Currently, the vaccination not only does not lose its value, but also becoming more in-demand allowing to prevent mass infection, disability, and mortality due to them, oncological and somatic diseases. The variety of medicinal vaccines is actively developed. The particular importance is given to the vaccination as a key mean to prevent the antibiotic resistance. That is why it is important for every health worker to know the up-to-date approaches to the immunization in whole, and especially for the risk groups, to understand and compare reasonably the risks of the infections and vaccinations, to be able to explain this to their patients and parents. The most important is a common understanding of the importance of the preventive vaccination of the health workers of all specialties and levels.

The epidemiological studies have shown an increase of congenital heart diseases in the industrial regions of the Russian Federation. The etiology and pathogenesis of most congenital heart defects are still unknown despite high achievements in cardiology and cardiac surgery. The purpose of this review is to analyze the literature data devoted to the influence of a complex of exogenous and endogenous factors, including the genetic ones, upon the development of the sporadic congenital heart diseases, which are not associated with the chromosomal abnormalities. It isshown that the sporadic congenital heart diseases associate with the women's social factors such as a low social status and level of education, smoking, and alcohol. The air pollution with nanoparticles smaller than 10 µm is a risk factor for the development of the congenital heart diseases and other pathological conditions of the cardiovascular system. It has been proven that the alleles and genotypes of polymorphic variants of genes GATA6, NPPB (rs198388 and rs198389), CYP1A1, CYP1B1, and NAT2 have the associative links with the congenital heart disease in the children.

The Epstein–Barr virus, which is persistent in the human organism throughout lifetime after the primary infection, is involved in the pathogenesis of a number of somatic chronic diseases. It is known that the virus successfully escapes the immune control, and has many mechanisms to regulate the components of immune system s as well. In our work, we summarized the current scientific data on the effect of the persistent Epstein-Barr virus on the function and quantity of T- and B-lymphocytes, NK cells, activity of the toll-like receptors, secretion of interleukins, interferons and other cytokines. The immunity dysfunction with the immunoactivation predominance leads to the formation of severe forms of chronic active Epstein–Barr virus infection such as the chronic mononucleosis, hemophagocytic lymphohistiocytosis. The immunosuppression is characteristic for the atypical course of the chronic active Epstein–Barr virus infection. The ability of some viral proteins to antigenic mimicry (that is, the homology of viral and human proteins) is the determining factor in the development of the chronic fatigue syndrome, multiple sclerosis and systemic lupus erythematosus. The Epstein–Barr virus is capable of the immortalization of the B-lymphocytes, including the autoaggressive ones, which leads to the formation of the chronic autoimmune diseases. Study of the development mechanisms of these diseases permits to develop the new, more effective, personalized prevention and treatment schemes, for example, using the targeting therapy.

The atopic dermatitis is a chronic inflammatory skin disease. The wide disease prevalence, increase in a number of the severe forms determine its high medical and social importance. The development of the dermatic inflammatory process in the patients is caused by the complex interaction of the genetic mechanisms, environmental factors, infectious agents, defects of the skin barrier and immunologic mechanisms. The various populations of the immunocompetent cells takes participation in the atopic dermatitis immunopathogenesis: Th1, Th2, Th9, Тh17, Th22, regulatory T-cells and cytokines secreted by them. This review states the data on the participation of a number of cytokines in the development of the immunopathological process in the presence of the atopic dermatitis. The revealing of the particularities of the disease pathogenesis based on the cytokine profile assessment and determination of the clinical course severity markers are the most relevant line of the clinical allergology and immunology for determination not only the disease prognosis but for the therapeutic targets in future as well.

The bronchial asthma is one of the most common chronical disease in the children; it has the high medical and social significance. The bronchial asthma onset is reported for 70–80% of the children in the early childhood. However, the disease in this age period is not diagnosed in many cases, and the patients are not treated efficiently. The purpose of the study is to try the Russian-language version of the questionnaire “Asthma Prediction Tool”. The questionnaire of 49 parents of the infants with the bronchial obstruction syndrome was carried out. According to the questionnaire results, it was determined that more than half of  the children (59.2%) had low risk, 36.7% – average risk and 4.1% – high risk to develop the bronchial asthma in the next 5 years. So, the questionnaire “Asthma Prediction Tool” may prove to be the convenient and useful additional tool in future to assess the risks of developing of this disease in the children for the nearest 5 years, correlating well with the usually assessed sign pattern, specifying the bronchial asthma in the patients suffered the bronchial obstruction syndrome in the early childhood.

