Summary: The study represents a review of publications covering molecular entity of intestinal permeability and changes causing its disorders. The current concepts on intestinal barrier, tight joints (TJ) and intestinal permeability under normal and pathological conditions are covered. Special attention has been given to molecular unions of tight joints; a role of dysregulation of the components of the TJ complex in the formation of oral tolerance and food allergy is disclosed. It is shown that the assessment of the intestinal epithelial barrier condition can be a significant diagnostic criterion to control the disease and to assess the effectiveness of treatment.

Summary: The review covers the findings of the epidemiological trial on urinary tract infections (UTI) incidence, acute and chronic pyelonephritis in children of Primorsky territory. The significant impact of environmental climatic and heliogeophysical risk factors on pyelonephritis morbidity in children was detected. The most significant factors of UTI development in Primorsky territory were defined. Clinical and laboratory features of this pathology in children at the present day are represented. The ideas of pathogenesis of bacterial inflammation in urinary tract have been broaden. The role of tumor necrosis factor-alpha and its type 2 soluble receptor in persistence and in progression of the inflammation process, which can lead to non-reversible sclerotic changes in kidney tissue, was determined. The role of nitrogen oxide in pathogenetic mechanisms of renal hypertension in children was determined. The complex rehabilitation of patients with local mineral water was considered. The obtained data can be used in further researches, in the work of children›s medical institutions of cities and regions of Primorsky territory.

 Summary: The lecture covers modern concepts of various causes of cardiac arrhythmias in children and adolescents. The working classification of arrhythmias, based on their pathophysiological mechanisms, is given. The diagnostics of cardiac arrhythmias including clinical criteria, various instrumental and ultrasound methods are presented. The group of life-threatening arrhythmias, which are considered to be one of the main causes of sudden cardiac death, is distinguished. Each type of lifethreatening arrhythmias is defined. Medical and surgical methods of treatment are presented.

Objective. The study objective is to analyze data of territorial subjects of the Russian Federation on idiopathic pulmonary arterial hypertension (IPAH) – nosology included in the list of life threatening and chronic advanced rare (orphan) diseases leading to the reduction in life expectancy of citizens or to their disability.

Methods: Based on regulatory legal acts and federal patients registry we have analyzed morbidity and mortality, level of satisfaction with medicines supply and medicines supply expenses for patients with IPAH.

Results: Criteria of level of satisfaction with drugs availability in patients with rare (orphan) diseases are suggested. Satisfaction with medicines supply is measured up against indicators and objectives of “Strategies for drug supply for the population of the Russian Federation for the period up to 2025 and a plan for its implementation”.

Conclusions: The need for further improvement of regulations of orphan diseases sphere, i.e. revision of their list and regional programs creation, is justified.

Objective: The study objective is to assess parents’ opinion and awareness of preventive vaccination issues.

Methods: Using the data of cross-sectional multicenter study the assessment of 1620 parents’ awareness of preventive vaccination issues was taken; we checked up on factors causing decline of trust in vaccination.

Results: The most respondents (n=1590) vaccinated children adhering to recommendation of National calendar, 25 % of parents additionally vaccinated them due to epidemic indications. The refusal of vaccination in 2 % of respondents was influenced by level of education (lower secondary education), family financial state (average or above the average), choice of mass media as prevailing source of information about vaccination. High level of credibility to the opinion of neighborhood pediatrician (91.7 %) and parents› interest in receiving additional information about vaccinations (71.2 %) were revealed.

Conclusions: Providing the accurate information on vaccination by doctors will enable to increase parents’ adherence to children vaccination.

Objective: The objective is to analyze the availability of vitamin D in children with allergic diseases (AD) in polymorphism of the vitamin D receptor (VDR) gene.

Methods: The main group included 130 children with allergic diseases aged from 1.5 to 16 y.o. The control group included 41 apparently healthy children aged from 1 to 10 y.o. The analysis of polymorphic markers FokI (rs2228570), BsmI (rs 1544410) and TaqI (rs 731236) of gene VDR was carried out by polymerase chain reaction method in realtime mode using detecting amplifier DT-96 and DNA-diagnostics sets. The assay of metabolite 25(OH) D (25-Hydroxyvitamin D2 and D3) was carried out by an immunoenzyme method.

