Clinical manifestations of familial exudative vitreoretinopathy in children with nucleotide sequence alterations in the FZD4 gene

Familial exudative vitreoretinopathy (FEVR)is a rare genetically heterogeneous disease with multiple types of inheritance (autosomal dominant, autosomal recessive, X-linked) and widely varying clinical features. Up to 40 % of cases of FEVR are associated with mutations of the FZD4 gene.Purpose: to investigate the clinical manifestations of FEVR in children with nucleotide sequence alterations in the FZD4 gene. Material and methods. The Helmholtz National Medical ResearchCenter of Eye Diseases and the ResearchCentre for MedicalGenetics conducted a joint in-depth ophthalmological examination of 18 patients aged from 3 weeks to 17 years with a diagnosis of FEVR, which included a detailed ophthalmoscopy under drug mydriasis, ultrasound and electrophysiological examination, photographic recording of fundus changes using RetCam and Fundus Foto. Molecular genetic examination was carried out by direct sequencing according to Sanger. Results. Nucleotide sequence alterations in the FZD4 gene were detected in 3 patients(16.7 %)from two unrelated families. In one family, a 12-year-old girl wasfound to display the firstsymptoms of ophthalmic pathology (reduced vision, strabismus) at the age of 3.5 years. In another family, the clinical manifestations of FZD4 gene mutations were observed in two children during the first year of life (at the age of 5 and 11 months).Conclusions. The clinical picture of 3 patients with detected changes in the nucleotide sequence of the FZD4 gene is characterized by early manifestation and bilateral asymmetric ophthalmoscopic damage. The results of the study indicate the need for a timely diagnosis of FEVR in young children, recommend an interdisciplinary approach to the study of the disease, which should contribute to a better understanding of pathogenesis, and the development of an effective diagnostic, treatment and rehabilitation algorithm.

The process of forming welded joints by high-voltage capacitor welding

The article substantiates the relevance of obtaining welded joints from dissimilar alloys with minimizing the processes responsible for the formation of intermetallic joints by using high-voltage capacitor welding with an induction-dynamic drive. A schematic diagram of high-voltage capacitor welding is given, the physical nature of the work is described. The parts to be welded are connected in series to an electric circuit and an inductor, which is a feature of this method. The physical processes and stages of high-voltage capacitor welding with an induction-dynamic drive are described. Comparison and analysis of high-speed photographic recording with oscillograms of current and voltage is carried out. The presence of an inductive resistance in the discharge circuit shifts the discharge current relative to the voltage. Confirmation of the hypothesis of formation of welded joints in the solid phase is based on the analysis of welded joints by scanning electron microscopy. Energy dispersive analysis of the welded joint zone did not reveal the diffusion of zinc into the AlMg2.5 alloy and magnesium into the B63 alloy. Energy dispersive analysis confirmed the assumption about the formation of a welded joint under conditions of wetting of molten metal by a pulsed electric arc, followed by displacement from the joint zone and plastic deformation of the heated metal. Recommendations are given for taking into account the polarity in high-voltage capacitor welding, depending on the thickness of the thinnest element.

THE ROLE OF THE UNDERSTOOD IN THE DEPOSIT OF THE EVIDENCE IN THE IMPLEMENTATION OF CRIMINAL PROCEDURAL PROOF IN THE PRE-TRIAL STAGES

The article is devoted to the study of procedural aspects related to the effectiveness of the functioning of the institution of attesting witnesses at the pre-trial stages in the framework of modern Russian criminal proceedings. The author considers the issues of the participation of the attesting witness as one of the subjects of proof in the conduct of investigative or other procedural actions, the list of which is enshrined in the current Criminal Procedure Code of the Russian Federation. Its role as a participant in the criminal process in the preservation (deposition) of evidentiary information in the implementation of "control" over the course and results of investigative actions carried out by the criminal prosecution authorities in the course of criminal proceedings is indicated. The situation concerning the improvement of the institution of attesting witnesses, in case the latter is not excluded by the legislator from the criminal procedure law, has been studied from various sides. Various points of view of both leading proceduralists and practitioners on the existing problem are considered: to preserve or exclude (cancel) the institution of attesting witnesses. In support of the author's position on the importance of witnesses as participants in criminal proceedings, examples from practice are given. The procedural status of an attesting witness and his main functions as a participant in the conduct of investigative actions in pre-trial proceedings are disclosed. The issues of the presence of attesting witnesses during the implementation of investigative actions and the use of technical means for fixing the latter have been studied. The features of video and photographic recording of the results of investigative actions in the volume with the participation of attesting witnesses and the obligation to further familiarize this participant with the received video material are revealed.

