The article is devoted to the history of the creation of the bone marrow transplantation department and the development of pediatric oncology in the Russian Federation.

The cases of sudden, unexpected child death against the background of relative clinical well-being, i.e., in the absence of apparent reasons take a special place in the structure of infant mortality. Sudden Infant Death Syndrome (SIDS), which is recognized as one of the leading causes of postnatal infant mortality in most developed countries, is the most common cause of unexpected out-ofhospital death of a child. Today SIDS remains one of the most mysterious problems in medicine. The lack of identifiable mechanisms causing SIDS has led to a large number of theories about the mechanisms responsible for death due to this syndrome. Also, sudden unexplained death in childhood (SUDC), which is the unexplained death of children over one- year-old, is currently distinguished among cases of unexpected death. The main clinical features of SUDC include: more common in boys; death occurs at night time, children are found in the early morning in a prone position, face down; children often have convulsions, including febrile ones in the clinical symptom complex during life. Several authors have noted that in some cases, the death due to SUDC resembles Sudden Death in Epilepsy (SUDEP), which is the leading cause of death in epilepsy. To date, it has already been shown that SUDEP is one of the forms of canalopathies characteristic of young children and it is associated with a high risk of sudden death. The mechanisms of thanatogenesis in SUDEP remain unknown. SIDS, SUDC, and SUDEP are a series of fatal syndromes united by multifactorial pathophysiological mechanisms, the causes of which are not fully understood. In fact, these syndromes represent a catastrophic multisystem failure, which is caused by an extremely unfavorable combination of autonomic, respiratory and cardiogenic disorders.

The article provides an overview of special literature, which gives an opportunity to redefine some issues of disability prevention in children with rare (orphan) diseases, which will improve the organizational measures in this area. The prevention of children’s disability is considered as a system of measures to protect the health of the mother and child throughout childhood. The early disability prevention system in children and support for families raising children with disabilities remain among the main priorities of the State social policy of the Russian Federation. The authors describe modern technologies for reducing the genetic burden in the population from the point of view of preventing hereditary and congenital pathologies. They identify the priority areas of disability prevention in children with rare (orphan) diseases, i.e. introduction of prenatal and preimplantation diagnostics; use of the Prenatal Consultation organizational model; conducting a wider screening for congenital and hereditary metabolic diseases with the inclusion of the most common nosological forms of rare (orphan) diseases; finding pathogenetic therapy methods; increasing the knowledge of pediatricians about rare (orphan) diseases.

Despite the long-term study of exudative otitis media, the disease is not well understood. The wide prevalence of exudative otitis media in children and the increase in the incidence during recent decades explain the interest of specialists in the etiology of the disease. The article presents current views on the role of premature birth, gastroesophageal reflux, systemic and local immunodeficiency, disorders in the antioxidant defense system, mucopolysaccharidosis, genetic predisposition, and other factors in otitis media development. Unfortunately, the cause of exudative otitis media cannot always be established, and it requires further experimental and clinical studies in order to develop effective approaches to etiopathogenetically justified, timely and successful treatment of the disease and its implementation in clinical medicine.

Having discussed current definitions and classification of pulmonary hypertension associated with congenital heart defects, the authors consider characteristic clinical symptoms and data of physical examination separately for every subgroup. An increase in pulmonary vascular resistance after radical correction and with small / concomitant defects leads to insufficient filling of the systemic ventricle and the progression of symptoms of low cardiac output , i.e. shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. At late stages, due to transient systemic hypotension the patients feel dizziness, lightheadedness and fainting, as well as signs of congestive right ventricular failure – peripheral edema, liver enlargement, ascites and swelling of the cervical veins. The patients with Eisenmenger syndrome develop cyanosis and signs of systemic complications – polycythemia, deformation of the distal phalanges of the fingers like «drumsticks» and «watch glasses», posture disturbance due to osteoarthropathy and scoliosis, pulmonary and paradoxical systemic thrombosis and embolism, bleeding, symptoms of gout and cholelithiasis, impaired renal function. Even mild pulmonary hypertension after Fontaine’s surgery causes venous hypertension with congestive heart failure, hydrothorax, protein deficiency enteropathy and plastic bronchitis, as well as insufficient blood flow to the systemic ventricle with low cardiac output, shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. All forms of pulmonary hypertension cause pain in the region of the heart, hemoptysis, pulmonary hemorrhage, arrhythmias, and developmental delay in children. The patient shall be examined «from head to toe.» The diagnostic value of auscultation depends on the complexity of the congenital malformation leading to pulmonary hypertension Pulse oximetry should be performed separately on the arms and legs at rest and exercise.

