The UV spectra of induced autofluorescence in the skin of children and adolescents with type 1 diabetes mellitus were studied and its relationships with sex, age, duration of diabetes, and chronic complications of the disease were evaluated. The data were presented as a 2D array, the fluorescence spectra were normalized, smoothed by the moving average method with a window of 10 nm, and renormalized taking into account the coefficients found. Significant differences in skin fluorescence spectra were found in children of different ages, with increasing the disease duration and depending on the sex of the patients. When developing non-invasive methods of monitoring the state of carbohydrate metabolism, it is necessary to take into account sex and age, disease duration, and the presence of complications of type 1 diabetes mellitus.

The article presents current information on vitamin D. It considers not only well-known recommendations for the administration of vitamin D in paediatric practice, but also the clinical aspects of the new guidelines – Vitamin D for disease prevention: a clinical practice guideline of the Endocrine Society 2024. The document was created using the results of the review of prioritized randomized placebo-controlled trials in general populations, taking into account systematic reviews of electronic databases of patients and their representatives (without an established indication for vitamin D treatment or 25[OH]D testing). The work assessed the effect of “empirical supplementation” defined as vitamin D intake at a dose that exceeds the Dietary Reference Intakes (DRI). This article considers two sections of above guidelines (obstetric and paediatric), alongside with the results of the analytical review, which present some scientific interest, deserve the attention of practicing physicians, confirm the importance of prevention in childhood, as well as indicate the need for further studies on the assessment of vitamin D status using long-term follow-ups and a personalized approach to the groups of children and adolescents that exact very careful attention. Apart from the new guidelines, attention is drawn to the urgency of the Russian National Program for Vitamin D Deficiency in Children and Adolescents of the Russian Federation: Modern Approaches to the Management, which outlines current treatment and preventive strategies for managing vitamin D deficiency in our country using the results of the multicenter study. It is important that Russian paediatricians have an evidence-based, specific and structured document with a brief look at the main strategic issues and operational decisions on the treatment and prevention of vitamin D hypovitaminosis in contrast to ongoing discussions in different countries of the world.

Introduction. The basis of preventive activities in pediatrics is medical examination, based on active dynamic monitoring of sick and healthy children. An important part of medical examination is preventive examinations, which are carried out with the aim of timely detection of deviations in the health status in children and the management of necessary treatment, preventive and health measures. Purpose of the study: based on the results of a dynamic 10-year observation, present the data on the health status of a single cohort of schoolchildren to update the main preventive areas for children’s practice over schooling process. Materials and methods. The study was conducted on the basis of a study of the health status of four hundred thirty seven schoolchildren at different stages of schooling (before entering school, at the end of first grade, during the transition to subject education and at the end of school education), according to a single protocol, on one sample and a single methodology with sequential multiple registration of selected indices of the health status in schoolchildren and annual analysis over a period of 10 years. Results. An analysis of the distribution of those examined cases by membership in health groups clearly demonstrated a decrease in the number of children in health groups I and II and an increase in schoolchildren in health groups III and IV. A dynamic assessment of diagnosed pathological conditions in their compliance with a certain ICD-10 class over a 10-year observation period revealed an increase in the proportion of schoolchildren suffering from diseases of the musculoskeletal system, the organ of vision, cardio- and endocrinopathies. Research limitations.The study has regional (Krasnoyarsk Territory) limitations and concerns 6 to 17 year children. Conclusion. The data obtained established the possibilities of early diagnosis in risk groups, and preventive measures aimed at developing healthy lifestyle skills in schoolchildren and their parents within the framework of priority national projects in Russia.

Recurrent acetonemic syndrome (AS) refers to pathological conditions predominantly in childhood with periodic ketotic crises with repeated (multiple) vomiting, which are caused by an increase in blood ketone bodies over 30 mg/l as a result of incomplete oxidation of fatty acids. and/or breakdown of ketogenic amino acids, as well as other metabolic disorders. The need to discuss the problem is due to the significant frequency of cases, including those requiring hospitalization of patients, the polyetiology of this condition, the complex and largely ambiguous mechanisms of development, the difficulties of differential diagnosis and the uncertainty of the prognosis. The article presents modern literature data on recurrent acetonemic syndrome with cyclic vomiting, drawing attention to the terminological aspects of the problem, discussing the most significant etiological factors and pathogenetic mechanisms of the development of crisis conditions, as well as diagnostic and therapeutic strategies. The article relates to discussion publications and is intended for pediatricians, general practitioners, pediatric endocrinologists, gastroenterologists, and emergency care specialists.

