In the year of the 80^th anniversary of the Victory in the Great Patriotic War, we remember the feat of medical workers of besieged Leningrad – courageous, steadfast and brave people. In the history of wars, large cities did not withstand the siege, they either surrendered or died out due to epidemics. But Leningrad lived, worked, fought. And together with their hometown, the employees of the Leningrad Pediatric Medical Institute (LPMI) – doctors, nurses, lab technicians, orderlies worked in extreme conditions, and students continued their studies in cold classrooms without electricity, while many of them were in a state of alimentary dystrophy. In this article, we will turn to unique materials that are stored in the archive of the Department of Pathological Anatomy with a course of forensic medicine named after prof. D.D. Lokhova of the Saint Petersburg State Pediatric Medical University – protocols of pathological anatomical and forensic autopsies, set out in the journals of the “Clinical Hospital of the Leningrad State Pediatric Medical Institute” for the period from September 8, 1941 to January 27, 1944. The autopsy protocols are a chronicle of the terrible Leningrad blockade – the most severe, massive and long-term extreme situation that people have ever experienced in modern history. The result of the prosector’s work is always a formulated final diagnosis. During the difficult years of the blockade, when due to malnutrition, both quantitative and qualitative, hypothermia, fatigue and mental trauma, there was a change in the course of many, including dangerous diseases, a slowdown in regeneration processes in wounds, an atypical clinical picture of acute infections took place. The correctness of diagnostics allowed to prevent the spread of infections, to treat various diseases in conditions of alimentary dystrophy, avitaminosis. Particularly close attention of the prosectors was directed to identifying signs of especially dangerous infections.

Introduction. Intraventricular hemorrhages (IVH) in extremely preterm newborns significantly affect the course of the early neonatal period and can significantly affect the outcomes of their care.

Aim. Study is to develop new methods for predicting intraventricular hemorrhages (IVH) in very preterm newborns.

Materials and methods. A total of 227 extremely preterm infants with a gestational age of less than 32 weeks were examined: Group 1 consisted of 125 infants with IVH of varying severity, including subgroup 1a – 80 with IVH of grade I, 1b – 39 with IVH of grade II, 1c – 6 infants with IVH of grade III; Group 2 consisted of 102 extremely premature infants without IVH. The analysis of the health status of mothers of newborn children, the course of the current pregnancy and childbirth, the course of the early neonatal adaptation period was carried out; laboratory parameters were studied: neuromodulin (GAP-43), β-arrestin-2, platelet indexes in children depending on IVH. Risk factors were calculated using the “Open Epi” program.

Results and discussion. Risk factors for the development of IVH include the absence or incomplete antenatal prevention of respiratory distress syndrome, severe asphyxia at birth, and invasive artificial ventilation. Factors that contribute to the prevention of IVH include placental insufficiency and intrauterine growth retardation syndrome. The significance of the concentration of GAP-43 and β-arrestin-2 in the blood serum of a newborn for the development of IVH was revealed, regardless of the degree of prematurity. The development of IVH in very preterm newborns is associated with high platelet granularity (p = 0.002) and low -thrombocrit (p = 0.004).

Conclusion. The results of the study made it possible to develop new methods for predicting IVH in extremely preterm patients to enable a personalized approach to their management.

The incidence of bronchopulmonary dysplasia in leading clinics around the world remains high and even increases in some countries, which directly depends on the gestational age and duration of respiratory therapy. Despite a significant increase in survival rates of premature infants and a decrease in the incidence of other diseases, the incidence of bronchopulmonary dysplasia remains high. Over the past three decades, the incidence of bronchopulmonary dysplasia in premature infants in different countries has varied from 11 to 50% and has become increasingly common due to improvements in nursing methods and, as a result, an increase in the survival of extremely premature infants. To date, there are no specific treatments to prevent the development of bronchopulmonary dysplasia. However, prevention of preterm birth and antenatal steroid prophylaxis, optimal respiratory approaches with preferences for non-invasive ventilation, and the use of exogenous surfactants reduce the incidence and risk of bronchopulmonary dysplasia. The article presents a clinical observation of a group of infants, which included 22 infants (9 girls, 13 boys) with the risk of developing or progressing bronchopulmonary dysplasia. All infants underwent treatment in the neonatal intensive care unit (NICU) setting of the Regional Perinatal Center at Nizhny Novgorod Federal City Clinical Hospital No. 40 (Avtozavodskoy District, Nizhny Novgorod) and received combination therapy. It has been established that the use of inhaled surfactant in premature infants showed positive outcomes.

Introduction. Among acute inflammatory diseases of the respiratory tract in children, the leading place belongs to acute bronchitis, the main symptom of which is cough. In the treatment of cough, combination drugs are widely used. They have a complex effect on various pathogenetic mechanisms.

Aim. To evaluate the efficacy and safety of a combination drug containing bromhexine, guaifenesin and salbutamol in children with acute non-obstructive and obstructive bronchitis.

Materials and methods. The study included 60 children with acute non-obstructive bronchitis of viral etiology aged 2 to 12 years and 30 children with acute obstructive bronchitis, having a mild course, aged 2 to 6 years. Children with acute non-obstructive bronchitis were divided into 2 groups; the main group – 35 children, receiving a combination drug containing bromhexine, guaifenesin and salbutamol in an age-appropriate dose for 4–5 days as part of the therapy, and a comparison group of 25 children receiving ambroxol as part of the therapy. The effectiveness was assessed based on a study of the dynamics of the main clinical symptoms of the disease.

Results and discussion. It was found that the administration of a combination drug containing bromhexine, guaifenesin and salbutamol showed clinical efficacy in children with non-obstructive bronchitis in 88.7% of cases, and in mild bronchial obstruction – in 86.6%, which was manifested by a pronounced positive dynamics in the main clinical symptoms of the disease, as well as a decrease in the development of bacterial complications. The drug is well tolerated (91.4% of cases) and rarely produces minor adverse effects.

