Cardiovascular disease (CVD) is one of the main reasons for the high mortality rate in the world. In recent years, the importance of vascular lesions of the heart and brain in the structure of causes of death among adolescents has increased. The main risk factor for deaths and serious life-threatening complications of CVD is overweight and, in particular, obesity. Over the past 20 years the prevalence of this risk factor among children aged 6–11 years has doubled, and among teens aged 12–19 years — tripled. It is expected that the urgency of the problem will increase in the future. Over the past 40 years the association of obesity with sleep-disordered breathing has been actively studied. Therewith, obstructive sleep apnea/hypopnea syndrome is considered as one of the main causes of the development of cardiovascular accidents in adults. This review presents the results of analysis of the problem of CVD development in children with sleep disorders assessing the pediatric aspect of this problem.

Sudden Infant Death Syndrome (SIDS) is one of the leading causes of postneonatal mortality of infants in most developed countries. This paper presents data on terminology and on the study of SIDS in developed countries, and also examines problems of SIDS diagnostic in
Russia in comparison with the results of own researches, analyzes risk factors, which are universal as for SIDS, and also for perinatal and infant death in general. The large variability of SIDS diagnosis in Russia suggests that this diagnosis often does not comply with international standard: post-mortem examination of community-acquired sudden death cases is often carried out by a forensic expert, but not by a children's pathologist, and they do not use virological and microbiological methods of investigation. In some cases, there is no infant's record or case record from children's polyclinic.

Background: At present, it is urgent to find ways to overcome the inefficiency of and intolerance to the methotrexate therapy in patients with juvenile idiopathic arthritis (JIA). A promising method is the use of TNF-alpha inhibitors as monotherapy.

Objective: Our aim was to evaluate the efficacy and safety of etanercept monotherapy and treatment with etanercept and methotrexate in patients with JIA without systemic manifestations.

Methods: Observational study with retrospective assessment of the treatment of patients who received etanercept — the treatment group (n = 55) and the combination of etanercept and methotrexate — the control group (n = 136). The efficacy was evaluated for 3 years using the pediatric criteria of the American College of Rheumatology (ACR), remission criteria by C. Wallace and index JADAS71.

Results: Total amount of patients with JIA without systemic manifestations under study is 191. As early as 1 month after the first etanercept injection, clinical and laboratory parameters of disease activity significantly decreased in 83 and 77% and functional ability of joints improved in 87 and 74% of patients treated with TNF- inhibitor and its combination with methotrexate. After 6 months, the improvement according to the ACR pediatric criteria 30/50/70 was recorded in 98/98/96 and 96/95/86%; inactive stage of the disease/remission was recorded in 44 and 24% of patients who received etanercept and etanercept + methotrexate. After 1 year, the improvement was recorded in 100 and 100/98/93% of patients in the treatment group and the control group and inactive stage of the disease/remission — in 65 and 43% of patients. In the course of etanercept + methotrexate therapy, infectious adverse events were more common. 12.7 and 7.3% of patients treated with etanercept and methotrexate discontinued their participation in the study during the first 6 months and 18 and 10% — during the first year. 8.4% of patients treated with the combination of etanercept and methotrexate discontinued their participation in the study during the second year.

Conclusion: Etanercept monotherapy is effective on a par with the combination therapy, but it has a higher safety profile and a lower therapy «survival».

Objective: Our aim was to study demographic and clinical characteristics of patients with systemic juvenile idiopathic arthritis (sJIA), timing of diagnosis and routing of the children after the onset of the disease according to the All-Russian Register of the Union of Pediatricians of Russia.

Methods: Retrospective study (1998–2015) analyzing indicators from 384 children with sJIA.

