The incidence of various types of cancer is steadily increasing. It is necessary to correctly analyze and interpret statistical data, given that the quality of medical care at all its stages is characterized not by the level of morbidity, but by the mortality rate. The leading centers of the Russian Federation can apply almost all the therapy methods to treat patients. At the same time, one of the limiting factors for increasing the survival rate is the detection of diseases mainly at the late stages, which dictates the need to increase oncological alertness and the widespread introduction of early screening diagnostics into healthcare practice. The development of many new targeted drugs has led to the fact that only a few have been shown to be effective in the treatment of only certain types of tumors. The development of bone marrow transplantation contributed to the development of other directions in medicine, which proved that the hematopoietic stem cell is a platform for personalized therapy for many diseases. The article describes the problems of cell therapy implementation in the treatment of cancer patients.

Intraventricular hemorrhage is the most common type of brain damage in infants with extremely low and very low body weight. The main source of intraventricular hemorrhages in premature infants are the vessels of the ventricular germinal zone, or germ matrix. Intraventricular hemorrhages are caused by many risk factors. The current concept is that predictors of development are classified into ante-, intra- and postnatal. The most significant among them is intrauterine infection, which can have a direct and indirect effect on the immature structures of the fetal brain. Also of great importance are the concomitant pathology of the cardiovascular system in the mother and complications of pregnancy and childbirth (placental abruption, rapid delivery, chronic placental insufficiency). On the part of the newborn, coagulation disorders in combination with immaturity of the hemostatic system, as well as pathology of the cardiovascular and respiratory systems are important. Predictors of intraventricular hemorrhages in premature infants (genetic, hemostasiological, morphological), require further study, analysis, and optimization of application in order to prevent and reduce the incidence of intraventricular hemorrhages.

The article discusses the principles of therapy and prospects for the treatment of interstitial lung diseases in children. Many of interstitial lung diseases occur with progressive fibrosis, so the use of new antifibrotic drugs is especially relevant now. These drugs include pirfenidone and nintedanib. Therapy of interstitial lung diseases using antifibrotic drugs in children is a promising new direction.

This study presents the results of treatment of 98 patients with risk of development of severe coronavirus disease (hereinafter COVID-19) with the first positive result of polymerase chain reaction test for SARS-CoV-2 in a day clinic of the multidisciplinary Z.A. Bashlyaeva Children’s Municipal Clinical Hospital. To prevent the clinical manifestation of COVID-19 and the progression of the main disease all these children were treated with virus-neutralizing monoclonal antibodies (sotrovimab 500 mg dissolved in 92 mL of 0.9% sodium chloride solution intravenously for 30 minutes, once, for children over 12 years old and with body weight over 12 kg; balanivimab 700 mg + etesivimab 1400 mg pre-dissolved in 250 mL of 0.9% sodium chloride solution intravenously for 30 minutes, once). Patients underwent a comprehensive clinical, laboratory and instrumental examination both initially and in dynamics for 3–7–11 days after therapy according to the developed clinical algorithm. The effectiveness of biological therapy in children with a risk of severe COVID-19 was noted in 100% of cases. None of the observed patients had either a clinical manifestation of COVID-19 or a relapse of the main chronic disease.

Bronchopulmonary dysplasia is the most common chronic lung disease in children under three years. Risk factors of bronchopulmonary dysplasia include prematurity, low birth weight, intrauterine growth restriction, and prolonged respiratory support in neonatal period. However, the incidence of bronchopulmonary dysplasia directly depends on the criteria for diagnosis and criteria for its withdrawal, since the most frequent current “new form” of the disease may not have clinically significant manifestations. This article summarizes data of bronchopulmonary dysplasia incidence in different medical centers in the Russian Federation. The incidence of bronchopulmonary dysplasia varied significantly from 0% to 87%. Increasing knowledge on incidence of bronchopulmonary dysplasia is important to determine ways to improve care of premature infants, to predict diseases of the respiratory system in these patients. It is possible to get reliable data in groups of patients homogeneous in terms of gestational age and birth weight if applying uniform approaches to the diagnosis and treatment of the disease.

