Development of the person of the future engineer as the result of productive training

The article notes a worldwide decline in the productivity of engineering education. The requirements for the engineer put forward by the world leader in the field of production of aviation, space and military equipment, the corporation The Boeing Company are presented. It is proposed to implement the standards of the Global Initiative CDIOВ статье отмечается всемирное падение продуктивности инженерного образования. Приводятся требования к инженеру, выдвигаемые мировым лидером в области производства авиационной, космической и военной техники, корпорацией The Boeing Company. Предлагается реализовать стандарты Всемирной инициативы CDI

PRAXIOCENTRALISM IN THE PROFESSIONAL STANDARD OF THE TEACHER (Continuation of the article)

Introduction. The main objective of the professional standard, systematizing the work function of a teacher, is to describe not personal qualities of teachers but the process and outcome of their activities. The logic of the development of the professional standard involves the analysis of teaching activities and evaluation of its results, which should possess the properties of measurability and diagnosability.The aim of the present publication is to identify praxiological foundations of the professional standard of the teacher via a comparative analysis of the requirementsand fundamental concepts of praxeology – a General theory of successful activities developed by domestic and foreign authors and reflected in monographic and dissertation researches.Methodology and methods of research. Methodological basis of research is the ideas of system, activity and praxiological approaches. Results. The authors prove that pedagogical praxeology as the science and practice of effective organization of pedagogical activity is a new mechanism to describe the characteristics of pedagogical activity and formation of complex of professional portrait of the teacher. Pedagogical praxeology as the mechanism: establishes the framework of competences of the teacher; defines the requirements for structured content, conditions and quality of work of the teacher, their qualifications and competences in the field of professional activity; provides the necessary compliance awareness of teacher’s requirements; describes methods of assessing the development of competencies of teachers; promotes the involvement of the teacher in the task of improving the quality of education; serves as the basis for constructing a continuous trajectory of increasing level of professional training of each teacher.Scientific novelty. The content of professional standards from the standpoint of the praxiological approach is investigated; conceptual ideas of praxeology, defining role and position of praxiocentralism in the professional standard are highlighted; criteria and indicators of an estimation of labour activity of the teacher from the point of view of its efficiency and effectiveness are given.Practical significance. This study contributes to the solution of theoretical and practical problems of correlation existing Federal educational state standards (FESS) and professional standards. This is especially important due to the possibility of subsequent approval and implementation into the pedagogical practices of the next generation of FESS developed on the basis of professional standards

A productive approach to building educational programs in mathematics for csto cadets

For the implementation of educational programs for joint training of cadets mathematics from the member countries of the Collective Security Treaty Organization (CSTO) it is proposed to rely on developed information technologiesРассматриваются вопросы реализации образовательных программ совместного обучения математике курсантов из стран-членов Организации Договора о коллективной безопасности (ОДКБ) с использованием информационных технологи

Value of determining the cerebrospinal fluid protein markers of amyloidosis and neurodegeneration in the diagnosis of vascular and neurodegenerative cognitive impairments

The article presents data on different forms of moderate cognitive impairments (MCI) and the specific features of their transformation to dementia. Cerebrospinal fluid (CSF) was investigated in 60 patients with the amnestic and neurodynamic types of MCI, in 15 patients with vascular dementia (VD), 50 patients with Alzheimer’s disease (AD), and 23 patients with mixed vascular and neurodegenerative dementia (MVND). The specific features of β-amyloid and τ-protein concentrations were established in the preclinical stages of dementia, which reflects the main components of the pathogenesis of neurodegeneration. In the amnestic form of MCI and AD, there was drastically decreased Aβ-42 and increased τ-protein levels in SCF. As cognitive impairments progressed, there was a rise in the concentration of τ-protein; its level correlated with the severity of dementia. In MND, the level of Aβ-42 was significantly reduced while the concentration of τ-protein was much increased; moreover, to a greater extent than in AD and VD. Cerebrovascular damage and neurodegeneration were related to each other and mutually worsened clinical and pathogenic effects

Особенности цитокинового баланса при инфекционных геморрагических колитах у детей

