The greatest threat to the health of the children exposed to radiation or born from irradiated parents after the accident at the Chernobyl NPP is the risk of developing stochastic radiation-induced pathology: genetic diseases, undifferentiated mental retardation, malignant neoplasms, etc. According to the provisions of international organizations (UNSCEAR, BEIR and ICRP), these effects can be caused by any dose, even a small one.

Stochastic effects, which are currently associated with ionizing radiation, occur as mutations and then they are expressed to the final clinical manifestations (oncological, genetic pathology) as hidden genome damage. The article describes the complex of risk factors of developing radiation-induced  diseases, namely, the phenomenon of genomic instability, the system of genomic DNA repair as a mechanism of anti-mutagenic protection, characteristic of gene expression of the TP53 gene network, immunological predictors of the risk of developing radiation-induced diseases for early diagnosis of the pathology and minimization of the effects of ionizing radiation.

The article presents the data on modern approaches to non-drug and drug treatment of hypertension in children and adolescents. The article analyzes the recommendations of the American Heart Association (2017) and the European Society of Hypertension (2016) for the treatment of arterial hypertension. The authors describe the possibilities of non-drug treatment by changing the diet and increasing the level of physical activity. The article presents data on multicenter randomized clinical studies on the use of the main groups of antihypertensive drugs in children and adolescents, recommendations on the target level of blood pressure in the treatment of hypertension. The authors present indications and contraindications to the prescription of antihypertensive drugs: angiotensinconverting enzyme inhibitors, angiotensin receptor blockers, calcium channel blockers. The concept of using β-blockers for the treatment of arterial hypertension is revised. The authors consider the treatment of refractory arterial hypertension and treatment of hypertension with proteinuria, metabolic syndrome, dyslipidemia.

The review of the scientific literature provides the recent data on vitamin D for children with allergy: atopic dermatitis and bronchial asthma. The article describes the studies of the adverse effects of hypovitaminosis D in children with allergy; it admits the positive effect of vitamin D in the standard therapy of children allergies, in particular the reduction in the frequency of exacerbations.

The study is devoted to the assessment of hemodynamic parameters in healthy newborns in the early neonatal period. The authors examined 76 healthy newborns aged up to 7 days. 12 (15.7%) of 76 children were diagnosed with intrauterine growth retardation of hypotrophic type and 14 children (18.49%) were premature. The authors evaluated the diameter of the outgoing tract of the left ventricle, pulmonary artery trunk, mitral and tricuspid valve rings. The disc method was used to determine the final diastolic volume of the left ventricle. The pulse Doppler was used to determine the integrated flow rate in the outflow tract of the left ventricle, the pulmonary artery trunk, on the mitral and tricuspid valves. After US there were calculated the stroke volume index, cardiac index, total peripheral vascular resistance and oxygen delivery index.

The results. It integral flow rate was found to be a key indicator of central hemodynamics, which determines the magnitude of the stroke volume. Body weight and the presence of functioning fetal communications do not have a significant impact on the indexed systemic blood flow in healthy newborns. There is a direct correlation between the integral blood flow velocity and the stroke volume index, which is characteristic of all intracardiac anatomical structures.

To study the health status of children of the first 12 months of life born after in vitro fertilization from single and multiple pregnancies, the authors analyzed obstetric and gynecological anamnesis of mothers, peculiarities of the procedure of extracorporal fertilization, the results of a prospective clinical observation of children under 1 year with the assessment of physical, neuro-psychological development, morbidity and disability. It was found that multiple pregnancy due after in vitro fertilization is a risk factor for preterm birth, birth of children with low body weight and the formation of perinatal pathology. Children born from a single pregnancy after in vitro fertilization, at the age of 1 year did not differ from children born from spontaneous  pregnancy, in terms of physical and neuropsychological development parameters, and absence of disability. Morbidity, disability, physical and neuropsychic development of children with a birth weight of less than 1,500 g from multiple pregnancies after in vitro fertilization did not differ from children from multiple natural pregnancies in the first 12 months of life. The procedure of in vitro fertilization is not a negative factor for the formation of the health of children born from single and multiple pregnancies. In this regard, the multiple pregnancy and the associated prematurity is the main factor affecting the health of children born after in vitro fertilization.

The objective. To analyze early and long-term results of surgical treatment of biliary atresia in children. 120 patients with biliary atresia underwent Kasai procedure from 2000 to 2018. The follow-up course varied from 6 months to 15 years. The authors assessed the survival rate of children with native liver and the survival rate without indications for liver transplantation in different age periods, as well as the frequency of surgical complications, bacterial cholangitis, portal hypertension, etc.

