This short communications compares the phenotypic properties of Candida species isolated from gut of children with reactive arthritis, such as secretory inhibitor of lysozyme and biofilm formation. A total of 65 clinical strains of Candida species isolated from the feces of healthy children and children with ReA were tested. SIL production of Candida by inhibiting lysozyme activity against Micrococcus luteus ATCC 15307 were studied (mg/mL*OD). Quantitative assessment of biofilm formation was performed using crystal violet binding assay method (CU). A significantly higher proportion of ReA strains were SIL-positive (77.8% vs. 40.0%) and BF-positive (64.4% vs. 30.0%) compared with non-ReA strains (P < 0.01). These results suggest that the functional significance of SIL production and biofilm formation must be estimated, of investigating the roles of intestinal of Candida in the pathogenesis of spondylarthritis, including reactive arthritis. It is imperative to delineate microbial factors that contribute to ReA development.

Chronic lung infection is the leading cause of death in patients with cystic fibrosis. The article highlights the data of a microbiological examination of children and adolescents with cystic fibrosis living in the Moscow region and the results of determining the antibiotic susceptibility of the main isolated pathogens. The purpose of the study was to study the spectrum of the microflora of the respiratory tract of children and adolescents with cystic fibrosis with the determination of the antibiotic sensitivity of the isolated strains of S. aureus and P. aeruginosa. Material and methods. A study was made of the discharge of the upper and lower respiratory tract in 127 (66.6%) pediatric patients with cystic fibrosis under the age of 18 years using the bacteriological method of research. Results and discussion. In children and adolescents with cystic fibrosis, S. аureus was isolated from the biological material of the upper and lower respiratory tract in 70.1% of cases, in 46.5% in monoculture and in 24% in association with other microorganisms. P. aeruginosa isolated in 23.6%, in monoculture 8.0%. In most cases, P. aeruginosa was associated with S. аureus. MRSA has been identified in 6.4% of children and adolescents with cystic fibrosis. The studied strains of S. aureus were 33% sensitive to benzylpenicillin, 25% to chloramphenicol and were resistant (75%) to ciprofloxacin. 56% of S. aureus strains remained sensitive to erythromycin. P. aeruginosa strains were resistant to ticarcillin/clavulanate), fluoroquinolones, third-generation cephalosporins, imipenem, and aztreonam. Conclusion. P. aeruginosa retains sensitivity to meropenem and aminoglycosides, and S. aureus to tigecycline, teicoplanin, vancomycin, linezolid, fosfomycin, and fusidic acid. Monitoring the antibiotic susceptibility of microorganisms isolated from patients with cystic fibrosis allows you to optimize the tactics of antimicrobial therapy, taking into account the revision of the criteria of the European Committee for the determination of sensitivity to antimicrobial drugs (EUCAST).

3D-technologies are a promising direction in the development of medicine. Numerous publications in recent years demonstrate the potential use of 3D-modeling in disease diagnosis, surgical planning, intraoperative navigation, development of individual implants, and specialist training. In this article, we present the experience of implementing 3D-technologies in the practice of a pediatric surgeon using three clinical cases where the use of 3D models based on computed tomography data allowed for more accurate diagnosis and surgical planning. The first patient was a 3-year-old boy with vascular compression of the left main bronchus and impaired ventilation of the left lung. After 3D-visualization of the area of interest, an arterial ligament causing the compression was visualized as the main cause. The second patient was a 6-month-old boy with confirmed bilateral chondromesenchymal hamartoma of the chest wall. The volume and staging of the surgical treatment, as well as the most suitable option for defect replacement, were developed after creating a polymer 3D-model of the child’s chest. The third patient was a 17-year-old girl with persistent hypercalcemia after removal of a parathyroid adenoma. A repeat computed tomography revealed an ectopic parathyroid tissue. The use of 3D-models and AR-technology for intraoperative navigation allowed for precise determination of the location of the pathological tissue. These clinical cases demonstrate the potential and effectiveness of using 3D-modeling in pediatric surgery and confirm that this is a promising direction in the development of medicine, opening up new possibilities for clinicians.