Conclusions. A combination drug containing bromhexine, guaifenesin and salbutamol has shown high clinical efficacy and good tolerability in both non-obstructive bronchitis and mild broncho-obstruction, which allows us to recommend it as one of the drugs of choice in the treatment of this pathology.

In the structure of general morbidity, respiratory allergic pathology occupies the third position, but according to forecasts of the World Health Organization, by 2050 it will take first place. According to statistics, the prevalence of AR in Russia is up to 24%. The number of AR cases in children is increasing, and almost half of AR patients develop symptoms before the age of 6 years. AR negatively affects the quality of life, school performance, and overall health. An important comorbid condition in allergic rhinitis in children is adenoid hypertrophy, which is closely related to the allergic process in the nasal cavity. The use of intranasal corticosteroids (mometasone furoate) as monotherapy significantly reduces both the symptoms of allergic rhinitis and the size of the pharyngeal tonsil. In combination with the leukotriene inhibitor montelukast mometasone furoate allows for better results and fewer relapses compared to monotherapy with mometasone intranasal administration alone. According to the analyzed clinical observations in patients with allergic rhinitis and adenoid hypertrophy, mometasone furoate and mometasone furoate and montelukast showed a good clinical effect. Based on the analysis of systematic reviews on the etiology, clinic and diagnosis of allergic rhinitis in combination with adenoid hypertrophy, data on the relationship between adenoid hypertrophy and allergic rhinitis and methods of their correction, as well as clinical studies on the efficacy and safety of mometasone furoate and montelukast, it is concluded that their use in a complex treatment of allergic rhinitis and adenoid hypertrophy in children.

Acute respiratory infections (ARI) can be called a global burden of modern pediatrics. The most frequent incidence of ARI, mainly of viral etiology, is experienced by children of the first 6 years of life: infants, as well as children of early and preschool age. This is explained primarily by the physiological characteristics of their immune system. In this regard, the issue of rational selection of an immunotropic drug with proven efficacy and safety, capable of both increasing resistance to infectious agents and increasing the effectiveness of the complex therapy of ARI, especially in the most vulnerable categories of patients from the point of view of the immune response, is becoming relevant. Among the numerous immunomodulatory drugs, a group of bacterial lysates stands out, attracting the attention of practicing physicians and researchers in the context of non-specific immunoprophylaxis and immunotherapy of ARIs. The use of drugs in this spectrum promotes the activation of innate and adaptive links of immunity, faster relief of symptoms of respiratory infection, and also reduces the risk of complications and, accordingly, reduces the need for antibacterial therapy. A special feature of the pharmacological action of this group of drugs is the induction of an adaptive immune response not only against pathogens whose antigens are part of bacterial lysates, but also an increase in resistance to many other bacterial and viral infectious agents. Among bacterial lysates, OM-85 is the leader in terms of evidence – the most studied modulator of anti-infective protection of the respiratory tract. Its effectiveness and safety have been demonstrated in numerous randomized clinical trials. Its effectiveness and safety have been demonstrated in numerous randomized clinical trials. The article presents data confirming the advantages of OM-85 over other immunotropic drugs.

Viral diseases of the respiratory tract are one of the most pressing health problems worldwide. Influenza viruses, parainfluenza, rhinoviruses, adenoviruses, herpes viruses, enteroviruses, ESNO and Coxsackie viruses, penetrating by airborne droplets and affecting mucous membranes, cause catarrhal inflammation at the sites of invasion, which is accompanied by rhinitis, pharyngitis, tonsillitis, laryngitis, tracheitis. Viruses determine a complex of immunological disorders leading to an imbalance of cellular and humoral immunity factors, a change in mucociliary clearance, which contributes to the chronic persistence of viruses and the attachment of bacterial infection. The authors consider the features of the clinical course of viral lesions of the respiratory tract, possible complications and approaches to the treatment of these conditions. In the absence of effective etiotropic treatment for most respiratory viral infections, the possibility of influencing the course of the disease by stimulating phagocytosis, T-lymphocytes, reducing the production of proinflammatory cytokines and increasing the level of immunoglobulins and interferons is of great importance. The authors discuss the prospects of using inosine pranobex, a synthetic purine derivative, as an immune–stimulating and non-specific antiviral agent in the treatment of inflammatory diseases of the respiratory tract caused by viral infection. The incidence of influenza and acute respiratory viral infections exhibits seasonal patterns, however in addition to sick contacts, the immune system status is also important, which explains the recurrence of viral infections in young children and adults with chronic somatic pathology accompanied by immunosuppression. At the same time, the physiological features of the early life immune system are primarily characterized by the insufficiency of cellular and humoral factors of local immunity. This circumstance takes on particular and important significance in the context of insufficient effectiveness of respiratory viral infections therapy with available antiviral and symptomatic drugs and allows to consider the prospects for using immunostimulating agents. The authors conclude that inosine pranobex is effective and safe in the treatment of a wide range of respiratory viral infections.

Introduction. Obesity is a pressing public health issue. An important task is the study of individual clinical variants of obesity in specific patient groups, including adolescent boys.

Aim. To study the heterogeneity of sleep quality indicators, emotional and behavioral characteristics, and eating behavior in adolescent boys with obesity.

Materials and methods. The study included 164 adolescents aged 14–17, comprising 137 with obesity (E66.0, ICD-10) and 27 healthy controls. Sleep quality was assessed using the Adolescent Sleep-Wake Scale (ASWS). Behavioral and emotional disorders were evaluated using the Achenbach questionnaire (Youth Self Report, YSR 11-18). Eating behavior was assessed using the Dutch Eating Behavior Questionnaire (DEBQ).

Results. Six distinct clusters were identified: (1) adolescents with thought problems and high aggression; (2) children with sleep and eating disorders, low social adaptation, and high aggression; (3) adolescents without significant issues; (4) socially adapted adolescents with internalized problems and emotional eating; (5) adolescents with eating disorders, deviant behavior, and aggression; and (6) socially adapted adolescents with restrictive eating behavior. Healthy adolescents predominantly belonged to the third cluster, while adolescents with obesity showed diverse cluster representation. Assignment to any cluster other than the third was a significant predictor of increased BMI z-score.