One of the current problems of perinatal neurology is the hypoxic-ischemic brain damage in newborns associated with the influence of the hypoxia upon the fetus, intranatal and postnatal asphyxia on one hand and a lack of the efficient therapy schemes on the other hand. Due to this, the purpose of this pilotstudy isto identify the effects of drug Cytoflavin, included into the complex therapy scheme for the newborns with the cerebral ischemia of II-III stages, on the blood acid-base balance. A retrospective analysis of the results of the complex therapy for 16 newborns with the moderate (14 children) and severe (2 children) brain ischemia was performed. Cytoflavin was included in the standard therapy schemes for all children at a dose of 2 ml/kg per day at a dilution of 5% glucose solution at the ratio of 1:5, intravenously, microfluidically for 20 hours for 3 days. In addition to the standard examination, the blood acid-base balance assessment using the follow-up microgasometric method was included (after 60 min and then every 6 hours until 72 hours of observation). All children had positive tendency to the arresting of the metabolic acidosis (in the form of the decrease of the base deficiency after 24 hours and increase of pH level (the level of 7.30 was reached by 12 hours of age in full-term newborns and 24 hour of age in the preterm newborns). The revealed positive changes in the time of the metabolic acidosis arresting along with the small volumes of the infusion and good tolerability are the cause for the planning of the subsequent, more large-scale studies.

Forthe child’s optimal growth and development, the normal provision with vitamin D isrequired. The vitamin D levelsin newborn depend directly on the mother’s provision with this vitamin, who is a single vitamin source for the child. The purpose of the study is to assess the provision with vitamin D in newborns of Arkhangelsk city in winter. 25(ОН)D determination in the blood serum of 55 newborns and their mothers was carried out using the method of the enzyme-linked immunosorbent assay at the laboratory of Arkhangelsk Children’s Regional Clinical Hospital. Resulting from the test carried out, only 4 (7%) children had the normal level of vitamin D, 36% (20 children) had the level corresponding to the deficiency: 14 (26% children) had the insufficiency of this vitamin, 17 (31% children) had a severe deficiency. The data obtained indicate the necessity to optimize the activities on the vitamin D deficiency prophylaxis in the pregnant women and support the prescription of vitamin D drugs to the newborns of Arkhangelsk city from the first days of the life.

The purpose of the study is to investigate the state of the upper gastrointestinal tract (UGIT) in various types of basic treatment of children’s bronchial asthma. There was conducted a clinical-instrumentalsurvey of children with bronchial asthma. They have been receiving a basic anti-inflammatory therapy inhaled glucocorticosteroids (IGCS) (1st group, n=33) or anti-inflammatory cromone drugs (2nd group, n=33) for 3 years and more. Most of the children with the bronchial asthma complained about the gastrointestinal problems, mostly abdominal pain; the heartburn was recorded reasonably oftener in the children of 1st group. While conducting endoscope survey, 97% of children of 1st group were revealed having the UGIT pathology. As the result of microscopy of gastric mucosa bioptic sample, most of the patients of 1st group appeared having fungal infection; the significant lymphoplasmacytic or eosinophilic infiltration in own layer of the gastric mucosa was determined. Moreover, H. pylori was revealed more frequently for the children of 1st group, and the level of invasion was higher. The data given indicate the need for measures limiting the entry of steroids into GIT, as well as the use of medicines that reduce damaging effect of IGCS on the mucous layers.

The article considersthe etiological factors, clinical manifestations and diagnostic signs of the alveolar hemorrhagic syndrome, which is rare in the pediatric clinical practice, using the example of the group of the patients with orphan lung pathological condition observed in the Pulmonology Clinic of the Institute. The prevailing number of the patients consists of the children with the rare disease; that is the idiopathic pulmonary hemosiderosis. That is why the clinical manifestations of the alveolar hemorrhagic syndrome in the presence of this pathological condition are discussed, the algorithm of the diagnostic measures and the long-term observation results (including the disease outcomes) for the children received the different variants of the immunosuppressive therapy are presented.