Results: Children with AD demonstrated a significantly increased degree of incidence of A-allele in the site of BsmI gene VDR and carriage of homozygous (A/A) and heterozygous (G/A) of its genotypes. The statistically significant decrease of 25 (OH) concentration was established in heterozygous variant A/G and in homozygous variant G/G of FokI site of gene VDR.

Conclusions: The findings lay the groundwork for development of individual approach to prevent vitamin D deficiency in children with AD.

Objective. The objective is to analyze molecular and genetic mechanisms of endothelial dysfunction development in children with allergic enteropathy.

Methods: We have studied the polymorphisms of endothelial nitric oxide synthase gene and the level of nitric oxide metabolites in coprofiltrates in 43 children aged from 6 months to 3 years with allergic enteropathy.

Results: The endothelial dysfunction in allergic enteropathy is associated with nitric oxide molecule. Carriage of the homozygous allele СС Т786С of the endothelial nitric oxide synthase gene raises the risk of IgE-independent food allergy development by 8.8 times.

Conclusions: The conducted study evidences the active participation of nitric-oxidergic mechanism in allergic inflammation development.

Objective: The study objective is to analyze the efficacy of antibacterial medication selection to treat community-acquired pneumonia (CAP) in children in an out-patient clinic and in a pulmonology department of an in-patient hospital.

Methods: Retrospective descriptive study for 2009–2017 in day-patient departments of pediatric clinics and pediatric pulmonology departments of in-patient hospitals of Primorskiy territory (760 cases).

Results: 50 % of children took antibiotics at pre-hospital stage (self-treatment). Penicillins were the most popular – 50.1 % (218 prescriptions), cephalosporins were used in 129 (29.7 %), macrolides – in 86 (18.8 %) of cases. The average duration of antibacterial treatment course of CAP was 10 days at pre-hospital stage. Both in the out-patient clinic and in the in-patient hospital, cephalosporins (58.4 and 58.3 %), macrolides (34.8 and 37.4 %) and aminoglycosides (6.3 and 3.4 %) were used more often.

Conclusions: The positive dynamics in the protocols of antibiotic therapy of CAP should be noted. The improvement of regulatory framework has led to the increase of rational prescriptions. It is necessary to continue improving of clinical guidelines and implementing pulmonology hospitals of microbiological monitoring that will allow improving the quality of antibacterial treatment.

Objective. The objective is to analyze the features of sensitization to food allergens in vegetarian children.

Methods: 210 children aged from 3 months to 17 years and 11 months old: vegetarians with at least 6 months vegetarian experience (n = 129) and children receiving a traditional diet (n = 81) were examined. The level of specific immunoglobulin E of blood serum to cow milk protein, soy, beef, pork, chicken, fish (cod), egg white and wheat was determined.

Results: The rate of sensitization to food allergen in vegetarian children did not statistically differ from that of a comparison group (18.6 against 17.3 %, p>0.05). The correlation analysis showed a slight association between vegetarian diet and the level of specific immunoglobulin E only for soy protein (r = 0.22, p<0.05).

Conclusions: The obtained results speak of higher risk of sensitization to soy in vegetarian children.

Objective: The objective is to assess the clinical efficacy of the inclusion of release-active medication of affinity purified antibodies to protein S100 in complex rehabilitation of infants with old acute obstructive bronchitis affected by perinatal pathology of central nervous system of hypoxic genesis.

Methods: 210 infants aged from 2–36 months, who suffered events of acute obstructive bronchitis, were examined. Along with clinical methods of examination, a computer bronchophonography and assessment of heart rate variability were performed.

Results: Additional inclusion of release-active medication ‘Tenoten for children’ in the complex of rehabilitation of these patients had a positive effect on autonomic balance and autonomic component of bronchial obstruction. At the same time, the short course of Tenoten (1 month) did not prevent recurrent events of bronchial obstruction.

