[Modern possibilities for transcatheter pulmonary valve replacement]. / Sovremennye vozmozhnosti transkateternogo protezirovaniya klapana legochnoi arterii.

The literature review is devoted to transcatheter pulmonary valve replacement. The authors summarize the indications, clinical data and current capabilities of transcatheter pulmonary valve replacement. The authors also overviewed modern valves for transcatheter pulmonary artery replacement. Effectiveness of transcatheter pulmonary valve implantation has been substantiated. Various studies comparing the outcomes of different valve systems for endovascular implantation were analyzed. The authors concluded the prospects for transcatheter pulmonary valve implantation.

[The mortality of children population].

The article presents comprehensive medical statistical analysis of indicators and causes of mortality of children population of the Russian Federation in 2017-2021. It is emphasized that in Russia, in conditions of extremely unfavorable demographic situation, the hyper actual task is to preserve life of every child. It is demonstrated that crucial role in mortality of children population is played by not only infant mortality and mortality of children aged 1-4 years, but also by mortality of children of older ages. The children population mortality still keeps gender and residence differences. The problem of reliable registration of infant mortality is to be revisited since part of newborns born alive are classified after birth as stillborn. Beginning from 2018, the first place was taken by the class "Injuries, poisonings and some other consequences of external causes" driving back the class "Individual conditions occurring in perinatal period". Thus, measures of preventing negative impact of social factors on children health continue to be an important component of modern system of health care of children population. The directions of measures reducing children mortality in Russia are proposed.

Are the Goals of Therapy Achievable in Patients with Rheumatoid Arthritis Receiving Upadacitinib in Real Clinical Practice?

The aim of the study was to evaluate the effectiveness of UPA in RA patients in real clinical practice after 3 and 6 months of therapy. The study included 63 RA patients with high activity of the disease. Activity was assessed according to the DAS28(ESR), DAS28(CRP), SDAI, CDAI; functional ability to HAQ; quality of life to the EQ-5D; disease activity according to the patient's RAPID-3 index; the level of depression and anxiety to the HADS scale. The effectiveness of therapy was evaluated after 3 (n = 45) and 6 (n = 31) months of UPA therapy. Remission or low activity of the disease by 3 months of therapy was achieved by most patients: remission of 69.8% of patients, low activity of the disease-16.3% of patients. Moderate or high activity persisted in 13.9% of patients. By the 6th month of UPA therapy, the number of remissions reached 90%, low activity 3.3%, moderate activity persisted in 6.7% of patients, high activity of the disease was not in any patient. 20% improvement in function was achieved in 71.8% of patients by the 3rd month of therapy and in 77.8% by the 6th month of treatment; the difference in average HAQ values by the 3rd month of therapy was 0.38 points, by the 6th month-0.58 points. After 3 months of follow-up, 31.1% of patients continued taking GC, by 6 months-24.2%. The dose of GC was reduced from an average of 7.23 to 5.6 mg/s. The percentage of patients requiring NSAIDs decreased from 95.2 to 35.6% and 33.3%, respectively. DMARDs continued to be received by 75.6% of patients by 3 months and 69.7% by 6 months of follow-up. Achieving remission or low activity of the disease in patients with RA receiving UPA in real clinical practice is possible in most patients. A rapid decrease in inflammatory activity is accompanied by a significant improvement in the functional state and quality of life of patients. UPA therapy reduces the need for the use of NSAIDs and reduces the dose of GC in a third of patients.

[The prevention of disability as a leading priority of caring of mother and child health].

