Carbohydrate metabolism plays an enormous role in the vital functions of a child’s body. Glucose is the main energy source for the development of the central nervous system starting from the intrauterine development period. This bibliographical review is devoted to such pathological conditions of newborns as hypo- and hyperglycemias and namely to the mechanisms of the influence of the named metabolic disorders on the central nervous system, the topography of the brain damages and clinical consequences. The analysis revealed that dysglycaemia is more common in children with an aggravated ante- and perinatal history, is combined with damage of brain structures and leads, even in subclinical form, to short- and long-term consequences of neuropsychiatric development, and therefore requires predictive and timely verification and correction taking into account individual characteristics.

The article discusses a clinical case of a combination of achalasia of the cardia complicated by candidal esophagitis, which led to severe dysphagia and a significant decrease in body weight of a patient suffering from hereditary motor and sensory neuropathy - Charcot-Marie-Tooth disease (CMT) type 4 E. The authors highlight the pathogenesis and clinical picture diseases, features of the course of achalasia in a patient with a hereditary neurological disease. In this clinical case, an example of a disease in a 12-year-old girl is considered. A feature of this patient was progressive dysphagia, which ultimately led to the almost complete impossibility of normal food intake and the development of nutritional insufficiency. CMT disease is a heterogeneous group of hereditary disorders affecting the peripheral nervous system, as a result of the progression of the disease, motor and sensory dysfunction develops, such patients are under the supervision of neurologists. The presence of atypical symptoms, including not only progressive muscle weakness and exhaustion, with sensory deficits in the distal parts of the limbs, but also the severity of dysphagia, which from episodic becomes permanent, prevents the passage of not only solid food, but also liquid through the esophagus, the occurrence of esophageal vomiting - should serve as the basis for a complete examination of the patient using methods that allow visualization of the esophagus. In the available domestic literature, no description of the combined course of the pathological conditions indicated in the observation was found; isolated cases are given in the foreign literature, so the description of this clinical observation is relevant for raising awareness and early diagnosis of gastrointestinal smooth muscle dysfunction against the background of polyneuropathy.

Sepsis as the most common infectious complication continues to cause severe and potentially fatal conditions in premature infants. A severe or “comorbid” condition manifests as a combination of pathologies in various organ systems involving multiple pathophysiological mechanisms. In this case, the authors of the article focus on the consequences of prematurity-associated comorbidity, that is, the consequences of those combined diseases to which small-for-gestational age infants with extremely low birth weight are prone and which should be recognized by specialists after the neonatal period, despite the difficulties of early diagnosis. This article presents a clinical case of neonatal sepsis with simultaneous involvement of the respiratory, nervous, digestive, and bone systems and other metabolic manifestations. By analyzing the literature on this topic, the authors show the connection between pathological conditions within comorbidity in a single patient to develop and improve the clinical thinking of physicians regarding a condition such as prematurity-associated comorbidity. The authors hope that this literature review with the description of a clinical case will be valuable in terms of practical applicability for clinicians working in outpatient and inpatient settings as well as for researchers. Key words: sepsis, comorbidity, microbiological monitoring, nosocomial flora, newborn, premature infant

Numerous scientific studies conducted over the past years expand our understanding of the physiological and pathophysiological effects of bilirubin. In this review of the literature, the authors, using the example of Gilbert’s syndrome, as a classic condition occurring with hyperbilirubinemia, discuss the results of clinical and experimental studies demonstrating the protective mechanisms and the protective role of elevated bilirubin concentration in relation to diseases accompanied by metabolic inflammation, oncological diseases, and a number of others. The authors focus on the hormonal function of bilirubin and its potential therapeutic effect discussed in recent scientific works. The purpose of this review of the literature is to expand the understanding of bilirubin from the clinician’s usual in the context of the end product of heme and antioxidant metabolism to a signaling molecule involved in the pathophysiology of many diseases.