Conclusion. The study revealed significant differences in emotional and behavioral characteristics among adolescents with obesity, emphasizing the importance of considering these factors when selecting treatment approaches.

Introduction. The choice of drugs for the treatment of intercurrent diseases in diabetes mellitus (DM) is limited: drugs containing glucose and sucrose increase glycemia. Drug composition may also include substances that are falsely recognized by glucometer instead of glucose, which can lead to error in correction of insulin therapy. Ibuprofen and paracetamol do not effect accuracy of glucose measurements by glucometer; interference of cough medicines and excipients have not been studied.

Aim. To evaluate the effect of popular antipyretic and cough medicines liquid drug forms used in children on the accuracy of glucose measurement using a glucometer.

Materials and methods. We tested samples of antipyretic syrups (ibuprofen, nurofen, paracetamol), syrups and solutions of cough medicines (lazolvan, bromhexine). Paracetamol syrup contained sucrose; all syrups but bromhexine contained glycerol; paracetamol and bromhexine syrups contained propyleneglycol. We added a drop of each drug sample to the low- and high-glucose control solutions (CS), and then measured the glucose concentration using a glucometer. The results were compared with the reference range for the corresponding CS. Each test was repeated 5 times.

Results. Adding of antipyretic syrups to CS led to an overestimation of the results regardless of the presence of sucrose in the syrup. Adding of lazolvan solution did not distort the result; but syrup increased it. Adding of bromhexine solution or syrup resulted in the same borderline overestimation of the result.

Conclusion. Patients with diabetes should avoid using medications in syrup form, even if they do not contain glucose and sucrose, since the excipients included in the syrup may falsely increase glycemia values determined by a glucometer. Our results suggest that the use of solutions is safer. It is important to take in account that active substances in some medications can also distort blood glucose measurement results.

The gut microbiome plays a key role in the developing body, being a crucial component of bidirectional functional axes. There is a cross-talk between the gut microbiota composition and immune system development, metabolism, neurogenesis, gastrointestinal integrity and many other processes. The mechanisms whereby this influence is exerted are actively discussed. The gut microbiota has been found to interact with the brain through the microbiota-gut-brain axis, regulating various physiological processes. The gut microbiota is considered to regulate neurodevelopment through three pathways: the immune pathway, the neuronal pathway, and the endocrine-systemic pathway, which overlap and cross-talk with each other. The gut microbiome is a system which undergoes dynamic changes, which pattern has certain regularities. During fetal development, the microbiome progresses over time in parallel with the complex, genetically determined process of neuronogenesis. New studies confirm the active impact of the intestinal microbiome and its metabolites on the neuropsychic development of children. However, homeostasis within microbiota-gut-brain system can be disrupted, which increases the risk of developing neuropsychiatric disorders, including autism spectrum disorders, and attention deficit hyperactivity disorder. The diversity and abundance of bacterial colonization are influenced by a number of factors, including infant nutrition. More emphasis has been rightly placed on the role of specific nutrients in the neurodevelopment of young children. Timely supplementation of critically important food components serves as a basis for developing approaches that ensure the prevention of a whole range of nervous system diseases. Given that breastfeeding rates are still below desirable levels, special attention should be paid to the infant formula components that affect infant health.

Introduction. Psoriasis is often accompanied by obesity, insulin resistance, arterial hypertension and dyslipidemia. It is noted that the higher the PASI score, the higher the risk of obesity in patients.

Aim. To study the polymorphism of the genes FADS1 rs174544, FADS1 rs174583; APOE rs769452, APOA1 rs1799837; FTO rs9939609; LIPC rs2070895 and the impact on nutritional status in children with psoriasis.

Materials and methods. In a single-center, cross-sectional cohort study, gene polymorphism was studied in 45 patients, average age 14.05 years. Unified examination protocol: PASI; Antro Plus WHO, MEDASS bioimpedance. Genotyping of genomic DNA samples was performed in real time on a DT-Prime amplifier (RT-PCR). Statistical analysis StatTech v. 3.1.8.

Results. PASI averaged 32.2 points. Average BMI was 22.6 kg/m² [20.288; 25.069], BMI Z-score was 1.706 [-0.175; 0.928]. Overweight and obesity are observed in adolescents according to weight Z-score (17.1%) and BMI (5.7%), mainly in boys. Children with obesity are characterized by CC alleles of FADS1 rs174544 and FADS1 rs174583; correlations with active cell mass (p = 0.034), its proportion (p = 0.013) and skeletal muscle mass (SMM) (p = 0.049). APOE rs769452 only homozygous variant Leu/ Leu occurs. In children with obesity, the CC allele of APOA1 rs1799837 is more common. The FTO rs9939609 gene was found in the AT and AA alleles in children with excess body weight and obesity. For TT carriers, the highest proportion of %SMM (p = 0.026). For %SMM (Mann-Whitney U-test), statistically significant differences were revealed (p < 0.05) between A / A and T / T (p = 0.028) A / T and T / T (p = 0.008).

Conclusions. Obesity and psoriasis, having different roots, can be regulated by the same genes. However, the relationship between psoriasis and obesity in children has not been fully studied and remains the subject of research and discussion in the scientific literature.

In newborns and young children, the skin is characterized by relative morphofunctional immaturity, a reduced degree of differentiation of structural elements and the thickness of the epidermal and dermal layers, resulting in increased susceptibility to exogenous factors and an increased risk of pathogens, which increases the likelihood of infectious and inflammatory processes. Diaper dermatitis is a common inflammatory skin disease that significantly reduces the quality of life of the child and his parents. The main symptoms are redness, swelling and discomfort in the diaper area. Effective treatment and prevention of diaper dermatitis require restoration and strengthening of the skin barrier, as well as ensuring sufficient skin hydration. This article examines the anatomical and physiological features of the skin of newborns and young children that determine its increased sensitivity and vulnerability to external influences. The factors contributing to the development of diaper dermatitis are analyzed in detail, including increased humidity, friction, exposure to urine and feces, as well as a violation of the barrier function of the skin. Particular attention is paid to the role of emollients-plus – modern products containing not only moisturizing and softening components, but also additional ingredients such as prebiotics, antioxidants and anti-inflammatory substances for the prevention and treatment of diaper dermatitis.