The review summarizes the data of foreign studies on the relationship of the taxonomic diversity of the oral microbiota and changes in its composition with the development of the metabolic syndrome in children with obesity. This information is of scientific interest from the point of view of the search for new targets and possible ways of conservative treatment in order to prevent and treat obesity through maintaining the microflora of the gastrointestinal tract.

The paper considers the role of maternal HLA-DR (Human Leukocyte Antigens-DR) and HLA-G (Human Leukocyte Antigen-G) loci in determining the risk of the formation of sporadic congenital heart defects without chromosomal diseases in the next generation. The HLA-G molecule expressed on trophoblast performs a protective function by blocking killer receptors on natural killer cells (NK cells). At the same time, the maternal alleles of HLA-DRB1 restrict the immune response to allogeneic antigens of the paternal embryo, which may affect the severity of inflammation in the mother-embryo system and through this mechanism induce the formation of heart disease.

Objective: to study the frequency distribution of the combinations of alleles and genotypes of HLA-G 3’UTR and HLA-DRB1 in women with children with sporadic congenital heart defects without chromosomal diseases. Children characteristics and research methods. There were formed 2 groups: Main Group (103 women with children with sporadic congenital heart defects without chromosomal diseases) and Control Group (103 women with conditionally healthy children). Genomic DNA was isolated by phenol-chloroform extraction. Typing of HLA-G 3’UTR 14-bp insertion/deletion was performed by amplification of polymorphic regions of genes by polymerase chain reaction with further electrophoretic detection in polyacrylamide gel 6.0. The frequency analysis of 14 alleles of the HLA-DRB1 gene was performed by real-time polymerase chain reaction. In the course of this work the authors identified predictor and protective combined genotypes.

Conclusion. HLA-DRB1 and HLA-G 3’UTR 14-bp ins/del (rs 1704) make a significant contribution to determining the risk of the formation of sporadic congenital heart defects without chromosomal diseases in the next generation.

Intraventricular hemorrhage remains a serious complication in infants and especially in preterm infants with gestational age up to 27 weeks.

Objective. To assess the risk factors for the development of intraventricular hemorrhage in deeply preterm infants with respiratory distress syndrome.

Materials and methods. We carried out a prospective controlled comparative study. The study included 104 newborns with respiratory distress syndrome with a gestational age of less than 32 weeks and a birth weight of less than 1500 g. Depending on the presence of intraventricular hemorrhage the patients were divided into groups: Group I : 56 preterm infants with intraventricular hemorrhage verified during the observation; Group II: 48 preterm infants without intraventricular hemorrhage

Results. The groups at birth were comparable in terms of weight and height. We identified the risk factors contributing to the development of intraventricular hemorrhage: the absence of antenatal prophylaxis of fetal respiratory distress syndrome (odds ratio (OR) 2.728; 95% CI 1.218–6.109), tracheal intubation in the delivery room (OR 5.714; 95% CI 1.610–20.28), the need for mechanical ventilation on the first day life (OR 2.713; 95% CI 1.154–6.377), forced mechanical ventilation (OR 9.818; 95% CI 1.039–92.86), > 20 manipulations in the first day of life (OR 2.747; 95% CI 1.240–6.089). Also, the authors determined the factors contributing to a decrease in the development of intraventricular hemorrhage: complete antenatal prevention of fetal respiratory distress syndrome (OR 0.35; 95% CI 0.149–0.825), less invasive administration of poractant-alpha at a dosage of 200 mg/kg (OR 0.161; 95% CI 0.033–0.787), ventilation with double control during inspiration (OR 0.159; 95% CI 0.032–0.784), chronic arterial hypertension in the mother during the present pregnancy (OR 0.185; 95% CI 0.037–0.919).

Conclusion. According to the results of the study the authors identified significant risk factors for the development of intraventricular hemorrhage in deeply preterm infants with respiratory distress syndrome.

At the first year of a child’s life begins a gradual transition to verticalization through the sequential development of anti-gravity postures. During the maintain of these poses occurs the active muscle contraction and appears a postural tremor.

