Dilated cardiomyopathy is a common cause of heart failure and the most common pathology in patients referred for heart transplantation. In childhood, difficulties arise in the differential diagnosis of various types of cardiomyopathies due to the lack of research methods such as cardiac magnetic resonance imaging, endomyocardial biopsy, and molecular genetic analysis in the routine practice of a doctor. In this regard, the pediatrician and pediatric cardiologist need to pay special attention to the clinical symptoms of heart failure, which are often disguised as anatomical and physiological characteristics in young children, and also be able to interpret and analyze the results of additional examination: hypertrophy of the atria and ventricles of the heart, signs of stress , disturbance of repolarization processes according to electrocardiography, determine cardiomegaly and disturbances of blood flow in the lungs according to X-ray examination, and also understand the significance of key parameters of transthoracic echocardiography. Early diagnosis of cardiomyopathies in children is extremely important, since all myocardial diseases can be associated with sudden cardiac death syndrome. The incidence of sudden death in children with dilated cardiomyopathy varies from 1.5 to 4%; in most cases, the cause of death is life-threatening cardiac arrhythmias, which also require early identification. Timely diagnosis and comprehensive treatment can improve the prognosis and quality of life of children with this pathology. In the article, we present a clinical case of difficulty in diagnosing dilated cardiomyopathy in combination with thrombophilia in a young child; later detection of the concomitant pathology led to complications such as left ventricular thrombosis and severe systolic dysfunction, which subsequently required serious and long-term anticoagulant and cardioprotective therapy.

Introduction. Modern preventive measures of skin diseases aim to maintain the protective skin barrier. The area that comes in direct contact with the diaper becomes excessively wet, the corneous layer is damaged, which results in a weakened barrier function of the epidermis and the occurrence of an inflammatory process, diaper dermatitis.

Aim. To study the clinical efficacy and safety of the use of disposable diapers and pull up pants in full-term and premature infants from birth to 1 year of age to prevent diaper dermatitis.

Materials and methods. A total of 45 full-term and premature children under 1 year old with various diseases, including skin eruptions in the area covered by the diaper were included in the study. The proposed care products were used for 14 days on the strict condition that they would be reapplied after defecation and during hygiene procedures in a timely manner. The skin status was assessed visually by a paediatrician from Day 1 to 14 of the study using the M. Odio skin assessment scale.

Results. The M. Odio scale evaluation of the skin around waist, buttocks, genitals, anal region, and inguinal folds in all children on Days 1, 7 and 14 showed 0 scores. No adverse events were reported during assessment of the skin status over 14 days of the use of care products.

Discussion. The observations were consistent with the previously published data demonstrating that maintenance of the functional characteristics of the skin in the area covered by the diaper and exposed to faecal and urinary enzymes, elevated temperature and humidity prevented the development of diaper dermatitis and skin diseases.

Conclusion. No signs of diaper dermatitis were detected in all 45 full-term and premature children under 1 year old, including children with various diseases, during 2 weeks of application of disposable Tanoshi Premium diapers and pull up pants in clinical practice. This fact proves the safety and good tolerance of disposable diapers by children under 1 year old provided that the skin care requirements were met. Tanoshi Premium diapers and pull up pants can be recommended for babies of different ages and body weights, including those with diaper dermatitis.

An analytical review of scientific literature, international acts and Russian regulatory documents on the issues of implementation of anti-epidemic actions in tuberculosis among the general population and, on a priority basis, in high-risk groups – migrants (refugees and resettlers) are presented. The review is aimed to raise awareness among readers about the RF regulatory environment on this issue, as well as the results of research on migrants’ access to the measures on early detection, diagnosis, prevention and treatment of tuberculosis in Russia. The features of identification of disease cases among migrants have been determined based on the results of observation of the formation of an epidemic situation in the border region of the Russian Federation under extreme conditions and development of a pandemic. In the period before, during the development and after the pandemic, 2268 newly diagnosed and recurrent tuberculosis patients were followed-up, who were identified using various approaches and diagnostic methods: radiation (fluorography + radiography), bacteriological, molecular and genetic, computed tomography, histological studies. There are interesting data on the use of immunological methods. The conclusions: the created regulatory environment to identify, treat and prevent the spread of tuberculosis in the country, including high-risk groups such as migrants/refugees, among whom there are many children, ensured the implementation of controlled anti-epidemic actions under extreme conditions conducive to the formation of an epidemic situation; extreme conditions, including a pandemic/ epidemic of other etiologies, affected the formation of the epidemic situation of tuberculosis, as well as among children and adolescents, in the region; the efficiency of  immunological methods, primarily the recombinant tuberculosis allergen, in the high-risk groups under consideration has been proven.

Introduction. Rhinosinusitis, along with adenoiditis, holds leading positions in terms of incidence among adults and children. About 10 million cases of rhinosinusitis per year are reported in Russia.

Aim. To study the effectiveness and safety of secretolytic drugs in the treatment of acute rhinosinusitis in different groups of patients, features of the course of rhinosinusitis in patients with concomitant disorders of the immune system.

Materials and methods. A total of 98 medical records of children with acute sinusitis that was diagnosed by an otolaryngologist during the initial examination based on complaints, past medical history data and paranasal sinuses x-rays were selected for analysis. Of these, 60 children aged 3 to 15 years (23 girls and 37 boys) were included in Group 1. 38 immunocompromised children aged 3 to 12 years (18 girls and 20 boys) were included in Group 2.

Results. In the group of patients without oncohematological diseases (Group 1), the average duration of bacterial sinusitis was from 6 to 12 days, if the standard treatment regimen was ussed; if therapy included Sinupret complex-action herbal medicine, children were ill for an average of 6.46 days, and the average duration of the disease in patients who did not receive the herbal medicine was 3 days longer, totalling to 9.54 days. Patients with various immunocompromising diseases, who received specific immunosuppressive therapy, on average were ill significantly longer – from 8 to 16 days on systemic antibacterial therapy. Moreover, the average duration of sinusitis in patients, who additionally used Sinupret was 7.5 days, which was almost 5 days less (12.4) than in patients, who received treatment without Sinupret herbal medicine. The article also presented the results of treatment of patients receiving several immunosuppressive drugs, who had a graft-versus-host reaction, cytopenia and other unfavorable factors.

Conclusion. The inclusion of the herbal medicine with complex action in the treatment regimen significantly improves the positive effect of therapy, reduces the duration and intensity of clinical presentations, even if a patient has immunological diseases and immunocompromising conditions.

