The necessity for system structuring is defined by entry into force of the new normative documents directed on improvement of availability and quality of medical care, introduction of orders and standards of medical care delivery, providing staging and recommended volume. The three-level system operating in Saint-Petersburg is defined by normative documents of regional level and provides staging of medical care delivery to children with infectious diseases — one of the most frequent children pathologies. The main objective of the present article was to analyse the activity of three-level structure of the establishments rendering medical care to children with infectious diseases, to define the main objectives carried out at each level. The analysis of the main indicators shows the necessity for improvement of the system through rationalization depending on quality and volume of rendered medical care.

Acute Enteric Infections (AEI) occupy one of the leading places in structure of infectious pathology at children's age. A variety of etiological agents (bacteria, viruses and protozoa) causing infectious diarrhoea, the high frequency of mixed infections, frequent failures and uneven course prove the necessity of search of ways for optimization of diagnostic and therapeutic approaches according to modern scientific data. The principles of clinical and laboratory diagnostics of AEI are considered in the article. Criteria of the assessment of a disease severity, predictive criteria of development of the life-threatening states connected with the exicosis are presented. The principles of AEI treatment are presented from the modern positions, the necessity of an integrated approach and staging in therapy with the personified choice of preparations considering weight, phase and clinical form of a disease, age of a child and a macroorganism condition by the time of the disease beginning are introduced. The necessity of a strictly differentiated approach to prescription of antibiotics is emphasized. The data on expediency of inclusion of probiotics to starting AEI therapy of virus aetiology are separately presented taking into account strain-specific efficiency that provides the reliable reduction of degree of intensity and duration of the main symptoms of a disease, and also has beneficial impact on a condition of microflora of a gastrointestinal path.

In the review of modern literature the influence of a bacterial factor, first of all — indigenous microflora — on formation of food tolerance and immune answer of a child as a whole is in detail considered. Formation of a microbiocenosis, food and autogenic tolerance happens under the general laws. In this process the primary colonization of intestines and formation of a microflora variety which, as results of the last researches show, comes to an end by 2–4 years of life of a child has special value. In the review the major factors influencing the formation of a child intestinal microbiota both antenatal and postnatal and for the first years of life, and also their interaction are considered. The influence of a bacterial factor on formation of food tolerance and sensibilization processes, and also mechanisms of this influence is in detail discussed. The data on features of a structure of intestinal microflora at children with food allergy including the results of own researches devoted to microbiocenosis at children with food allergy and to development of tactics of management of children with this pathology are provided. The main directions and possibilities of dietary influence on biocenosis structure at children with this pathology are considered.

The DALY indicator (Disability Adjusted Life Years) is widely used for the assessment of a contribution of various diseases to losses of the population health and of the analysis of activity efficiency of health care systems. Background: Research objective was to evaluate dynamics of the DALY indicator as a result of premature mortality of the child population of Tomsk region during 2008-2012. Patients and methods: DALY calculation as a result of premature mortality of the child population of the region during the period from 2008 to 2012 is made. While calculating the data of Territorial body of Federal service of state statistics for the Tomsk Region (Rosstat) which included a sex of the dead, day, month and year of death, place of residence, cause of death, day, month and year of birth of the dead was used. The number and gender and age structure of the population of the Tomsk Region according to Rosstat data were also considered. Results: The DALY decrease by 16,5% in child population at the age of 0–17 years is observed in the Tomsk region during the period from 2008 to 2012. Following the results of 2012, DALY in studied population made 833.1 (82.3 ± 5.4) years for 10 thousand of corresponding population that is 1,1 times lower than totally in the Russian Federation, and 3.4 times lower than the universal indicator of 2010. Conclusion: The received result reflects the activity of health care system of the region, directed on integration of synthesis of theoretical and practical knowledge. Decrease in DALY among the child population in the territory of the region is promoted by a number of factors: continuous quality improvement of the medical care rendered to children, target programs on motherhood and childhood health protection realized in the territory of the region, and also functioning of the federal scientific and educational institutions promoting introduction of modern techniques of medical care delivery in practical health care.

Key words: demography, DALY, health assessment of the child population.

Mucopolysaccharidosis is the group of hereditary metabolic disorders; it is characterized by accumulation of glycosaminoglycans owing to storage of specific lysosomal enzymes. Background: Research objective was to study the influence of enzyme replacement therapy on a somatic state and psychomotor development of children with mucopolysaccharidosis type I and II of various severity in dynamics and to estimate its efficiency. Patients and methods: The data of five years' supervision over 13 patients with mucopolysaccharidosis type I and II is used in the research. During the work the therapy efficiency analysis is made by the following criteria: data of objective examinations, ultrasound investigation of liver, spleen and heart, quantitative determination of excretion of urine glycosaminoglycans, assessment of articular and abarticular affection by JADI scale, assessment of social age and social coefficient by Doll's scale. Results: The reliable distinctions in the contents of urine glycosaminoglycans and also by results of an objective assessment of the liver and spleen sizes and of ultrasonic research of the spleen area in 6 and 12 months of treatment in comparison with basic data are received. The reliable decrease in social coefficient indicator at the first stage of therapy is registered, and then distinctions have insignificant character. There was no essential dynamics of the articular status on treatment that is connected with process stabilization. There are no reliable evidences of both positive and negative dynamics on myocardium involvement, ultrasonic characteristics of the sizes of hepatic lobes. Conclusion: Enzyme replacement therapy is an effective method of treatment of somatic manifestations of various types of mucopolysaccharidosis.