Conclusions: Probably, patients with recurrent events of acute bronchial obstruction in combination with pronounced functional disorders of vegetative state require longer courses of release-active medication which normalized the activity of the central nervous system.

Objective: The objective is an optimization of medical support of children after transplantation of liver and kidneys based on analysis of frequency, etiology and nature of infectious complications.

Methods: From 2017 to 2019, 40 transplantations of solid organs were performed in various medical institutions at 38 children, having lived in Novosibirsk region: 23 liver transplantation, 13 kidney transplantation and 2 single-stage transplantation of a part of the liver and the kidney. Imunosuppressive protocol components were lymphocyte non-exhaustion monoclonal antibodies, glucocorticosteroids, calcineurin inhibitor, mycophenolates. Besides immunosuppression right after the transplantation, 78.9 % of patients received Valgancyclovirum under inpatient treatment, and 65.8 % of patients continued taking it under outpatient treatment. In all cases, patients received Co-trimoxazole and Fluconazole for preventive purposes.

Results: Right after the organs transplantation 44 cases of infectious complications of different etiology were detected. 39 infectious complications were diagnosed during early post-operative period, including 24 – after the liver transplantation, 15 – after the kidney transplantation. 17 longterm infectious complications were registered, in most cases (64.7 %) – after the liver transplantation. In general there is no significant difference in the frequency of complications after transplantations.

Conclusions: Patients, having undergone the transplantation of solid organs, need a dynamic monitoring of markers of different infections. Taking into account persistent nature of infectious complications and high frequency of their occurrence, especially during the first six years, patients need a preventive prescription of Valgancyclovirum and Co-trimoxazole as well as prevention of urinary system infections after the kidney transplantation.

Objective: The objective is to analyze epigenetic and clinical features of course of the cow’s milk protein allergy (CMPA) and lactase deficiency (LD) in infants.

Methods: We have assessed the factors contributing to the formation of CMPA and LD in children with calculation of attributable risk rate. Intensity of clinical symptoms was measured with CoMiSS.

Results: The analysis according to CoMiSS showed the presence of general gastrointestinal symptoms in patients with CMPA and LD. The assessment of attributable risk factors gave the opportunity to create a scale of CMPA development.

Conclusions: The invented scale will help to extract infants from the risk group and take complex of preventive measures.

Objective: The objective is to analyze the influence of nutrition on permanent dentition processes in children of early and middle childhood age.

Methods: The study of the permanent dentition time periods in children going to municipal budgetary educational institutions of Vladivostok was performed. The sanitary audit of everyday nutrition was carried out. The causeeffect relations between nutrition and permanent dentition were identified.

Results: First permanent teeth started erupting in girls in 4.5 y.o. The analysis showed children nutrition deficiency and imbalance in nutrient content of their diets. The portion of nutrition factor influence on permanent dentition in girls of early childhood age was 36.0 ±3.2Influence of nutrition on permanent dentition in children living in Vladivostok, in boys of early childhood age – 38.4±3.7 %, in girls of middle childhood age – 36.0±4.4 %, in boys of middle childhood – 36.0±0.2 %.

Conclusion: The nutrient deficiency and dietary imbalance of a child serves as risk factors of the permanent dentition disorders. Developing a diet for children it is important to consider their physiological requirements at each age with mandatory inclusion of products containing sufficient amount of macroand microelements.

Objective: The objective is to develop an algorithm of initial antimicrobial treatment of urinary tract infection (UTI) concidering the etiological factor Enterococcus faecalis, having spontaneous resistance to cephalosporins.

Methods: The analysis of 6438 results of urine bacteriological examination of children with UTI, having received the treatment in multi-field children’s hospital for 9 years, and clinical manifestations of UTI associated with E. faecalis in 181 patients was carried out. Addirionally, antimicrobial susceptibility testing was carried out in 60 cultures of E. faecalis.

Results: E. faecalis is occurred to be the second etiological factor of UTI in order of importance in children and had high priority in its development in newborns. The detected diagnostic markers of UTI associated with E. faecalis, and its antimicrobial susceptibility are represented in the author-developed algorithm of the initial antimicrobial treatment.