The physical development indicators in  the context of  the social and hygienic researches are important health parameters of  the children’s population and indices of the social well-being of the society. With the purpose to define the modern trends of the children physical development using the random sampling technique, 6,207 school children (3,225 boys and 2,982 girls) aged from 7 to 17 years were examined in the various districts of Saint-Petersburg. The somatometry (body height, weight and ribcage circle) was carried out and the arithmetic mean values (M), and error in means (m) were calculated. The data analysis has shown that in all age groups, except for 11–12-year-old children, the indicators of the body height are higher for the boysthan those for the girls. The pubertal growth acceleration of the body height in girls becomes perceptible during the period from 9 to 13 years of age; in boys, it starts after 11 years and it is prolonged up to 15 years of age. The girls’ body weight is lower in all groupsthan that in the boys; only at the age from 11 to 13 years, the body weight is practically equal to the indicators of the boys of the same age. The ribcage circle indicators are also higher in boys than in girls in all age groups. The analysis of the annual somatometric indicator increase has revealed the considerable decrease in the value of gain in the body height in both the boys and girls on the 11th year of life as well as the body weights in boys that demands the additional analysis of cause factors. The revealed trends of the physical development of schoolchildren in the conditions of the megalopolis demand the in-depth analysis of the factors defining these tendencies with the subsequent development and carrying out of the preventive measures.

The article is devoted to the rare disease of the lysosomal storage disease group – Fabry’s disease. The disease is associated with the sphingolipids dysmetabolism, is caused by the accumulation of the globotriosylceramide (Gb₃ ) and othersphingolipidsin the organism tissues and cells; it is characterized by the progression and severity of the course. The diagnostic results of 6 patient children aged from 5 to 17 years are analyzed; 2 boys and 4 girls from 3 families. The hereditary burden with a large number of the disease cases, 16 patients in 3 families including 6 children, comes under notice. All 6 children were diagnosed with Fabry’s disease based on the genealogical analysis as well as biochemical and molecular genetic examination. The activity of α-galactosidase A enzyme in the blood leukocytes was significantly decreased in two boys, insignificantly decreased in two sisters, and was normal in two girls. When performing the molecular genetic analysis, 3 mutations in exon 5 of GLA gene were identified. It has been established that the damages of cardiovascularsystem and nervoussystem, kidneys and visual organ, depression of the perspiratory gland function shall be considered as the first clinical signs of the disease in the children; it seems likely that the angiokeratoma appearance is characteristic only for boys. The presence of the non-specific symptoms and signs of the connective tissue dysplasia is noteworthy. The emphasis is made towards the importance of the early Fabry’s disease diagnosis, as it is essential for the timely (prior to appearance of the clinical symptoms and signs) beginning of the pathogenic treatment with the enzyme replacement drug.

This article is devoted to the study of the incidence and structure of the genetic syndromes associated with congenital heart diseases in Azerbaijan. The results of observation of 430 children with congenital heart diseases, which have been stayed in the Child Department of Scientific Surgery Center named after Academician M.A. Topchubashov during 2010-2015 period, have been analyzed. It was demonstrated that the incidence of the chromosomal and monogenic pathological conditions is 6.5±1.2% (28 children) among the above population. The chromosomal syndromes were diagnosed in 20 (4.7±1.0%) children, monogenic ones – in 8 (1.9+0.7%) children. The chromosomal pathological condition was mostly presented with the Down’s syndrome (in 12 patents). As to the monogenic syndromes, it was mostly the heterotoxic syndrome (4 children). These data testify that in spite of the multifactorial genesis of the most of the congenital heart diseases and role of the unfavorable factors of the antenatal period, the genetic component influences with a great importance upon the prevalence rate of the malformations.

The tubulopathies are a group of the diseases varied in the origin, which manifest with the disorder of tubular transport of the organic matters, water or electrolytes. The article presentsthe clinical observations of different variants of the primary tubulopathiesin the children: the nephrogenic diabetesinsipidus(the hemizygous mutation isidentified in gene AVPR2), distalrenal tubular acidosis(SLC4A1 gene mutation is identified) and renal glycosuria. The dynamics of the clinical signs, phases of the diagnostic search and differential diagnostics have been described. The difficultiesin the therapy selection were analyzed for each case; the tactics of the furtherfollow-up was discussed.

