The article is devoted to urgent pediatric problem of comparing traditional and innovative approaches to the study of predisposition to diseases. It is noted that in the era of the formation of personalized pediatrics, along with the introduction of the latest diagnostic and therapeutic technologies, it is important to take into account the classic achievements, including those associated with the ideology of diathesis developed in the Russian pediatrics – concepts of hereditary predispositions. The article contains data on changing classification of diathesis. Within the framework of the 4P medical paradigm and, first of all, personalized medicine, the authors discuss molecular genetic and other high-tech potential methods of the disease risk assessment. Based on the concept of the space-time bioecological continuum of transitional health states, in particular, using the example of the atopic march, the authors consider the approaches to multi-level research, adaptation mechanisms and their breakdowns, and possible development of appropriate prognostic biomarkers. Using the example of biophenotypes of bronchial asthma and variants of manifestations of disenergetic states, the authors pay their attention to the importance of identifying the individual characteristics of pathological processes in children. The authors have concluded that the main task of personalized pediatrics is the development of methodology used for designing a personal health management program based on the principles of a new strategy for diagnosing, monitoring and controlling individual (including genomic) health, with the formation of a genetic passports for each child.

The article analyzes the literature on the development of views on the definition, approaches to diagnostics and tactical measures in case of suddenly manifesting conditions in children of the first year of life, accompanied by respiratory failure, changes in muscle tone and skin color. The article contains data from the clinical guidelines of theAmericanAcademyof Pediatrics 2016. The above guidelines gave introduced a new term “brief resolved unexplained event” (BRUE) and distinguished two groups of infants – with low and high risk of unfavorable outcome. The article contains a definition of a “life-threatening event”, which refers to events in children of the first year of life who have experienced a rapidly resolved unexplained condition and met the criteria of low risk. The authors describe the approaches to the diagnosis and prevention of pseudo-life-threatening events in infants. The authors call the pediatric community to discuss and present their own view on the problem of definition and tactical measures necessary for identifying quickly resolved unexplained events in children of the first year of life who meet the criteria of low risk.

The article reflects the genetic variants of polycystic kidney disease, describes the modern strategy for the treatment of polycystic kidney disease in children and adults. The authors present the results of clinical trials of vasopressin V2 receptor antagonists (tolvaptan, liksivaptan), a multi-kinase inhibitor (tezevatinib), somatostatin analogues (lankreotide, octreotide), statins (pravastatin), mTOR inhibitors (everolimus, sirolimus), metformin in patients with autosomal recessive and autosomal polycystic kidney disease. The authors discuss the factors determining the prognosis and outcome of these diseases.

Rheumatoid uveitis is a serious problem in rheumatology and ophthalmology due to the peculiarities of the disease – an undistinguished beginning, chronic course and a high incidence of disabling complications. The article analyzes various data on the development and nature of uveitis in children with juvenile idiopathic arthritis. The authors describe the analysis of the results of various studies of this disease. They consider the features and results of treatment of children with rheumatoid uveitis using genetically engineered biological drugs.

Epstein – Barr virus, related to herpes viruses, causes infectious mononucleosis during the initial infection; after recovery, the virus persists in the body throughout lifetime. The presence of clinical symptoms and viral load in a patient in 6 months after the infectious mononucleosis disease indicates the formation of chronic active Epstein – Barr viral infection. Hemophagocytic lymphohistiocytosis, posttransplantation lymphoproliferative disease and chronic fatigue syndrome, which has a polyetiological nature, are also associated with the activation of the persistent Epstein – Barr virus. Most of these diseases develop in children due to their physiological immunodeficiency and are accompanied by high mortality – up to 50%. Immune mechanisms, in addition to the virus itself, play a leading role in the pathogenesis of the diseases. The article summarizes all existing approaches to the treatment of chronic Epstein – Barr virus-associated diseases. The authors have analyzed the effectiveness of these approaches on the basis of various published studies. These diseases are treated with etiotropic antiviral drugs – nucleoside analogs, nonspecific immunotherapy, targeted therapy with monoclonal antibody preparations, immune cellular CD8+ therapy. In case of ineffectiveness of these methods, the alternative bone marrow transplantation is used. The article highlightes promising areas for the development of new approaches to the treatment of Epstein – Barr virus-associated diseases.