The aim of this work is to analyze the epidemiology of congenital malformations in different populations in comparison with the data of the regional register of congenital malformations of the Moscow Region. The article provides a brief description and results of the work of international organizations that collect and analyze data on the prevalence and structure of congenital anomalies — EUROCAT (European network of population-based registries for the epidemiological surveillance of congenital anomalies) and ICBDSR (The International Clearinghouse for Birth Defects Surveillance and Research). For countries where there are no monitoring systems for congenital malformations recording, the Modell World Database of Congenital Diseases (MGDb) has been developed, which makes it possible to calculate the estimated population prevalence of congenital malformations in these regions. According to international registers, the most common groups of congenital malformations are malformations of the cardiovascular, genitourinary, central nervous systems and malformations of the extremities. To date, the epidemiological picture of the prevalence and structure of congenital malformations around the world looks stable.
The article analyzes data from the population-based register of congenital malformations in the Moscow Region from 2011 to 2019, with a coverage of 83% of births in the region. The overall prevalence of congenital malformations was 25.42 per 1000 births. It is determined that the structure of congenital malformations in the Moscow Region is comparable to the data of international studies, but one should pay attention to the lower frequency of most groups of malformations, which is most likely due to insufficient registration of malformations. The Moscow Region register of congenital malformations provides relevant and qualitative data on the frequency and structure of the congenital malformations in the region, which allows, if necessary, to extrapolate obtained prevalence rates to other constituent entities of the Russian Federation as well.

Congenital malformations are not only a medical, but also a serious social problem, since this pathology is a leading cause of perinatal, neonatal, and infant morbidity, disability, and mortality.
Purpose. To analyze the variation dynamics in the incidence of groups of congenital malformations, the structure of congenital malformations according to the average incidence, and the consistency of changes in the incidence of individual groups of congenital anomalies in children of Chelyabinsk based on the results of epidemiological monitoring in 2012–2017.
Material and methods. To assess the frequencies of congenital malformations, we used the monitoring data of the Regional Medical
and Genetic Consultation Office in Chelyabinsk for the period from 2012 to 2017, for Down syndrome (T21) from 2012 to 2018. The total number of newborns over the six-year period was 102,308, of which 2101 children were registered with congenital malformations, including live-born, stillborn children, and fetuses with malformations identified through prenatal diagnostics. Clinical data on newborns was grouped into 11 congenital malformations categories based on the International Classification of Diseases. The statistical analysis of the monitoring results was carried out using methods of descriptive statistics, samples comparisons, correlation, and cluster analysis.
Results. The incidence of all registered congenital malformations in newborns in Chelyabinsk was 20.7 per 1000 births (95% CI: 17.4–25.4) during the study period. In newborns of the Chelyabinsk city in 2012–2017, the prevalence of congenital malformations of the circulatory system (42.8%), chromosomal abnormalities (11.9%), and anomalies of the musculoskeletal system (10.3%) was observed. The relative frequency of Down syndrome for the period from 2012 to 2018 ranged from 1.51‰ to 2.42‰, considering both children born with this pathology and the data on prenatal diagnostics.
Conclusion. The hierarchy of congenital malformations was reproduced from year to year with a predominance of circulatory anomalies, chromosomal anomalies, and musculoskeletal anomalies of the system. The overall incidence of all congenital malformations in Chelyabinsk in 2012–2017 does not exceed the average values for the Russian Federation, while the incidence of Down syndrome exceeded the average national level.

Premature infants born before 32 weeks of gestation with very low and extremely low birth weights are at higher risk for several neurodevelopmental disorders, including cerebral palsy and sensory, cognitive, and behavioral problems. The more complications are observed in the neonatal period, the higher is the likelihood of long-term complications of prematurity. Studying the factors that influence the outcome of neurological development is the only way to develop better treatment approaches.
Purpose. Assessment of correlations between the brain ultrasonography data in children with very low and extremely low birth weights at birth with perinatal and neonatal risk factors, to predict neurodevelopmental outcomes and neuropsychic disorders at 2 years of corrected age.
Material and methods. The material of the study was premature babies with very low and extremely low birth weights (≤32 weeks, ≤1500 g) who were born and received treatment and care in two large perinatal centers in Yerevan. Neurodevelopmental outcome in children was assessed using the Bayley-III developmental scale at 2 years of age.
Results. 64% of ex-premature babies had completely normal development. 10% had cerebral palsy, 21% of children had a speech delay, 5% developed mental deficiency. Socio-emotional and behavioral problems were more common, especially in the social and practical areas (27% and 25%, respectively). 85.7% of children with motor disorders and 60% of children with speech delay had concomitant behavioral disorders. Deviations in behavior were found in 34.4% of children with normal (mental, motor, speech) development. More than 9% of children had very good results, 2% had a high level of mental development, and 3% — a high level of motor development.
Conclusion. Predictors for the development of motor disorders and cerebral palsy were Grade 2 and 3 intraventricular hemorrhages, cystic periventricular leukomalacia, ventriculomegaly. Ventriculomegaly at term equivalent age, expansion of the extracerebral space and interhemispheric fissure led to combination of cerebral palsy and delayed speech development.

Vascular endothelial growth factor is a consequence of angiogenesis links, while at the same time it exhibits different activity under changes in breast milk storage conditions.

Purpose. To study the vascular endothelial growth factor (vascular endothelial growth factor, VEGF) in breast milk and peripheral blood in women with different states of health in the dynamics of lactation, to assess the effect of physical exposure on this indicator.