Extraintestinal manifestations occur in every second child with Crohn’s disease, can affect any organ system, and determine the type of therapy and prognosis of the disease. The skin is the most frequent “target organ” of all extraintestinal manifestations, the spectrum of skin lesions is extremely clinically and pathogenically heterogeneous. Metastatic Crohn’s disease (MBC) is a rare extraintestinal manifestation of the disease, approaches to the diagnosis and management of which are not standardized in either adults or children. The most frequent description of MBC in the literature is skin damage, although interstitial lung damage, bone and muscle damage, and ENT organs, could be destructive. MCD lesions vary in morphology and can arise anywhere on the skin, rarely undergo reverse development, require special approaches to therapy. The primary diagnosis of such elements is extremely difficult, often delated, biopsy is required for a definitive diagnosis - the detection of epithelioid granuloma and its elements outside the gastrointestinal tract. Therapeutic approaches to MCD are not standardized now, they are carried out according to the protocols of CD management, and the effect of the use of oral steroids, a-TNFa drugs is shown. A case of metastatic Crohn’s disease in a 16-year-old teenager with an unusual localization - the nasal septum is described. The destructive nature of the lesion with the formation of perforation of the nasal septum determined the difficult path of differential diagnosis with Wegener’s granulomatosis. Morphological examination of the biopsy of the nasal mucosa revealed epithelioid cells of sarcoid-like granuloma. Systemic glucocorticosteroids use (GCS) led to epithelialization of the nasal septum defect and a decrease in the activity of CD in the intestine mucosa.

Аnnotation. A long-term persistent infection of the lower respiratory tract with cystic fibrosis and, as a consequence, the need for regular use of antibacterial drugs inevitably leads to changes in the composition of the intestinal microflora. At the same time, the most dangerous are toxigenic strains of Clostridium difficile. In turn, excessive growth of C. Difficile leads to increased sensitivity of enterocytes to its toxins. The leading pathogenicity factors of C. Difficile are exotoxins A (TcdA), B (TcdB) and binary toxin. TcdA and TcdB are enterotoxins acting on intestinal enterocytes, which leads to inflammation and necrosis of the mucous membrane. The binary toxin forms a complex on the enterocyte membrane that penetrates the cytoplasm, disrupts the functioning of the cell, leads to its death, and also enhances the adhesion and colonization of C. Difficile. One of the most accessible methods for determining the presence of toxin-producing C. Difficile in feces is immunochromatographic examination. The frequency of such a complication in patients with MV is unknown, the literature presents isolated cases of such a complication in Russia. There is one known case of fulminant pseudomembranous colitis (lethal). A clinical case of fulminant pseudomembranous colitis with recovery is presented. Conclusion. Patients with cystic fibrosis are at risk for the development of this complication, especially patients with a history of intestinal resection against the background of meconium ileus. In about 5% of cases, there is a fulminant course of pseudomembarnous colitis. In this form, the duration of the disease can be several hours. Lethality in the lightning-fast course reaches 58-70%. This option presents the greatest difficulty in terms of diagnosis due to atypical clinical symptoms. In particular, the diarrheal syndrome may not be pronounced or absent at all. Such patients often show symptoms of an acute abdomen, which makes differential diagnosis difficult. This is confirmed in our clinical case. It is necessary to increase awareness and alertness of doctors regarding the risk of developing this formidable complication in patients with Сystic fibrosis

The article presents current data on the issue of primary ciliary dysfunction. Primary ciliary dysfunction and its variant, Kartagener`s syndrome, is a rare pathology. Despite the presence of chronic respiratory symptoms in patients from the neonatal period, pediatricians’ awareness of this disease remains low. The article presents the own clinical observation of a patient with Kartagener`s syndrome.

The paper presents literature data and our own results of surgical treatment of children with a rare form of Meckel’s diverticulum in combination with congenital diaphragmatic hernia and intestinal malrotation. Of the 47 children observed in the clinical bases of the Department of Hospital Surgery of the Tashkent Medical Pediatric Institute in 2010-2022, 1 child was diagnosed with this anomaly.