The study of cytokine production and its genetic regulation in diseases of various pathogenesis in childhood, which include several mechanisms of inflammation - this is autoimmune against the background of celiac disease, type 1 diabetes and CAI, lymphoproliferative against the background of oncohematological diseases, microbial-inflammatory against the background of chronic pyelonephritis and cystic fibrosis and metabolic against the background of obesity and a decrease in bone mineral density is necessary to expand understanding of pathogenesis, predict variants of the clinical course of diseases (clinical phenotypes) and complications, as well as response to therapy. The literature review is devoted to the analysis and interpretation of data on the effect of vitamin D supply and its genetic regulation on the course of diseases, combined according to the leading pathogenetic mechanism of inflammation into autoimmune, microbial, and lymphoproliferative models.

Objective. To study clinical, anamnestic and laboratory features and to determine risk factors for necrotizing enterocolitis (NEC) in premature infants. Patients and methods. A retrospective analysis of the medical records of 47 premature infants with very low and extremely low birth weight (VLBW/ELBW) and NEC was performed. Newborns were divided into an early-onset group (n = 22) and a lateonset group (n = 25). Groups were compared for perinatal status, clinical symptoms, laboratory findings, and clinical outcomes. As a control group, 50 children without NEC were selected. Risk factors for the development of NEC were determined using multivariate logistic regression analysis. Results. When comparing groups with early- and late-onset NEC, it was noted that in the group with early onset, the number of children with the Apgar score at 1 minute ≤3, stage 3 NEC, surgical treatment, intraventricular hemorrhage ≥ grade 3, apnea and fever or hypothermia was higher. Multivariate logistic regression analysis showed that impaired enteral nutrition tolerance, early sepsis, severe anemia, and hemodynamically significant patent ductus arteriosus were independent risk factors for earlyonset NEC in VLBW/ELBW preterm infants (p < 0.05). Conclusion. Neonates with enteral intolerance, early sepsis, severe anemia, or hemodynamically significant patent ductus arteriosus are at higher risk of developing NEC. Key words: necrotizing enterocolitis, early diagnosis, premature, newborns

Heterotopic pancreas (HPG) is an aberrant anatomical malformation that is most commonly located in the upper gastrointestinal tract. The presence of an aberrant pancreas in most cases is asymptomatic, but in a number of clinical situations it is accompanied by symptoms that require drug therapy, sometimes surgical or endoscopic intervention. Analysis of the clinical manifestations and symptoms of BPH is important in choosing the tactics of treating patients. The difficulty lies in the fact that there is not enough information on BPH in the literature, there are no data from a study in a large cohort of patients with BPH. The purpose of the publication is to review clinical cases, including our own observation, and compare them with a systematic review of the literature in order to draw conclusions about the features of clinical manifestations, pathomorphosis and modern principles of treatment of the disease.

Introduction . The circadian rhythms of the nasociliary system of children with bronchial asthma have not been practically studied. Objective . To evaluate seasonal variations of circa-annual rhythms of the nasociliary system in healthy children and children with year-round bronchial asthma. Materials and methods . 134 children were examined; the main group consisted of 99 children aged 1.5–7 years with mild and moderate bronchial asthma, the control group was represented by 45 healthy children of the same age. The biorhythms of the nasociliary system were studied for three years, 4 times during each year (in October, January, April, July): smears-prints from the nasal mucosa were studied with the calculation of the specific gravity of neutrophils, eosinophils, cylindrical and squamous epithelium as a percentage, the calculation of the cytolysis index of cells and the average destruction index for each type of cell. To study the near-seasonal rhythms, the KOSINOR program and the computer system of intra-laboratory control “VlCC” were used. Results . The presence of seasonal changes in cytological parameters of the nasal mucosa with the maximum values of the average values of the average destruction of the flat, cylindrical epithelium, neutrophils in January and acrophases in January was revealed. If eosinophils on the nasal mucosa were not detected in healthy children, then the presence of this type of cells was noted in children with asthma throughout the year. The period of fluctuations of the average destruction index in the flat epithelium (1.7 months) was 4 times shorter, and in the cylindrical epithelium (21.6 months) – 3.5 times longer in children with asthma compared with healthy children. The periods, amplitude and mesor of the average neutrophil destruction index in children with asthma and healthy peers did not differ significantly. Acrophases and bathyphases of the average index of destruction of the flat, cylindrical epithelium, neutrophils in patients with asthma and healthy children were recorded at different times. Conclusions . The study showed that in children with AD there are no patterns of rhythmological organization in the work of the nasociliary system, characteristic of healthy children.