The prevention of disability in children and support of families fostering disabled children and children with limited capacities in health are included into number of main priorities of the state social policy of the Russian Federation. Thereupon, the project "Childhood Decade" is an important stage that includes social care of disabled children and children with limited capacities in health and their integration in modern society according the Decree of the President on implementation of state policy in the field of family and children care. The underestimation of children disability is supposed to be present produced by such causes as social motivation of family, complicacy of legal official registration, harsh requirements of medical social expertise services, inadequate awareness of medical staff about criteria of disability. According the WHO European Regional Bureau, in Russia there are 250 000 children with limitations of life activity mainly caused by somatic pathology, who have no status of disabled person and no state social support. The prevention of children disability is considered as a system of measures of caring mother and child health all along childhood. The priority directions of prevention considering causes of development of disability and its structure according particular disease are marked. The data is presented related to modern technologies of decreasing genetic load on population from position of prevention of hereditary, inborn and orphan diseases. In particular studies non-infectious pathology acquiring greater significance in development of disability in children of various age groups since neonatal period is considered. The new directions of research are proposed to affect the processes of incapacitation of child on more profound pathogenic level and to develop organization of preventive activities in this area.

[All-Russian Consensus on Diagnosis and Treatment of Celiac Disease in Children and Adults]. / Vserossiiskii konsensus po diagnostike i lecheniyu tseliakii u detei i vzroslykh.

The paper presents the All-Russian consensus on the diagnosis and treatment of celiac disease in children and adults, which has been elaborated by leading experts, such as gastroenterologists and pediatricians of Russia on the basis of the existing Russian and international guidelines. The consensus approved at the 42nd Annual Scientific Session of the Central Research Institute of Gastroenterology on Principles of Evidence-Based Medicine into Clinical Practice (March 2-3, 2016). The consensus is intended for practitioners engaged in the management and treatment of patients with celiac disease. Evidence for the main provisions of the consensus was sought in electronic databases. In making recommendations, the main source was the publications included in the Cochrane Library, EMBASE, MEDLINE, and PubMed. The search depth was 10 years. Recommendations in the preliminary version were reviewed by independent experts. Voting was done by the Delphic polling system.

[The evaluation of quality of implementation of preventive medical examinations of underage persons in the Russian Federation].

The analysis was implemented concerning informational statistic data characterizing health of children population of different age groups in the Russian Federation on the basis of results of dispensarization in its federal okrugs and subjects in 2014. The purpose of the study was to discover ways and modes of developing and increasing efficiency of preventive examinations of underage population. The following indices were analyzed: coverage of children population by preventive medical examinations, distribution according health groups and medical groups for physical culture involvement, level and stricture of established total and primary morbidity, rate of dispensary registration, requirements in additional consultations, examinations and treatment in out-patient condition, day hospital, day-and-night hospital and also coverage with all these medical services. In the most of the subjects of the Russian Federation a high level of coverage of underage population with preventive medical examinations is registered. The percentage of healthy children population in the Russian Federation comprises more than one third of all covered by dispensarization. The significant variations in indices of rate of healthy children and children with functional disorders and chronic diseases in subjects of the Russian Federation is determined by quality and accessibility of medical care at the regional level. The established total and primary morbidity of children population in significant percentage (more than one third) of the subjects has a level higher than a national one. The leading causes of morbidity in children are diseases of respiratory system, musculoskeletal system, nervous system. In adolescents, these causes are diseases of musculoskeletal system, eye diseases and diseases of respiratory system. Despite high prevalence of chronic pathology in children population of the Russian Federation, the guidelines concerning treatment and rehabilitation on the basis of results of preventive medical examinations were developed in inadequate scope. The regional characteristics of the results of dispensarization are established. These results made it possible to sort out the most unfavorable territories and to determine defects in its organization and absence of continuity between medeical institutions providing the given type of medical services.

[Technologies for Complex Intelligent Clinical Data Analysis].

