The article discusses the major steps in development of health and social care for the child population in Russian Federation. Principal legislative and regulatory acts covering the medical care provision are specified. Two regional organizational models of health and social care for the child population were presented, they have shown their effectiveness. Principle problems for the development of systematic approach to the organization of health and social care for children in Russia are listed. Moreover, main directions for its development and nearest prospects associated with the implementation of program activities carried out within the framework of the Decade of Childhood.

Background. Immunological potency of 13-valent pneumococcal vaccine (PCV-13) in children with systemic juvenile idiopathic arthritis (SJIA) is still unstudied. Estimates of the genetically engineered biologic drugs (GEBD) effects on pneumococcal vaccination results also remain controversial.

Objective. The aim of the study was to explore the PCV-13 efficacy in patients with SJIA and who is on treatment with monoclonal antibodies against interleukin 6 receptor (tocilizumab) and interleukin 8 receptor beta (canakinumab).

Methods. The study included patients under the age of 18 with SJIA in remission or active form of disease vaccinated with PCV-13. The vaccine was administered in single dose of 0.5 ml intramuscularly in patients on treatment with GEBD or 3 weeks before GEBD administration for the first time (for patients with active disease). Vaccination was considered effective at achievement of the minimum protective level of antibodies to capsular polysaccharide of pneumococcus (anti-SPP IgG; ≥ 7 U/ml) or increase of anti-SPP IgG level ≥ 2 times in 4 weeks after vaccination. The anti-SPP IgG levels were measured with enzyme immunoassay.

Results. The study included 53 patients (27 girls) in remission of SJIA and 25 (16 girls) in active disease. Median age was 13.3 and 10.8 years respectively. Tocilizumab/canakinumab was administrated in 43/10 and 18/7 patients respectively. Minimum significant anti-SPP IgG level and two-fold increase in anti-SPP IgG level were recorded in 49/53 (92%) and 32/53 (60%) patients with SJIA in remission, as well as in 22/25 (88%) and 18/25 (72%) patients in active disease respectively. PCV-13 immunological potency in patients with SJIA in remission and in active disease (in those who were initially administrated and who did not receive GEBD) did not differ.

Conclusion. PCV-13 vaccination allows to achieve protective antibodies level in most of the patients with SJIA in children population regardless of the disease stage and the history of GEBD administration.

Background. Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. It is more typical among Turks, Jews, Armenians, Arabs and nationalities permanently living in the Mediterranean area. Crimean Tatars were not considered as the population where FMF may occur until 2016.
Objective. The aim of the study was to describe the clinical course and outcomes of familial Mediterranean fever in Crimean Tatar children.
Methods. We have studied data from medical records of children under the age of 18 with the diagnosis of FMF verified according to the Eurofever/PRINTO 2019 criteria. The illness onset characteristics were estimated on the last admission to the hospital, as well as aspects of management.
Results. The median age of FMF diagnosis was 9.5 (4; 14) years, time from the first clinical manifestations to diagnosis establishment was 5.5 (2; 9) years. The primary clinical manifestations of SSL were fever and arthritis (n = 16), erysipelas rashes (n = 9/16), peritonitis (n = 8/16), pleurisy (n = 1/17). All patients had knee arthritis, and 4/16 had hip arthritis. 12 children with FMF at debut were diagnosed as acute respiratory infection, 2 — as teething, 2 — as juvenile arthritis. The M694V variant of MEFV gene were revealed in 14/16 patients (3 in homozygous state), M680I and V726A variants were revealed once each. Parents of 8/16 patients were near related (cousins and second cousins). Colchicine intolerance was diagnosed in 2/16 patients, resistance — in 4/16 patients. Genetically engineered biologic drugs (GEBD) were prescribed for 6 patients (canakinumab in 4 cases, tocilizumab in 2 cases). Colchicine and/or GEBD therapy was effective in all patients (lesser frequency, duration and severity of episodes; improvement of laboratory signs of disease activity).
Conclusion. Heterozygous pathological variant M694V of MEFV gene is the most common among Crimean Tatar patients with FMF, when the most frequent clinical signs are fever and arthritis. Every third patient has received GEBD therapy. This therapy was effective in all cases.

