Background. In the article, a clinical case of managing a comorbid 74-year-old patient with a history ofischemic heart disease, who was hospitalized in a vascular center due to myocardial infarction, with subsequently developed acute kidney injury against the background of chronic kidney disease is presented.

Objective. The purpose of describing this clinical case is to demonstrate the importance of assessing and considering the significance and modifiability of risk factors for the development of chronic kidney disease, as well as their timely correction. The clinical example illustrates the connection between impaired kidney function and the progression of cardiovascular pathology.

Results. The patient described in the clinical case, despite a long history of hypertension, did not receive ambulatory combined antihypertensive therapy in accordance with actual clinical recommendations, but received a monopreparation from the class of angiotensin-converting enzyme inhibitors without achieving the target blood pressure. In 2012, the patient underwent coronary artery stenting due to unstable angina, after which she received only angiotensin-converting enzyme inhibitors and beta-blockers from the drugs recommended by clinicalguidelines and did not receive antithrombotic and lipid-lowering therapy. The patient was diagnosed with type 2 diabetes mellitus 20 years ago without subsequent control of glucose levels and choice of hypoglycemic therapy. In addition, the patient had modifiable risk factors of cardiovascular diseases and chronic kidney disease: long smoking history, obesity. Upon admission to the hospital, coronary angiography was performed with intravenous administration of iodine-containing contrast medium, according to the results of which stenosis of the right coronary artery was detected, and its stenting was performed with drug-coated stents. During the hospitalization period, there was an increase in the level of creatinine and urea in the blood serum with maximum values of 570.7 μmol/L and 37.1 mmol/L respectively. When these changes were detected, the patient was consulted twice by a nephrologist, and a decision was made to conduct sessions of replacement kidney therapy due to severe azotemia with persistent restoration of kidney function. Upon stabilization of the patient’s condition, she was discharged from the hospital with recommendations to continue the program hemodialysis. However, even upon admission to the hospital, the patient’s creatinine level was 260 μmol/L, which may indicate that chronic kidney disease had been developing for a long time before myocardial infarction and before the administration of X-ray contrast medium during coronary angiography.

Conclusion. Thus, due to the management of the patient at the outpatient stage without compliance with clinical recommendations, lack of correction of modifiable risk factors for chronic kidney disease and cardiovascular pathology, it was not possible to prevent acute kidney injury against the background of chronic kidney disease and myocardial infarction due to late diagnosis of chronic kidney disease.

Objective. To study the effect of immunosuppressive therapy based on a reduction in standard dosages and target values of tacrolimus concentrations on the results of the postoperative period in recipients with a transplanted kidney.

 Materials and methods. The study included 176 kidney recipients who are under outpatient supervision of a nephrologist at the Samara Center for Organ and Tissue Transplantation. In accordance with the purpose and objectives of the study, the recipients were divided into two groups. The first group (control) included 78 patients receiving standard immunosuppressive therapy with calcineurin inhibitors (tacrolimus prolonged action) in combination with standard dosages of mycophenolic acid. The target concentration of tacrolimus in this group was 7-8 ng/ml. The second group (the main one) included 98 patients who received immunosuppressive therapy according to the tacrolimus prolonged action scheme in reduced dosages in combination with mycophenolic acid. The target concentration of tacrolimus in this group was 5-6 ng/ml. Both groups were comparable by gender, age, type and duration of stay on renal replacement therapy, as well as by the main diagnosis that led to chronic kidney disease.

Results. In the course of the study, it was revealed that the minimization of IST leads to an improvement in the long-term results of transplantation. In the late postoperative period, in the group with reduced doses and target values of tacrolimus concentration, the creatinine level remained significantly lower than in the control subgroup. There was also a significant difference in the glomerular filtration rate – in the study group it was significantly higher by 20% than in the control group. A decrease in the target concentrations of tacrolimus in the postoperative period significantly led to a decrease in the frequency of dose-dependent side effects, which subsequently led to a significant improvement in the quality of life of recipients in the control group.

Background. Nutritional status reflects the relationship between consumption and metabolism of nutrients, in the form of objective parameters of the body, its biological media and components.