The aim of the study was to study the features of cytokine balance and their effect on clinical and laboratory parameters in infectious hemorrhagic colitis in children. Materials and methods. In the Department of intestinal infections in 2018 observed 28 children with infectious haemorrhagic colitis. The diagnosis was established taking into account clinical recommendations; the severity of the disease was determined by the Clark index; the etiology was verified according to bacteriological and molecular studies of feces, serological methods. In the acute period of the disease, the concentration of cytokines in the blood serum — TNF-a, IL-4, IL-6, IL-10 was studied by enzyme immunoassay. Mann-Whitney method, Kruskal-Wallis dispersion analysis, Pearson correlation method we used to estimate the obtained data.Results. The age structure of patients was dominated by young children (53.6%). Mild form of the disease was diagnosed in 32.1% of patients, moderate — in 67.9%. A high level of IL-4 was observed against a relatively low level of TNF-a, IL-6 and IL-10. Significantly higher concentrations of TNF-a and IL-6 were found in children with moderate form of the disease. The maximum values of IL-6 were observed in young children, IL-10 — in infants. In the moderate form of the disease, negative correlations of IL-4 with the number of leukocytes (r = –0.46; p = 0.05) and platelets (r = –0.48; p < 0.05) in the hemogram noted. Early age patients were found to have correlations of TNF-a with the relative number of rod neutrophils (r = 0.62; p < 0.01). There was a significant correlation of IL-4 with the relative number of segmental neutrophils (r = 0.49; p < 0.05). The relationship of TNF-a with the severity of the disease (r = 0.42; p < 0.05) revealed. The data on the possible suppressive effect of TNF-a and IL-10 on the synthesis of urea and the relationship of IL-6 with reactive changes in the liver obtained.Conclusion. Studies of cytokine balance in infectious hemorrhagic colitis in children showed the severity of systemic inflammatory response with the activation of Th-2 immune response. The correlation of TNF-a with the severity of the disease was established, which may have diagnostic and prognostic value.Цель исследования — изучение особенностей баланса цитокинов и их влияния на клинико-лабораторные показатели при инфекционных геморрагических колитах у детей.В отделении кишечных инфекций в 2018 г. наблюдали 28 детей с инфекционными геморрагическими колитами. Диагноз устанавливали с учетом клинических рекомендаций; тяжесть заболевания определяли по индексу Кларка; этиологию верифицировали по данным бактериологического и молекулярных исследований кала, серологических методов. В острый период заболевания исследовали концентрацию сывороточных цитокинов — ФНО-a, ИЛ-4, ИЛ-6, ИЛ-10 методом ИФА. При оценке полученных данных использовали метод Манна-Уитни, дисперсионный анализ Краскела-Уоллиса, метод корреляции Пирсона. В возрастной структуре больных доминировали дети раннего возраста (53,6%). Легкая форма заболевания диагностирована у 32,1% пациентов, среднетяжелая — у 67,9%. Отмечен высокий уровень ИЛ-4 на фоне относительно низкого уровня ФНО-a, ИЛ-6 и ИЛ-10. Достоверно более высокая концентрация ФНО-a и ИЛ-6 обнаружена у детей со среднетяжелой формой. Максимальные значения ИЛ-6 наблюдались у детей раннего возраста, ИЛ-10 — у детей грудного возраста. При среднетяжелой форме заболевания отмечены отрицательные корреляции ИЛ-4 с количеством лейкоцитов (r = –0,46; p = 0,05) и тромбоцитов (r = –0,48; p < 0,05) в гемограмме. У пациентов раннего возраста выявлены корреляции ФНО-a с относительным количеством палочкоядерных нейтрофилов (r = 0,62; p < 0,01). Отмечена корреляция ИЛ-4 с относительным количеством сегментоядерных нейтрофилов (r = 0,49; p < 0,05). Выявлена связь ФНО-a с тяжестью заболевания (r = 0,42; p < 0,05). Получены данные, позволяющие предположить подавляющее влияние ФНО-a и ИЛ-10 на синтез мочевины и возможную связь ИЛ-6 с реактивными изменениями в печени.Исследования цитокинового баланса при инфекционных геморрагических колитах у детей свидетельствовали о выраженности системной воспалительной реакции с активацией Th-2 иммунного ответа. Установлена корреляция ФНО-a с тяжестью заболевания, что может иметь диагностическое и прогностическое значение