Introduction. The paediatric tuberculosis screening coverage rate can vary significantly depending on its calculation and planning methodology, which will ultimately affect the formation of rates in the reporting period.

Aim. To review the results of annual immunological screening and the methodology for calculating the preventive tuberculosis screening coverage rates for the metropolitan population aged 1–17 years.

Materials and methods. The data from 2023 healthcare facility reports on preventive screening of children aged 1–17 years in Moscow using a skin test with purified tuberculosis allergen-standard solution and a skin test using recombinant tuberculosis allergen-standard solution were used.

Results. In accordance with the 2023 population screening program for early detection of tuberculosis in Moscow, the number of children screened for tuberculosis using immunoassay tests amounted to 1,900,739 (96.4% coverage of the assigned population). According to the data of Federal Statistical Monitoring (FSN) Form No. 30, 1,921,590 children (including 718,272 children aged 1–7 years) were screened for early detection of tuberculosis in Moscow healthcare facilities in 2023 (97.5% coverage rate against the program target), and 1,916,255 children were screened in healthcare facilities with assigned population (97.2% coverage rate against the program target). The preventive pediatric tuberculosis screening made it possible to identify 45 new cases of tuberculosis among children and adolescents, 86 new cases of residual post-tuberculosis changes, and also to form a high-risk group among children with altered tuberculosis allergen sensitivity (3,772).

Conclusions. The paediatric tuberculosis screening coverage rate depends on its calculation methodology; it should be planned and calculated based on the number of the assigned population to be served by healthcare facilities.

Introduction. Any contact with a TB patient is the leading risk factor for developing TB disease in children and adolescents, and availability of a family/close relative contact matters the most for its realization. TB foci with multidrug-resistant and extensively drug-resistant Mycobacterium tuberculosis (MDR/XDR MBT) strains that are present at the source of infection require special attention as the most epidemically dangerous ones.

Aim. To identify the major reasons for the development and late diagnosis of TB disease in adolescents from the infection foci, and to find possible ways to address identified issues.

Materials and methods. A retrospective cohort study (2021 to 2023) was conducted. 90 medical records of adolescents were analysed. In 53 cases, contacts with a TB patient were identified (58.9%). The statistical relationship between the studied risk factors that could affect the late detection of the TB disease was calculated using the Pearson χ2 test.

Results. It has been established that a lack of proper follow-up medical care over exposed persons at the tuberculosis dispensary (TBD) (violations of the procedures for follow-up checkups, preventive chemotherapy) and missed opportunities in mass screening in the primary health care (PHC) facilities are the main reasons for late detection of the disease, especially when several risk factors are combined, and clinical signs of the disease are present. TB screening methods applied at the infection foci have proven to be most effective when implemented on a regular basis. The disease can manifest during long intervals between medical checkups in the absence of pronounced clinical symptoms of the disease and in a torpid course, which identifies inconsistency between TB diagnostic techniques (screening) and its clinical form (widespread, complicated processes).

Conclusion. To solve this issue, the following measures are required: mandatory follow-ups of exposed persons at the TBD regardless of legal representative / patient’s wishes; determination of the conditions for isolation of a source of infection from a contact adolescent until bacterial excretion is completed; personalized follow-up care and thorough medical checkups of exposed persons, if MDR/XDR MBT strains are identified at the source of infection; improvement of interdisciplinary collaboration between primary health care facilities.

Introduction. Knowing the patient’s genetic profile, it is possible to predict the likelihood of developing food allergies.

Aim. The study was to conduct a study in children with allergic enteropathy: vitamin D levels, genotypic distribution of polymorphic variants of the VDR gene, the content of fecal biomarkers (zonulin, I-FABP and b-defensin 2).

Materials and methods. The study included 30 toddlers: 15 children with allergic enteropathy (main group) and 15 healthy children (control group). In the blood serum, 25(OH)D was determined by enzyme immunoassay, and the real-time polymerase chain reaction method was used to identify VDR gene polymorphisms. The content of fecal biomarkers (zonulin, b-defensin 2, I-FABP) was estimated in coprofiltrates using the enzyme-linked immunosorbent assay method.

Results. There was a statistically significant decrease in the level of 25(OH)D in children of the main group compared with healthy children (21.4 ng/ml and 32.4 ng/ml, respectively, p = 0.006). In the coprofiltrates of children in the main group, an increase in the concentration of zonulin, b-defensin 2 and I-FABP was noted compared to the control (1.6 ng/ml and 0.6 ng/ml (p < 0.001), 0.3 ng/ml and 0.1 ng/ml (p = 0.003), 0.42 ng/ml, and 0.19 ng/ml (p < 0.001), respectively). In children with allergic enteropathy, a high frequency of occurrence of the homozygous genotype T/T polymorphism TaqI (rs731236) of the VDR gene has been recorded. The content of 25(OH)D is inversely correlated with b-defensin 2 and I-FABP (rs = – 0.673, p < 0.001 and rs = – 0.399, p = 0.029, respectively).

Conclusions. In patients with allergic enteropathy, insufficient levels of 25(OH)D, increased concentrations of the studied markers in feces, and an increased prevalence of the homozygous T/T genotype of the TaqI polymorphic variant (rs731236) of the VDR gene were detected.

