Analysis of the opportunities for improving energy efficiency in public buildings

Reducing energy consumption and maintaining an appropriate indoor air quality in buildings is a global trend. This is achieved in several ways - improving the building envelope thermal characteristics; fuel base replacement; introduction of smart energy consumption tracking systems. The aim of this paper is an analysis of the impact of energy efficiency measures introduced in buildings that provide health services. Measures have been taken to improve the energy performance of the envelope, as well as to modernize the system for the production and distribution of thermal energy. One complete year after energy savings measures implemented the total energy savings of 37.9% have been achieved. Further economic assessment has been made regarding the profitability of the applied measures

Моделирование атмосферной колонны установки первичной переработки нефти с целью улучшения качества дизельной фракции

Объектом исследования является атмосферная колонна установки первичной переработки нефти. Целью работы является промежуточное циркуляционное орошение, исследование влияния расходов и температур возвратов промежуточных циркуляционных орошений основной атмосферной колонны на качество и количество получаемой дизельной фракции из основной атмосферной колонны с помощью математической модели действующей установки фракционирования нефти.The object of the study is the atmospheric column of a primary oil refinery. The aim of the work is intermediate circulating irrigation, a study of the influence of the costs and return temperatures of intermediate circulatory irrigation of the main atmospheric column on the quality and quantity of the diesel fraction obtained from the main atmospheric column using the mathematical model of an operating oil fractionation unit

Deglutitive Inhibition, Latency Between Swallow and Esophageal Contractions and Primary Esophageal Motor Disorders

Swallowing induces an inhibitory wave that is followed by a contractile wave along the esophageal body. Deglutitive inhibition in the skeletal muscle of the esophagus is controlled in the brain stem whilst in the smooth muscle, an intrinsic peripheral control mechanism is critical. The latency between swallow and contractions is determined by the pattern of activation of the inhibitory and excitatory vagal pathways, the regional gradients of inhibitory and excitatory myenteric nerves, and the intrinsic properties of the smooth muscle. A wave of inhibition precedes a swallow-induced peristaltic contraction in the smooth muscle part of the human oesophagus involving both circular and longitudinal muscles in a peristaltic fashion. Deglutitive inhibition is necessary for drinking liquids which requires multiple rapid swallows (MRS). During MRS the esophageal body remains inhibited until the last of the series of swallows and then a peristaltic contraction wave follows. A normal response to MRS requires indemnity of both inhibitory and excitatory mechanisms and esophageal muscle. MRS has recently been used to assess deglutitive inhibition in patients with esophageal motor disorders. Examples with impairment of deglutitive inhibition are achalasia of the LES and diffuse esophageal spasm

Система безопасности в Российской Федерации

В данной статье рассматривается система пожарной безопасности в России. Система пожарной безопасности - это целостная, комплексная система мер, включающая в себя законодательную базу, меры профилактики и тренировки, а так же систему оповещения о возникновении пожара.This article discusses the fire safety system in Russia. The fire safety system is a holistic, comprehensive system of measures, which includes the legal framework, preventive measures and training, as well as a fire alert system

Исследование проблемы выхода из строя клапанов 3-х ступенчатого газопоршневого компрессора корпорации Ariel

Цель работы – рассмотреть основную причину остановки компрессорной установки на ГКС Казанского НГКМ и предложить решение для минимизации данной поломки. В процессе исследования проводились обзор литературы, анализ и подбор В результате исследования был подобран фильтр механической очистки и режим работы компрессора для устранения жидкости из газа.The purpose of the work is to consider the main reason for stopping the compressor unit at the GCS of the Kazan Oil Gas Condenser and suggest a solution for minimizing this breakdown. During the research, literature review, analysis and selection were conducted. The study was used to select a mechanical cleaning filter and compressor operating mode to remove liquid from the gas

Discovery of four recessive developmental disorders using probabilistic genotype and phenotype matching among 4,125 families.