The results. The survival rate of children with native liver within 1 year was 82.7%; within 2 years – 57.72%; 3 years – 49.6%; 5 years – 42.1%; over 10 years – 33.25%. In case of effective surgery the authors noted the restoration of stool color, jaundice relief and gradual normalization of bilirubin level during the 1st year. The activity of gamma-glutamyl transferase and transaminases in most children increased in the early postoperative period and, then, gradually decreased. The most frequent postoperative complications were episodes of cholangitis and portal hypertension. During the first year, episodes of cholangitis were detected in 50 (42.3%) of 119 children, regardless of the effectiveness of the Kasai procedure. 56 children (47.75%) had signs of portal hypertension during the first year of life; its frequency increased to 70% by the age of 5–10 years.

Conclusion. In most cases, Kasai procedure prolongs life with the native liver, allowing you to postpone transplantation and it is an effective method of treatment of biliary atresia in children. The most frequent complications are cholangitis and portal hypertension, the early detection and timely correction of them is an integral part of the treatment of these patients.

The issue of perinatal injury of the central nervous system and birth trauma draws close attention of various specialists.

The objective: to identify the effect of extragenital pathology of the pregnant woman and obstetric factors on the formation of perinatal CNS injuries and traumatic injuries of the newborn.

Materials and methods. The authors studied ante- and intranatal risk factors for perinatal CNS  damage and birth trauma in 183 babies in severe or moderately severe condition (main group) born in the City Clinical Perinatal Center in 2011–2017. There were assessed the clinical manifestations and results of instrumental studies. The control group was formed by the case – control type of 183 women in labor and their children without birth injuries.

The results. There were revealed the most significant risk factors for the formation of perinatal CNS injury and birth trauma of the newborn: a high frequency of pregnancy complications (100%), extragenital diseases (90.2%), urogenital infections (33.3%), umbilical cord pathology (33.3%) and other complications of labor (42.1%).

The conclusion. The complications of pregnancy and childbirth, extragenital diseases, urogenital infections contribute to the frequency of perinatal CNS damage and development of birth trauma.

The objective. To calculate the reference intervals of the content of organic acids in the urine of the children of different age groups based on the results of gas chromatography with mass spectrometry; to find out their diagnostic value as biochemical markers of organic acidemia and other metabolic disorders.

Materials and methods. The group consisted of 80 children with suspected organic acidemia. The control group consisted of 443 practically healthy children of different ages. The urine samples were analyzed for the content of 28 organic by gas chromatography with mass spectrometry.

The results. The authors established reference intervals of 28 organic urine acids. The differences in the reference intervals defined in this work were isolated from those obtained in other studies. There were identified 37 children with abnormalities in the spectrum of organic acids according to the results of the analysis of the urine samples of patients by gas chromatography-mass spectrometry. 23 patients were diagnosed with various forms of organic acidemia on the basis of characteristic clinical and laboratory data and changes in the indicators of organic acids.

The conclusion. The method of gas chromatography with mass spectrometry has proved its effectiveness  and reliability for the diagnosis of hereditary diseases from the group of organic acidemias.

The objective: to study clinical and microbiological features of the injuries of the respiratory organs and to assess the effectiveness of elimination therapy in chronic sinus infection in children in the regional center for cystic fibrosis. The specialists of Omsk center for cystic fibrosis made a retrospective analysis of 48 case histories and assessed clinical and functional parameters depending on the age and microbiological status of the respiratory tract. They presented a comparative assessment of the effectiveness of the original and generic tobramycin inhalation.

The results. An association of microorganisms was identified in the majority of bacterial cultures of cystic fibrosis patients’ sputum (66.7%). Among the clinically relevant pathogens there were Staphylococcus aureus (18.9%) and Pseudomonas aeruginosa (16.6%) with the significant prevalence of non-mucoid forms. From 2011 to 2016 the amount of Pseudomonas aeruginosa strains, sensitive to all usable antibiotics, decreased, especially significant sensitivity decline was registered to amikacin – 21.35% and сiprofloxacin – 26.1%. The children infected by Pseudomonas  aeruginosa had more deviations of lung function measured by the computed bronchophonography and spirometry, they more often had changes at chest computed tomography than patients free of Pseudomonas aeruginosa infection. Usage of generic tobramycin instead of original tobramycin didn’t lead to the elevation of respiratory syndrome exacerbation rates, didn’t increase requirement in the intravenous antibacterial treatment and didn’t influence to the degree of microbial colonization of sputum. The conclusion. There was noted an increase in the resistance of Pseudomonas aeruginosa to anti-pseudomuscular drugs, which justifies the introduction of highly concentrated inhaled forms of antibiotics. Replacing the original inhaled tobramycin with a generic drug did not cause a deterioration in the clinical and functional status of the patients and did not change their microbiological status.