Results: The majority of patients live in the Central and Volga Federal Districts — 157 (40.9%) and 68 (17.7%) patients, respectively. The ratio of girls to boys is 1.25:1; 281 (73.2%) children got sick under the age of 5. In 35 (9.1%) children the disease is hereditary. The trigger factor in 1/3 of patients was an infection. At the onset of the disease, fever was recorded in all patients, rash — in 190 (49.5%), enlargement of the liver and/or spleen — in 150 (39.1%), and serositis — in 56 (14.6%) patients. 17 (4.4%) children had 5 manifestations of systemic disease, 117 (30.5%) — 4, 64 (16.7%) — 3, 73 (19.0%) and 73 (19.0%) — 2 and 1. 175 (45.6%) children had arthritis at the onset of the disease and extra-articular manifestations were observed for 6 months. 7 (1.8%) patients were hospitalized to the specialized rheumatology departments, 50 (13.0%) — to the cardiologic departments, and 190 (49.5%) — to the pediatric departments. 250 (65.1%) patients were diagnosed with an infectious disease. None of the patients was diagnosed with sJIA within first 6 weeks. The average duration of the disease from the onset to diagnosis was 2.0 ± 15.5 (0–139) months, from the onset to the admission to a special department of the federal research center — 8.0 ± 29.3 (0–146) months, from diagnosis to the admission to the federal research center — 3.0 ± 28.0 (0–138) months; 50 (13.1%) patients were hospitalized without a diagnosis (established at the federal research center).

Conclusion: According to the All-Russian Register, sJIA is characterized by aggressive beginning at an early age with severe extra-articular manifestations and late addition of the articular syndrome. In most cases, the verification of diagnosis and admission of patients to the specialized rheumatology departments of the federal research center were carried out in later periods.

Background: The use of genetically engineered biological agents in patients with juvenile idiopathic arthritis (JIA) allows to achieve good results in treatment. However, their efficacy in patients refractory to basic antirheumatic drugs, requires further analysis.

Objective: To study the efficacy and safety of the combination therapy by adalimumab with methotrexate and of the monotherapy by methotrexate in children with JIA.

Methods: It was conducted a perspective (annual) comparative research with the analysis of the results of treating children with polyarticular seronegative JIA refractory to basic immunosuppressive therapy. The efficacy was assessed by ACRpedi criteria and by dynamics of values of cellular and humoral immunity indicators. Safety was determined by the number of adverse effects.

Results: The combination therapy by methotrexate with adalimumab for children with JIA (n = 14) in a shorter time than the monotherapy by methotrexate (n = 17) induced arthritis remission. After 46 weeks of treatment, the remission of arthritis and the normalization of laboratory findings were reported in 6 (43%) patients treated by adalimumab with methotrexate, and in 3 (18%) children — methotrexate (p = 0.233); adverse effects — in 4 (29%) and 14 (82%) cases (p = 0.004), respectively. No serious adverse effects have been registered.

Conclusion: The prescription of adalimumab in combination with methotrexate in children with JIA, refractory to basic immunosuppressive therapy, allows to achieve rapid remission of the disease while preserving the effect in a significant number of patients within 46 weeks of therapy.

Background: Vitamin D is involved in important physiological processes. Vitamin D deficiency is a risk factor for the formation of various pathological conditions. We are interested in the study of vitamin D sufficiency of the population of various age groups living in the northern regions where the risk of vitamin D deficiency is caused by low insolation.

Objective: To evaluate vitamin D sufficiency of the population
of the city of Arkhangelsk in different age groups.

Methods: In the prevalence (cross-sectional) uncontrolled study we examined the concentration of 25-OH Vitamin D — 25(OH)D — in the blood serum of residents of both sexes in Arkhangelsk in spring-autumn period 2013–2014. Moderate vitamin D deficiency was determined at the content of 25(OH)D in the range of 20–30 ng/ml, deficiency — at 10–19 ng/ml, severe deficiency — at < 10 ng/ml.

Results: The study included children under 3 years (n = 155), school children of 6–7 years (n = 80), adolescents of 13–15 years (n = 184), university students of 18–22 years (n = 88), and adults of 24–60 years old (n = 85). The lack of vitamin D is found in 38 (25%), 16 (20%), 54 (29%), 28 (32%), and 35 (41%); deficiency — in 35 (22%), 39 (49%), 127 (69%), 44 (50%), and 25 (29%); severe deficiency — in 12 (8%), 18 (22%), 1 (1%), 1 (1%), and 3 (4%) of patients, respectively.

