The article presents the text of the authors' report at the plenary session of the XX Congress of Pediatricians of Russia (dated February 16, 2018) dedicated to the centenary of the Soviet state mother and child welfare system. The features of its formation and development were described. The most important achievements in the field of child health care were outlined. Attention is focused on the personalities of the first facilitators of pediatric healthcare in Soviet Russia. Authors summarise the findings resulting from the history of the Soviet pediatric service.

The training program for a modern pediatrician involves the study of Pedagogy in medical residency in specialty 31.08.19 Pediatrics. The urgency of such training for the future doctor is uncontroversial today. Currently the experience of studying this discipline has been gained in Russian medical universities. At the same time this work requires further improvement and exchange of experience with colleagues.

The article presents current data on the etiology, pathogenesis, and epidemiology of systemic lupus erythematosus (SLE). The SLE diagnosis details are considered with a description of each examination technique. Moreover, an assessment of reliability level of both evidence and recommendations for each thesis-recommendation are discussed thoroughly. The aspects of differential diagnosis and criteria for the diagnostic quality of SLE are revealed.

This lecture for the system of postgraduate medical education analyzes causes, types, key links of pathogenesis, and manifestations of the main typical forms of liver pathology — liver failure, hepatic coma, jaundice, cholemia, acholia, cholelithiasis, and their complications in children. To control the retention of the lecture material, case problems and multiple-choice tests are given.

The rational use of antibacterial drugs in children implies an adequate choice of the necessary medication, its dosing regimen, and the duration of treatment in order to achieve maximum efficacy and minimize toxic effects. The knowledge of pharmacokinetic and pharmacodynamic profiles of the antibacterial drug plays a crucial role for optimizing the dosing regimen. The strategy of individual choice of the dosing regimen, taking into account the principles of pharmacokinetics and pharmacodynamics, can be especially effective in patients with the expectedly changed parameters of pharmacokinetics and in infections caused by bacteria strains with low sensitivity to antibiotics. The review presents a contemporary view of pharmacokinetic and pharmacodynamic profiles of antibacterial drugs most commonly used in pediatrics and their relationship to the clinical efficacy of the administered therapy.

Background. Cystic fibrosis is a hereditary disease that occurs as a result of mutations in the regulator gene of chloride ion transmembrane transport (CFTR). Finding mutations in the CFTR gene is necessary for identification of the clinical features of cystic fibrosis.

Objective. Our aim was to identify genotype-phenotype correlations between mutations of the first class of pathogenicity and clinical manifestations of cystic fibrosis based on studying the prevalence and structure of CFTR gene mutations.

Methods. The study included children under 18 years with cystic fibrosis admitted to hospital between 2013 and 2017. Biallelic mutations in the CFTR gene were the noninclusion criterion. The CFTR gene variants were analyzed by next-generation sequencing method.

Results. In 125 patients with cystic fibrosis, 59 different variants of the CFTR gene were detected, 11 of them not previously described. The most common was the deletion c.1521_1523del found in 98 (39.2%) of the 250 analyzed CFTR gene alleles and the deletion c.1545_1546del found in 22/250 (8.8%) alleles. It has been shown that the mutation c.1545_1546del, p.Y515* was more often found in children of the Chechen nation — odds ratio (OR) 139 (95% confidence interval 15–1,257). It has been established that meconium ileus, pancreatic deficiency and cirrhosis are more common in patients with mutations of the first category of pathogenicity: OR 3.9 (95% CI 1.0–15.0), 4.4 (95% CI 1.8–11.1), and 351 (95% CI 17.5–7,046), respectively. The association of CFTR gene mutations with the development of bronchiectases and polypous pancinusitis has not been found.

Conclusion. Correlations between the genotype and clinical manifestations of cystic fibrosis in Russian children with CFTR gene mutations of the first class of pathogenicity have been established.

Background. Children of preschool and school age are at risk of developing vitamin deficiency. Screening of the vitamin provision of children remains an urgent problem of pediatrics. Objective. Our aim was to determine the prevalence of low excretion of watersoluble vitamins among healthy preschool and school-age children.

