Kazan State Medical Academy

Objective: to analyze the possibilities and study the informative value of computed tomography (CT) and magnetic resonance imaging (MRI) in visualizing the clinical manifestations of neurofibromatosis. Material and methods. Based on retrospective analysis of the medical records, a group of types 1 and 2 neurofibromatose patients observed at the Children’s Republican Clinical Hospital was formed. In total, 35 children with a confirmed or clinically suspected disease of phacomatoses group were examined. The patients met the following inclusion criteria: age from 0 to 18 years inclusive, СT and MRI multi-system manifestations, characteristic visual changes. Results. Brain and spinal cord study revealed focal areas of signal intensity (77.14%), optic nerve gliomas with a spread to chiasm (25,00%), peripheral neurofibromas (34.29%). Chest lesions included plexiform neurofibromas paravasally in the mediastinum and paravertebrally (5.71%), peripheral neurofibromas (2.86%). Examination of the abdominal cavity and retroperitoneal space showed peripheral neurofibromas (2.86%), and multi-node volumetric masses (2.86%). In the lower limbs, the following lesions were found: plexiform neurofibromas with limb soft tissue lesions (5.71%), fibrous dysplasia (5.71%). MRI showed a higher sensitivity in assessing central nervous system damage due to the high tissue contrast, which makes it possible to assess in detail the size, localization and growth pattern of the nervous tissue tumors, as well as their relation to the surrounding nervous structures. CT is preferable for evaluating visceral structures of the abdominal cavity and retroperitoneal space, detecting bone and thoracic lesions mediated by neurofibromatosis. Conclusion. A multi-modal approach integrating the use of CT and MRI provides a comprehensive visual representation of neurofibromatosis manifestations.

Background. There is a complex interaction of endogenous and exogenous factors in rosacea pathogenesis. Existing methods of analysis often do not consider the multifactorial nature of dermatosis, which complicates effective management of patients. Objective. To identify the main relationships between the development factors and rosacea severity building a Bayesian network model original for this dermatosis, as well as to evaluate the results of topical therapy with metronidazole. Material and methods. The study was performed from 2023 to 2025 in Kazan. It involved 117 patients with papulopustular rosacea, median age 35.0 [24; 45] years. The studied parameters included: unchangeable (general characteristics, anamnestic data) and changeable (laboratory indicators, morphofunctional parameters and microbiota content of the facial skin) factors. Obtained parameters — clinical characteristics and dermatological indices. At the first stage of the study, a reconstruction of non-parametric Bayesian network was carried out using a modified algorithm that ensures reproducibility of the model due to clear threshold criteria (Spearman’s rank correlation coefficient equal 0.7 and above) and cycle elimination stages. At the second stage, a group of patients (n=52) with papulopustular subtype of rosacea of mild and moderate course was formed from the total sample, and these subjects underwent 12-week topical therapy with 1% metronidazole gel. Results. With the use of a new network approach for rosacea, key factors influencing the course of dermatosis were identified, 4 clusters of features describing the influence of hormonal, biochemical, immunohematologic and microbial pathogenesis factors were formed. Topical therapy of patients (n=52) with 1% metronidazole gel was effective in 86.5% (n=45) cases. Adverse local reactions were characterized by a mild degree of severity and did not require withdrawal of the drug. Conclusion. The proposed multicomponent Bayesian rosacea model with methodological reproducibility is useful for understanding the relationships between pathogenesis factors and clinical manifestations of dermatosis. Topical therapy of papulopustular rosacea with 1% metronidazole gel has shown high effectiveness, safety and favorable tolerability.

Antimicrobial resistance (AMR) is one of the most serious threats to global public health in the 21st century. According to the World Health Organization, in 2019, the bacterial AMR caused 4.95 million deaths around the world, creating unprecedented calls for healthcare systems. The economic burden of the problem continues to grow rapidly — it is predicted that by 2050 additional expenses associated with the AMR can reach 1 trillion US dollars. Of particular concern is the fact that in outpatient practice up to 30 % of antibiotic prescriptions are unreasonable, and under stationary conditions, the situation is aggravated by the use of empirical therapy with excessively wide spectrum drugs. The analysis of the work of a multidisciplinary hospital during the six -month period revealed the high efficiency of the interdisciplinary model of interaction between a clinical pharmacologist and a bacteriological laboratory. The clinical pharmacologist demonstrated its key role in ensuring rational antimicrobial therapy through a systematic audit of appointments, the implementation of the principles of de-escalation and the provision of expert consultations. His work made it possible not only to optimize treatment regimen, but also to reduce the level of unreasonable anxiety of medical personnel, contributing to the refusal of the practice of prescribing antibiotics from anxiety. The bacteriological laboratory confirmed its essential role as a source of objective diagnostic and epidemiological data. In addition to the routine conduct of bacteriological crops and determining sensitivity to antimicrobial drugs, the laboratory organized a system of constant resistance of resistance, analyzing data on the allocation and locals of the excretion of pathogens. The interactions of experts were manifested in the creation of a closed antimicrobial therapy management cycle. The introduction of the operational information system made it possible to reduce the time between the release of the pathogen and the correction of therapy from 7 to 2 days. The clinical pharmacologist got the opportunity to request additional studies of sensitivity to the antibiotics of the reserve for complex cases, while the laboratory began to inform in advance of the release of polyuretic strains in advance. A significant achievement was the use of local resistance data to optimize both empirical therapy and the dosage form of the hospital. The analysis of the accumulated data made it possible to rationalize the purchases of antibiotics, focusing on pathogens prevailing in the hospital and their resistance profiles. The results confirmed that interdisciplinary interaction is an effective tool for combating the AMR, which provides both improving the quality of medical care and saving resources, and should be considered as a mandatory component of the modern healthcare system.