Conclusion: The high incidence of vitamin D deficiency of varying severity is determined in all age groups of the population living in the city of Arkhangelsk. The findings indicate the necessity of the development and implementation of target regional and national programs for the prevention, early detection, and correction of vitamin D deficiency and associated disorders in the Russian Federation.

Background: Cancer is the second most common cause of death in children. The organization of cancer care for children requires further reforming and rational use of available resources.

Objective: Our aim was to assess the provision of federal districts (FD) with cancer specialists and relevant bedspace needed for providing medical care for children with newly diagnosed malignant disease.

Methods: The ecological study investigated reports of regional ministries and departments of health of 82 subjects of the Russian Federation for 2013. It was analyzed the morbiden (newly diagnosed cases of cancer) of children aged 0–17 years, the provision of bedspace, doctors, diagnostic and therapeutic technologies.

Results: In 2013 it was registered 3378 children with cancer. The average incidence rate in Russia amounted to 12.4 per 100 thousand of the child population, in the RF subjects index values ranged from 5.5 to 21.8. 1705 (50.5%) patients were sent to federal clinics. In total, in 2013 in Russia there were 51 children's oncology departments with 2021 beds (including beds in non-core departments), therewith, the provision of bedspace varied by FD from 0.40 to 1.13 per 10 thousand of the child population. Treatment of children with cancer was carried out by 390 doctors, 138 (35.4%) among them did not have a certificate of Pediatric Oncology. The provision of doctors varied by FD from 0.06 to 0.20 per 10 thousand of the child population. The availability of modern diagnostic tools in the RF remains at a relatively low level. As a result, 76.6% of all newly diagnosed cancers were stage III–IV.

Conclusion: It is required the improving of the vertical and the amount of specialized medical care for children with newly diagnosed cancer.

Objective: On the basis of international requirements to create a Russian version of the international instrument Pediatric Quality of Life Inventory (PedsQL) 3.0 Cardiac Module, used for assessment of the quality of life in children with cardiovascular diseases.

Methods: In a prospective study it was assessed the reliability of the questionnaire (calculating Cronbach's alpha coefficient), its validity — by comparing the responses of patients who had a different ejection fraction (construct validity), and comparing the responses on the scales of PedsQL Cardiac Module questionnaire with the scales of the common PedsQL Generic Core Scale questionnaire (convergent validity). The questionnaire sensitivity was determined by calculating the difference between the values of quality of life of children at the moment of their first hospitalization (1st point) and in 4 months after the initiation of combination therapy (2nd point).

Results: The study involved 61 children with heart failure (chronic myocarditis — 25, dilated cardiomyopathy — 36). The median of the age of patients was 12 (4; 15) years. The questionnaire proved to be reliable (the values of Cronbach's alpha coefficient for different units ranged from 0.75 to 0.91) and satisfactory constructive and convergent validity. Therewith, children with ejection fraction <60% had lower values of quality of life than children with higher ejection fraction. Increase in assessment of quality of life in the course of the administered treatment pointed to the questionnaire sensitivity to changes in patients' condition.

Conclusion: Satisfactory psychometric characteristics of the Russian version of PedsQL 3.0 Cardiac Module questionnaire allow to use it for research in the field of quality of life in children with heart failure.

The article provides information on disorders of nutritional status (malnutrition) in children with chronic heart failure (congenital heart diseases, cardiomyopathy), discusses the causes of growth retardation and failure to thrive. The factors of the risk of nutritional
deficiency development and the possibility of its correction with the use of nutritional products with a specially given composition. It is presented the clinical observation of an infant with congenital heart disease, aortic coarctation and patent ductus arteriosus, chronic heart failure 2B-A st. and severe malnutrition. The correction of nutritional status using a specialized formula for enteral nutrition in the course of adequate medical therapy allowed to improve the general condition of a patient and positive dynamics of body weight.