Results. A review of the literature is presented. To assess nutritional status, available anthropometric parameters that have proven clinical and prognostic significance (body weight, body mass index, etc.) are used. However, these indicators do not fully reflect the component composition of the body, which is critically important for some diseases and conditions. A more informative indicator is the somatic protein pool – the reserve/physiological reserve of amino acids in the body, which provides a pool of skeletal muscle mass. Muscle tissue is the main reserve of amino acids in the body; it becomes a donor of the necessary protein for repair and to meet increased needs in the acute phase of the disease/treatment of patients with hypercatabolism in chronic diseases, injuries, oncopediatrics, etc. An important criterion for assessing nutritional status is the analysis of the risk of sarcopenia. Sarcopenia (decreased muscle mass) and dynapenia (decreased muscle strength) lead to increased risk of disease, mortality, and poor quality of life. Sarcopenia can occur not only in hypercatabolism, but also in obese children, which increases the risks of insulin resistance and metabolic syndrome, while the unfavorable prognosis is greatly enhanced. This indicates the need to use various methods for assessing muscle mass/strength, as well as identifying the risk of developing sarcopenia for adequate differentiated nutritional support and other rehabilitation factors depending on the etiological factors. In the correction of sarcopenia, it is important to take into account its etiopathogenesis. Increasing protein in the daily diet is effective in slowing or preventing muscle catabolism and sarcopenia, including in obese children. However, restoration of skeletal muscle mass and prevention of sarcopenia is not an isolated increase in protein in the diet, but a nutritious diet with a sufficient content of easily digestible protein and the search and possible elimination of the causes of sarcopenia and factors contributing to its aggravation.

Background. The combined pathology of acute respiratory syndrome with gastrointestinal disorder is highly relevant. At this moment there were only a few studies for children on the subjects of the role of respiratory viruses and gastrointestinal pathogens with examination the materials (oropharynx and intestines). That’s why the certain role of particular pathogens in their combination are not completely determined.

Objectives. The purpose of the study was to determine the role of viruses for children with acute respiratory syndrome with gastrointestinal disorder, depending on age and time of year in the period of COVID-pandemic. To evaluate the efficacy of the popular oral probiotics (oil drops dosage form) based on the results of clinical and laboratory tests in the complex therapy of acute respiratory syndrome with gastrointestinal disorder.

Materials and methods. An open prospective observational study was conducted at out-patient’s clinics in the period of COVID-pandemic (04.2022-04.2023) and involved 100 children at the age of 1 month to 18 years. All children were examined on the subject of nucleic acids viruses (multiplex PCR diagnostics) from the oropharynx and feces. Additionally, there were evaluated the efficacy of probiotics. Group 1 (n = 50) received Adiarin® Probio at a dose of 6 drops 1-2 times a day. Group 2 (n = 50) received BioGaia® at a dose of 5 drops 1-2 times a day.

Results. The results of the study showed high efficiency and sensitivity of the conducted probiotic therapy. However, for group 1, there was established greater sensitivity in viral infections management based on the test results of pharyngeal and fecal materials. At the same time, the width (95%) confidence interval of the Group 1 was less than for the Group 2, which means greater statistical reliability for the results interpretation.

Conclusion. The study revealed differences in the seasonal circulation of viruses ib the period of COVID-pandemic, the change of the main pathogens in the oropharynx and feces. From the point of view of clinical practice, it is worth giving preference to probiotics which let treat patients shorter and need less number of additional medicines, as a result it means less drug and economic overloading for patients.

The purpose of the work is the relevance of multilevel influence of deficiency of some nutrients on children's health. Nowadays, "unexplained" hemorrhagic syndrome is quite common in children. At the same time, standard methods of examination of patients do not allow to establish the cause of such pathology. Only a targeted study of the primary hemostasis will reveal dysaggregative thrombocytopathy. Often thrombocytopathies in children have an erased clinical picture and are not diagnosed in time. It is now known that increased platelet aggregation can be regarded as a predictor of the development of pathological disorders in the cardiovascular and other systems. Also increased platelet aggregation can lead to small vessel wall remodeling. The mechanisms of platelet activation are different. They depend on blood plasma composition, functional state and structure of both platelet membranes and functional state of endothelium. The clinical picture of platelet disorders of the hemostasis system is heterogeneous and includes hemorrhagic, ischemic and thrombotic manifestations. Diseases characterized by platelet disorders are not limited to congenital conditions. Often, against the background of any disease develops thrombocytopathies of an acquired nature. Abnormalities of platelet hemostasis are observed in patients of different profiles. The results of research of blood test results, level of serum iron, ferritin, vitamin B12, vitamin D demonstrated the presence of deficiency and insufficiency of microelements in children. The article presents data from laboratory studies reflecting abnormalities in platelet function on the background of vitamin B9, B12, D deficiency and decreased iron reserves. The results indicate the relevance of timely versatile examination of the pediatric population for the diagnosis of deficiencies of the main micronutrients and platelet diseases acquired on this background.