IDENTIFICAÇÃO DE SÍTIOS DE REPRODUÇÃO DE AEDES AEGYPTI COM AERONAVE REMOTAMENTE PILOTADA (ARP)

Um drone e seus complementos de voo são denominados Sistema de Aeronave Remotamente Pilotada (RPAS - Remotely Piloted Aircraft System), sendo uma ferramenta com ampla gama de aplicações em diversas áreas. A pesquisa prospectou novas possibilidades de uso de RPAS com enfoque no diagnóstico e monitoramento de locais de reprodução de Aedes aegypti. Para isso, objetos considerados como potenciais criadouros de larvas de mosquito foram distribuídos em ambientes que permitiam maior ou menor detecção visual dos alvos (embalagens/recipientes) em quatro ambientes: solo coberto com gramínea seca, solo exposto, solo coberto com gramínea de porte baixo e solo coberto com gramínea de porte alto. Foi utilizado RPAS, Phantom 4 Pro com dispositivo móvel e o programa nativo da RPA para os voos. Sobrevoamos alvos para registro fotográfico em quatro alturas do solo (20m, 30m, 60m e 80m). A detecção visual dos alvos foi realizada por um grupo de 10 pessoas denominado júri. O Júri aferiu a maior ou menor probabilidade de detecção de alvos, em função de três variáveis: tipo de alvo, tipo de ambiente e altura de tomada da fotografia aérea. Fotografias obtidas a 30 metros de altura representaram o maior número de alvos identificados (30% dos alvos). Os alvos mais identificados foram pneu, garrafa PET, latas de cerveja e latas de tinta. Os menos identificados foram vasilhas plásticas coloridas e garrafas de cerveja. A pesquisa colaborou para o aperfeiçoamento de procedimentos operacionais de controle e combate a endemias e epidemias, que poderão identificar possíveis criadouros do mosquito por meio de RPA, monitorando áreas de difícil acesso que ofereçam risco a integridade física das pessoas. Palavras-chave: drone; geotecnologias; arboviroses; dengue. Identification of reproduction sites of Aedes aegypti with remote pilot aircraft (ARP) ABSTRACT: A drone and its flight accessories are called Remotely Piloted Aircraft System (RPAS - Remotely Piloted Aircraft System), being a tool with a wide range of applications in several areas. The research explored new possibilities for the use of RPAS with a focus on the diagnosis and monitoring of breeding sites for Aedes aegypti. For this, objects considered as potential breeding grounds for mosquito larvae were distributed in environments that allowed greater or lesser visual detection of targets (packages / containers) in four environments: soil covered with dry grass, exposed soil, soil covered with low grass. and soil covered with tall grass. Was used RPAS, Phantom 4 Pro with an Ipad Mini 4 mobile device and the DJI GO program for flights. We fly over targets for photographic recording at four heights from the ground (20m, 30m, 60m and 80m). The visual detection of the targets was carried out by a group of 10 people called a jury. The Jury assessed the greater or lesser probability of target detection, depending on three variables: type of target, type of environment and height of aerial photography. Photographs taken at a height of 30 meters represented the largest number of targets identified (30% of the targets). The most identified targets were tires, pet bottles, cans of beer and cans of paint. The least identified were colored plastic canisters and beer bottles. The research helped to improve operational procedures for controlling and combating endemics and epidemics, which may identify possible mosquito breeding sites through RPA, monitoring areas of difficult access that pose a risk to people's physical integrity. Keywords: drone; geotecnologies; arbovírus; dengue.

LOW-COST 3D ACQUISITION OF GEOMETRIC DATA FOR LIVING HERITAGE: ATTEMPTING TO RECORD THE PUDHU MANDAPAM, MADURAI

The driving forces behind the rapid development of accessible 3d modelling acquisition are generally economic. As the requirements for on-site data acquisition technology become cheaper and more user friendly, opportunities for the geographic dislocation of expertise become more viable. In effect, much of the diagnosis of a monuments’ morphology or condition can be made remotely, as a virtual model is constructed. This potential portability serves to reduce the impact, invasiveness and cost of survey and documentation processes. In cases of contested heritage conservation practices, the simple act of photographic recording can cause concern. However, photogrammetric recording is eminently advantageous for its capacity to provide non-destructive means to consider degradation and condition mapping as well as to record and monitor change over time. Here, two rapid surveys taken with portable 360° cameras a year apart, demonstrate the potential value and limitations of deploying recent techniques in order to deliver credible or useful survey data in a highly complex pillared hall that is intensively occupied.