High prevalence of pathological conditions of a visual organ at modern school students is the objective medical and social prerequisite for development of the effective measures directed on prevention and correction of disorders and diseases of a visual organ of children of school age. Background: The aim of the study was to establish the prevalence of disorders and diseases of a visual organ at school students for development of the medical and organizational actions directed on their prevention and correction. Patients and methods: The analysis of medical documentation, the extracting from registration forms of the children's policlinics and medical offices of schools, is carried out (1332 extracted sheets of pupils of a primary, middle and advanced school age of basic high comprehensive schools of Moscow). Results: Authors prepared conceptual model on prevention and correction of disorders and diseases of a visual organ of school students for development of the medical and organizational actions directed on prevention of revealed ophthalmopathy. Conclusion: The use of this conceptual model and also its separate structural components is directed on increase of the level of knowledge of medical workers and optimization of their work on identification and reduction of number of risk factors of disorders of a visual organ of pupils of general education institutions. It will allow to carry out work on prevention and correction of the revealed disorders and diseases of a visual organ of school students at better level throughout the entire period of their training at school.

The relevance of a problem of autism spectrum disorder in children and the modern view on etiology and pathogenesis of these states are revealed in the article. Autism classification according to the International classification of diseases of the 10th revision adopted in Russia and important changes of a new classifier of the American psychiatric association concerning autism spectrum disorders are considered. The difficulties connected with diagnostics of autism spectrum disorders in children, autism comorbidity and some other psychiatric nosologies and the necessity of detailed differential diagnostics for a circle of these diseases are mentioned. Autism spectrum disorders are presented from the point of view of clinical genetics, the necessity of medical genetic consultation in diagnosing is proved. Definition of complex and essential autism is given. A number of widespread genetic syndromes with the description of clinical characteristics and molecular genetic mechanisms underlying the pathogenesis is allocated in the group of complex autism. Difficulties of molecular genetic confirmation of the diagnosis are revealed, the algorithm of search of mutations and the short description of methods of diagnostics are given. The efficiency of standard procedure of molecular genetic diagnostics at each stage, according to literary data, is shown in the group of children with essential autism. The opportunities and advantages of a method of the chromosomal micromatrix analysis as one of available modern methods of molecular genetic diagnostics in the group of children with autism spectrum disorders are highlighted on the example of extended microdeletion and microduplicational syndromes.

Aversion to food is observed at children at different stages of their development since the neonatality period. This state can be caused by several reasons. First, by coexisting diseases among which there are pathology of a gastrointestinal path (gastroesophageal reflux disease, malabsorption syndrome), neurologic (damage at ischemia or hemorrhage of a brain axis and cervical cord, neuromuscular diseases) and logopedic pathology (ankyloglossia), metabolic diseases (organic aciduria) and food intolerance. Secondly, disorder of eating behaviour can be connected with organic pathology, for example with gullet and stomach developmental malformations (esophageal achalasi, short gullet, pyloristenosis), congenital pathology of cardiovascular system, respiratory organs, liver. Thirdly, aversion to food can take place against complete health without visible objective reasons and, as a rule, is interfaced to the wrong feeding. For this reason the determination of possible reasons of eating disorder is fundamental for the achievement of clinical effect. However, besides treatment of the main disease the complex behavioural and nutritional correction which is considered in this article is justified for all patients with aversion to food.

Background: To summarize the experience in application of tocilizumab at system variant of juvenile idiopathic arthritis in the Republic of Bashkortostan. Patients and methods: In cardiorheumatologic department of Republican Pediatric Clinical Hospital of Ufa there were 9 children (6 boys and 3 girls) aged from 2,5 to 13,8 years (middle age — 8,2 years) under supervision. Middle age in a disease onset made 4,5 years. All patients were exposed to the combined antirheumatic therapy before prescription of tocilizumab. Despite the therapy, the patients still had a high degree of activity of a disease that was the basis for prescription of tocilizumab. The average single dose of a preparation made 11,2 mg/kg for children with body weight under 30 kg, 8,7 mg/kg for children over 30 kg. Results: 8 children were exposed to the continuous therapy with tocilizumab, therapy duration fluctuated from 6 to 52 (on the average 17,8) months. Treatment was stopped at 1 patient because of insufficient effect and at 1 patient because of the development of the undesirable phenomena. The minimum improvement defined as achievement of ACR_pedi30 criterion was reached in all patients throughout the entire period of treatment by the preparation. The prescription of tocilizumab allowed to stop the system manifestations of a disease within 1 month of treatment in all children. The JADAS-27 index decreased twice (from 33,6 to 16,5) in 1 month from the beginning of the therapy. From the 3rd month of treatment all patients showed the normalization of indicators of the peripheral blood characterizing the activity of inflammatory process (ESR, contents of leukocytes and haemoglobin, SRP serumal level). In 3 and 6 months from the therapy beginning 7 patients reached 50%, and 5 and 6 — 70% improvement, respectively. Two children didn't reach 70% improvement in view of remaining insignificant or moderately active articulate syndrome. Conclusion: The analysis of the research results showed high efficiency of tocilizumab concerning system manifestations and activity of inflammatory process. At the same time 3 patients still suffered the moderate articular syndrome in the form of persistent oligoarthritis, 1 patient — in the form of polyarthritis.