Диагностическое и прогностическое значение нейронспецифичной енолазы и белка S-100 при вирусных энцефалитах у детей

Objective – to study the dynamics of the level of neuron-specific enolase (NSE) and S-100 protein in blood serum and cerebrospinal fluid in children with various features of viral encephalitis course and outcome and to define their role in diagnostics and prognosis.Material and methods. 48 children aged from 1 month to 15 years were examined. Analysis of the level of NSE and S-100 protein was performed by the method of hardphase immune-enzyme analysis.Results. A reliable increase of the level of S-100 protein in blood serum was identified in comparison with the control group in both acute and reconvalescence periods. During acute period higher values of NSE in blood serum were found in children with herpesvirus encephalitis of various etiologies in comparison with varicella zoster encephalitis, and during reconvalescence period – higher values of S-100 protein. In case of the most severe course of the disease the level of NSE in cerebrospinal fluid was reliably higher in comparison with patients in severe condition. Children with convulsive syndrome demonstrated higher concentration of NSE in cerebrospinal fluid than patients without convulsions. In case of formation of stable neurologic deficiency during the disease outcome there was identified an increase of NSE concentration in blood serum in comparison with the recovered patients. It was also identified that if the values of NSE were >7.0 ng/ml, it was possible to predict unfavourable outcome of viral encephalitis with sensitivity of 61.54% and specificity of 71.43%. The highest values of neuron-specific proteins in blood serum were found in the dead patient with herpes encephalitis.Conclusion. Analysis of NSE and S-100 protein in pediatric viral encephalitis is characterized by diagnostic and prognostic significance, demonstrates their role in pathogenesis and can be used for therapy correction.Цель: изучить динамику уровня нейронспецифичной енолазы (NSE) и белка S-100 в сыворотке крови и ликворе у детей с различным характером течения и исхода вирусного энцефалита и определить их роль в диагностике и прогнозе.Материалы и методы: обследовано 48 детей в возрасте от 1 месяца до 15 лет. Исследование уровня NSE и белка S-100 осуществляли методом твердофазного иммуноферментного анализа.Результаты: установлено достоверное увеличение уровня белка S-100 в сыворотке крови по сравнению с контрольной группой, как в остром периоде, так и в периоде реконвалесценции. У детей с герпес-вирусными энцефалитами различной этиологии по сравнению с ветряночным энцефалитом в сыворотке крови в остром периоде обнаружены более высокие значения NSE, в стадии реконвалесценции более высокие значения белка S-100. При крайне тяжелом течении заболевания в ликворе уровень NSE был достоверно выше по сравнению с пациентами в тяжелом состоянии. У детей с судорожным синдромом концентрация NSE в ликворе была выше, чем у больных без судорог. При формировании в исходе заболевания стойкого неврологического дефицита выявлено увеличение концентрации NSE в сыворотке крови по сравнению с выздоровевшими пациентами. Установлено, что при значениях NSE, превышающих 7,0 нг/мл, можно прогнозировать неблагоприятный исход вирусного энцефалита с чувствительностью 61,54% и специфичностью 71,43%. Максимально высокие значения НСБ в сыворотке крови обнаружены у умершего пациента с герпетическим энцефалитом.Заключение. Исследование NSE и белка S-100 при вирусных энцефалитах у детей обладает диагностической и прогностической значимостью, указывает на их роль в патогенезе и может быть использовано для коррекции терапии

Эффективность и безопасность препарата нусинерсен в рамках программы расширенного доступа в России

