Background. Cholestatic liver diseases represent a pressing issue in modern pediatric gastroenterology. They significantly impact the quality of life of both children and their families, often lead to disability and high mortality rates, and are a primary indication for liver transplantation. In pediatric practice, various liver diseases can manifest with cholestasis, a pathological process characterized by impaired bile synthesis or flow, leading to the accumulation of bile components (bile acids, bilirubin, cholesterol) in the body and progressive damage to liver tissue. This pathological condition can be caused by anatomical obstructions, developmental anomalies of the biliary tract structures, infections of various etiologies, metabolic diseases, or genetically determined defects in enzymatic and transport systems.

Objective. The purpose of this study is to provide a clinical example of a rare genetic disorder, progressive familial intrahepatic cholestasis type 3 (PFIC-3). These defects disrupt the processes of bile acid synthesis and excretion and cause damage to hepatocytes and epithelial cells of the bile ducts. One rare genetic pathology is Progressive Familial Intrahepatic Cholestasis type 3. This is an autosomal recessive disease caused by defects in the ABCB4 gene, which encodes the MDR3 transport protein responsible for phospholipid secretion into bile. The disease manifests in early childhood and is characterized by progressive cholestasis, intense pruritus, failure to thrive, and a high risk of developing liver cirrhosis. Diagnosing Progressive Familial Intrahepatic Cholestasis type 3 presents significant challenges due to the absence of pathognomonic clinical signs and requires the use of molecular genetic methods for verification.

Conclusion. The article presents a clinical case of Progressive Familial Intrahepatic Cholestasis type 3 in a girl, diagnosed at the age of 1 year and 6 months, with a confirmed homozygous mutation c.2534G>T in the ABCB4 gene. For the first time in Russian practice, the experience of using odevixibat – a selective inhibitor of the intestinal bile acid transporter (IBAT) – in this category of patients is described. 

Background. Acid sphingomyelinase deficiency (Niemann – Pick disease) leads to the intracellular accumulation of sphingomyelin within the mononuclear phagocyte system, including liver, spleen, lungs and bone marrow. Liver involvement represents one of the earliest and persistent visceral manifestations of the disease. Foamy macrophages featuring prominent cytoplasmic vacuolation are generated within the liver. Intracellular accumulation of pathological substrates in hepatocytes results in several pathological changes, including inflammation and cytokine release, remodeling of the sinusoidal network, periportal and bridging fibrosis and progressive portal hypertension. Dyslipidemia, associated with Niemann – Pick disease, is characterised by elevated levels of various lipid fractions. Gradual lipid accumulation leads to the liver enlargement (hepatomegaly) and the development of hepatic steatosis. Hepatomegaly, biochemical abnormalities and progressive fibrosis significantly impair the patients' quality of life and adversely affect the prognosis of the disease.

Objective. To analyze current data on the pathogenesis, clinical manifestations, morphological features, and treatment of liver damage in Niemann – Pick disease types A, A/B, and B.

Materials and methods. A systematic review of publications from 2013-2025 on the clinical presentation, histopathology, and treatment of acid sphingomyelinase deficiency was conducted, including the impact of enzyme replacement therapy on liver pathology.

Results. The liver is a key target organ in Niemann – Pick disease types A, A/B, and B. The spectrum of liver changes includes hepatomegaly, parenchymal infiltration with foamy macrophages, progressive fibrosis, cirrhosis, hepatic steatosis, and the possible development of portal hypertension and liver failure requiring transplantation. The severity of liver damage and the age at onset of changes depend on the disease phenotype. Liver failure, in addition to respiratory disease, is the leading cause of death in chronic forms of Niemann – Pick disease. Modern enzyme replacement therapy can reduce liver volume, improve biochemical parameters, and slow the progression of fibrosis.

Conclusion. Early diagnosis and pathogenetic therapy are key to slowing the progression of liver disease in acid sphingomyelinase deficiency.

Background. Lysosomal acid lipase deficiency (LALD) is a chronic, progressive disease based on a defect in the LIPA gene encoding lysosomal acid lipase, leading to impaired lipid metabolism. Currently, enzyme replacement therapy (ERT) has been developed.

Objective. To evaluate the dynamics of clinical and laboratory symptoms of LALD in children on the background of ERT with sebelipase alpha.

Materials and methods. A retrospective analysis of the medical records of 5 children with LALD (boys – 2, girls – 3) who received sebelipase alpha therapy according to the instructions was carried out. The dynamics of anthropometric parameters were assessed (height, weight, body mass index (BMI), SDS BMI, biochemical blood test with assessment of transaminase levels (ALT, AST), lipidogram, ultrasound examination of abdominal organs, liver elastometry.