Background. Breast milk is the best nutrition for a baby. If breastfeeding is not possible, the choice of infant formula is of paramount importance. It is important that the composition of the formula reflects the composition and effects of breast milk. A new approach to adapting infant formula is a special technology for fermenting the milk base. It has been demonstrated that as a result of fermentation, partial breakdown of macronutrients occurs, which increases the biological value and facilitates the digestion of the dairy product. Also, during the fermentation of the milk base by bacterial strains, their metabolic products are formed – various metabolically active compounds, postbiotics, which bring health benefits to the host organism.

Objective. To evaluate the scientific evidence presented in the national and international literature on the biological value of fermented foods, the effect of the fermentation process on macronutrients, and the benefits of using a fermented milk base in infant formula.

Results. Analysis of the literature demonstrates high scientific interest in the topic under review. The benefits of fermented foods are due, among other things, to the formation of active metabolites of lactic acid bacteria, postbiotics. The class of postbiotics includes a wide range of compounds, for example: peptides, amino acids, fatty acids, breast milk oligosaccharides, etc. The quality and quantity of postbiotics is determined by the type of bacteria used to ferment the product, as well as the characteristics of the ripening technology. The use of a partially fermented milk base to create infant formula is an innovative approach in adapting breast milk substitutes because it allows us to get closer to the composition and effect of breast milk. One of the postbiotics formed as a result of Lactofidus™ fermentation is the oligosaccharide 3’-galactosyllactose (3’-GL), identical in structure and properties to 3’-GL of breast milk. In vivo and in vitro studies have shown the safety and good tolerability of infant formulas containing 3'-GL, as well as their positive effects on the child’s body at the local and systemic levels, in particular, in combination with prebiotics scGOS/lcFOS (9:1).

Background. Hemorrhagic disease of the newborn is a pathological condition in newborns and children during the first months of life, caused by vitamin K deficiency of dependent blood coagulation factors and manifested by increased bleeding. The incidence of hemorrhagic disease of the newborn varies in different regions of the world, in Russia, according to the literature data, it ranges from 0.25 to 1.7%. Hemorrhagic disease of the newborn is characterized by the development of bleeding and hemorrhage, which negatively affect the function and further development of a number of organs and systems of the child, especially the central nervous system and the gastrointestinal tract. Based on the etiology of the development of the disease described above, HDN is classified into primary (idiopathic) and secondary. Depending on the age of onset, early is distinguished – it manifests itself in the first 24 hours of life, classical – develops on the 2-7th day and late, manifesting itself after 1 week of life (rarely occurs at the age of up to 6 months of life, and some authors indicate the age children under 1 year of age) hemorrhagic disease of the newborn forms. The key priority of neonatologists is to single out from the number of born children, newborns who are at risk for HRD for the purpose of prophylactic administration of vitamin K to them, which will prevent severe undesirable consequences of the disease. A genetic study using logistic regression analysis confirmed a higher risk of intraventricular hemorrhages in newborns with polymorphisms in the VKORC1 genes, which is responsible for the binding of vitamin K1 to cysteine, and thus for a decrease in its concentration in carriers of this gene. Despite the variability in approaches to doses, methods and timing of administration, parenteral administration of vitamin K is the only reliable and preferred way to prevent this disease. In the case of the development of hemorrhagic disease of the newborn, modern approaches to the diagnosis and treatment of this pathology have been developed.

Results. The article presents modern approaches to the prevention, diagnosis and therapy of vitamin K-dependent hemorrhagic disease of the newborn.

Background. Di Giorgi syndrome is grouped under the term chromosome 22q11.2 deletion syndrome (22q11.2DS). It refers to combined primary immunodeficiencies with syndromal manifestations. The phenotype of 22q11.2DS is unusually variable, even among relatives. The symptoms of 22q11.2DS can vary in both severity and type. Some features may be identified at birth, such as cleft palate or congenital heart defect, while others are diagnosed in older children. The article presents data from the current literature, our own data, and a description of our clinical observation of an 8-year-old child with 22q11.2DS.

