[Clinical guidelines «Precocious puberty¼].

The precocious puberty is an urgent problem of pediatric endocrinology characterized by clinical and pathogenetic heterogeneity. The appearance of secondary sex characteristics before the age of 8 years in girls and 9 years in boys requires timely diagnosis and the appointment of pathogenetically justified treatment in order to achieve the target indicators of final growth and prevent social deprivation. The developed clinical guidelines are the main working tool of the practitioner. They briefly and structurally present the main information about the epidemiology and modern classification of рrecocious puberty, methods of its diagnosis and treatment based on the principles of evidence-based medicine.

[Assessment of physical development and diet of primary school children in Smolensk].

The growth and development of children largely depends on the diet. Irrational and irregular nutrition can contribute to the development of nutritional-dependent diseases of children and adolescents, the formation of overweight and obesity. The aim of the research - the assessment of physical development and diet of primary schoolchildren living in Smolensk. Material and methods. 817 schoolchildren aged 7-10 have been surveyed, of whom 403 (49.3%) were girls and 414 (50.7%) were boys. In the process of the study, the length and body mass have been measured, body mass index (BMI) has been calculated. Assessment of the physical development of children has been carried out according to the standards of the World Health Organization (WHO) using the software product WHO AnthroPlus (2009). Z-score values for body weight for age, body length for age, and BMI for age have been calculated. In the process of the study, children were questioned. A questionnaire on the diagnosis of the lifestyle of children, developed by specialists from the University of Cologne, made it possible to evaluate the mode and structure of nutrition of schoolchildren. Results and discussion. It has been revealed that the average values of mass-growth indicators of Smolensk primary schoolchildren were higher compared with the standard WHO population. Deviations of physical development in the studied sample of schoolchildren were more concerned with body weight than growth, while obesity in boys was detected significantly more often than in girls (11.9 vs 5.2%, χ2=10.465, p=0.002). It has been established that the majority of primary schoolchildren (84.8%) ate 3-5 times a day. 92.7% of schoolchildren have breakfast at home. In the school canteen, only 55.6% of schoolchildren ate regularly. In the interval between dinner and supper, the majority (96.8%) of the respondents had a snack, of which 43.7% used patties, cookies or sweets for snacking. Almost half of primary schoolchildren (45.4%) admitted that they had food intake just before bedtime. Moreover, boys did this significantly more often than girls (50.8 vs 41.0%, χ2=5.209, p=0.023). Conclusion. In order to prevent the formation of overweight and obesity among primary schoolchildren, it is necessary to introduce a complex of preventive measures. Among them, there are rationalization of children's nutrition in the family and school, optimization of the day regime and physical activity of children, psychological support for children with overweight and obesity, the introduction of modern educational and information technologies for the formation of a healthy lifestyle of children.

[Open, non-comparative phase III clinical study to evaluate the efficacy and safety of sapropterin in patients with phenylketonuria and hyperphenylalaninemia].

BACKGROUND: Phenylketonuria (PKU) is an autosomal recessive inherited disease associated with impaired metabolism of the amino acids phenylalanine (Phe) and tyrosine. The main criterion for diagnosis of PKU is high blood Phe level determined during neonatal screening. In case where PKU patient is responsive to tetrahydrobiopterin treatment, sapropterin restores the impaired activity of the enzyme phenylalanine hydroxylase, resulting in the stimulation of normal Phe metabolism and thereby enhancing patient tolerance to natural products. AIM: The present open, non-comparative clinical study was initiated to assess the degree and frequency of response after 8-day sapropterin administration and assess the safety of 6-week sapropterin treatment in patients with PKU and hyperphenylalaninemia. PATIENTS AND METHODS: The study enrolled 90 patients with PKU. The criterion of response to 8-day sapropterin therapy was the reduction of Phe blood levels ≥ 30% compared with the baseline value. RESULTS: Positive response to treatment was observed in 30 (33.3%) patients (95% CI 23.7-44.1). The mean percentage change in Phe blood levels after the 8-day response test period compared to Phe levels prior to dosing was 14.1 ± 28.4% in the overall subject population (95% CI 8.2-20.1) and 44.3 ± 15.1% in the subpopulation of patients with a positive response (95% CI 38.6-49.9). During the study, adverse events were reported in 24 (26.7%) patients in the overall population in 16 (53.3%) patients in the subpopulation who had a response. CONCLUSION: The study results confirmed the efficacy and safety of sapropterin therapy in patients with PKU, which is consistent with international clinical trials data.

[The characteristics of lipid metabolism in children with congenital hypothyroidism against the background of substitutive therapy with L-thyroxin].

The article presents the results of study of lipid metabolism. The sample consisted of 40 children with congenital thyroid deficiency at different stage of disease compensation and receiving substitutive therapy. Such disorders of lipid metabolism as hypertriglyceridemia and decrease of concentration of high density lipoproteins are established. The results testify the increased risk of atherogenic tendency. The hyper- and dyslipidemia are more often registered in patients at the stage of sub-compensated thyroid deficiency. The correlation of dosage of thyroxin with application of L-carnitine contributes to normalization of indicators of lipid metabolism.