This paper presents the results of the implementation of the drug circulation system, in particular, drug provision, in the medical information system. The role of a clinical pharmacologist in the formation of algorithms for using the basic principles of the drug circulation system at various levels (procurement department, pharmacy, clinical departments to provide medicines to a medical organization) is shown.

Aim: to present clinical guidelines describing the clinical, laboratory, endoscopic and histological characteristics of autoimmune gastritis (AIG) and to provide information support for decision-making on diagnosis, prognosis assessment and choice of management tactics for patients with AIG. Key points. AIG is an organ-specific autoimmune disease characterized by the presence of antibodies to parietal cells and/or intrinsic factor, in which inflammation and atrophy are limited to the gastric mucosa, functionally manifesting as hypo- and achlorhydria. There is evidence of an increase in the incidence of AIG worldwide. AIG is often associated with autoimmune thyroiditis (up to 40 % of cases), type 1 diabetes mellitus, and autoimmune polyglandular syndrome. Data on laboratory diagnostic methods, esophagogastroduodenoscopy, and histological examination of biopsies are presented, with an analysis of the endoscopic and histological picture typical of AIG. When AIG is combined with H. pylori infection, eradication therapy is recommended. In addition to symptomatic treatment and treatment of anemia, long-term rebamipide therapy is recommended to potentiate the protective properties of the gastric mucosa. Endoscopic monitoring of patients with AIG is recommended at intervals of once every 3 years.

Extracellular vesicles produced by mesenchymal stem cells (MSC-EVs), including exosomes, microvesicles, and apoptotic bodies, are key mediators of intercellular communication and have attracted increasing attention in recent years as potential therapeutic agents for neurological disorders. Predominantly preclinical investigations, including in vitro and animal model studies, demonstrate that MSC-EVs can enhance axonal growth, promote regeneration of nerve fibers and remyelination, and modulate inflammatory processes in injured nervous tissue. These effects have been observed across multiple neurological conditions, including spinal cord injury, traumatic brain injury, ischemic stroke, Alzheimer’s disease, Parkinson’s disease, and multiple sclerosis, though primarily in experimental settings. Owing to their ability to carry biologically active molecules and to cross the blood–brain barrier, MSC-EVs have shown potential as vehicles for targeted delivery of therapeutic molecules to the central nervous system. However, the overwhelming majority of evidence remains preclinical, and clinical translation is limited by the scarcity of completed, rigorously controlled human trials. To advance toward clinical application, further research is required to standardize methods for vesicle isolation, characterization, and delivery, establish optimal dosing regimens, and develop robust quality control standards. A more comprehensive understanding of MSC-EVs signaling mechanisms may facilitate the future development of therapeutic strategies, contingent upon rigorous validation in well-designed clinical studies.

Noonan-like syndrome with anagen hair loss is a RAS pathology and is characterized by craniofacial features resembling Noonan syndrome, a spectrum of cardiovascular abnormalities, cognitive deficits and behavioral distorders, short stature usually associated with growth hormone deficiency, a unique combination ectodermal disorders. It results from a missense variant (c.4A>G, c.Ser2Gly) in the SHOC2 gene, located on chromosome 10q25, which encodes a protein that facilitates signaling through the RAS mitogen-associated protein kinase pathway, triggering a number of cellular processes. The prevalence of the syndrome is less than 1 in 1,000,000 (worldwide). Evidence has been obtained that disturbances in the level of expression and activation of the Ras family signaling pathway and its downstream kinases, such as Raf/MEK/ERK1-2, contribute to the pathogenetic mechanisms of the development of autoimmune diseases. Description of a clinical case. We present a previously undescribed clinical case of the development of juvenile arthritis associated with Crohn’s disease in a 13-year-old girl with Noonan-like syndrome with hair loss in the anagen phase. At birth, the patient was diagnosed with a congenital heart defect (multiple atrial septal defect), corrected at 4 years, articular syndrome debuted at 8 years 4 months, intestinal syndrome at 10 years 4 months. Genetic analysis was carried out only at 10 years 7 months, DNA sequencing results were obtained and a pathogenic variant of the nucleotide sequence of the SHOC2 gene was identified in a heterozygous state (c.4A>G, p.Ser2Gly). As a result of the analysis of clinical data, laboratory and instrumental studies, a diagnosis was made: “Juvenile arthritis (pauciarticular variant) associated with Crohn’s disease (ulcerative bauginitis, ulcerative ileitis, catarrhal jeunitis, aphthous left-sided colitis, erosive gastritis). Noonan-like syndrome with hair loss in the anagen stage 1. Hypopituitarism. Partial growth hormone deficiency. Condition after ASD repair.” The girl received treatment: a course of glucocorticosteroids (methylprednisolone) with gradual withdrawal, methotrexate, adalimumab and mesalazine with positive dynamics. Conclusion. Pediatricians need to remember the possibility of developing autoimmune diseases in children with NPS, the need for their early diagnosis, timely referral to specialists and adequate therapy.