The purpose of the study was dynamic assessment of academic performance and higher mental functions of schoolchildren in elementary school and during transition to subject-based education. The test-based analysis of higher mental functions in schoolchildren with recurrent respiratory infections (RRI) at the stage of primary education has shown an increase in the ratio of schoolchildren with low values according to the offered tests characterising activity and independence in cognitive and social activities (the WAM method), motivation and academic performance (the “Landolt’s correction test”), logical thinking (a test for the development of logical and cognitive processes by Leontiev A.N.) and indicators of attention and performance of the child (the Toulouse-Pieron test). The characteristic behavioural features of children with RRI during the transition to subject-based education are a decrease in communication skills (the “Man in the rain” drawing test) and a high level of anxiety in 70% of the schoolchildren (Spielberger-Khanin test).

Congenital cytomegalovirus infection is the most common congenital infection, occurs in 0.6-5% of newborns worldwide, and is considered the leading non-genetic cause of sensorineural hearing loss in children. Most newborns with a manifest form of the disease develop psychomotor and cognitive disorders, and about half of them develop visual impairment.The purpose of publication: to present a clinical case of severe congenital cytomegalovirus infection in the manifest period with multisystem manifestations (hemorrhagic syndrome, encephalitis, myocarditis, hepatitis, splenomegaly) and follow-up data at the age of 1 year.Conclusion. this clinical example demonstrates diagnostic and therapeutic difficulties in the acute period of the disease and a significant list of diseases resulting from infection. Based on the information presented, the importance and necessity of close attention to the results of a serological study of pregnant women during the entire gestation period with an assessment of the antibody titer to cytomegalovirus infection in dynamics are updated. Specific antiviral therapy, prescribed on the basis of a life-threatening course of a severe manifest form of the disease, made it possible to achieve positive dynamics in the acute period, eradicate the virus and prevent severe outcomes, including death.

Intrauterine hypoxia and asphyxia during childbirth are among the leading causes of neonatal deaths in the structure of «Separate conditions arising in the perinatal period» according to the Ministry of Health of the Russian Federation. The main causes of asphyxia are chronic intrauterine hypoxia and acute fetal hypoxia (most often in the intrapartum period). The article observes current data on antenatal and intrapartum risk factors for the development of asphyxia in newborns. Risk factors (modifiable and non-modifiable) triggering intrauterine hypoxia are discussed, as well as methods of management and prevention of asphyxia to prevent the subsequent development of hypoxic-ischemic encephalopathy. The importance of the problem under consideration is due to the need to find promising «tools» for managing neonatal and infant mortality, as well as preventing the long-term consequences of hypoxic brain damage.

Hereditary motor sensory neuropathies (HMSNs) are the most common degenerative disorders of the peripheral nervous system in children. In this pathology, degenerative lesions of the myelin sheaths and/or axons lead predominantly to distal paralytic amyotrophy, affecting mainly the lower extremities, and are accompanied by areflexia. A clinical case of HMSN type I (Charcot–Marie–Tooth disease, type I A) with an early (up to 1 year) onset of the disease is described. The clinical features of this observation are a very early onset of the disease with a debut before the age of 1 year, the absence of pronounced amyotrophies and characteristic deformities of the feet («hollow foot»). To diagnose this genetic disease, a molecular genetic study was carried out. As a result of DNA sequencing (panel «Neuromuscular diseases»), data were obtained on the presence of a duplication of a segment of chromosome 17 with approximate boundaries of 14005424 – 15162520 bp, covering sections of several genes, including the PMP22 gene. Chromosomal micromatrix targeted analysis revealed a microduplication of the short arm (p) region of chromosome 17 from position 14076430 to position 15441811, covering the 17p12 region. The duplication zone also includes the PMP22 gene, the duplication of which is the cause of Charcot–Marie–Tooth disease, type 1A. At this time, there are no methods of pathogenetic therapy for HMSN, therefore, early diagnosis is of great practical importance, which makes it possible to start rehabilitation measures and prevent the disease in burdened families in a timely manner, based on medical genetic counseling and prenatal DNA diagnostics.

