Some features of the bioelectric activity of the muscles with prolonged hypokinesia

The effects of prolonged hypokinesia, brought on by confinement to bed and the attendant lack of motor activity, on the bioelectric activity of muscles are studied. Electromyographic measurements of amplitude and frequency indicators of muscular bioelectric activity were analyzed

Monitoring of effectiveness of rehabilitation-pedagogical work on the development of verbal communication of preschoolers with hearing impairments

The purpose of this article is to acquaint the participants of the rehabilitation-educational process (administration, specialists in methods, defectologists, surdopedagogues, logopedists and parents) with the suggested algorithm and the system of monitoring the effectiveness of rehabilitation-pedagogical work on the development of speech communication of preschool children with hearing impairments.В статье дается определение понятия "мониторинг", характеризуются разные виды мониторинга и рассматривается система мониторинга эффективности коррекционно-педагогической работы по развитию речевого общения дошкольников с нарушением слуха

Osteogenesis imperfecta as a cause of death

Osteogenesis imperfecta (OI) is a rare heterozygous connective tissue disordercaused by mutations in genes that affect collagen components (in most cases mutations in COL1A1 и COL1A2 genes). The current classification system includes 15 types of OI, one of which (type II) is characterized by 100% intrauterine or perinatal mortality. The structure of mortality in other OI types is poorly understood because of the heterogeneity of clinical symptoms and the severity of connective tissue damage. W present a clinical case of type III osteogenesis imperfecta, complicated by generalized osteoporosis with multiple fractures of vertebrae and tubular bones and progressive kyphoscoliosis. Late-initiated treatment led to progression of the disease and led to cardiopulmonary insufficiency and death of the patient. Our clinical case highlights the importance of timely diagnosis, treatment and regular observation in patients with OI

Efficiency estimation of mammographic screening among women's population Khanty-Mansiysk for the period 2008-2016

Early diagnosis of breast cancer certainly affects the reduction in mortality from this disease. the article shows and analyzes the indicators that determine the effectiveness of mammographic screening among the female population of Khanty-Mansiysk in the period from 2008 to 2016. the screening procedure for breast cancer in the period under review covered 22681 women of age groups 40-49 years old, 50 years and older. All examined women underwent a mammogram in direct and oblique projections at intervals of 2 years. Analysis of the results showed the relevance and significance of breast cancer screening. In the 40-49 age group, the detection rate of breast cancer per 1000 surveyed was 3.7, in the 50-year-old group and the scare, the corresponding figure was 5.1. the article also substantiates the importance of controlling the incidence of breast cancer in patients of different age groups.Ранняя диагностика рака молочной железы (РМЖ), безусловно, влияет на снижение смертности от данного заболевания. В статье приведены и проанализированы показатели, определяющие эффективность маммографического скрининга среди женского населения г. Ханты-Мансийска в период с 2008 по 2016 годы. Процедура скрининга РМЖ в рассматриваемый период охватила 22681 женщин возрастных групп 40-49 лет, 50 лет и старше. Всем обследованным женщинам была проведена маммография в прямой и косой проекциях с интервалом в 2 года. анализ результатов показал актуальность и значимость скрининга РМЖ. В группе женщин 40-49 лет показатель выявления РМЖ на 1000 обследованных составил 3,7, в группе 50 лет и старше соответствующий показатель составил 5,1. В статье также обосновывается важность контроля заболеваемости РМЖ у пациентов разных возрастных групп

10-year results of the breast cancer screening program in Khanty-Mansiysk state region-Ugra

The main aim of this study is to evaluate the quality of the Breast Cancer Screening Program performed in the Khanty-Mansiysk State Region - Ugra. The screening covers women over 40 years old. The screening interval is 2 years, with two-view mammography and single reading as the standard. During 2007-16 within the Program, 451139 women were screened in the region. The screening coverage rate (2015-16 round) was 41%. 9,3% of screened were referred for further assessment. The screen detection rate was 3.5 per 1000 screened. The test sensitivity for the first round was estimated as 80%. A 42% increase in newly diagnosed stage t1n0M0 breast cancer and 21% reduction in breast cancer detected with tumour size over 20 mm were observed compared to expected absolute number from the 2002-06 trend. Mammographic screening has contributed to a significant reduction in breast cancer mortality – 23,7% reduction using absolute mortality results. Continuous 10-year mammographic screening in Ugra has contributed to a significant improvement in the early disease diagnosis and, as a result, a reduction in absolute mortality rate from breast cancer were observed. In clinical terms, since the findings to date suggest a benefit of the screening, the main aim should be to improve coverage of the population with screening.Цель исследования – оценка качества проведения маммографического скрининга рака молочной в Ханты-Мансийском автономном округе Югре в период с 20007 по 2016 год. Стратегия скрининга: возраст – старше 40 лет, интервал между обследованиями – 2 года, 2 проекции каждой молочной железы, одно прочтение маммограмм (single reader). В 2007- 2016 гг. было обследовано 451139 женщин. дополнительное обследование рекомендовано в 9.3% случаев. Показатель выявления рака молочной железы при скрининге составил 3.5 на 1000 обследованных. охват целевого населения скрининговой маммографией за раунд (2015-2016 гг.) - 41%. Чувствительность скринингового теста - 80%. В целом было отмечено, что в 2016 году фактически абсолютное количество впервые выявленных женщин с РМЖ 1 стадии (T1N0M0) на 42% больше в сравнении ожидаемыми цифрами, в стадии т2+ - на 21% меньше прогнозируемого. Фактическое количество женщин, умерших от РМЖ в 2016 году, на 23.7% ниже, чем прогнозируемое. таким образом, Улучшение ранней диагностики рака молочной железы, как результат реализации постоянно действующего маммографического скрининга в ХМАО - Югре обеспечивает дальнейшее снижение смертности от рака молочной железы среди женщин в возрасте старше 40 лет при условии продолжения увеличения охвата скринингом целевого населения

