BACKGROUND Mucopolysaccharidosis type Ⅱ(MPS Ⅱ)is a chronic inherited disease with multiorgan involvement,a progressive course,and restricted life expectancy.AIM To evaluate the predictors of fatal outcomes in MPS Ⅱ...BACKGROUND Mucopolysaccharidosis type Ⅱ(MPS Ⅱ)is a chronic inherited disease with multiorgan involvement,a progressive course,and restricted life expectancy.AIM To evaluate the predictors of fatal outcomes in MPS Ⅱ patients.METHODS In the retrospective cohort study,the clinical,laboratory data and enzyme replacement therapy(ERT)(84.2%)of about 160 patients were extracted and analyzed from the Russian MPS Ⅱ registry,with death as a primary outcome.We compared patients who died(n=20;12.5%)with severe form(n=13;68.4%)and attenuated form(n=6,31.6%)to 140 alive patients.RESULTS Fatal outcomes occurred in 5%,35%,20%,and 40%of patients before 10,10-14,15-19,and≥20 years.The most common causes of death were cardiovascular(29.4%),respiratory failure(17.6%),including pneumonia(17.6%),and their associations(17.6%)and MPS Ⅱ progression(11.8%).Acute or chronic respiratory failure was in 53%.Died patients had higher birth weight,higher age of diagnosis,and start of ERT.Hydrocephalus,hydrocephalus bypass surgery,epilepsy,difficulty swallowing,and impaired movement after 12 years of age were significantly more common in the deceased patients.Cox regression analysis has revealed the following time-dependent covariates of the lethal outcome:1^(st)-year psychomotor development delay,delayed mental and speech development,hydrocephalus,swallow disorders,impossible walking at age>12 years,respiratory disorders,tracheostomy,neuronopathic form.CONCLUSION Increased birth weight,delayed diagnosis and the start of ERT,and development of neuronopathic form with impossible walking after 12 years were the main predictors of the fatal outcome.展开更多
The Russian Banquet-Treasures of Dining from the Moscow Kremlin Museums Collection presents the 500-year history of Russian food culture from the 16th century to the 20th century,allowing the visitors to see the chang...The Russian Banquet-Treasures of Dining from the Moscow Kremlin Museums Collection presents the 500-year history of Russian food culture from the 16th century to the 20th century,allowing the visitors to see the changes and origins of Russian food from the Middle Ages of the Tsarist era to the modern era.展开更多
The study examines the socio-cultural context of Russian music in the decades immediately preceding and following the October Revolution of 1917,focusing on several aspects:personalities representing pre-and post-revo...The study examines the socio-cultural context of Russian music in the decades immediately preceding and following the October Revolution of 1917,focusing on several aspects:personalities representing pre-and post-revolutionary Russian(in exile)and Soviet(at home)musical activity and culture;second-tier national composers;“industrial music”of the early Soviet period;and Soviet cultural management in music behind the Iron Curtain.Portraits of composers little known to Western listeners and academic audiences are presented.The spectrum of musical styles and aesthetics is considered.Issues of ethnic diversity and religious affiliation of Russian composers are relevantly explored.Songs of the First Revolution(1905),the Civil War,and Soviet mass songs are briefly touched upon.The scope and choice of themes of the study provide a holistic approach to topics that are usually addressed separately:before or after the 1917 Revolution;classical music or folk/popular songs.展开更多
BACKGROUND Mucopolysaccharidosis typeⅥ(MPSⅥ)is a chronic,progressive,inherited disease with multiorgan involvement and a restricted life expectancy.AIM To investigate the epidemiological,clinical,and genetic charact...BACKGROUND Mucopolysaccharidosis typeⅥ(MPSⅥ)is a chronic,progressive,inherited disease with multiorgan involvement and a restricted life expectancy.AIM To investigate the epidemiological,clinical,and genetic characteristics of patients with mucopolysaccharidosis type 6 and their outcomes using the Russian Federation's national registry,as per the Russian registry,and compare them with previously published data.METHODS In a retrospective cohort study,clinical,laboratory data,molecular genetic analysis results,and enzyme replacement therapy(ERT)data were extracted and analyzed from the Russian MPSⅥregistry for 53 patients,comprising 26 males(49.1%)and 27 females(50.9%).RESULTS The median age of first symptoms was 2 years,ranging from the first months of life to 20 years.A positive family history of MPSⅥwas reported in 19/53(35.8%)patients,a negative family history in 24(45.3%),and missing information in 10(18.9%).The main features of the disease were hepatomegaly(n=23;60.5%),splenomegaly(n=15,39.5%),involvement of otolaryngological organs(n=24/33;72.7%),umbilical and inguinal hernia(n=19/36;52.8%),heart involvement(n=26/32;81.3%)with valve involvement(n=25/26;96.2%)and linear growth delay(n=30/39,76.9%).Two patients(3.8%)died.The most common variants identified in the ARSB gene were c.454C>T and c.194C>T.At the time of data collection,ERT had ever received 48/53(90.5%)patients.CONCLUSION No correlation was observed between the age of onset of the first symptoms,the severity of clinical manifestations,enzyme activity,or nucleotide variants in the ARSB gene.展开更多
Chinese companies are transitioning from“manufacturing export”to brand export and from“value-for-money competition”to“value competition”,continuously expanding their presence in Russian-speaking markets.
