nPendred syndrome （PS） is characterized by autosomal recessive inheritance of goiter associated with a defect of iodide organification, hearing loss, enlargement of the vestibular aqueduct （EVA）, and mutations of ...nPendred syndrome （PS） is characterized by autosomal recessive inheritance of goiter associated with a defect of iodide organification, hearing loss, enlargement of the vestibular aqueduct （EVA）, and mutations of the SLC26A4 gene. However, not all EVA patients have PS or SLC26A4 mutations. Two mutant alleles of SLC26A4 are detected in 1/4 of North American or European EVA populations, one mutant allele is detected in another 1/4 of patient populations, and no mutations are de-tected in the other 1/2. The presence of two mutant al-leles of SLC26A4 is associated with abnormal iodide or-ganification, increased thyroid gland volume, increased severity of hearing loss, and bilateral EVA. The pres-ence of a single mutant allele of SLC26A4 is associated with normal iodide organification, normal thyroid gland volume, less severe hearing loss and either bilateral or unilateral EVA. When other underlying correlations are accounted for, the presence of a cochlear malformation or the size of EVA does not have an effect on hearing thresholds. This is consistent with observations of an Slc26a4 mutant mouse model of EVA in which hearing loss is independent of endolymphatic hydrops or in-ner ear malformations. Segregation analyses of EVA in families suggest that the patients carrying one mutant allele of SLC26A4 have a second, undetected mutant allele of SLC26A4, and the probability of a sibling hav-ing EVA is consistent with its segregation as an autoso-mal recessive trait. Patients without any mutations are an etiologically heterogeneous group in which siblings have a lower probability of having EVA. SLC26A4 muta-tion testing can provide prognostic information to guide clinical surveillance and management, as well as the probability of EVA affecting a sibling.展开更多
ased on Quasi-Vortex-Lattiee method, a program is presented to com-pute the aerodynamic forces for nonplanar wing with wing-tip sails. By using thisprogram, the aerodynamic force is calculated and the sails are design...ased on Quasi-Vortex-Lattiee method, a program is presented to com-pute the aerodynamic forces for nonplanar wing with wing-tip sails. By using thisprogram, the aerodynamic force is calculated and the sails are designed for an aircraftwith rectangular wing of 8.6 aspect ratio. The calculation results show that thosewing-tip sails, whose total area is 3. 1 percent of the aircraft’s basic wing area, will haveremarkable effect on reducing induced drag, and the lift-dependent drag factor can bereduced by about 18.5 to 21 .5 percent. Wind tunnel tests are conducted in NH-2 windtunnel of Nanjing Aeronautical and Astronautical University, and the results demon-strate the correctness of the above calculation results. The influences of sail parameterson performance and handling qualities of aircraft are also analyzed.展开更多
The present research aims to identify the possibilities of rehabilitation of building facades in the centre of Chisinau, through the use of high-performance materials, use of high-performance technologies in exec...The present research aims to identify the possibilities of rehabilitation of building facades in the centre of Chisinau, through the use of high-performance materials, use of high-performance technologies in execution, reduction of facade renovation costs, etc. In the process of organising the rehabilitation works of historical buildings of architectural value, we are dealing with the following: the value of the rehabilitation works of a historical building is very high, the duration of the rehabilitation is important, the attitude of the society is insufficient, the lack of strategies for the rehabilitation of buildings at the municipality level. In order to achieve this objective, we propose to research the following tasks: definition of the concept of rehabilitation of buildings in the Historic Centre of Chisinau;legislative, normative assurance of rehabilitation of historical heritage in the Republic of Moldova;analysis of the situation in Chisinau on the example of historical buildings with limestone finishes and examination by non-destructive methods of facades;solutions for rehabilitation of facades of historical buildings by comparing two types of technologies. In conclusion, emphasis will be placed on the choice of the most efficient method in terms of material, technology and cost.展开更多
基金Supported by NIH intramural research funds Z01-DC-000039,Z01-DC-000060 and Z01-DC-000064,NIH grants R01-DK43495 and P30-DK34854Kansas State University CVM-SMILE and the Kansas City Area Life Science Institute
文摘nPendred syndrome （PS） is characterized by autosomal recessive inheritance of goiter associated with a defect of iodide organification, hearing loss, enlargement of the vestibular aqueduct （EVA）, and mutations of the SLC26A4 gene. However, not all EVA patients have PS or SLC26A4 mutations. Two mutant alleles of SLC26A4 are detected in 1/4 of North American or European EVA populations, one mutant allele is detected in another 1/4 of patient populations, and no mutations are de-tected in the other 1/2. The presence of two mutant al-leles of SLC26A4 is associated with abnormal iodide or-ganification, increased thyroid gland volume, increased severity of hearing loss, and bilateral EVA. The pres-ence of a single mutant allele of SLC26A4 is associated with normal iodide organification, normal thyroid gland volume, less severe hearing loss and either bilateral or unilateral EVA. When other underlying correlations are accounted for, the presence of a cochlear malformation or the size of EVA does not have an effect on hearing thresholds. This is consistent with observations of an Slc26a4 mutant mouse model of EVA in which hearing loss is independent of endolymphatic hydrops or in-ner ear malformations. Segregation analyses of EVA in families suggest that the patients carrying one mutant allele of SLC26A4 have a second, undetected mutant allele of SLC26A4, and the probability of a sibling hav-ing EVA is consistent with its segregation as an autoso-mal recessive trait. Patients without any mutations are an etiologically heterogeneous group in which siblings have a lower probability of having EVA. SLC26A4 muta-tion testing can provide prognostic information to guide clinical surveillance and management, as well as the probability of EVA affecting a sibling.
文摘ased on Quasi-Vortex-Lattiee method, a program is presented to com-pute the aerodynamic forces for nonplanar wing with wing-tip sails. By using thisprogram, the aerodynamic force is calculated and the sails are designed for an aircraftwith rectangular wing of 8.6 aspect ratio. The calculation results show that thosewing-tip sails, whose total area is 3. 1 percent of the aircraft’s basic wing area, will haveremarkable effect on reducing induced drag, and the lift-dependent drag factor can bereduced by about 18.5 to 21 .5 percent. Wind tunnel tests are conducted in NH-2 windtunnel of Nanjing Aeronautical and Astronautical University, and the results demon-strate the correctness of the above calculation results. The influences of sail parameterson performance and handling qualities of aircraft are also analyzed.
文摘The present research aims to identify the possibilities of rehabilitation of building facades in the centre of Chisinau, through the use of high-performance materials, use of high-performance technologies in execution, reduction of facade renovation costs, etc. In the process of organising the rehabilitation works of historical buildings of architectural value, we are dealing with the following: the value of the rehabilitation works of a historical building is very high, the duration of the rehabilitation is important, the attitude of the society is insufficient, the lack of strategies for the rehabilitation of buildings at the municipality level. In order to achieve this objective, we propose to research the following tasks: definition of the concept of rehabilitation of buildings in the Historic Centre of Chisinau;legislative, normative assurance of rehabilitation of historical heritage in the Republic of Moldova;analysis of the situation in Chisinau on the example of historical buildings with limestone finishes and examination by non-destructive methods of facades;solutions for rehabilitation of facades of historical buildings by comparing two types of technologies. In conclusion, emphasis will be placed on the choice of the most efficient method in terms of material, technology and cost.