The new coronavirus infection COVID-19, as a rule, in children and teenagers has a lighter course compared to adults. However, in a number of comorbid patients, this disease can be severe and even end fatally. This concomitant pathology includes obesity, which became even more common in the population, including in childhood, during the COVID-19 pandemic and has a number of common pathogenetic mechanisms of inflammation with this infection. The article considers such a clinical case in a 17-year-old teenager with an unfavorable outcome, the purpose of demonstrating which was to determine the risks of a severe course of a new coronavirus infection.

Goal. Currently, there is an increasing interest in the treatment and improvement of the quality of life of patients with rare chronic diseases, and one of the urgent issues remains the problem of pain syndrome assessment and prevention. The purpose of the review article was to study the problem of pain and its assessment in children with epidermolysis bullosa. Theoretical justification. In the article, the problem of pain is considered as a complex biopsychosocial phenomenon, according to the results of a theoretical review, the main provisions of the concept of the multifactorial nature of pain are presented, which must be taken into account when selecting tools for assessing pain when working with gene dermatoses (epidermolysis bullosa) in childhood. Based on the analysis of Russian and foreign studies, a number of the most problematic areas in the assessment of pain in children are identified. Results and their discussion. According to the results of the analysis of a number of studies, the description of the main manifestations of epidermolysis bullosa and possible causes of pain is given. The biopsychosocial multifactorial nature of pain is considered, its neurobiological, psychological and functional consequences are presented, and on the basis of the research presented, conclusions are drawn for the first time about the principles of selecting the most effective tools for assessing pain and its impact on the vital activity of children with a chronic incurable disease. Pain assessment should include children’s previous experiences and expectations, coping strategies and pain relief resources, and communication features with caring adults. All forms of epidermolysis bullosa are characterized by pain and discomfort, and their elimination plays a key role in terms of the patient’s well-being and quality of life.

Genodermatoses are a group of hereditary heterogeneous diseases, the distinctive feature of which is the predominant damage to the skin and its appendages. Genodermatoses are distinguished by their variability, a wide range of complications and concomitant pathology from other organs and systems of the body. For timely diagnosis and prescription of symptomatic therapy, patients with genodermatoses require observation by a multidisciplinary team of specialists. Purpose of the study: present the rationale for an integrated approach to diagnosis, treatment and preventive monitoring for genodermatoses using the example of congenital epidermolysis bullosa and ichthyosis. Methods: An analysis of extracutaneous manifestations and complications of such genodermatoses as congenital epidermolysis bullosa and ichthyosis was carried out from foreign and domestic scientific sources. The spectrum of complications and manifestations of internal organs in genodermatoses is described, the topic of symptomatic treatment and management of patients with congenital epidermolysis bullosa and ichthyosis in the neonatal period is highlighted. Recommendations for the symptomatic treatment of skin manifestations and complications of the gastrointestinal tract and musculoskeletal system are described. Results: Based on the analyzed literary sources and our own clinical experience, it can be argued that with genodermatoses, not only the skin, but also other organs and systems of the body are involved in the pathological process. Timely diagnosis of the disease is extremely important for patients with these diseases, since symptomatic treatment of genodermatoses must begin as early as possible. Congenital epidermolysis bullosa and ichthyosis are serious diseases from the group of genetic skin diseases, characterized by great variability in clinical manifestations and a wide range of extracutaneous complications. Conclusions: Preventive observation and treatment of patients with genodermatoses is a complex problem and its solution requires the involvement of a multidisciplinary team of specialists, which should include a dermatologist, pediatrician/therapist, geneticist, gastroenterologist, surgeon, ophthalmologist, otorhinolaryngologist, oncologist, dentist, orthopedist, rehabilitation specialist, psychologist, palliative care physician and qualified nurse.