There is a gradual increase in the proportion of renewable energy sources.Green hydrogen has the potential to become one of the major energy carriers in the future.The Russian Federation,in partnership with countries ...There is a gradual increase in the proportion of renewable energy sources.Green hydrogen has the potential to become one of the major energy carriers in the future.The Russian Federation,in partnership with countries in the Asia-Pacific region and especially China,has the potential to play a significant role in green hydrogen market.This study assessed the potential of developing green hydrogen energy based on wind power in the Far Eastern Federal District(FEFD)of the Russian Federation.Empirical wind speed data were collected from 20 meteorological stations in 4 regions(Sakhalinskaya Oblast’,Primorskiy Krai,Khabarovskiy Krai,and Amurskaya Oblast’)of the FEFD.The Weibull distribution was used to predict the potential of green hydrogen production.Five different methods(Empirical Method of Justus(EMJ),Empirical Method of Lysen(EML),Maximum Likelihood Method(MLE),Power Density Method(PDM),and Median and Quartiles Method(MQM))were used to determine the parameters(scape factor and scale factor)of the Weibull distribution.We calculated the total electricity generation potential based on the technical specifications of the three wind turbines:Senvion 6150 onshore,H165-4.0 MW,and Vestas V150-4.2 MW.The results showed that Vladivostok,Pogibi,Ilyinskiy,Yuzhno-Kuril’sk,Severo-Kuril’sk,Kholmsk,and Okha stations had the higher potential of green hydrogen production,of which Vladivostok exhibited the highest potential of green hydrogen production using the wind turbine of H165-4.0 MW,up to 2.56×10^(5) kg/a.In terms of economic analysis,the levelized cost of hydrogen(LCOH)values of lower than 4.00 USD/kg were obtained at Yuzhno-Kuril’sk,Ilyinskiy,Pogibi,and Vladivostok stations using the wind turbine of H165-4.0 MW,with the values of 3.54,3.50,3.24,and 2.55 USD/kg,respectively.This study concluded that the FEFD possesses significant potential in the production of green hydrogen and,with appropriate investment,has the potential to become a significant hub for green hydrogen trading in the Asia-Pacific region.展开更多
BACKGROUND Gallbladder and biliary tract cancer(GBTC)is a highly aggressive malignant tumor with a high fatality rate.The global incidence and mortality of GBTC continue to increase,presenting a significant challenge ...BACKGROUND Gallbladder and biliary tract cancer(GBTC)is a highly aggressive malignant tumor with a high fatality rate.The global incidence and mortality of GBTC continue to increase,presenting a significant challenge to public health.Strategies for preventing and controlling GBTC in Brazil,Russian Federation,India,China and South Africa(BRICS)countries offer valuable lessons for other developing nations.AIM To investigate GBTC burden trends in BRICS countries and perform an ageperiod-cohort(APC)analysis of Global Burden of Disease(GBD)from 1990-2021.METHODS Data on the incidences and crude incidence rates,the number of deaths and crude mortality rates,and the age-standardized incidence rate(ASIR)and agestandardized mortality rate(ASMR)of GBTC were obtained for BRICS countries from the GBD study 2021.Joinpoint regression analysis was employed to examine the trends in disease burden from 1990 to 2021.The APC model was utilized to assess the age,period,and birth cohort effects on the changes in GBTC disease burden worldwide and in the BRICS countries during the same time frame.Bayesian APC analysis was used to estimate the future burden.RESULTS The increases in incidence and deaths were 101.09%and 74.26%,respectively,compared with 1990.The ASMRs in Brazil,Russia,and China decreased,while those in India and South Africa increased.Among the BRICS countries,in most age groups in Brazil,Russia,India,and South Africa,the crude incidence and mortality rates in women were higher than those in men,whereas in China,the situation was the opposite.Joinpoint regression analysis revealed that from 1990 to 2021,the overall ASIR of gallbladder and bile duct cancer exhibited a declining trend.Although the incidence rate in China showed an increasing trend,the mortality rate exhibited a declining trend,which became more pronounced over time.CONCLUSION In BRICS countries,the number of incident cases and deaths from GBTC increased between 1990 and 2021,primarily due to rapid population growth.Nevertheless,the ASIR and ASMR declined during the same period.展开更多
The Chinese-Russian Workshop on Biophotonics and Biomedical Optics 2023 was held online twice on 18–21 September and 25–26 September 2023.The bilateral workshop brought together both Russian and Chinese scientists,e...The Chinese-Russian Workshop on Biophotonics and Biomedical Optics 2023 was held online twice on 18–21 September and 25–26 September 2023.The bilateral workshop brought together both Russian and Chinese scientists,engineers,and clinical researchers from a variety of disciplines engaged in applying optical science,photonics,and imaging technologies to problems in biology and medicine.During the workshops,two plenary lectures and twenty invited presentations were presented.This special issue selects some papers from both Russian and Chinese sides,consisting of one review and seven original research articles.展开更多
