The historical and chronological sequence of congresses of the All-Union Society of Pediatricians presented in the article reflects  the significance of scientific works, Society charters, reports, resolutions of congresses and reports of the Society chairmen. The discussion of the most topical problems of scientific and clinical pediatrics, presentation of the problems of medical care for children's population in the pediatric forums contributed to the decrease of infant morbidity and mortality, preservation of the lives of millions of children in the USSR.

Neurogenetics is a thriving young science greatly contributing to the generally accepted concept of the brain development in health and disease. Thereby; scientists are not only able to highlight new key points in traditional ideas about the origin of diseases; but also to completely rethink their view on the problem of pathology development. In particular; new data on neurogenetics of perinatal affections of the central nervous system (CNS) has appeared. Genetic factors in varying degrees affect perinatal hypoxic-ischemic CNS affections.

Prematurity determination stays the most studied among them. Nevertheless; there is increasing evidence of significant epigenetic regulations of neuro-expression caused by hypoxia; malnutrition of a pregnant woman; stress; smoking; alcohol; drugs that either directly pathologically affect the developing brain; or form a brain phenotype sensitive to a perinatal CNS affection. New data obliges to change the approaches to prevention of perinatal CNS affections.

Respiratory viral infections in infants and preschool children are often accompanied by repeated episodes of wheezing; which is associated with an increased risk of asthma development. Mechanisms of connection between a viral infection and the subsequent development of allergic inflammation in the respiratory tract are insufficiently studied. Unfortunately; despite the existence of such connection; it is difficult to predict the risk occurrence on the basis of clinical symptoms. Determination of nitrogen oxide in the exhaled air; of the number of eosinophils in the peripheral blood and of bronchoalveolar lavage fluid as well as of IgE was suggested as predictors of allergic inflammation in the airways. But these surrogate markers in patients including children with asthma have a moderate diagnostic accuracy. Their use as a marker of airway eosinophilia can cause a significant amount of false-positive and false-negative results. Recent studies have shown that determination of serum periostin more accurately confirms eosinophilic airway inflammation. The possibility to study this marker in different variants of wheezing in children as a possible predictor of asthma development is discussed based on its reliability; stability and a small variation in determination of eosinophilic inflammation in the airways with symptoms of bronchial obstruction.

The lecture gives characteristics of leukaemia: tumours of hematopoietic cells, its types, etiology and pathogenesis key links, clinical manifestations and mechanisms of its development, caused systemic disorders, principles of its diagnosis and treatment.

Background: Addition of genetically engineered biological agents in the paradigm of juvenile idiopathic arthritis (JIA) treatment significantly increased the efficacy of the antirheumatic therapy in patients with this severe chronic disease.

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

Methods. The open prospective study included patients with JIA without systemic manifestations treated with etanercept.

The treatment was carried out on the basis of the rheumatological department of the Scientific Center of Children's Health (Moscow) in the period from December 2009 to August 2014. To assess the therapy efficacy in a year, we used the improvement definition for pediatric patients of the American College of Rheumatology (ACR_pedi) 30, 50, 70, and 90. Reaching the stage of inactive disease/remission were recorded under С. Wallace criteria and threshold value of the JIA activity index (JADAS71).

Results. Totally, the study included 197 patients. After 1 year from the treatment beginning, the ACR_pedi30 improvement was reported in 179 (90.9%) patients, ACR_pedi50 — in 177 (89.8%), ACR_pedi70 — in 168 (85.3%), and ACR_pedi90 — in 135 (68.5%). The stage of inactive disease/remission under C. Wallace criteria was ascertained in 132 (67.0%), under JADAS71 index — in 92 (46.7%) patients.

Conclusion. After 1 year of etanercept treatment, the stage of inactive disease/remission and the improvement under ACR_pedi90 criterion have been reported in almost half (45.7%) of patients with juvenile idiopathic arthritis without systemic manifestations. The predictors of a high response to etanercept were a short disease duration, less number of used disease-modifying antirheumatic drugs, and a low level of C-reactive protein in the blood serum before etanercept prescription.

Background. Infectious diseases remain the leading causes of morbidity and mortality worldwide. Against the background of ever-increasing resistance of bacteria, it is necessary to develop effective measures aimed at the structure optimization of consumption of antimicrobial agents. Therefore, the pharmacoepidemiological data describing either the consumption of antimicrobial agents or the resistance level to them is required.