Numerous epidemiological studies demonstrate that cystic fibrosis, the most common orphan disease in the world, can not occur in isolation, but can be combined with other serious diseases. The most common such combination in all populations is traditionally cystic fibrosis and celiac disease, which, on the one hand, differ in the leading mechanism of inflammation, in cystic fibrosis - chronic microbial inflammation, in celiac disease - autoimmune, on the other hand, these two diseases have a number of common pathogenesis links, in particular, realizing the syndrome of malabsorption, which only complicates the timely diagnosis of these combined pathological conditions. In addition, the authors of the literature review focus on clinical examples of late detection of autoimmune gluten intolerance against the background of cystic fibrosis. They also consider the combination of cystic fibrosis with other severe, disabling diseases (rheumatoid arthritis, phenylketonuria, oncological diseases), which sometimes require a serious change in therapeutic tactics.

Relevance. In the context of the pandemic, schoolchildren were transferred to a distance learning format. Forced isolation, prolonged stay at the computer, physical inactivity, high mental stress had an impact on their health. Of practical interest is the analysis of the frequency of chronic diseases during a pandemic in a cohort of adolescents. Purpose: to estimate the incidence of chronic diseases among adolescents during the COVID-19 pandemic. Materials and methods: a prospective, cohort, non-randomized study of adolescents (15-17 years old), (n=221): 116 girls, 105 boys were examined in accordance with the order of the Ministry of Health of the Russian Federation of August 10, 2017 N 514n “On the Procedure for Conducting Preventive Medical Examinations minors”, a comparison was made between the results of the survey in 2019 and 2021. Results. The frequency of mild protein-energy malnutrition increased from 15.1% (2019) to 19.6% (2021), p=0.02. The number of overweight children decreased in the general group from 14.1% to 10.1%, p=0.04, and in the group of boys from 17.2% to 10.6%, p=0.04. Obesity of the I-II degree in the observation groups did not have significant changes in the number of diagnosed cases, however, in the group of boys in 2021, obesity of the III degree was detected. According to the results of observation against the backdrop of a pandemic in adolescents, the frequency of chronic diseases has significantly increased: pathology of the organ of vision - by 1.4 times, pathology of the musculoskeletal system - by 2.8 times, pathology of the nervous system - by 7 times, gynecological diseases - by 1, 7 times. Conclusion. The overall frequency of detected pathology increased by 1.6 times, due to diseases of the eyes, blood system, musculoskeletal system, endocrine, nervous and reproductive systems. It is likely that physical inactivity and a high level of stress, causing a long-term hormonal imbalance, played a negative role during the period of covid restrictions. At present, thanks to the opening of rehabilitation departments providing specialized medical care within the framework of the compulsory medical insurance system, this category of patients throughout the Russian Federation will have the opportunity for personalized correction of the identified pathology.

Proper treatment of neonatal seizures is crucial for reducing long-term neurological disorders. None of the currently used drugs to treat neonatal seizures has sufficient evidentiary base to make an unambiguous conclusion about its effectiveness. There are no results of large-scale studies that allow choosing an anticonvulsant drug for starting therapy and an alternative to it, in case the drug of choice is ineffective. The review summarizes the data on the effectiveness of anticonvulsants of different pharmacologic classes and considers their benefits, drawbacks, and possible mechanisms of variability in effectiveness when used in newborns. The review also analyzes approaches to the duration of the anticonvulsant therapy and discusses the problems and prospects of clinical trials for increasing the effectiveness and safety of anticonvulsant therapy in newborns.

The description of risk factors for disorders of calcium and phosphorus homeostasis and vitamin D metabolism in preterm infants with very low and extremely low birth weight seems highly relevant today. Patients in this category often develop metabolic bone disease (osteopenia of prematurity). The search for highly specific and early clinical, metabolic, and genetic markers is necessary for timely diagnosis, therapy, and prevention of this disease. This review analyzes the associations of a certain VDR genotype with preterm birth and related complications, particularly with disorders of calcium and phosphorus homeostasis. The data are presented on the effect of VDR genetic variants not only on clinical signs, but also on various links in the pathogenesis of calcium and phosphorus homeostasis disorders in women who gave birth to preterm infants very low and extremely low birth weight and in their children. Key words: vitamin D, C-terminal telopeptides of type I collagen (Betta-Cross-Laps), metabolic bone disease, prematurity, very low birth weight, extremely low birth weight, osteocalcin, parathyroid hormone, vitamin D receptor (VDR) gene, fibroblast growth factor (FGF23)

