Temperature, composition and age of the Kara Sea Shelf sediments in the area of the Marre-Sale Geocryological Station

The paper presents results of the study of the uppermost 20 m-thick layer of the near-Yamal shelf bottom sediments, penetrated in May 2014 by two VSEGINGEO boreholes equipped with LРС loggers, with an aim of the temperature regime dynamics monitoring in the nearshore bottom sediments, both for the research purposes and in as much as the data add value to the forthcoming hydrocarbon resource development on the Russian continental shelf. On the basis of the temperature variation observations during three summer months of 2014, it has been established that marine silty clays and aleurites composing the bottom sediment section, represent relict frozen deposits subjected to cryogenic metamorphism in the subaerial exposure environment. Diatom assemblages occurring in aleurite and clayey deposits consist exclusively of the marine extinct species typical of the Early Eocene Pyxilla gracilis diatom zone. A modern marine sublittoral diatom assemblage is found inhabiting the sands of the upper part of the onshore borehole section

Population-genetic structure of the residents of Central Russia

In the territory of Central Russia, the presence of a certain, ordered system of grouping of elementary populations (raions) has been established, according to the data on the frequencies of surnames, using various methods of multivariate statistics (cluster analysis, multidimensional scaling, factor analysis). All the raions of the Belgorod oblast are combined into four clusters, corresponding to real geographical location of the populations: central cluster (12 raions), western (4 raions), south-eastern (3 raions), eastern (2 raions). Geographical distances between them play a significant role in the formation of genetic division between elementary population

Novel GLIS3 mutation in patient with neonatal diabetes mellitus and congenital hypothyroidism (NDH-syndrome)

Mutations in the GLIS3 gene encoding the GLIS3 transcription factor are cause of a rare syndromic form of neonatal diabetes mellitus (NDM) with congenital hypothyroidism. Additional features include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay and other anomalies. This disease in foreign literature is called NDH-syndrome (Neonatal diabetes and Hypothyroidism syndrome).We present the description of a patient with this syndrome with novel homozygous GLIS3 mutation.Our patient is a female, who was born with a weight of 1680 gr, length of 44 cm to consanguineous parents. She developed diabetes on 2 day after birth, requiring continuous intravenous insulin. On day 5 of life hypothyroidism was identified. ­Thyroid anatomy was normal on ultrasound scan. NDH syndrome was suspected.Genetic analysis revealed a novel homozygous mutation c.1836delT, p.Ser612ArgfsTer33 in exon 5 in GLIS3 gene.To date, the patient is followed up for 4 years in total. Currently, growth retardation, psychomotor and speech development persist. Carbohydrate metabolism and thyroid profile has been subcompensated against the background of replacement therapy. No other components of the syndrome have been identified.In this report, we have demonstrated the features of the neonatal diabetes mellitus in a patient with a defect in the GLIS3 gene. Early genetic verification of the diagnosis contributes to the timely starting of personalized therapy, can improve the quality of life of such patients, and, given the nature of inheritance, is necessary for medical genetic counseling of the family

Состояние радиационной обстановки на территории Орловской области, подвергшейся радиоактивному загрязнению вследствие аварии на Чернобыльской АЭС