The article considers the main pathological processes in the outer and middle ear, which are detected at the primary paediatric visit. A particular focus has been placed on the importance of otoscopy and the need for sustainable routing of a child with ear pain. The basic principles for the treatment of acute external and otitis media in children are outlined, and indications for additional examinations and systemic antimicrobial therapy are determined. The benefits of topical combination ear drops in the treatment of acute external and otitis media are presented. The benefits of combination ear drops in the treatment of ear pathology in children from 6 years of age are demonstrated by examples of clinical studies and retrospective analysis of clinical efficacy. Clinical cases from our own observations are presented: a 7-year-old patient with acute non-perforative otitis media and a 12-year-old patient with acute exacerbation of chronic external otitis, myringitis. The combination ear drops containing chloramphenicol, beclomethasone dipropionate (anhydrous), clotrimazole and lidocaine hydrochloride monohydrate demonstrated high clinical efficacy. The combination drug with a beneficial and clinically proven effect can help solve the issue of otitis in children from 6 years of age. The authors have developed a treatment and prevention reminder for ear pathologies in children, which will help the paediatrician explain the main principles of treatment and prevention of otitis media to parents in an easily accessible form. It was concluded that ear pathology is a current and multi-etiological challenge that requires attention and expert knowledge of a paediatrician. The active parental involvement in ear pathology prevention in their child will help their children to grow physically healthy.

Introduction. Type 1 diabetes in children creates difficulties and requires certain conditions for control while attending school/ kindergarten, while children should receive education without any restrictions.

Aim. To identify problems and assess the needs of children with type 1 diabetes mellitus and their parents during their stay in preschool and general education organizations.

Materials and methods. A sociological online survey of 684 parents of children with diabetes was conducted in September- October 2023 in 8 federal districts of Russia. The electronic questionnaire contained 117 questions on 9 main blocks of studying the problems of children staying in schools/kindergartens.

Results. The age of children is from 2 to 18 years (median – 9.9 years); gender of children: female – 50.9%, male – 49.1%; diabetes experience – from 1 month. up to 15 years (median – 3.6 years). Parents consider the main problems to be difficulties with organizing meals (54.7%), administering insulin (52.8%), controlling glycemia (44.9%), their own fear (41.2%), and mistrust of teachers (20.8%) and nurses (11.1%), administrative barriers (29.4%). Teachers do not have sufficient knowledge about diabetes in children (78.5%), teaching aids (53.0%), regulations on their responsibilities when accompanying children (46.0%), 54.8% of them are afraid, 87.9% cannot help a child in an emergency. Parents are concerned about the lack of nurses (80.1%) and a separate room for self-monitoring (47.7%) at school. According to parents, educational organizations need awareness-raising work (85.8%) and support from an assistant/tutor (56.5%) for children with diabetes.

Conclusion. It is possible to solve the identified problems through the cooperation of teachers, parents and children, endocrinologists, psychologists, social workers and government authorities who can remove barriers and create conditions for a comfortable stay for children with diabetes in educational organizations.

Obesity is a global problem in pediatrics. Scientific interest in the study of this disease in childhood and adolescence is due to a number of factors: a steady increase in the number of patients in most countries of the world, unfavorable long-term consequences and complications, its progression into adulthood. These circumstances dictate the need for health professionals to search for new methods of treatment and prevention. The basis of treatment is a comprehensive approach, including normocaloric diet, increased physical activity, behavioral therapy and psychological support. However, these methods are often ineffective. In recent years, medications, particularly glucagon-like peptide-1 agonists (the drug liraglutide), have been approved for effective weight loss. However, there is some clinical stagnation on the part of the medical community regarding its prescription due to the lack of clear criteria determining the validity and increasing the effectiveness of treatment. On the basis of clinical observations, to propose criteria determining the possibility of prescribing a domestic analog of liraglutide (enligria) to improve the effectiveness of treatment of obesity in adolescent children. Four clinical cases demonstrated the high efficacy of liraglutide with respect to leveling cardiometabolic risks regardless of the degree of obesity and increasing adherence to lifestyle modification principles in patients with a metabolically “healthy” obesity phenotype. Also, its use was associated with facilitating the management of different types of eating disorders. Additional criteria determining the validity of liraglutide prescription in the therapy of obesity in adolescent children were proposed. It will contribute to a wider introduction of this drug into practical healthcare.

The development of post-infectious pathology is determined by the type of pathogen, the genesis of damage to the intestinal barrier and the premorbid background of the child. On the scale of recent epidemiological events, attention is drawn to the SARS-CoV-2 virus, which has tropism for cells of the gastrointestinal tract, distorts the composition and function of the intestinal microbiome. The importance of the functions of intestinal microbes in the genesis and outcome of SARS-CoV-2 infection has been highlighted. It has been proven that a microbiota disorder with a new coronavirus infection affects not only the duration and severity of the infectious process, but also the risk of the formation of systemic diseases and malignant neoplasms. After the acute phase of SARS-CoV-2 infection, post-COVID-19 syndrome may occur, the pathophysiology of which has not yet been fully established. Although it is believed that the main reason for its appearance is the diversification of the intestinal microbial landscape against the background of the direct effect of SARS-CoV-2 on the ACE2 receptors of intestinal epitheliocytes, followed by a pathoimmune response and impaired hemostasis. Established post-COVID-19 dysbiosis underlies functional gastrointestinal disorders with pronounced impairment in the brain-gut-microbiota axis and imbalance of the migrating motor complex, leading to the development of constipation. Post-infectious constipation debut within the first 3 months after an acute infection. The main goal of therapy for post-infectious constipation is to achieve regular painless defecation with soft-consistency stool and prevent feces. Numerous foreign studies and clinical guidelines indicate that stimulating laxatives are effective and safe in the treatment of constipation, include the children’s practice. Sodium picosulfate is considered one of the topical drugs of this group of laxatives.

Breast milk is the “gold standard” for feeding infants for the first years of life and affects the children health both at an early age and in subsequent periods of their life. However, there are times when women are unable to breastfeed for the following reasons: a woman cannot make milk or has health problems, or lose interest in breastfeeding, or should be back at work, etc. This brings up a legitimate question: how to choose among a large number of infant formulas for healthy infants the one that will fit and be safe for a child to use, taking into account their gestational age, as well as a large number of individual “risk facts” such as high or low body mass and height parameters, features of a childbirth (“vaginal”, operative delivery), etc. Most often, the choice falls on cow-milk-based infant formula. After all, one should not forget about alternative options such as goat-milk-based formulas. Historical evidence shows that patients with rickets were fed with goat milk and it was believed to affect brain functions. Interest in the beneficial effects of goat’s milk has been steadily increasing to this day, given that the biological active substances present in goat’s milk are also present in women’s milk in larger quantities. The _β-casein fraction is its main component, which makes it as similar to breast milk in structure as possible. In addition to proteins, fats are components that contribute to the excellent digestibility of goat milk. Their distinctive feature is smaller size as compared to milk fats from other animals. This article explores all the benefits of goat milk and its effect on all aspects of infant health.