The aim of the research. To study the features of cardiovascular system disorders in post-covid syndrome (PCS) in children and adolescents after a mild form of coronavirus infection (COVID-19). Material and methods. From 260 children and adolescents after a mild form of COVID-19, a total of 30 patients aged 7–17 years with cardiac manifestations of PCS were selected. Therewith, 32 patients with an uncomplicated form of the disease were selected to form a comparison group. In 3 and 6 months after disease onset, a comprehensive examination of patients was performed with a questionnaire on the subjective scale for MFI-20 assessment asthenia (Multidimensional Fatigue Inventory-20), electrocardiography (ECG), echocardiography; daily monitoring of ECG and blood pressure. The biochemical blood test included assay of creatine phosphokinase-MB (CPK-MB), troponin I and lactate dehydrogenase (LDH). Results. The incidence of PCS with cardiac manifestations amounted to 11.5 %. After 3 months from the disease onset, complaints of pain and discomfort in the chest, palpitations, fatigue, and poor exercise tolerance persisted. Asthenic syndrome was diagnosed in 70 % of patients. The “general asthenia” indicator totalled14 [12; 16] points (p<0.001) and was associated with the age of patients (r=+0.5; p<0.05). Arrhythmic syndrome and conduction disorders were detected in 67% of children. Labile arterial hypertension and hypotension occurred in 23 % of the adolescents. The increase in CPK-MB remained in 17% of the children, LDH – in 10%. In the sixth month after the onset of the disease, there were no significant differences in the results of the examination in the observation groups. However, a decrease in the level of resistance within 6 months was recorded in 43.3% of the schoolchildren with PCS (p<0.001). Conclusion. The data obtained indicate the need for early verification of cardiopathies in children with COVID-19, determination of a set of therapeutic and rehabilitation measures as well as ECG monitoring.

A special form of streptococcal infection is streptococcal toxic shock syndrome (STS), characterized by rapid development of symptoms and high mortality. Patient O., 14 years old, was taken to the infectious diseases department of OGAUZ DBNo. 1 by the SMP team with complaints of shortness of breath, vomiting, loose stools in a state of moderate severity due to intoxication syndrome. Diagnosis upon admission: Acute infectious gastroenteritis of moderate severity. Acute respiratory infections rhinopharyngitis, acute bronchitis, pneumonia (?), DN1. During examination in the UAC, anemia, leukocytosis, acceleration of ESR, in the biochemical blood analysis – an increase in CRP, in the coagulogram — increased INR, APTT, RFMC, decreased PTI, in urine tests – protein, erythrocytes, on the X–ray — bilateral pleural effusion, in the tank. sputum culture — Streptoccocus oralis 10/3 KOE/ml, PCR SARS-CoV-2: negative, blood test for antistreptolysin-O (ASL-O): 800 IU/ml (norm up to 200 IU/ml), blood for sterility 19.05.20: no bacterial microflora growth was detected. After receiving laboratory data, the diagnosis was made: Acute glomerulonephritis?, Аcute intestinal infection. Double-sided hydrothorax. Internal combustion engine. Anemia of the 1st degree. The final diagnosis: Acute post-streptococcal glomerulonephritis with a debut in the form of streptococcal toxic shock syndrome, a period of extensive clinical and laboratory changes, with a decrease in the debut of kidney function in the form of acute renal failure, recovery period. Against the background of the treatment (2 courses of antibiotic therapy (cefotaxime, amoxicillin), infusion therapy, pulse therapy with metipred (5 pulses), double transfusion of freshly frozen plasma, prednisone, lasix, veroshpiron, enap, curantil, heparin, and other accompanying therapy), pronounced positive clinical and laboratory dynamics was noted. She was hospitalized for 43 days, of which 9 days were in the intensive care unit (5 days on a ventilator). On the 44th day, the child was discharged in a satisfactory condition with recommendations under the supervision of a pediatrician, a pediatric nephrologist at the place of residence