Research objective is retrospective analysis of radiation conditions in the Oryol region during 1986- 2015 and assessment of efficacy of the carried out sanitary and preventive activities for population protection against radiation contamination caused by the Chernobyl NPP accident.Article materials were own memoirs of events participants, analysis of federal state statistic surveillance forms 3-DOZ across the Oryol region, f-35 “Data on patients with malignant neoplasms, f-12 “Report on MPI activities”. Risk assessment of oncological diseases occurrence is carried out on the basis of AAED for 1986- 2014 using the method of population exposure risk assessment due to long uniform man-made irradiation in small doses. Results of medical and sociological research of genetic, environmental, professional and lifestyle factors were obtained using the method of cancer patients’ anonymous survey. Data on "risk" factors were obtained from 467 patients hospitalized at the Budgetary Health Care Institution of the Oryol region “Oryol oncology clinic”; a specially developed questionnaire with 60 questions was filled out.The article employs the method of retrospective analysis of laboratory and tool research and calculation of dose loads on the Oryol region population, executed throughout the whole period after the accident.This article provides results of the carried out laboratory research of foodstuff, environment objects describing the radiation conditions in the Oryol region since the first days after the Chernobyl NPP accident in 1986 till 2015.We presented a number of activities aimed at liquidation of man-caused radiation accident consequences which were developed and executed by the experts of the Oryol region sanitary and epidemiology service in 1986-2015. On the basis of the above-stated one may draw the conclusions listed below. Due to interdepartmental interaction and active work of executive authorities in the Oryol region, the population of the territories impacted by radioactive contamination is provided with conditions in compliance with radiation safety requirements and hygienic specifications.Since January 1987 according to radiation and hygienic monitoring in the Oryol region, the local foodstuff did not exceed the hygienic specifications on radiation. In the territories included in the zone of radioactive contamination, the economic activities may be carried out without special protective measures for decrease of caesium-137 and strontium-90 content in produce, there are no restrictions on picking of wild-growing berries, herbs and mushrooms.Being direct participants of development and carrying out of activities for the region protection in case of emergency situation at the radiation hazardous site, we believe that the data presented in this article may be useful both for experts and general population.Целью исследования являлся ретроспективный анализ радиационной обстановки на территории Орловской области за период 1986-2015 гг. и оценка эффективности проведенных санитарно-профилактических мероприятий по защите населения от радиационного загрязнения в результате аварии на Чернобыльской АЭС. Материалом для статьи являлись собственные воспоминания участников тех событий, формы федерального государственного статистического наблюдения 3-ДОЗ по Орловской области, ф-35«Сведения о больных злокачественными новообразованиями», ф-12 «Отчет о работе ЛПУ». Оценка риска возникновения онкологических заболеваний проведена на основании СГЭД за 1986-2014 гг. в соответствии с методикой оценки радиационного риска у населения за счет длительного равномерного техногенного облучения в малых дозах. Результаты медико-социологического исследования генетических, средовых, профессиональных факторов и образа жизни получены методом анонимного анкетирования больных онкологическими заболеваниями. Сведения о факторах риска получены у 467 пациентов, находящихся на стационарном лечении в БУЗ Орловской области «Орловский онкологический диспансер», с заполнением специально разработанной анкеты из 60 вопросов.В статье использован метод ретроспективного анализа лабораторных, инструментальных исследований и расчетов дозовых нагрузок на население Орловской области, выполненных на протяжении всего периода после аварии.В настоящей статье приведены результаты проведенных лабораторных исследований пищевых продуктов, объектов внешней среды, характеризующие радиационную обстановку на территории Орловской области с первых дней после аварии на Чернобыльской АЭС в 1986 г. и до 2015 г. Нами представлен объем мероприятий, направленных на ликвидацию последствий техногенной радиационной аварии, которые были разработаны и выполнены специалистами санитарно-эпидемиологической службы Орловской области в 1986-2015 гг. На основании вышеизложенного можно сделать следующие выводы. На территории Орловской области в результате широкого межведомственного взаимодействия и активной работы исполнительной власти обеспечено проживание населения на территориях, подвергшихся радиоактивному загрязнению, с соблюдением требований и гигиенических нормативов радиационной безопасности.По данным радиационно-гигиенического мониторинга, с января 1987 г. в Орловской области в продовольственном сырье и пищевых продуктах, выращенных на территории области, не выявлялись превышения гигиенических нормативов по радиологическим показателям. Хозяйственная деятельность на территориях, отнесенных к зоне радиоактивного загрязнения, может проводиться без применения специальных защитных мероприятий по снижению содержания цезия-137 и стронция-90 в производимой продукции, отсутствуют ограничения на сбор дикорастущих ягод, трав, грибов.Являясь прямыми участниками разработки и реализации мероприятий по защите региона при возникновении чрезвычайной ситуации на объекте, представляющем радиоактивную опасность, полагаем, что данные, представленные в данной статье, могут быть полезны как специалистам, так и населению

Scale-dependent plant diversity in Palaearctic grasslands: a comparative overview

Here we present an extensive overview of plant diversity values in Palaearctic grasslands for seven standard grain sizes from 0.0001 to 100 m². The data originate from 20 studies, including the Field Workshops of the Eurasian Dry Grassland Group (EDGG), ranging geographically from Spain in the west to Siberia in the east, from Sicily in the south to Estonia in the north and from the sea coast up to 3100 m a.s.l. The majority of data is from dry grasslands (Festuco-Brometea, Koelerio-Corynephoretea, Cleistogenetea squarrosae), but there are also some mesic, wet, saline, acidic, alpine and Mediterranean grasslands included. Among others, we compiled data from 1795 1-m², 1109 10-m² and 338 100-m² plots. In all cases we present mean, minimum and maximum richness for the seven grain sizes, plus, in cases where also terricolous bryophytes and lichens had been recorded, the same values for total “plant” species richness, non-vascular plant species richness and fraction of non-vascular plants. The maximum richness values were 82, 101 and 134 for all “plants”, and 79, 98 and 127 vascular plants at grain sizes of 1 m², 10 m² and 100 m², respectively (all in Transylvania, Romania). Our overview comprises new, hitherto unpublished world records of vascular plant species richness at the scales of 0.0001 m² (9) and 0.001 m² (19, both shoot presence), from meso-xeric, basiphilous grasslands in Navarre, Spain, which is much higher than the previously known maxima. The highest values of non-vascular plant richness at 1 m², 10 m² and 100 m², respectively, were 49, 64 and 64, respectively (all in Sedo-Scleranthenea communities of Öland, Sweden, and Saaremaa, Estonia). In general, the dry, alpine and Mediterranean grasslands were much richer than the studied mesic, wet or saline grasslands at any spatial scale. The presented set of mean, minimum and maximum values and their metadata is publically available and will be continuously updated. These data can serve as a reference of “normal” richness, both in fundamental and applied research. To facilitate the application, we provide an easy formula based on the power-law species-area relationship that allows the estimation of richness values at intermediate grain sizes not included in our dataset. In conclusion, our data emphasise the role of Palaearctic grasslands as global hotspot of small-scale vascular plant diversity, while at the same time highlighting that in some grassland types also the bryophyte and lichen diversity can be extraordinarily high