Discovery of most autosomal recessive disease-associated genes has involved analysis of large, often consanguineous multiplex families or small cohorts of unrelated individuals with a well-defined clinical condition. Discovery of new dominant causes of rare, genetically heterogeneous developmental disorders has been revolutionized by exome analysis of large cohorts of phenotypically diverse parent-offspring trios. Here we analyzed 4,125 families with diverse, rare and genetically heterogeneous developmental disorders and identified four new autosomal recessive disorders. These four disorders were identified by integrating Mendelian filtering (selecting probands with rare, biallelic and putatively damaging variants in the same gene) with statistical assessments of (i) the likelihood of sampling the observed genotypes from the general population and (ii) the phenotypic similarity of patients with recessive variants in the same candidate gene. This new paradigm promises to catalyze the discovery of novel recessive disorders, especially those with less consistent or nonspecific clinical presentations and those caused predominantly by compound heterozygous genotypes

Prevalence and architecture of de novo mutations in developmental disorders.

The genomes of individuals with severe, undiagnosed developmental disorders are enriched in damaging de novo mutations (DNMs) in developmentally important genes. Here we have sequenced the exomes of 4,293 families containing individuals with developmental disorders, and meta-analysed these data with data from another 3,287 individuals with similar disorders. We show that the most important factors influencing the diagnostic yield of DNMs are the sex of the affected individual, the relatedness of their parents, whether close relatives are affected and the parental ages. We identified 94 genes enriched in damaging DNMs, including 14 that previously lacked compelling evidence of involvement in developmental disorders. We have also characterized the phenotypic diversity among these disorders. We estimate that 42% of our cohort carry pathogenic DNMs in coding sequences; approximately half of these DNMs disrupt gene function and the remainder result in altered protein function. We estimate that developmental disorders caused by DNMs have an average prevalence of 1 in 213 to 1 in 448 births, depending on parental age. Given current global demographics, this equates to almost 400,000 children born per year

How to select patients for anti-reflux surgery? The ICARUS guidelines (International Consensus regarding preoperative examinations and clinical characteristics assessment to select adult patients for AntiReflUx Surgery)

Objective: Anti-reflux surgery can be proposed in patients with gastro-esophageal reflux disease, especially when proton pump inhibitor use leads to incomplete symptom improvement. However, to date, international consensus guidelines on the clinical criteria and additional technical examinations used in patient selection for anti-reflux surgery are lacking. We aimed at generating key recommendations in the selection of patients for anti-reflux surgery. Design: We included 35 international experts (gastroenterologists, surgeons and physiologists) in a Delphi process and developed 37 statements that were revised by the Consensus Group, to start the Delphi process. Three voting rounds followed where each statement was presented with the evidence summary. The panel indicated the degree of agreement for the statement. When 80% of the Consensus Group agreed (A+/A) with a statement, this was defined as consensus. All votes were mutually anonymous.Results: Patients with heartburn with a satisfactory response to PPIs, patients with a hiatal hernia (HH), patients with esophagitis LA grade B or higher and patients with Barrett’s esophagus are good candidates for anti-reflux surgery. An endoscopy prior to anti-reflux surgery is mandatory and a barium swallow should be performed in patients with suspicion of a HH or short esophagus. Esophageal manometry is mandatory to rule out major motility disorders. Finally, esophageal pH (+/- impedance) monitoring off PPI is mandatory to select patients for anti-reflux surgery, if endoscopy is negative for unequivocal reflux esophagitis. Conclusion: With the ICARUS guidelines, we generated key recommendations for selection of patients for anti-reflux surgery

Bi-allelic Loss-of-Function CACNA1B Mutations in Progressive Epilepsy-Dyskinesia.