Necrotizing enterocolitis (NEC) is one of the most severe diseases in preterm newborns. Despite numerous studies analyzing NEC, many aspects of its etiology, pathogenesis, diagnosis, and treatment are still poorly understood. NEC diagnosis at early stages remains extremely challenging. It is early diagnosis that ensures timely treatment initiation and reduces mortality. There is a clear need for early diagnostic biomarkers of NEC, since it will improve treatment outcomes and expand our understanding of NEC pathogenesis. This literature review summarizes information on laboratory and instrumental diagnostics of NEC, which can facilitate the identification of new biomarkers. Key words: necrotizing enterocolitis, preterm infants, newborn, diagnosis

Objective. To determine the diagnostic value of fatty acid binding protein (I-FABP) in premature infants with necrotizing enterocolitis (NEC). Patients and methods. A prospective study was conducted that included 38 premature infants. The children were divided into 2 groups. Group I included 18 children with NEC, 20 premature infants were included in control group II. Patients underwent clinical and laboratory examination, radiography of the abdominal cavity and measurement of the level of I-FABP in serum. Results. Serum I-FABP levels were significantly higher in children from the main group at all stages of the study: at birth, at the beginning of enteral feeding and at the time of diagnosis of NEC (p < 0,001). There is a correlation between the I-FABP level and the NEC stage. Conclusion. Sequential measurements of serum I-FABP levels can be a useful marker for early diagnosis and prediction of disease severity in NEC. Key words: necrotizing enterocolitis, early diagnosis, biomarker, premature, newborns

The priority of the national health policy is to preserve the life and a high level of quality of life for every premature baby. The clinical focus is on children born with ELBW. Among this category of children, NEC makes the main contribution to the structure of infant mortality. Based on the analysis of the literature, the authors conclude that the study of cell markers that characterize different depths of damage to enterocytes allows: to assess the likelihood of developing NEC in infants with ELMT; conduct early diagnosis of NEC; rule out NEC in neonates with similar symptoms; predict the course of NEC; propose and substantiate personalized approaches to correcting the low supply of 25(OH)D; to analyze the influence of candidate genes on the implementation of NEC, its outcomes, and 25(OH)D metabolism. To assess damage at the level of the enterocyte, the authors selected the intestinal fraction of fatty acid binding protein (I-FABP) for literature analysis. To determine the depth of damage to intercellular junctions of the intestine - the expression of transmembrane (claudin-2, claudin-3, claudin-4, occludin) and cytoplasmic (zonulin) tight junction proteins. Analysis of the results of studies on the expression of fecal calprotectin, lipocalin-2 (LCN2) and eosinophilic neurotoxin, showing the activity of local inflammation, was carried out in order to assess both the risk of NEC and its course. Intestinal damage is associated with impaired 25(OH)D metabolism, and metabolic bone disease in preterm infants with damage to the intestinal barrier up to NEC is recorded ten times more often at the stage of nursing in the NICU. A huge number of studies have shown a decrease in survival, an increase in the risk of severe complications against the background of a low supply of 25(OH)D in the preterm population. The authors analyze the relationship between 25(OH)D availability, taking into account the influence of exogenous and endogenous factors, the nature of damage to the intestinal wall and the implementation of NEC, and focus on the existing preventive and therapeutic approaches to prescribing various doses of vitamin D in preterm infants with NEC.

The approaches to pharmacotherapy of neonatal seizures are always empirical and vary across the globe. There is still no consensus on this issue because of the highly variable efficacy of anticonvulsants traditionally used in newborns. Currently, none of these drugs has sufficient evidence to make an unambiguous conclusion on their efficacy and safety. Therefore, the search for new anticonvulsants for newborns is highly relevant. This review aims to summarize existing literature surrounding anticonvulsants that can potentially be used in newborns. It discusses advantages and disadvantages, as well as their utility in neonatal care. It also summarizes the latest information on neonatal seizures and outlines future directions of research to create new therapeutic strategies for neonatal seizures. Key words: anticonvulsant, treatment, neonatal seizures, newborns, anticonvulsant therapy, anticonvulsants, seizures