The article is devoted to a review of the literature on the study of intestinal microbiota in chronic viral hepatitis in children. The results of recent studies of microbial diversity of colon contents using 16S ribosomal RNA sequencing are presented. Scientific research in recent years has proven the influence of changes in the microbiota and its individual representatives on the formation of complications in patients with chronic viral hepatitis, the development of fibrosis/cirrhosis. Changed quantitative and species ratios of microorganisms can lead to the launch of pathological reactions in the intestinal-liver axis system. A significant reduction in the number of bacteria such as Clostridia and Bacteroidia (Faecalibacterium, Roseburia inilinivorans, Roseburia intestinalis, Coprococcus comes) causes low production of short-chain fatty acid butyrate, which can interfere with the functioning of tight junction proteins. Leaks and increased intestinal permeability create conditions for the formation of inflammation due to the activity of pro-inflammatory cytokines. Violation of the intestinal barrier function creates the opportunity for the penetration of bacteria, lipopolysaccharide complexes, bacterial ligands and metabolites, leading to the development of immunopathological reactions. These changes in the pediatric population are less noticeable than in the adult cohort of patients, since the clinical course of chronic viral hepatitis in children has a smoother course. However, periods of exacerbation, which are characterized by clinical and laboratory changes, may maintain the risk of developing fibrogenesis activity in older age groups. Data on intestinal microbial diversity in adult patients with chronic viral hepatitis B and C are presented separately. The possibilities of using pre- and probiotic therapy in the complex treatment of chronic liver diseases in adults and children are considered.

Pediatric skin has some peculiarities that may determine why children are more likely to develop some skin conditions. In pediatrics, contact dermatitis is one of the most prevalent skin diseases involving impaired epithelial barrier. To reduce skin inflammation, decrease in frequency, duration, and number of contacts with triggering factors along with skin barrier restoration are needed. The Anti-inflammatory hormonal topical therapy represents the main treatment method. This article demonstrated several clinical cases of contact dermatitis in toddlers that were treated with methylprednisolone aceponate accompanied by ceramides and showed high therapy effectiveness in toddlers. A case of treatment of a child aged 1 year, 3 months with asymmetrical widespread hyperemic spots on the back surface of the body, mainly in the lumbar region; a case of treatment of a 2-year-old child with multiple hyperemic spots located on the anterior and, to a greater extent, posterior surface of the lower extremities, having clear edges, accompanied by focal dryness and moderate peeling at the edges; as well as a case of treatment of a child aged 1 year 11 months with hyperemic asymmetrical dry spots of irregular shape on the posterior and medial surface of the left forearm, which tend to merge and have clear edges. The use of topical corticosteroid in combination with ceramides in pediatrics leads to effective and fast inflammation decrease along with visible skin restoration.

Proactive therapy in dermatology is defined as a long-term intermittent application of anti-inflammatory topical agents to maintain a stable and long-term remission in chronic recurrent dermatoses. Proactive therapy is prescribed after reactive treatment in atopic dermatitis, chronic dermatoses, and involves emollients, topical corticosteroids, and topical calcineurin inhibitors. Intermittent therapy allows to control the course of the disease and prolong remissions, which has a positive effect on the patients’ quality of life. The duration of therapy is determined on a case-by-case basis, taking into account the severity of dermatosis and relapse rates. Thus, proactive treatment for atopic dermatitis can last from 12–16 weeks (mild level) to 52 weeks and longer in severe cases. The anti-inflammatory effect of pimecrolimus and tacrolimus has been shown to prevent the production and release of pro-inflammatory cytokines (IL2, IL3, IL4, IL8, IFN_γ, TNF, GM-CSF) and to inhibit T-lymphocyte proliferation, and topical calcineurin inhibitors prevent the release of inflammatory mediators (histamine, tryptase) from activated mast cells. In addition, the active substance of tacrolimus and pimecrolimus binds to the protein receptor macrophilin-12 (FKBP-12). The review article looks at whether the outcomes of the recent studies show the effectiveness of proactive therapy, increase in relapse rates in children with atopic dermatitis while using pimecrolimus cream, 1% and emollients after the inflammatory process has been stopped. In-house data on the analysis of the relapse rates in 22 patients (9 girls and 13 boys) aged 6 to 14 years with moderate atopic dermatitis, who were under observation from 2020 to 2022, are presented. Analysis of study results and our own observations demonstrated that such complex external treatment with topical calcineurin inhibitors and emollients can reduce the rate of exacerbations of atopic dermatitis and improve the patients’ quality of life.

Introduction. Currently, there are insufficient studies to demonstrate the effect of vitamin D status on the production of proinflammatory cytokines in children with allergic diseases, which precipitated this study.

Aim. To analyse interferon_-γ, interleukin-17A and 33 profiles in children with asthma according to serum calcidiol levels before and during cholecalciferol subsidies.

Materials and methods. A total of 71 children aged 3 to 18 years were examined: 51 patients with asthma and 20 children in the control group. A 3-month cholecalciferol course at a prophylactic dose specified in the national program was prescribed to all children. 25(OH)D, interferon-_γ, interleukin-17A and 33 levels were twice determined in the examined children.

Results and discussion. Median 25(OH)D levels in patients with asthma did not reach the levels of healthy children either before or after cholecalciferol subsidies at prophylactic doses. Vitamin D intake led to a decrease in interferon-_γ levels in healthy children from 3.07 [2.29; 4.81] pg/ml to 2.18 [1.74; 3.45] pg/ml (p < 0.05). In patients with asthma, such changes were not detected in the initial population, however, after cholecalciferol subsidies their interferon-_γ levels were significantly higher than those in healthy children: 3.11 [0.89; 5.0] pg/ml and 2.18 [1.74; 3.45] pg/ml, respectively (p < 0.05). Assessment results of interleukin-17A levels in children with asthma showed that the median cytokine levels were significantly higher before the cholecalciferol subsidies than after them: 2.03 [0.1; 10.01] pg/ml and 0.96 [0.1; 12.87] pg/ml, respectively (p = 0.03). The median interleukin-17A levels in children with asthma were significantly higher than in healthy children, both before and during the cholecalciferol subsidies. The median interleukin-33 levels were significantly higher in children with asthma as compared to healthy children, both before and during vitamin D subsidies.

Conclusion. Our results suggest that cholecalciferol has a modulatory effect on interferon-_γ and interleukin-17A in patients with asthma. Interleukin-33 levels did not change significantly in children with asthma on cholecalciferol.

Aim. To study the effectiveness of a combined immunomodulatory drug consisting of complex immunoglobulin preparation and recombinant interferon-_α2b in children of the first three years.

Materials and methods. There were examined 55 children with acute respiratory viral infection of the upper respiratory tract from 2 months to 3 years. Of these, the main group consisted of 30 children receiving, as part of complex therapy, a combined immunomodulatory drug (Kipferon) in the form of rectal suppositories for children under one year old – 1 suppository per day, for children over one year old – 1 suppository twice a day for 5–7 days. The comparison group included 25 children receiving symptomatic therapy for acute respiratory viral infection, which did not differ in the compared groups

Results. It was revealed that the prescription of a combined immunomodulatory drug leads to a positive clinical effect in 90.0% of cases. While taking the drug, there is a more rapid relief of fever and symptoms of intoxication, as well as local inflammatory manifestations of acute respiratory viral infection (hyperemia of the pharynx, rhinitis, cough), which led to a shortening of the duration of the disease by 1.3 times and a reduction in the need for antibiotics by 2.4 times.

Discussion. The results of the study, which showed significant advantages of including a combined immunomodulatory drug in the acute respiratory viral infection treatment regimen, are comparable with the available literature data.