Methods. The study was conducted in March-April 2017. We determined the urinary excretion (fasting morning portion collected during 30–120 min after night-time urination) of metabolites of vitamins C, B₁, B₂, and B₆ in healthy children. Riboflavin (vitamin B₂ metabolite) was determined spectrophotometrically by titration with a riboflavin-binding apoprotein; 4-pyridoxyl acid (vitamin B₆ metabolite) and thiamine (vitamin B₁ metabolite) — by fluorescent method, ascorbic acid (vitamin C metabolite) — by visual titration with Tillman’s reagent. The excretion considered to be low (equivalent to vitamin deficiency) when thiamine excretion was < 7, 10, 11, and 12 μg/h and riboflavin < 6, 9, 10, and 13 μg/h in children aged 3–5, 6–8, 9–11, and above 12 years, respectively; 4-pyridoxylic acid — < 40, 60, and 70 μg/h in children aged 3–5, 6–8, and ≥ 9 years, ascorbic acid — < 0.2 and 0.4 mg/h in children aged 3–11 and ≥ 12 years, respectively.

Results. Metabolites were excreted in 39 children (20 girls), 14 of them aged 4–6 years and 25 children aged 7–14 years. A low level of ascorbic acid excretion was found in 13 (33%) children, of thiamine — in 24 (62%), of riboflavin — in 16 (41%), of 4-pyridoxyl acid — in 26 (67%). Low excretion of at least one vitamin metabolite was detected in 30 (77%) children, of 3 or more metabolites simultaneously — in 15 (39%).

Background. There are limited data on the efficacy of long-term enzyme replacement therapy (ERT) in children with mucopolysaccharidosis (MPS).

Objective. Our aim was to study the efficacy and safety of long-term ERT in children with MPS type I, II, and VI.

Methods. We analyzed the results of ERT with laronidase, idursulfase, and galsulfase in children with MPS type I, II, and VI admitted to the federal research center from January 2007 to November 2016. The response rate was assessed by the level of normalized urinary excretion of glycosaminoglycans (GAGs) (the ratio of GAGs concentration to urine creatinine) recalculated in percent (%) exceedance of the upper limit of normal for the corresponding age. Data on the administered therapy and its results, including adverse events, is extracted from the medical records of in-patients.

Results. The results of treatment (intravenous infusions, intervals between administrations from 4 to 10 days) were studied in 33 children (5 of them were girls) with MPS type I (n = 4; laronidase at a dose of 0.58 mg/kg), II (n = 26; idursulfase at a dose of 0.5 mg/kg), and VI (n = 3; galsulfase at a dose of 1 mg/kg). A decrease in the normalized urinary excretion of GAGs from 376% (172; 791) to 54% (0; 146) exceedance of the upper limit of normal for the age (p < 0.001) was noted in the course of ERT lasting (median) 27 (14; 41) months. A decrease in the normalized GAGs excretion below the upper limit of normal for the age was established in 12/33 (36%) patients. ERT-associated adverse events were identified in 12 patients; one case required a two-fold therapy interruption. The development of nephrotic syndrome in the course of ERT in patients with severe MPS II was first described.

Conclusion. Long-term ERT in children with MPS type I, II, and VI is characterized by acceptable efficacy and safety. Key words: children, mucopolysaccharidosis, enzyme replacement therapy, laronidase, idursulfase, galsulfase, glycosaminoglycans.

Atopic dermatitis is a common chronic inflammatory skin disease characterized by a recurring course and progressive decrease in the quality of life. Recent studies in this area demonstrate the multifaceted pathogenesis of atopic dermatitis. Interaction of such factors as epidermal dysfunction, immune system disorders, and the consequences of genetic mutations contributes not only to the development of the disease but also to its progression and chronic course. The article presents various components of the etiopathogenesis of atopic dermatitis, describes the role of lipids, thereby the new therapeutic targets are revealed to specialists.

Background. Solid tumors in children are one of the most common childhood malignancy, second only to hemoblastosis. Among solid tumours, about 5% are bone sarcomas: osteosarcoma (3%) and Ewing's sarcoma (2%). Atypicality of the these diseases course makes an early diagnosis a real challenge.

Case Reports. The article presents six clinical observations of patients with bone sarcomas. We demonstrate the difficulties in diagnosing of this disease group which is associated with the absence of both specific symptoms and  distinct clinical picture.

Conclusion. Pediatricians and pediatric surgeons should take into account the possibility of atypical course of bone sarcomas in children. Low cancer alertness is the reason for a significant delay in establishing the correct diagnosis which contributes to the tumour process generalization and reduces the chances of achieving remission while increasing the cost of treating such patients.