Results. 3 (60%) children had signs of malnutrition and a decrease in SDS BMI of less than -1 before starting therapy. In 2 (40%) children, after 1 year of ERT, this indicator returned to normal, in 1 child after 2 years. Before the start of ERT, all children had an increase in ALT levels: in 3 (60%) children – up to 2 norms, and in 1 child – from 2 to 3 norms and more than 4 norms, AST: in 4 (80%) children – less than 2 norms, in 1 child – more than 3 norms. After 1 year from the start of ERT, all children (100%) showed a decrease in the level of transaminases, after 3 years normalization of these indicators was registered in 4 (80%) children, in 1 child transaminases remained elevated during 4 years of follow-up, although they had lower values than before the start of ERT. After 1 year from the start of therapy, all children showed a reduction in liver size. In 1 child, organ size returned to normal after 4 years of therapy, and in 4 (80%) children, hepatomegaly persisted throughout the follow-up, although it became significantly less than before the start of ERT. The METAVIR fibrosis score before the start of FGT in 2 (40%) children was F1, in 1 child – F2, and in 1 child – F3. After 2 years, it decreased to F0 in all children. The steatosis index in 3 (60%) children is S3, and in 2 children it is S2. Signs of steatosis in 1 child persisted for 8 years of therapy at S3 level, in 1 child for 4 years at S2 level, in 2 (40%) children after 2 years the index of steatosis decreased from S2 to S1, in 1 child – from S3 to S0. There were no signs of liver damage progression on the background of ERT in any child. The size of the spleen was normal in 3 (60%) children 3 years after the start of therapy. No increase in lipid levels above baseline values was detected in any child on the background of ERT: total cholesterol and LDL exceeded the standard values more often, while the level of TG normalized after 3 years of PHT in all children. HDL in 1 child remained below normal even after 4 years of therapy.

Conclusion. A comparative analysis of the clinical, laboratory and instrumental parameters of 5 children with LALD who received sebelipase alpha for 3 years (2 children), 4 years (2 children) and 8 years (1 child) allows us to conclude that there are positive dynamics against the background of therapy in all patients. This is manifested by normalization of body weight-growth indicators, reduction in the size of the liver and spleen, decrease in cytolysis markers, improvement or stabilization of structural changes in the liver parenchyma. There is no progression of dyslipidemia, although the levels of cholesterol and LDL remain elevated in most measurements, but below the initial values, which is explained by a complex mechanism of regulation of lipid metabolism.

Background. Neonatal hyperammonemia is a condition caused by an increased content of ammonia in the blood of a newborn, both as a result of certain hereditary metabolic diseases and occurring against the background of severe pathology of the perinatal period. Neonatal hyperammonemia is defined as serum ammonia concentration exceeding 100 μmol/L for full-term infants and exceeding 150 μmol/L for preterm infants. A level exceeding 360 μmol/L (600 micrograms per deciliter (mcg/dL)) is considered a critical threshold, which is associated with a high risk of irreversible changes in the brain, liver, kidneys and other organ systems, and requires immediate initiation of extracorporeal detoxification methods. Upon entering the central nervous system, ammonia triggers a cascade of pathological reactions. The pivotal event in this case will be the overproduction of glutamine in astroglia. The relevance of neonatal hyperammonemia is underscored not only by its life-threatening nature and by the complexity of timely diagnosis due to the non-specific clinical presentation, but also by a significant risk of multiple organ failure and fatal outcome in the case of the delayed correction of hyperammonemia. Regardless of the cause, neonatal hyperammonemia can become life-threatening and lead to irreversible damage to cells of the central nervous system, liver and other organs.

Results. This review analyzes the current understanding of the etiopathogenesis of neonatal hyperammonemia, including the mechanisms of neuro- and cytotoxicity, the classification of etiological factors, and clinical semiotics. Special attention is paid to the differential diagnostic algorithm, which makes it possible to distinguish between primary defects of the urea cycle, organic acidemia and transient conditions. Intensive care protocols, including pharmacological methods of ammonia binding, indications for extracorporeal detoxification, and a long-term patient management strategy are considered in detail. It is emphasized that the outcome of the disease is directly determined by the timeliness of diagnosis and the immediate initiation of pathogenetic therapy.