Conclusions. Considering the high clinical efficacy and safety of the combined immunomodulatory drug Kipferon, it can be recommended for widespread practical use in acute respiratory viral infections of the upper respiratory tract in young children.

The prevalence of allergic diseases is increasing at rates corresponding to epidemic’s spread rates. Cow’s milk protein allergy (CMPA) is one of the most common food allergies in infancy. Its clinical variants remain hard-to-diagnose diseases due to their multiple clinical faces. Food allergy can develop along the pathway associated with both IgE and non-IgE, or mixed one. Non-IgEassociated variants of food allergies, due to the lack of accurate and specific laboratory markers, can cause significant difficulties in making a diagnosis. The search for new diagnostic markers continues. Faecal calprotectin, a calcium-binding leukocyte protein consisting of a complex of two monomers S100A8 and S100A9, may be one of them. Faecal calprotectin concentration is directly proportional to the number of leukocytes migrating into the intestinal wall, due to which faecal calprotectin becomes an accurate, non-invasive and sensitive indicator reflecting the level of inflammation in the intestines. Today, serum and faecal calprotectin are used as laboratory markers, and the latter, being more accessible and not critical for collection material is widely known as a diagnostic marker of inflammatory bowel diseases. Some studies suggest that faecal calprotectin may be used to diagnose cow’s milk protein allergy in young infants when other diagnostic tests are not available. Data from various studies remain contradictory. Further studies to investigate the possibilities of using faecal calprotectin as a marker of the gastrointestinal food allergy in young children is needed.

Introduction. According to ophthalmologists, the number of patients with signs of asthenia and asthenopia has increased substantially over the past few years. It is a known fact that good nutrition, adequate intake of vitamins and minerals with diet, compliance with work-rest regime is a necessary condition for the prevention and elimination of clinical presentations of these pathologies.

Aim. To assess the results of the use of biologically active food additives Doppelherz^® Kinder Omega-3 for children from 7 years old and Doppelgerz^® Kinder multivitamins for children in paediatric population with signs of asthenia and asthenopia.

Materials and methods. A total of 90 children aged 7-12 years with signs of reactive asthenia and asthenopia were included in the study. Patients in group 1 received the biologically active food additive Doppelherz^® Kinder Omega-3 for children from 7 years old; patients in group 2 – Doppelgerz^® Kinder multivitamins for children; patients in group 3 – did not receive any biologically active food additives or vitamins.

Results. The CISS questionnaire showed a reduction in the severity of signs of asthenopia in group 1, less pronounced reduction of signs in group 2, and insignificant reduction in group 3. The Swanson scale showed a decrease in the degree of inattention in group 1, a decrease in the degree of inattention and impulsivity in group 2. I.K. Shats questionnaire showed that asthenia decreased from severe to moderate in group 1, and from severe to a fatigue reaction in group 2. In all groups that complied with the general recommendations, an increase in the relative accommodation reserve was observed. Evidence from pupillography indicates decreased accommodation tension, which was more pronounced in group 2 as compared with group 1, and insignificant in group 3. The Norn test and the tear meniscus height values confirmed an improvement in the tear film status, which was more pronounced in group 1 than in group 2.

Discussion. There are three main directions of the treatment strategy for asthenia: etiopathogenetic, nonspecific and symptomatic therapy. Targeted addition of vitamin and mineral complexes to a child’s diet is a reasonable and integral part of the pathogenetic treatment of asthenia and asthenopia.

Conclusion. A decrease in signs of asthenopia, improvement of the tear film state, reduction in signs of inattention due to increased concentration, and improvement of memory was observed in the group of patients who used the biologically active food additives with omega-3 for children from 7 years old, as compared to the control group. A decrease in signs of asthenia, and improvement of the tear film state was observed in the group of patients who used multivitamins for children — chew strips with raspberry flavour.

Introduction. Intestinal microbiota is a complex and unique system in its composition, performing a huge number of diverse functions in the body. Its formation begins in utero.

Aim. To study the features of the parietal and cavity intestinal microbiota in children born to mothers suffering from moderate bronchial asthma, depending on the mode of delivery.

Material and methods. A prospective longitudinal study was carried out, which included children from the first day of life to 12 months. A total of 68 children at high risk of atopy (HRA) from mothers with asthma were included, of whom 22 were born by cesarean section and 46 were born through the vaginal birth canal. Qualitative and quantitative analysis of GM was carried out by real-time PCR with group- and species-specific primers: in the examined children, the dynamics of 4 phylums including 31 microorganisms was assessed. The child’s feces and brush-biopsy were taken from the rectum at 7 control points of measurement.

Results and discussion. The data obtained showed that the method of delivery has an impact on the formation of the intestinal microbiota: in children born by cesarean section, in the parietal microbiota, starting from the age of 1–2 months, and in the cavity microbiota – 3–4 months, representatives of phylum Firmicutes (Clostridium leptum gr m.). In children born through the vaginal birth canal, all the first 8 months of life in the cavity and parietal microbiota were dominated by representatives of phylum Bacteroidetes (Bacteroides spp., Prevotella spp.). Clostridium leptum can be a marker of an inflammatory process in the intestinal mucosa in children at high risk of atopy. The research conducted over the past few years has found that Clostridium leptum promotes the secretion of anti-inflammatory (IL-10 and IL-12) and inhibition of the production of pro-inflammatory (IL-8) cytokines, and also boosts the number of Treg cells.

Conclusion. The mode of delivery in children with a high risk of atopy is an additional epigenetic factor that influences the nature of both the parietal and cerebrospinal microbiota.

Introduction. Acute respiratory infections cover majority of pediatric outpatient visits and hospitalizations, especially during the cold and flu epidemic season.

Aim. To evaluate the effect of Baс-Set Cold/Flu probiotic on the clinical manifestations and severity of acute respiratory infections (ARI) along with standard symptomatic therapy during periods of increased morbidity (November 2023 – March 2024).

Materials and methods. This study was an open-label, observational, multicenter study. The number of subjects was 234: 111 volunteers in the first group (experimental) and 123 volunteers in the second group (control) aged from 2 to 10 years inclusive (mean age 5.79) with a diagnosis of ARI. Patients of the first group took Bac-Set  Cold/Flu probiotic along with standard symptomatic therapy in comparison with control group which took a standard symptomatic therapy only. Statistical analysis was performed using the R programming language.

Results and discussion. The use of probiotic significantly reduced the number of complications and the prescription of antibiotic therapy by more than three times (5% in the probiotic group versus 17% in the control group). A significant difference was revealed in the total duration of the disease (the maximum period from the first symptom of fever to the fact of complete recovery, – absence of fever within last 48 hours) between groups (Mann- Whitney test, p-value = 0.017). In the experimental group, the average duration of illness was 6.2 ± 3.7 days, in the control group – 7.2 ± 3.1 days. A statistically significant difference in the duration of fever was also revealed (Mann-Whitney  test, p-value = 0.006). In the control group, on average, fever lasted 1 day longer. The median maximum temperature in the probiotic group was 0.5 degrees lower at the 2nd visit compared to the control group. The use of the probiotic did not affect the symptoms of cough, nasal congestion and the color of nasal discharge, but a trend was recorded for the effect on the symptoms of sore throat.