UNLABELLED: The paper presents the system for intelligent analysis of clinical information. Authors describe methods implemented in the system for clinical information retrieval, intelligent diagnostics of chronic diseases, patient's features importance and for detection of hidden dependencies between features. Results of the experimental evaluation of these methods are also presented. BACKGROUND: Healthcare facilities generate a large flow of both structured and unstructured data which contain important information about patients. Test results are usually retained as structured data but some data is retained in the form of natural language texts (medical history, the results of physical examination, and the results of other examinations, such as ultrasound, ECG or X-ray studies). Many tasks arising in clinical practice can be automated applying methods for intelligent analysis of accumulated structured array and unstructured data that leads to improvement of the healthcare quality. AIMS: the creation of the complex system for intelligent data analysis in the multi-disciplinary pediatric center. MATERIALS AND METHODS: Authors propose methods for information extraction from clinical texts in Russian. The methods are carried out on the basis of deep linguistic analysis. They retrieve terms of diseases, symptoms, areas of the body and drugs. The methods can recognize additional attributes such as "negation" (indicates that the disease is absent), "no patient" (indicates that the disease refers to the patient's family member, but not to the patient), "severity of illness", disease course", "body region to which the disease refers". Authors use a set of hand-drawn templates and various techniques based on machine learning to retrieve information using a medical thesaurus. The extracted information is used to solve the problem of automatic diagnosis of chronic diseases. A machine learning method for classification of patients with similar nosology and the methodfor determining the most informative patients'features are also proposed. RESULTS: Authors have processed anonymized health records from the pediatric center to estimate the proposed methods. The results show the applicability of the information extracted from the texts for solving practical problems. The records ofpatients with allergic, glomerular and rheumatic diseases were used for experimental assessment of the method of automatic diagnostic. Authors have also determined the most appropriate machine learning methods for classification of patients for each group of diseases, as well as the most informative disease signs. It has been found that using additional information extracted from clinical texts, together with structured data helps to improve the quality of diagnosis of chronic diseases. Authors have also obtained pattern combinations of signs of diseases. CONCLUSIONS: The proposed methods have been implemented in the intelligent data processing system for a multidisciplinary pediatric center. The experimental results show the availability of the system to improve the quality of pediatric healthcare.

[Pediatric Health Quality Assessment in Different Regions of Russian Federation].

Background: The quality of pediatric healthcare is a cornerstone for good maternal and infant health. Aims: To evaluate the quality of healthcare in secondary and tertiary regional pediatric hospitals in the Russian Federation. Methods: Healthcare quality assessment was performed in 21 pediatric hospitals (tertiary, n=5; secondary, n=16) of four regions. The WHO recommendations were used. Results: In all regions, similar traits of inpatient pediatric healthcare determining a poor quality were observed. These included low preparedness for emergent care at admission departments; a high rate of unjustified hospitalization due to lack of clear indications for inpatient care; a widespread polypharmacy and unnecessary painful procedures and treatment; inadequate unjustified antimicrobial and parenteral therapy. Conclusions: The revealed identity of problems in different regions of the country allows to consider a common strategy to overcome them, which, obviously, should primarily involve education of medical personnel, restructuring of hospital beds to increase day care beds, increasing the clinical expert work in hospitals.

[Nutritional management of kidney diseases in children].

The prevalence of various kidney diseases in children remains high in recent decades. Adequate nutrition management can enhance the effectiveness of drug treatment, slow the frequency of relapses andprevent the progression of the disease. The article is devoted to modern approaches to diet therapy in various kidney diseases in children with the defeat of tubular and glomerular appa ratus. For the first time the therapeutic diets for children with various kidney diseases are presented. Particular attention is paid to diet therapy in nephrotic syndrome (steroid-responsive and steroid-refractory). Dietary approaches with modern formulas for enteral nutrition in cases of steroid therapy complications in children with renal insufficiency (in predialysis stage and on dialysis) are described. Differentiated nutritional approaches for patients with different types of crystalluria are separately presented.

[Medical and Social Problems of Assisted Reproductive Technologies from the Perspective of Pediatrics].