In total the authors reviewed 83 patient records: disease development anamnesis, results of the standard clinical, radiological and laboratory examinations.  Tuberculosis in adolescents  found during routine examination is characterized by a high epidemic risk (45% of patients were sputum positive), severe clinical forms of the disease with the pronounced residual abnormalities and therefore need of surgical treatments. Patients with the clinical signs of pulmonary disease (pneumonia, bronchitis, recurrent acute respiratory infections) belong to the risk group requiring evaluation for tuberculosis including with the use of immunological skin tests.

The objective. To assess the incidence of insufficient supply of water-soluble vitamins in vegetarian children using non-invasive methods (excretion with the urine), depending on the intake of the vitamin-mineral complex. In the winter-autumn period we examined 38 preschool and school-aged vegetarian children (3–16 years old) living in Moscow and being on vegetarian diet. The 1st group consisted of 25 children who did not take vitamins. The 2nd group included 13 children who took a complex of 12 vitamins and 9 minerals for 1–3 weeks. Vitamin status was assessed by excretion of ascorbic acid, thiamine, riboflavin, and 4-pyridoxyl acid with morning urine collected on an empty stomach.

The results. Only 1 child from the 1st group had a lack of vitamin C due to the sufficient intake of fresh vegetables and fruits. A vitamin-mineral complex noticeably improved the provision of vitamins B. The children of the Group 1, had a reduced excretion of vitamins B1, B2 and B6 more often as compared to the children of Group 2: 44.0–64.0% vs 0–30.8%. 2/3 of children-vegetarians of Group 2 were adequately provided with three vitamins of group B, which is 2.7 times higher than children who did not receive vitamins. A deficiency of three vitamins was detected in 7 children (28%) who did not take a vitamin-mineral complex.

The conclusion. A vitamin-mineral complex in the diet is a necessary condition for maintaining optimal micronutrient status of vegetarian children.

Information on the prevalence of allergic and non-allergic bronchial asthma among children of preschool age is limited due to insufficient amount of studies. At the same time, determining the phenotype of the disease is the key to choosing the optimal therapy and determining the prognosis.

The objective: to establish the prevalence of allergic and non-allergic bronchial asthma in children of 3–6  years living in the urban areas of the Altai Territory, and to study the sensitization spectrum. The authors conducted a momentary population-based study, consisting of two stages. At the screening stage, the study included 3205 children of 3–6  years attending preschool educational institutions. Symptoms of bronchial asthma were determined using the ISAAC questionnaire. At the clinical stage, the diagnosis was verified by allergists based on the diagnostic criteria of GINA. The allergic phenotype of asthma was established with a positive prick test and / or at a level of specific blood IgE of > 0.35 kE / l to at least one allergen.

The results. 5.7% of urban children of 3–6 years suffered bronchial asthma. Allergic asthma prevails over non-allergic one – 4 and1.7%, respectively. The condition of most children is sensitized to 2 allergens and more, the most often case was a sensitization to house dust mite Dermatophagoides pteronyssinus (42.9%) birch pollen (34.3%) and cat epithelium (27.5%).

The conclusion. 5.7% of children of 3–6  years living the in urban areas of the Altai Territory suffered bronchial asthma with a predominance of allergic phenotype.

Moscow is a city of federal significance with a high population of children and, accordingly, a large number of primary patients detected annually.

The objective of the study is to analyze the main indicators characterizing the medical care for children with cancer in Moscow and the Moscow Region.

Materials and methods. There were analyzed the operational reports of the Moscow Department of Health and the Ministry of Health of the Moscow Region for 2013–2017.

The results. In 2013–2017 there were identified 18,090 primary patients in the Russian Federation, among them 1807 (10%) in the analyzed locations: 1315 (7.3%) in Moscow, 492 (2.7%) in Moscow Region. For 5 years the number of primary patients in Moscow increased by 18.1%, and in the Moscow region it decreased by 2.3%. The morbidity of the children aged 0–17  years in Moscow increased by 5.3%, while in the Moscow Region the morbidity decreased by 20%. The mortality rate in 2016–2017 in Moscow decreased by 37.5%, while in the Moscow region – by 50%. There were no actively identified patients in Moscow in 2016–2017, and in the Moscow region their percentage decreased from 34.2 to 7.3.

The conclusion. The incidence of malignant neoplasms of the child population in Moscow and the region is slightly lower than the average for the Russian Federation. At the same time, the levels of morbidity and mortality in Moscow and the Moscow region differ significantly. This indicates obvious defects in diagnostics and accounting, which is confirmed by the discrepancy between these operational reports and the Federal State Statistics Service. District pediatricians demonstrated low oncological alertness.

