Розвиток телебачення Конотопщини

Історія конотопського телебачення цікава і трагічна, тісно пов’язана з історією розвитку вітчизняного телебачення, з різними подіями в історії України і людства, з видатними науковцями і політичними діячами, письменниками і композиторами, робітниками і селянами Конотопщини. При цитуванні документа, використовуйте посилання http://essuir.sumdu.edu.ua/handle/123456789/32401История конотопcкого телевидения интересная и трагическая, тесно связана с историей развития отечественного телевидения, с различными событиями в истории Украины и человечества, с выдающимися учеными и политическими деятелями, писателями и композиторами, рабочими и крестьянами Конотопщины. При цитировании документа, используйте ссылку http://essuir.sumdu.edu.ua/handle/123456789/3240

INFLUENCE OF THE CREATIVE ECONOMY ON MODERN MANAGEMENT OF CREATIVE INDUSTRIES

The features of the modern approach to the organization of working process in creative industries, where the key is the concept of “teal organization” distinctive quality characteristics of which are self-government and self-organization, implying independent implementation of the work processes and projects realization by the employees, have been considered. The rules of creating competent communication with the creative class, people who are the driving force of the creative economy, play an essential role in organizing the management of creative industries in modern society. The functioning of “teal organizations” exclusively within the framework of a creative economy, where the greatest value is the realization of the creative potential of the employee, has been examined in the article

Зміни показників фізичного стану жінок 19–35-літнього віку при самостійних заняттях оздоровчими видами фізичної культури

The article represents the comparative analysis results of physical data of 19–35 year-old women before and after 4 months self-training with using of different health-improving kinds of physical culture (athletic gymnastic, swimming, shaping).Представлены результаты сравнительного анализа морфофункциональных показателей женщин 19–35 лет до и после самостоятельных занятий различными оздоровительными видами физической культуры (атлетическая гимнастика, плавание, шейпинг).Сравнительный анализ показателей физического состояния до и после эксперимента выявил наличие общих и специфических особенностей проявления тренировочного эффекта в условиях занятий различными видами физической культуры.Надані результати порівняного аналізу морфо-функціональних показників жінок 19–35 років до та після занять різними оздоровчими видами фізичної культури (атлетична гімнастика, плавання, шейпінг). Порівняльний аналіз показників фізичного стану до і після експерименту виявив наявність загальних і специфічних особливостей прояву тренувального ефекту в умовах занять різними видами фізичної культури

Sphingolipid dysregulation due to lack of functional KDSR impairs proplatelet formation causing thrombocytopenia.

Sphingolipids are fundamental to membrane trafficking, apoptosis, and cell differentiation and proliferation. KDSR or 3-keto-dihydrosphingosine reductase is an essential enzyme for de novo sphingolipid synthesis, and pathogenic mutations in KDSR result in the severe skin disorder erythrokeratodermia variabilis et progressiva-4 Four of the eight reported cases also had thrombocytopenia but the underlying mechanism has remained unexplored. Here we expand upon the phenotypic spectrum of KDSR deficiency with studies in two siblings with novel compound heterozygous variants associated with thrombocytopenia, anemia, and minimal skin involvement. We report a novel phenotype of progressive juvenile myelofibrosis in the propositus, with spontaneous recovery of anemia and thrombocytopenia in the first decade of life. Examination of bone marrow biopsies showed megakaryocyte hyperproliferation and dysplasia. Megakaryocytes obtained by culture of CD34+ stem cells confirmed hyperproliferation and showed reduced proplatelet formation. The effect of KDSR insufficiency on the sphingolipid profile was unknown, and was explored in vivo and in vitro by a broad metabolomics screen that indicated activation of an in vivo compensatory pathway that leads to normalization of downstream metabolites such as ceramide. Differentiation of propositus-derived induced pluripotent stem cells to megakaryocytes followed by expression of functional KDSR showed correction of the aberrant cellular and biochemical phenotypes, corroborating the critical role of KDSR in proplatelet formation. Finally, Kdsr depletion in zebrafish recapitulated the thrombocytopenia and showed biochemical changes similar to those observed in the affected siblings. These studies support an important role for sphingolipids as regulators of cytoskeletal organization during megakaryopoiesis and proplatelet formation

Опыт создания полнотекстовой базы данных «МедЛиб» в научно-медицинской библиотеке Сибирского государственного медицинского университета

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Whole genome sequencing reveals that genetic conditions are frequent in intensively ill children.

PURPOSE: With growing evidence that rare single gene disorders present in the neonatal period, there is a need for rapid, systematic, and comprehensive genomic diagnoses in ICUs to assist acute and long-term clinical decisions. This study aimed to identify genetic conditions in neonatal (NICU) and paediatric (PICU) intensive care populations. METHODS: We performed trio whole genome sequence (WGS) analysis on a prospective cohort of families recruited in NICU and PICU at a single site in the UK. We developed a research pipeline in collaboration with the National Health Service to deliver validated pertinent pathogenic findings within 2-3 weeks of recruitment. RESULTS: A total of 195 families had whole genome analysis performed (567 samples) and 21% received a molecular diagnosis for the underlying genetic condition in the child. The phenotypic description of the child was a poor predictor of the gene identified in 90% of cases, arguing for gene agnostic testing in NICU/PICU. The diagnosis affected clinical management in more than 65% of cases (83% in neonates) including modification of treatments and care pathways and/or informing palliative care decisions. A 2-3 week turnaround was sufficient to impact most clinical decision-making. CONCLUSIONS: The use of WGS in intensively ill children is acceptable and trio analysis facilitates diagnoses. A gene agnostic approach was effective in identifying an underlying genetic condition, with phenotypes and symptomatology being primarily used for data interpretation rather than gene selection. WGS analysis has the potential to be a first-line diagnostic tool for a subset of intensively ill children

Identification of rare sequence variation underlying heritable pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis. Deleterious variation within components of the transforming growth factor-β pathway, particularly the bone morphogenetic protein type 2 receptor (BMPR2), underlies most heritable forms of PAH. To identify the missing heritability we perform whole-genome sequencing in 1038 PAH index cases and 6385 PAH-negative control subjects. Case-control analyses reveal significant overrepresentation of rare variants in ATP13A3, AQP1 and SOX17, and provide independent validation of a critical role for GDF2 in PAH. We demonstrate familial segregation of mutations in SOX17 and AQP1 with PAH. Mutations in GDF2, encoding a BMPR2 ligand, lead to reduced secretion from transfected cells. In addition, we identify pathogenic mutations in the majority of previously reported PAH genes, and provide evidence for further putative genes. Taken together these findings contribute new insights into the molecular basis of PAH and indicate unexplored pathways for therapeutic intervention