Due to its cardinal differences from the adult oncology the children's oncology takes a special place in the national cancer control program. The article analyzes up-to-date problems and suggests their solutions. It contains statistical indicators and a three-level model of medical care for children with cancer. The tasks of the national program stated in the article reflect the author's point of view and are to be further discussed.

The fetal neurobehaviour helps to evaluate the integrativity of the brain function. The distinctive signs of the fetal motor patterns allow us to diagnose developmental disorders at early stages.

The purpose is to review literature related to the study of the integrative activity of the central nervous system, the development and use of modern methods of fetal neuropsychological ultrasound diagnostics - the antenatal test of neurodevelopment after A. Kurjak (Kurjak Antenatal Neurodevelopmental Test - KANET).

The results and conclusion. Using ultrasound of pregnant women at different gestation periods helps us to directly observe and evaluate fetal movements reflecting activity of the maturing central nervous system. The KANET test based on the observation of the fetal motor pattern according to the Prechtl’s method allows us to evaluate the fetal motor activity and predict development and functioning of the central nervous system after birth. The fetal antenatal neuropsychological diagnostics is used for timely detection of the disorders of central nervous system and early implementation of preventive and curative measures for the consequences of CNS damage: neurological, emotional and behavioral disorders in older children.

The article considers the issue of cystic fibrosis – a monogenic autosomal recessive disease. It describes the history of the CFTR gene discovery, the further search for modifier genes to explain the variability of the clinical manifestations of cystic fibrosis. The review discusses problems of connective tissue dysplasia and somatic pathology, which is formed due to the connective tissue dysmorphogenesis in patients with cystic fibrosis; and also the article contains justification for the connection between the formation of severe fibrosis of the lungs and liver and the presence of clinical and genetic markers of connective tissue dysplasia. The author assumes that the clinical and genetic polymorphisms of connective tissue influence the course of cystic fibrosis, formation of bronchiectasis, interstitial pneumofibrosis, cystic fibrosis dysplasia, liver fibrosis and cirrhosis.

Bronchiectasis is characterized by a chronic and abnormal expansion of the respiratory tract; it is caused by impaired clearance of various microorganisms and recurrent infection. The cause of 26%–53% cases of bronchiectasis is unknown, which means that their etiology and pathogenesis are to be studied. This review summarizes current knowledge of epidemiology, pathophysiology, clinical features and management of children with bronchoectatic disease. Recent data allow us to improve our understanding of this heterogeneous disease, for example, the role of the interaction of the immune system and microbiota in the pathogenesis of bronchoectatic disease.

The article presents classification and epidemiology of acute renal injury in newborns and children. According to the international multicentre epidemiological studies, the development of acute renal injury is the main and independent risk factor of newborn mortality. Pediatric nephrology has discussed the evolution of the epidemiology of acute renal injury from primary kidney disease to the secondary one due to another systemic disease or its treatment in children.

The article presents data of experimental and clinical studies devoted to the influence of vitamin D on the origin and progression of the cardiovascular diseases: arterial hypertension, atherosclerosis, coronary heart disease, cardiac rhythm disturbances. The results of the studies indicate that a pronounced deficiency of vitamin D is highly correlated with sudden cardiac death, cardiovascular catastrophes, and overall mortality. The decrease in vitamin D is followed by an increase in total cholesterol, low-density lipoproteins and triglycerides, an increase in the heart rate and the level of systolic blood pressure. The Meta-analyzes of randomized controlled trials demonstrated that the addition of vitamin D to treatment and rehabilitation measures of the patients with cardiovascular disease helps to reduce overall mortality.

Objective. To determine frequency of retinopathy cases in profoundly preterm newborns treated at Gorodkov Ivanovo Scientific Research Institute of Maternity and Childhood in 2016-2017, as well as to evaluate impact of the risk factors on disease severity in the conditions of specialized medical care. We examined 344 profoundly preterm newborns with gestational age of less than 32 weeks. 83 children (24.1%) were diagnosed with retinopathy of preterm newborns. A high quality of medical care was proved by a low percentage of children with severe forms of retinopathy in preterm newborns and absence of blindness in the children. A low gestational age, low Apgar score at the end of the first minute of life and traditional artificial lung ventilation are the most significant factors of severe retinopathy of preterm newborns.

