Vol 78, No 5 (2023)

The focus on the interests of children has always been inherent in the Russian State, but the most effective measures have been implemented already in this century: 4 years before WHO announced the Global Strategy for Mothers and Children in 2016, Russian President Vladimir Putin signed the Decree on the National Strategy for Children for 2012–2017, in 2017. The President announced the “Decade of Childhood”, and in 2020 The priority of childhood was enshrined in the Constitution of the Russian Federation. In the Russian Federation, the most child-oriented system of children’s healthcare has been built, in medical and scientific organizations of the pediatric profile of the Russian Federation, specialists with medical, biomedical, psychological, pedagogical, and social education are specially trained to work with children of different ages, and scientific research in the field of pediatrics is carried out by specialized researchers within the framework of special programs focused on the needs of children’s healthcare. A detailed analysis of the structure and content of the projects implemented in 2022 has been carried out. Scientific projects involving children compared to 2018, when the implementation of the “Decade of Childhood” program began, which indicates that scientific research in the field of children’s health and development is voluminous, but uncoordinated. Many topics are carried out simultaneously by several scientific and medical or educational organizations, but they are not coordinated in unified scientific and methodological approaches. At the same time, there are systemic problems, to solve which it is necessary to switch to network scientific research of population health; to strengthen the integration of research teams both in the context of interdepartmental cooperation (the Ministry of Education and Science of the Russian Federation and the Ministry of Health of the Russian Federation — Rospotrebnadzor — Federal Medical and Biological Agency), and joint scientific work with other ministries (the Ministry of Education of the Russian Federation, the Ministry of Sports of the Russian Federation, the Ministry of Culture of the Russian Federation, etc.); as well as to improve financial mechanisms for managing scientific activities.

Background. One of the four important components of the formation of cognitive functions is somatic health. But to date, there are no population studies that consider the relationship with cognitive functions and school performance of a large range of somatic factors, which allows us to compare the strength of their hypothetical contribution to cognitive functioning with each other. This study is the second part of a population-based study, the first part of which is presented in the previous publication "A Single-Stage Population-Based Study of the Prevalence of Mild Cognitive Impairment in Children of Secondary School Age". Aims — to determine the main patterns in the relationship between cognitive-academic and somatic factors in a cohort of Russian children, 5th grade students at school. Methods. In Russian schoolchildren of the 5th grades of municipalities representing cities of all federal districts of the Russian Federation, the links with integrative cognitive success, the number of subtests performed at the level of mild cognitive impairment, the results of individual cognitive subtests, academic performance and the leading hand factor were analyzed — the following somatic factors: the presence of skin pathology, bronchial asthma, orthopedic, ophthalmological disorders, visual acuity, body mass index, parameters of the study of the function of external respiration, electrocardiography, ultrasound examination of the thyroid gland, laboratory blood tests. Results. The results of the survey of 1036 participants, 51% of them girls, were admitted to the analysis. It has been established that iron content is directly related to integrative cognitive success and school performance, the relationship is especially strong between subgroups of iron content above and below 26.4 mmol/l. Clinical levels of erythrocytes are more strongly associated with integrative cognitive success and individual cognitive functions than other factors: in erythropenia cognitive parameters are worse. The presence of thyroid cysts directly correlates with some of the worst parameters of cognitive activity. High body mass index and low hemoglobin are associated with poorer academic performance. Conclusions. The results of the study for the first time on a cohort of Russian schoolchildren showed a connection with cognitive activity and school performance of a number of somatic factors, including iron content, which requires further in-depth study.