Objective. To conduct a comparative analysis of the incidence of acute kidney injury in infants with rotavirus, norovirus, and COVID-19 associated with gastrointestinal syndrome and to determine its association with clinical predictors and noninvasive biomarkers.

Materials and methods. A total of 148 children aged 1 month to 1 year, hospitalized at the Z. A. Bashlyaeva Children's City Clinical Hospital with a diagnosis of acute infectious gastroenteritis of various etiologies, were examined. Patients were divided into three groups: Group 1: patients with rotavirus infection (RVs, n = 40), Group 2: patients with norovirus infection (NoVs, n = 25), and Group 3: patients with the gastrointestinal form of COVID-19 (n = 83). All patients underwent standard laboratory testing, including urine ELISA testing for NGAL, KIM-1, L-FABP, and IL-1. Clinical symptoms, hospitalization duration, degree of exsiccosis, and clinical predictors of acute kidney injury were assessed.

Results. The risk of developing acute kidney injury in patients with RVs and NoVs was 60% and 52%, respectively, compared to 32.5% for COVID-19 (p < 0.05). Vomiting was the leading symptom of COVID-19. RVs is associated with the most severe diarrhea and significant acid-base balance changes, with a later hospitalization time proving to be a critical factor (p < 0.01). The median NGAL was 40.1 ng/ ml in patients with AKI versus 5.8 ng/ml in patients without AKI (p < 0.001), for KIM-1 it was 0.404 ng/ml versus 0.049 ng/ml, and for L-FABP it was 3.928 ng/ml versus 0.098 ng/ml. IL-1 showed no significant differences (p = 0.250). Elevated NGAL and KIM-1 levels were associated with more severe disease, the degree of exsiccosis, and acidosis. L-FABP and IL-18 also increased in AKI but were less sensitive than NGAL. KIM-1 was a marker of tubular damage in acidosis (pH < 7.30). These results highlight the importance of NGAL and KIM-1 in assessing the risk of developing and the severity of renal injury.

Conclusion. The etiology of viral diarrhea determines the pathogenetic variant of AKI and requires a differentiated approach to its diagnosis. NGAL is a universal early marker of AKI risk, while KIM-1 verifies tubular damage during acidosis later in the disease. The data obtained can be used for personalized patient management depending on the etiology of diarrhea.

Background. Currently, in Russia, intravenous enzyme replacement therapy is used for the treatment of mucopolysaccharidosis type II. In recent months, enzyme replacement therapy administered via the cerebral ventricles (intracerebroventricular administration) has become available. The main drawback of this therapy is its inability to cross the blood-brain barrier. This means that available medications can target either the somatic manifestations of the disease or the neurological ones. For effective therapy aimed at alleviating all symptoms of the disease, it is necessary to administer two enzyme replacement therapies: intravenously, to address somatic symptoms, and intracerebroventricularly, to alleviate neurological manifestations of the disease.

Objective. This article is dedicated to new approaches in the treatment of mucopolysaccharidosis type II with a next-generation drug capable of crossing the blood-brain barrier. Two types of receptors are utilized for the passage of recombinant idursulfase through the blood-brain barrier: transferrin receptors and insulin receptors. Two drugs have been registered worldwide, each targeting a specific type of receptor. Materials and Methods. This article analyzes literary sources describing the mechanism by which drug molecules overcome the blood-brain barrier via transferrin receptors, as well as evaluates the impact of pabinafusp alpha on the neurological and somatic status of patients with mucopolysaccharidosis type II.

Conclusions. Pabinafusp alpha represents an innovative approach to the treatment of Hunter syndrome, providing the ability to cross the blood-brain barrier. This opens new horizons for therapy, allowing a single drug to comprehensively address both somatic and neurological manifestations of the disease. Studies show that pabinafusp alpha can improve neurological symptoms and the quality of life for patients and their families. Thus, this drug demonstrates promising results and may become the treatment of choice for patients with mucopolysaccharidosis type II.

Background. Palliative care for children with palliative status is aimed at maximizing the quality of life for chronic and severe diseases that cannot be treated radically. In this area, not only the symptoms of the disease are taken into account, but also the individual needs of each child and his family. The growing number of children with life-limiting conditions requires the introduction of new models of care that go beyond standard protocols and include comprehensive, personalized management.

