Strategic priorities of regional investment activity

© Authors. The purpose of the paper is to study the regional investment strategy development based on the identified strategic priorities for its development. Methods of the problem under research include statistical and methods of system and logical analysis, comparative and retrospective methods, methods of analysis and synthesis, methods for conjugate effects accounting. The paper presents the study results of the regional investment activities strategic priorities, taking into account its investment environment functioning features. The conducted study is based on the most important strategic documents, reflecting the long-term goals of regional development. In order to justify the possibility of implementing the strategy, the conditions for implementing investment projects aimed at the regional infrastructure developing and its socio-economic development indices improving are considered. The studies were carried out on the analysis basis of the major national economic complexes investment structure in the region. The paper also presents the region's target state indicators in the context of various forecast scenarios for its development, which include inertial, basic and optimistic ones. At the same time, a basic scenario aimed at overcoming resource constraints is considered in the context of the regional economic complexes. The conclusions are made on the compliance of the regional investment activities' strategic priorities with the potential for its development. The paper materials are of practical value for specialists engaged in the development, analysis and implementation of the regional investment strategy

Глутаровая ацидурия типа 1 у детей. Клиническое представление 46 случаев, диагностированных в России

Background. Glutaric aciduria type 1 is an autosomal recessive disease caused by mutations in the GCDH gene, which encodes the enzyme glutaryl‑CoA dehydrogenase. Metabolic crisis in type 1 glutaric aciduria is an acute life‑threatening condition that requires careful diagnosis with a number of other conditions and the immediate initiation of pathogenetic therapy.Materials and methods. Clinical manifestations, neuroimaging characteristics of the disease were studied in 46 patients with diagnosed glutaric aciduria type 1 confirmed by biochemical and molecular genetic methods. Methods: gas chromatography with mass spectrometry, tandem mass spectrometry, Sanger sequencing, chromosomal microarray analysis of the exon level.Results and discussion. A retrospective analysis of anamnestic and clinical data was carried out, and the nature and age of disease manifestation, provoking factors, a spectrum of clinical manifestations and neuroimaging data were assessed.Conclusion. How initiated treatment prevents progression of neurological symptom relief and patient adaptation. With the help of the goal, it is necessary to inform pediatricians, neurologists and neuroradiologists about this feature of the course of glutaric aciduria type 1 in order to increase the clinical alertness of this disease.Введение. Глутаровая ацидурия типа 1 – аутосомно‑рецессивное заболевание, обусловленное мутациями в гене GCDH, кодирующем фермент глутарил‑КоА дегидрогеназу. Метаболический криз при глутаровой ацидурии типа 1 – это острое жизнеугрожающее состояние, требующее тщательной дифференциальной диагностики с рядом других состояний и незамедлительного начала патогенетической терапии.Материалы и методы. Клинические проявления, нейровизуализационные характеристики болезни изучены у 46 пациентов с подтвержденным биохимическими и молекулярно‑генетическими методами диагнозом глутаровой ацидурии типа 1. Методы: газовая хроматография с масс‑спектрометрией, тандемная масс‑спектрометрия, секвенирование по Сэнгеру, хромосомный микроматричный анализ экзонного уровня.Результаты и обсуждение. Проведен ретроспективный анализ анамнестических данных, клинических, а также оценены характер и возраст манифестации болезни, провоцирующие факторы, спектр клинических проявлений и нейровизуализационные данные.Заключение. При отсутствии массового неонатального скрининга крайне важное значение имеет ранняя диагностика болезни, так как своевременно начатое лечение поможет предотвратить прогрессирование неврологической симптоматики и способствовать адаптации пациентов. С этой целью необходимо информировать врачей‑педиатров, неврологов и нейрорадиологов об особенностях протекания глутаровой ацидурии типа 1 для повышения клинической настороженности в отношении данного заболевания

Strategic priorities of regional investment activity

© Authors. The purpose of the paper is to study the regional investment strategy development based on the identified strategic priorities for its development. Methods of the problem under research include statistical and methods of system and logical analysis, comparative and retrospective methods, methods of analysis and synthesis, methods for conjugate effects accounting. The paper presents the study results of the regional investment activities strategic priorities, taking into account its investment environment functioning features. The conducted study is based on the most important strategic documents, reflecting the long-term goals of regional development. In order to justify the possibility of implementing the strategy, the conditions for implementing investment projects aimed at the regional infrastructure developing and its socio-economic development indices improving are considered. The studies were carried out on the analysis basis of the major national economic complexes investment structure in the region. The paper also presents the region's target state indicators in the context of various forecast scenarios for its development, which include inertial, basic and optimistic ones. At the same time, a basic scenario aimed at overcoming resource constraints is considered in the context of the regional economic complexes. The conclusions are made on the compliance of the regional investment activities' strategic priorities with the potential for its development. The paper materials are of practical value for specialists engaged in the development, analysis and implementation of the regional investment strategy