The paper analyzes nutritional support for a child with disseminated mucormycosis against the background of newly diagnosed diabetes mellitus. Mucormycosis is an aggressive fungal infection, especially dangerous for immunocompromised individuals, which include patients with primary and secondary immunodeficiencies. Diabetes mellitus acts as the main disease leading to secondary immunodeficiency, especially in the presence of ketoacidosis. Pain syndrome, cosmetic defect of the hard and soft palate, lack of ability to eat in the usual way against the background of the underlying disease contributed to the development of progressive cachexia. Gastrostomy and specialized enteral nutrition gave a chance for recovery. All medications were administered through a gastrostomy tube without causing pain. The dynamics of nutritional status were monitored using bioimpedancemetry data. With nutritional support from mixtures based on deeply hydrolyzed protein with medium-chain triglycerides at the beginning of feeding through a gastrostomy, it was possible to restore the mucous membrane of the gastrointestinal tract. Subsequently, the patient received specialized mixtures recommended for carbohydrate metabolism disorders. A balanced composition of enteral nutrition made it possible to achieve a stable blood glucose level, correct hypoproteinemia, hypoalbuminemia and hypokalemia that arose during therapy with an antifungal agent. Restoration of swallowing and removal of gastrostomy is possible with stable positive dynamics of nutritional status, control over the underlying disease and restoration of cosmetic defects.

Introduction. The clinical manifestations of acute surgical diseases of the abdominal organs in children may be similar to acute intestinal infections. To date, there remains a need to optimize the therapeutic and diagnostic algorithm in children due to the emergence of “new” and expanding the range of rare nosologies of “acute abdomen” with atypical clinical manifestations of diseases for certain age groups of children.

Aim. To optimize the treatment and diagnostic algorithm for children with acute surgical diseases of the abdominal organs and acute infectious diseases.

Materials and methods. The study included 437 children with acute surgical diseases of the abdominal cavity and acute infectious diseases. All patients underwent examinations at the preoperative stage in accordance with the clinical protocol for the diagnosis and treatment of acute surgical diseases in children. The volume of surgical intervention was determined during laparoscopy. The average age of the patients was 10.23 ± 3.3 years. The prehospital period is 15.9 ± 8.5 hours. The preoperative period was 14.6 ± 7.6 hours. The coincidence of diagnoses at admission/clinical was 413/331 (80%).

Results and discussion. Three variants of clinical and instrumental manifestations of acute surgical diseases of the abdominal cavity in combination with acute intestinal infections in children have been identified. Reliable differences between individual indicators of clinical and anamnestic, instrumental and intraoperative data in groups of patients with acute surgical and infectious diseases have been proved.

Conclusion. The atypical course of acute abdominal symptoms in children from the younger age group is due to a predominance of systemic inflammatory responses over local symptoms in 30% of cases. It is justified to intensify diagnostic measures, such as expanding the indications for laparoscopy, in children from younger age groups.

Lysosomal acid lipase deficiency (LALD, cholesterol ester storage disease, OMIM #278000) is a rare autosomal recessive disorder resulting from underlying LIPA gene mutations, leading to impaired hydrolysis of cholesterol esters and triglycerides within lysosomes. Common presenting symptoms include hepatomegaly, dyslipidemia, increased transaminases, steatosis, and in severe forms – progressive liver fibrosis and early atherosclerosis. The presented clinical case describes the management of LALD in an 8-year-old girl, characterized by early age onset, absence of typical delay in physical development and pronounced polymorphism of clinical manifestations. For several years, gastrointestinal symptoms combined with anemia, nosebleeding, and fatigue, which were evaluated as functional disorders prevailed. The diagnostic search was initiated late, despite the presence of hepatomegaly, persistent hypercholesterolemia, and elevated transaminases. The diagnosis was verified based on decreased enzymatic activity of lysosomal acid lipase and results of the molecular genetic analysis (variant c.894G>A in the LIPA gene in the homozygous state). Liver steatosis and fibrosis progression was observed in the absence of timely initiation of enzyme replacement therapy. A clinical example demonstrates the difficulties of early diagnosis of lysosomal storage diseases in pediatric practice. The case highlights the diagnostic challenges due to phenotypic variability and clinical similarities with more common disorders, including familial hypercholesterolemia. The article discusses differential diagnostic approaches, including exclusion of viral hepatitis, Wilson's disease, autoimmune liver diseases, and hereditary metabolic diseases. The need for early diagnosis, family screening and optimization of access to enzyme replacement therapy has been underlined. The article also outlines promising directions in the development of neonatal screening programs and areas of targeted therapy, including gene technologies. This case emphasizes the need to increase the alertness of clinicians and introduce systemic diagnostic algorithms into clinical practice.

Eosinophilic esophagitis is a rare chronic inflammatory disease of the esophagus, which is entered into the list of orphan and rare diseases of the Ministry of Health of the Russian Federation in 2024. In recent years, the disease has become increasingly important in clinical practice, especially in pediatrics, as its prevalence is rapidly growing throughout the world. It is reported that eosinophilic esophagitis is associated with allergic diseases such as food allergy, atopic dermatitis, bronchial asthma and allergic rhinitis, and represents a significant clinical and diagnostic challenge due to the similarity of symptoms with gastroesophageal reflux disease. Such non-specific symptoms as regurgitation, nausea and vomiting, abdominal pain while eating are observed in young children, and complaints of dysphagia are registered in older children. It is quite difficult to diagnose eosinophilic esophagitis due to the lack of specific symptoms and markers, as well as the need for invasive diagnosis confirmation using endoscopy and biopsy examinations. This results in frequent cases of understaging or late diagnosis, which, in turn, can worsen the prognosis and patient’s quality of life. The article presents clinical cases of eosinophilic esophagitis in young children, assessment of symptoms, diagnostic search, and principles of therapy choice.

Introduction. The close interest of a wide range of pediatricians in the problems of bronchial asthma (BA) is due to its prevalence, difficulties in selecting basic therapy, and the high frequency of hypovitaminosis D.

Aim. To analyze the frequency of vitamin D deficiency and the effectiveness of its correction by including cholecalciferol preparations in the standard step therapy regimen for bronchial asthma in children.

Materials and methods. The study involved 75 children and adolescents from the Stavropol Territory suffering from BA, aged

12.4 [9.5; 14.8] years. From 2018 to 2021, a two-stage study of vitamin D status was conducted in patients – initially and after completion of a 3-month intake of cholecalciferol aqueous solution (CCF).