The aim of the work was to study and describe the elements of personalized care that optimize clinical and functional indicators and improve the standard of living of children with palliative status. To achieve this goal, the following tasks were solved: studying the theoretical foundations of palliative care and legislative norms; analyzing the features of therapeutic nutrition, taking into account individual characteristics; identifying the importance of interdisciplinary cooperation between specialists; integrating psychological and spiritual support; assessing the impact of adapting measures to different conditions of care; the study of the role of social support through public organizations; as well as the development of approaches to an objective assessment of the effectiveness of personalized events. The paper will examine in detail the theoretical foundations of palliative care for children, the specifics of personalization of therapeutic nutrition, mechanisms of interdisciplinary interaction, methods of integrating psychological and spiritual support, as well as the practice of adapting measures to different conditions of medical care.

Results. Special attention is paid to social support and evaluation of the effectiveness of integrated approaches aimed at improving clinical and functional indicators and the quality of life of children with palliative status. In general, the conducted research shows that a personalized approach to palliative care for children requires the combined efforts of medical professionals, psychologists, social services and public organizations to create an integrated, adaptive and patient-oriented package of measures. Only with this approach is it possible to provide high-efficiency support, reduce the level of suffering and achieve the most comfortable quality of life for children with palliative status and their families.

Background. Traditionally, supportive therapy is used to reduce severe reactions and complications during chemotherapy for acute leukemias. One of the frequent complications is the development of toxic hepatitis, which requires dose reduction or chemotherapy discontinuation until biochemical parameters improve.

Objective. To evaluate the effectiveness of prophylactic administration of a drug with hepatoprotective properties during drug therapy for acute lymphoblastic leukemias in children.

Materials and methods. A retrospective, single-center, selective, controlled observation of 76 children with acute B-lineage lymphoblastic leukemia was conducted, and the children were divided into two groups based on whether or not they had received a hepatoprotective agent for preventive purposes. A unified international scale was used to assess the degree of hepatotoxicity in accordance with the RUSSCO clinical guidelines, and the type of damage was calculated based on the known ratio of ALT and alkaline phosphatase (R criterion).

Results. The predominant type of liver damage against the background of polychemotherapy of acute leukemia turned out to be hepatocellular (R > 5) – in the first 86.2% and the second group – 95.7%. In all cases, against the background of drug therapy for leukemia, an increase in liver enzymes (ALT, AST) of various degrees was recorded during the induction and consolidation of remission. In children of the second group (receiving ursodeoxycholic acid simultaneously with the start of treatment for acute leukemia), mild degrees of hepatotoxicity were registered predominantly, whereas in children without such, the third degree was registered in 51.7% of cases, and the first degree – in only 3.4% of cases.

Conclusion. The predominant type of liver damage in drug therapy for acute lymphoblastic leukemia in children is hepatocellular. The use of a hepatoprotective agent in combination with drug therapy for acute lymphoblastic leukemia reduces the incidence of severe hepatotoxicity. 

Background. Fatty liver disease is a term that combines pathological conditions, the common feature of which is the accumulation of lipids in the liver. Fatty liver disease includes several groups of diseases, of which metabolically associated fatty liver disease is the most common in the pediatric population. The term "fatty liver" was first introduced in 1836 by the English scientist Thomas Addison to describe the hepatic histology in a patient with a history of alcohol use disorder. Ten years later, Austrian pathologist Carl von Rokitansky described the histological features of fatty liver disease in children, which was associated with excessive food intake. For over a century, scientists and researchers studied the etiology and pathophysiology of fatty liver disease, as well as its link with fibrosis and cirrhosis. In 1980, the American pathologist Jurgen Ludwig and his colleagues coined the term "non-alcoholic steatohepatitis", while his compatriot, physician and pathologist Fenton Schaffner, introduced the term "non-alcoholic fatty liver disease" in 1986. New knowledge has been accumulated about the multifactorial genesis of metabolically associated fatty liver disease, the role of epigenetic and genetic factors in the development and progression of the disease, which has led to the need to revise terminology and approaches to diagnosis. In 2023, the term nonalcoholic fatty liver disease was proposed instead of non-alcoholic fatty liver disease, as well as updated diagnostic criteria. Currently, there are no approved clinical guidelines for metabolically associated fatty liver disease in children in the Russian Federation. In 2025, a draft of clinical guidelines for the diagnosis and treatment of metabolically associated fatty liver disease in children (non-alcoholic fatty liver disease) was published, which specifies the main criteria for the diagnosis of metabolically associated fatty liver disease in childhood.

Conclusion. This review presents modern ideas about the classification of pediatric metabolically associated fatty liver disease, pays attention to a comparative analysis of diagnostic approaches reflected in foreign and domestic recommendation documents, as well as issues of prevention and treatment of metabolically associated fatty liver disease in children.