The article presents a literature review over the last few years devoted to the health status and development peculiarities of children born using assisted reproductive technologies (ART) procedures. The statistics shows an explosive increase in the frequency of ART application as a fertility treatment method. The presented data analysis reflects the perinatal outcomes after ART in children, the frequency of congenital malformations and genetic diseases in this population, possible long-term malconditions and pathologies in children born using ART. The overwhelming majority of investigators consider the adverse effect of ART on a child's body to be the result of prematurity and multiple pregnancy (transfer multiple embryos followed by partial reduction). It is stated that the widespread introduction of ART may contribute to the vertical transmission of parental infertility factors in the population. The application of ARTprocedures in some cases is associated with controversial ethical and legal issues (surrogacy, oocyte donation). Further improvements in ART procedures (preimplantation training, medical and genetic diagnosis, reducing the frequency of multiple pregnancy) cannot be stated as an alternative to the general medical and social prevention of reproductive disorders in adolescents and youth.

[The regularities and tendencies of infant and children mortality in the Russian Federation].

The article presents dynamics and causes of infant and children mortality in the Russian Federation during post-soviet period (1990-2012) in conditions of implementation of demographic policy and reforming of health care system. The data of official statistics of Rosstat (1990-2012) is analyzed. The comparative analysis with similar indicators in countries of European Union was made. The significant decreasing of infant mortality more than twice at the expense of all its components (early neonatal, neonatal, post-neonatal mortality) and because ofalmost all causes (conditions of perinatal period, infectious diseases, respiratory organs diseases, malformations, accidents) is established In the Russian. Federation, the characteristic of this indicator is decreasing of neonatal mortality and mortality and increasing of post-neonatal mortality in contrast with countries of European Union where its decreasing occurs just at the expense of late losses. It is demonstrated that this particularity is conditioned by under-registration of infants died in early neonatal period. The higher level of infant mortality in rural territories is established. However, the gap between urban and rural indicators shortens. In the Russian Federation infant mortality has regional characteristics and the Siberian and Far-East region are the most unfavorable ones. The mortality of children aged before 5 decreases. However, its level is still higher than in countries of European Union. Among causes of death of children in this age group the first places are for external factors and significance of malignant neoplasms increases. Therefore, development of system of mother and child health care in the Russian Federation made it possible to significantly decrease infant and children mortality.

[The main principles of concept of development of palliative care to children and adolescents].

The article substantiates actuality of elaboration of the concept of development of palliative care of children and adolescents. The analysis of legislative and normative legal base, international documents, scientific studies related to this issue are analyzed. The situation in the regions ofthe Russian Federation is examined concerning organization of palliative care of children and its forms, provision of pharmaceuticals and specialized equipment and professional training of specialists. The demand of children population in palliative care in Russia is calculated according data of 2012. The results of carried out study are used as a basis of determining measures of development of system of palliative care of children and adolescents in the Russian Federation to propose as a foundation for the National concept as a necessary condition for organization of effective and efficient service.

[The automated analysis of anti-nuclear antibodies using technique of indirect reaction of immunofluorescence with application of HEP-2-cells].

The identification of antinuclear antibodies in blood serum based on indirect reaction of immunofluorescence using cells of line HEp-2 (IRIF HEp-2)--a "golden standard" and key screening technique of laboratory diagnostic of autoimmune rheumatic diseases. The automated systems of interpretation of samples offluorescence promote standardization and increase effectiveness of detection of content of antinuclear antibodies with IRIF HEp-2 technique. The study was organized to comparatively analyze automated and visual interpretation of results of IRIF HEp-2 in detection of content antinuclear antibodies in patients with rheumatic diseases. The level of antinuclear antibodies in blood serums of 1178 patients with rheumatic diseases was detected using IRIF HEp-2 technique. The results of IRIF HEp-2 were evaluated by visual microscopy and using automated platform "AKLIDES". The degree of consistency of positive/negative results of detection (k = 0.5), types (k = 0.7) and titers/intensity of fluorescence (k = 0.45) of antinuclear antibodies under automated and traditional interpretation of IRIF HEp-2 was "good". The discordance of positive/negative results of analysis of content of IRIF HEp-2 was established in 18.5% of patients. The automated technique more often detected homogeneous (37.6%) and speckled (32.3%) fluorescence of nucleus. At the same time, there were no differentiation of type of fluorescence in 21.4% of patients. The visual technique detected mixed type of fluorescence in blood serums of most of the patients (72.8%). The mixed fluorescence was identified by system "AKLIDES" as homogeneous (40.5%), speckled (32.7%), nucleolar (2.4%), centromeric (0.9%), undifferentiated (23.5%). Under visual analysis of samples of fluorescence with undifferentiated type of fluorescence was identified as mixed (79.8%), homogeneous (5.9%) and speckled (14.3%). The titers of antinuclear antibodies less than 1:160 associated with intensity of fluorescence 0/B±; 1:160-0, B±, +, ++; more than 1:1280--+++, ++++. In common practice the automated system "AKLIDES" permits identifying positive/negative results of detection of content of antinuclear antibodies comparably with "classic" visual technique of interpretation of IRIF HEp-2 and prognosticate maximal finite titer of antinuclear antibodies in serums in patients with rheumatic diseases according intensity of fluorescence. To confirm results of automated evaluation of types of nuclear fluorescence and to specify titers of antinuclear antibodies it is recommended to apply additional expert visual analysis of positive samples of fluorescence.

