BACKGROUND Fabry disease is a kind of lysosomal storage disease resulting from deficient activity of the lysosomal hydrolase alpha-galactosidase A(GLA). A mutation in the GLA gene leads to a loss of activity of alpha-...BACKGROUND Fabry disease is a kind of lysosomal storage disease resulting from deficient activity of the lysosomal hydrolase alpha-galactosidase A(GLA). A mutation in the GLA gene leads to a loss of activity of alpha-galactosidase A. Some drugs,such as hydroxychloroquine, can cause pathological changes similar to those usually seen in Fabry disease.CASE SUMMARY We report the case of a 41-year-old female patient who was diagnosed with undifferentiated connective tissue disease in 2008. Hydroxychloroquine treatment started 2 years ago, and proteinuria and hematuria increased. Renal biopsy demonstrated renal phospholipidosis. Zebra bodies and myelin figures were found by renal electron microscopy and were initially thought to be indicators of Fabry disease. A genetic analysis of the patient and her family members did not reveal mutations in the GLA gene, supporting a diagnosis of hydroxychloroquine-induced renal phospholipidosis.CONCLUSION This report reveals one of the adverse effects of hydroxychloroquine. We should pay more attention to hydroxychloroquine-induced renal phospholipidosis.展开更多
Considering sharp increase of central integration scale of intermittent energy generation, power system couldn’t rely on the determined model based operation schedule to assure its reliable load supply. Then, this ar...Considering sharp increase of central integration scale of intermittent energy generation, power system couldn’t rely on the determined model based operation schedule to assure its reliable load supply. Then, this article presents a new load supply adequacy evaluation method, which based on continuous security constrained generation optimization, and provides the functional architecture design of online multi-level short term load supply adequacy evaluation system.展开更多
With the development and application of energy Internet technology,the collaborative interaction of“source network,load and storage”has becomethe development trend of power grid dispatching.The large-scale access of...With the development and application of energy Internet technology,the collaborative interaction of“source network,load and storage”has becomethe development trend of power grid dispatching.The large-scale access of renewableenergy on the load side,the unified management of adjustable loads,and theparticipation of multiple parties in energy operations have put forward requirementsfor the safety,credibility,openness,and transparency of the load dispatchingenvironment.Under the environment of carbon emission reduction,the paperproposed an architecture of the scheduling data blockchain,based on the in-depthstudy of blockchain.Moreover,smart contracts are used to realize the applicationscenario of load dispatching instruction evidence on the blockchain.The contentand storage mode of scheduling instruction evidence on blockchain are studied.And different storage modes are adopted according to the actual needs.Andthe smart contract system realizes the evidence generation of power dispatchinginstruction.This is the basis for the normal circulation of power dispatchinginstruction evidence.The research significance of this paper is highlighted as follows.The data and information generated in the power dispatching process arestored as evidence.On the one hand,it can provide a basis for settlement betweenpower production and dispatching companies and power users.On the other hand,it can prepare for distributed transactions in the power grid under the environmentof carbon emission reduction.展开更多
To address the imbalance problem between supply and demand for taxis and passengers,this paper proposes a distributed ensemble empirical mode decomposition with normalization of spatial attention mechanism based bi-di...To address the imbalance problem between supply and demand for taxis and passengers,this paper proposes a distributed ensemble empirical mode decomposition with normalization of spatial attention mechanism based bi-directional gated recurrent unit(EEMDN-SABiGRU)model on Spark for accurate passenger hotspot prediction.It focuses on reducing blind cruising costs,improving carrying efficiency,and maximizing incomes.Specifically,the EEMDN method is put forward to process the passenger hotspot data in the grid to solve the problems of non-smooth sequences and the degradation of prediction accuracy