The clinical and genetic features of the rare, atypical form of the autosomalrecessive Gitelman syndrome with manifestation atschool age are presented. The disease manifestsin child with the hypomagnesemia, hypermagnesium, hypomagnesic limbs convulsions, metabolic alkalosis, hypokalemia, hypocalcemia, hypoparathyroidism associated with the cerebral bilateral calcifications in the frontal lobes, basal ganglia, cerebellum, and decreased intelligence. The diagnosis is confirmed by recognition of the homozygous mutation in gene SLC12А3.

One of the leading positions among the infant diseases belongs to the pathology of the respiratory tract. The causes of the obstructive bronchitis in children are very diverse including those, which can be associated with the disorder of the normal immune system functioning or immunodeficiency state. The purpose of the study is to determine the nature of the immune disorders in the children with the obstructive bronchitis, to assess the efficiency of the immunocorrecting therapy. For 2 groups of the children with the obstructive bronchitis, the comparative study with determination of the cytokine and immunoglobulin levels in the blood serum against the background of standard therapy (1st group) and against the background with immune stimulator ismigen included into the therapy was carried out; the ismigen is a multivalent antigenic complex of the bacterial lysates. The efficiency of the immune-enhancing therapy carried out towards the immune system condition of children during the comprehensive treatment of the obstructive bronchitis was shown. The practical importance of the work is to optimize diagnosis and immunocorrection in obstructive bronchitis in children, accompanied by the disorder of the body’s immunoreactivity.

The tuberous sclerosis is a systemic orphan disease. According to the literature data, the article summarizes the clinical and genetic features, presents the large and small criteria for the tuberous sclerosis diagnostics in children. The authors provide the comprehensive description of the kidney pathological condition specific for the tuberous sclerosis, which complicates considerably the course and determines the disease prognosis. The clinical observation of two tuberous sclerosis cases in the infants with very early detection of the polycystic kidney disease, quick growth of the kidney cysts and increase of the kidney volume, with formation of the nephrogenic arterial hypertension is presented. The TSC2/PKD1 syndrome (“adjacent gene syndrome”), which is a consequence of the simultaneous mutation of the adjacent genes on the short arm of chromosome 16, is the most probable in both cases.

The possibility to carry out the alternative measures for the children with the urinary tract infection is considered in addition to the antibiotic therapy, which has become insufficient in recent years due to the steady growth of the antibiotic resistance as well as owing to the creation of the conditions for population of the urogenital tract with the uropathogenic bacteria. Constantly expanding range of the antibiotics, which provides a cytotoxic effect on the kidney tissue, significantly increases the likelihood of the nephrotoxicity of the drugs. The normalization of the urodynamics and biocenosis of the mucous membranes, as well as so-called receptor therapy and mucin layer recovery usually in combination with some etiotropic influence renders the favorable medical and preventive effect.

The functional gastrointestinal disorders in infants occur rather often. About half of the children has at least one of such signs of the functional gastrointestinal disorders as the spitting up, torminae, constipations, and the combination of several signs is ordinary. The formation mechanisms of the functional disorders are multivarious and they are determined not only by the anatomical and physiological particularities but by the autonomic and humoral regulation imbalances of the child’s organism as well. The protein and lipid composition of the mixture based on the goat's milk, Kabrita® Gold, contributes to the digestion improvement. The mixture can be recommended for the formula-fed infants with the functional gastrointestinal disorders in the absence of the atopy signs.

Forthe harmonious development of the child at any age, the dietshall be balanced in terms of the proteins, fats and carbohydrates, and it shall include the sufficient amount of the macro- and microelements as well. The micronutrients are necessary for the functioning of all organs and systems, are part of many enzymes, hormones, immune factors. Numerous domestic and foreign studies indicate to the insufficient attention to this problem; that contributesto the development of deficiency conditions. The article considersthe issues associated with the introduction of products of industrial production enriched with the mineral elements in the nutrition of children.

The long-term cooperation of the two scientific institutions contributing to the professional development of pediatricians is based on the long-term scientific relations of the Department of Pediatrics of the Russian Medical Academy of Continuing Professional Education and the Moscow Research Institute of Pediatrics and Pediatric Surgery (now the Research Institute of Pediatrics named after Academician Yu.E.Veltischev). The role of remarkable pediatric scientists – G.N. Speransky, Yu.E. Veltstschev, M.S. Ignatova, N.A. Korovina in the development of these links and pediatric science in Russia.