Background. The development of central nervous system (CNS) malformations may occur due to antenatal infection. Specific pathomorphological changes in CNS structures require further study.
Clinical Case Description. The autopsy morphological study of the brain of a child who had antenatal toxoplasma and cytomegalovirus infections and who died at the age of 2 years was performed. Thickening of the pia mater with cloudy surface due to edema and focal sclerosis, generalized thrombosis of superior sagittal sinus, sinus rectus and sinus transverses, absence of corpus callosum, major commissural fibers, opened third ventricle and vermis of cerebellum agenesia, as well as mixed forms of obstructive hydrocephalus were revealed. Diffuse gliosis with glial cysts in the periventricular zones were revealed at histological examination of the alba. Pseudocysts filled with toxoplasms were identified in the cysts’ wall. Signs of productive vasculitis were noted. Comparison of pathomorphological changes in the brain and internal organs with anamnestic data of the patient (toxoplasma and cytomegalovirus infection during recent pregnancy, brain abnormality of the fetus on the second ultrasound screening at 21–22 weeks of gestation) and the results of histological examination of placenta was carried out to prove the correlation of antenatal infection with the development of brain anomalies. Pathognomonic signs of cytomegalovirus infection and toxoplasmosis were revealed in the placenta: cytomegalic cells, vascular thrombosis, white infarcts, fibrinoid necrosis of basal lamina, placental hypoplasia. Toxoplasmic cysts were detected in chorionic villae stroma, parietal thophoblast, amnion, decidual tissue.
Conclusion. Antenatal viral-parasitic infection may be the cause of brain anomalies of the fetus. However, the child had no specific signs of cytomegalovirus infection (such as cytomegalic cells — owl eye cells), even when the placenta had pathomorphological changes typical for this infection. It can be assumed that the development of brain anomaly in this child could be associated with toxoplasma infection.

Background. Mixed connective tissue disease (Sharp syndrome) is the rare chronic autoimmune pathology combining various features of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, dermatomyositis and high antibody titer to nuclear ribonucleoprotein. The mixed connective tissue disease may evolve into other systemic diseases over time. Description of any cases of mixed connective tissue disease and its evolution in Russian patients has not been published previously.

Clinical Case Description. The results of observations of the child with clinical and immunological signs of the mixed connective tissue disease followed by the progression of systemic scleroderma symptoms and development of Sjogren's syndrome in the short period of time are presented in the article. Improvement (such as pain attenuation, increase in volume of movements in affected joints, decrease of Raynaud syndrome manifestations duration) was observed on treatment (methotrexate 10 mg/week with subsequent addition of prednisolone 0.75 mg/kg/day).

Conclusion. Timely diagnostics of clinical signs of the systemic diseases debut is crucial for correct patient routing and for achieving of disease improvement.

The article contains the analysis of optimal patterns of supplemental feeding in infants from the point of view of the concept of the First 1000 days and the possibility of fat metabolism disorders prevention. The correlation between the early supplemental feeding (before 4 months of age) with the risk of obesity is noted mainly in socially deprived families with premature termination of breastfeeding. If supplemental feeding was sustained until 5–6 months of age as well as breastfeeding, the risk of metabolic disorder development was minimal. The time and procedure for implementation of supplemental feeding according to the formula «nutrition under the control of the child» is discussed. Specific attention was paid to the excess sugar content in nutritional support for infants as the factor associated with the development of obesity in children and adolescents.

Juvenile localized scleroderma (JLS) is a group of childhood diseases with the main symptom — skin and subcutaneous structures lesions, without any organ involvement. There is active (inflammatory) and fibrotic phase in development of JLS. The JLS treatment during active phase (when skin lesions are reversible) is the most effective. The management is determined by the area and depth of skin lesions, appearance and spread of new lesions, presence of extracutaneous signs of the disease. Topical and systemic immunosuppressants are the basic therapy for JLS. The use of antibiotics is not suggested. Clinical scores (LoSCAT), ultrasound, thermography and magnetic resonance imaging are recommended to estimate the treatment efficacy.

General information on atopic dermatitis (AD), its history and epidemiological significance are presented. Multiple etiopathogenetic factors affecting disease manifestation are identified. The absence of specific tests, laboratory and histological studies, that can help in correct AT diagnosis, is mentioned. Most of AT diagnostic schemes clarify main (major) and additional (minor) signs of disease. Clinical manifestations of AD minor signs crucial for revealing of subacute and chronic course, as well as onset of incomplete remission of disease are described. Topical AD management regimens are presented. Pathogenetic verifications for emollients and regenerating agents use are presented.