Breast milk is the best food for a baby of the first year of life. To pass successfully from feeding by breast milk or formula tothe introduction of complementary food, a child need to learn to perceive the taste of new products offering to him. We have evaluated mouthfeel of complementary food in 30 children aged between 4 and 12 months of life, which were divided into 3 groups according to the administered complementary food. The observation period was 30 days. The accounting of the perception of new complementary food was carried out basing on the assessment of the child's emotional status and tolerability of the complementary food product. As a result, in the first group of children the refusal of the vegetable intake (broccoli, squash) occurred to 4.1 ± 0.94 days from the start of the product administration. Intestinal colic and defecation disorders were observed in 2 (16.6%) children of this group, but they were not registered after the re-examination of infants. In the second group, the positive perception of buckwheat dairy-free porridge rich in 3 polyunsaturated fatty acids was observed ranging from 3.0 ± 0.97 days from the start of its administration. In this group 1 (8.3%) child responded by the appearance of posseting and 2 (16.6%) — by defecation disorders. After 2 weeks 1 (8.3%) child still had defecation disorders. In the third group, children, starting from the 2nd day of the intake of pureed meat, ate this product with pleasure, disorders of the gastrointestinal tract have not been reported. Thus, studied complementary food has a good taste, hypoallergenic balanced composition and allows to diversify the diet of infants.

The article presents current data on the peculiarities of the etiology and pathogenesis of diaper dermatitis in newborns and infants. The role of enzymes in the gastrointestinal tract, of bacterial and fungal agents in its development is showed. The data on peculiarities of the differential diagnosis of skin diseases involving the anogenital area is given. The peculiarities of psoriasis manifestation with typical inversion of skin pathological process in infancy are noted. The results of clinical studies on the efficacy and safety of dexpanthenol in the treatment of diaper dermatitis are highlighted, the basic principles of its prevention are announced.

The article considers philosophical questions of neonatal screening technology. The main focus is on ethical and methodological issues that inevitably arise when expanding the number of scanned nosologies and applying genetic research methods. Questions concerning the existing discrepancy between technical capacity and the practical level of healthcare delivery and the probabilistic nature of results obtained by molecular testing are analyzed in terms of methodology. Access to information about the DNA-testing of newborns and the linkage between neonatal screening and prenatal diagnostics are among the most topical ethical problems raised within this article. One of the purposes of this article is to draw the attention of the public — especially it concerns current and prospective parents and volunteer organizations — to these contemporary problems.

Leukoencephalopathy with predominant lesion of the brain stem, spinal cord and high blood lactate in the MR spectroscopy is a hereditary disease characterized by slowly progressing cerebellar, pyramidal disorders, disorders involving the lesion of posterior columns of the spinal cord and highly specific changes according to the data of MRI and magnetic resonanse spectroscopy of the brain and spinal cord. This disease is one of nuclear inherited mitochondrial encephalomyopathies, caused by mutations in the DARS2 gene and characterized by mitochondrial aspartyl-tRNA-synthetase deficiency. This article presents the record of the disease description, its genetic basis, clinical features and diagnostic criteria. The article describes two clinical cases of the disease in 9-year and 17-year old girls.

The article describes the individual peculiarities of a rare disease — hemiplegic migraine — in 3 patients (two girls aged 2 and 14 years old and a boy of 16 years). In common clinical aspect there was a correlation between the migraine-attack and the slight head trauma in all patients. Attack symptoms were almost identical: hemiparesis, aphasia, ataxia. The family history for migraine was burdened in both girls. A genetic testing in the boy and in the smallest girl demonstrated CACNA1A gene mutation, in the teen girl — ATP1A2 gene mutation. The electroencephalograms in all patients during the acute phase presented signs of hemipcortical brain dysfunction. The magnetic resonance imaging revealed prominent but reversible hemicortical oedema. The repeated MRI studies diagnosed nonrelevant for this disease hemicortical atrophy (girl 2 years) and atrophy of the cerebellum (the boy). Due to the rarity of the disease so far there are no clear guidelines for its treatment and prevention. In view of the pathogenesis for the prevention patients were prescribed medications changing the activity of cytoplasmic calcium and sodium canals.

The high prevalence of acute otitis media in children confirms the urgency of finding new treatment methods of the disease. In view of the problem of antibiotic resistance, the question of using topical antimicrobial preparations with high bioavailability and able to solve the problem of acute otitis media treatment at different stages of the disease is becoming particularly important.