3,386 research outputs found
Inter-reciprocity applied to electrical networks
No full textElectrical Engineering, Mathematics and Computer Scienc
'Twas on the beach at Brighton one fine Summer day, I met this handsome man who stole my heart away [first line of chorus]
No full textstrophic with choruspiano and voiceTo Wm. Lingard, Author & Singerads on inside back cover for J.L. Peters stock353-3Johns Hopkins University, Levy Sheet Music Collection, Box
053, Item 027W.D. Raphaelson.Sung by LingardPaleri[?
'Twas on the beach at Brighton one fine Summer day, I met this handsome man who stole my heart away [first line of chorus]
No full textstrophic with choruspiano and voiceTo Wm. Lingard, Author & Singerads on inside back cover for J.L. Peters stock353-3Johns Hopkins University, Levy Sheet Music Collection, Box
053, Item 027W.D. Raphaelson.Sung by LingardPaleri[?
Feasibility Study of a Balanced Upper Arm Orthosis based on Bending Beams
No full textPeople with neuromuscular diseases request an orthosis close to the body for assistance with their arm movements. This paper proposes a concept for a passive arm support that is close to the body and is based on bending beams. Simulations resulted in the final configuration and dimensions of the beams, optimised to balance an arm. One Carbon-fibre-reinforced polymer beam with dimensions 0.22x0.0041x0.0027m at the medial side and one at the lateral side of the upper arm delivers the required energy for balancing the arm. Experimental evaluation of a prototype demonstrated the technical principle; more than 87% of the moment around the shoulder was balanced between 0 and 1.1rad. A second prototype was built for preliminary evaluation of the concept in relation to the body. The width of the elastic and structural elements was more than four times smaller than in present arm supports. From this it was concluded that bending beams have the potential to make an orthosis that is closer to the upper arm than current orthoses.BMEBioMechanical EngineeringMechanical, Maritime and Materials Engineerin
Incidents of Travel in Egypt, Arabia Petraea, and the Holy Land. by J.L. Stephens, author of Incidents of travel in Greece, Turkey, Russia, and Poland.Edinburgh published by William and Robert Chambers. and sold by all booksellers 1839.
No full textPreface: as Prefatory noticeDedication:Content description: Detailed contentsPagination: PP4+119PVolumes: 1Edition:6thText Genre:Journa
J.L. Brierly and The Modernization of International Law
No full textIn this Article, the author provides an analysis of a classic of international law, The Law of Nations, by J.L. Brierly. The author describes Brierly as an international legal scholar whose modernization of international law involves an emphasis on fact and complexity, an emphasis that is ultimately little more than a gesture. The author then examines the narrative structure of The Law of Nations and indicates the normative messages disclosed in Brierly\u27s telling of the story of international law. Finally, the author describes Brierly\u27s effort to describe international law as occupying a political realm while Brierly\u27s evolutionary optimism made him anything but a political realist. In short, the author sees in Brierly\u27s promises of complexity and realism a thinly veiled simplicity that would be subsumed into the orthodoxies of international legal thought
Experimental application of a dynamic observer to capture and predict the dynamics of a flat-plate boundary layer
No full textThe recent approach, proposed by Guzman-Inigo et al. \cite{GuzmanInigo2014}, using System Identification to derive a Reduced Order Model from snapshots of a flow is applied to a transitional boundary layer growing over a flat-plate. It is shown that such an approach can indeed be applied to experimental PIV snapshots. Using a proper learning dataset and a proper local sensor, it is shown that the evolution of boundary layer can be properly estimated from the time evolution of the local probe and with no more than ten POD modes for the Reduced Order Model. The influence of the various parameters on the efficiency of the system identification technique is discussed
Rett syndrome
No full textRett syndrome is a rare genetic disorder affecting predominantly females resulting in severe physical and mental disability. In this Primer, Gold and colleagues review the epidemiology, mechanisms, diagnosis and treatment as well as highlight the quality of life of individuals with Rett syndrome. © Springer Nature Limited 2024..
Rett syndrome (RTT) is a severe, progressive, neurodevelopmental disorder, which affects predominantly females. In most cases, RTT is associated with pathogenic variants in MECP2. MeCP2, the protein product of MECP2, is known to regulate gene expression and is highly expressed in the brain. RTT is characterized by developmental regression of spoken language and hand use that, with hand stereotypies and impaired ambulation, constitute the four core diagnostic features. Affected individuals may present multiple other neurological impairments and comorbidities, such as seizures, breathing irregularities, anxiety and constipation. Studies employing neuroimaging, neuropathology, neurochemistry and animal models show reductions in brain size and global decreases in neuronal size, as well as alterations in multiple neurotransmitter systems. Management of RTT is mainly focused on preventing the progression of symptoms, currently improved by guidelines based on natural history studies. Animal and cellular models of MeCP2 deficiency have helped in understanding the pathophysiology of RTT and guided the development of trofinetide, an IGF1-related compound, which is an approved drug for RTT, as well as of other drugs and gene therapies currently under investigation
Rett Syndrome: Revised diagnostic criteria and nomenclature
No full textObjective: Rett syndrome (RTT) is a severe neurodevelopmental disease that affects approximately 1 in 10,000 live female births and is often caused by mutations in Methyl-CpG-binding protein 2 (MECP2). Despite distinct clinical features, the accumulation of clinical and molecular information in recent years has generated considerable confusion regarding the diagnosis of RTT. The purpose of this work was to revise and clarify 2002 consensus criteria for the diagnosis of RTT in anticipation of treatment trials. Method: RettSearch members, representing the majority of the international clinical RTT specialists, participated in an iterative process to come to a consensus on a revised and simplified clinical diagnostic criteria for RTT. Results: The clinical criteria required for the diagnosis of classic and atypical RTT were clarified and simplified. Guidelines for the diagnosis and molecular evaluation of specific variant forms of RTT were developed. Interpretation These revised criteria provide clarity regarding the key features required for the diagnosis of RTT and reinforce the concept that RTT is a clinical diagnosis based on distinct clinical criteria, independent of molecular findings. We recommend that these criteria and guidelines be utilized in any proposed clinical research
Trofinetide. Glycine-proline-glutamate (GPE) analogue, Treatment of Rett syndrome, Treatment of fragile X syndrome
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