Purpose: To identify the features of postural tremor while holding the first antigravity postures in children with motor development disorders and to develop a new method for its diagnosing in children in the first six months of life. During the first year of life, the child gradually moves to verticalization through the sequential development of anti-gravity postures. To maintain these postures, the child actively contracts muscles, causing postural tremor.

Objective. To reveal the features of postural tremor while holding the first antigravity postures in children with motor development disorders and to develop a new diagnostic method in the first six months of life. Children characteristics and research methods. The authors examined 33 children with impaired motor development and 10 children without neurological pathology at the age of 3–5 months. All children underwent neurological examination and study of postural tremor according to our method.

Results. The authors established the features of postural tremor in children with impaired motor development at the age of 3-5 months compared with healthy children, manifested in amplitude increase. The authors presented their own for recording postural tremor in children of the first six months of life at the stage of mastering the first antigravity postures. There are presented the results of the analysis postural tremor in children of 3-5 months with impaired motor development in comparison with healthy children. The article presents a new method for diagnosing impaired motor development in children of the first six months of life using the results of postural tremor research. The high diagnostic value of the developed method is shown

Objective. To evaluate anatomical tracheal parameters of fetus and premature infant for clarifying the permissible and safe depth of insertion and «thin catheter» diameter during minimally invasive administration of surfactant.

Material and methods. Autopsy examination of fetuses and newborns with extremely low birth weight, who died during their first 168 hours of life. The study describes only 26 cases, respiratory system malformations are excluded. The authors measured the distance from the glottis to the bifurcation, the perimeter of the trachea under the ligaments, in the middle part and at the level of the bifurcation with the calculation of the trachea diameter.

Results. The average body weight was 684.6 ± 160.8 g, gestational age varied from 21 to 33 weeks and averaged 25 weeks. The distance from the glottis to the bifurcation was 34.31 ± 5.28 mm, and the diameter in the upper, middle and lower thirds was 3.53 ± 0.64 mm, 3.41 ± 0.63 and 3.69 ± 0.78 mm respectively

Application of the results: registration of these indicators when choosing a catheter for minimally invasive administration of a surfactant and the depth of its introduction into the trachea in newborns with extremely low body weight will help to avoid such complications, as one-lung administration of surfactant and its regurgitation from the trachea during the procedure, thereby increasing the efficiency and safety of the method

Conclusion. The length and diameter of trachea in neonates with extremely low birth weight in the early neonatal period depend on anthropometric values and gestational age at birth, mean length is 34.31±5.28 mm.

Idiopathic nephrotic syndrome is the most common glomerulopathy in children, with a prevalence of approximately 16 per 100,000 of child population worldwide. Any chronic glomerular disease has the same type of development mechanism. Regardless of the damaging factor, after the death of a significant part of the nephrons, there occurs a steady decrease in the glomerular filtration rate, while morphologically we most often determine focal segmental glomerulosclerosis. Studying the causes of focal segmental glomerulosclerosis is an urgent problem in pediatric nephrology. Recently, there has been discussed the role of the cation channels of the potential receptor TRPC of podocytes in the development of proteinuria and focal segmental glomerulosclerosis. The article provides data on the role of TRPC receptors in the pathogenesis of focal segmental glomerulosclerosis. The authors present their our own data demonstrating gene expression of the cationic channels family of the potential receptor TRPC1, TRPC3, TRPC4, TRPC5 and TRPC6 in children with idiopathic nephrotic syndrome, depending on the morphological picture of the disease and sensitivity to steroid therapy.

Objective. To determine clinical course and state of cellular immunity in young children with rotavirus infection.

Children characteristics and research methods. The scientists examined children without infectious pathology and with rotavirus infection (20 patients in each group) using general clinical methods. Rotavirus infection was diagnosed by polymerase chain reaction and immunochromatography. Cellular immunity parameters were determined by flow cytometry.

Results. All the children under observation had a moderate form of the disease with symptoms of exsicosis of the II degree. Changes in the immune status were mainly of a regulatory, adaptive nature, which contributed to the favorable course of rotavirus infection in children, however, the dynamics of the number of cells expressing Toll-like receptors indicates the immunosuppressive properties of rotavirus.

Conclusion. Currently, rotavirus infection in young children is typical with watery diarrhea as the most pronounced and long-lasting clinical symptom. Shifts in immunogram indices in general indicate a deficiency of the cellular link of immunity and a violation of its regulation with simultaneous activation of the immune system in an effort to achieve the eradication of the rotavirus with immunosuppressive properties.