caused by excessive numerical differences,while dealing with the eigenmodal EMD.Next,a spatial attention mechanism is constructed to capture the characteristics of passenger hotspots in each grid,taking passenger boarding and alighting hotspots as weights and emphasizing the spatial regularity of passengers in the grid.Furthermore,the bi-directional GRU algorithm is merged to deal with the problem that GRU can obtain only the forward information but ignores the backward information,to improve the accuracy of feature extraction.Finally,the accurate prediction of passenger hotspots is achieved based on the EEMDN-SABiGRU model using real-world taxi GPS trajectory data in the Spark parallel computing framework.The experimental results demonstrate that based on the four datasets in the 00-grid,compared with LSTM,EMDLSTM,EEMD-LSTM,GRU,EMD-GRU,EEMD-GRU,EMDN-GRU,CNN,and BP,the mean absolute percentage error,mean absolute error,root mean square error,and maximum error values of EEMDN-SABiGRU decrease by at least 43.18%,44.91%,55.04%,and 39.33%,respectively.展开更多
Apple maggot fly, Rhagoletis pomonella (Walsh) is a major pest causing considerable economic losses of fruits in North America. During the development of international trade, apple maggot fly has become a threat to ...Apple maggot fly, Rhagoletis pomonella (Walsh) is a major pest causing considerable economic losses of fruits in North America. During the development of international trade, apple maggot fly has become a threat to Chinese agriculture. In this study, CLIMEX and ArcGIS were used to predict the potential geographical distribution of apple maggot fly in China. The parameters used in CLIMEX for apple maggot fly were derived from ecological data and the present geographical distribution of apple maggot fly in North America. Then the potential distribution map in China was presented based on the adjusted values of these parameters. The results showed that apple maggot fly has a wide potential distribution area in China; 47.5% of 748 meteorological stations presented high or medium suitability of pest establishment. These high suitable stations are mainly located in northeast, southwest and northwest of China, such as Liaoning, Shandong, Gansu and Shaanxi Provinces. These areas are also the central regions of apple, pear and peach production in China. Two hundred and twenty-five stations (30.1%) in westem and southern China, such as Tibet, Qinghai, Guangdong, Guangxi, Hainan and Taiwan, were unsuitable for establishment of apple maggot fly populations. In order to prevent the introduction of apple maggot fly in China, the present plant quarantine measures should be enhanced, especially in the areas with high suitability for the presence of apple maggot fly.展开更多
基金Supported by the Dongguan Social Science and Technology Development Project,No.2018507150461629
文摘BACKGROUND Fabry disease is a kind of lysosomal storage disease resulting from deficient activity of the lysosomal hydrolase alpha-galactosidase A(GLA). A mutation in the GLA gene leads to a loss of activity of alpha-galactosidase A. Some drugs,such as hydroxychloroquine, can cause pathological changes similar to those usually seen in Fabry disease.CASE SUMMARY We report the case of a 41-year-old female patient who was diagnosed with undifferentiated connective tissue disease in 2008. Hydroxychloroquine treatment started 2 years ago, and proteinuria and hematuria increased. Renal biopsy demonstrated renal phospholipidosis. Zebra bodies and myelin figures were found by renal electron microscopy and were initially thought to be indicators of Fabry disease. A genetic analysis of the patient and her family members did not reveal mutations in the GLA gene, supporting a diagnosis of hydroxychloroquine-induced renal phospholipidosis.CONCLUSION This report reveals one of the adverse effects of hydroxychloroquine. We should pay more attention to hydroxychloroquine-induced renal phospholipidosis.
文摘Considering sharp increase of central integration scale of intermittent energy generation, power system couldn’t rely on the determined model based operation schedule to assure its reliable load supply. Then, this article presents a new load supply adequacy evaluation method, which based on continuous security constrained generation optimization, and provides the functional architecture design of online multi-level short term load supply adequacy evaluation system.
基金supported by Science and Technology Program of State Grid Corporation of China under Grant(No.5100-202155319A-0-0-00).
