100 research outputs found

    Research of Planning Features of the Territory of the Estate Grebnitskikh in the Village of Orekhovno

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    Секция II : Строительные конструкции зданий и сооружений, современные методы расчета и проектированияРассмотрено современное состояние усадебно-парковой зоны усадьбы Гребницких в д. Ореховно. Приведены результаты натурных обследований территории, расположения зданий и зеленых насаждений. Предложен план организации предпроектных работ по восстановлению и адаптации усадьбы.=The modern state of the manor-Park zone of the Grebnitskih estate in the village of Orekhovno is considered. The results of field surveys of the territory, the location of buildings and green spaces are presented. The plan of the organization of pre-project works on restoration and adaptation of the estate is offered

    Research of Planning Features of the Territory of the Estate Grebnitskikh in the Village of Orekhovno

    Get PDF
    Секция II : Строительные конструкции зданий и сооружений, современные методы расчета и проектированияРассмотрено современное состояние усадебно-парковой зоны усадьбы Гребницких в д. Ореховно. Приведены результаты натурных обследований территории, расположения зданий и зеленых насаждений. Предложен план организации предпроектных работ по восстановлению и адаптации усадьбы.=The modern state of the manor-Park zone of the Grebnitskih estate in the village of Orekhovno is considered. The results of field surveys of the territory, the location of buildings and green spaces are presented. The plan of the organization of pre-project works on restoration and adaptation of the estate is offered

    Morphometric parameters of hepatocytes of three zones of the rat liver lobule in the recovery period after mechanical injury of the lower leg

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    The aim of the study – to investigate the dynamics of morphometric parameters of liver hepatocytes in the recovery period after mechanical injury of the lower leg in rats.Цель исследования – исследовать динамику морфометрических показателей гепатоцитов печени в восстановительном периоде после механической травмы голени у крыс

    Организация деятельности противотуберкулезных медицинских учреждений в условиях пандемии COVID-19

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    The article analyzes the organization of work of N.V. Postnikov Samara Regional Clinical TB Dispensary in 2020. The special procedure has been developed for COVID-19 tests in patients referred to TB department. All cases of the new coronavirus infection (COVID-19) in tuberculosis patients that occurred in the Samara region in 2020 (n = 31) were studied. Infection control activities are the following: separate filter boxes have been provided for personnel and patients in each department; two observational departments have been organized where patients are admitted depending on the type of medical care. The polymerase chain reaction has been used to examine patients for COVID-19 before admission to TB hospital for planned medical care, and express tests for immunoglobulins M and G have been used when patients were admitted in an emergency. Of the 30 cases of intravital detection of concurrent infections (tuberculosis + COVID-19), in 56.7% (17 people), COVID-19 was treated in the in-patient unit specializing in treatment of the new coronavirus infection, and in 43.3% (13 people), treatment was outpatient; lethality made 1/30 (3.33%).Проанализирована организация работы ГБУЗ «Самарский областной клинический противотуберкулезный диспансер им. Н. В. Постникова» в 2020 г. Разработан алгоритм обследования пациентов на COVID-19 при направлении в туберкулезное отделение. Изучены все случаи новой коронавирусной инфекции (COVID-19) у больных туберкулезом в Самарской области в 2020 г. (n = 31). Меры инфекционной безопасности: организованы раздельные фильтр-боксы для сотрудников и пациентов в каждом структурном подразделении; выделены два обсервационных отделения в зависимости от формы оказания медицинской помощи. Обследование пациентов на COVID-19 перед госпитализацией в туберкулезный стационар проводилось методом полимеразной цепной реакции при оказании медицинской помощи в плановой форме, экспресс-методом определения иммуноглобулинов M и G – при оказании помощи в неотложной/экстренной форме. Из 30 случаев прижизненного выявления сочетанной патологии (туберкулез + COVID-19) в 56,7% (17 человек) лечение COVID-19 осуществлялось в условиях стационара для оказания медицинской помощи пациентам с новой коронавирусной инфекцией, в 43,3% (13 человек) – амбулаторно, летальность составила 1/30 (3,33%)

    Adverse events in families with hypertrophic or dilated cardiomyopathy and mutations in the MYBPC3 gene