Bone sarcomas account for 7% of malignant tumors in children, and osteosarcomas make up 35–50% of them. Many patients are diagnosed at spread stages of the disease, which dictates the need to search for new approaches to treatment. At the same time, the optimal therapeutic strategy is unknown, the results of treatment of such children remain unsatisfactory. Part I of this article describes currently used treatment regimens. Part II presents achievements in the field of personalized therapy. Thus, changes in treatment approaches illustrate the vector of development of the treatment principles of such complex diseases, being fatal for most patients a few years ago.

Integrity and permeability of the intestinal barrier in premature children plays an important role digestive disorders and such terrible complications as necrotizing enterocolitis.
Objective. To determine the dynamics of the level of fecal zonulin in premature babies during the first 2 weeks of life and establish the connection between its values and other indicators characterizing the status of extremely premature children.
Materials and methods. There were examined 2 groups of children: Group 1 consisted of 108 premature infants with gestational age of 22–32 weeks and Group 2 (control) included 27 full-term newborns. The level of fecal zonulin was determined by enzyme-linked immunosorbent analysis in dynamics on the 3rd, 7th and 14th days of life.
Results. The premature children have a multiple increase in fecal zonulin levels within 14 days of life compared to healthy full term children. Levels of fecal zonulin on the 7th day of life were significantly higher in children who developed necrotizing enterocolitis on the 10±4.7 days of life.

The Objective of this article is to assess the influence of the factors of pregnancy, childbirth and the early neonatal period on the development of acute kidney damage in premature newborns with very low and extremely low body weight. In addition, this disease significantly affects the prognosis and quality of life of the child. In addition, acute kidney damage significantly increases mortality among patients of the intensive care unit for newborns. There has been established a connection between acute hypoxia in childbirth, extremely low body weight at birth, intrauterine infection and III severity on the NTISS scale with the development of acute kidney damage in the early neonatal period. Early detection of this condition requires a comprehensive study of the mechanisms of its development and possible risk factors.

Objective. To identify risk factors for acute cardiac surgery-associated kidney damage in 214 newborns and infants with congenital heart defects.
Results. 54.7% of 95 newborns and 46.2% of 119 infants have acute cardiac surgery-associated kidney injury. There have been determined statistically significant risk factors of acute cardiac surgery-associated kidney injury: in newborns – the level of lactate in blood plasma more than 2.5 mmol/l and artificial lung ventilation (87.1% as compared to 39.1% without these factors; 93.8 and 46.8% accordingly; р<0.001), in infants – level of lactate in blood plasma more than 2.5 mmol/l before surgery as compared to those without these factors (96.4% as compared to 30.8% without this factor; р<0.001).

Objective: to analyze the parameters of the hemostasis system and the results of molecular genetic testing in patients with a single functional ventricle. The study included 102 patients. All the patients underwent a staged surgical hemodynamic correction of a single functional ventricle. The authors performed a retrospective analysis of patient records in order to identify the episodes of thrombosis. The incidence of thrombotic complications at different stages of hemodynamic correction in the examined patients with a single functional ventricle was 12.7%. The indicators of plasma link hemostasis in the observed patients have been characterized by a balance of hemostatic reactions in the group of children with thrombosis and without. The results of a molecular genetic study demonstrated that the carrier of the heterozygous genotype of 20210GA factor II gene in patients with a single functional ventricle increased the risk of thrombotic complications 16 times (15.4% in patients with thrombosis versus 1.1% in the group without thrombosis; odds ratio 16.0; 95% confidence interval 1.34–191.24; p=0.028). All patients with thrombosis in the history revealed a homozygous condition according to variant 10976GG factor VII gene (p=0.017).
Conclusion: molecular genetic analysis of polymorphic variants of the hemostatic system in patients with a single functional ventricle is required to predict the risk, timely prevention and correction of thrombotic complications during the surgical treatment of congenital heart disease.

