD children against pathogens of the respiratory spectrum.

The socio-economic burden of the consequences of perinatal central nervous system damage in preterm infants remains an urgent healthcare issue. The review discusses the modern concepts of cerebral ischemia pathogenesis in prematures, the peculiarities of diagnosing structural brain lesions, and the ability to predict motor deficits in the future. The possibilities of assessing mental, motor development and social adaptation disorders of premature infants with consequences of perinatal pathology in the early stages of ontogenesis are described.. The modern views on rehabilitation and restorative treatment of premature infants with combined perinatal pathology are highlighted. The characteristics of non-invasive methods for early diagnosis of central nervous system pathology and therapeutic methods with the rationale for choosing preventive and curative measures and the further prediction of the disease course and outcome are given. The world experience of multidisciplinary rehabilitation of premature infants with combined perinatal pathology is highlighted.