This paper conducted a more comprehensive review and comparative analysis of the two heavy to blizzard processes that occurred in the Beijing area during December 13-15,2023,and February 20-21,2024,in terms of compreh...This paper conducted a more comprehensive review and comparative analysis of the two heavy to blizzard processes that occurred in the Beijing area during December 13-15,2023,and February 20-21,2024,in terms of comprehensive weather situation diagnosis,forecasting,and decision-making services,and summarized the meteorological service support experience of such heavy snow weather processes.It was found that both blizzard processes were jointly influenced by the 700 hPa southwesterly warm and humid jet stream and the near-surface easterly backflow;the numerical forecast was relatively accurate in the overall description of the snowfall process,and the forecast bias of the position of the 700 hPa southwesterly warm and humid jet stream determined the bias of the snowfall magnitude forecast at a certain point;when a deviation was found between the actual snowfall and the forecast,the cause should be analyzed in a timely manner,and the warning and forecast conclusions should be updated.With the full cooperation of relevant departments,it can greatly make up for the deviation of the early forecast snowfall amount,and ensure the safety and efficiency of people's travel.展开更多
Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive condition associated with exocrine pancreatic insufficiency,and is characterized by hypoplastic nasal alae,mental retardation,sensorineural hearing loss,s...Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive condition associated with exocrine pancreatic insufficiency,and is characterized by hypoplastic nasal alae,mental retardation,sensorineural hearing loss,short stature,scalp defects,dental abnormalities and abnormal hair patterns. Growth hormone deficiency,hypopituitarism,and impaired glucagon secretion response to insulin-induced hypoglycemia have been reported. Congenital heart defects have also been described in this condition. Mental retardation is typically moderate to severe in patients with JBS; however,normal intelligence can occur. In the pancreas,there is a selective defect of acinar tissue,whereas the islets of Langerhans and ducts are preserved. Diabetes has been reported in older children,suggesting the progressive nature of pancreatic disease. The molecular basis of JBS has recently been mapped to chromosome 15q15-q21 with identified mutations in the UBR1 gene. We report the case of a 7-year-old female with pancreatic insufficiency and mild phenotypic features,in whom the diagnosis of JBS was established using recently described molecular testing for the UBR1 gene.展开更多
[Objective]The research aimed to analyze formation reason of ＂ 0902＂ blizzard in northeast China. [Method]By using timely observation data,NCEP reanalysis data and Doppler radar data at Baishan station,blizzard proc...[Objective]The research aimed to analyze formation reason of ＂ 0902＂ blizzard in northeast China. [Method]By using timely observation data,NCEP reanalysis data and Doppler radar data at Baishan station,blizzard process in southeast part of northeast China during 12-13 February,2009 was analyzed. [Result]Snowfall zone of the blizzard process was wide,snowfall was more,snowfall gradient was big,and snowfall time relatively concentrated. These characteristics reflected that the blizzard process had significant convection characteristics. Baroclinic disturbance at high-altitude straight frontal zone and ground warm frontogenesis caused by eastward movement and northward advancement of North China low vortex at low altitude were the circulation characteristics in the process. Water vapor from the sea went northward as southwest airflow,and strongly converged in blizzard zone,which provided sufficient water vapor condition for the blizzard. Before heavy snowfall occurred,there was accumulation process of heat and energy. Conditional symmetric instability was main unstable mechanism of the blizzard. During heavy snowfall period,ascending branch of secondary vertical circulation at exit zone of high-altitude jet coupled with ascending branch of secondary vertical circulation of warm frontegenesis at low layer,inducing strong development of the vertical motion. Doppler radar intensity echo revealed that it was easy to generate blizzard in the area where echo intensity was consistently above 20 dBz. Strong wind velocity convergence zone at radical velocity field especially adverse wind zone was favorable for the generation of blizzard. [Conclusion]The research could provide reference for blizzard forecast in northeast China.展开更多
Johanson-Blizzard syndrome (JBS) is a rare genetic disorder characterized by multiple craniofacial abnormalities, intellectual disability, sensorineural hearing loss, pancreatic exocrine insufficiency, and involvement...Johanson-Blizzard syndrome (JBS) is a rare genetic disorder characterized by multiple craniofacial abnormalities, intellectual disability, sensorineural hearing loss, pancreatic exocrine insufficiency, and involvement of other organ systems to varying degrees. Patients with JBS may require surgical intervention to address the underlying phenotypic abnormalities. The many craniofacial abnormalities found in patients with JBS are a concern for the anesthesiologist. We present the case of an adult patient with JBS who is undergoing implantation of a leadless pacemaker. Considering the many cardiac and craniofacial abnormalities in these patients, the anesthesiologist should order diagnostic tests such as echocardiography to assess cardiac function, as well as be prepared to perform advanced airway techniques for difficult airways. The anesthetic provider should be aware of the varied phenotypic expression of JBS and should individualize the anesthetic plan to each patient. Prior medical literature on the anesthetic management of these patients is scarce and limited to pediatric patients. This is the first case report addressing anesthetic concerns in an adult patient with JBS.展开更多
文摘This paper conducted a more comprehensive review and comparative analysis of the two heavy to blizzard processes that occurred in the Beijing area during December 13-15,2023,and February 20-21,2024,in terms of comprehensive weather situation diagnosis,forecasting,and decision-making services,and summarized the meteorological service support experience of such heavy snow weather processes.It was found that both blizzard processes were jointly influenced by the 700 hPa southwesterly warm and humid jet stream and the near-surface easterly backflow;the numerical forecast was relatively accurate in the overall description of the snowfall process,and the forecast bias of the position of the 700 hPa southwesterly warm and humid jet stream determined the bias of the snowfall magnitude forecast at a certain point;when a deviation was found between the actual snowfall and the forecast,the cause should be analyzed in a timely manner,and the warning and forecast conclusions should be updated.With the full cooperation of relevant departments,it can greatly make up for the deviation of the early forecast snowfall amount,and ensure the safety and efficiency of people's travel.