The development of agro-industrial complex is important for ensuring national food security and national health.The development of rural areas is subject to the development of agriculture and local infrastructure,as w...The development of agro-industrial complex is important for ensuring national food security and national health.The development of rural areas is subject to the development of agriculture and local infrastructure,as well as the availability of various services.This study selected 15 indicators in 2021 to analyze the employment and development levels in rural areas of 71 regions of the Russian Federation using the analytical grouping method.The results indicated that 20 regions(Group 1)had the highest percentage of rural population(33.10%).The percentage of population engaged in agriculture had the highest value(12.40%)in 31 regions(Group 2).Moreover,20 regions(Group 3)had the highest investments in fixed assets at the expense of municipal budget(11.80 USD/person).Increasing the investments in fixed assets carried out from the budget of the municipality can improve the employment level in rural areas.Then,we used cluster analysis to divide 14 regions of the Volga Federal District in the Russian Federation into 3 clusters.Cluster 1 covered Kirov Region and Republic of Mari El;Cluster 2 included Ulyanovsk Region,Saratov Region,Nizhny Novgorod Region,Perm Territory,Orenburg Region,Chuvash Region,and Republic of Mordovia;and Cluster 3 contained Republic of Tatarstan,Samara Region,Udmurtian Republic,Penza Region,and Republic of Bashkortostan.Results indicated that the 2 regions of Cluster 1 need to increase the availability of resources and natural gas and improve the investment attractiveness of rural areas.The 7 regions of Cluster 2 needed to develop infrastructure,public services,and agricultural production.We found the highest employment level in rural areas,the largest investments in fixed assets at the expense of municipal budget,the largest residential building area per 10,000 persons,and the largest individual residential building area in the 5 regions of Cluster 3.This study makes it possible to draw up a comprehensive regional development program and proves the need for the development of rural areas,which is especially important for the sustainable development of the Russian Federation.展开更多
This year marks the 75th anniversary of China-Russia diplomatic relations.Russian President Vladimir Putin paid a state visit to China in May.The heads of state of both China and Russia held a sincere,friendly and inf...This year marks the 75th anniversary of China-Russia diplomatic relations.Russian President Vladimir Putin paid a state visit to China in May.The heads of state of both China and Russia held a sincere,friendly and informative meeting,signed and issued a joint statement,and witnessed the signing of more than 10 important cooperation documents.These bilateral relations have shown a sound momentum of mutual benefit,in-depth coordination and long-term stability.展开更多
This article aims to study the history of scientific investigations in China in the nineteenth century,made by Russian scientists who ranged in status and position including official agents,the directors of the Russia...This article aims to study the history of scientific investigations in China in the nineteenth century,made by Russian scientists who ranged in status and position including official agents,the directors of the Russian Magneto-Meteorological Observatory in Beijing,the members of the Russian Orthodox Mission(ROM),and others.The article gives a detailed analysis of the unique expeditions of H.Fritsche(1839–1913),director of the Russian Magneto-Meteorological Observatory in Beijing,and Archimandrite Palladius(1817–1878),head of the ROM to the northeast part of China.During those expeditions,existing maps were corrected and new maps were made.The article also covers the history of natural science investigations,made by other members of missions,doctors,and attached persons.This article employs sources from Russian archives(Saint Petersburg Branch of the Archive of the Russian Academy of Sciences,Russian State Historical Archive,Archive of the Foreign Policy of the Russian Empire),which were rarely used by the scientific community,and adopts an empirical approach based on objectivity and historicism to the sources and research.展开更多
Russia’s reindeer population accounts for two thirds of the world’s total.There is a strong and resilient population of reindeer on the tundra,and reindeer herders inherit and transmit the unique culture of the nort...Russia’s reindeer population accounts for two thirds of the world’s total.There is a strong and resilient population of reindeer on the tundra,and reindeer herders inherit and transmit the unique culture of the north.Reindeer products have become the subject of innovative developments in the biopharmaceutical and healthcare products industry owing to their unique raw material properties.Because deer antlers and blood are widely used in traditional Chinese medicine,significant quantities of Arctic reindeer products are likely to be sold in China.Strengthening understanding of the Russian Arctic reindeer industry will help Chinese companies invest in the Russian Arctic,promote Sino-Russian Arctic cooperation on green biomanufacturing,and lead to the development of new products that promote human health.展开更多
In our class,we have already learned some articles of Aesthetism.The famous aesthetes,such as Walter Pater,Oscar Wilde,Benedetto Croce,all give us huge impressions.And Russian Formalism is also very important school o...In our class,we have already learned some articles of Aesthetism.The famous aesthetes,such as Walter Pater,Oscar Wilde,Benedetto Croce,all give us huge impressions.And Russian Formalism is also very important school of thoughts.As they all study art,literature and form,I would like to know the relationship between them.In this paper,first I will show some of my own understandings on Both Aesthetism and Russian Formalism.Then I will compare them to find similarities and differences.At last,I will say something about my preference.展开更多
Animal Farm,full of symbolism,is regarded as a classic portrayal of anti-utopianism in the real world and a satire on the Russian Revolution.George Orwell expresses his views of totalitarianism by using animals to rep...Animal Farm,full of symbolism,is regarded as a classic portrayal of anti-utopianism in the real world and a satire on the Russian Revolution.George Orwell expresses his views of totalitarianism by using animals to represent the major characters from the period of the Russian Revolution,from Karl Marx and Tsar Nicholas II to Stalin and Trotsky.His characters and scenar io paint a vision of a world of communism in Soviet Union,and Animal Farm chronicles the events and people of the Russian Revolution.Based on the historical background of the novel,this thesis is to reveal the real-life counterparts that the fictional characters in Animal Farm represent,thus uncovering the true meaning of the story.展开更多