First description of a type v osteogenesis imperfecta clinical case with severe skeletal deformities caused by a mutation p.119C> T in IFITM5 gene in Russia

Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder. Main clinical manifestations include recurring pathological fractures and progressive skeletal deformation. Five types of OI are distinguished based on clinical symptoms. In most cases, the disease is caused by mutations in the COL1A1 and COL1A2 genes, leading to a defect of type 1 collagen synthesis, which is the main component of the bone matrix. Up to 5% of patients with OI have a mutation in IFITM5 gene, which leads to the development of OI type V. Approximately 150 cases of the OI type V are described in the literature, and mutation c.-14C T in IFITM5 gene is found in most of the cases. Only 5 patients have a c.119C T: p.S40L.mutation. Pathogenesis of OI type V is not fully understood. It is assumed that mutations in the IFITM5 gene cause impaired osteoblastogenesis, decreased bone mineral density and multiple low-traumatic fractures. There is probably a phenotype-genotypic correlation in cases with different mutations of the IFITM5. However, it is currently difficult to assess the relationship in view of the variability of the characters and the low prevalence of the OI type V. We present the first description in Russia of the clinical case of an adult patient with OI type V due to a rare mutation p.119C T: p.S40L in the IFITM5 gene

Features of Laboratory Design for Measuring Environmental and Working Environment Parameters

Статья посвящена особенностям проектирования лабораторий, деятельность которых направлена на измерения (испытания, исследования) параметров окружающей и рабочей сред в условиях ФГАОУ ВО «Севастопольский государственный университет».The article is devoted to the design features of laboratories whose activities are aimed at measuring (testing, research) the parameters of the environment and working environ-ments in the conditions of the Sevastopol State University

Analysis of the Procedure for Confirming the Conformity of Alcoholic Beverages in Accordance with the Requirements of the Technical Regulations of the EAEU TR 047

Статья посвящена анализу и разработке алгоритмов подтверждения соответствия алкогольной продукции в соответствии с требованиями технического регламента ТР ЕАЭС 047: алгоритм декларирования алкогольной продукции и алгоритм сертификации алкогольной продукции.The article is devoted to the analysis and development of algorithms for confirming the conformity of alcoholic beverages in accordance with the requirements of the technical regulations of the EAEU TR 047: the algorithm for declaring alcoholic beverages and the algorithm for certifying alcoholic beverages

Rare genetic diseases of the bone tissue: the case of a family with osteogenesis imperfecta and X-linked hypophosphataemia

Osteogenesis imperfecta (OI) and X-linked hypophosphataemia (XLH) are rare genetic diseases, which lead to childhood-onset bone fragility, low-trauma fractures and limb deformities. OI occurs as a result of impaired type 1 collagen synthesis at different stages, depending on the type of a genetic mutation, which leads to bone strength impairment. In most cases OI is a disorder with an autosomal dominant inheritance. However, there are also cases of autosomal recessive inheritance. To date, 16 types of OI are distinguished, with type 2 being the most severe due to 100% mortality rate in neonatal and perinatal periods. XLH is characterized by altered bone mineralization due to impaired phosphorus absorption and reabsorption, as a result of mutations in the PHEX gene. The bone tissue softens, and this process is accompanied by deformities in long tubular bones. In this article we describe the family, in which both diseases are presented, despite their rarity. The case is investigated from points of view: the clinicians and the patients perspective

КЛИНИЧЕСКИЙ СЛУЧАЙ ГРИППА А(H1N1), ОСЛОЖНЕННОГО ОСТРЫМ ДИССЕМИНИРОВАННЫМ ЭНЦЕФАЛОМИЕЛИТОМ

The defeat of the central nervous system in influenza reflects the properties of both the pathogen itself and the complex pathogenetic mechanisms of the influenza infectious process.  Existing modern  theories do not fully explain the pathological conditions of influenza in the central nervous system, which is still accompanied by ambiguous clinical arguments about the direct cytopathic effect of the influenza virus on neural tissue with the development of encephalitis. Another rare complication of the flu is acute disseminated encephalomyelitis. The autoimmune mechanism of the development of this disease is universally recognized, despite the continuing difficulties of diagnosis in the absence of oligoclonal antibodies in blood plasma and spinal cerebral fluid in the majority of patients.Поражение центральной нервной системы при гриппе отражает свойства как самого возбудителя, так и сложные патогенетические механизмы гриппозного инфекционного процесса. Существующие современные теории до конца не объясняют патологические состояния при гриппе, реализуемые в ЦНС, что до сих сопровождается неоднозначностью клинических рассуждений о прямом цитопатическом  действии вируса гриппа  на нервную ткань с развитием энцефалита. Другим редким осложнением гриппа является острый диссеминированный энцефаломиелит. Аутоимунный механизм развития данного заболевания является общепризнанным, несмотря на сохраняющиеся сложности диагностики при отсутствии олигоклональных антител в плазме крови и спино-мозговой жидкости у большей части пациентов