Technological advances in the field of medicine, which received an additional impetus to development during the COVID-19 pandemic, and subsequent organizational changes, increased requirements for high efficiency and quality of work of medical organizations, often continue to have a significant impact on the well-being of medical workers even after the pandemic in the absence of sound management. The work environment is a key factor determining the well-being and health of medical workers. Increasing the competence of managers in determining strategies for preventive solutions to the problem of an unfavorable working environment is the main condition for reducing the spread of professional burnout syndrome and personnel shortages in medicine. A well-structured work environment, organizational and communicative cultures of medical organizations and improved working conditions of medical workers are significant determinants of the effectiveness and quality of healthcare.

Introduction. Growing antibacterial resistance of urinary tract infection pathogens in children and the risk of relapses necessitate finding the safest ways to improve treatment outcomes. Aim. To evaluate the effectiveness of taking Canephron ® N in combination with antibacterial therapy in children aged 1 to 3 years with acute uncomplicated pyelonephritis to increase the effectiveness of therapy with a reduced risk of parenchyma damage and faster recovery of renal function. Materials and methods. A prospective, open, randomized study was conducted in 2 groups of sick children: Group 1 (50 children) received antibacterial therapy and Canephron ® N for 10–14 days, Group 2 (50 children) – only antibacterial therapy. The duration of observation of patients was 60 days, the period of active treatment was 10–14 days, follow-up was 1.5 months. Clinical and laboratory data, partial renal functions, enzymuria (ALP, LDH, GGT), metabolic disorders were assessed. Results and discussion. The average duration of clinical manifestations and urinary syndrome in Group 1 was 2–3 days less than in patients in Group 2. A decrease in the inflammatory process was accompanied by normalization of the functional state of the kidneys by day 15 in 85% of patients in Group 1, which was significantly better (p < 0.05) than in Group 2 (49%). In patients of Group 1, in contrast to patients of Group 2, the excretion of oxalates and calcium in urine also decreased (p < 0.05). Particular attention should be paid to the faster normalization of the level of fermenturia in patients of Group 1. Conclusions. Combination therapy with the inclusion of a herbal preparation contributed to a faster elimination of clinical manifestations of the disease, a decrease the activity of the inflammatory process and the severity of urinary syndrome, reduced fermenturia, oxalate-calcium crystalluria and improved the processes of tubular reabsorption.

Introduction. Today, complex drugs are used as effective medications to relieve broncho-obstructive syndrome. Due to their composition, they can effectively influence mucociliary clearance, relieve bronchospasms, and improve sputum rheology. Aim. To conduct a comparative evaluation of the efficacy and safety of a complex mucolytic drug and other cough treatment options in children with broncho-obstructive syndrome. Materials and methods. A total of 167 children aged 3 to 15 years with obstructive bronchitis (mean age 7.9 ± 1.4 years) were observed. Children aged 4 to 8 years (91 children [54.5%]) represented the largest portion of the sample. The patients were divided into 2 groups: Group 1 (93 children) received Jocet® as their sole cough drug; Group 2, the control group (74 children), received a combination of mono-preparations: a bronchodilator combined with an expectorant and/or mucolytic drug. Results. The study investigated the safety profile of a complex mucolytic drug (Jocet®). The analysis of clinical observations showed no reported adverse events in 97% of patients, which evidenced high tolerability of the drug. On Days 6–7 of treatment, the severity of clinical symptoms in Group 1 was significantly lower than in Group 2. Group 1 also demonstrated faster improvement in auscultation findings and transformation of cough into productive wet cough; Group 2 showed a similar effect on average 0.9 days later. Conclusions. The use of Joсet® allowed to effectively and safely achieve the intended effect in children with broncho-obstructive syndrome as compared to children, who received a combination of drugs (a bronchodilator and an expectorant). Based on the study results, we would advise to include this drug in pediatric practice as an effective pathogenetic agent to treat obstructive bronchitis.