Translators of the Sino-Russian crude oil pipeline: They are the translators who enabled communication between the Chinese staff and the Russian staff during the construction and operation of the SinoRussian crude oil...Translators of the Sino-Russian crude oil pipeline: They are the translators who enabled communication between the Chinese staff and the Russian staff during the construction and operation of the SinoRussian crude oil pipeline, including during such crucial tasks like the launching and the reception of the pig traps and the process of transporting the oil. As ‘messengers’ connecting the Chinese and the Russians, and as a necessity in facilitating the transportation of oil from Russia to China, they make great contributions towards ensuring the safe and efficient operation of the Sino-Russian crude oil pipeline.展开更多
This paper presents the past,present and future status of the Russian VLBI network "Quasar".This network has been developed for at least 20 years by the Institute of Applied Astronomy of the Russian Academy ...This paper presents the past,present and future status of the Russian VLBI network "Quasar".This network has been developed for at least 20 years by the Institute of Applied Astronomy of the Russian Academy of Sciences(IAA RAS). The equipment installed currently at each "Quasar" station and the facilities for processing observational data are described briefly. The latest results are presented, including the Earth orientation parameters(EOP) determined from the "Quasar" VLBI data and comparison with those from other processing centers. Our future plans to extend the "Quasar" VLB I network to the east and to the west will be carried out by establishing the Ussurijsk station in the Far East and the RussianCuban station in the west. They are considered in the context of the Russian section activity in the GGOS project. The expected improvement of the EOP determination accuracy can be obtained within the future configuration of the "Quasar" network,which is proved by simulation.展开更多
Phosphorite has become increasingly important as the raw material for phosphatic fertilizer. Phosphorite nodules are widespread in the Kursk complex deposit (Russian platform). Genesis of these nodules has long been a...Phosphorite has become increasingly important as the raw material for phosphatic fertilizer. Phosphorite nodules are widespread in the Kursk complex deposit (Russian platform). Genesis of these nodules has long been a matter of debate, and this has hampered understanding of the mechanism and controls in the formation of the nodules. In this paper, we report the petrographical, mineralogical, and geochemical data of the Lebedinsky phosphorite nodules. Petrographic study reveals complex phosphatic cement resulting in the replacement of apatite minerals around quartz grains. The main mineral composition consists of quartz, phosphate minerals (apatite, francolite mainly), feldspar, iron hydroxides and carbonate minerals. These results, when combined with available data, are used to address the origin of the phosphorite nodules. The nodules are characterized by the universal presence of biogenic and chemical signatures which is phosphorus crown around crystal grains. The structure of the nodules is massive. Their texture is depicted by basaltic cement and concretion, which consists mainly of apatite and its varieties, with general formula: Ca_10 (PO_4, CO_3) 6(F,OH,Cl). Variation of mineralogy appears dependent on geological setting. Microscopic observations of biogenic fossils in Lebedinsky phosphorite favor a chemical and biogenic origin of phosphorites. Weathering has been suggested to be capable of liberating as much as 20 %-35 % P_2O_5 from sedimentary rocks. Previous investigations demonstrate that weathering of the Proterozoic substratum was the main cause in the formation of Fe, Mo, Mn, Pb, Zn, and P in the Russian platform. We therefore suggest that both weathering and biochemical processes have been positive in the formation of the Lebedinsky phosporite nodules. However, whether continental weathering or oceanic bio-chemical processes are more relevant in the phosphorite accumulation remains undetermined.展开更多
We studied the allelic frequency distributions and statistical forensic parameters of 21 new short tandem repeat(STR)loci and the amelogenin locus,which are not included in the combined DNA index system(CODIS),in a Ru...We studied the allelic frequency distributions and statistical forensic parameters of 21 new short tandem repeat(STR)loci and the amelogenin locus,which are not included in the combined DNA index system(CODIS),in a Russian ethnic minority group from the Inner Mongolia Autonomous Region,China.A total of 114 bloodstain samples from unrelated individuals were extracted and co-amplified with four fluorescence-labeled primers in a multiplex polymerase chain reaction(PCR)system.Using capillary electrophoresis,the PCR products of the 21 STR loci were separated and genotyped.A total of 161 alleles were observed in the Russian ethnic minority group,and corresponding allelic frequencies ranged from 0.0044 to 0.5965.The 21 non-CODIS STR loci of the Russian ethnic minority group were characterized by high genetic diversity and therefore may be useful for elucidating the population's genetic background,for individual identification,and for paternity testing in forensic practice.展开更多
To address the issues of investment appeal in the Russian electric power industry,this study analyzes the dynamics of corporate governance,including permanent redistribution of property and compliance with the Russian...To address the issues of investment appeal in the Russian electric power industry,this study analyzes the dynamics of corporate governance,including permanent redistribution of property and compliance with the Russian Corporate Governance Code,in wholesale and territorial-generating companies.The increasing concentration of property in the hands of the state and its implications for investors are also noted.This study reveals the violations of essential principles,and the substantial differences in corporate governance practices,in the best-and worst-performing companies.Additional standards for better corporate governance practices to benefit the investors in the context of the current Environmental,Social,and corporate Governance(ESG)agenda are proposed.This study provides a new insight at the development of corporate governance in Russian power generating companies through property redistribution and compliance with corporate governance principles.展开更多