Breast milk is considered to be the best nutrition for infants, as it provides all the nutrients necessary for proper development during the first 6 months of life. Objectives: to study the level of immunoglobulins of the main classes in breast milk and peripheral blood in women with different health status in lactation dynamics. The IgA, sIgA, IgE, IgM, IgG parameters in breast milk and peripheral blood were determined in 3-5 days after delivery and in 1, 3 and 6 months of lactation.

Results. The paper presents data on the level of immunoglobulins in 96 women with different health status. It demonstrates that female milk contains the main classes of immunoglobulins which have reverse dynamics of secretion dependent on the lactation term.

Abstract. Different neuropsychical pathologies are revealed in preterm infants during the subsequent stages of ontogenesis. Violation of the phospholipids composition of cell membranes is one of the essential pathogenetic mechanisms of the psychoneurological pathology.
Objective: to study the content of phospholipid fractions of cord blood lymphocytes in preterm infants at different gestation terms.
The spectrum of phospholipids was determined by thin layer chromatography in 39 healthy full-term newborns and in 65 preterm children (28-36 weeks). We revealed an increased content of sphingomyelin in cord blood lymphocytes in preterm infants. The highest rates were found in children with a gestation term of less than 32 weeks (p = 0.004 compared with full-term infants). Sphingomyelin, unlike other phospholipids of cell membranes, is concentrated mainly in the brain, which indicates its special role for CNS activity.
Recently, there has been formed the idea on the importance of lipid components of fat globule membranes of breast milk for optimal neurological development of children. Given the changes in the sphingomyelin content in the cell membranes of preterm newborns and its important structural and functional role for CNS activity, we believe that special attention should be paid to its content in the formulas for preterm infants among standard components.

The frequency of cholelithiasis, including in children, is constantly growing. For the moment there is no treatment algorithm of this disease after cholecystectomy, which determines the urgency of this problem. The article presents the pathogenetic justification of substitution therapy with enzyme drugs, sparing therapy with antacids, hepatoprotectors and cholespasmolytics, as well as probiotics in the early postoperative period in children with cholelithiasis who underwent cholecystectomy. There are data proving fallacy of prescribing ursodeoxycholic acid during this period. Particular attention is paid to the mechanism of action of drugs and the possibility of their use for the prevention and / or treatment of postcholecystectomy syndrome in children of different age groups. We describe our personal experience in the use of drugs in children with cholelithiasis, who underwent cholecystectomy.

Purpose of the study: to determine main parameters of the heart rhythm in children with type 1 diabetes, depending on the night level of glycemia. We performed bifunctional monitoring of glycemia and ECG in 50 patients with type 1 diabetes from 10 to 17 years. Depending on the glycemic parameters summarized in all patients we divided 3 groups: Group 1 (n=11) – sites of hypoglycemia (≤3.9 mmol/l), Group 2 (n=35) – optimal level of glycemia (4.0-9.0 mmol/l) and Group 3 (n=45) – sites of hyperglycemia (> 9 mmol/l). According to the results of glycemic monitoring, 96% of children do not have glycemic target marks at night and 20% of patients have asymptomatic night hypoglycemia. When analyzing the parameters of Holter ECG monitoring, the greatest value of heart rate, duration of the QTc interval, as well as atrial ectopic activity were often recorded during periods of hypoglycemia. Hyperglycemia also causes pathologic elongation of the ventricular electric systole and a greater number of ventricular extrasystoles. Thus, night hypo- and hyperglycemia in children and adolescents with type 1 diabetes leads to an extension of the QTc interval, an increase in the number of extrasystoles with the highest severity and frequency in hypoglycemia. We noted a high level of functioning of the sympathetic department of the autonomic nervous system and the central contour of heart rhythm regulation in children and adolescents with type 1 diabetes mellitus in hypoglycemia.

Purpose. To identify the characteristics of the amino acid profile in children with various disturbances of the motor activity of the gastrointestinal tract. We examined 100 patients treated at the diagnostic department of the Children’s Republican Clinical Hospital of Tatarstan. The main group consisted of 80 patients, 40 of whom were diagnosed with functional constipation, and 40 patients were diagnosed with endoscopically verified gastroesophageal reflux disease. The control group consisted of 20 somatically healthy children with no signs of pathology of the gastrointestinal tract. We determined the content of amino acids in dry blood stains using liquid tandem chromatography-mass spectrometry on Agilent Technologies 6410 Triple Quad LC/MC (USA). We performed statistical analysis of the results using GraphPad InStat (version 3.05; 2000) and Statistica 10.0.