The occurrence of non-epileptic hyperkinetic movements in the context of developmental epileptic encephalopathies is an increasingly recognized phenomenon. Identification of causative mutations provides an important insight into common pathogenic mechanisms that cause both seizures and abnormal motor control. We report bi-allelic loss-of-function CACNA1B variants in six children from three unrelated families whose affected members present with a complex and progressive neurological syndrome. All affected individuals presented with epileptic encephalopathy, severe neurodevelopmental delay (often with regression), and a hyperkinetic movement disorder. Additional neurological features included postnatal microcephaly and hypotonia. Five children died in childhood or adolescence (mean age of death: 9 years), mainly as a result of secondary respiratory complications. CACNA1B encodes the pore-forming subunit of the pre-synaptic neuronal voltage-gated calcium channel Cav2.2/N-type, crucial for SNARE-mediated neurotransmission, particularly in the early postnatal period. Bi-allelic loss-of-function variants in CACNA1B are predicted to cause disruption of Ca2+ influx, leading to impaired synaptic neurotransmission. The resultant effect on neuronal function is likely to be important in the development of involuntary movements and epilepsy. Overall, our findings provide further evidence for the key role of Cav2.2 in normal human neurodevelopment.MAK is funded by an NIHR Research Professorship and receives funding from the Wellcome Trust, Great Ormond Street Children's Hospital Charity, and Rosetrees Trust. E.M. received funding from the Rosetrees Trust (CD-A53) and Great Ormond Street Hospital Children's Charity. K.G. received funding from Temple Street Foundation. A.M. is funded by Great Ormond Street Hospital, the National Institute for Health Research (NIHR), and Biomedical Research Centre. F.L.R. and D.G. are funded by Cambridge Biomedical Research Centre. K.C. and A.S.J. are funded by NIHR Bioresource for Rare Diseases. The DDD Study presents independent research commissioned by the Health Innovation Challenge Fund (grant number HICF-1009-003), a parallel funding partnership between the Wellcome Trust and the Department of Health, and the Wellcome Trust Sanger Institute (grant number WT098051). We acknowledge support from the UK Department of Health via the NIHR comprehensive Biomedical Research Centre award to Guy's and St. Thomas' National Health Service (NHS) Foundation Trust in partnership with King's College London. This research was also supported by the NIHR Great Ormond Street Hospital Biomedical Research Centre. J.H.C. is in receipt of an NIHR Senior Investigator Award. The research team acknowledges the support of the NIHR through the Comprehensive Clinical Research Network. The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR, Department of Health, or Wellcome Trust. E.R.M. acknowledges support from NIHR Cambridge Biomedical Research Centre, an NIHR Senior Investigator Award, and the University of Cambridge has received salary support in respect of E.R.M. from the NHS in the East of England through the Clinical Academic Reserve. I.E.S. is supported by the National Health and Medical Research Council of Australia (Program Grant and Practitioner Fellowship)

Heterozygous Variants in KMT2E Cause a Spectrum of Neurodevelopmental Disorders and Epilepsy.

We delineate a KMT2E-related neurodevelopmental disorder on the basis of 38 individuals in 36 families. This study includes 31 distinct heterozygous variants in KMT2E (28 ascertained from Matchmaker Exchange and three previously reported), and four individuals with chromosome 7q22.2-22.23 microdeletions encompassing KMT2E (one previously reported). Almost all variants occurred de novo, and most were truncating. Most affected individuals with protein-truncating variants presented with mild intellectual disability. One-quarter of individuals met criteria for autism. Additional common features include macrocephaly, hypotonia, functional gastrointestinal abnormalities, and a subtle facial gestalt. Epilepsy was present in about one-fifth of individuals with truncating variants and was responsive to treatment with anti-epileptic medications in almost all. More than 70% of the individuals were male, and expressivity was variable by sex; epilepsy was more common in females and autism more common in males. The four individuals with microdeletions encompassing KMT2E generally presented similarly to those with truncating variants, but the degree of developmental delay was greater. The group of four individuals with missense variants in KMT2E presented with the most severe developmental delays. Epilepsy was present in all individuals with missense variants, often manifesting as treatment-resistant infantile epileptic encephalopathy. Microcephaly was also common in this group. Haploinsufficiency versus gain-of-function or dominant-negative effects specific to these missense variants in KMT2E might explain this divergence in phenotype, but requires independent validation. Disruptive variants in KMT2E are an under-recognized cause of neurodevelopmental abnormalities