Conclusion. The use of a probiotic reduced the number of complications and the prescription of antibiotic therapy by more than three times, the duration of the disease and fever.

Duchenne muscular dystrophy is an orphan disease that belongs to the group of severe hereditary diseases affecting primarily skeletal muscles, characterized by a progressive disabling course. Duchenne muscular dystrophy is an X-linked recessive disease caused by mutations in the DMD gene, resulting in the absence or insufficient function of dystrophin. The disease has several stages with early and late clinical manifestations, as well as characteristic laboratory markers – increased activity of creatine phosphokinase, ALT and AST. The clinical case presented in the article demonstrates the late diagnosis of the early outpatient stage of Duchenne muscular dystrophy. The existing clinical symptoms: a slight delay in physical development in the first year of life, the appearance of tightness in the calf muscles from the age of 2.5 years, long-term observation by an orthopedist and correction of the existing equinovalgus deformity of the feet are classic clinical markers of the early outpatient stage of the disease. The addition of motor awkwardness in the dynamics, the use of the Gowers maneuver when standing up, changes in posture in the form of moderate kyphosis and pronounced lordosis, changes in gait with an expanded support base, periodic walking “on tiptoes” did not serve as a reason for further examination of the boy in terms of excluding muscular dystonia. Carrying out a clinical examination in a preschool institution allowed the neurologist to diagnose increased levels of CPK by 200 times and LDH by 100 times. Subsequently, during a molecular genetic examination at the Research Centre for Medical Genetics, a deletion of exons 46–52 of the DMD gene (NM_004006.2:c.(6614+1_6615-1)_(7660+1_7661-1)del) in a hemizygous state was discovered, which confirmed the diagnosis of Duchenne muscular dystrophy. Pathogenetic treatment has been started.

Introduction. Serologic tests are the main tool to screen for celiac disease. The serologic tests for tissue transglutaminase (tTG-IgA) antibodies combined with the assessment of the total serum IgA concentration is recommended as a primary test. Other serological markers, in particular IgA and/or IgG antibodies against deamidated gliadin peptides (DGP-IgG/IgA) and native gliadin (AGA-IgA/IgG), are not recommended for use in diagnosing celiac disease in immunocompetent patients.

Aim. To determine the diagnostic value of serological markers during the initial examination for celiac disease in children.

Materials and methods. The sensitivity, specificity, positive and negative predictive value of tTG-IgA/IgG, DPG-IgA/IgG, AGA-IgA/IgG were calculated for a group of 1247 children.

Results. Celiac disease was diagnosed in 101 patients of the study group. Tissue transglutaminase IgA antibodies demonstrated the highest sensitivity, specificity, positive and negative predictive values of 98.02, 99.48, 94.29 and 99.82%, respectively. DPG-IgA/IgG, AGA-IgA were inferior in sensitivity and specificity to tTG-IgA, the positive predictive value of DGP-IgG and AGA-IgA was 34.62 and 34.21%, respectively. AGA-IgG showed the worst specificity (40.3%) and positive predictive values (12.41%).

Discussion The low predictive value of the positive results of AGA IgA and DPG IgG determines the extremely high percentage of false-positive results for these tests. In none of the cases of an isolated elevation in AGA and DPG antibodies the diagnosis of celiac disease was confirmed, if tissue transglutaminase IgA antibody concentrations were normal.

Conclusion. The results of our study are fully consistent with the position of modern clinical guidelines, demonstrating the maximum diagnostic value of tissue transglutaminase IgA antibody during examination for celiac disease. Other antibodies are not recommended for use in the screening process to avoid unnecessary referrals of patients for endoscopic examination and to reduce healthcare costs.

Introduction. Increased protein and energy requirements, as well as intoxication symptoms in patients with malignant neoplasms (MN), spinal muscular atrophy (SMA) and cystic fibrosis (CF) determine the need to use specialized protein detoxification nutrition in their diet.

Aim. To evaluate the tolerability and clinical efficacy of the domestic specialized food product of dietary therapeutic and dietary preventive nutrition Protein detoxification cocktail NUTRIO (liquid mixture) in children with orphan diseases, over 2 years old. Materials and methods. We observed 18 children with rare diseases, aged from 2 years 11 months to 16 years. 6 children had a confirmed diagnosis of MN, 10 – SMA, 2 – CF. All children received the NUTRIO Detox Protein Cocktail twice a day: children aged 3 to 7 years old – ½ serving (100 ml), and aged 8 to 16 years – 1 serving (200 ml). The observation period was 1 month.

Results. It was found that all patients (100% of patients) highly rated the taste of the product. There were no refusals from the product. No allergic, dyspeptic or other side effects were noted during the observation period. All patients showed positive dynamics of nutritional status, an increase in the level of total protein and albumin in the blood, a decrease in markers of intoxication and inflammation, an improvement in the quality of life, increased endurance, physical activity and an improvement in the psycho-emotional background.

Discussion. The results we obtained correlate well with the authors’ data on the clinical efficacy, safety and significant economic benefits of using this diet.

Conclusions. The use of the domestic specialized food product of dietary therapeutic and dietary preventive nutrition Protein detoxification cocktail NUTRIO in diet therapy of children with malignant neoplasms, SMA and CF is effective for correcting their nutritional status and improving the quality of life.

Introduction. The improvement and prediction of functional status in patients with lymphatic malformations of the head and neck, particularly in neck masses, remains a pressing socio-economic concern. As of the publication of this article, no comprehensive scientific studies have explored the cause-and-effect relationships that impact the functional status of this specific patient demographic.

Aim. To elucidate the cause-and-effect relationships impacting the functional status of patients with cervical lymphatic malformations and to develop a predictive model for their functional decline.

Materials and methods. A retrospective cohort study was conducted, involving a detailed analysis of the functional status of 115 paediatric and adolescent patients aged 1 month to 17 years, treated for cervical lymphatic malformations at the Dmitry Rogachev National Medical Research Center for Pediatric Hematology, Oncology, and Immunology, from May 2012 to December 2022. The analysis utilised the Cologne Disease Score (CDS), varying according to the histological type of the lymphatic malformations (microcystic, macrocystic, or mixed) and the staging of the de Serres classification.

Results and discussion. The study involved 115 patients with a median age of 2.1 years (ranging from 0.4 to 5.5 years). It was determined that the ‘Mixed lymphatic malformations type,’ ‘Stage V of the de Serres classification,’ and ‘Need for surgical treatment’ were clinically and statistically significant prognostic factors for the deterioration of functional status in these patients, reducing the CDS scores by 0.976 to 4.514 points, respectively. These findings supported the development of a predictive model for worsening functional status in this group.

Conclusion. The predictive model formulated during this research accounts for the histological type, location, and treatment modality, and is recommended for clinical application within specialised medical institutions.