Results. We found differences in the amino acid composition of blood of the patients with impaired motor activity of the digestive tract as compared to healthy children. The level of leucine was increased to the greatest extent in the study group. The revealed changes correlated with the severity of the disease.

Conclusions. Disorders in the motor activity of the digestive organs are accompanied by changes in the amino acid profile that are associated with the severity of functional constipation and gastroesophageal reflux disease. Some amino acids, in particular leucine and arginine, can be used to diagnose secondary mitochondrial dysfunction in pediatric gastroenterology along with conventional biochemical and chromatomass-spectrometric markers.

In order to study the peculiarities of children’s adaptation to the impact of moderate chronic stress we examined 177 healthy children from 10 to 12 years, who began to study in various institutions. We studied the volume of the educational load, academic performance, parameters of vegetative homeostasis, personal characteristics and the style of adaptive behavior. The cardiovascular system was evaluated by daily ECG monitoring and blood pressure. The duration of follow-up was 2.5 years. Adaptation of children, regardless of the type of educational institution, was accompanied by a tension of the vegetative regulation mechanisms. The children with formed adaptive personal resources and a balanced state of the autonomic nervous system adapted most successfully. At the beginning of adaptation we observed increase in blood pressure in 25.4% of children, and arterial hypertension in 3.3% of children. In 24 months there was found sinus bradycardia in 8.5% of children. Regardless of the type of institution, the depletion of adaptive capacity was associated with abnormalities in the state of the children’s health, low training effectiveness and poor tolerance of educational load. Thus, we believe that personal characteristics, styles of adaptive behavior and the state of vegetative regulation mechanisms should be taken into account when preparing recommendations for the training and organization of medical and psychological-pedagogical support for students.

Objective: to determine the role of the indicators of lipid peroxidation and antioxidant protection in the pathogenesis of perinatal hypoxia in premature newborns.

Methods. The authors studied two groups of premature newborns: 24 infants born at 34-36 weeks of gestation (late premature children), and 7 children with gestation age of 32-34 weeks (moderately premature children). The authors assessed the levels of ceruloplasmin, total antioxidant activity and malonic dialdehyde in the blood serum.

Results. Comparison of the studied indicators revealed higher levels of antioxidant protection and malonic dialdehyde in late premature newborns, which can be associated with greater activity of metabolic processes and resistance to oxidative stress in this group.

The authors studied such factors as the sex of the child and the degree of asphyxia at birth and their impact on the values of lipid peroxidation and antioxidant activity. They found that the male infants had a significant increase in the level of ceruloplasmin and antioxidant activity in comparison with the female infants. The children with moderate asphyxia at birth had the highest values of malonic dialdehyde and anti-oxidative protection.

Conclusion. The processes of lipoperoxidation and antioxidant protection are optimally balanced in late premature infants, thus, we believe that they better adapted in conditions of hypoxia.

Objective. To study the effect of virulence and antibiotic sensitivity of K. pneumoniae on the course and outcome of localized and generalized forms of infection in newborns.
The authors studied 25 samples of K. pneumoniae isolated from the blood (12 isolates) and feces (13 isolates) of the children with various forms of neonatal infection. Group 1 consisted of 12 children with bacteriologically proven neonatal sepsis, K. pneumoniae was isolated of their blood. Group 2 included 13 children with localized bacterial infection in the form of pneumonia, K. pneumoniae was isolated from their feces. The PCR method was used to determine the virulence factors of the isolates of K. pneumoniae-rmpA, aerobactin and colibactin. The sensitivity of K. pneumoniae to antibiotics was determined by the Kirby-Bauer method. The double disk method was used to determine the ability of K. pneumoniae to produce extended-spectrum β-lactamases (ESBL).
Results. In Group 1 the isolates of K. pneumoniae produced ESBL in 8 children out of 12. The bacteria were sensitive to meropenem, amikacin and ciprofloxacin in 4 cases. One child demonstrated resistance to meropenem. The remaining 4 isolates were sensitive to the third-generation cephalosporins protected by aminopenicillins, amikacin, meropenem and ciprofloxacin. The rmpA gene was determined in the K. pneumoniae isolates in 6 children. The “string-test” of these colonies of K. pneumoniae in all cases gave a positive result. The genes of siderophores, aerobactin and colibactin were found in 3 isolates. Aerobactin and colibactin produced only rmpA-bearing strains. 3 isolates (23%) of K. pneumoniae produced ESBL in Group 2. In 8 out of 13 cases there was rmpA–gene and genes of aerobactin and colibactin in 11 and 7 cases accordingly. The “string-test” was positive in 8 cases, and there were only rmpA-positive bacteria. Siderophores were detected both in rmpA-positive and rmpA-negative isolates. The microbes produced BLBR and were rmpA-positive in 2 children. In one case, the isolates had neither the characteristic virulence factors, nor BLBR.
Conclusion. The risk of developing localized and generalized forms of neonatal klebsiella infection is largely determined by microbiological features of the microorganism, its resistance and virulence. We observed clinical variants of the disease caused by K. pneumoniae, which simultaneously had two properties: high aggressiveness and resistance to antibiotic therapy.