Lyell’s syndrome is one of the most serious diseases of a toxic-allergic nature and it presents a real threat to children life. The article presents literature data and clinical observations of Lyell’s syndrome in children, the etiological cause of which was medication, often used in pediatric practice for viral infections – 0.05% eye bonafton ointment, paracetamol. Based on the analysis of literature data and our own observations, we propose the algorithm of therapeutic measures in children with Lyell’s syndrome, which includes the administration of glucocorticoids, immunoglobulins for intravenous administration, hyposensitizing agents, correction of water and electrolyte balance, local treatment, etc.

In recent years the technology of new generation sequencing technology (NGS) has become an important diagnostic tool in pediatrics. In the genetic pediatric clinic there were analyzed the results of the use of full excome sequencing in 42 children with the retardation of mental and physical development and / or abnormalities of various organs and systems. There was established a primary genetic diagnosis in 19 patients, and thus, the effectiveness of exomic sequencing was 45%, which is slightly higher than the effectiveness of NGS given in the literature sources. The article presents clinical observations of the cases of primary, or double diagnosis, prognostic secondary variant, examples of errors in the interpretation of sequencing data. The authors emphasize the importance of studying family mutations not only among the parents, but also other relatives of the patient. In particular, in case of the identification of X-linked genetic variants, the authors justify the necessity of analysis in three family generations.

In the era of modern medicine, there are formed the fundamental scientific principles in the diagnosis of a wide range of diseases that are based on traditional instruments: laboratory and functional studies, light microscopy, immunofluorescence, and electron microscopy. However, in recent decades these methods have become insufficient to verify various variants of the diseases, especially with an atypical clinical picture. The article discusses the capabilities of the nCounter technology from the company Nanostring Technologies, it describes in detail the capabilities of the technology in the diagnosis of various diseases, and also the authors present their own experience of using diagnostics in children with nephrotic syndrome.

Marfan syndrome was described more than 125 years ago, but it still remains a very complex and urgent medical problem.

The objective. To evaluate the nature and severity of cardiovascular complications of Marfan syndrome. In the Republic of Crimea, there was analyzed the follow-up study of 15 patients with Marfan syndrome: 1 – 3 years old – 1 child (6.7%), 3–7 years old – 3 children (20%), 8–12 years old – 3 children (20%), 13– 17 years old – 8 children (53.3%). In 2 cases there was the familial form of the disease.

The methods. The diagnosis of Marfan syndrome was based on the study of genealogical data, analysis of the morpho-phenotype and the results of the clinical examination. The severity of cardiovascular complications was assessed according to clinical and instrumental studies. The results. Heart failure was manifested by mitral valve insufficiency and expansion of the ascending aorta. The article contains own clinical observations of a teenager with acute aortic dissection, which led to the death due to the parents refusing the operation. Three other children successfully underwent surgery, in particular, surgery after Bentall and David. The remaining children with an aortic aneurysm are still followed-up and do not require surgery.

The conclusion. These clinical examples prove that parents are not always aware of the severity of the child’s condition. It is important to emphasize that the cooperation of the parents, the cardiologist and the patient himself contributes to the preservation of life, improvement of its quality and duration.

The article reflects the modern views on food protein induced proctocolitis: prevalence, pathophysiological mechanisms of intestinal damage, clinical manifestations, main food allergens that contribute to the development of this pathological condition, approaches to diagnosis and diet therapy. The article describes a clinical case of food protein induced proctocolitis in a child of the 1st year of life.

Respiratory infections are mainly caused by viruses or bacteria that often interact. In the upper respiratory tract there is a wide range of commensals and potential pathogenic bacteria that, together with other bacteria, viruses and fungi, form complex ecological networks known collectively as “the microbiome”. It is assumed that this community is constantly exposed to synergistic and competitive interspecific interactions. The microbiome of the respiratory tract is a factor of respiratory health, which changes (respiratory viral infections, inappropriate use of antibiotics) can lead to bacterial complications and the spread of respiratory tract infections in. In the light of current guidelines for limiting the use of systemic antibiotics in case of uncomplicated acute respiratory tract infections, a real alternative is the use of topical antimicrobial agents, for example, thiamphenicol glycinate acetylcysteinate. The article presents the results of the use of the drug in respiratory infections of the upper and lower respiratory tract.

The article deals with the formation of eating behavior in children with the use of complementary foods. It contains the data on new types of complementary foods, and the possibility of their use in children of the 1st year of life. The article presents the results of clinical studies of new types of products for younger children that are developed with innovative technologies.