Objective. The aim of the study is to analyze the clinical features of children with 22q11.2DS on the basis of the parents' database.

Materials and methods. We analyzed a database of 135 children with 22q11.2DS maintained by parents of patients living in the Russian Federation. Mathematical processing of the data was performed in Microsoft Office Excel tables.

Results. In analyzing the clinical features in children with 22q11.2DS, we found that congenital heart malformations and anomalies were the most common (81%), of which conotruncal malformations accounted for the majority (57%), as well as congenital malformations of other organs and systems (33%), gait disorders (21%), thymus hypo-/aplasia (18%), hernias (17%), ophthalmologic pathology (14%), hearing loss (6%), and dental enamel hypoplasia (3.7%). Among other congenital malformations and anomalies, bone (37%), genitourinary (24%), and gastrointestinal (17%) malformations were the most common. There was a high incidence of cleft palate (74%) among bone malformations. Children with 22q11.2DS have a high incidence of ophthalmologic pathology: astigmatism (50%), retinal angiopathy (20%), iris coloboma (5%), and strabismus (15%).

Conclusion. The analysis of the questionnaire database allowed us to identify the main and additional diagnostically significant symptoms of 22q11.2DS, which should be paid attention to during the initial examination of any patient with suspected immunodeficiency. Special diagnostic attention should be paid to immunodeficient children born before 2023. Due to the fact that an increasing number of patients with 22q11.2DS survive to adulthood, the development of algorithms for the prognosis of this disease is important.

Background. 68% of women in Russia use modern methods of preventing unintended pregnancy, which is higher than the world average (57%) and comparable to indicators in developed countries (62%). Researchers also state that in the Russian Federation there is a fairly high rate of use of modern reversible contraceptives (46%), of which 30% are combined oral contraceptives, and intrauterine systems (both copper-containing and levonorgestrel-containing) account for 14%. Other long-acting hormonal releasing systems with various methods of administration are preferred by only 1% of those declaring a desire to use one or another method of hormonal contraception. According to researchers, such a small percentage is associated with a low level of patient awareness of existing advanced methods of contraception - hormonal releasing systems. At the same time, modern research leaves no doubt that the effectiveness of hormonal releasing systems reaches the highest level not only due to the active component, but also due to adherence to the method, eliminating the factor of ″unforced user error″, i.e. due to the lack of need for control over use by the woman.

Results. The article presents arguments in favor of long-acting hormonal contraception, as well as data demonstrating the benefits of using a subcutaneous etonogestrel-containing single-rod implant – highly effective long-acting reversible contraception (LARC). Data are presented on the difficulties that arise when acquiring the practice of installing and removing an implant, and the myths that exist, including in the medical environment, regarding the use of a subcutaneous etonogestrel-containing single-rod implant in various groups of patients.

Background. Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. Adalimumab is the drug of choice for polyarthritis, HLA-B27-associated arthritis, psoriatic arthritis, as well as for the association of arthritis with enthesitis, uveitis. It is of interest to evaluate the efficacy and safety of the genetically engineered biological drug adalimumab in patients with polyarticular rheumatoid factor seronegative juvenile idiopathic arthritis living in the Samara region.

Objective. The aim of the study is to evaluate the efficacy and safety of the genetically engineered biological drug adalimumab in patients with polyarticular seronegative juvenile idiopathic arthritis in the Russian Federation living in the Samara region.

Materials and methods. A retro- and prospective study of 32 (8 boys, 24 girls) patients with polyarticular juvenile idiopathic arthritis, seronegative in the Russian Federation, was carried out. The disease was diagnosed using the ILAR diagnostic criteria. The study was conducted from 2018 to 2022 on the basis of the Samara Regional Clinical Cardiological Dispensary V. P. Polyakov. Analysis of the effectiveness of the therapy was carried out during 6 visits with an interval of 70 days and included an assessment of the functional ability using the CHAQ questionnaire. The indicators of the effectiveness of therapy included: achievement of 50-90% improvement in drug remission of the disease.