Objective: to improve the methods of treatment of community-acquired pneumonia in children with recurrent respiratory diseases, taking into account the state of mucosal immunity. The authors examined 65 children from 3 to 7 years with community-acquired pneumonia and recurrent respiratory diseases. The main group consisted of 25 children receiving Fenspiride (4 mg / kg for 7 days) and immunomodulator Azoximer bromide (sublingually, 6 mg tablets twice a day for 10 days) in the complex anti-inflammatory therapy. The comparison group included 20 children receiving only Fenspiride (4 mg / kg for 7 days) as part of complex therapy. Control group included 20 children receiving only traditional therapy. We determined the levels of pro-inflammatory (IL-8, IL-1, α-factor tumor necrosis) and regulatory cytokines (IL-10 and γ-interferon) in induced expectoration by an immune enzyme analysis. We found that due to the therapy the imbalance of both pro-inflammatory and regulatory cytokines was leveled in the main group against a background of positive clinical efficacy that is manifested by reduction of the febrile period, faster relief of respiratory syndrome and local pulmonary changes, and 2 times reduction in the frequency of recurrent respiratory diseases.

Objective: to evaluate the diagnostic significance of measuring the thickness of the intima-media complex (IMC) in children with autosomal dominant familial hypercholesterolemia for early diagnostics and prompt treatment of atherosclerosis.
Materials and methods. The study included 109 children – 64 children with familial hypercholesterolemia and 45 healthy children. Both groups were divided into 2 subgroups according to the age – from 3 to 8 years and from 9 to 18 years. We measured the intimamedia thickness (IMT) of the common carotid artery in all the children. To evaluate IMT we used an ultrasound scanner HD11XE (Philips, USA) with a linear (3–12MHz) sensor IMT.
Results. We found a statistically significant difference (p=0.012) of the IMT of the common carotid artery in children with familial hypercholesterolemia (0.61 ± 0.02 mm) in comparison with the control group (0.49±0.02 mm), starting from the age of 9 years. There were no sex differences of IMT in patients older than 9 years.
Conclusion. We found that children with familial hypercholesterolemia have higher values of IMT already from the age of 9 as compared with healthy children; the increase in TCIM is an additional criterion for the early diagnostics of atherosclerosis and evaluation of cardiovascular risk. These results emphasize the relevance of lipid-lowering therapy for patients with familial hypercholesterolemia in childhood, before the first signs of atherosclerosis appear.

Objective: to evaluate the condition of the pancreas in children with cystic fibrosis using complex ultrasound.

We examined 80 children from 3 to 18 years: 50 children with mixed cystic fibrosis and 30 conditionally healthy children. The complex ultrasound consisted of gray scale US, color dopplerography, energy dopplerography and pulse-wave dopplerography.

Results. A complex study of such parameters as splenic vein diameter, volume and linear blood flow with postprandial response help to obtain more complete picture of pancreatic damage in children with cystic fibrosis. This highly sensitive and specific method with nutritional load has no contraindications, it is non-invasive, readily available, and it does not require large costs and opens up wide prospects in diagnosing the structural condition of the pancreas in children with cystic fibrosis.

Objective: to evaluate the clinical and epidemiological features of chronic adenoiditis and adenotonzillitis in children with nasopharyngeal carriage of the Epstein-Barr virus, cytomegalovirus and human herpesvirus 6. We conducted open comparative study, which involved 200 patients undergoing conservative and surgical treatment (adenotomy, adenotonzillotomy) at the ENT department of the Medical Center “Mart” (Kazan).