Objective: Our aim was to assess the antimicrobial load in term and preterm newborns during their stay in the maternity wards and/or in the intensive care units (ICU).

Methods. A retrospective cross-sectional study has been carried out. The analysis included data of 419 newborns from 5 medical centers. The study has been carried out as part of a multi-purpose program on antibiotic resistance containment being held in St. Petersburg from 2014.

Results. The antimicrobial load on the child's body expressed in days of the antimicrobial therapy was 1,838 per 1,000 patient-days in children staying in the ICU for not more than 16 days, and 1,434 per 1,000 patient-days when staying in the ICU for more than 16 days. The average duration of the antimicrobial therapy is 28 days in the ICU and 5 days out the ICU (department for newborns, physiological departments). The most commonly, the children were treated with ampicillin — 384 (92%), gentamicin — 254 (61%), and fluconazole — 150 (36%). The prevalence of off-label prescriptions was 41% (of 1,557 analyzed prescriptions), unlicensed prescriptions — 10%. Congenital infections were the main reasons for prescription of antimicrobial drugs — 225 (62%).

Conclusion. The high anti-bacterial load in newborns has been established. The indicator 'days of antimicrobial therapy' in the ICU is 4 times higher than that in the United States. The prevalence of off-label and unlicensed prescriptions is comparable with foreign data.

Background: Children with type 1 diabetes (T1D) are at high risk for severe influenza.

Objective. Our aim was to assess immunogenicity and safety of a trivalent subunit immunoadjuvant influenza vaccine in children with T1D.

Methods. The prospective study carried out from September to November 2014 included children with T1D at the age from 3 to 17 years vaccinated against influenza by a trivalent subunit immunoadjuvant influenza vaccine and unvaccinated due to parental refusal. Anti-influenza virus antibodies were determined by passive hemagglutination reaction. Vaccine safety was assessed by a number of adverse events occurred within 6 days after its administration. Observation was carried out during 1 year after vaccination (to October-November 2015 inclusive).

Results. Of 780 children with T1D followed-up by endocrinologists in Nizhny Novgorod region, the study included 94 children — 44 vaccinated and 50 unvaccinated matched by sex and age. As for influenza A/H1N1/California/07/09 virus strain, the seroprotection rate (antibody titre > 1:40) was 84%, seroconversion rate (4-fold increase in antibody titre) was 66%, seroconversion factor (average increase of the antibody titre and 95% confidence interval) was 20.6 (10.4–30.9), as for influenza A/H3N2/Texas/50/12 virus, it was 98%, 41%, and 9.9 (3.2–16.6), as for influenza B/Massachusetts/2/12 virus, it was 77%, 49%, and 7.7 (4.0–11.4), respectively. After 1 year, the conditionally protective antibody titres (> 1:40) to influenza A/H1N1/California/07/09 virus were in 32/43 (74%), to influenza A/H3N2/Texas/50/12 virus — in 38/43 (88%), to influenza B/Massachusetts/2/12 virus — in 25/43 (58%) vaccinated children, in unvaccinated ones — 23/49 (47%) 37/49 (76%), and 6/49 (12%), respectively. Mild local adverse events were observed in 5/44 (11%), general — in 1/44 (2%), intercurrent diseases — in 8/44 (18%) vaccinated children.

Conclusion. Immunogenicity and safety of a trivalent subunit immunoadjuvant influenza vaccine in children with T1D have been confirmed.

Background. Blocking of B lymphocytes producing autoantibodies in systemic lupus erythematosus (SLE) may be an effective strategy for pathogenetic treatment, including in children.

Objective: Our aim was to assess the efficacy and safety of rituximab treatment (chimeric monoclonal antibodies to CD20) in children with severe SLE refractory to glucocorticosteroids and immunosuppressants.

Methods. We studied the case histories of children with SLE admitted to the rheumatology department of the SCCH between 2004 and 2013. The treatment results were assessed by the SELENA SLEDAI Disease Activity Index, Damage Index scale, and dynamics of the values of laboratory disease activity indices.