Background. Measles is a pressing problem in modern medicine. At present, there is an epidemic problem with measles. The main reason for the current situation is considered to be a decrease in the coverage of the population with planned immunization. It is known that the acute period of measles is accompanied by severe catarrhal syndrome, intoxication and exanthema. In addition, the disease often occurs with various complications. Complications associated with damage to the central nervous system are usually considered separately, in particular: measles meningitis, encephalitis, acute disseminated encephalomyelitis (ADEM), subacute sclerosing panencephalitis (SSPE). Lesions of the nervous system in measles can occur in the acute period (meningitis, encephalitis), which indicates a direct connection with the action of the measles virus and the inflammatory reactions associated with it. Delayed damage to the nervous system (ADEM, PSPE) is also possible, pathogenetically based on immune-pathological reactions and largely realized in the presence of a genetic predisposition in a particular individual. In the second case, the measles virus can be rationally considered as a significant trigger for the manifestation of these neurological manifestations. The presence of damage to the central nervous system in measles, both in the acute period and in delayed implementations, is an alarming moment that requires special detailed monitoring of the patient.

Results. We present a clinical observation of the development and successful treatment of measles meningitis in a 12-year-old patient. The patient was not vaccinated against measles, due to the refusal of legal representatives for personal reasons. The course of measles in the patient was classical – pronounced intoxication syndrome, Stimson's catarrhal triad were noted. On the third day of the febrile period, the patient noted the appearance of a typical exanthema of a maculopapular nature with stages from top to bottom. Manifestations of typical enanthem in the form of Belsky – Filatov – Koplik spots were also recorded. The patient was hospitalized on the 6th day from the onset of the disease. Verification of the diagnosis was carried out by detecting early serological markers (IgM) to the measles virus. By the 9th day, against the background of symptomatic treatment, clinical stabilization of the condition was noted – persistent normalization of body temperature, regression of catarrhal syndrome. On the 11th day, negative dynamics were noted, manifested by the resumption of fever, the development of cephalgia, meningeal syndrome. After neuroimaging, a lumbar puncture was performed. Cytosis – 165 cells, lymphocytic (78% – lymphocytes; 22% – neutrophils). The course of measles meningitis is documented. For anti-inflammatory purposes, the patient was prescribed systemic glucocorticosteroids (in a standard dosage of 2 mg/kg of prednisolone) for a short course. Against the background of the complex pathogenetic therapy, by the 13th day it was possible to achieve stable normalization of body temperature, regression of neurological symptoms. On the 23rd day, the patient was punctured again, the cerebrospinal fluid was sanitized (cytosis – 10 cells). On the 24th day from the onset of the disease, the patient was discharged in a satisfactory condition for further observation by the local pediatrician.

Conclusion. This clinical case demonstrates the relevance of the measles problem at the present stage. Practicing doctors are required to be alert to the possibility of the implementation of neurological complications of measles.

Background. Despite advances in recent years, treatment outcomes for pancreatic cancer remain modest. The search for ways to overcome the immune resistance of pancreatic cancer and the need to optimize treatment strategies have drawn attention to the potential combined use of chemotherapy and the cytokine gene therapy drug tumor necrosis factor-thymosin-α1 recombinant.

Оbjective. To evaluate the efficacy and safety of therapy with tumor necrosis factor-thymosin-α1 recombinant in a patient with pancreatic cancer undergoing chemotherapy.

Material and methods. Patient I., 52, presented to the clinic in August 2025 for stage III cT4N1M0 pancreatic cancer. A percutaneous biopsy of the pancreatic tumor was performed (August 5, 2025). The diagnosis was confirmed histologically, revealing ductal adenocarcinoma, G3. According to the immunohistochemistry study of August 15, 2025: BRCA1 wt, BRCA2 wt, PALB2 wt. The oncology board prescribed courses of chemotherapy with the FOLFIRINOX regimen. To reduce the likelihood and severity of undesirable side effects associated with chemotherapy, the patient sought simultaneous cytokine gene therapy. The patient's condition was monitored using the Karnofsky status, CEA, CA19-9, and tumor necrosis factor-alpha levels. Computed tomography with intravenous contrast was used to assess the tumor response to treatment according to RECIST 1.1 criteria.