Conclusions: The algorithm of the initial antimicrobial treatment of UTI caused by E. faecalis will help to prescribe a sustainable, early antimicrobial treatment at the stage of microbe identification.

Objective: The objective is a comparative analysis of vitamin D content and level of proinflammatory cytokines in infants born by mothers with endocrine disorders, and in infants of women with normal body weight.

Methods: The clinic-anthropometrical and laboratory observation of 78 children born in Stavropol Regional Clinical Perinatal Centre: 36 infants were born by women with obesity and 18 – by women with gestational diabetes mellitus associated with obesity, 24 – by women who did not have obesity and clinical and laboratory signs of intolerance to glucose (control group). The level of calcidiol and interleukin 1β and 6 were determined in blood serum.

Results: The content of vitamin D in infants born by healthy women was higher than in infants born by mothers with gestational diabetes mellitus associated with obesity and by mothers with obesity. Infants born by mothers with obesity had higher level of proinflammotary cytokines than those of control group.

Conclusions: Children, born by mothers with endocrine disorders, have lower level of calcidiol and require postnatal prevention of vitamin D deficiency. Also it is necessary to take vitamin D during the pregnancy to prevent complications in pregnancy and neonatal pathology.

Objective: The objective is to analyze clinical and laboratory features of infectious mononucleosis (IM) depending on disease etiology.

Methods: 102 children with IM aged from 1 to 15 y.o. were examined. Polymerase chain reaction and immunoenzyme method were used to verify disease etiology.

Results: In patients with Epstein–Barr virus and mixed infection, the most pronounced manifestations of lymphoproliferative syndrome and tonsillitis, high content of atypical mononuclear cells in peripheral blood are detected. Aspects of IM of cytomegaloviral etiology were notable for the prevalence of toxic syndrome along with other less pronounced clinical and laboratory manifestations of the disease.

Conclusions: The detected features of the aspects of IM depending on etiological factor allow to prognosticate a variant and a character of the disease course and to early improve the therapy.

Objective: The objective is to assess the influence of cardiac rehabilitation (CR), represented by self-controlled walking in middleaged men with different onsets of coronary heart disease (CHD), on the muscular strength of the hand flexors, muscle mass and 6-minute walking distance.

Methods: 90 men aged from 45 to 65 with the same prescriptive CHD with different onsets: myocardial infarction (MI) – 56 patients, or stable angina – 34 patients were examined. After 9 months of CR we have assessed the muscular strength of the hand flexors (MSHF), have run the bioelectrical impedance analysis and 6-minute walking test, have determined the level of testosterone in blood serum.

Results: In patients with CHD with MI as an onset, MSHF of the right hand increased from 43 to 49 DAN at the average, and from 41 to 46 DAN – of the left hand; equity proportion of muscle tissue increased from 48.4 to 48.8% , 6-minute walking distance – from 450 to 500 meters. In patients with CHD with stable angina as an onset, the proportion of muscle tissue decreased from 48.2 to 47.3 %, 6-minute walking distance – from 405 to 400 meters without significant improvement of MSHF.

Conclusions: 9-month CR in patients after MI had a positive effect on MSHF, muscle tissue and stamina as opposed to the patients with stable angina.

Objective: The objective is to analyze the seroepidemiology and resistance of Streptococcus pneumoniae in children over 5 y.o.

Methods: 57 patients with pneumonia and 186 healthy children were examined. The induced sputum and nasopharyngeal swabs were analyzed with culture and molecular assays.

Results: S. pneumoniae incidence among healthy children was 15.1 %. Serotype flora was represented by 4 serotypes and 6 serogroups with prevalence of 19F, 37 (for 17.8 % each), 6АВС, 3 (for 10.7 % each). 17.8 % of strains were antibiotic resistant, four of them was 19F serotype, and one was of the 6th serogroup. All of them were multiresistant: to macrolides, lincosamides, tetracyclines, cephalosporins of II generation along with moderate resistance to penicillin. In children with pneumonia, pneumococcocus was detected in 24.6 % of cases: serotypes 19F (64.3 %), 19А (21.4 %), 23F (14.3 %). 57.1 % of strains were multiresistant and was of 19F and 19А serotypes. Phenotype of the resistance was identic to the described above except the resistance to amoxicillin: all strains were resistant to it. All given isolates were moderately resistant to ceftriaxone.