Background. Irrational selection of biological therapy leads to its inefficiency and early cancellation, aggravating the course of psoriasis and worsening the quality of life, which has negative socio-economic consequences associated with the cost of initiating and providing the patient with a new biological agent. Currently there are no clearly defined strategies and unified protocols for therapeutic tactics regarding the choice and switching of biological drugs in children with psoriasis, which requires research aimed at studying the survival of biological therapy in order to draw up evidence-based recommendations with a high evidence base. Aims — study aims to determine survival rate and significant predictors of biological therapy discontinuation. Methods. A retro-prospective observational study was conducted, which included patients with psoriasis vulgaris, aged 4 to 18 years, who had previously received or needed biological therapy. The data of patients over an 8-year period (from 2015 to 2023) were analyzed. Follow-up was performed from initiation to discontinuation of biological therapy, in cases where outcomes were unknown data was censored. Analysis of therapy survival was carried out using the Kaplan–Meier method with curve construction, the assessment of intergroup differences was carried out using a log rank test. Identification of significant predictors of biological therapy discontinuation was carried out using the Cox multivariate regression method. Results. 430 cases of biological therapy were selected from patients with psoriasis vulgaris aged 5 to 18 years. The highest survival rate of biological therapy was obtained for ustekinumab — 54.9 months, the lowest values — for etanercept (26.7 months). The survival values of adalimumab — 33.9 months and secukinumab — 34.5 months did not statistically significantly differ from each other (p = 0.387). Predictors of early discontinuation of biological therapy were established: presence of a burdened family history (HR = 3.861, p = 0.006); delayed prescription of biological therapy — > 2 years from the date of diagnosis (HR = 1.447, p = 0.045); long-term (> 6 months) use of methotrexate (HR = 3.085, p < 0.001) or cyclosporine (HR = 4.538, p < 0.001) previous to the biological treatment; the presence of comorbidity (inflammatory bowel diseases (HR = 4.938, p = 0.001), metabolic syndrome (HR = 3.947, p < 0.001) or psoriatic arthritis (HR = 2.337, p < 0.001). Conclusions. Proved the inexpediency of long-term immunosuppressive treatment with non-biological drugs and the need for early prescription of biological therapy. In the presence of a burdened family history and disease duration > 2 years since the diagnosis of psoriasis, ustekinumab or secukinumab are recommended as first-line biological therapy in children, the use of which is also associated with a longer duration of treatment and a lower risk of developing adverse events in patient with metabolic syndrome. In active Crohn’s disease, adalimumab is most recommended, followed by a possible switch to ustekinumab. Secukinumab is the drug of choice for patients diagnosed with psoriatic arthritis.

Background. In Russian scientific literature there is a limited amount of data on the prevalence of sensitization to various types of allergens in children. The determination of sensitization profiles is important for the formation of personalized measures for patients. Aim — to reveal the prevalence of sensitization profiles to allergen extracts and components in children with different variants of the atopic phenotype, taking into account regional characteristics. Methods. A cross-sectional study was conducted in 160 children from 0 months to 17 years 11 months with symptoms of allergic rhinitis, food allergy, atopic dermatitis, living in the Moscow metropolitan area. The sensitization to 9 extracts of allergens was analyzed in all children using the ImmunoCAP method, component resolved diagnostics was held with “Allergochip ISAC ImmunoCAP” in 143 patients. Results. sensitization to birch pollen was detected most often (68%), less often (52%) — to timothy grass, (48.1%) — to mugwort. Among food allergens, most often (55% each) children were sensitized to extracts of egg white, wheat flour, less often — cow’s milk (48%), Atlantic cod (15.6%). Analysis of molecular sensitization showed that most often patients were sensitized to Bet v 1 (64%), Aln g 1 (50.3%), Сor 1.0101 (48.3%), Fel d 1 (42.7%) cases. Sensitization to cat/dog allergen components was detected in 79.7% patients. Asymptomatic sensitization to cat allergens was observed in 35.8%, to dog — in 40% patients. More than half of the patients had sensitization to the PR-10 components. From 2 to 10% of patients had sensitization to LTP, most often to the Jug r 3 walnut component in 10.5% patients. Conclusion. The profiles of molecular sensitization in children with various variants of the atopic phenotype were clarified, the prevalence of sensitization to extracts and components of pollen, household, food and animal allergens, the incidence of true and asymptomatic sensitization were determined.

Primary immunodeficiency (PID) constitute a polymorphic group of genetic life-threatening disorders. APDS (activated phosphoinositide 3-kinase δ) represents a rare PID caused by monoallelic gain of function defects in the PIK3CD gene, or monoallelic loss of function defects in the PIK3R1 and PTEN genes. Disease symptoms usually manifest early in life and include recurrent bacterial infections, non-malignant and malignant lymphoproliferation, persistent herpes virus infections and a whole spectrum of autoimmune manifestations. Immunological features include T and B lymphocytes defects. APDS treatment including immunoglobulin substitution and immunosuppression does not always lead to complete remission of the disease. Hematopoietic stem cell transplantation is a curative option leading to disease resolution in 75–80% of the patients. In 2023 FDA approved leniolisib — selective PI3Kδ inhibitor — for APDS treatment. In the clinical trial the preparation demonstrated safety and efficacy for the patients with APDS ages 12 and above. Approval of the drug created specifically to treat this rare variant of PID opens a new era of the targeted treatment for the patients with this orphan disease.