Results. At the 1st stage of treatment, all 3 (100%) patients were over 12 years old and received low doses of ICS each time they used SABA without constant basic therapy. At the 2nd stage of therapy, all 9 (100%) patients used LRA drugs in age-appropriate dosages. At the 3rd stage, 1 (5.3%) child received treatment with medium doses of ICS, 10 (52.6%) children used a fixed combination of ICS-LABA, and 8 (42.1%) a combination of ICS + LRA.

The vitamin D status after taking cholecalciferol preparations increased from 18.3 ng/ml to 35.4 ng/ml. Vitamin D deficiency was initially detected in 44 (58.7%) children, and after the correction course – only in 3 (4.0%) people (p < 0.001).

Initially, the median AST was 16.0 [12.0; 20.5] points, and at the end of the study 20.0 [16.0; 24.0] points (p < 0.004).

Conclusion. Step therapy for asthma should be prescribed in according to current clinical guidelines. ICS should be used more widely as initial basic therapy for asthma, reducing the number of people receiving monotherapy with ALR.

In recent years, probiotics have gained increasingly greater attention of clinicians due to their use in the prevention and treatment of multiple diseases. At the same time, the number of myths imposed by different media is also growing. The main mechanisms of action of probiotics include improvement of the mucosal barrier function, direct antagonistic activities against pathogens, inhibition of bacterial adhesion and invasion in the intestinal epithelium, enhancing of immunity and central nervous system regulation. Normal gut microbiota plays a critical role in maintaining the human health. Gut microbiota imbalance and dysbiosis may give rise to such diseases as infections, autoimmune diseases, allergies, etc. Probiotics seem to be a promising approach to the prevention and even relief of symptoms of such clinical conditions as an adjuvant therapy by preserving the normal gut microbiota balance and boosting the immunity. The present review refutes common myths that prevent from recommending and using probiotics for various indications. To date, stronger evidence in favour of their clinical use is provided in the prevention of gastrointestinal disorders, antibiotic-associated diarrhea, allergy and respiratory infections. Numerous in vitro studies, preclinical and clinical trials showed the significance of the human microbiota, improvement and restoration of its functions by probiotic treatment. In this review, we tried to summarize current knowledge on probiotics’ use and therapeutic potential in different diseases of not only digestive system, but also outside the gastrointestinal tract.

Atherosclerosis, which begins early in life, accounts for a significant share of cardiovascular diseases in adulthood. At the same time, the prevalence of obesity, high blood pressure and dyslipidemia among children in certain regions of the Russian Federation reaches double figures. New order No. 211n of the Ministry of Health of Russia provides for the targeted lipid screening of children aged 6 and 10 years, however specific clinical and anamnestic grounds and criteria for being qualified as children at risk have not yet been regulated. This paper presents an analytical review of federal regulatory acts, current clinical guidelines and national multicenter studies (2014–2024), which gave rise to a list of indications for targeted screening for lipid profile in children and adolescents. The proposed checklist includes 17 clinical, anamnestic and phenotypic criteria grouped into eight blocks: (1) hereditary predisposition, (2) phenotypic markers for hyperlipidemia, (3) the presence of overweight/obesity, (4) resistant hypertension, (5) severe metabolic and systemic diseases, (6) congenital and acquired valvular heart diseases, (7) modifiable behavioural factors, (8) earlier cardiotoxic conditions. The presence of one key or two additional symptoms serves as a basis for an extended lipid panel testing and subsequent follow-up care. The presented model does not have a regulatory status and reflects the point of view of the authors' team; it is intended for expert discussion, improvement and further clinical validation. The proposed tool can be easily integrated into electronic health records, automating patient routing and creating the groundwork for prospective studies of Russian pediatric cohorts.

Lower urinary tract dysfunction in children is a complex set of disorders affecting not only physiological, but also neuropsychiatric aspects of development. Understanding the pathophysiological mechanisms underlying overactive bladder and other forms of lower urinary tract dysfunction allows for a more effective approach to the diagnosis and treatment of these conditions. It is important to remember that the delay in the development of the nervous regulation of urination, the peculiarities of brain functioning and concomitant psychological factors play a key role in the onset and maintenance of symptoms of lower urinary tract dysfunction. The key point in the successful management of children with urinary dysfunction is an integrated approach that takes into account all factors affecting the function of the bladder and intestines, as well as the psycho-emotional state of the child. Careful history collection, detailed examination and adequate interpretation of the results make it possible to avoid erroneous diagnoses and prescribe effective treatment. Timely detection and medical correction of not only urological, but also neuropsychiatric problems in children with urinary dysfunction is the key to improving the child’s quality of life and preventing the development of long-term psychological and social consequences. Underestimation of psychoemotional factors can significantly reduce the effectiveness of treatment and lead to the chronization of the process. Further research in this area is needed to develop more effective and personalized approaches to the treatment of urinary dysfunction in children, taking into account both physiological and psychosocial aspects.

Introduction. Familial hypercholesterolemia (FH) is the most common hereditary disorder of lipid metabolism and a significant risk factor for the early development of cardiovascular diseases. Effective detection of FH in childhood allows timely initiation of therapy and significantly improves the prognosis.

Aim. To evaluate the effectiveness of targeted, universal and combined screening for the detection of new cases of familial hypercholesterolemia in children, including determining the sensitivity, specificity and predictive value of the methods.

Materials and methods. 521 children aged 11–13 years were examined. All participants underwent a questionnaire to identify a family history of cardiovascular diseases and a study of the lipid profile (TC, LDL, HDL, TG). The diagnosis of probable FH was established according to the Simon–Broome criteria. Sensitivity, specificity, positive and negative predictive values, as well as ROC analysis were used to evaluate the effectiveness.