[Maintenance and health promotion of adolescent--pledge of sustainable development of society and state (current status of the issue)].

The article presents submitted data of population dynamics of adolescent (10-17 years old) in Russian Federation over the period of 1995-2012 In the presence of reduction by 8.2 million of adolescent population the top-priority task of adolescent health and life maintenance was declared. The article deals with physical growth and development statistics of schoolchildren of 15-19 years old: as opposed to peers of 80-es increase of length, body weight and circumference of chest as well as reduction of muscle strength is observed. On the ground of the data analysis of authoritative statistical reporting the increase of morbidity rate by factor of 1.4 was detected over the last 10 years. It has been established that the true morbidity level is above the authoritative by the factor of 1.5. The number of adolescent at the age of 10-15 referred to 1st and 2nd health groups decreased almost on 20%, at the same time pathological processes become chronic. The reproductive and mental health of adolescent at the age of 15-17 years was analyzed over the last 10 years. The rate of mental health disorders in adolescent living in country is more than in the peers living in cities by the factor of 1.2-1.4. The rate of period disorders, salpingitis and oothecitis increased in girls under 10 years and ones at the age of 15-17. The rate and structure of child disability was analyzed. It was fixed that leading causes of adolescent disability are mental disorders, diseases of nervous system, congenital anomalies. The most commonly encountered seed of physical dysfunction is capacity to study. It is demonstrated that there is misreporting on both child disability in totally and adolescent disability in Russian Federation. After the analysis of particular provisions of legislation concerning medical and social issues of child disability the week points were detected. The morbidity rate of adolescent was studied: primary cause of death in adolescent is extrinsic factor (more than 70%), the leading one is suicide. Road traffic accidents and accidental poisoning comes then. The medical and social consequences of high morbidity, disability and mortality rates of adolescent were considered as labour, reproductive and military potential of society and state. Short-term and long-term plans focused on decrease of morbidity, disability and mortality rates of adolescent were formulated on the basis of submitted data. search for solution is supposed to be nationwide and local as well (public health service, education, social protection).

[Trends of infant and child mortality in the Russian Federation in the period of 1990-2012].

OBJECTIVE: Dynamics and causes of infant and child mortality in the Russian Federation in post-Soviet period (1990-2012) under conditions of demographic policy implementation and healthcare system reforming are presented. METHODS: Federal State Statistics Service's official statistics data (1990-2012) had been analyzed. The data was compared with similar rates of European Union countries. RESULTS: It's been revealed that infant mortality decreased (more than in 2 times) (from 17.4 per thousand to 8.6 per thousand) through all its level indicators (early neonatal, neonatal, post-neonatal mortality) and due to almost all basic reasons (perinatal disorders, infectious diseases, respiratory diseases, congenital anomalies, accidents). Infant mortality rate in rural areas is higher (10.1 per thousand and 8.1 per thousand), however, the gap between rates in rural regions and in cities is decreasing. In Russian Federation the infant mortality has regional distinctions, thus Siberian and Far Eastern regions are the least advantaged areas. Mortality of children under 5y.o. is declining (from 21.3 per thousand to 10.6 per thousand), but its rate is still higher than in European Union countries (3.5 per thousand). External factors occupy the top position as a death cause of infants and toddlers; the number of clinical cases with a diagnosis of malignant neoplasm increases. CONCLUSION: Thus, improving of the system of motherhood and childhood protection in the Russian Federation has resulted in significant reduction of infant and child mortality rate.