To improve the serious situation of soil degradation in China, tests were carried out to study the improvement effects of Russian Melilotus officinalis on the low base saturation acid soil, the alkaline-saline soil an...To improve the serious situation of soil degradation in China, tests were carried out to study the improvement effects of Russian Melilotus officinalis on the low base saturation acid soil, the alkaline-saline soil and the heavy metal contaminated soil. The results showed that M. officinalis could improve the low base saturation acid soil, increase the salt, alkali content, mineral nutrition and organic nutrient, which indicated that M. officinalis could be planted in the acid soil, and had a significant ecological effect on soil improvement. M. officinalis achieved a remarkable effect on improving the alkaline-saline soil （pH 8.5-9.5） that the soil desalted, dealkalized and had more organic nutrient. Moreover, M. officinalis could improve the heavy metal contaminated soil and had the ability to enrich and transform heavy metal elements, especially Cd and Ni, thereby presenting significant repair effects on soil improvement. Therefore, Russian M. officinalis had multiple-effects on degraded soil improvement, so it is worthy of application and popularization.展开更多
文摘BACKGROUND Mucopolysaccharidosis type Ⅱ(MPS Ⅱ)is a chronic inherited disease with multiorgan involvement,a progressive course,and restricted life expectancy.AIM To evaluate the predictors of fatal outcomes in MPS Ⅱ patients.METHODS In the retrospective cohort study,the clinical,laboratory data and enzyme replacement therapy(ERT)(84.2%)of about 160 patients were extracted and analyzed from the Russian MPS Ⅱ registry,with death as a primary outcome.We compared patients who died(n=20;12.5%)with severe form(n=13;68.4%)and attenuated form(n=6,31.6%)to 140 alive patients.RESULTS Fatal outcomes occurred in 5%,35%,20%,and 40%of patients before 10,10-14,15-19,and≥20 years.The most common causes of death were cardiovascular(29.4%),respiratory failure(17.6%),including pneumonia(17.6%),and their associations(17.6%)and MPS Ⅱ progression(11.8%).Acute or chronic respiratory failure was in 53%.Died patients had higher birth weight,higher age of diagnosis,and start of ERT.Hydrocephalus,hydrocephalus bypass surgery,epilepsy,difficulty swallowing,and impaired movement after 12 years of age were significantly more common in the deceased patients.Cox regression analysis has revealed the following time-dependent covariates of the lethal outcome:1^(st)-year psychomotor development delay,delayed mental and speech development,hydrocephalus,swallow disorders,impossible walking at age>12 years,respiratory disorders,tracheostomy,neuronopathic form.CONCLUSION Increased birth weight,delayed diagnosis and the start of ERT,and development of neuronopathic form with impossible walking after 12 years were the main predictors of the fatal outcome.
文摘The Russian Banquet-Treasures of Dining from the Moscow Kremlin Museums Collection presents the 500-year history of Russian food culture from the 16th century to the 20th century,allowing the visitors to see the changes and origins of Russian food from the Middle Ages of the Tsarist era to the modern era.
文摘The study examines the socio-cultural context of Russian music in the decades immediately preceding and following the October Revolution of 1917,focusing on several aspects:personalities representing pre-and post-revolutionary Russian(in exile)and Soviet(at home)musical activity and culture;second-tier national composers;“industrial music”of the early Soviet period;and Soviet cultural management in music behind the Iron Curtain.Portraits of composers little known to Western listeners and academic audiences are presented.The spectrum of musical styles and aesthetics is considered.Issues of ethnic diversity and religious affiliation of Russian composers are relevantly explored.Songs of the First Revolution(1905),the Civil War,and Soviet mass songs are briefly touched upon.The scope and choice of themes of the study provide a holistic approach to topics that are usually addressed separately:before or after the 1917 Revolution;classical music or folk/popular songs.
文摘BACKGROUND Mucopolysaccharidosis typeⅥ(MPSⅥ)is a chronic,progressive,inherited disease with multiorgan involvement and a restricted life expectancy.AIM To investigate the epidemiological,clinical,and genetic characteristics of patients with mucopolysaccharidosis type 6 and their outcomes using the Russian Federation's national registry,as per the Russian registry,and compare them with previously published data.METHODS In a retrospective cohort study,clinical,laboratory data,molecular genetic analysis results,and enzyme replacement therapy(ERT)data were extracted and analyzed from the Russian MPSⅥregistry for 53 patients,comprising 26 males(49.1%)and 27 females(50.9%).RESULTS The median age of first symptoms was 2 years,ranging from the first months of life to 20 years.A positive family history of MPSⅥwas reported in 19/53(35.8%)patients,a negative family history in 24(45.3%),and missing information in 10(18.9%).The main features of the disease were hepatomegaly(n=23;60.5%),splenomegaly(n=15,39.5%),involvement of otolaryngological organs(n=24/33;72.7%),umbilical and inguinal hernia(n=19/36;52.8%),heart involvement(n=26/32;81.3%)with valve involvement(n=25/26;96.2%)and linear growth delay(n=30/39,76.9%).Two patients(3.8%)died.The most common variants identified in the ARSB gene were c.454C>T and c.194C>T.At the time of data collection,ERT had ever received 48/53(90.5%)patients.CONCLUSION No correlation was observed between the age of onset of the first symptoms,the severity of clinical manifestations,enzyme activity,or nucleotide variants in the ARSB gene.