Ocrelizumab in treatment of primary-progressive multiple sclerosis: systematic review

Aim. To analyze the efficacy, safety and pharmacoeconomic indicators of ocrelizumab in adult patients with primary progressive multiple sclerosis (PPMS). Methods. An information search was conducted in the databases Embase, PubMed, Cochrane and eLibrary.ru. The levels of evidence were determined in the studies. Results. Therapy with ocrelizumab compared with placebo characterized by a decrease in the rate of progression of the disease. Treatment with ocrelizumab was associated with a significant slowdown in progression compared to other drugs: rituximab, fingolimod, myelin basic protein peptide 82–98, intravenous immunoglobulin; plasmapheresis / plasma metabolism, corticosteroids, general irradiation of lymphoid tissue, and other most common adverse events: infusion reactions, nasopharyngitis, upper tract respiratory and urinary tract infections, headaches. Life years and quality-adjusted life years for patients receiving ocrelizumab were 16.11 and 3.33, compared with 15.61 and 2.75 for patients receiving better supportive care, respectively. The annual average potential impact on the budget for 1 patient with PPMS in the treatment of ocrelizumab for 5 years ranged from $ 18,300 to 44 200. Conclusions. Ocrelizumab is the only drug that has proven its clinical efficacy in the previously non-curable type of multiple sclerosis, PPC, with risk profile acceptable with respect to clinical benefits

Влияние медико-социальных факторов на отношение родителей к специфической профилактике инфекционных заболеваний у детей Саратовской области в период пандемии новой коронавирусной инфекции

Objective: to analyze the influence of medical and social factors on the attitude of parents to the specific prevention of infectious diseases in children during the pandemic of a new coronavirus infection. Material and methods. Anonymous questioning of parents on the attitude to vaccination was conducted. A specially designed questionnaire included questions about attitudes towards vaccines included in the National Immunization Schedule, as well as those not included in it, in particular, the desire of parents to vaccinate their children against COVID-19 was assessed. In total, 153 people aged 17 to 42 took part in the survey — parents of children aged 1 to 14 years. Results. All parents under 18 years of age support vaccination in accordance with the National Vaccination Calendar; 52.1% of parents aged 18—25 years (p < 0.001), 55.6% of parents aged 26—35 years (p < 0.001 compared to parents under 18 years old), and 66.7% of parents over 35 years old (p < 0.001 compared to parents under 18 years old and parents 18—35 years old). Conclusion. The main medical and social factors associated with the refusal to carry out preventive vaccinations for their children are: the age of parents under 35, incomplete higher education and trust in information received on the Internet. The results obtained indicate the need to determine the «risk group» among parents who express doubts about the need for vaccination. It is necessary to conduct educational programs for them in order to form a positive attitude towards the specific prevention of infectious diseases.Цель: анализ влияния медико-социальных факторов на отношение родителей к специфической профилактике инфекционных заболеваний у детей в период пандемии новой коронавирусной инфекции. Материалы и методы. Проводилось анонимное анкетирование родителей по вопросам отношения к вакцинопрофилактике. Специально разработанная анкета включала в себя вопросы: об отношении к вакцинам, входящим в Национальный календарь профилактических прививок, а также не входящим в него, в частности, оценивалось желание родителей вакцинировать своих детей против COVID-19. Всего в анкетировании приняли участие 153 человека в возрасте от 17 до 42 лет — родители детей в возрасте от 1 года до 14 лет. Результаты. Поддерживают вакцинацию в соответствии с Национальным календарём прививок все родители младше 18 лет; 52,1% родителей в возрасте 18—25 лет (р < 0,001 по сравнению с родителями младше 18 лет), 55,6% родителей в возрасте 26—35 лет (p < 0,001 по сравнению с родителями младше 18 лет), и 66,7% родителей старше 35 лет (p < 0,001 по сравнению с родителями младше 18 лет и родителями 18—35 лет). Заключение. Основными медико-социальными факторами, ассоциированными с отказом от проведения профилактических прививок своим детям являются: возраст родителей до 35 лет, неоконченное высшее образование и доверие к информации, полученной в сети Интернет. Полученные результаты свидетельствуют о необходимости определения «группы риска» среди родителей, высказывающих сомнения в пользе вакцинопрофилактики. Для этой группы родителей требуется проведение образовательных программ с целью формирования позитивного отношения к специфической профилактике инфекционных заболеваний

Клинические рекомендации по оказанию медицинской помощи пациентам с болезнью Помпе

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