[Palivizumab: four seasons in Russia].

In 2010, the Russian Federation (RF) registered palivizumab--innovative drug, based on monoclonal antibodies for passive immunization of seasonal respiratory syncytial virus (RSV) infection in children of disease severe progress risk group, which include primarily premature infants, children with bronchopulmonary dysplasia and hemodynamically significant congenital heart disease. Currently, palivizumab is included in the list of recommended medicines and medical care standards of different countries, including Russia. In the review the results of Russian research on the progress of RSV infection, its epidemiology and immunization experience gained over the 2010-2014 period are summarized in relation to the foreign data. During the four epidemic seasons palivizumab immunization covered more than 3,200 children of severe RSV infection risk group with a progressive annual increase in the number of patients who received the drug. Geography of palivizumab immunization is also greatly expanded in our country during this time. If during the first two seasons measures of immunization were taken mainly in Moscow and St. Petersburg, at the present time, thirty one territorial entities of the Russian Federation have the experience in the drug application. Analysis of the results of RSV infection immunization (made in several regions) confirms the high clinical efficacy and palivizumab safety already demonstrated in international studies. In addition, the analysis presents the potential to improve the efficiency of the integrated RSV infection immunization programs, realizing in the establishment of high-risk child group register, adequate counseling for parents, as well as the development of the routing of patients and coordination of interaction between different health institutions during the immunization.

[The condition and problems of adolescents' health in Russia].

The article presents data concerning health condition of adolescents aged 15-19 years in the Russian Federation during 2002-2012. The official statistical data concerning morbidity, disability and mortality of adolescents were analyzed. In children of this age group increasing of morbidity, disability and decreasing of mortality are established. The special studies demonstrated that true indicators of morbidity of adolescents exceed official indicators up to 1.5-2 times. The morbidity of adolescents is characterized by increasing of socially significant pathology and diseases related to behavioral risk factors. In adolescent age, the unfavorable tendencies in psychic and reproductive health are observed. In the structure of disability mental disorders and behavior disorders, diseases of nervous system and inherited malformations are in the lead. The principal limitation of vital activity is "capacity to learning". It is demonstrated that in the Russian Federation there is underestimation of cases of disability among children and adolescents.

[The organizational principles and technologies of prevention of the XXI century].

The article considers organizational principles, technologies and criteria of effectiveness of actual preventive activities considering indicators of health condition of children and adolescents. The structures participating in implementation of general and specific prevention among children and adolescents are presented. The structures' functions are enumerated concerning development of succession and unity of preventive environment. The particular attention is paid to development of informational and telecommunication technologies in the fields of health care legal support of health care of children and adolescents, manpower development in area of preventive pediatrics.

[Evaluation of premature infants nutritional status by air plethysmography: first Russian prospective study].

In this article results of first Russian study on premature infants body composition in association with trend of their physical development within the first month and according to way of feeding. 30 preterm newborns without signs of intrauterine hypotrophy at birth with gestational age less than 35 month were examined by air body plethysmography. The infants were examined reaching term corrected age. Less levels of fat-free mass and increased percentage of fat were found in premature infants. The negative association between gestational age and the specific content of fat was found. This increases the risk of long-term metabolic diseases in preterm infants. It is found that preterm infants fed by fortified human milk have a more optimal body composition (less body fat levels) than infants fed by formula. The found consistent patterns confirm the necessity of supplying preterm infants with human milk for more adequate formation of plastic processes.