Objective. To analyze the results of clinical and laboratory studies of children born to women with syphilitic infection, and select the most clinically significant criteria to optimize the diagnosis of early congenital syphilis.
Methods. A comprehensive clinical and laboratory examination of 547 newborns born to women with a syphilitic infection.
Results. The diagnostic sensitivity of the enzyme immunoassay with the determination of specific IgM was 86.6%, the polymerase chain reaction with the detection of Treponema pallidum DNA was 91.6%.
Conclusion. The examination of newborns from mothers with a syphilitic infection can be optimized by reducing the number of laboratory techniques (microprecipitation reaction and passive haemagglutination reaction), subject to the mandatory determination of specific IgM and IgG in the first day of life, followed by further confirmation of diagnostics (IgG titer dynamics and the determination of the presence of T. pallidum DNA, as well as instrumental methods of research).

The authors studied microbial composition and metabolic activity of the intestinal microbiota in 20 babies born by caesarean section (main group) and 20 babies born vaginally (control group). There were isolated bifidobacteria from meconium at a concentration of 105– 1010 CFU/g by the bacteriological method in 4 (20%) children of the main group and 6 (30%) children in the control group. The gas-liquid chromatography revealed the metabolic activity of various representatives of the intestinal microflora in children of both groups, regardless of the mode of delivery; the indicators of metabolic activity were significantly lower than in the infants. The children in both groups had similar absolute and relative content of short-chain fatty acids in meconium. The data of gas-liquid chromatography indicate the metabolic activity of the meconium microbiota, which is dominated by anaerobic microbial populations and there is a high proportion of proteolytic and conditionally pathogenic flora. The data obtained confirm the process of intrauterine translocation of microbes from mother to child and indicate the importance of the microflora of the future mother in the process of forming the intestinal microbiota of the child.

The authors studied the effect of selenium on the dynamics of immune system indicators in children with autoimmune thyroiditis. They examined 31 children (average age of 11.16 ± 0.59 years). Group I included 17 children who took selenium (100 μg per day for 6 months) along with the basic treatment. Group II (n=14) took L-thyroxin. The control group included 15 healthy children of the same age. The average level of selenium in children of Group I and II was 69.23 ± 1.52 μg / l at the beginning of the study, in the control group it was 114.8 ± 3.18 μg / l. Before treatment, children in Group I and II had T-cell suppression, the average level of all cytokines (especially TNF-α and IL-6) was significantly higher than in practically healthy children.
The study demonstrated that the level of the thyroid tissue antibodies decreased significantly (p=0.001) with an increase in the level of selenium in the blood serum. By the end of the study the content of IgA (p=0.012) and IgG (p=0.044) in Group I, as well as the number of lymphocytes CD3 + (p=0.008), CD4 + (p=0.015), CD16 + / 56 + (p=0.010) significantly increased. The authors observed statistically significant decrease in the levels of TNF-α (p=0.028), IL-6 (p=0.002) and IL-1β (p=0.009) in children who took selenium in addition to the main treatment. Thus, the results of the study suggest that selenium in the complex therapy of autoimmune thyroiditis significantly reduces the titer of antithyroid antibodies and positively affects a number of important indicators of immune homeostasis in children.

The anatomical dimensions of the internal organs are relatively stable and constitutionally determined. The connection between the somatotype and the size of the internal organs in adolescents - boys and girls - remains virtually unexplored. So, the purpose of this work is to compare the size of the internal organs separately in the group of young men and girls with lepto-, meso-and hypersome somatotype. The study involved 421 teenagers aged from 13 to 17 years: 225 boys and 196 girls. Their somatotypes were determined according to the patented method of V.A. Melnik, I.I. Salivon, N.I. Polina, and the sizes of abdominal organs and thyroid gland were determined using ultrasound morphometry. Their comparisons revealed the mosaic distribution of differences in the size of internal organs in children with different types of constitution. The authors discussed possible reasons of this difference: a methodological error in visualizing the size of internal organs, the influence of thyroid hormones, general biological and evolutionary factors, and the effect of narrow (in terms of gender and age) selection of the subjects. Thus, an orientation towards the constitutional features of the organism as a factor influencing the results of ultrasound examination of internal organs should be optional until the mechanisms and structure of these features are clarified in detail.

