Rare case of type II glycogen storage disease

The article presents information about a rare case of Pompe disease. It is a glycogen storage disease. During the third screening of a pregnant woman, the ultrasonography of the fetus’s heart revealed the myocardial hypertrophy of the left ventricle perceived as posthypoxic. After delivery, the newborn underwent the ultrasound examination and molecular genetic studies. Firstly, the hepatomegaly and cardiomegaly were diagnosed. Then an infantile form of Pompe disease was found. The patient got enzyme replacement therapy without positive result. The death occurred at the age of 2 years and 5 months as a result of cardiovascular disease failure. Macroscopically, the sizes of the internal organs were increased. The microscopic examination demonstrated glycogen deposition in the myocardium, skeletal muscles, mucous membranes of the organs of the gastrointestinal tract, liver, kidney, spleen and adrenal gland

Adenomatous lesions of the major duodenal papilla in conjunction with pancreatic heterotopia

The study was carried out on the material of 327 pathoanatomical autopsies taking into account the sex, age, clinical data, pathoanatomical, and clinical diagnoses. The material is divided into two groups: The 1st group - without an ectopic pancreatic tissue in the major duodenum papilla (279 cases) and the 2nd group - with different variants of ectopic pancreatic tissue (48 cases). In each of them, the distribution was carried out according to sex, age, and cause of deat

Evaluation of rat brain morphology following the induction of acute meningitis treated with Ceftriaxone

The present study aimed to assess the histological and immunohistochemical characteristics of the brain of rats that underwent induced acute purulent pneumococcal meningitis after antibiotic therapy with Ceftriaxon

Дирофиляриоз: двухстороннее поражение плевры в сочетании с паразитарной пневмонией под маской туберкулеза

The article describes a rare clinical case of pulmonary dirofilariasis (caused by Dirofilaria repens) manifesting as a combination of bilateral pleural lesions with recurrent exudative pleurisy and parasitic pneumonia mimicking tuberculosis. Based on published data, the pathogenetic mechanisms of the disease development and its specific course are described.Представлено редкое клиническое наблюдение легочного дирофиляриоза (возбудитель Dirofilaria repens), проявляющегося сочетанием двухстороннего поражения плевры с рецидивирующим экссудативным плевритом и паразитарной пневмонией, протекающих под маской туберкулезного процесса. По данным литературы описаны патогенетические механизмы формирования болезни и особенности течения

Density‐ and size‐dependent mortality in fish early life stages

The importance of survival and growth variations early in life for population dynamics depends on the degrees of compensatory density dependence and size dependence in survival at later life stages. Quantifying density‐ and size‐dependent mortality at different juvenile stages is therefore important to understand and potentially predict the recruitment to the population. We applied a statistical state‐space modelling approach to analyse time series of abundance and mean body size of larval and juvenile fish. The focus was to identify the importance of abundance and body size for growth and survival through successive larval and juvenile age intervals, and to quantify how the dynamics propagate through the early life to influence recruitment. We thus identified both relevant ages and mechanisms (i.e. density dependence and size dependence in survival and growth) linking recruitment variability to early life dynamics. The analysis was conducted on six economically and ecologically important fish populations from cold temperate and sub‐arctic marine ecosystems. Our results underscore the importance of size for survival early in life. The comparative analysis suggests that size‐dependent mortality and density‐dependent growth frequently occur at a transition from pelagic to demersal habitats, which may be linked to competition for suitable habitat. The generality of this hypothesis warrants testing in future research.publishedVersio

Эффективность бенрализумаба при тяжелой эозинофильной бронхиальной астме: промежуточный анализ результатов проспективного исследования BEST в условиях реальной клинической практики в России