The association of pertussis with various respiratory infections in children is the leading factor determining the complicated course and unfavorable outcome of the disease.

Objective. To analyze clinical and immunological features of the combined course of pertussis and rhinovirus infection.

Children characteristics and research methods. The authors observed 20 patients: 10 (50%) children were under the age of 1 year, 5 (25%) children of 1–3 years old, 3 (15%) children of 4–6 years, 2 (10%) children of 7 -14 years old.

Results. The rhinovirus infection developed mainly at 1-2 weeks of illness in 18 (90%) children. Bronchitis developed in 11 (55%) children, pneumonia – in 4 (20%). Special attention was drawn to the low content of NK cells in 82.4% of patients when assessing the subpopulation composition of lymphocytes. The cytokine profile was characterized by a low level of interferon-gamma and interferon-alpha production – in 94.4 and 61.1% of patients, respectively.

Conclusion. The combination of pertussis and rhinovirus infection in children contributes to the uneven course of the disease, the frequent development of bronchopulmonary complications.

Objective. To describe clinical and laboratory features of COVID-19 in children admitted to the hospital.

Children characteristics and research methods. The authors carried out a retrospective observational study, which included 204 children admitted to the Kazan hospital with a diagnosis of “new coronavirus infection” in the period from May 1 to October 30, 2020.

Results. It was revealed that all hospitalized children had RNA of the SARS-CoV-2 virus (100%) in a smear from the nasopharynx and oropharynx detected by PCR and only 50% of patients were diagnosed with lung tissue lesions by computed tomography. The clinical picture of COVID-19 in children most often corresponds to the classic manifestations of a respiratory viral infection. Risk factors for the development of severe forms, described in adults, were recorded only in isolated cases in our study.

Conclusion. The clinical picture of COVID-19 in children most often corresponds to the classic manifestations of a respiratory viral infection. Risk factors for the development of severe forms, described in adults, were recorded in isolated cases in our study.

The article discusses the possible causes of polycystic posthemorrhagic ventriculomegaly in infants, as well as predictors of this pathological process. When choosing a treatment method the doctor shall take into account not only the etiology of the disease, but also age parameters, concomitant pathology. An important aspect in the approach to the treatment of polycystic posthemorrhagic multilevel hydrocephalus includes the determination of the biomechanical properties of the craniospinal system, especially the compliance of the brain and resorption of cerebrospinal fluid. Particular attention is paid to the timely correction of complications, including the analysis of inflammatory changes markers in the craniospinal system, which serve as predictors of the outcome of cerebrospinal fluid resorption. The authors also noted that the violation of the cerebrospinal fluid outflow from the cranial cavity in children may depend on a large number of «extracerebral» factors. They consider the stages of neurosurgical care to patients in this group on a clinical example. This example and literature data demonstrate that children with age develop changes not only in the craniospinal segment, which is responsible for the transformation of free fluid, but also in the extracranial cavities. Thus, we are able to recommend an integrated approach for the management of newborns with intracranial hemorrhage, complicated by posthemorrhagic hyporesorbent and occlusive hydrocephalus, with the justification of the optimal neurosurgical tactics for managing drainage-dependent patients, the course of the disease in which is accompanied by a multicomponent inflammatory syndrome.

Anomalies in the biliary tract development are a relatively rare pathology in the practice of a pediatric surgeon. A combination of two or more defects of the biliary tract is even less common. The article presents literature data and own clinical observation of a child with a combination of a cyst of the common bile, or hepatic, duct (common bile duct) and an additional bile duct. The anatomical structure of the biliary tract was identified before surgery using magnetic resonance cholangiography. Based on the analysis of literature data and our own observation, we propose an algorithm for diagnostic measures in children with malformations of the biliary tract, which helps to avoid intraoperative injuries.

A group of experts, leading Russian specialists in the field of pediatric endocrinology prepared updated clinical guidelines for the diagnosis and treatment of type 1 diabetes mellitus in children. The article presents the main provisions of clinical guidelines for diagnosis and differential diagnosis, treatment, organization of medical care, specific and acute complications in children with type 1 diabetes mellitus. These clinical recommendations and concerted actions in practical work will allow pediatricians and pediatric endocrinologists to improve the diagnosis, treatment and quality of medical care for children with type 1 diabetes.