The authors describe their own clinical observations of children with non-syndromic craniosynostosis. They note that knowledge of this pathology by local pediatricians and a comprehensive examination of the skeletal system, in particular, the skull of the newborn, contributes to the early diagnosis of craniosynostosis. Timely direction to genetic and neurosurgeon allows for early correction preventing undesirable effects of craniosynostosis.

The article discusses the problem of allergic bronchopulmonary mycoses, which occur in children much more often than they are diagnosed. Their treatment is a challenge, and the prognosis is very problematic. In addition to the most frequent cause of Aspergillus, allergic bronchopulmonary mycoses can be caused by various fungi. The clinical example demonstrates the difficulty of diagnosing and treating a child with allergic bronchopulmonary mycosis caused by a rare type of filamentous fungi Paecilomyces lilacinus.

The article justifies the use of interferon inducers in a clinical practice of pediatrician. The authors isolated a group of derivatives of acridone acetic acid, which effectiveness is associated with the features of pharmacokinetics and pharmacological activity. They demonstrated preventive and therapeutic efficacy of cycloferon in the complex treatment of children with influenza and other acute respiratory viral infections, herpes virus, arbovirus, rotavirus infections, viral hepatitis and tick-borne encephalitis due to its immunomodulatory potency. The article analyzed the use of cycloferon in frequently sick children, patients with allergic diseases, chronic tonsillitis, etc.; also it showed a decrease in the frequency of relapses of both infectious and allergic diseases, the possibility of combined use with other etiotropic and symptomatic drugs.

The article discusses issues related to the introduction of complementary feeding in light of new ideas about its role in preserving child’s health and development. The most controversial topic is protein and carbohydrates consumption, which is significantly changing with the introduction of complementary feeding, both in quantitative and qualitative terms. The results of foreign and domestic studies on the analysis of actual nutrition of children over 6 month old as well as new practical recommendations for introducing complementary feeding are presented. It was found that the actual protein amounts in the recommended diets for children older than 6 months of age in the USA and EU countries significantly exceeds the consumption standards established abroad (FAO / WHO; EFSA, 2017), but is close to the recommended level of protein consumption in domestic practice. Article also reviews the importance of fruit juices in the nutrition of children in the first year of life and gives the rationale for changing tactics of introducing complementary feeding in the pediatric practice in Russia.

The life and scientific heritage of Professor V.I. Molchanov are presented. The article noted that in 1923–1950 he headed the Department of Children’s Diseases of the 1st Moscow State University (since 1930, the 1st MMI). It was emphasized that in 1909 he defended his doctoral thesis on the topic: «The adrenal glands and their changes in diphtheria.» One of the first to study the state of the nervous system in acute infections, particularly scarlet fever, which allowed him to study the pathogenesis of the «scarlet heart», white dermographism in scarlet fever. It is shown that he was the first in the world (1932) to establish a pathogenetic link between scarlet fever and rheumatism. The merit of V. I. Molchanov is the development of a clinic for a number of endocrine disorders in childhood. In his monograph «Disorders of growth and development in children» (1927), a classification of endocrinopathies is proposed, recognized both in the USSRand abroad. Vasily Ivanovich was the first chairman (since 1936) of the committee for the study of children’s rheumatism, chairman of the Moscow Society of Pediatric Physicians since 1941. The pedagogical activities of Professor V.I. Molchanov. The prominent national pediatric school founded by him with some bright representatives of it is covered. Many of the students of Molchanov later became heads of the departments of pediatrics at medical schools in the country and prominent scientists. The article highlights the merits of some of them. It is shown that the image of this extraordinarily talented person, who glorified Russian pediatrics outside of Russia, and now is for the younger generation a vivid example of disinterested service to science.