Results. Targeted screening revealed 5 out of 15 cases of FH (sensitivity – 33.3%, specificity – 85.8%, PPV – 6.5%). Universal screening provided 100% sensitivity and specificity. The combined approach also showed 100% sensitivity with a specificity of 85.8% and a PPV of 17.2%. ROC analysis demonstrated the maximum diagnostic accuracy of the combined method (AUC = 1.00).

Conclusion. Universal and combined approaches are superior to targeted screening in terms of the effectiveness of detecting FH in the pediatric population. Combined screening makes it possible to achieve high-quality diagnostics with reasonable use of resources. The data obtained confirm the expediency of introducing expanded programs for the detection of FH in the framework of the prevention of cardiovascular diseases in children.

The article presents an overview of the current understanding of lysosomal storage diseases (LSD) – a group of rare genetic disorders characterized by abnormal accumulation of macromolecular degradation products within cell organelles. The clinical manifestations of LSD vary significantly depending on the type of disorder and may include liver damage, neurological symptoms, and involvement of other organs. To illustrate the unique features of clinical course and mechanisms of liver injury associated with different types of LSD, individual nosologies have been reviewed, including Gaucher’s disease, Niemann-Pick disease, lysosomal acid lipase deficiency, gangliosidoses, and mucopolysaccharidoses, all of which show substantial differences in their pathogenesis and clinical presentations. Diagnostic criteria were described, focusing on measurements of enzyme activities and identification of pathogenic genotypes, alongside specialized biomarkers utilized for confirming diagnoses. Diagnosis of LSDs relies on assessing enzyme activity or metabolite concentrations in biological samples, while molecular-genetic testing is employed to verify the diagnosis. In this review, various therapeutic strategies are discussed, encompassing enzyme replacement therapy (ERT), substrate reduction therapy, and liver transplantation when severe organ involvement occurs. Early detection and prompt initiation of treatment are emphasized as crucial steps to prevent irreversible organ damage. Additionally, the prospects of applying gene therapy, which has shown positive outcomes in experimental studies, are highlighted. This approach opens up new possibilities for innovative treatments aimed at restoring normal enzyme function and preventing disease progression. The presented data offer a comprehensive insight into the issues surrounding the diagnosis and management of LSDs, which is important for both clinicians and researchers engaged in the study of rare diseases.

Introduction. Increased protein and energy requirements, high levels of inflammatory response, osteopenia, and endogenous intoxication syndrome in patients with cerebral palsy determine the need to use therapeutic enteral mixtures with a metabolic focus (EMF) in their nutrition.

Aim. Evaluate the clinical efficacy and tolerability of the use of domestic therapeutic nutrition (nutritive support (NS) – ESMN Detoxifying Protein Cocktail NUTRIO and Bone Tissue Cocktail (protein) in children with cerebral palsy.

Materials and methods. A comparative prospective study was conducted at the State Healthcare Institution of the Tyumen Region, the Children’s and Rehabilitation Center Nadezhda. 39 children with cerebral palsy aged 3 to 18 years with a severity of motor impairment of GMFCS III-V were observed. All patients were divided into 4 groups depending on their age and nutritional support: 1^st main (n = 5) aged 3–8 years with NS; 1^st control (n = 5) without NS; 2^nd main (n = 21) aged 9–17 years with NS; 2^nd control (n = 8) without NS. The study period was 30 days. Before and After the study, the general condition of the children, nutritional status, anthropometry, densitometry, as well as blood parameters and markers of inflammation and intoxication were assessed.

Results. Against the background of nutritional support, the following was observed: statistically significant increase in body weight, height, z-score of body weight, mainly due to skeletal muscle mass; an increase in the level of protein and albumin in the blood; a decrease in markers of intoxication and inflammation; an increase in bone mineral density.

Conclusions. The Protein Detoxification Cocktail NUTRIO and Bone Tissue Cocktail (protein) in diet therapy for children with cerebral palsy is effective in correcting their nutritional status, preventing the development of osteopenia, reducing inflammation and intoxication and improving the quality of life.

Currently, more than 70% of hereditary diseases can be considered rare, since their occurrence in the population is only 2%. Regular epidemiological studies show that the frequency of detection of hereditary diseases in the pediatric population is increasing. In connection with this trend, diagnostics is also improving. Congenital adrenal cortical deficiency has been included in neonatal screening since 2006. The presented clinical case demonstrates a combination of hereditary diseases in one patient, in this case, congenital dysfunction of the adrenal cortex and von Willebrand disease. The described clinical example is the result of observation of a child from birth to 15 years of age, with the features of the combined course of two diseases, the selection of drug therapy. The diagnosis of congenital dysfunction of the adrenal cortex was made from birth and confirmed genetically, the child received therapy with mineralocorticoids and glucocorticoids. At the age of 8 months, against the background of an Addisonian crisis, a deficiency of coagulation factor 8 was revealed, in connection with which the drug Factor 8 was added to the therapy, and from the age of 13 – Human coagulation factor VIII human factor von Willebrand. Since the parents did not adhere to medical recommendations, there was no stable compensation for congenital dysfunction of the adrenal cortex, so at the age of 13, the National Medical Research Center of Endocrinology in Moscow adjusted the treatment regimen with hormones. At the age of 15, he was admitted to the Department of Endocrinology of the N.N. Silishcheva Children’s Clinical Hospital with complaints of dizziness, weakness and lethargy. The examination revealed mild anemia, in connection with which iron III hydroxide polymaltose and folic acid were added to the therapy. Against the background of treatment, the patient showed positive dynamics, however, the child requires special monitoring due to the threat of developing a crisis of adrenal insufficiency against the background of stress and infection.

Introduction. Polymorphisms of CYP3A4 and CYP3A5 genes are currently poorly understood as biomarkers of antipsychotic efficacy and safety. There are no studies of these factors in children taking antipsychotics for conduct disorder.