VISUAL ETHNOGRAPHY OF IVAN LOPATIN (ON THE MATERIALS OF THE AMUR EXPEDITION OF 1913)

Статья посвящена актуальной, но слабо разработанной в отечественной гуманитарной науке теме, – визуальной антропологии как форме научного познания, средству фиксации и репрезентации материалов. Как известно, определяющую роль в освоении фронтирных регионов страны в имперский период играли профессиональные исследователи и путешественники, активно использовавшие в своей деятельности фотографическую технику. Многочисленные фотодокументы, собранные ими в научных экспедициях рассредоточены по фондам центральных и региональных музейных и архивных институций. Данное исследование фокусируется на визуально-антропологических материалах из экспедиции известного этнографа Ивана Алексеевича Лопатина 1913 года, хранящихся в архиве Общества изучения Амурского края, и изученных автором статьи в ходе исследовательских работ лета 2020 года. Многие из рассматриваемых фотодокументов, являясь уникальными свидетельствами культурной эволюции народностей Приамурья на рубеже XIX–XX вв., вводятся в научный оборот впервые. Путем сопоставления текстовых и визуальных архивов исследователя выделяются и анализируются ключевые этнографические образы, зафиксированные им среди орочей и гольдов (нанайцев): явления материальной и духовной культуры, промысловые процессы и физические типы. Особо рассматриваются и опережающие свое время методологические открытия И. А. Лопатина в области полевой работы этнографа: проведение параллельной письменной и фотографической фиксации материалов в процессе экспедиционного исследования для последующего использования фотодокументов в качестве иллюстраций к основным положениям научных публикаций. Фотографическое творчество И. А. Лопатина анализируется в историко-антропологическом ключе, в сопоставлении с тематически смежными исследованиями других авторов и академическими парадигмами соответствующего периода. Делается вывод об архивном фотонаследии ученого как информативном историко-этнографическом источнике, визуально отображающем не только культуру снимаемого, но и снимающего; передающем не только этнографическую информацию, но и ее образно-эмоциональный контекст. Исторический опыт И. А. Лопатина показывается в качестве вполне актуального методического примера для сегодняшних визуально-антропологических практик. The article is devoted to a topical but poorly developed topic in the Russian humanities – visual anthropology as a form of scientific knowledge, a means of fixing and representing materials. It is well known, that the decisive role in the development of the frontier regions of the country during the imperial period was played by professional researchers and travelers, who actively used photographic technology in their activities. Numerous photographic documents collected by them during scientific expeditions are scattered among the funds of central and regional museum and archival institutions. This study focuses on visual anthropological materials from the expedition of the famous ethnographer Ivan Alekseevich Lopatin in 1913, stored in the archives of the Society for the Study of the Amur Region, and studied by the author of the article during research work in the summer of 2020. Many of the photographic documents under consideration, being unique evidence of the cultural evolution of the peoples of the Amur region at the turn of the 19th–20th centuries, are introduced into scientific circulation for the first time. By comparing the textual and visual archives of the researcher, the key ethnographic images recorded by him among the Orochs and Golds (Nanai) peoples are distinguished and analyzed: phenomena of material and spiritual culture, fishing processes and physical types. The methodological discoveries of Ivan Lopatin in the field of the ethnographer's field work are also specially considered: parallel written and photographic recording of materials in the process of expeditionary research for the subsequent use of photographic documents as illustrations to the main provisions of scientific publications. The photographic work of Ivan Lopatin is analyzed in a historical and anthropological vein, in comparison with thematically related studies of other authors and academic paradigms of the corresponding period. The conclusion is made about the scientist's archival photographic heritage as an informative historical and ethnographic source, visually reflecting not only the culture of the photograph, but also the one who is filming; transmitting not only ethnographic information, but also its figurative and emotional context. The historical experience of Ivan Lopatin is shown as a completely relevant methodological example for today's visual anthropological practices.