Objective: The analysis of clinical-laboratory peculiarities and the dynamics of glycemia clarifying the terms of blood glucose stabilization during the early neonatal period in children having risk factors of the development of such state.Methods: Open, retrospective , continuous, single-center study was conducted. It was aimed at getting preliminary data that is important for planning further tactics of examining newborns having risk factors on hypoglycemia development (kids having large body weight at birth, children whose mothers have diabetes, newborns having intrauterine growth retardation).The target group of the survey included 522 newborns of both genders in the early neonatal period (from 0 to 7 days of life).Results: There is data on the frequency of first indicated hypoglycemia in newborns having risk factors of the development of such state, clinicallaboratory peculiarities, the dynamics of glycemia during early neonatal period, and also the period of blood glucose stabilization (the age of reaching normoglycemia). Neonatal hypoglycemia was detected in 40.2% of examined children. Laboratory manifestation during the first day of life was noticed 62.9% of cases with the further normalization of glucose level in blood in 24 hours in 73.3% of newborns. Significant reduction of blood glucose was observed in 54.8% of cases requiring hypoglycemia therapy with parenteral prescription of 10% glucose solution. Hypoglycemia jugulated against oral correction of 5% glucoses solution in 40.5 % of cases. 4.8% of children got the level of glucose stabilized after introduction of supplementary feeding.Conclusion: The results of the newborns having risk factors on hypoglycemia development (kids having large body weight at birth, children whose mothers have diabetes, newborns having intrauterine growth retardation) examinations are presented. The peculiarities of neonatal hypoglycemia are: the absence of a typical clinical symptoms among 89% of children. Asymptomatic course of hypoglycemia confirms the importance and necessity of glycemia monitoring in the early neonatal period in order to conduct timely and adequate therapy.

The aim of the research. To identify the features of the onset of type-1 diabetes in children and adolescents residing in the territory of a closed city through studying the following manifestations: medical history characteristics, the gender composition and age of patients, the season of the appearance of the initial symptoms as well as clinical manifestations and indicators of carbohydrate metabolism during the initial visit to an endocrinologist. Material and methods. While carrying out the work, the data of the regional registry of diabetes mellitus for the past ten years were studied as well as the information from outpatient records of children and adolescents with type-1 diabetes living in a closed territorial administrative unit in the Krasnoyarsk Territory. Anamnestic and clinical laboratory data were analysed, a comparative analysis with similar data of children in the Krasnoyarsk Territory and Russia was carried out. Results. The study revealed approximately the same quantity of sick children of both genders. The onset of the disease was more oft en noted in the spring-autumn period, the trigger mechanism was viral infection in the overwhelming number of cases. At the moment of manifestation, the bulk of the children had harmonious physical development, the indicators of carbohydrate metabolism indicated significant decompensation. Conclusion. The article presents features of the course of the type-1 diabetes manifestation period in the studied cohort in relation to the age groups of children and the disease onset seasonality. For a broader understanding of the features of the type-1 diabetes onset in children and adolescents living in a closed city, it is necessary to continue research using data on the child population living in similar conditions.