Material and methods. 96 lactating women were enrolled in the study. The sampling of breast milk and peripheral blood for the laboratory part of the study was carried out 3–5 days after birth, then after 1, 3, and 6 months of lactation. To assess the influence of physical factors on the level of VEGF in breast milk, different modes of temperature exposure were used: freezing and long-term (up to 6 months) storage, heating in a microwave oven up to +60°C.

Conclusion. It has been shown that the VEGF level does not change in samples obtained from women with different states of health during pregnancy and lactation. Preservation of milk at a low temperature affects the reduction of this protein by more than 4 times. Rapid heating of samples by microwave exposure (up to +60°C) leads to partial inactivation of the protein.

Obesity is one of the high-priority problems in modern pediatrics. To study early cardiovascular risks in adolescent children with obesity, 85 subjects with exogenous constitutional obesity at the age of 12–18 years were examined. The patients were divided into two groups: the first group, 41 patients with uncomplicated primary exogenous constitutional obesity, the second — 44 patients with obesity and metabolic syndrome; controls included 20 healthy adolescents of the same age with normal body weight. Based on a comprehensive study of the structural and functional state of the myocardium and intracardiac hemodynamics, it was found that the early significant criteria for cardiovascular risk in obese adolescent children were insulin resistance (HOMA-IR over 3.4), labile arterial hypertension, impaired relaxation, and/or concentric LV remodeling.

While for adult athletes, increasing blood pressure is one of the main reasons for withdrawing from sports for medical reasons, for children this issue is only being studied. Prevalence, risk factors for arterial hypertension and target organ involvement in young (under 18 years old) athletes’ population of different sports specialization and skill levels have not yet been determined.
The purpose of this work was an attempt to determine the arterial hypertension prevalence depending on sports affiliation among highly trained young athletes. It was determined that in athletes the blood pressure’s level, as well as some markers of hidden hypertension, vary significantly (0–23%) depending on diagnostics method, sports specialization, and professional experience.

It is known that blood pressure in children at rest depends on height, but this fact has not been considered during the exercise stress test.
Purpose. To determine the maximum value of blood pressure in young elite athletes, depending on the height and gender.
Characteristics of children and research methods. The study examined 2313 young elite athletes, members of the national teams of the Russian Federation in 40 sports disciplines (average age — (15.5 ± 1.4) years; 45% of young men). All subjects underwent PWC170 cycle ergometry, blood pressure was measured manually at each stage of testing, and its maxima were registered throughout the testing.
Results. The maximum blood pressure values in boys were higher than in girls: systolic blood pressure (195 ± 25) vs (175 ± 20) mmHg, p< 0.001, diastolic blood pressure (80 ± 11) vs (80 ± 10) mmHg, p< 0.05. There was relationship between the maximum value of the blood pressure on exercise and height (r= 0.55; p< 0.001). There were proposed normative tables for the maximum values of systolic blood pressure during exercise, depending on height.
Conclusion. In the young elite athletes, the maximum value of blood pressure during stress test depends not only on the gender, but also on the height. The maximum values of systolic blood pressure during exercise in tall young elite athletes can reach 250 mmHg in boys and 210 mmHg in girls.

Purpose. To present data on testing the program, which allows optimizing audiological monitoring in children with risk factors for the development of hearing loss and deafness.
Characteristics of children and research methods. 217 children who underwent audiological monitoring at the consultative аnd diagnostic center in Morozov Children’s City Clinical Hospital were examined. The research was a prospective longitudinal study with cross-sectional elements. The children were divided into 2 groups. The first group (main) consisted of 136 children who underwent audiological monitoring based on the developed program, and the second (comparison) included 81 children (the program was not applied). Statistical software packages SPSS and Epi info were used to process the obtained data.
Results. In the main group, the most common age of children with primary treatment was (3.0 ± 0.5) months, and in the comparison group — (6.0 ± 0.5) months. In the main group, in the period of (3.0 ± 0.5) months of life, out of 129 children, neurosensory hearing loss was detected in 27 (21.0%), in the period of (6.0 ± 0.5) in 19 of 134 (14.0%), and in the periods of (9.0 ± 0.5) and (12.0 ± 0.5) in 19 (13.4%) and 5 of 136 children, respectively. In 22 children out of 136, hearing decline was transient. In the comparison group at the age of (3.0 ± 0.5) months, neurosensory hearing loss was detected in 2 children, at (6.0 ± 0.5) months in 4 out of 15, at (9.0 ± 0.5) in 1 child out of 25, and at (12.0 ± 0.5) in 9 patients out of 35 children.
Conclusion. The application of the developed program will allow neonatologists and primary care physicians to fully comply with the deadlines for audiological monitoring.