Systematizing clinical experience in the use of biological treatments in patients with severe bronchial asthma in real world settings with assessment of clinical outcomes and quality of life level is of great interest and value in practical pulmonology.The aim of the BEST study (Real World Evidence of Benralizumab in Eosinophilic Severe AsThma in Russia) was to evaluate effectiveness of benralizumab by control and quality of life associated with respiratory status in patients with eosinophilic phenotype of severe asthma in real clinical practice in Russia.Results. The results obtained through 16 weeks of benralizumab therapy in patients with severe eosinophilic asthma (SEA) were analyzed. All 59 enrolled patients showed significant improvement in the asthma control questionnaire (ACQ-5) score, the quality of life associated with respiratory status according to the St. George Respiratory Questionnaire (SGRQ), and subjective assessments of the well-being of patients and the disease severity (PGIC and PGIS). Treatment with benralizumab contributed to a reduction in the oral glucocorticosteroids intake throughout the study period.Conclusion. In real clinical practice, treatment with benralizumab results in significant improvement in disease control and the quality of life in patients with severe eosinophilic asthma and is characterized by a favorable safety profile.Актуальной задачей современной практической пульмонологии является систематизация опыта применения генно-инженерных биологических препаратов в терапии пациентов с тяжелой бронхиальной астмой (ТБА) в реальной клинической практике с оценкой результатов эффективности и показателей качества жизни (КЖ) больных.Целью исследования Real World Evidence of Benralizumab in Eosinophilic Severe AsThma in Russia (BEST) являлось определение эффективности контроля над заболеванием и КЖ, связанного с состоянием дыхательной системы, у пациентов с ТБА эозинофильного фенотипа на фоне применения бенрализумаба в условиях реальной клинической практики в России.Результаты. У пациентов с ТБА проанализированы промежуточные результаты исследования BEST, полученные через 16 нед. после начала терапии бенрализумабом. У всех больных, включенных в исследование (n = 59), достигнуто значимое улучшение оценки по результатам теста по контролю над БА (Asthma Control Questionnaire – ACQ-5), повышение КЖ, связанного с состоянием дыхательной системы, которое определялось с помощью опросника госпиталя Святого Георгия по оценке качества жизни больных респираторными заболеваниями (St. Georges Respiratory Questionnaire – SGRQ), и субъективных оценок самочувствия пациентов (шкала самооценки пациентом изменения своего состояния (Patients’ Global Impression of Change – PGIC) и шкала самооценки пациентом тяжести заболевания (Patients’ Global Impression of Severity – PGIS)). При терапии бенрализумабом отмечено сокращение приема пероральных глюкокортикостероидов в течение всего периода наблюдения.Заключение. При использовании бенрализумаба в реальной клинической практике существенно улучшаются контроль над ТБА эозинофильного фенотипа и КЖ пациентов при благоприятном профиле безопасности терапии

MONITORING ANALYSIS OF EFFICIENCY OF PROFESSIONAL COMPUTERS AND SYSTEM REQUIREMENTS FOR THE ORGANIZATION OF ELECTRONIC-EDUCATIONAL ENVIRONMENT OF HIGHER EDUCATION INSTITUTIONS IN THE CONDITIONS OF EDUCATIONAL INCLUSION

Introduction: in the process of training specialists in various fields of activity, one of the strategic directions is the education of persons with disabilities. The main task is to make higher education more “sensitive” towards students with disabilities. To give them more freedom of choice, based primarily on the desire of students to get an interesting profession for themselves. One of the main activities on this path is the elimination of all kinds of barriers in education, based on the use of information and communication technologies. Multipurpose use of computer equipment in working with students with HIA allows the introduction of the main classical didactic principles for the implementation of the process of training and education (science, systematic and consistent, conscientiousness and strength of training, students' activity principles and use of visual aids), and also increase the effectiveness of the principle of accounting for age and individual characteristics of students. In the conditions of professional activity in educational organizations, there is a tendency towards the request of high-performance computing means - personal computers (PCs), workstations united by telecommunications computer networks, and the organization faces the problem of choosing the priority of investment. The question is to be asked which information technology object is more priority for the formation of an electronic educational environment. At the same time, on the one hand, it makes sense to make an assumption about investing in technological equipment, the physical deterioration of which (in many cases - extreme) and obsolescence contribute to the deterioration of the quality of the organization of educational activities, its technological capabilities and appreciation. On the other hand, scientific and technical progress offers new solutions and functionalities in the form of modern information technologies (IT). However, in order to achieve maximum  IT, monitoring and evaluation of the computing power of the PC is necessary. In this regard, the goal of the research is to conduct a monitoring analysis of the effectiveness of professional computers and system requirements for the timely identification of obsolete personal computers and forecasting the potential life of personal computers in an educational organization to determine the readiness of a university to create and maintain an inclusive educational environment.Materials and methods: for monitoring the effectiveness of professional computers and system requirements for the organization of the electronic educational environment of the university in terms of educational inclusion, the educational process was divided into three areas: "Engineering graphics", "Programming" and "Computer Science". Within each area, a reference software product was identified - the product that is most often used in this area of study and / or the most costly in terms of resources consumed. For the “Engineering graphics” specialty, the reference product was “AutoCAD”, for the “Programming” specialty - “Microsoft Visual Studio”, for the “Informatics” specialty - “Microsoft Office”.Results: Forecasting in our study is presented as the construction of a trend line (a graph of a prediction function). Using this function, approximate ratings of software products of subsequent versions were calculated, which made it possible to track the physical and moral obsolescence of classrooms.Discussion and Conclusions: long-term experience of using information technologies in the learning process proves the readiness of an educational organization to effectively create an inclusive educational environment, with the goal of developing successful socialization and professional development in students with disabilities