Results. We obtained positive result of herpesvirus test in 65% (95% CI 58.4–71.6), 130/200 cases. There were registered 92 cases of the Epstein-Barr virus infection (70.8% [95% CI 63–78.6]), 46 – cytomegalovirus (35.4% [95% CI 27.2–43.6]) and 56 – human herpesvirus 6 (43.1% [95% CI 34.6–51.6]). 100% of patients with herpesvirus 6 had recurrent respiratory diseases, an increase in the cervical group of lymph nodes; 36.4% (95% CI 16.4–56.4%), 8/22 of these patients did not need operative treatment.  Patients with the Epstein-Barr virus, cytomegalovirus and their combination more often complained of hypertrophy of the pharyngeal tonsil, recurrent otitis and a decrease in hearing, p<0.01. Patients with Epstein-Barr virus infection (11.9% [95% CI 2.2–21.6]), 5/42) were more often dissatisfied with the treatment results.

Conclusion: effective options of treatment and prevention of hypertrophy of the lymphoid tissue of the pharyngeal ring should include etiotropic therapy of herpesvirus infections.

This is the first report of a clinical case of subcutaneous adiponecrosis of a newborn with acute early neonatal period from a mother with risk factors in the form of diabetes, hypoglycemia during pregnancy, chronic infections, emergency surgical delivery due to acute fetal hypoxia. The article contains algorithm of the differential diagnostics of diabetic embryo-fetopathy and metabolic disorders in a newborn from a mother with diabetes.

Intestinal yersiniosis is a sporadic disease for the Republic of Bulgaria, and mixed forms of infection with other pathogens are extremely rare, they occur in severe form with progressive dehydration and development of different complications. Purpose: to present a clinical case of mixed infection – enteritis – caused by Yersinia enterocolitica and Astrovirus, in a 1-year old child hospitalized with acute diarrheal syndrome of infectious genesis to the Varna Hospital for Infectious Diseases. The study used clinico-epidemiological, laboratory data and information from medical documentation. Results. The patient was diagnosed with enteritis caused by Yersinia enterocolitica and Astrovirus, a mixed form of infection. As a result of the treatment, the child quickly recovered, and the control fecal samples did not give rise to pathogenic bacteria and did not show the presence of viral antigens. Conclusion: mixed infections caused by intestinal pathogens represent an actual problem and they are an object of scientific research. We described the clinical case of a mixed infection with a normal course in a medium-severe form, without complications, and it resulted in complete recovery of the patient after a 5-day course of antibiotic therapy.

Purpose: to evaluate effectiveness of Oscillococcinum in the treatment of acute respiratory viral infections (ARVI) in children of different age groups.

We observed 140 children from 1 to 14 years with mild to moderate-severe acute respiratory viral infection. 80 children (Group 1) were treated with Oscillococcinum, 60 children (Group 2) were treated symptomatically. Group 1 was divided into Subgroup 1А (40 children of 1–5 years old) and Subgroup 1B (40 children >5–14 years old). Group 2 was divided into Subgroup 2А (30 children of 1–5 years old) and Subgroup 2B (30 children >5–14 years old). The follow-up period was 7–10 days. Clinical efficacy was assessed by the severity of ARVI symptoms in scores from 0 to 2. All adverse events of the therapy were recorded. Also we evaluated disappearance of ARVI symptoms within 48 hours after the beginning of the therapy.

Results: Oscillococcinum reduced the duration of ARVI in children of different age groups. During the first two days the symptoms of acute respiratory viral infection disappeared in 13 (16.3%) children of Group 1 receiving oscilococcinum and in 4 (6.7%) patients from Group 2 (OR = 2.7, 95 % CI 0.8–8.8, p <0.001).

Conclusion: Oscillococcinum is an effective and safe drug to treat ARVI in children of different age groups.

The article analyzes a regulatory base for admission of children and adolescents to physical culture, sports, conformity with the standards of preparedness for work and defense, recommended tests for determining tolerance to physical activity. The article reveals issues of admission to various types of physical activity: ways to assess tolerance to physical exercises, the right for admission to various types of physical activity.

Abstract. Proper nutrition is the key factor of growth and development of the child. Early artificial feeding and irrational introduction of complementary food is one of the problems of infants leading to development of functional disorders of the gastrointestinal tract, impaired immunological protection and manifestation of atopic diseases. We described the effects of the main pre-biotic components of functional nutrition in children of early age. Also we demonstrated the importance of prebiotics for the formation of intestinal microflora, including the development of local mucosal homeostasis due to immunomodulatory action.