Перинатальная патология центральной нервной системы занимает ведущие позиции в структуре детской заболеваемости, что диктует необходимость поиска оптимальных подходов к ранней диагностике гипоксически-ишемических и травматических поражений центральной нервной системы, в том числе с использованием технологий математического моделирования. Цель работы состоит в создании и апробации математической модели дифференциальной диагностики гипоксически-ишемических и травматических поражений нервной системы у детей с использованием метода кариометрии. В исследование были включены 290 доношенных детей первого года жизни: первая группа сравнения включала 120 новорожденных с гипоксически-ишемическим поражением центральной нрвной системы, вторая – 120 новорожденных с ее травматическим поражением, контрольная группа включала 50 здоровых детей. Всем детям в возрасте 1, 3, 6, 9 месяцев и 1 года оценивались физическое и нервно-психическое развитие, двигательные функции по методу Л. Т. Журбы. На первом месяце жизни проводились нейросонография головного мозга, рентгенологическое обследование головы и шейного отдела позвоночника в двух проекциях. В возрасте 1, 3 и 6 месяцев выполнялось морфологическое исследование лимфоцитов (кариометрия). Для создания математической модели дифференциальной диагностики гипоксически-ишемических и травматических поражений нервной системы были взяты 12 показателей нейросонографии и 4 показателя морфологии лимфоцитов периферической капиллярной крови (площадь и периметр ядра, площадь и периметр клетки). В построении диагностической модели использовали самообучающуюся искусственную нейронную сеть, работа которой воспроизведена при помощи созданного программного приложения. Исследование показало, что полученная нами модель проста в использовании, экономит время на постановку диагноза, обладает высокой степенью распознавания: специфичность модели – 89,2%, чувствительность 92%. Perinatal pathology of the Central nervous system (CNS) occupies a leading position in the structure of childhood morbidity, which dictates the need to find optimal approaches to the early diagnosis of hypoxic-ischemic and traumatic CNS lesions, including using mathematical modeling technologies. Objective: to create and test a mathematical model for differential diagnosis of hypoxic-ischemic and traumatic lesions of the nervous system in children using the karyometry method. The study included 290 full-term infants of the first year of life: the first comparison group included 120 newborns with hypoxic – ischemic CNS damage, the second group-120 newborns with traumatic CNS damage, the control group included 50 healthy children. All children aged 1, 3, 6, 9 months and 1 year were evaluated for physical and neuropsychic development, motor functions according to the method of L. T. Zhurba. In the first month of life, neurosonography of the brain, x-ray examination of the head and cervical spine in two projections were performed. Morphological examination of lymphocytes (karyometry) was performed at the ages of 1, 3 and 6 months. To create a mathematical model for differential diagnosis of hypoxic-ischemic and traumatic lesions of the nervous system, 12 indicators of neurosonography and 4 indicators of peripheral capillary blood lymphocyte morphology (area and perimeter of the nucleus, area and perimeter of the cell) were taken. A self-learning artificial neural network was used in the construction of the diagnostic model, the operation of which was reproduced using the created software application. The study showed that the model we obtained is easy to use, saves time for diagnosis, and has a high degree of recognition: the model specificity is 89,2%, and the sensitivity is 92%.

Objective. To determine the incidence of metabolic syndrome components in adolescents born prematurely, depending on the presence of eating disorders. Patients and methods. Group I (n = 58) included adolescents who were born preterm. In group II (n = 65), there were adolescents born full-term. The mean age of participants was 12.3 years. To diagnose metabolic syndrome, the criteria of the International Diabetes Federation (2009) were used. Eating behavior of adolescents was studied using the Dutch Eating Behavior Questionnaire (DEBQ). Results. Overweight was statistically significantly more frequent among adolescents born preterm (36% versus 9% in the comparison group, OR 5.7; 95% CI 2.2–15.2) as well as visceral fat accumulation (36% versus 6%, OR 8.8; 95% CI 2.9–27.2) and stage 1 hypertension (35% versus 2%, OR 33.8; 95% CI 4.4–261.3). Eating disorders in adolescents born prematurely differed by gender: boys were more likely to have eating disorders (emotional and external eating, and their combination, 73%) than girls (36%, OR 4.4; 95% CI 1.5–13.2). Conclusion. The realization of metabolic risk factors in adolescents born preterm is affected not only by eating disorders but also by premature birth itself. Key words: metabolic syndrome, prematurity, eating behavior, adolescents born preterm