文摘Chinese companies are transitioning from“manufacturing export”to brand export and from“value-for-money competition”to“value competition”,continuously expanding their presence in Russian-speaking markets.
文摘There is a gradual increase in the proportion of renewable energy sources.Green hydrogen has the potential to become one of the major energy carriers in the future.The Russian Federation,in partnership with countries in the Asia-Pacific region and especially China,has the potential to play a significant role in green hydrogen market.This study assessed the potential of developing green hydrogen energy based on wind power in the Far Eastern Federal District(FEFD)of the Russian Federation.Empirical wind speed data were collected from 20 meteorological stations in 4 regions(Sakhalinskaya Oblast’,Primorskiy Krai,Khabarovskiy Krai,and Amurskaya Oblast’)of the FEFD.The Weibull distribution was used to predict the potential of green hydrogen production.Five different methods(Empirical Method of Justus(EMJ),Empirical Method of Lysen(EML),Maximum Likelihood Method(MLE),Power Density Method(PDM),and Median and Quartiles Method(MQM))were used to determine the parameters(scape factor and scale factor)of the Weibull distribution.We calculated the total electricity generation potential based on the technical specifications of the three wind turbines:Senvion 6150 onshore,H165-4.0 MW,and Vestas V150-4.2 MW.The results showed that Vladivostok,Pogibi,Ilyinskiy,Yuzhno-Kuril’sk,Severo-Kuril’sk,Kholmsk,and Okha stations had the higher potential of green hydrogen production,of which Vladivostok exhibited the highest potential of green hydrogen production using the wind turbine of H165-4.0 MW,up to 2.56×10^(5) kg/a.In terms of economic analysis,the levelized cost of hydrogen(LCOH)values of lower than 4.00 USD/kg were obtained at Yuzhno-Kuril’sk,Ilyinskiy,Pogibi,and Vladivostok stations using the wind turbine of H165-4.0 MW,with the values of 3.54,3.50,3.24,and 2.55 USD/kg,respectively.This study concluded that the FEFD possesses significant potential in the production of green hydrogen and,with appropriate investment,has the potential to become a significant hub for green hydrogen trading in the Asia-Pacific region.
基金Supported by the Key Project of Science&Technology Development Fund of Tianjin Education Commission for Higher Education,No.2024ZD023.
文摘BACKGROUND Gallbladder and biliary tract cancer(GBTC)is a highly aggressive malignant tumor with a high fatality rate.The global incidence and mortality of GBTC continue to increase,presenting a significant challenge to public health.Strategies for preventing and controlling GBTC in Brazil,Russian Federation,India,China and South Africa(BRICS)countries offer valuable lessons for other developing nations.AIM To investigate GBTC burden trends in BRICS countries and perform an ageperiod-cohort(APC)analysis of Global Burden of Disease(GBD)from 1990-2021.METHODS Data on the incidences and crude incidence rates,the number of deaths and crude mortality rates,and the age-standardized incidence rate(ASIR)and agestandardized mortality rate(ASMR)of GBTC were obtained for BRICS countries from the GBD study 2021.Joinpoint regression analysis was employed to examine the trends in disease burden from 1990 to 2021.The APC model was utilized to assess the age,period,and birth cohort effects on the changes in GBTC disease burden worldwide and in the BRICS countries during the same time frame.Bayesian APC analysis was used to estimate the future burden.RESULTS The increases in incidence and deaths were 101.09%and 74.26%,respectively,compared with 1990.The ASMRs in Brazil,Russia,and China decreased,while those in India and South Africa increased.Among the BRICS countries,in most age groups in Brazil,Russia,India,and South Africa,the crude incidence and mortality rates in women were higher than those in men,whereas in China,the situation was the opposite.Joinpoint regression analysis revealed that from 1990 to 2021,the overall ASIR of gallbladder and bile duct cancer exhibited a declining trend.Although the incidence rate in China showed an increasing trend,the mortality rate exhibited a declining trend,which became more pronounced over time.CONCLUSION In BRICS countries,the number of incident cases and deaths from GBTC increased between 1990 and 2021,primarily due to rapid population growth.Nevertheless,the ASIR and ASMR declined during the same period.
文摘The Chinese-Russian Workshop on Biophotonics and Biomedical Optics 2023 was held online twice on 18–21 September and 25–26 September 2023.The bilateral workshop brought together both Russian and Chinese scientists,engineers,and clinical researchers from a variety of disciplines engaged in applying optical science,photonics,and imaging technologies to problems in biology and medicine.During the workshops,two plenary lectures and twenty invited presentations were presented.This special issue selects some papers from both Russian and Chinese sides,consisting of one review and seven original research articles.