The aim of the research. To analyse the level of oxytocin in the blood serum of premature and full-term newborns in various types of feeding (according to the Department of Pathology of Newborns and Premature Infants of the Krasnoyarsk Regional Clinical Center for Maternal and Child Health). Material and methods. A single-centre open-label prospective study including 24 preterm infants (at 26 to 36 weeks of gestation) and 14 full-term newborns was carried out. The levels of oxytocin in the blood serum of the examined children on various types of feeding (breastfeeding and artificial) were analysed in dynamics on days 5-6 and 11-12. Results. It has been established that among full-term children, the average values of oxytocin did not have significant differences between the subgroups of breastfeeding and artificial feeding and significantly exceeded the levels of oxytocin in premature infants. The most likely reason for the low level of OT should be considered the higher vulnerability to multiple external influences (examinations and manipulations, temperature, humidity, incubator conditions, care specifics, etc.) that accompany newborns with a small gestational age. Suppression of OT secretion at the stage of the general adaptation syndrome may be due to a pronounced activation of the sympathetic-adrenal system in premature infants in response to the stress situation with an increase in the level of adrenaline and norepinephrine. Analysis of the level of OT in premature infants in various types of feeding depending on body weight at birth in BF and AF showed an inverse relationship between the level of the studied hormone and body weight at birth (the lower the birth weight, the higher the level of OT on the 5th-6th day of postnatal life). Conclusion. In modern literature, data on the physiological effects of oxytocin and the participation of the hormone in the course of a number of pathologies are being actively updated. New findings from recent studies point to the role of oxytocin not only in labour but also in multiple effects on the fetus and newborns. Maternal oxytocin can reach fetal neurons across both the placental and blood-brain barriers. At the same time, the physiological significance of the influence of oxytocin on the brain and the dependence on the type of feeding in newborns are insufficiently studied and are a subject of discussion, but mainly in experimental studies

In world pediatric practice, protracted bacterial bronchitis is the leading cause of chronic cough in preschool-age children. However, in Russia this nosology is not often diagnosed, and in real clinical practice children are observed for doubtful and ambiguous diagnoses. In 2021, the Russian Ministry of Health updated the clinical guidelines “Bronchitis” for pediatric age category with inclusion of protracted bacterial bronchitis in the list of bronchitis variants with designation of disease criteria: wet cough lasting at least 4 weeks, auscultatory symptoms of bronchitis in the absence of signs of chronic lung and bronchial pathology, cough relief after 2 weeks of therapy with an antibacterial drug active against H. influenzae, S. pneumoniae, M. catarrhalis. The article presents a review of the literature on protracted bacterial bronchitis in the historical aspect, and also reflects the criteria of the disease, epidemiology, etiology, pathogenesis, clinical presentation, methods of examination. Particular attention is paid to the treatment and debatable scientific and practical issues in the study of protracted bacterial bronchitis in the light of the clinical guidelines of the Ministry of Health of the Russian Federation. It is emphasized that the etiotropic treatment of protracted bacterial bronchitis is antibacterial therapy. The effectiveness of antibiotics for this disease has been proven in systematic reviews. Therapy is prescribed empirically. The first-line drugs of choice is oral amoxicillin/clavulanic acid. Preference is given to drugs in the form of dispersible tablets. The duration of therapy remains a subject of scientific debate. According to the clinical guidelines of the Russian Federation Ministry of Health, the course of treatment should not exceed 2 weeks, in case of ineffectiveness and/or relapse of protracted bacterial bronchitis, the course of antibiotic therapy may be prolonged to 4–6 weeks.

The hemostasis system is a complex structure that includes the fibrinolysis system, and Yes this is correct coagulation and anticoagulation parts. Due to the multicomponent nature, it becomes relevant to study the key changes in the functioning of signaling pathways, and develop new diagnostic methods and modern drugs with high selectivity. One of the ways to solve this problem is the development of molecular recognition elements capable of blocking one of the hemostasis systems and/or activating another. Aptamers can serve as ligands for targeting specific clinical needs, promising anticoagulants with minor side effects and significant biological activity. Aptamers with several clotting factors and platelet proteins are used for the treatment of thrombosis. This review is focused on the aptamers used for the correction of the hemostasis system, and their structural and functional features. G-rich nucleic acid aptamers, mostly versatile G-quadruplexes, recognize different components of the hemostasis system and are capable of correcting the functioning.