Results. The patient was prescribed cytokine gene therapy with tumor necrosis factor-thymosin-α1 recombinant (rTNF-α1) according to the regimen developed at the clinic. During combination therapy, the patient noted a gradual improvement in his overall well-being, a decrease in pain severity, and was able to not only perform feasible housework but also partially return to his professional activities. Treatment toxicity associated with the use of chemotherapy did not exceed grades 1-2 according to CTCAE 5.0 (nausea and grade 1-2 leukopenia). No additional side effects associated with the use of rTNF-α1 were noted. When assessing the tumor process according to RECIST 1.1 criteria, after 5 courses of cytokine gene therapy and 9 courses of chemotherapy, a partial response was achieved, a decrease in target lesions by 36,9% from the initial level was noted. The patient currently continues to receive combination therapy with rTNF-α1 and chemotherapy. The patient's survival from diagnosis is 7+ months.

Conclusion. Further clinical studies are needed to determine the role of combination therapy with tumor necrosis factor-thymosin-α1 recombinant and chemotherapy in patients with pancreatic cancer.

Background. Osteoporosis is a systemic metabolic skeletal disorder, most common in older and geriatric patients, leading to enhanced bone fragility and a consequent increase in fracture risk. In Russia osteoporosis is estimated to affect at least 16 million people, and, by 2050, the annual incidence of osteoporotic proximal femur fractures is projected to exceed 200,000 cases. Pharmacological treatment of osteoporosis aims to enhance bone strength and reduce fracture risk.

Results. This review highlights denosumab, a fully human monoclonal antibody to RANKL, as a promising therapeutic option for osteoporosis. The efficacy of denosumab in the treatment of osteoporosis has been demonstrated in several Phase II and III clinical trials. The administration of the drug was associated with an early, significant, and sustained increase in bone mineral density across all skeletal sites. Denosumab is an antiresorptive agent; by binding to RANKL, thereby it inhibits osteoclast activation and subsequent bone resorption, resulting in an increase in bone mass and strength in both cortical and trabecular bone. In contrast to bisphosphonates, where bone mineral density reaches a plateau after 4-5 years despite sustained anti-fracture efficacy, denosumab therapy demonstrated a progressive and sustained increase in bone mineral density over 10 years of continuous use. In addition to postmenopausal osteoporosis, the efficacy of denosumab has been demonstrated in the treatment of male osteoporosis. Denosumab also demonstrated efficacy in both aromatase inhibitor-induced and glucocorticoid-induced osteoporosis. Long-term denosumab therapy is associated with a reduced incidence of diabetes mellitus. The effects of denosumab on bone tissue and bone turnover are fully reversible. Since denosumab does not interact directly with bone tissue and, in contrast to bisphosphonates, is not incorporated into the bone matrix, its therapeutic effect is maintained only during its presence in the systemic circulation. Long-term denosumab treatment may be associated with a range of adverse effects, such as drug-induced osteonecrosis of the jaw and atypical femoral fracture. With 10 years of denosumab therapy, a favorable skeletal benefit-risk profile was maintained, with one atypical femoral fracture and one case of drug-induced osteonecrosis of the jaw occurring per 281 and 40 prevented osteoporotic fractures, respectively. Denosumab is an effective and relatively safe medication for the long-term treatment of various forms of osteoporosis, requiring strict adherence to the dosing schedule and a well-defined treatment cessation strategy. The first denosumab biosimilar was launched on the Russian market in April 2025. This product has completed all stages of evaluation, including in vitro physicochemical and biological characterization, preclinical studies of biosimilarity and safety compared to the reference product, and clinical trials.

Objective. To summarize the available data on the impact of iron deficiency on innate and adaptive immunity, focusing on the molecular mechanisms and clinical implications for patients with inborn errors of immunity. This article is a comprehensive review dedicated to describing the co-regulatory significance of iron in the formation of immune mechanisms, highlighting the role of iron deficiency as a factor in the dysregulation of various arms of the immune response.

Materials and methods. The review presents an analysis of the role of iron homeostasis in regulating the immune system. A literature search (1975-2025) was conducted in the PubMed/MEDLINE, Scopus, Web of Science, Google Scholar, and eLibrary databases. Publication selection was based on thematic relevance, belonging to peer-reviewed sources, and full-text availability. The goal of this synthesis is to provide a comprehensive picture of current knowledge.

Results. This literature review demonstrates the critical role of iron in immune system function. Iron deficiency, a global health problem, comprehensively disrupts the immune response. The impact of iron deficiency on humoral and cytokine profiles remains a subject of debate. On the part of innate immunity, IRP-dependent impairment of neutrophil differentiation and a reduced pool of dendritic cell precursors in the bone marrow are observed. This leads to defects in adaptive immunity: impaired activation of CD4+ T cells and suppression of clonal expansion of CD8+ T lymphocytes due to blockade of the tricarboxylic acid cycle and aspartate deficiency. The discovered mechanisms indicate that iron deficiency can serve as a predictor of severe infections and an ineffective vaccine response in patients with inborn errors of immunity, underscoring the need to monitor iron metabolism parameters in their management.

