Background: Late- onset Tay- Sachs disease (LOTS) is an adult- onset, autosomal recessive, progressive variant of GM2 gangliosidosis, characterized by involvement of the cerebellum and anterior horn cells. Objective: ...Background: Late- onset Tay- Sachs disease (LOTS) is an adult- onset, autosomal recessive, progressive variant of GM2 gangliosidosis, characterized by involvement of the cerebellum and anterior horn cells. Objective: To determine the range of visual and ocular motor abnormalities in LOTS, as a prelude to evaluating the effectiveness of novel therapies. Methods: Fourteen patients with biochemically confirmed LOTS (8 men; age range 24 to 53 years; disease duration 5 to 30 years) and 10 age- matched control subjects were studied. Snellen visual acuity, contrast sensitivity, color vision, stereopsis, and visual fields were measured, and optic fundi were photographed. Horizontal and vertical eye movements (search coil) were recorded, and saccades, pursuit, vestibulo- ocular reflex (VOR), vergence, and optokinetic (OK) responses were measured. Results: All patients showed normal visual functions and optic fundi. The main eye movement abnormality concerned saccades, which were “ multistep,” consisting of a series of small saccades and larger movements that showed transient decelerations. Larger saccades ended earlier and more abruptly (greater peak deceleration) in LOTS patients than in control subjects; these changes can be attributed to premature termination of the saccadic pulse. Smooth- pursuit and slow- phase OK gains were reduced, but VOR, vergence, and gaze holding were normal. Conclusions: Patients with late- onset Tay- Sachs disease (LOTS) show characteristic abnormalities of saccades but normal afferent visual systems. Hypometria, transient decelerations, and premature termination of saccades suggest disruption of a “ latch circuit” that normally inhibits pontine omnipause neurons, permitting burst neurons to discharge until the eye movement is completed. These measurable abnormalities of saccades provide a means to evaluate the effects of novel treatments for LOTS.展开更多
In this paper,we present the simplification of Sachs formulas for the measurement and calculation of the residual stresses of the cylinder only with the plane stresses. Furthermore, we present the method for the measu...In this paper,we present the simplification of Sachs formulas for the measurement and calculation of the residual stresses of the cylinder only with the plane stresses. Furthermore, we present the method for the measurement and calculation of the residual stresses of the cylinder not only with the finite length but with the longitudinal stress. These can be applied to the investigation on the residual stresses of the autofretted gun tube.展开更多
The prosthesis is an artificial device that can replace an organ of a human body member to restore a compromised function. It is necessary following the removal of a human organ, which can occur as a result of an illn...The prosthesis is an artificial device that can replace an organ of a human body member to restore a compromised function. It is necessary following the removal of a human organ, which can occur as a result of an illness, trauma or congenital malformation. The trans-tibia prosthesis, in particular, allows the amputee patient to recover the impaired function and regain autonomy, while facilitating their daily social integration. The trans-tibia prosthesis consists of a socket, a sleeve, connecting elements and a prosthetic foot. Each of these components plays a very important role. Among these components, the prosthetic foot usually called “SACH foot” is very often replaced due to cracking and therefore has a fairly short lifespan. At the Center for Equipment and Rehabilitation of Kabalaye (CERK), the SACH foot made using polyurethane and wood is imported and is given to patients with reduced mobility. The aim of this article is twofold, on the one hand, to make a social and pathological study of trans-tibia amputees in relation to the use of the SACH foot prosthesis, on the other hand, to compare this foot with a new prosthetic foot proposed and which is manufactured using extruded polystyrene. The result of prosthetic tests carried out on twenty-four amputees showed that the foot manufactured using extruded polystyrene is better in terms of resistance, bulk and adaptability to active amputees.展开更多
Helicenes are the simply connected helicenic (geometrically non-planar ) polyhexs. In this paper, we give a construction method for concealed non-Kekulean helicenes with h=14, and rigorously prove that there are exact...Helicenes are the simply connected helicenic (geometrically non-planar ) polyhexs. In this paper, we give a construction method for concealed non-Kekulean helicenes with h=14, and rigorously prove that there are exactly 3115 concealed non-Kekulean helicenes with h=14.展开更多