Food whims, some food refusals, food selectivity are issues for which parents often seek advice from a paediatrician. It is difficult to identify the reasons of the issues for just a few minutes of a visit. But the greatest challenges in the diagnosis arise when parents complain that they fail to feed both breastfed and non-breastfed young infants. Anorexia in young infants may be of a psychological and/or organic origin. The most complex and early forms often have a mixed etiology. A detailed history taking reveals cases of forced feeding, frequent changes of formula, and mother’s emotional instability. The article presents several clinical cases with different reasons for food refusals in children for which parents sought advice from a doctor. The first clinical case: a 11-year-old boy with complaints about lack of weight gain, as his weight did not exceed 6.8 kg, extremely slow growth rates. The child was diagnosed with moderate eosinophilic infiltration of the esophagus with eosinophils up to 10–12 per high-power field (hpf). Second clinical case: a 7-month-old girl M. with complaints about decreased appetite, food refusal, lack of interest in food and thirst, regurgitation during feeding. The examination showed eosinophilic infiltration of the duodenum up to 50 eosinophils per hpf. In the stomach there were up to 5 eosinophils per hpf. Third case: a boy Ya. with complaints about restlessness, more in the evening and at night, waking up frequently at night, regurgitation, hiccups, and periods of bloating. The article presents modern options for using specialized food products for this category of children according to the cause of anorexia.

The surface of the eyeball presents a unique microbiocynosis, which is composed of various bacteria including viruses. Proceeding in the body itself. Infectious conjunctivitis in children is one of the most frequent and widespread processes, which determines the relevance of this problem. Inflammatory diseases can be both independent diseases and a reflection of negative processes of the eye.

Aim. To evaluate the efficacy of picloxidine dihydrochloride in the composition 0.05% eye drops in the treatment of infectious conjunctivitis in children according to the results of conjunctival cavity microbiosis study by chromatography-mass spectrometry.

Materials and methods. The study included 61 patients (104 eyes) with infectious conjunctivitis aged from 1 to 9 years (5.02 ± 0.25). The state of conjunctival cavity microbiocenosis was studied in all patients before treatment by chromato-mass spectrometry. All patients were prescribed instillations of picloxidine dihydrochloride as a part of eye drops 0.05% into conjunctival cavity with the frequency of 4 times a day.

Results. A more severe course of conjunctivitis was recorded in children with less Bifidobacterium spp.

Discussion. Dysbiosis of conjunctival sac microbiome reflects the reserve potential of the immunity of the bulbar conjunctiva. Due to emergence of 4 types of pathogenic microflora, cytomegalovirus (20% of patients) and a decrease in all types of normal flora, a significant increase in opportunistic microorganisms (12 types), 2 to 59.5-fold increase on average is recorded, and every 3^rd patient is diagnosed with an increased titer of total endotoxin, which indicates the decreased reserve potential of the immunity of the bulbar conjunctiva.

Conclusion. Antiseptic with picloxidine dihydrochloride 0.05% in the eye drops Piktorid-SOLOpharm is a broad spectrum drug in the treatment of infectious conjunctivitis in children without significant side effects.

Introduction. In recent years there has been an increase in the number of cases of postoperative adhesive intestinal obstruction among children after surgical interventions due to congenital anomalies of the gastrointestinal tract, traumatic injuries and abdominal masses. The laparoscopic technology opportunities are directly related to the type of obstruction and the severity of the patients’ condition on admission. The rationale for laparoscopic technologies will help improve the outcomes of surgical treatment of children with various types of intestinal obstruction.

Aim. To evaluate the effectiveness of laparoscopic technologies in a group of children with intestinal obstruction.

Materials and methods. The study included 88 patients who underwent surgery for intestinal obstruction from 2008 to 2022 at the age of 1 month up to 18 years (7.75 ± 4.62). The patients were divided into two groups: Group I – obstructive intestinal obstruction (51) and Group II – strangulating intestinal obstruction (37). Methods for personalized evaluation of the options for endosurgical interventions were created based on the coefficients of the Bayesian decision rule characteristics.

Results and discussion. The method for personalized evaluation of the options for endosurgical intervention completion in the form of laparoscopic version was proposed on the basis of the analysis of clinical and anamnestic data, outcomes of instrumental approaches to preoperative diagnosis and laparoscopy in a group of patients with intestinal obstruction. Laparoscopic technology has proved to be effective in the groups of patients with obstructive and strangulation intestinal obstruction in 70 and 62.2% of follow-ups, respectively.

Conclusion. The effectiveness of laparoscopic technologies in children with intestinal obstruction depends on the patients’ group-specific decision rule characteristics of the method for personalized evaluation of the options for endosurgical interventions and accounts for 66% (Sn – 90%; Se – 85%; ACC – 85.8%). The method for personalized evaluation of the options for endosurgical interventions is a highly informative test for a group of patients with intestinal obstruction (AUC – 92.2%).

Acute respiratory diseases are more and more being registered among the population. According to Rospotrebnadzor, the incidence of acute respiratory infections in the Russian Federation in 2023 amounted to 15911.94 per 100.000 of population. Whooping cough plays a special role in the structure of acute respiratory infections. The largest increase is noted in the last months of 2023. In total, 20.497 cases of whooping cough were registered in the Russian Federation for 9 months of 2023. Whooping cough is an acute anthroponotic infection caused by whooping cough bacteria (Bordetella pertussis), accompanied by catarrhal phenomena in the upper respiratory tract and paroxysmal spasmodic cough. In young children, severe forms of whooping cough are still the most common, leading to a fatal outcome. One of the severe complications in children of the first years of life is encephalopathy, with persistent organic brain damage. In adolescents and adults, whooping cough often occurs in atypical forms and is manifested by a prolonged cough, for which they receive, as a rule, ineffective therapy from a pediatrician, therapist, allergist and otorhinolaryngologist. Whooping cough refers to diseases controlled by specific prevention. However, for children of an earlier age, infection from older children in the family or from adults is typical. This article presents a clinical observation of a severe course of whooping cough in a child aged 1 month, the disease proceeded with a paroxysmal cough, mainly nocturnal, with long reprises and redness of the face, ending with apnea of varying severity and duration. Determination of the severity of the course of pertussis infection is based on a complex of clinical and laboratory data. An early and effective method of etiological verification of whooping cough in children of the first year of life is PCR (polymerase chain reaction) of nasal/oropharyngeal smears.

Introduction. Endoscopic ear surgery is a promising treatment for petrous bone cholesteatoma in children. Endoscopic assistance provides better visualization and the ability to manipulate in hidden areas of the formed cavity and facilitate the removal of cholesteatoma matrix from hard-to-reach areas.

Aim. o systematize the accumulated knowledge about the clinical presentations of cholesteatoma of the temporal bone pyramid in children, review the modern methods of treatment and possible complications, and assess the necessity of applying endoscopic imaging devices to monitor hard-to-reach places in the temporal bone pyramid.