Results. We analyzed the results of rituximab treatment in 12 patients for 24 weeks, 11 of them — for 48 weeks. The preparation was administered intravenously at a dose of 375 mg/m² of the body surface area to administer 4 times weekly (n = 10) or 2 times with an interval of 2 weeks (n = 2). After 48 weeks of rituximab treatment, SELENA SLEDAI index values decreased from 16 (11, 21) to 1 (0, 2) (p < 0.001), Damage Index — from 3.5 (1.0, 5.0) to 1.0 (0.0, 3.0) (p = 0.024), complement component C4 concentration increased from 0.13 (0.04, 0.19) to 0.43 (0.18, 0.50) g/l (p = 0.017), the number of antibodies to double-stranded DNA decreased from 73.5 (11.5, 245.0) to 1.9 (0.0, 40.0) IU/ml (p = 0.004). Depletion of B lymphocytes was recorded in all patients after 24 and 48 weeks of rituximab treatment. Prednisolone dose was reduced from the initial 0.80 (0.43, 1.00) mg/kg per day to 0.20 (0.17, 0.30) after 48 weeks (p = 0.025). Disease exacerbations during 48 weeks have not been reported. Such serious adverse events as pneumonia (n = 2), neutropenia (n = 5), Herpes zoster infection (n = 2), hypogammaglobulinemia (n = 8) were noted.

Conclusion. High efficacy and satisfactory risk-benefit profile of rituximab treatment in children with severe SLE refractory to glucocorticosteroids and immunosuppressants have been shown.

 Differentiated Approach to Non-Bacterial Osteomyelitis Treatment in Children: the Retrospective Study Results Background. Low efficacy of the therapy of children with non-bacterial osteomyelitis remains a topical problem of modern pediatrics and rheumatology.

Objective: Our aim was to assess the efficacy and safety of non-bacterial osteomyelitis treatment in children.

Methods. A retrospective study of the case records of children with non-bacterial osteomyelitis has been carried out. The therapy efficacy (remission) was assessed by the following criteria: absence of fever, pain, clinically active lesions, laboratory disease activity. Laboratory and radiological methods were carried out during diagnosis of the pathological process, and subsequently every 3–6–12 months to assess disease activity depending on the initial injury extent of the skeleton. Clinical activity assessment was carried out by means of a visual analogue scale.

Results. We analyzed treatment results of 52 children (68 therapy courses) with non-bacterial osteomyelitis. Nonsteroidal anti-inflammatory drugs were administered to 19 patients, sulfasalazine — to 7, methotrexate — to 9, pamidronic acid — to 18, inhibitors of tumour necrosis factor (TNF) α  — to 15. Remission was achieved in 10 (53%) 4 (57%) 4 (44%), 16 (89%), and 11 (73%) patients (p = 0.001), respectively. Adverse events during treatment occurred in 21 (40%) children. No serious adverse events were reported.

Conclusion. The efficacy of different regimens has been shown. It was found that pamidronic acid drug and TNF  inhibitors were the most effective in treating children with non-bacterial osteomyelitis.

Neurocutaneous melanosis is part of a group of hereditary diseases characterized by large and/or multiple pigmented nevi, melanosis or melanoma of the pia mater, with no evidence of malignant skin lesions and involvement of other organs. The disease was described over 150 years ago, but its pathogenesis has not been studied yet, and treatment methods have not been developed yet. Different disease courses due to the pronounced polymorphism of clinical symptoms complicate the diagnosis, and the low efficacy of the symptomatic treatment worsens the disease prognosis. The article describes the experience of managing children with phakomatoses not similar to each other neither in debut and course nor in response to the therapy and prognosis. Early diagnosis of neurocutaneous melanosis in children allows to carry out timely symptomatic treatment and dynamical monitoring, and to improve the survival of patients.

The article presents a case of a successful canakinumab application (preparation of monoclonal antibodies to 1 interleukin) for undifferentiated autoinflammatory syndrome in a patient with refractoriness to oral glucocorticosteroids and genetically engineered biological drugs with a different action mechanisms. After 8 weeks of canakinumab therapy, systemic manifestations were cropped, and laboratory parameters of the disease activity were normalized. During the entire care period (12 months), neither serious adverse events nor increased incidence of acute respiratory infections have been reported.