Extracorporeal membrane oxygenation is a modern therapeutic strategy aimed to stabilize vital function in a patient suffering from severe circulatory failure and refractory hypoxia.
Purpose. To describe two clinical cases of venoarterial extracorporeal membrane oxygenation in neonates with mеconium aspiration syndrome, complicated by persistent pulmonary hypertension and refractory hypoxia during medical evacuation.
Material and methods. The authors performed a comprehensive analysis of the condition specifics based on clinical and instrumental work up.
Results. We demonstrated that early application of venoarterial extracorporeal membrane oxygenation in neonates with meconium aspiration syndrome promotes the quickest possible stabilization and reverse of hypoxia and low cardiac output.
Conclusion. Venoarterial extracorporeal membrane oxygenation in neonates with meconium aspiration syndrome and refractory hypoxemia is a life-saving procedure, that can be applied during medical evacuation by qualified medical staff.

Spinal muscular atrophy is a hereditary disease of the nervous system with damage to the motor neurons of the anterior horns of the spinal cord, leading to the development of atrophy and progressive muscle weakness. The article presents a clinical case of type 2 spinal muscular atrophy in a child aged 7 years. For the first time, the diagnosis of spinal muscular atrophy was made and substantiated by the molecular genetic method at the age of 1.5 years. At the age of 2.5 years, the diagnosis of spinal muscular atrophy was confirmed at the Veltischev Research and Clinical Institute for Pediatrics, Moscow. Over the next years, the patient experienced a rapid regression of previously acquired skills. The child ceased to stand with support, to sit independently. Contractures of the knee joints and ankle joints appeared; 2nd degree scoliosis of the spine; dislocation of the head of the left hip joint; symptoms of chronic ventilation failure. Once a year, planned hospitalization was carried out in the neurological department of the children’s hospital at the place of residence to conduct supportive therapy. The patient was repeatedly hospitalized at the Research and Clinical Institute for Pediatrics. In connection with the invariable indications for the treatment of this disease, according to the conclusion of the case conference of the Research and Clinical Institute of Pediatrics, pathogenetic treatment with risdiplam at a dose of 5 mg per day according to the program of premarketing use of this drug was started 4 months ago. While the patient continues to take risdiplam, the disease is stable and the general condition and motor function in the upper extremities improve. Adverse events while taking the drug are not observed.

The publication presents a clinical case of cystic fibrosis with multiple organ lesions in a 9-year-old child. The medical and social significance of cystic fibrosis is due to a significant decrease in the quality of life of patients, the need for long-term treatment, its cost, constant clinical supervision, early disability of patients, and limited life expectancy.
In the presented clinical case, an early age of cystic fibrosis onset, a severe course with damage to the bronchopulmonary system, intestines, pancreas, liver and the development of pronounced pancreatic, protein-energy, respiratory failure, as well as anemia is noted. Despite the early diagnosis of the disease, the child developed multiple organ failure due to the presence of concomitant diseases, which determines the unfavorable prognosis.

Mutations in the PRKAG2 gene lead to hypertrophic cardiomyopathy in combination with Wolff—Parkinson—White syndrome. The cause of the development of heart damage is the deposition of glycogen in the myocardium and the conduction system of the heart with a violation of the metabolism of adenosine monophosphate-activated protein kinase. A feature of PRKAG2 hypertrophic cardiomyopathy is progressive conduction disturbances with the development of atrioventricular blockade and ventricular preexcitation syndrome with a high frequency of paroxysmal supraventricular tachycardia against the background of additional atrioventricular fenestrations (Wolff—Parkinson—White syndrome). Progressive heart failure, high frequency of sudden cardiac death at a young age are characteristic. The features of diagnosis and treatment are considered.

The nature of hereditary cardiac conduction diseases can be determined by using genetic testing. However, due to the absence of large studies, as well as the phenotypic variability of the electrocardiographic and clinical manifestations in patients with bradyarrhythmias, and a positive genetic test there are difficulti to evaluate its effectiveness. The article presents indications for genetic examinations in children with bradyarrhythmias, developed in the Children’s Center for Cardiac Arrhythmias and summarizes the recommendations for patients with cardiac conduction disorders.

This article is devoted to the 150th anniversary of the birth of Evgeniy Petrovich Radin, a prominent scientist and figure of the Soviet health care. In 1927, he was the head of the State Scientific Institute of Health Protection of Children and Adolescents, founded by People’s Commissariat for Health of the RSFSR — the State Institute for Health Protection of Children and Adolescents named after the 10th anniversary of the October Revolution (later the Moscow Research Institute of Pediatrics and Children Surgery, at present it is the Veltischev Research and Clinical Institute for Pediatrics at the Pirogov Russian National Research Medical University) — which position he held until 1931. The article reflects the well-known and little-known facts of Radin’s complicated biography, the variety of his great talent and his invaluable contribution to the establishment of the Institute, which has remained the flagship of the Russian pediatric science for almost a century.