Materials and methods. The results of surgical treatment of petrous bone cholesteatoma (PBC) in the immediate and long-term postoperative periods in children were assessed. The study included 26 children aged 8 to 18 years (average age 13.1), 21 boys and 5 girls. Dynamic follow-up after surgery was at least two years. The function of facial muscles was assessed using the House – Brackmann scale before and after surgery after 6 months, and a pure-tone audiogram was performed before and after surgery after 6 months. The approaches, options for completing the operation, complications, and relapse rates are presented.

The discussion of the results. In the postoperative period, 2 people (7.7%) had normal hearing according to audiometry results, 1 (3.8%) had I degree of hearing loss, 5 (19.2%) had II degree of hearing loss, 6 (23.0%), III degree of hearing loss, in 7 (27.0%) IV degree of hearing loss, in 5 (19.2%) deafness. Function in the postoperative period was normal in 15 (57.0%) and from House – Brackmann grades II to VI in 11 (43.0%). Recurrence of cholesteatoma was detected in eight patients (30.0%) after 1–2 years.

Conclusion. In children, early diagnosis of cholesteatoma of the petrous bone is important to prevent the risk of complications. The use of endoscopic assistance when removing cholesteatoma of the petrous bone provides advantages: the ability to remove the cholesteatoma matrix in hidden areas, provides a larger viewing angle and control of these areas, thereby minimizing the risks of intraoperative complications, allowing for maximum sanitization of the pathological process.

This paper specifically examines MVP “Ailey Robot Improves Verbal Skills” as a tool for preschool aged children’s verbal skills development. Initial project aims to develop competent verbal skills as an aid tool for simple verbal correction in native (Russian) language and the associative object thinking improvement for children aged 3 years old, as well as is objected to increase the vocabulary of older children (up to seven aged ones). One of most significant activities for preschoolers is a role-playing game with a plot helping children to orient in social relations and estimate human activity’ values and motives through the acceptance of a certain role. With technological development, online platforms and applications are becoming the most accessible and increasingly popular method of teaching children from 3 to 7 years old. The content of thematic levels in the application covers various areas of daily life, which helps the child develop important situational and social skills, while the game format increases internal motivation to learn. The “Ailey Robot Improves Verbal Skills” service uses artificial intelligence models to create an exciting and effective learning environment for children and serves as an informative auxiliary tool for parents and speech specialists. In this paper we aimed to review this online application’s main features, analyze its performance, and identify its pros & cons.

Introduction. The relevance of the introduction of a system for evaluating medicinal prescriptions to control the safety of the use of antibiotics and concomitant therapy is due to the emergence of electronic medical records in multidisciplinary children’s hospitals.

Aim. To assess the potential risks of adverse reactions to antibiotics using an implemented retrospective cohort analysis of antimicrobial drug prescriptions in critically ill children.

Materials and methods. The analysis of the effect of antibiotic prescriptions on the potential risk of HP in the development of a critical condition in children was performed as part of a prospective observational study at the Morozov Children’s City Clinical Hospital from 02/01/2020 to 09/01/2021 in 100 children aged 0 to 17 years. To evaluate the prescribing lists, a twostage retrospective cohort analysis method was used to assess the potential risks of HP for antibiotics in the development of a critical condition in children with nosocomial infection. The patients were divided into 2 cohorts in 2 stages: at stage 1, an analysis was performed in 1 cohort “outcome HP+” of 30 children with HP on antibiotics and 2 cohort “outcome HP–” of 70 children without HP on antibiotics. At stage 2, an analysis was carried out in the same cohorts with an additional sampling criterion “availability of concomitant therapy” for 40 patients.

Results and discussion. The possibilities of taking into account additional potential risks of HP for antibiotics when making an appointment list in children with nosocomial infection with the development of a critical condition are shown. In the group of children receiving concomitant therapy, the highest relative risk of developing HP for antibiotics was revealed when using tigecycline (OR 7.24; 95% CI 0.64–8.21) and meropenem (OR 1.84; 95% CI 1.01–3.36).

Conclusion. The study shows the possibilities and prospects for the development of a risk-based approach when choosing combinations of antimicrobial drugs when making an appointment sheet based on an electronic medical record of an inpatient.

Recurrent acetonemic syndrome (AS) refers to pathological conditions predominantly in childhood with periodic ketotic crises with repeated (multiple) vomiting, which are caused by an increase in blood ketone bodies over 30 mg/l as a result of incomplete oxidation of fatty acids. and/or breakdown of ketogenic amino acids, as well as other metabolic disorders. The need to discuss the problem is due to the significant frequency of cases, including those requiring hospitalization of patients, the polyetiology of this condition, the complex and largely ambiguous mechanisms of development, the difficulties of differential diagnosis and the uncertainty of the prognosis. The article presents modern literature data on recurrent acetonemic syndrome with cyclic vomiting, drawing attention to the terminological aspects of the problem, discussing the most significant etiological factors and pathogenetic mechanisms of the development of crisis conditions, as well as diagnostic and therapeutic strategies. The article relates to discussion publications and is intended for pediatricians, general practitioners, pediatric endocrinologists, gastroenterologists, and emergency care specialists.

Niemann – Pick disease type A/B (NPAB) is a rare severe inherited disease from the group of accumulation diseases with a defect in the acid sphingomyelinase gene (Niemann-Pick disease types B, A/B) (ASMD). Symptoms of damage to the nervous system and internal organs manifest in infancy, leading to disability, fatalities in childhood. NPAB is so far incurable. Optimal management of the disease requires a multidisciplinary team of physicians, specialists. The basis of therapy is the elimination of existing/forming complications, symptomatic treatment. Enzyme replacement therapy as a means of modifying the course of this disease is expected to slow down the progression of pathologic manifestations of the disease not related to the central nervous system lesions. Single cases of hematopoietic stem cell transplantation (HSCT) have been described in the treatment of ASMD, which is one of the new methods aimed at normalizing the level of acid sphingomyelinase, blood parameters, as well as reducing the severity of pathological visceral manifestations. However, the development of complications during HSCT, absence of positive therapeutic effect in severe CNS lesions does not allow to widely implement this method. Taking into account the contradictory data on the efficacy of HSCT in ASMD, further clinical studies are required. Analysis of 2 clinical cases of NPAB in children from the same family allowed us to reveal differences in the course and outcomes of the disease at verification of the diagnosis at birth followed by HSCT. Difficulties in diagnosing this extremely rare pathology, which requires a multidisciplinary approach, justify the need to improve methods of early diagnosis, including the organization of genetic risk determination, introduction of prenatal genetic testing before pregnancy.

Diagnostic and treatment facilities in pediatric oncology have reached a very technological advances in a short period of time, contributing to increased relapse-free and overall survival rates. Obviously, the key factors are the improvement of diagnostic and screening programs, anticancer and concomitant therapy, including personification of clinical guidelines for monitoring and correction of early and late complications in time. Issues regarding the quality of life of patients, including social, psychological rehabilitation and aspects of reproductive potential, are becoming highly actual. The critical importance is that an informed multidisciplinary team of specialists at all stages of anticancer treatment should be involved. This article presents the main algorithms and the most significant issues for pediatricians in the diagnosis and supervision of patients with an oncological diagnosis, using the example of medulloblastoma, as the most common malignant tumor of the central nervous system in children. The proposed recommendations are based on a retrospective analysis of pediatric patients with relapsed and refractory forms of medulloblastoma (n = 270) who received antitumor therapy in the period from 07/01/1993 to 07/01/2023, as well as international clinical data.