Immature brain structures of very premature infants determine the features of the echographic picture at birth, as well as in the neoand postneonatal period. The article presents an analysis of the results of ultrasound studies of the brain (from birth to 18 months of corrected age) in comparison with the clinical and laboratory data of 489 very premature infants born with very low and extremely low body weight. The comparison of neurosonographic data and the results of clinical examination indicate that the echographic picture of the brain structures of very premature infants may differ from that of the mature infants and it does not require medical correction in the absence of neurological manifestations. The most characteristic echographic features in the first years of life are moderate dilatation of the intrathecal spaces, lateral ventricles and cisterns of the brain. Intraventricular hemorrhages in deeply premature infants occur with a high frequency, while degree III intraventricular hemorrhages, as well as periventricular and diffuse leukomalacia, are prognostically unfavorable factors leading to the development of severe neurological defects.

Craniosynostosis is a premature closure of the skull sutures, manifested by deformation of the head requiring surgical treatment. An instrumental examination of the state of the cranial sutures is necessary for the differential diagnosis of craniosynostosis and benign positional deformities of the skull in infants. Traditionally, radiation methods, such as X-ray and computed tomography with three-dimensional reconstruction, are used for this purpose. Over the past two decades, we have accumulated a large amount of data on the high information content of the ultrasound method in assessing the state of the cranial sutures in children. Ultrasound examination is widely available, easy to perform, reproducible; it does not require sedation of the patient and does not carry radiation exposure, which is especially important when examining young children. A negative result of the study makes it possible to exclude the diagnosis of craniosynostosis, while the detection of suture fusion serves as an indication for referring the child to visit a surgeon and further examination. The ultrasound method should be more widely used as a screening method for detecting head deformity and suspicion of craniosynostosis in children of the first year of life.

Currently, in pediatric practice, a special place is occupied by patients with severe allergic lesions of the gastrointestinal tract, which often manifest immediately after the birth of a child. Food protein-induced enteropathy reduces the quality of life of the child and family; it is difficult to diagnose and to treat. We present a clinical case of a patient with food protein-induced enteropathy, complicated by severe B12-deficiency anemia and edema syndrome. In the tactics of patient management, attention is paid to the late diagnosis of the underlying disease and complications, the complexity of prescribing diet therapy and the lack of alertness regarding the diagnosis of malabsorption associated with food intolerance.

Atopic dermatitis is one of the most common dermatological diseases in infants and young children. The disease is characterized by a chronic recurrent course with itching, erythema, lichenification and desquamation. Typical cases are diagnosed on the clinically available diagnostic criteria. However, similar clinical manifestations are found in a number of other diseases that can simulate, accompany or complicate the course of atopic dermatitis. They include inflammatory skin conditions, infections, parasitic diseases, genodermatoses, immunodeficiencies and malignancies, as well as nutritional disorders, graft-versus-host disease, and drug disease. Knowledge of the spectrum of these diseases and their distinctive features is crucial for the correct and timely diagnosis of atopic dermatitis, the selection of the optimal treatment.

The article provides an overview of current data on clinical features, modern diagnostic methods and therapeutic strategies for primary sclerosing cholangitis in children with inflammatory bowel disease. Primary sclerosing cholangitis can be asymptomatic for a long time, acquiring a progressive character with liver cirrhosis. The disease is one of the most important risk factors for the development of cholangiocarcinoma. The article presents a diagnostic algorithm for increased transaminases in the serum of the children with inflammatory bowel disease, with an emphasis on various variants of hepatobiliary pathology including primary sclerosing cholangitis. The widespread application of the pediatric scale SCOPE (Sclerosing Cholangitis Outcomes in Pediatrics index) will allow us to timely diagnose and prescribe treatment for a patient with primary sclerosing cholangitis at an early stage improving the prognosis of the disease.

Hemolytic-uremic syndrome is a serious problem in pediatrics and pediatric nephrology. Hemolytic-uremic syndrome is one of the leading causes of acute kidney injury with potential transformation into terminal chronic kidney disease. Currently, the endothelial dysfunction is strongly associated with changes in the serotonergic system in the pathogenesis of hemolytic-uremic syndrome. There are few studies that have revealed an increase in the blood plasma serotonin concentration in children with hemolytic-uremic syndrome, but its role in the pathogenesis of chronic kidney disease has been insufficiently studied. The progressive course of hemolytic-uremic syndrome, up to the terminal stage of renal failure, requires the search for markers of renal tissue damage as prognostically significant factors for the development of nephrosclerosis, which is of particular importance for optimizing the management of such children.