Introduction. Spinal muscular atrophy is a severe neuromuscular disease characterized by rapid progression of muscle weakness and early death. Pathogenetic therapy with nusinersen can significantly change the course of the disease and enable the patient to acquire new skills. The study of the efficacy and safety of nusinersen therapy in patients with type 1 spinal muscular atrophy should be continued. The aim of the study was to assess the safety and efficacy of nusinersen therapy in patients with type 1 spinal muscular atrophy for 6 months as part of an expanded access program at federal and regional healthcare facilities. Materials and methods. Patients with type 1 spinal muscular atrophy received nusinersen therapy under the expanded access program (NCT02865109). Patients were evaluated before starting treatment and 6 months after starting treatment. Overall motor development and motor function was assessed using the Hammersmith Infant Neurological Status Scale Part 2 and the Philadelphia Pediatric Hospital Neonatal Motor Scale. Results. 41 children aged 6 to 38 months received nusinersen therapy between July 2019 and March 2020. At the time of analysis, all patients were alive and continued treatment. The average improvement on the Hammersmith Hospital Neurological Status Scale was 3.7 points after 6 months of therapy compared to baseline (n = 39, p <0.001). The mean improvement on the Children’s Hospital of Philadelphia Scale for the Diagnosis of Motor Function in Newborns was 9.8 after 6 months of therapy from baseline (n = 30, p <0.001). Conclusion. The efficacy and safety of nusinersen have been demonstrated in real clinical practice in Russia in the treatment of early-onset spinal muscular atrophy.Введение. Спинальная мышечная атрофия – тяжелое нервно-мышечное заболевание, характеризующееся быстрым прогрессированием мышечной слабости, ранним летальным исходом. Применение патогенетической терапии нусинерсеном может существенно изменить течение болезни и дать возможность пациенту приобрести новые навыки. Изучение эффективности и безопасности терапии нусинерсеном у пациентов со спинальной мышечной атрофией 1-го типа неообходимо продолжать. Цель исследования – оценить безопасность и эффективность терапии нусинерсеном пациентов со спинальной мышечной атрофией 1-го типа в течение 6 мес в рамках клинической практики в лечебно-профилактических учреждениях федерального и регионального уровней. Материалы и методы. Пациенты со спинальной мышечной атрофией 1-го типа получали терапию препаратом нусинерсен в рамках программы расширенного доступа (NCT02865109). Оценка пациентов проводилась до начала лечения и через 6 мес после начала лечения. Оценивали общее двигательное развитие, моторную функцию – с помощью Шкалы оценки неврологического статуса больницы Хаммерсмит у детей раннего возраста, часть 2, и Шкалы детской больницы Филадельфии для диагностики двигательных функций у новорожденных. Результаты. Терапию препаратом нусинерсен получил 41 ребенок в возрасте от 6 до 38 мес в период с июля 2019 по март 2020 г. На момент анализа все пациенты были живы и продолжали лечение. Среднее улучшение по Шкале оценки неврологического статуса больницы Хаммерсмит составило 3,7 балла через 6 мес терапии по сравнению с исходным уровнем (n = 39, р <0,001). Среднее улучшение по Шкале детской больницы Филадельфии для диагностики двигательных функций у новорожденных составило 9,8 балла через 6 мес терапии по сравнению с исходным уровнем (n = 30, р <0,001). Заключение. Продемонстрированы эффективность и безопасность нусинерсена в условиях реальной клинической практики в России при терапии спинальной мышечной атрофии 1-го типа с ранним началом

The efficacy and safety of nusinersen within the expanded access program in Russia

Introduction. Spinal muscular atrophy is a severe neuromuscular disease characterized by rapid progression of muscle weakness and early death. Pathogenetic therapy with nusinersen can significantly change the course of the disease and enable the patient to acquire new skills. The study of the efficacy and safety of nusinersen therapy in patients with type 1 spinal muscular atrophy should be continued. The aim of the study was to assess the safety and efficacy of nusinersen therapy in patients with type 1 spinal muscular atrophy for 6 months as part of an expanded access program at federal and regional healthcare facilities. Materials and methods. Patients with type 1 spinal muscular atrophy received nusinersen therapy under the expanded access program (NCT02865109). Patients were evaluated before starting treatment and 6 months after starting treatment. Overall motor development and motor function was assessed using the Hammersmith Infant Neurological Status Scale Part 2 and the Philadelphia Pediatric Hospital Neonatal Motor Scale. Results. 41 children aged 6 to 38 months received nusinersen therapy between July 2019 and March 2020. At the time of analysis, all patients were alive and continued treatment. The average improvement on the Hammersmith Hospital Neurological Status Scale was 3.7 points after 6 months of therapy compared to baseline (n = 39, p <0.001). The mean improvement on the Children’s Hospital of Philadelphia Scale for the Diagnosis of Motor Function in Newborns was 9.8 after 6 months of therapy from baseline (n = 30, p <0.001). Conclusion. The efficacy and safety of nusinersen have been demonstrated in real clinical practice in Russia in the treatment of early-onset spinal muscular atrophy