Conclusion. Iron deficiency in immunocompromised patients creates a vicious cycle, worsening existing immunodeficiency and increasing the risk of infections. Monitoring iron metabolism parameters and its timely correction should be considered an important component of the diagnostic algorithm in the comprehensive management of such patients.

Background. In recent years, glucagon-like peptide-1 receptor agonists (GLP-1a) have emerged as pivotal therapeutic agents in the management of metabolic disorders, particularly type 2 diabetes mellitus and obesity. This is due to their ability to improve glycemic control and facilitate weight loss by enhancing insulin secretion, suppressing glucagon release and delaying gastric emptying by promoting prolonged satiety and reducing appetite. However, despite their clinical efficacy, therapy with GLP-1RAs has been associated with an increased risk of gallbladder disease, especially with prolonged use and higher doses.

Objective. The aim of this study is to evaluate current clinical data regarding the incidence and severity of adverse effects involving gallbladder and biliary tract in patients receiving GLP-1α therapy, to evaluate potential prevention strategies and to assess the efficacy of ursodeoxycholic acid (UDCA) in this patient population. The objective of this review is to examine the potential mechanisms through which GLP-1 receptor agonists may contribute to the development of biliary tract diseases. Recent systematic reviews, meta-analyses and Randomized Controlled Trials (RCTs) indicate an increased risk of gallbladder and biliary tract diseases with GLP-1 receptor agonist therapy. The risk is highest with high-dose and long-term therapy, as well as in patients treated for weight loss and type 2 diabetes mellitus (T2DM). The increased risk of gallbladder diseases associated with GLP-1 receptor agonists therapy has important clinical implications necessitating careful monitoring of the gallbladder and biliary tract before initiation, as well as of patients receiving GLP-1 receptor agonist therapy. UDCA positively affects choleresis, reduces the inflammation and improves metabolic parameters; therefore, it is recommended for the prevention and treatment of adverse events associated with GLP-1 receptor agonist therapy.

Background. Currently, targeted therapy is the most promising method of treating malignant neoplasms. It is mainly used as a secondline therapy, along with classical methods of chemotherapy and radiation therapy. Among the areas of targeted cancer therapy, the most intensively developing areas are monoclonal antibodies and low-molecular-weight biologically active substances, tyrosine kinase blockers, folic acid receptors and serine/threonine kinase blockers. There is another area of targeted therapy, which is based on the selective activation of the cellular component of the immune system, primarily macrophages. Macrophages and T-lymphocytes are the main, genetically determined link for the elimination of tumor cells using natural pathophysiological mechanisms. The purpose of this work is to evaluate the oncoprotective effect of low–molecular-weight chitosan on an in vivo model of a transferable malignant tumor and to investigate the effectiveness of the therapeutic effect of chitosan in leukopenia induced by cytostatics and gamma radiation.

Materials and methods. Cytostatic leukopenia: 15 outbred nonlinear laboratory ICR (CD-1) mice (males) With an average body weight of 20-22 g, they were divided into 3 groups of 5 animals in each group. Cytostatic leukopenia was modeled by a single intraperitoneal injection of cyclophosphamide solution to animals of all groups at the rate of 250 mg cyclophosphamide per 1 kg of animal body weight. Radiation leukopenia: 10 outbred nonlinear laboratory ICR (CD-1) mice (males) With an average body weight of 20-22 g, they were divided into 2 groups of 5 animals in each group. Radiative leukopenia of moderate severity was modeled by a single exposure to gamma braking radiation at a dose of 0.9 Gy on an ILU-10 pulsed linear accelerator. Investigation of the antitumor activity of low-molecularweight chitosan on an in vivo transferable tumor model: a cyclophosphamide-resistant solid form of mouse lymphosarcoma RLS 40 was used as a transferable tumor. To form a solid tumor, 22 outbred nonlinear laboratory ICR (CD-1) mice (males) were transplanted with lymphosarcoma from RLS 40 mice.

