When measurements are performed in high speed, small-scale compressors, the use of curved glasswindows is required in order to avoid any mismatch between the measurement window and the casing. However, the glass curva...When measurements are performed in high speed, small-scale compressors, the use of curved glasswindows is required in order to avoid any mismatch between the measurement window and the casing. However, the glass curvature leads to optical distortions, which hinder acceptable measurementsand can even prevent the acquisition of any data. Thus, an original optical assembly, which consistsin inserting a simple and inexpensive corrective window between the frontal lens of the anemometerand the shroud window, is proposed. The way of determining the geometric characteristics and theposition of this corrective window, which restores very acceptable fool, is presented in the paper. Thereliability of this corrective optical assembly is highlighted by comparative measurements in a test case.Using such an optical setting, L2F measurements were realized along a section, downstream of the inletguide vane (IGV) of a transonic compressor stage. The spatial resolution leads to a good descriptionof the interaction of the wake with the oblique shock emanating from the leading edge of the rotor.A phenomenological study of the wake/shock interaction with a change of frame is realized using thestreamwise equation of the transport of vorticity.展开更多
BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients wi...BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients with DCM contributes to variable disease severity and complicates overall prognosis,which can be very poor.AIM To identify pathogenic genes in DCM through pedigree analysis.METHODS Our research team identified a patient with DCM in the clinic.Through invest-igation,we found that the family of this patient has a typical DCM pedigree.High-throughput sequencing technology,next-generation sequencing,was used to sequence the whole exomes of seven samples in the pedigree.RESULTS A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered.The mutation was completely consistent with the clinical information for this DCM pedigree.Sanger sequencing was used to further verify the locus of the mutation in pedigree samples.These results were consistent with those of high-throughput sequencing.CONCLUSIONS ANK2p.F3067L is considered a novel and potentially pathogenic gene mutation in DCM.展开更多
文摘When measurements are performed in high speed, small-scale compressors, the use of curved glasswindows is required in order to avoid any mismatch between the measurement window and the casing. However, the glass curvature leads to optical distortions, which hinder acceptable measurementsand can even prevent the acquisition of any data. Thus, an original optical assembly, which consistsin inserting a simple and inexpensive corrective window between the frontal lens of the anemometerand the shroud window, is proposed. The way of determining the geometric characteristics and theposition of this corrective window, which restores very acceptable fool, is presented in the paper. Thereliability of this corrective optical assembly is highlighted by comparative measurements in a test case.Using such an optical setting, L2F measurements were realized along a section, downstream of the inletguide vane (IGV) of a transonic compressor stage. The spatial resolution leads to a good descriptionof the interaction of the wake with the oblique shock emanating from the leading edge of the rotor.A phenomenological study of the wake/shock interaction with a change of frame is realized using thestreamwise equation of the transport of vorticity.
基金Supported by the Jilin Provincial Healthcare Talent Special Program,No.2019SCZT08.
文摘BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients with DCM contributes to variable disease severity and complicates overall prognosis,which can be very poor.AIM To identify pathogenic genes in DCM through pedigree analysis.METHODS Our research team identified a patient with DCM in the clinic.Through invest-igation,we found that the family of this patient has a typical DCM pedigree.High-throughput sequencing technology,next-generation sequencing,was used to sequence the whole exomes of seven samples in the pedigree.RESULTS A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered.The mutation was completely consistent with the clinical information for this DCM pedigree.Sanger sequencing was used to further verify the locus of the mutation in pedigree samples.These results were consistent with those of high-throughput sequencing.CONCLUSIONS ANK2p.F3067L is considered a novel and potentially pathogenic gene mutation in DCM.