Background. Dysfunctions of hemostasis include conditions occurring both with hemorrhagic syndrome and in the form of thrombosis, the number of which is growing due to the intensification of the therapy. The relevance of the topic is determined by the prevalence of these pathological conditions in newborns, the peculiarities of the hemostasis system in the neonatal period, as well as the difficulties of diagnosis and interpretation of laboratory parameters. Aim. Studying the features of clinical manifestations and genetic markers in newborns with clinical thrombosis on the background of primary thrombophilia. Materials and methods. A retrospective continuous analysis of the case histories of newborn children with thrombophilia who were hospitalized at the Krasnoyarsk Regional Clinical Center for Maternity and Childhood Protection in the period from January 2014 to January 2020 was carried out. Results. The debut of thrombosis in newborns is represented mainly by venous thrombosis of various localizations. Genetic mutations were identified in these patients, which are mainly associated with the work of the folate and methionine cycles (MTHFR: g.677CT, MTHFR: g.1298AC, MTRR: g.66AG and MTR: g.2756AG). A complicated course was noted in the presence in the genome of the combinations MTHFR: g.677CT, MTR: g.2756AG and MTRR: g.66AG and/or homozygous carriage of MTHFR: g.677CT, MTHFR: g.1298AC. The main trigger was peripheral vascular catheterization. Conclusion. A decrease in the levels of natural anticoagulants in newborns with some physiological features of hemostasis deserves close attention of neonatologists and clinicians, requires an integral assessment of the hemostasis system and additional examinations, including the analysis of genetic changes not only in the hemostasis system, but also in folate and methionine cycles.

Introduction. Intrauterine hypoxia and asphyxia during childbirth are the leading causes of infant mortality in the structure of certain conditions arising in the perinatal period.Purpose. To study the involvement of negative events for the fetus in the ante- and intranatal periods in the occurrence of perinatal asphyxia in newborns and identify the main modifiable risk factors that will allow formulating preventive strategies in the development of child hypoxia.Materials and methods. A retrospective assessment of the course of the ante- and intranatal period was carried out according to 50 case histories. Nominal data are presented with indication of absolute and relative values. Sets of quantitative indicators are described by the values of the median (Me) and the lower and upper quartiles (Q1–Q3). The χ2 test was used to compare unrelated samples. Differences were considered statistically significant at p < 0.05. Statistical data processing was carried out using the Microsoft Office 2021 software package.Results: In the group of children with moderate and severe asphyxia at birth, the median gestational age was 36 and 33 weeks. The main ante- and intranatal risk factors for asphyxia of newborns were established in the following percentage: fetoplacental insufficiency – 32%; premature rupture of membranes and medical abortions in history, 30% each; first pregnancy, anemia, obesity, hypertension, smoking, maternal age > 35 years, 18% each; isthmic-cervical insufficiency – 16%; history of antenatal death, threatened miscarriage, vaginitis, 14% each; acute respiratory infections during pregnancy, assisted reproductive technologies (IVF), uterine fibroids – 12% each; oligohydramnios – 8%; polyhydramnios – 6%.Conclusion. The structure of factors that can contribute to the development of asphyxia in newborns is diverse. Understanding the involvement of modifiable risk factors determines the need to build a strategy and tactics to reduce their impact on the development of the pathology under consideration.

12-13 июня 2021 г. КОМПЛЕКСНЫЙ В СОПРОВОЖДЕНИИ ДЕТЕЙ С ИЗБЫТОЧНЫМ ВЕСОМ И ОЖИРЕНИЕМ: ПОЗИЦИЯ СПЕЦИАЛИСТА ПО ПЕДИАТРИИ, ЭНДОКРИНОЛОГИИ И ДИЕТОЛОГИИ, ПРАКТИКУЮЩЕГО НА БАЗЕ ДЕТСКОГО

The precocious puberty is an urgent problem of pediatric endocrinology characterized by clinical and pathogenetic heterogeneity. The appearance of secondary sex characteristics before the age of 8 years in girls and 9 years in boys requires timely diagnosis and the appointment of pathogenetically justified treatment in order to achieve the target indicators of final growth and prevent social deprivation. The developed clinical guidelines are the main working tool of the practitioner. They briefly and structurally present the main information about the epidemiology and modern classification of рrecocious puberty, methods of its diagnosis and treatment based on the principles of evidence-based medicine.

Childhood obesity is an urgent problem of pediatric endocrinology due to the widespread occurrence, the development of metabolic complications and their steady tracking into adulthood. The developed clinical guidelines are the main working tool of the practitioner. They briefly and structurally present the main information about the epidemiology and modern classification of obesity, methods of its diagnosis and treatment based on the principles of evidence-based medicine.