基金Supported by Grant from the German Research Foundation (DFG) to MZ
文摘Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive condition associated with exocrine pancreatic insufficiency,and is characterized by hypoplastic nasal alae,mental retardation,sensorineural hearing loss,short stature,scalp defects,dental abnormalities and abnormal hair patterns. Growth hormone deficiency,hypopituitarism,and impaired glucagon secretion response to insulin-induced hypoglycemia have been reported. Congenital heart defects have also been described in this condition. Mental retardation is typically moderate to severe in patients with JBS; however,normal intelligence can occur. In the pancreas,there is a selective defect of acinar tissue,whereas the islets of Langerhans and ducts are preserved. Diabetes has been reported in older children,suggesting the progressive nature of pancreatic disease. The molecular basis of JBS has recently been mapped to chromosome 15q15-q21 with identified mutations in the UBR1 gene. We report the case of a 7-year-old female with pancreatic insufficiency and mild phenotypic features,in whom the diagnosis of JBS was established using recently described molecular testing for the UBR1 gene.
文摘[Objective]The research aimed to analyze formation reason of ＂ 0902＂ blizzard in northeast China. [Method]By using timely observation data,NCEP reanalysis data and Doppler radar data at Baishan station,blizzard process in southeast part of northeast China during 12-13 February,2009 was analyzed. [Result]Snowfall zone of the blizzard process was wide,snowfall was more,snowfall gradient was big,and snowfall time relatively concentrated. These characteristics reflected that the blizzard process had significant convection characteristics. Baroclinic disturbance at high-altitude straight frontal zone and ground warm frontogenesis caused by eastward movement and northward advancement of North China low vortex at low altitude were the circulation characteristics in the process. Water vapor from the sea went northward as southwest airflow,and strongly converged in blizzard zone,which provided sufficient water vapor condition for the blizzard. Before heavy snowfall occurred,there was accumulation process of heat and energy. Conditional symmetric instability was main unstable mechanism of the blizzard. During heavy snowfall period,ascending branch of secondary vertical circulation at exit zone of high-altitude jet coupled with ascending branch of secondary vertical circulation of warm frontegenesis at low layer,inducing strong development of the vertical motion. Doppler radar intensity echo revealed that it was easy to generate blizzard in the area where echo intensity was consistently above 20 dBz. Strong wind velocity convergence zone at radical velocity field especially adverse wind zone was favorable for the generation of blizzard. [Conclusion]The research could provide reference for blizzard forecast in northeast China.
文摘Johanson-Blizzard syndrome (JBS) is a rare genetic disorder characterized by multiple craniofacial abnormalities, intellectual disability, sensorineural hearing loss, pancreatic exocrine insufficiency, and involvement of other organ systems to varying degrees. Patients with JBS may require surgical intervention to address the underlying phenotypic abnormalities. The many craniofacial abnormalities found in patients with JBS are a concern for the anesthesiologist. We present the case of an adult patient with JBS who is undergoing implantation of a leadless pacemaker. Considering the many cardiac and craniofacial abnormalities in these patients, the anesthesiologist should order diagnostic tests such as echocardiography to assess cardiac function, as well as be prepared to perform advanced airway techniques for difficult airways. The anesthetic provider should be aware of the varied phenotypic expression of JBS and should individualize the anesthetic plan to each patient. Prior medical literature on the anesthetic management of these patients is scarce and limited to pediatric patients. This is the first case report addressing anesthetic concerns in an adult patient with JBS.