The review article is devoted to the problem of the variability of the intestinal microbiota in children with various diseases of the gastrointestinal tract (GIT) of non-infectious origin, which is one of the current areas of modern pediatric gastroenterology. More often, in practical pediatrics, during the treatment of non-infectious diseases of the gastrointestinal tract, doctors often do not prescribe medications for the intestinal microbiota. In connection with this, complications subsequently develop in functional activity in this system, which is most often classified as a separate, unrelated pathology. The scientific articles by Russian and foreign authors devoted to the study of intestinal microbiota variability in children with non-infectious diseases of various origins were analyzed. According to the results of the study, it was noted that in non-infectious inflammatory processes, as a result of endogenous and exogenous factors, there is a decrease in the quantity and quality of microorganisms, mainly producing SCFA, which affects the course of the underlying disease. At the same time, the question of rational prescription of biotechnological and gene therapeutic drugs, together with traditional drugs containing live bacteria and substances that provide the necessary conditions for the growth and development of normal intestinal microbiota (pro-, pre-, syn- and symbiotics), also remains open. Analysis of scientific and literary data has shown that in children with non-infectious gastrointestinal diseases of various origins, there is a decrease in the quantitative composition of the intestinal microbiota due to microorganisms producing SCFA, such as Clostridium spp. and Faecalibacterium prausnitzii, against the background of a decrease in probiotic bacteria. These changes lead to disproportions in the qualitative composition of the intestinal microbiota. Therefore, these patients should be treated with biologics containing Lactobacillus rhamnosus, Lactobacillus gasseri, Lactococcus lactis, Leuconostoc mesenteroides, and Bifidobacteria.

Interested in the problem of cytopenic conditions in children is associated with a various causes of them. The spectrum of differential diagnostic search is very wide: hemoblastoses, myelodysplastic and paraneoplastic syndromes, aplastic anemia. It is necessary for assessment of the main cause of cytopenia to pay attention on the patient’ common status (his complaints, anamnestic data — features of the start of the disease, previous diseases, concomitant somatic pathology, constant medication). Also on the complex and features of the clinical manifestations, the functional state of organs and systems, and in particular the liver, hematological changes in peripheral blood and bone the brain. The combination of immune thrombocytopenic purpura with autoimmune hemolytic anemia are named Fisher — Evans syndrome (FES). The article presents a clinical case of the combination Fisher — Evans syndrome and preceding B-lymphoma in a preschool child, and also reflects the main etiopathogenetic mechanisms of development this pathology. Fisher — Evans syndrome (SFE) is a rare autoimmune disease characterized by the immune thrombocytopenia (ITP) and Coombs–positive autoimmune hemolytic anemia (AIGA), which can develop simultaneously or sequentially, and in some cases are combined with immune neutropenia. The primary and secondary variants of Fisher — Evans syndrome are described. The secondary is the background of the following pathology: Epstein — Barr viral, cytomegalovirus, immunodeficiency virus, hepatitis C, parvovirus, varicella zoster virus, mycoplasma infection, tuberculosis), immunodeficiency and lymphoproliferative conditions, rheumatoid, autoimmune and malignant diseases such as systemic lupus erythematosus (SLE), antiphospholipid syndrome (AFLS), Sjogren’s syndrome, immunoglobulin A deficiency (IgA), autoimmune lymphoproliferative syndrome (ALPS), general variable immune deficiency (OVIN), lymphogranulomatosis (LGM), chronic lymphoblastic leukemia (CLL), autoimmune thyroiditis, autoimmune hepatitis, Takayasu arteritis, systemic sclerosis. The difficulties of searching causes are and indicated this diagnosis of exclusion. The question remains: is B-lymphoma the cause or accomplice of SFE in a child?

In ENT clinical practice, there are cases of a disease similar to those ones of chronic adenotonsillitis, but insensitive to the designated conservative therapy. This disease was first described in 1987 and is called PFAPA syndrome. Its symptoms periodicity and its regression when corticosteroids are prescribed were established. Adenotomy/adenotonsillectomy can eliminate the symptoms of the disease or significantly reduce the frequency of relapses. The histological features of the structure of the palatine and pharyngeal tonsils, as well as the characteristics of the PFAPA-syndrome, were revealed. Clinical Case Description: patient K., 3 years old, repeated cases of fever were recorded in the child from the age of 1 year 3 months. They were initially regarded as manifestations of acute respiratory viral infections, complicated by adenoiditis, pharyngitis, aphthous stomatitis and cervical lymphadenitis, with a frequency of 1.5–2 months, 6–7 episodes per year. The antiviral therapy was prescribed without any effect. A prednisolone test (1.5 mg/kg) was positive; it led to a rapid normalization of body temperature and condition improvement. The adenotonsillectomy was done. Surgical intervention and the postoperative period were uneventful. The follow-up period was without any recurrence of the disease for more than 1 year. Patient F., 6 years old, had, the recurrent (each 1.5–2 months), acute respiratory viral infections, adenotonsillitis, accompanied by lymphadenitis and febrile fever. A prednisolone test was positive. The patient was diagnosed with PFAPA syndrome by the age of 6. The adenotonsillectomy was done. No disease relapses were noted in the follow-up.

Progressive familial intrahepatic cholestasis is a group of infrequent hereditary diseases caused by the defect in the transport of bile acids, leading to the rapid development of the liver’s cirrhosis. The infrequent occurrence of this pathology creates significant difficulties in the diagnostic search for the causes of intrahepatic cholestasis. The article demonstrates a clinical case of progressive familial intrahepatic cholestasis in a newborn child. We used the descriptive method of clinical-anamnestic and laboratory-instrumental data, obtained from the medical history of a full-term newborn who was treated in the perinatal center of the Regional Clinical Hospital (Omsk). Starting from the first days of life, signs of liver failure with a progressive increase drew: indirect hyperbilirubinemia followed by predominance of direct bilirubin, hemorrhagic syndrome with signs of disturbance of both external and internal hemostasis pathways, and hypoproteinemia. At the second stage of nursing, monitoring was carried out laboratory and instrumental studies and using results we excluded the surgical and infectious nature of the pathology: hepatitis B and C, cytomegalovirus infection. In a diagnostic searching we were looking for the most common genetically determined liver diseases. Using massive parallel sequencing for mutations in 47 genes which have the hereditary nature of liver damage, we excluded tyrosinemia, galactosemia, Gaucher’s disease, and alpha-1-antitrypsin deficiency. According to the results of a liver biopsy performed at the clinic of the federal center of the St Petersburg State Pediatric Medical University detected progressive familial intrahepatic cholestasis (Byler’s disease). This case belongs to the category of orphan diseases with a complex diagnostic search, has a familial nature of inheritance (patient’s brother dead due to certain hereditary liver’s disease) and requires molecular genetic confirmation.