文摘The development of agro-industrial complex is important for ensuring national food security and national health.The development of rural areas is subject to the development of agriculture and local infrastructure,as well as the availability of various services.This study selected 15 indicators in 2021 to analyze the employment and development levels in rural areas of 71 regions of the Russian Federation using the analytical grouping method.The results indicated that 20 regions(Group 1)had the highest percentage of rural population(33.10%).The percentage of population engaged in agriculture had the highest value(12.40%)in 31 regions(Group 2).Moreover,20 regions(Group 3)had the highest investments in fixed assets at the expense of municipal budget(11.80 USD/person).Increasing the investments in fixed assets carried out from the budget of the municipality can improve the employment level in rural areas.Then,we used cluster analysis to divide 14 regions of the Volga Federal District in the Russian Federation into 3 clusters.Cluster 1 covered Kirov Region and Republic of Mari El;Cluster 2 included Ulyanovsk Region,Saratov Region,Nizhny Novgorod Region,Perm Territory,Orenburg Region,Chuvash Region,and Republic of Mordovia;and Cluster 3 contained Republic of Tatarstan,Samara Region,Udmurtian Republic,Penza Region,and Republic of Bashkortostan.Results indicated that the 2 regions of Cluster 1 need to increase the availability of resources and natural gas and improve the investment attractiveness of rural areas.The 7 regions of Cluster 2 needed to develop infrastructure,public services,and agricultural production.We found the highest employment level in rural areas,the largest investments in fixed assets at the expense of municipal budget,the largest residential building area per 10,000 persons,and the largest individual residential building area in the 5 regions of Cluster 3.This study makes it possible to draw up a comprehensive regional development program and proves the need for the development of rural areas,which is especially important for the sustainable development of the Russian Federation.
文摘This year marks the 75th anniversary of China-Russia diplomatic relations.Russian President Vladimir Putin paid a state visit to China in May.The heads of state of both China and Russia held a sincere,friendly and informative meeting,signed and issued a joint statement,and witnessed the signing of more than 10 important cooperation documents.These bilateral relations have shown a sound momentum of mutual benefit,in-depth coordination and long-term stability.
文摘This article aims to study the history of scientific investigations in China in the nineteenth century,made by Russian scientists who ranged in status and position including official agents,the directors of the Russian Magneto-Meteorological Observatory in Beijing,the members of the Russian Orthodox Mission(ROM),and others.The article gives a detailed analysis of the unique expeditions of H.Fritsche(1839–1913),director of the Russian Magneto-Meteorological Observatory in Beijing,and Archimandrite Palladius(1817–1878),head of the ROM to the northeast part of China.During those expeditions,existing maps were corrected and new maps were made.The article also covers the history of natural science investigations,made by other members of missions,doctors,and attached persons.This article employs sources from Russian archives(Saint Petersburg Branch of the Archive of the Russian Academy of Sciences,Russian State Historical Archive,Archive of the Foreign Policy of the Russian Empire),which were rarely used by the scientific community,and adopts an empirical approach based on objectivity and historicism to the sources and research.
基金a phased result of both the research on“Strategic Competition and Cooperation in the Arctic among China,Russia,and the United States from the Perspective of Sustainable Development”(Grant no.20BGJ045)the research project on the fusion of Arctic biological resource products and traditional Chinese medicine culture(Grant no.21JD004A)。
文摘Russia’s reindeer population accounts for two thirds of the world’s total.There is a strong and resilient population of reindeer on the tundra,and reindeer herders inherit and transmit the unique culture of the north.Reindeer products have become the subject of innovative developments in the biopharmaceutical and healthcare products industry owing to their unique raw material properties.Because deer antlers and blood are widely used in traditional Chinese medicine,significant quantities of Arctic reindeer products are likely to be sold in China.Strengthening understanding of the Russian Arctic reindeer industry will help Chinese companies invest in the Russian Arctic,promote Sino-Russian Arctic cooperation on green biomanufacturing,and lead to the development of new products that promote human health.
文摘In our class,we have already learned some articles of Aesthetism.The famous aesthetes,such as Walter Pater,Oscar Wilde,Benedetto Croce,all give us huge impressions.And Russian Formalism is also very important school of thoughts.As they all study art,literature and form,I would like to know the relationship between them.In this paper,first I will show some of my own understandings on Both Aesthetism and Russian Formalism.Then I will compare them to find similarities and differences.At last,I will say something about my preference.
文摘Animal Farm,full of symbolism,is regarded as a classic portrayal of anti-utopianism in the real world and a satire on the Russian Revolution.George Orwell expresses his views of totalitarianism by using animals to represent the major characters from the period of the Russian Revolution,from Karl Marx and Tsar Nicholas II to Stalin and Trotsky.His characters and scenar io paint a vision of a world of communism in Soviet Union,and Animal Farm chronicles the events and people of the Russian Revolution.Based on the historical background of the novel,this thesis is to reveal the real-life counterparts that the fictional characters in Animal Farm represent,thus uncovering the true meaning of the story.
文摘Translators of the Sino-Russian crude oil pipeline: They are the translators who enabled communication between the Chinese staff and the Russian staff during the construction and operation of the SinoRussian crude oil pipeline, including during such crucial tasks like the launching and the reception of the pig traps and the process of transporting the oil. As ‘messengers’ connecting the Chinese and the Russians, and as a necessity in facilitating the transportation of oil from Russia to China, they make great contributions towards ensuring the safe and efficient operation of the Sino-Russian crude oil pipeline.