The article describes the impact of technological devices – gadgets – on the early cognitive and speech development in children. In early childhood, object-activity communication is the leading form of activity; its influence on the speech development is determined by the interaction of the child with the adult (object-oriented actions, the understanding of the purpose of objects, the use of verbal communication). The play activity of preschoolers helps to enhance communication and interaction with peers. Long and uncontrolled interaction with gadgets is connected with insufficient attention of parents and caregivers, which disrupts the process of communication and cognition, in particular, slows down the process of speech development. Foreign pediatric associations recommend eliminating the use of mobile devices in children aged 0–2 years and limiting their use to one hour per day for children aged 3–5 years. Most researchers identify a connection between the long-term use of gadgets by preschoolers, delayed development of endogenus attention, social interaction, distortion of cognitive development, delayed speech development and such positive effects as a high level of exogenus attention development, quick response to external stimuli.

For 10 years the authors analyzed 165 clinical cases of children with atopic dermatitis, which manifested in the first year of life. According to the 10-year follow-up, the authors revealed different outcomes of the disease, depending on the severity of the course, clinical forms of dermatitis and the timing of its onset. Favorable outcome with complete remission was observed in 30.9% of cases and was more common in children with a mild course of atopic dermatitis, acute exudative forms and with the onset of the disease at the age over 6 months. The transition to chronic forms was noted in 14.5% of cases and more often took place in children with a moderate severe course of the disease, erythematous-squamous forms and the onset of the disease at the age of 4–6 months. The development of respiratory forms of allergy (allergic rhinitis and bronchial asthma) was noted in 54.6% of cases and was more often detected in patients with a moderate severe and severe atopic dermatitis, erythematous-squamous forms and exudative forms with lichenification and onset of the disease in the first 3 months of life.

Inhibitor-protected cephalosporins are an important tool against hospital infections caused by extended spectrum β-lactamase producers. At the same time, the relative deficiency of the basic antibiotic in combination with the β-lactamase inhibitor sulbactam (1: 1) may be associated with the risk of therapeutic failure.

Objective. To compare therapeutic effect of various regimens for prescribing inhibitor-protected cephalosporins in children with severe bacterial infection.

Children characteristics and research methods. The authors compared clinical efficacy of cefotaxime / sulbactam, cefoperazone / sulbactam, cefepime / sulbactam at maximum doses and main component-fortified-cefepime / sulbactam. The study included 92 patients aged from 2 months to 12 years with appendicular peritonitis, acute purulent osteomyelitis, complicated hospital infections of the lower respiratory tract, exacerbation of chronic purulent otitis media and chronic urinary tract infections. It was found that inhibitor-protected cephalosporins with a 1: 1 ratio of components more often necessitated a change in therapy. The use of drugs initially containing a basic antibiotic in a high dose (component ratio 2:1) or additionally fortified with a basic antibiotic demonstrated a higher therapeutic efficacy.

Allergen-specific immunotherapy is a pathogenetic method for the treatment of IgE-mediated allergic diseases.

Objective. To evaluate effectiveness of allergen-specific immunotherapy in 471 children with allergic rhinitis and bronchial asthma, as well to analyze the dynamics of the cytokine profile at baseline and 1 year after the start of treatment in 86 children. The research was supported by a grant from the International Scientific Council for Young Scientists of Kazan State Medical University.

Results. Analysis of the data obtained demonstrated that allergen-specific immunotherapy is a highly effective method of treating children with allergic rhinitis and atopic bronchial asthma. The dynamics of the level of the studied cytokines during treatment indirectly indicates an increase in the functional activity of adaptive subpopulations of regulatory T-lymphocytes (T-reg) and B-lymphocytes (B-reg) with a decrease in the activity of Th2 lymphocytes, which may indicate the reconstruction of the immune response and possible disease-modifying effect.

The article describes the experience of diagnostics and treatment of chronic diseases of the veins of the lower extremities in 164 female patients.

Objective. To study the results of diagnosis and treatment of varicose veins in adolescent and adult females.