It is quite a challenge to evaluate the nutritional status of children with infantile cerebral palsy. The violation of the growth and development of this category of children has a polyfactorial genesis, and the approach to nutritional correction must be comprehensive. Only a complex solution with a verified algorithm for evaluation of the anthropometric indicators, knowledge of the features of physical development, actual nutrition, body composition and laboratory markers in children with cerebral palsy can reflect the real patient’s nutritional status and help to carry out the necessary correction.

The article discusses modern data on medical care for children with phenylketonuria. There are the directives and standards for neonatal screening, primary and specialized medical care for patients with phenylketonuria. The authors consider medical and social model of providing specialized nutrition and the indicators of medical care quality for children with phenylketonuria.

Tuberous sclerosis is a multisystem genetic disorder caused by unregulated activation of a mammalian rapamycin target (mTOR), leading to the growth of hamartomas in various organs. Skin lesions associated with tuberous sclerosis often develop at early age, which is an important diagnostic criterion. It is the pediatricians who can contribute to an early diagnostic process, to develop treatment strategy and create follow-up care for patients. Possible treatment options for skin lesions due tuberous sclerosis include pharmacological (e.g. local or systemic mTOR inhibitors) and non-pharmacological (surgical removal, laser therapy) methods.

An effective system of children’s and youth sports, selection and preparation of a the reserve for national teams predetermines the success of athletes in international competitions. Therefore, the development of medical and biological support of children’s sports is an important part of sports medicine. It is proved that when building a training process for different ages, it is necessary to take into account the adaptation processes, as well as the energy supply of the child’s organism. Based on numerous studies, we can conclude that mitochondria play the most important role in the process of energy supply, as well as carnitine, a substance that is a carrier of long chain fatty acids into the cytosol, an activator of beta oxidation and the Krebs cycle. Timely detection of factors adversely affecting and limiting physical activity at the macro level (overtraining, incompatibility of physical activity with the body capabilities) and at the micro level (carnitine deficiency), the ability to eliminate these factors and the adequate use of correction tools help to achieve high results in sports and preserve the health of the athlete.

The authors, in the form of discussion, express their own opinion on one of the current problems of the concept of “scientific school”. The change in the old ideas about the characteristic features of the scientific school is now logical and reflects the turbulent processes in modern pediatrics.

The scientific school with its own peculiarities, is headed by a leader (or leaders), a well-known scientist, a highly educated professional with encyclopedic knowledge, to form a fundamentally new scientific and practical direction in medicine and pediatrics. Training of highly qualified specialists proficient in modern research methods is an inseparable part of the scientific school. In our country the formation and development of scientific schools on topical issues in pediatric science and practice are associated with the names of outstanding scientists – coryphaeus of domestic pediatrics. The article focuses on the experience of Moscow scientific schools that have their own history, stages of development, undoubted influence on pediatrics both in our country and abroad.

The article describes medical, scientific, pedagogical activity of the outstanding Kazan scientist, Honored Scientist of the RSFSR, Yefim Moiseevich Lepsky. Ye.M. Lepsky made a great contribution both to the development of the Kazan pediatric school and to the development of Russian pediatrics in general.

The article presents modern points of view on the assessment of сognitive functions of the children and adolescents with epilepsy who take antiepileptic drugs (AEDs). The authors compare various AEDs which are used in modern clinical practice in Russia and abroad, their impact on cognitive functions, discuss the psychometric tools which are used in Russia to assess the dynamics of cognitive functions in this group of patients.

Idiopathic pulmonary arterial hypertension is a progressive, incurable disease of unclear etiology; it is characterized by intensive remodeling of the pulmonary vascular bed with a predominant lesion of the distal pulmonary arteries and arterioles. A diagnostic criterion of the disease is an increase in the average pressure in the pulmonary artery more than 25 mm Hg. Arterial hypertension is difficult to diagnose, especially at the early stages due to non-specific clinical symptoms. The article describes the clinical case of diagnostics and treatment of a child with primary pulmonary hypertension of high degree.