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    <p>Abstract</p> <p>Background</p> <p>Mutations in <it>MYBPC3 </it>encoding myosin binding protein C belong to the most frequent causes of hypertrophic cardiomyopathy (HCM) and may also lead to dilated cardiomyopathy (DCM). <it>MYBPC3 </it>mutations initially were considered to cause a benign form of HCM. The aim of this study was to examine the clinical outcome of patients and their relatives with 18 different <it>MYBPC3 </it>mutations.</p> <p>Methods</p> <p>87 patients with HCM and 71 patients with DCM were screened for <it>MYBPC3 </it>mutations by denaturing gradient gel electrophoresis and sequencing. Close relatives of mutation carriers were genotyped for the respective mutation. Relatives with mutation were then evaluated by echocardiography and magnetic resonance imaging. A detailed family history regarding adverse clinical events was recorded.</p> <p>Results</p> <p>In 16 HCM (18.4%) and two DCM (2.8%) index patients a mutation was detected. Seven mutations were novel. Mutation carriers exhibited no additional mutations in genes <it>MYH7</it>, <it>TNNT2</it>, <it>TNNI3</it>, <it>ACTC </it>and <it>TPM1</it>. Including relatives of twelve families, a total number of 42 mutation carriers was identified of which eleven (26.2%) had at least one adverse event. Considering the twelve families and six single patients with mutations, 45 individuals with cardiomyopathy and nine with borderline phenotype were identified. Among the 45 patients, 23 (51.1%) suffered from an adverse event. In eleven patients of seven families an unexplained sudden death was reported at the age between 13 and 67 years. Stroke or a transient ischemic attack occurred in six patients of five families. At least one adverse event occurred in eleven of twelve families.</p> <p>Conclusion</p> <p><it>MYBPC3 </it>mutations can be associated with cardiac events such as progressive heart failure, stroke and sudden death even at younger age. Therefore, patients with <it>MYBPC3 </it>mutations require thorough clinical risk assessment.</p

    Gene expression throughout a vertebrate's embryogenesis

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    Abstract Background Describing the patterns of gene expression during embryonic development has broadened our understanding of the processes and patterns that define morphogenesis. Yet gene expression patterns have not been described throughout vertebrate embryogenesis. This study presents statistical analyses of gene expression during all 40 developmental stages in the teleost Fundulus heteroclitus using four biological replicates per stage. Results Patterns of gene expression for 7,000 genes appear to be important as they recapitulate developmental timing. Among the 45% of genes with significant expression differences between pairs of temporally adjacent stages, significant differences in gene expression vary from as few as five to more than 660. Five adjacent stages have disproportionately more significant changes in gene expression (&gt; 200 genes) relative to other stages: four to eight and eight to sixteen cell stages, onset of circulation, pre and post-hatch, and during complete yolk absorption. The fewest differences among adjacent stages occur during gastrulation. Yet, at stage 16, (pre-mid-gastrulation) the largest number of genes has peak expression. This stage has an over representation of genes in oxidative respiration and protein expression (ribosomes, translational genes and proteases). Unexpectedly, among all ribosomal genes, both strong positive and negative correlations occur. Similar correlated patterns of expression occur among all significant genes. Conclusions These data provide statistical support for the temporal dynamics of developmental gene expression during all stages of vertebrate development

    COL4A1 Mutations Cause Ocular Dysgenesis, Neuronal Localization Defects, and Myopathy in Mice and Walker-Warburg Syndrome in Humans

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    Muscle-eye-brain disease (MEB) and Walker Warburg Syndrome (WWS) belong to a spectrum of autosomal recessive diseases characterized by ocular dysgenesis, neuronal migration defects, and congenital muscular dystrophy. Until now, the pathophysiology of MEB/WWS has been attributed to alteration in dystroglycan post-translational modification. Here, we provide evidence that mutations in a gene coding for a major basement membrane protein, collagen IV alpha 1 (COL4A1), are a novel cause of MEB/WWS. Using a combination of histological, molecular, and biochemical approaches, we show that heterozygous Col4a1 mutant mice have ocular dysgenesis, neuronal localization defects, and myopathy characteristic of MEB/WWS. Importantly, we identified putative heterozygous mutations in COL4A1 in two MEB/WWS patients. Both mutations occur within conserved amino acids of the triple-helix-forming domain of the protein, and at least one mutation interferes with secretion of the mutant proteins, resulting instead in intracellular accumulation. Expression and posttranslational modification of dystroglycan is unaltered in Col4a1 mutant mice indicating that COL4A1 mutations represent a distinct pathogenic mechanism underlying MEB/WWS. These findings implicate a novel gene and a novel mechanism in the etiology of MEB/WWS and expand the clinical spectrum of COL4A1-associated disorders

    Heavy and light roles: myosin in the morphogenesis of the heart

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    Myosin is an essential component of cardiac muscle, from the onset of cardiogenesis through to the adult heart. Although traditionally known for its role in energy transduction and force development, recent studies suggest that both myosin heavy-chain and myosin lightchain proteins are required for a correctly formed heart. Myosins are structural proteins that are not only expressed from early stages of heart development, but when mutated in humans they may give rise to congenital heart defects. This review will discuss the roles of myosin, specifically with regards to the developing heart. The expression of each myosin protein will be described, and the effects that altering expression has on the heart in embryogenesis in different animal models will be discussed. The human molecular genetics of the myosins will also be reviewed
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