Results. As a result of the study, it was found that low-molecular-weight chitosan has therapeutic efficacy in cytostatic leukopenia, and its effectiveness was more pronounced with enteral administration. Based on the results of the study, it can be argued that enteral administration of low-molecular-weight chitosan, starting 2 days after administration of cytostatic, may be a very promising way to compensate for cytostatic myelosuppression and may significantly improve the effectiveness of treatment and quality of life of cancer patients. There is a pronounced therapeutic effect from the use of low-molecular-weight chitosan in compensation of moderate-grade radiation leukopenia. Low molecular weight chitosan has a pronounced oncoprotective effect on an experimental model of a solid form of mouse lymphosarcoma.

The volume and weight of a solid tumor in the experimental group 1 is almost two times lower than in the control group.

Conclusion. The data obtained allow us to consider low molecular weight chitosan as a very promising auxiliary agent in the treatment of malignant tumors by traditional methods of chemoradiotherapy. Low molecular weight chitosan is a part of enterosorbents. Due to its unique technology, this component is stable in the environment of the small and large intestines at a pH of up to 8.2, which distinguishes it from analogues that are stable only at acidic pH values. Compensation of cytostatic and radiation leukopenia by taking an aqueous solution of low molecular weight chitosan as part of enterosorbents. It can be an effective and safe way to reduce the complications of chemoradiotherapy and improve the quality of life of cancer patients. Preliminary data on the assessment of the oncoprotective effect of low-molecular-weight chitosan and enterosorbents based on it allow us to consider it as a safe means of preventing cancer, especially at the initial stage, but this requires further research on various models of transferable tumors.

Background. Stroke, especially in the acute phase, leads to significant cognitive impairment. At the same time, such an intense psychologically traumatic event exacerbates the course of cerebrovascular process. Numerous studies have demonstrated that post-stroke depression affects approximately one-third of stroke survivors and serves as a predictor for significantly worse functional recovery, recurrent stroke and death. Post-stroke depression and dementia adversely affect the rehabilitation process, impair the recovery of activities of daily living and quality of life, as well as are associated with a significantly poorer prognosis.

Objective. To predict the recovery of the ability to self-care in stroke patients depending on the severity of depression

Materials and methods. A study of 199 patients with stroke in the acute period (2.2 ± 0.4 days) was conducted. Dynamic observation was performed after 12.4 ± 0.9 days. The severity of neurosomatic status was studied on the NIHSS scale, on the SHRM scale and the Bartel index; on the MRS scale, on the Beck depression scale, on the Spielberger anxiety scale, on the MMSE scale. The severity of arthropathy was assessed by X-ray and ultrasound examination. The serum levels of TRAIL and sCD95 were determined

Results. The established prognostic significance of the severity of depressive disorders in the formation of restrictions on the restoration of the ability to self-care, as well as masking criteria for cognitive defect in patients with ischemic stroke dictates the need for early involvement of medical psychologists not only at the hospital level, but also for the continuous management of patients at the outpatient stage.

Background. Extrapulmonary tuberculosis represents organ and tissue damage caused by Micobacterium tuberculosis. It can occur in the absence of pulmonary tuberculosis or it can be associated with concurrent pulmonary involvement. Tuberculosis can affect any organ system, with the exception of muscle tissue, nails and hair. The potential sites of involvement include peripheral lymph nodes, bones, kidneys, eyes and middle ear. Following primary infection, notwithstanding successful therapy, the infection can reactivate at any time and in any part of the body. Extrapulmonary tuberculosis in pregnant women presents unique diagnostic and therapeutic challenges due to physiological changes during pregnancy and the often nonspecific clinical manifestations. Extrapulmonary tuberculosis can affect various organs, including lymph nodes, bones, kidneys, and the reproductive system, complicating timely diagnosis. The condition poses risks to both mother and fetus, such as preterm birth, low birth weight, and maternal morbidity. Early detection and appropriate treatment are crucial to improve outcomes. Understanding the features of extrapulmonary tuberculosis in pregnancy is essential for healthcare providers to ensure prompt management, prevent complications, and safeguard the health of both mother and child.

Results. A comprehensive clinical case analysis was conducted in a scientific article on a pregnant woman with extrapulmonary tuberculosis, identifying the features of the disease course, evaluating diagnostic and therapeutic approaches, and determining the factors influencing pregnancy outcomes and the health of the mother and fetus.

Conclusion. In a patient with multiple infectious episodes and signs of chronic inflammation, including caseous lymphadenitis, a comprehensive and long-term monitoring is necessary. If tuberculosis is confirmed, anti-tuberculosis therapy should be initiated. Timely diagnosis and treatment are crucial to ensure a favorable outcome for both mother and child.