The main way of prevention of allergic diseases at children of early age is use of hypoallergenic formulas on the basis of partial hydrolysis of protein. Background: The aim of the study was to develop scientifically grounded methods of primary prevention of allergic diseases at children of the first year of life on the basis of data of complex clinical and immunological researches. Patients and methods: 92 children from the group of high risk of development of the allergy (from the birth to 12 months of life) were watched. Results: Among the children receiving hypoallergenic formula on the basis of partial hydrolysis of protein the symptom of allergic forms of pathology at the age of 3 months met by 3 times less than in a group of children receiving standard milk formulas on the basis of integral protein of cow milk. Both at children on breast feeding and receiving hypoallergenic formula, slight symptoms of atopic dermatitis took place whereas a moderate and severe course twice more often was met at children receiving standard milk formulas. Conclusion: Specialized hypoallergenic formula provides physiological needs of children of the first year of life in nutrients and prevents early development of clinical symptoms of food allergy at children with high risk of development of the atopy.

Background: The aim of the study was to to estimate traits of the morphofunctional changes of a gastroduodenal mucous membrane at chronic inflammatory diseases of the upper digestive tract at preschoolers with various level of amylolytic activity of saliva. Patients and methods: 145 children at the age of 3–7 years with chronic inflammatory diseases of a stomach and duodenum are surveyed. The amylolytic activity of the mixed saliva by Smith-Roy-Ugolev amylolytic method was determined at patients. 2 clinical groups with account of median (the 50th percentile) distribution of amylolytic activity of saliva are created: the first — with indicators below the median (n =72), the second — with indicators above or equal to the median (n =73). The endoscopic research (all children), histological research bioptates (n =63), morphometric analysis of a duodenum mucous membrane (n =26) were applied for the purpose of assessment of a morphofunctional condition of a stomach and duodenum. Results: It is established that predominant duodenum involvement, high frequency of isolated duodenitis, subatrophic and dystrophic changes of a duodenal mucous membrane, bigger intensity and depth of inflammatory process against lower level of nonspecific tissue protection, existence of signs of an allergic inflammation are characteristic for patients with low amylolytic activity of saliva. Conclusion: Determination of amylolytic activity of the mixed saliva at children with inflammatory diseases of a stomach and duodenum is available, noninvasive informative diagnostic test orienting in assessment of a character and extent of involvement of a gastroduodenal mucous membrane and allowing in due time to reveal the patients needing the profound inspection of gastroduodenal area and also to exclude food allergy.

In the article the most frequent problems of breast feeding on a pediatric site are considered. The assessment is given to quality of knowledge and participation extent of local crew staff in «mother-child» system when forming lactation dominant at a nursing woman, correction variants of this work are offered. Among the main obstacles for feeding the child by breast milk there is mother anxiety concerning the amount of lactated milk. The authors of the article pay attention to necessity and possibility of the differentiated approach to the baby cry, and also to importance of mother training in order to make her understand the so-called hungry cry. The balanced approach to supplementary feeding prescription, and in case of its need for the purpose of breast feeding maintenance, the use of a dummy imitating the act of breastfeeding are necessary.

The case of clinical observation over twin brothers (boys E. and N) suffering juvenile idiopathic arthritis is presented in the article. Boys fell ill with a difference in 1 year and 5 months at the age of 1 year and 9 months and 3 years 2 months, respectively. The disease proceeded wavily with the periods of remissions and exacerbations. Resistant articulate syndrome developed in July and November, 2009, respectively. Both children received NSAID, methotrexate combination in a dose of 10 mg/m²/week and sulphasalazine in a dose of 20 mg/kg of body weight per day. To the boy E. it was also prescripted prednisolon in a dose of 10 mg per day. Duration of antirheumatic therapy made 7 and 3 months, respectively. The therapy with a blocker of T-lymphocytes costimulation, abatacept, was initiated for the twins due to the inefficiency of treatment by immunodepressants and prednisolon at the boy E. In 6 weeks of the treatment 50% improvement, in 24 weeks — 90% improvement by criteria of the American College of Rheumatologists (ACR_pedi) were registered. The undesirable phenomena weren’t observed.