文摘With the development and application of energy Internet technology,the collaborative interaction of“source network,load and storage”has becomethe development trend of power grid dispatching.The large-scale access of renewableenergy on the load side,the unified management of adjustable loads,and theparticipation of multiple parties in energy operations have put forward requirementsfor the safety,credibility,openness,and transparency of the load dispatchingenvironment.Under the environment of carbon emission reduction,the paperproposed an architecture of the scheduling data blockchain,based on the in-depthstudy of blockchain.Moreover,smart contracts are used to realize the applicationscenario of load dispatching instruction evidence on the blockchain.The contentand storage mode of scheduling instruction evidence on blockchain are studied.And different storage modes are adopted according to the actual needs.Andthe smart contract system realizes the evidence generation of power dispatchinginstruction.This is the basis for the normal circulation of power dispatchinginstruction evidence.The research significance of this paper is highlighted as follows.The data and information generated in the power dispatching process arestored as evidence.On the one hand,it can provide a basis for settlement betweenpower production and dispatching companies and power users.On the other hand,it can prepare for distributed transactions in the power grid under the environmentof carbon emission reduction.
基金Project supported by the National Natural Science Foundation of China(Nos.62162012,62173278,and 62072061)the Science and Technology Support Program of Guizhou Province,China(No.QKHZC2021YB531)+3 种基金the Natural Science Research Project of Department of Education of Guizhou Province,China(Nos.QJJ2022015 and QJJ2022047)the Science and Technology Foundation of Guizhou Province,China(Nos.QKHJCZK2022YB195,QKHJCZK2022YB197,and QKHJCZK2023YB143)the Scientific Research Platform Project of Guizhou Minzu University,China(No.GZMUSYS202104)the 7^(th) Batch High-Level Innovative Talent Project of Guizhou Province,China。
文摘To address the imbalance problem between supply and demand for taxis and passengers,this paper proposes a distributed ensemble empirical mode decomposition with normalization of spatial attention mechanism based bi-directional gated recurrent unit(EEMDN-SABiGRU)model on Spark for accurate passenger hotspot prediction.It focuses on reducing blind cruising costs,improving carrying efficiency,and maximizing incomes.Specifically,the EEMDN method is put forward to process the passenger hotspot data in the grid to solve the problems of non-smooth sequences and the degradation of prediction accuracy caused by excessive numerical differences,while dealing with the eigenmodal EMD.Next,a spatial attention mechanism is constructed to capture the characteristics of passenger hotspots in each grid,taking passenger boarding and alighting hotspots as weights and emphasizing the spatial regularity of passengers in the grid.Furthermore,the bi-directional GRU algorithm is merged to deal with the problem that GRU can obtain only the forward information but ignores the backward information,to improve the accuracy of feature extraction.Finally,the accurate prediction of passenger hotspots is achieved based on the EEMDN-SABiGRU model using real-world taxi GPS trajectory data in the Spark parallel computing framework.The experimental results demonstrate that based on the four datasets in the 00-grid,compared with LSTM,EMDLSTM,EEMD-LSTM,GRU,EMD-GRU,EEMD-GRU,EMDN-GRU,CNN,and BP,the mean absolute percentage error,mean absolute error,root mean square error,and maximum error values of EEMDN-SABiGRU decrease by at least 43.18%,44.91%,55.04%,and 39.33%,respectively.
文摘Apple maggot fly, Rhagoletis pomonella (Walsh) is a major pest causing considerable economic losses of fruits in North America. During the development of international trade, apple maggot fly has become a threat to Chinese agriculture. In this study, CLIMEX and ArcGIS were used to predict the potential geographical distribution of apple maggot fly in China. The parameters used in CLIMEX for apple maggot fly were derived from ecological data and the present geographical distribution of apple maggot fly in North America. Then the potential distribution map in China was presented based on the adjusted values of these parameters. The results showed that apple maggot fly has a wide potential distribution area in China; 47.5% of 748 meteorological stations presented high or medium suitability of pest establishment. These high suitable stations are mainly located in northeast, southwest and northwest of China, such as Liaoning, Shandong, Gansu and Shaanxi Provinces. These areas are also the central regions of apple, pear and peach production in China. Two hundred and twenty-five stations (30.1%) in westem and southern China, such as Tibet, Qinghai, Guangdong, Guangxi, Hainan and Taiwan, were unsuitable for establishment of apple maggot fly populations. In order to prevent the introduction of apple maggot fly in China, the present plant quarantine measures should be enhanced, especially in the areas with high suitability for the presence of apple maggot fly.