文摘This paper presents the past,present and future status of the Russian VLBI network "Quasar".This network has been developed for at least 20 years by the Institute of Applied Astronomy of the Russian Academy of Sciences(IAA RAS). The equipment installed currently at each "Quasar" station and the facilities for processing observational data are described briefly. The latest results are presented, including the Earth orientation parameters(EOP) determined from the "Quasar" VLBI data and comparison with those from other processing centers. Our future plans to extend the "Quasar" VLB I network to the east and to the west will be carried out by establishing the Ussurijsk station in the Far East and the RussianCuban station in the west. They are considered in the context of the Russian section activity in the GGOS project. The expected improvement of the EOP determination accuracy can be obtained within the future configuration of the "Quasar" network,which is proved by simulation.
文摘Phosphorite has become increasingly important as the raw material for phosphatic fertilizer. Phosphorite nodules are widespread in the Kursk complex deposit (Russian platform). Genesis of these nodules has long been a matter of debate, and this has hampered understanding of the mechanism and controls in the formation of the nodules. In this paper, we report the petrographical, mineralogical, and geochemical data of the Lebedinsky phosphorite nodules. Petrographic study reveals complex phosphatic cement resulting in the replacement of apatite minerals around quartz grains. The main mineral composition consists of quartz, phosphate minerals (apatite, francolite mainly), feldspar, iron hydroxides and carbonate minerals. These results, when combined with available data, are used to address the origin of the phosphorite nodules. The nodules are characterized by the universal presence of biogenic and chemical signatures which is phosphorus crown around crystal grains. The structure of the nodules is massive. Their texture is depicted by basaltic cement and concretion, which consists mainly of apatite and its varieties, with general formula: Ca_10 (PO_4, CO_3) 6(F,OH,Cl). Variation of mineralogy appears dependent on geological setting. Microscopic observations of biogenic fossils in Lebedinsky phosphorite favor a chemical and biogenic origin of phosphorites. Weathering has been suggested to be capable of liberating as much as 20 %-35 % P_2O_5 from sedimentary rocks. Previous investigations demonstrate that weathering of the Proterozoic substratum was the main cause in the formation of Fe, Mo, Mn, Pb, Zn, and P in the Russian platform. We therefore suggest that both weathering and biochemical processes have been positive in the formation of the Lebedinsky phosporite nodules. However, whether continental weathering or oceanic bio-chemical processes are more relevant in the phosphorite accumulation remains undetermined.
基金Project supported by the Program for New Century Excellent Talents of the Ministry of Education,China (No. NECT-10-0687)the Fun-damental Research Funds for the Central University (No. 2011jdgz20)the Shaanxi Provincial Science and Technology Research and Development Project Fund (No. 2012K16-08-01),China
文摘We studied the allelic frequency distributions and statistical forensic parameters of 21 new short tandem repeat(STR)loci and the amelogenin locus,which are not included in the combined DNA index system(CODIS),in a Russian ethnic minority group from the Inner Mongolia Autonomous Region,China.A total of 114 bloodstain samples from unrelated individuals were extracted and co-amplified with four fluorescence-labeled primers in a multiplex polymerase chain reaction(PCR)system.Using capillary electrophoresis,the PCR products of the 21 STR loci were separated and genotyped.A total of 161 alleles were observed in the Russian ethnic minority group,and corresponding allelic frequencies ranged from 0.0044 to 0.5965.The 21 non-CODIS STR loci of the Russian ethnic minority group were characterized by high genetic diversity and therefore may be useful for elucidating the population's genetic background,for individual identification,and for paternity testing in forensic practice.
基金the State Assignment Project (No. FWEU-2021-0001) of the Fundamental Research Program of the Russian Federation 2021–2030
文摘To address the issues of investment appeal in the Russian electric power industry,this study analyzes the dynamics of corporate governance,including permanent redistribution of property and compliance with the Russian Corporate Governance Code,in wholesale and territorial-generating companies.The increasing concentration of property in the hands of the state and its implications for investors are also noted.This study reveals the violations of essential principles,and the substantial differences in corporate governance practices,in the best-and worst-performing companies.Additional standards for better corporate governance practices to benefit the investors in the context of the current Environmental,Social,and corporate Governance(ESG)agenda are proposed.This study provides a new insight at the development of corporate governance in Russian power generating companies through property redistribution and compliance with corporate governance principles.
基金Supported by the Special Fund for Agro-Scientific Research in the Public Interest of China(200903031)the Project of Science and Technology Department of Jilin Province(20070413)
文摘To improve the serious situation of soil degradation in China, tests were carried out to study the improvement effects of Russian Melilotus officinalis on the low base saturation acid soil, the alkaline-saline soil and the heavy metal contaminated soil. The results showed that M. officinalis could improve the low base saturation acid soil, increase the salt, alkali content, mineral nutrition and organic nutrient, which indicated that M. officinalis could be planted in the acid soil, and had a significant ecological effect on soil improvement. M. officinalis achieved a remarkable effect on improving the alkaline-saline soil （pH 8.5-9.5） that the soil desalted, dealkalized and had more organic nutrient. Moreover, M. officinalis could improve the heavy metal contaminated soil and had the ability to enrich and transform heavy metal elements, especially Cd and Ni, thereby presenting significant repair effects on soil improvement. Therefore, Russian M. officinalis had multiple-effects on degraded soil improvement, so it is worthy of application and popularization.