Dismantling a multi-storey building in the conditions of a built-up area: engineering and economic indicators

Introduction. In the contemporary world, the number of multi-storey and high-rise buildings goes up, and this is the case of central districts of large cities. This renewal of built environments in megalopolises takes the form of development of unoccupied spaces, which grow smaller year after year, and liquidation of buildings whose life cycle is coming to an end. In this regard, there arises a problem of choosing the building demolition method, since traditional techniques, used worldwide, do not allow for the liquidation of buildings that have more than 15 floors. Therefore, given the growing number of floors, densely built-up areas, and tighter safety regulations, there is a need to improve dismantling technologies, including tighter environmental safety requirements applied to dismantling works, and this necessity substantiates the relevance of this research. The subject of this work is the engineering and economic indicators of the element-wise deconstruction and mechanical demolition, performed as a sequential set of works that end in the elimination of a multi-storey building, having various design features. The purpose of this study is to explore the engineering and economic indicators of dismantling works carried out in densely built-up urban areas, taking into account work arrangement features, space-planning and design characteristics of a building to be liquidated. Materials and methods. Given the findings of the field observations that represent photographic recording and timing, measurements were taken in respect of the time needed to perform the dismantling work in the process of liquidation of a multi-storey building. The method of mathematical analysis was employed to obtain the engineering and economic indicators of dismantling works. The work space, the scope and amount of work were identified using AutoCAD, a 2D modeling system. Results. The co-authors have identified the features of the element-wise dismantling and mechanical demolition of a multi-storey building, dependences between engineering and economic indicators, as well as the characteristics of buildings. Conclusions. The research, performed by the co-authors, allow to identify the bottlenecks of such methods of dismantling a multi-storey building, as its element-wise dismantling and mechanical demolition. The results of the work can be used to streamline the building dismantling technology, with regard to the features of surrounding buildings and the characteristics of the building to be liquidated.

THE INFLUENCE OF THE PECULIARITIES OF THE ORGANIZATION OF RECREATIONAL AREAS ON THE SUSTAINABLE DEVELOPMENT OF THE LANDSCAPE AND ECOLOGICAL FRAMEWORK OF THE CITY (ON THE EXAMPLE OF KHARKIV CITY)

The article examines the process of degradation of the landscape-ecological framework of the city due to the dominance of the human-centered approach to the urban recreational areas’ organization. Using the methods of cartographic analysis, full-scale photographic recording, synthesis and abstraction, a study of the recreational territories of Kharkov city was carried out and three main types of recreational objects development were identified: small recreational objects (up to 10 hectares), which completely lost the opportunity for sustainable development, self-creation and are completely dependent from human withdrawal; medium-sized district and city parks, which are being reorganized into urban parks and amusement parks with a high level of recreational load; large elements of the landscape-ecological framework, which are degrading due to the complete absence of the recreational organization of the territory. It was also determined that the main reasons for the loss of sustainable development by recreational facilities are: lack of a comprehensive strategy of the system of landscape and recreational areas development for the city; making situational decisions about the reconstruction of recreational facilities in the city; inconsistency of the park’s type within its place in the structure of the landscape-ecological framework of the city; preference for the organization of park areas with high recreational loads; irregular organization of recreational areas in the city. The main steps of the strategy for the integrated development of the city landscape-recreational system are proposed, which are as follows: determining the level of value of a recreational facility in the structure of the city landscape-ecological framework, assessing the sustainability of a recreational facility development at the present stage, predicting the types of recreational organization of territories, the prospective level of recreational load, and the level of domestication of the object in order to preserve its sustainable development in the future.

Morphometric traits of selected heart veins of standard and mutational colour variants of American mink (Neovison vison)

The aim of the study was to estimate the values of metric traits of selected mink heart veins of standard and mutation colour variants. The study was conducted on 342 hearts of seven-month-old males and 405 hearts of seven-month-old females. Mink colour, resulting from mutation or from crossbreeding mutational colour variants with each other. Metric traits of the coronary sinus, great cardiac vein, middle cardiac vein, posterior vein, and small cardiac vein were determined by photographic recording of the sub-epicardial picture of these vessels after filling them with a water-dilutable dye for acrylic paints together with a 30% food gelatin solution. In addition, the number of the veins forming the great cardiac vein and that of the posterior veins of the left ventricle were determined. Based on the measurement of the width and length of the mink hearts, the heart shape was determined. Differences were found between the traits of the mink heart vessels of standard and mutational colour variants, but they did not allow to formulate clearly the thesis about the effect of mutations on these traits.

Performance Analysis of Photographic Equipment Used on the ISS RS from 2001 to 2021 to Perform Visual Observations of the Earth’s Surface. Features of Photographic Recording Using Ultra-Long Focus Optics.

The paper analyzes the characteristics of digital cameras with a set of inter-changeable lenses and extenders, which were part of the ISS RS standard equipment from 2001 to 2020 and were used by Russian cosmonauts to per-form visual observations of the Earth's surface in the interests of environmental examination of various areas and objects, improvement of methods of observation, evaluation and forecast of potentially hazardous or catastrophic phenomena. The paper also presents the features of photographic recording of ground objects using ultra-long focus optics.