Patient characteristics and research methods. The authors carried out a prospective study, they analyzed health condition of a group of adolescents and examined this group in 5 years later (the adult part of the study). The authors used clinical examination for diagnostics (disease assessment according to CEAP classification), ultrasound examinations, telephone interviews. Re-examination of the patients showed good long-term results of treatment. There were no re-operations.

Results. According to the data of a five-year follow-up, interventions were performed in 4 (4%) cases in the study group. The proportion of cured patients remained without significant changes.

Conclusion. 1. Phlebology of childhood is characterized by the predominance of uncomplicated forms of varicose veins, with a simultaneous high concern about the disease of the patient and parents. 2. Timely surgical and laser interventions and the prescribed nonsurgical treatment program allow achieving a lasting improvement in the majority of patients with good long-term results. 3. Observation of patients with chronic venous diseases during their transition to the adult age group shows the preservation of good results in the majority of cases.

Multisystem inflammatory syndrome in children associated with COVID-19 is a new disease, information first appeared in April 2020. There were reported cases of hospitalized children with unusual symptoms, manifested primarily by fever and multisystem inflammation from the pediatricians in the UK and other countries during the coronavirus infection pandemic (COVID-19). Some of these children were in critical condition with symptoms of shock and multiple organ failure, and some children had manifestations similar to Kawasaki disease. Despite the fact that COVID-19 in children is relatively easy, some of the «convalescents» after 2–6 weeks develop symptoms largely identical to the manifestations of severe forms of new coronavirus infection in adults. The report presents own clinical cases of multisystem inflammatory syndrome that developed in children after acute form of a new coronavirus infection. The authors discuss the possible clinical variants of the syndrome, its origin and outcomes.

Neuroblastoma is a malignant embryonic tumor of childhood originating from progenitor cells of the sympathetic nervous system. It accounts for 50% of all tumors in the first year of life with a high mortality rate. The development of the tumor process in this nosology is based on genetic disorders that may be associated with segmental breakdowns in chromosomes or a change in their number. The presence of aberrations of 1p and 11q loci serves as a criterion for determining the risk group, and amplification of the NMYC oncogene is an indicator of the aggressiveness of the disease. The main cause of death in neuroblastoma is hepatomegaly as a result of liver metastasis and the development of severe life-threatening complications. The presented case demonstrates a positive outcome of the disease in a newborn child with prognostically unfavorable factors, with early complex treatment, including polychemotherapy, surgical removal of the tumor and bone marrow transplantation.

Objective. To describe a case of atrial flutter in a newborn.

Results. The child in the maternity hospital was found to have a heart rhythm disorder of the type: atrial flutter, constant form. Examination revealed an atrial septal defect and an increase in the level of natriuretic peptide. Subsequently, the child was diagnosed with atrial flutter, constant form. NK 0-1. Open oval window. Treatment with drugs from the group of antiarrhythmics, antioxidants and cardiac glycosides made it possible to avoid pronounced hemodynamic disorders.

Conclusion. The presented case demonstrates that timely diagnosis and medical correction prevent possible complications.

Cardiomyopathy (CMP) is classified into familial and non-familial, which reflects the need to study the genetic basis of the disease. The article describes a clinical case of a familial form of non-compact cardiomyopathy in combination with a dilated form of cardiomyopathy. The article provides data of echocardiographic and MRI studies. The diagnosis was confirmed by genetic research, there was revealed a mutation in the MYH7 gene p.IIe201Thr in a heterozygous state, which is associated with the development of non-compact cardiomyopathy and dilated form of cardiomyopathy.

Exanthema in children accompanies various diseases. Often, polymorphic elements on the skin occur against the background of infectious diseases. Until the second part of the XXth century, children’s classical infectology described only 6 diseases in children characterized by spotty and maculopapular rash. Now there are more than two dozen exanthemes in children with an “infectious origin”. The article considers 2 clinical cases of a relatively rare annular erythema in patients of 1 year 2 months and 11 months associated with chronic herpetic infections. The article presents photo documents of the dynamics of the skin process and laboratory parameters against the background of antiviral therapy.

Objective. To describe the case of diffuse B-cell lymphoma in a child with HIV infection.