Aim. To identify associations of CYP3A4*22, CYP3A5*3 carriers with the effectiveness and safety of antipsychotics in children with conduct disorder

Materials and methods. Eighty-four children aged 7–12 years hospitalised for conduct disorders were included in the study. All patients received antipsychotics for correction of conduct disorder. Observation was carried out for 14 days, the efficacy of pharmacotherapy was assessed using CGAS, CGI-S, CGI-I scales; safety was evaluated using the UKU SERS scale. All patients were genotyped for CYP3A4*22 (rs35599367) and CYP3A5*3 (rs776746) polymorphisms.

Results. Carriers of the CYP3A5*1 (GA + AA) allele differed in lower severity of oppositional defiant behaviour on the CGI-S scale (3 [3; 4] vs. 4 [3; 4] points; p = 0.040) at the time of inclusion in the study. Also, CYP3A5*1 (GA + AA) was associated with less improvement on the CGI-I scale on day 5 (4 [3; 4] vs. 3 [3; 4] points; p = 0.026). CYP3A4*22 carriers reported a greater score on the UKU Mental Disorders subscale at day 5 (1 [0.25; 2] vs. 0 [0; 1], p = 0.026). Also, CYP3A4*22 carriers had higher scores on the UKU ‘Other HP’ subscale at 14 days (0 [0; 1] vs 0 [0; 0], p = 0.023). In carriers of the active CYP3A5*1 allele (genotypes GA + AA), the SAS scale score on day 5 was on average significantly higher compared to CYP3A5*3 GG carriers (0 [0; 1] vs 0 [0; 0] respectively, p = 0.019).

Conclusion. It was found that carriers of the ‘wild’ allele of CYP3A5*1 responded worse to pharmacotherapy of conduct disorders after 5 days. Significant associations of CYP3A4*22 carriers with worse tolerability of pharmacotherapy were found.

Introduction. Cardiac arrhythmias have not been considered as complications of whooping cough so far. The relevance of the study is conditioned by the need to improve the tactics of management of infants with whooping cough, having the most severe course of the disease, taking into account the identified arrhythmias, as well as the lack of study of this problem.

Aim. To develop an algorithm for the differential diagnosis of cardiac arrhythmias in infants with whooping cough to determine the tactics of their treatment and follow-up, taking into account the presumed pathogenesis of arrhythmias.

Materials and methods. The study included infants (n = 56; 55.4% – male) infants with whooping cough hospitalized in the intensive care unit of Federal Research and Clinical Center for Infectious Diseases. Using functional methods of diagnostics in cardiology and results of 24-hour recording from the intensive care unit monitors we have for the first time compared the heart rhythm with episodes of respiratory rhythm disturbance affected by this disease.

Results. In 83.9% of patients with whooping cough, the ECG parameters were normal. Apnea due to spasmodic coughing was observed in 23 children (41.1%) and was accompanied by desaturation and bradycardia. According to Holter ECG monitoring, all children had sinus bradycardia, atrial rhythm episodes, and extrasystoles. Five patients (8.93%) had sinus arrest with a maximum rhythm pause of 3020 milliseconds. No pathological arrhythmias were detected in the absence of respiratory rhythm disturbance. We suggested a connection between asystole and apnea with parasympathetic hyperactivity, increased sensitivity of the heart to vagal influences, and identified two main groups of patients with whooping cough based on the possible pathogenesis of cardiac arrhythmias.

Conclusions. The identification and differential diagnosis of cardiac arrhythmias in infants with whooping cough is important in determining their treatment and follow-up. Further research is needed to study this issue.

Introduction. A review of studies on the influence of a doctor’s appearance on the level of patient trust showed that in most cases, the doctor’s appearance is of great importance to the patient. The reason for conducting this study was the lack of specific regulatory documents governing the appearance of pediatric health workers, and the small number of domestic studies devoted to the influence of a pediatrician’s external image on the level of children and their parents.

Aim. To study the perceptions, expectations and preferences of children and their parents regarding the appearance of medical professionals.

Material and methods. The subjects of the study are children from 3 to 17 years old (145 people) and their parents aged 20–57 years (175 people). Survey and image methods were applied. A standardized questionnaire was developed for the survey in two versions: for children and for adults. The children’s questionnaire was illustrated with photographs borrowed from the Internet.

Results. The image of the “ideal” doctor from the point of view of the majority of parents surveyed is a doctor of any gender and nationality, with natural hair color, without tattoos, piercings, brightly painted nails, bright makeup and large jewelry. The parents considered the color and material of the medical uniform, as well as the presence of a medical cap, not so important. The image of the “ideal” doctor from the point of view of the majority of the interviewed children: the doctor is a woman in a plain surgical suit, without a medical cap, with natural-colored hair removed, without tattoos on exposed areas of the body, with an acceptable bright manicure.

Conclusion. The conservative style of the doctor is preferred by all respondents. 82% of respondents attach great importance to the appearance of the doctor.

Introduction. Recurrent respiratory infections in childhood are a pressing medical and social problem. Nutrition is important for the functioning and development of the child’s immune system.

Aim. To assess the diet and provision of children with recurrent respiratory infections with vitamins (A, E, D, C, B6, B12, folic acid) and minerals (Zn, Fe, Mg, Ca, P).

Materials and methods. The study included 65 children aged 3 to 8 years, including 50 children with recurrent respiratory infections, 15 children – the control group.

Results. The study among children with recurrent respiratory infections aged 3–8 years revealed a high frequency of deficiency of vitamins D (69%), A (40%), E (35%) and minerals Zn (70%) and Fe (44%). Moreover, the deficiency of vitamins D, A and Zn was most pronounced in children of the main group, compared with the control group (p < 0.05). The results of the survey of parents showed that children with recurrent respiratory infections often (82%) have a low and selective appetite. When assessing the actual nutrition in the structure of the diet, a low frequency of consumption of fish, meat and dairy products, vegetables was noted. Most children of the main group had violations in the diet, such as lack of breakfast, excess carbohydrates and fast food.

Conclusion. The dietary features we have established can negatively affect the vitamin and mineral status of children with recurrent respiratory infections. According to the results of the study of the levels of vitamins and minerals in children with recurrent respiratory diseases, the most frequent violations were found for zinc, vitamin D, A and iron.