Cystic dysplasia is a heterogeneous group of diseases, with 12–15% share in the structure of congenital kidney anomalies and 8–10% share in the structure of the causes of chronic renal failure in children. The article presents the results of observation of patients with polycystic kidney disease. To study the clinical features of the course of various forms of cystic dysplasia in children we analyzed the histories of children with autosomal recessive and autosomal dominant polycystic kidney disease. We revealed clinical, laboratory and instrumental features of the course of various types of cystic renal dysplasia.

We described a clinical case of Lafora disease, a rare genetically determined disease (mutations in the EPM2Aor EPM2Bgenes) from the group of progressive myoclonus-epilepsy forms with an autosomal recessive type of inheritance. The symptoms of the beginning of Laphora disease are similar to the symptoms of idiopathic generalized epilepsy. The absence of the effect of treatment with antiepileptic drugs, the manifestation of new types of seizures, the progression of cognitive impairments allowed us to exclude idiopathic generalized epilepsy with isolated convulsive seizures and juvenile myoclonic epilepsy. The case described in the article indicates the need for the neurologist to be aware of rare genetic syndromes and alertness to progressing myoclonus-epilepsies.

Neonatal thyrotoxicosis is 1% of all cases of thyrotoxicosis in children and adolescents and it is mostly determined by the mother’s Graves’ disease. The most dangerous manifestations of neonatal thyrotoxicosis are intrauterine growth retardation, tachycardia, and heart rhythm disturbances. Timely diagnostics and beginning of treatment are of great importance due to the high risk of fatal cardiac disruption in the acute phase of the disease and its serious consequences for the body. The article presents a clinical case of a patient with neonatal thyrotoxicosis diagnosed only at the age of 1 month, despite the mother’s burdened anamnesis; it contains a plan for diagnostic search and tactics of child management.

There are publications on the association of celiac disease with intussusception and intestinal obstruction in adults. Although intussusception is the most common cause of intestinal obstruction in children, it is rarely considered in association with celiac disease. The article presents clinical cases of a rare manifestation of celiac disease with acute surgical pathology – acute intestinal obstruction due to the intestinal intussusception and obstruction. Children with intussusception with non-established etiology should be examined for celiac disease, especially when they have the appropriate symptoms – growth retardation, anemia, bloating, increased transaminase levels and other manifestations of celiac disease, and in case the child’s age is not entirely characteristic of the intussusception.

The article presents the analysis of the health status of children born prematurely during the first year of their life. There is the comparison of morbidity rates for five years of the operation of the catamnesis center of Kazan. Profoundly premature children demonstrated the highest level of morbidity with more frequent pathologies of the central nervous system, respiratory organs, eyes and anemia of premature children. In 2017 there was a decrease in the frequency and severity of respiratory diseases (bronchopulmonary dysplasia with chronic respiratory insufficiency 1.6 times), the nervous system (severe ischemic and hypoxic-hemorrhagic lesions of CNS 2.7 times), eyes and its adnexa (a complicated retinopathy of premature children 1.7 times) in extremely premature infants.

The article describes surgical diseases of newborns after a trauma of central nervous system in the perinatal period. There is a detailed diagnostic and therapeutic tactics for injuries of internal organs. There is a clinical case of natal injury, leading to severe multi-organ failure and death.

The complicated course of acute otitis media in children not vaccinated against Streptococcus pneumoniae is progressively increasing. Up to 95% of children suffer at least one episode of acute otitis media during the first 7 years of life. Complications of this disease become a heavy burden for both patients, often leading to disability, and healthcare in general. The errors in antibiotic therapy of acute otitis media at the outpatient stage continue to be a serious problem. We present an analysis of the complications of acute otitis media in children and demonstrate the ways of solving this difficult problem.

The article describes the experience of implementing the “Emergency pediatrics” educational program with the use of simulation training methods, mobile simulation equipment, and distance educational technologies. The program is aimed at improving the doctors’ professional competence in emergency medical care for children. This format of the training helps to train the largest number of doctors in accordance with the international standards for emergency medical care on-the-job. The educational program makes it possible to assess the level of initial and final knowledge, to monitor professional skills, to determine the degree of the doctor’s readiness for emergency medical care for children. Based on the results of the incoming test control and assessment of basic cardiopulmonary resuscitation skills, we can conclude that the pediatricians have insufficient theoretical and practical skills of emergency care. As a promising direction, we plan to create a Register for sudden cardiac arrest, the Register of survival after sudden cardiac arrest in children at hospital and outside the hospital, which improve the quality of emergency and urgent care for children.