Results. A 16-year-old child with HIV infection and generalized lymphadenopathy was diagnosed with diffuse large B-cell lymphoma, stage III, negative central nervous system. The patient was diagnosed on the basis of the results of immunohistochemical study of biopaths of the tissue of the lymph nodes of the submandibular and right axillary region. The study in order to identify markers of active infection caused by the Epstein-Barr virus, cytomegalovirus and human herpesvirus type 6, made it possible to exclude the active phase of diseases that are the main infectious causes of lymphoproliferative syndrome. At the time of hospitalization, the patient was treated with antiretroviral therapy of the combined drug Eviplera. There was no HIV RNA in the blood, the level of CD4 lymphocytes was 0.4·109/l. The BFM chemotherapy regimen was used, which was accompanied by positive dynamics in the form of normalization of body temperature, reduction in the size of lymph nodes.

Conclusion. This case shows that immunohistochemical examination of tissue biopsies of enlarged lymph nodes is of decisive importance in the diagnosis of lymphoma, the main non-infectious cause of lymphoproliferative syndrome in patients with HIV infection.

Idiopathic hemosiderosis of lung tissue is a rare disease with lung damage caused by massive hemorrhage into the lung tissue, with iron-containing hemosiderin deposition. Mainly it occurs in childhood. The article describes the clinical case of idiopathic hemosiderosis of lung tissue in a 3 years 11 months old child. The girl was observed for anemia of unclear etiology, she was repeatedly treated at a hospital and received erythrocyte transfusions. In the future, respiratory syndrome joined the anemic syndrome. It should be noted that a correctly collected anamnesis, timely diagnosis of the disease and the appointment of immunosuppressive therapy make it possible to prevent the rapid progression of the process and the development of complications, including pneumosclerosis.

This article describes a clinical case of a rare Schwachman–Diamond syndrome. It covers the features of the clinical picture of the disease and the laboratory examinations. A multidisciplinary approach for the purpose of early diagnosis, timely initiation of complex treatment, including nutritional therapy, prescription of enzyme preparations and granulocyte colony-stimulating factor, makes it possible to improve the quality of life and prognosis in such patients.

Objective. To describe a long-term follow-up of a patient with a rare genetic disease – syndromic diarrhea, or trichohepatoenteric syndrome.

Results. From the first months of life, the child was diagnosed with incurable diarrhea syndrome, which led to the development of malabsorption syndrome, retardation of physical and psychomotor development. Long-term follow-up revealed the progression of malabsorption syndrome, metabolic and endocrine disorders against the background of increasing morphological changes in the intestine. Only a genetic study of the patient and his parents made it possible to formulate the final diagnosis: «Syndromic diarrhea (trichohepatoenteric syndrome, nucleotide variant g.31929071C> T homozygous in the SKIV2L gene) with crown-like syndrome».

Conclusion. The combination of incurable chronic diarrhea syndrome with facial dysmorphism, skin and hair abnormalities is important for this diagnosis.

Mucopolysaccharidosis (MPS) type III (Sanfilippo syndrome) is a lysosomal storage disease inherited in an autosomal recessive manner, it is characterized by the accumulation of heparan sulfate in the cells of the body, which leads to the development of multiple organ failure. It occurs with a frequency of 1: 70 thousand newborns. There are 4 subtypes of the disease: A, B, C, D. Cognitive and neurological disorders are the earliest symptoms of the disease. Delay (loss) of speech development, regression of acquired skills, hyperreactivity, autistic character traits, mental retardation, sleep disturbance, epilepsy are noted; aggressive behavior is formed, the ability to move is impaired, hepatomegaly appears. There are observed the violations of other organs and systems – the organ of vision, bone, respiratory, cardiovascular systems, ENT organs, dental problems. Clinically, the subtypes of mucopolysaccharidosis III are practically indistinguishable. The average age at diagnosis is 2–6 years. Diagnostic search includes qualitative and quantitative determination of glycosaminoglycans in urine. It should be remembered that with mild variants of the disease, these indicators may be within the normal range. In the future, it is necessary to assess the activity of the corresponding enzymes in leukocytes, plasma or serum, in skin fibroblasts. Genetic testing is then performed to identify the mutation of the corresponding defective gene. Differential diagnosis should be carried out with other types of mucopolysaccharidosis, mucolipidosis, gangliosidosis, multiple sulfatase deficiency, some rheumatoid diseases.

The article highlights the modern problems of nutrition for junior athletes. The authors outline the role of the nutritional plan as an integral part of sports training, as well as a factor in the healthy growth and development of a young athlete. They describe the features of the nutritional status of athletes. The article notes the need for educational programs in the field of balanced nutrition, the use of nutritional supplements and sports products among junior athletes.