Impact of Sanction Pressure in Developing Countries on Industrial Development

The article analyzes the chronology of the main stages of sanctions pressure on Iran and Russia. The impact of sanctions on the main indicators of industrial development is considered. The main response measures to the impact of development indicators were also considered.В статье проанализирована хронология основных этапов санкционного давления на Иран и Россию. Рассмотрено влияние санкций на основные показатели развития промышленности. Также были рассмотрены основные меры реагирования на влияние индикаторов развития

Evaluation of mass spectrometric indicators for the differential diagnosis of inherited disorders of organic acid metabolism in children

The objective. To calculate the reference intervals of the content of organic acids in the urine of the children of different age groups based on the results of gas chromatography with mass spectrometry; to find out their diagnostic value as biochemical markers of organic acidemia and other metabolic disorders.Materials and methods. The group consisted of 80 children with suspected organic acidemia. The control group consisted of 443 practically healthy children of different ages. The urine samples were analyzed for the content of 28 organic by gas chromatography with mass spectrometry.The results. The authors established reference intervals of 28 organic urine acids. The differences in the reference intervals defined in this work were isolated from those obtained in other studies. There were identified 37 children with abnormalities in the spectrum of organic acids according to the results of the analysis of the urine samples of patients by gas chromatography-mass spectrometry. 23 patients were diagnosed with various forms of organic acidemia on the basis of characteristic clinical and laboratory data and changes in the indicators of organic acids.The conclusion. The method of gas chromatography with mass spectrometry has proved its effectiveness  and reliability for the diagnosis of hereditary diseases from the group of organic acidemias

Carnitine metabolic features in young athletes

Objective: to reveal differences in the carnitine metabolism of children going in for various sports. The peripheral blood levels of free (FC) and bound carnitine (BC) were studied; a BC/FC ratio was calculated using liquid chromatography-electrospray ionizationtandem mass spectrometry in 121 children, including 46 ice-hockey players, 48 swimmers, and 27 healthy schoolchildren. The study established that the levels of FC in the group of ice-hockey players were 29,9±0,95 μmol/L, which was significantly lower than those in the group of swimmers (36,29±0,84 μmol/L) at p<0,001. In the ice-hockey players and swimmers, the BC/FC ratio was 0,54±0,02 and 0,46±0,02, respectively (the differences were significant; p<0,01. Analysis of gender-related differences revealed the lowest FC level in the group of female ice-hockey players and the highest BC/FC ratio in that of male ice-hockey players. This suggests that the swimmers, cyclical sports representatives (who perform mainly aerobic activity), have a more effective cellular energy

Specific features of the clinical and laboratory diagnosis of Lesch—Nyhan syndrome and current therapy options

The rare hereditary diseases encountered in the practice of pediatricians, pediatric neurologists, and geneticists include Lesch—Nyhan syndrome associated with purine metabolic disorders caused by mutations in the HPRT1 gene encoding the enzyme hypoxanthine-guanine phosphoribosyltransferase (HGPRT). HGPRT deficiency gives rise to hyperuricemia, hyperuricemic nephropathy, and a CNS lesion reminiscent of dystonic or hyperkinetic infantile cerebral paralysis concurrent with a propensity to have autoaggressive behavior. The diagnosis is based on a range of history data, characteristic neurological symptoms, the detection of high blood and urine uric acid levels, and the identification of pathogenic mutagens in the HPRT1 gene. Early diagnosis contributes to timely therapy and assists in medical genetic counseling. The paper describes a clinical case of a boy with Lesch-Nyhan syndrome and elucidates the problems of diagnosis and treatment of this disease

Diagnostic value of blood coenzyme Qlo levels in children with mitochondrial diseases

То establish the diagnostic value of a change in the indicator coenzyme Qlo, its blood levels were tested in 15 children with mitochondrial diseases (Group 1). A comparison group consisted of 13 children with neurodegenerative diseases (Group 2). A control group included 29 healthy children of the same age. In Group 1 patients, the mean coenzyme Qlo level (0,56+0,06 mcmol/1) did not differ from that in the control group. However, these patients had a considerably lower coenzyme Q10/cholesterol ratio (0,10+0,01; /K0,001). The patients in the older age subgroup were found to have a significantly lower coenzyme Qlo level, which seems to reflect the progressive nature of the abnormality. There was a trend for the coenzyme Q10/cholesterol ratio to decline with age. Group 2 children had higher blood coenzyme Qlo levels (1,53+0,23; /K0,001) than Group 1 and an increased Q10/cholesterol ratio (0,31+0,04; /K0,001). It is advisable to continue the investigation to provide a rationale for using low coenzyme values as an additional criterion for the differential diagnosis of mitochondrial diseases

Regionalism in Ukraine: Historic Evolution, Regional Claim-Making, and Centre–Periphery Conflict Resolution

This chapter examines the historical constitution of the present territory of Ukraine and its administrative-territorial system, identity and regional cleavages and the evolution and dynamics of claim-making and center-periphery contention related to them in different regions, namely Transcarpathia, Crimea and Donbas, since the late 1980s. It examines different forms of accommodation of claims such as an asymmetric state structure in the case of Crimean autonomy, power devolution, free economic zones, subsidies and budget disbursements, power-sharing at the national level, and local and regional legislation on historical memory and languages. Beyond the widely acknowledged role of external intervention in the escalation of conflict in eastern Ukraine in 2014, the chapter focuses on the long-term nonviolent contention related to regional cleavages prior to the escalation of the conflict and political exclusion. The chapter shows that while there has been an increasing identification with Ukrainian citizenship and support for decentralization since 2014, important regional differences in terms of historical memories, attitudes to the Euromaidan, and the nature of the ongoing conflict remain and may be loci of vulnerability to future regional mobilizations. The effect of the ongoing reforms in decentralization and democratic governance on the resolution of center–periphery conflicts and the accommodation of regional claims remains to be seen