Conclusions: Serotype flora of pneumococci in healthy children of school age was distinct in significant variety with high incidence of ‘mucoid’ serotypes 3 and 37 (28.6 %). In pneumonia, pneumococci of 19F and 19A types occurred significantly frequently. The main resistant serotype of S. pneumoniae in both groups was 19F with lowered sensitivity to beta-lactam antibiotics.

Objective. The objective is to develop methods of a process of forensic medical examinations in neglected schoolchildren cases.

Methods: 70 forensic medical examinations of children and adolescents were studied, whom legal representatives were accused of neglecting needs of their foster children. Criminal cases and medical documents were analyzed.

Results: Neglected schoolchildren had low neuropsychic development, high level of anxiety, negative emotional state, were dissatisfied with family situation. The structure of their disease was characterized by impaired psychological development, advanced stage of pulmonary diseases, dental caries, anemia, potential risk of tuberculosis infection. 40 % of children had weigh deficit, 28.6 % were retarded, and 44.3 % had decreased muscle strength.

Conclusions: Children were divided into two risk groups depending on neglecting of their needs: children from multiple children single parenting families and children whose mothers avoided medical observation during pregnancy. The range of factors indicating needs neglecting were detected: malnutrition, disregard of hygiene care and sanitary regulations at home, lack of medical care, lack of appropriate living conditions and learning environment, disregard of safety and supervision, absence of day regimen. The comprehensive assessment of children health condition under forensic medical examinations with account of obtained data will allow an enforcer to make a well-founded decision when investigating crimes, which are punishable under Criminal Rule 156 of the Russian Federation.

Objective: The objective is to improve non-invasive diagnostic algorithm (NDA) to identify food allergy (FA) in infants since their first months of live.

Methods: We have done clinical and anamnestic examination and subsequent follow-up of 26 infants aged from 1 week old to 9 months old having admitted with complaints about colics, diarrhea, abdominal distension and skin rashes. Primary screening was performed using CoMiss tool, then anamnesis data was collected according NDA.

Results: FA was diagnosed in 34.6 % of infants due to improved NDA. Case follow-up suggested the presence of FA in 46.1% of patients.

Conclusions: The questionnaire showed a high clinical effectiveness and uniqueness, exceeding current alternatives for non-invasive diagnostics of malabsorption syndrome in infants.

Summary: In recent years doctors and medical institutions are increasingly in the focus of law enforcement bodies, the number of criminal cases against doctors and the number of cases bringing to administrative responsibility of medical institutions is growing. Despite the fact that defects in the provision of medical care are detected in half of the cases, a direct cause-effect relation and the determination of the severity of harm to health are detected in isolated cases. The right legal evaluation of action (inaction) of a medical worker in a specific situation can be given under methodologically correctly performed examination.

Summary: The brief literature summary covering the congenital nephrotic syndrome (CNS) and two clinical observations of this disease are represented. Symptom complex of nephrotic syndrome manifested by a massive proteinuria, edemas, hypoproteinemia during the first three months after childbirth was the essential in establishing a diagnosis in both patients. In most cases CNS is caused by genetically-mediated defects of the glomerular filtration barrier components. In patients, clinical manifestations (phenotype) were absolutely similar, and etiology (genotype, other reasons) was different. The expert analysis of medical documentation detected diagnostic and therapeutic errors in patient management which in the second observations have led to the death. Based on the described cases, it is shown the necessity of complex approach to examining children with CNS to early diagnose supplemental syndrome variations of pathology, to determine vital and nephrologic prognosis for the disease and to develop therapy management (personalized therapy).

Summary: A case of Shwachman–Diamond syndrome with typicl symptoms of this disease (bone, intestinal, hematological) diagnosed in at an early age is described. Authors believe that the mainstreaming of discipline on orphan diseases with interdisciplinary diagnostic and medical approaches in training programs including post-graduate training is a compelling need to train a modern professional in the field of pediatrics.