Results. Efficacy, safety, and good tolerability of the anticytokine genetically engineered biological drug adalimumab in the treatment of children with polyarticular seronegative juvenile idiopathic arthritis in the Russian Federation have been proven.

Conclusion. The results obtained allow us to conclude that it is reasonable to use adalimumab in patients with refractoriness to immuno-suppressive therapy used for the treatment of juvenile arthritis and consider this approach promising in pediatric rheumatology.

Background. Crohn's disease is a chronic relapsing disease of the gastrointestinal tract of unclear etiology, characterized by transmural, segmental, granulomatous inflammation with the development of local and systemic complications. Currently, early diagnosis of Crohn's disease remains a challenge. In some cases, several years pass from the moment the first symptoms appear to the diagnosis. The recognition of nosology is difficult due to the variety of clinical manifestations due to the involvement of several sections of the gastrointestinal tract in the pathological process at the same time, as well as the addition of extraintestinal manifestations that can be considered as independent nosological forms. One of the most severe skin lesions in Crohn's disease is pyodermagangrenosum – progressive skin necrosis that occurs against the background of systemic pathology and is characterized by the formation of deep ulcers with purulent-necrotic discharge, often associated with diseases of the internal organs.

Objective. The aim of the work is to analyze a diagnostically complex clinical case from real medical practice, when Crohn's disease was detected after the onset of pyodermagangrenosum.

Materials and methods. The article describes a clinical case with Crohn's disease and pyodermagangrenosum, which is an extraintestinal symptom associated with the activity of inflammatory bowel disease. A feature of this case is the onset of the disease with skin lesions against the background of the absence of pathognomonic gastrointestinal symptoms at the onset of the disease. To establish the diagnosis, the patient underwent a comprehensive examination, including laboratory and endoscopic (esophagogastroduodenoscopy, colonoscopy with examination of the terminal ileum), diagnostics, X-ray examination (tubeless enterography) and morphological verification (biopsy of the skin and intestines).

Conclusion. Our clinical observation shows the importance of careful history taking (episodes of recurring abdominal pain, intermittent diarrhea) and the need for timely examination of the intestine in unclear cases. The clinical case also demonstrates the importance of a multidisciplinary approach and continuity between doctors of various specialties in the diagnosis of Crohn's disease. Timely diagnosis of this disease and adequate treatment make it possible to achieve clinical, endoscopic and histological remission, avoid complications, maintain patients' ability to work and improve the quality of life of patients.

Background. Every year, the incidence of ischemic stroke (IS) in the Russian Federation is steadily increasing, often affecting people of employable age. At a young age, the typical causes of stroke are much less common than in the general population. A group of young patients suffered from ischemic stroke (IS) needs to be provided with an extended medical examination involving genotyping methods aimed at the development of personalized measures of primary and secondary prevention.

Objective. To use our software developed for mobile devices, which allows us to calculate the risk of IS taking into account the genetic predisposition of the patient thereby to develop a scheme for the pathology prevention.

Materials and methods. The genetic, clinical and laboratory results of 280 patients examination have been analyzed. Group I consisted of patients with IS (n = 180) aged 22 to 45 years (mean age 33.4 ± 6.57, including 38 patients who experienced recurrent IS. Group II included patients with IS (n = 50) aged 52 to 100 years (mean age 73.4 ± 8.24 years). Group III, the control one, consisted of apparently healthy individuals (n = 50) aged 20 to 43 years (mean age 31.5 ± 5.82 years). All patients underwent computed tomography of the brain, ultrasound examination of the brachiocephalic arteries, and echocardiography. Pharmacogenetic investigations as well as venous blood tests were once performed in all the subjects to reveal a genetic predisposition to thrombophilia.

Results. The programme for the Android operating system has been designed which allows calculating the risk of IS development as well as the risk predicting coefficient of the recurrent IS, on the basis of the DNA tests obtained to reveal a genetic predisposition to so called 'vascular catastrophes' (21.10.2020 the programme 'Prediction and individualized prevention of ischemic stroke' was included in the register of computer programmes, registration No. 2020663019). Applying our product, we have implemented a new approach to the secondary prevention in stroke survivors.

Conclusion. The use of remote monitoring devices can dramatically help in the implementation of tailor-made IS prevention programmes. The genetic passport of the patient will make it possible to carry out an adequate pathogenetic therapy and prevention of acute cerebral circulation disorders.