Celiac disease is a chronic disease, with predominant damage to the small intestine and provoked by gluten consumption in genetically predisposed individuals. Previous large-scale studies suggest a possible role of the gut microbiota in the pathogenesis of this disease. Changes in the qualitative and quantitative characteristics of microbiota of different genesis can lead to impaired regulation of immune response, which, in turn, may contribute to the development of gluten intolerance. In this context, it is relevant to analyse the data on changes in the intestinal microbiota in celiac disease and the possibility of its correction with probiotics as one of the strategies for the treatment of children with celiac disease, suffering from celiac disease.

Justification. In recent years, great progress in the treatment of cystic fibrosis has been associated with the use of pathogenetic therapy (since 2012 - in world practice, since 2020 in the Russian Federation). Against the background of the use of which the nutritional status, the functional state of the lungs improves, but there is no complete restoration of the impaired functions of the organs, in particular, the external secretory function of the pancreas is restored only in young children with the timely appointment of targeted therapy The purpose of the study: to increase the effectiveness of correction of enzyme replacement therapy by an individualized approach. Methods. 140 children (boys - 81, girls - 59) with MV aged from 1 year to 18 years (average age 12.9±4.8 years) were examined. The study was conducted in 2022 in the Department of Cystic Fibrosis Scientific and «Research Clinical Institute of Childhood of the Ministry of Health of the Moscow Region» (clinical base of the scientific and clinical Department of Cystic fibrosis Research Center for Medical Genetic). Inclusion criteria - diagnosis of cystic fibrosis, chronic pancreatic insufficiency, severe. Exclusion criteria - short bowel syndrome, exacerbation of the disease. The doses of pancreatin per day were evaluated using the calculation method: units/g of fat in food and intestinal syndrome before and after correction using an algorithm of an individualized approach to calculating enzyme therapy. The observation period is 3 months Design: single-center, prospective, comparative Results. The study showed that the majority of 82 patients (59%) in the general group received less than 2000 U/g of fat, which is less than the reference values (2000-4000 U/g). The median daily dose was 1500 U / g of fat, which can be attributed to the peculiarities of dosing and the need for pancreatin in the Russian pediatric population of patients with cystic fibrosis. The results are consistent with the data of recent studies indicating an acceptable dosage range of pancreatin 1000-4000 units / g of fat in food. In the course of the study, an algorithm for individualized correction of enzyme replacement therapy was developed and the effectiveness of this approach was shown. Conclusion. When calculating pancreatin for the fat content in food, the dose was below the recommended values of 2000-4000 units / g and amounted to 1500 units / g, which indicated that it did not meet the needs of children in enzymes and required individual correction. This served as the basis for the development of an algorithm for individualized correction of enzyme replacement therapy. The effectiveness of this approach using computer technology (inpatient program for doctors and mobile version for patients), in the form of reduction / relief of intestinal syndrome, is shown. To increase compliance and to train parents to control diet and enzyme replacement therapy, it is advisable to use computer programs and mobile applications to calculate the diet and dosage of pancreatin for each meal.

The article covers in detail the history of the creation and construction of the Morozov Children’s City Clinical Hospital of the Moscow Department of Health. The development of medical care for children is described, from the organization of outpatient care and infectious diseases departments in the first half of the 20th century to highly specialized medical care in the 21st century. The main results of clinical, scientific and educational activities, achievements of scientific pediatric schools, and the contribution of researchers working at the Morozov Hospital to various fields of pediatrics and pediatric surgery are presented.

The organization of large congresses with international participation dedicated to the problems of children’s healthcare is an opportunity for doctors of the Russian Federation and other countries to share knowledge and experience on saving a child’s life and treating diseases from an early age of the patient through direct communication. A systematic approach can be considered the most effective for solving problems in all areas, including children’s healthcare, and the congress of pediatricians helps doctors go beyond the boundaries of the usual and the familiar, learn new things, and get ideas for further professional growth. While creating masterpieces of the world’s cultural heritage, great artists and composers multiplied their talent, honing their skills through training and hard work. Pediatrics is a healing art that requires the physician to regularly improve professional knowledge and competencies. Art that gives the world the most significant and valuable masterpieces - healthy children. This determined the name of the next congress of Children’s Doctors of the Moscow Region - “Pediatrics as an Art”.