联合利华公司（Unilever PLC）表示,已同意以22.7亿欧元的价格从Bain Capital Private Equity和高盛集团（Goldman Sachs Group Inc.）手中收购韩国护肤品公司Carver Korea。联合利华称,Carver将大大增强联合利华在亚洲北部市场的地位...联合利华公司（Unilever PLC）表示,已同意以22.7亿欧元的价格从Bain Capital Private Equity和高盛集团（Goldman Sachs Group Inc.）手中收购韩国护肤品公司Carver Korea。联合利华称,Carver将大大增强联合利华在亚洲北部市场的地位。亚洲是全球最大的护肤品市场。2016年,Carver实现3.21亿欧元销售额,利息﹑税项﹑折旧﹑摊销前收益（EBITDA）为1.37亿欧元。展开更多
文摘Background: Late- onset Tay- Sachs disease (LOTS) is an adult- onset, autosomal recessive, progressive variant of GM2 gangliosidosis, characterized by involvement of the cerebellum and anterior horn cells. Objective: To determine the range of visual and ocular motor abnormalities in LOTS, as a prelude to evaluating the effectiveness of novel therapies. Methods: Fourteen patients with biochemically confirmed LOTS (8 men; age range 24 to 53 years; disease duration 5 to 30 years) and 10 age- matched control subjects were studied. Snellen visual acuity, contrast sensitivity, color vision, stereopsis, and visual fields were measured, and optic fundi were photographed. Horizontal and vertical eye movements (search coil) were recorded, and saccades, pursuit, vestibulo- ocular reflex (VOR), vergence, and optokinetic (OK) responses were measured. Results: All patients showed normal visual functions and optic fundi. The main eye movement abnormality concerned saccades, which were “ multistep,” consisting of a series of small saccades and larger movements that showed transient decelerations. Larger saccades ended earlier and more abruptly (greater peak deceleration) in LOTS patients than in control subjects; these changes can be attributed to premature termination of the saccadic pulse. Smooth- pursuit and slow- phase OK gains were reduced, but VOR, vergence, and gaze holding were normal. Conclusions: Patients with late- onset Tay- Sachs disease (LOTS) show characteristic abnormalities of saccades but normal afferent visual systems. Hypometria, transient decelerations, and premature termination of saccades suggest disruption of a “ latch circuit” that normally inhibits pontine omnipause neurons, permitting burst neurons to discharge until the eye movement is completed. These measurable abnormalities of saccades provide a means to evaluate the effects of novel treatments for LOTS.
文摘In this paper,we present the simplification of Sachs formulas for the measurement and calculation of the residual stresses of the cylinder only with the plane stresses. Furthermore, we present the method for the measurement and calculation of the residual stresses of the cylinder not only with the finite length but with the longitudinal stress. These can be applied to the investigation on the residual stresses of the autofretted gun tube.
文摘The prosthesis is an artificial device that can replace an organ of a human body member to restore a compromised function. It is necessary following the removal of a human organ, which can occur as a result of an illness, trauma or congenital malformation. The trans-tibia prosthesis, in particular, allows the amputee patient to recover the impaired function and regain autonomy, while facilitating their daily social integration. The trans-tibia prosthesis consists of a socket, a sleeve, connecting elements and a prosthetic foot. Each of these components plays a very important role. Among these components, the prosthetic foot usually called “SACH foot” is very often replaced due to cracking and therefore has a fairly short lifespan. At the Center for Equipment and Rehabilitation of Kabalaye (CERK), the SACH foot made using polyurethane and wood is imported and is given to patients with reduced mobility. The aim of this article is twofold, on the one hand, to make a social and pathological study of trans-tibia amputees in relation to the use of the SACH foot prosthesis, on the other hand, to compare this foot with a new prosthetic foot proposed and which is manufactured using extruded polystyrene. The result of prosthetic tests carried out on twenty-four amputees showed that the foot manufactured using extruded polystyrene is better in terms of resistance, bulk and adaptability to active amputees.
文摘Helicenes are the simply connected helicenic (geometrically non-planar ) polyhexs. In this paper, we give a construction method for concealed non-Kekulean helicenes with h=14, and rigorously prove that there are exactly 3115 concealed non-Kekulean helicenes with h=14.
文摘联合利华公司（Unilever PLC）表示,已同意以22.7亿欧元的价格从Bain Capital Private Equity和高盛集团（Goldman Sachs Group Inc.）手中收购韩国护肤品公司Carver Korea。联合利华称,Carver将大大增强联合利华在亚洲北部市场的地位。亚洲是全球最大的护肤品市场。2016年,Carver实现3.21亿欧元销售额,利息﹑税项﹑折旧﹑摊销前收益（EBITDA）为1.37亿欧元。
