8,839 research outputs found

    Immunoglobulin light chain amyloidosis

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    Primary light chain amyloidosis is the most common form of systemic amyloidosis and is caused by misfolded light chains that cause proteotoxicity and rapid decline of vital organ function. Early diagnosis is essential in order to deliver effective therapy and prevent irreversible organ damage. Accurate diagnosis requires clinical skills and advanced technologies. The disease can be halted and the function of target organs preserved by the prompt reduction and elimination of the plasma cell clone producing the toxic light chains in the bone marrow. Heart damage is the major determinant of survival, and staging with cardiac biomarkers guides treatment. Two-thirds of patients can benefit from treatment with improved quality of life and extended survival. Future efforts should be directed at early diagnosis, improving the tolerability and efficacy of anti-plasma cell therapy, accelerating recovery of organ function via promoting resorption of amyloid deposits, and developing novel approaches to counter light chain proteotoxicity

    Amyloidosis: pathogenesis and new therapeutic options.

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    The systemic amyloidoses are a group of complex diseases caused by tissue deposition of misfolded proteins that results in progressive organ damage. The most common type, immunoglobulin light chain amyloidosis (AL), is caused by clonal plasma cells that produce misfolded light chains. The purpose of this review is to provide up-to-date information on diagnosis and treatment options for AL amyloidosis. Early, accurate diagnosis is the key to effective therapy, and unequivocal identification of the amyloidogenic protein may require advanced technologies and expertise. Prognosis is dominated by the extent of cardiac involvement, and cardiac staging directs the choice of therapy. Treatment for AL amyloidosis is highly individualized, determined on the basis of age, organ dysfunction, and regimen toxicities, and should be guided by biomarkers of hematologic and cardiac response. Alkylator-based chemotherapy is effective in almost two thirds of patients. Novel agents are also active, and trials are ongoing to establish their optimal use. Treatment algorithms will continue to be refined through controlled trials. Advances in basic research have led to the identification of new drug targets and therapeutic approaches, which will be integrated with chemotherapy in the future. J Clin Oncol 29:1924-1933. (C) 2011 by American Society of Clinical Oncolog

    Common Mode Currents in DC Power Routers

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    The grid reinforcement and energy redirection needs have led to the emergence of Back-To-Back Voltage Source Converter (BTB-VSC) based dc power routers. This paper investigates the low frequency Common Mode Currents (CMCs) that arise in the system if the employed BTB-VSCs have an un-isolated ac path connected in parallel to their output ports. Simulation results are presented to show a sensitivity analysis of lower order harmonics in CMC with respect to the operating active and reactive power of the dc router, dc link voltage, link resistance, modulation method and pole capacitance. Experimental results are shared to show existance of lower order CMC in 3-wire ac link operating in parallel with the dc power router and these are mitigated using zero sequence controller

    Modeling, Control, and Operation of an M-DAB DC-DC Converter for Interconnection of HVDC Grids

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    Future high-voltage direct-current (HVDC) networks based on voltage source converters (VSCs) will have different structures (asymmetric monopolar, bipolar, or symmetric monopolar), voltage levels, control, and protection schemes. Therefore, dc-dc converters are needed to interconnect those VSC-HVDC grids and several technical issues on their control and operational systems must be adequately addressed. A dc-dc converter based on a modular-dual active bridge (M-DAB) converter is suggested to reach a desirable interconnection of the HVDC grids and regulate power flow (PF) between them. A dynamic averaged model is proposed for the M-DAB converter and its stability is analyzed using the Lyapunov function. Moreover, a new local controller based on nonlinear control theory is proposed for the M-DAB. The new M-DAB local controller is integrated with the energy management system (EMS), by updating the PF equations, to create a complete control structure. Considering the CIGRE DCS3 HVDC test system and the studied M-DAB, static, dynamic simulation, and experimental studies are conducted and the dc-dc converter and the performance of the designed controllers and the EMS are examined and validated.Green Open Access added to TU Delft Institutional Repository 'You share, we take care!' - Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Intelligent Electrical Power Grid

    Restructuring the existing medium voltage distribution grids using DC systems

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    Green Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.DC systems, Energy conversion & Storag

    Cold cracking in DC-cast high strength aluminum alloy ingots: An intrinsic problem intensified by casting process parameters

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    For almost half a century the catastrophic failure of direct chill (DC) cast high strength aluminum alloys has been challenging the production of sound ingots. To overcome this problem, a criterion is required that can assist the researchers in predicting the critical conditions which facilitate the catastrophic failure of the ingots. This could be achieved at first glance by application of computer simulations to assess the level and distribution of residual thermal stresses. However, the simulation results are only able to show the critical locations and conditions where and when high stresses may appear in the ingots. The prediction of critical void/crack size requires simultaneous application of fracture mechanics. In this paper, we present the thermo-mechanical simulation results that indicate the critical crack size distribution in several DC-cast billets cast at various casting conditions. The simulation results were validated upon experimental DC-casting trials and revealed that the existence of voids/cracks with a considerable size is required for cold cracking to occur.Materials Science & EngineeringMechanical, Maritime and Materials Engineerin

    A Dynamically Reconfigurable Recursive Switched-Capacitor DC-DC Converter with Adaptive Load Ability Enhancement

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    Multiple voltage conversion ratio (VCR) recursive switched-capacitor (SC) dc-dc converters, based on several basic 2:1 converters, are widely used for on-chip power supplies due to their flexible VCRs for higher energy efficiency. However, conventional multiple VCR SC converters usually have one or more 2:1 converters unused for some VCRs, which results in lower power density and chip area wastage. This article presents a new recursive dc-dc converter system, which can dynamically reconfigure the connection of all on-chip 2:1 converter cells so that the unused converters in the conventional designs can be reused in this new architecture for increasing the load-driving capacity, power density, and power efficiency. To validate the design, a 4-bit-input 15-ratio system was designed and fabricated in a 180-nm BCD process, which can support a maximum load current of \text{0.71}\,\text{mA} and achieves a peak power efficiency of 93.1% with 105.3\,\mu \text{A/mm} {2} chip power density from a 2-V input power supply. The measurement results show that the load-driving capacity can become 6.826×, 2.236×, and 2.175× larger than the conventional topology when the VCR is 1/2, 1/4, and 3/4, respectively. In addition, the power efficiency under these specific VCRs can also be improved considerably.Green Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Electronic Instrumentatio

    From DC nano- and microgrids towards the universal DC distribution system - a plea to think further into the future

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    The traditional ac power system is challenged by the increased amount of distributed energy resources. Enormous changes and investments are necessary in order to achieve an ac smart grid capable of coping with the challenges introduced. In dc, solutions seem to be more straightforward since most of the distributed energy sources and most of the loads connected to the low voltage grid operate with dc. For this reason, dc distribution systems should be considered as an alternative with significant potential. Nevertheless, nowadays research is mainly focused on local dc nano- and microgrids. This paper introduces relevant aspects related to dc distribution networks. A wide field of opportunities and challenges are briefly exposed.Accepted Author ManuscriptOld - EWI-ESE-DC&S DC systems & StorageElectrical Power Processin

    Power Disequilibrium Suppression in Bipolar DC Distribution Grids By Using A Series-Parallel Power Flow Controller

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    The unbalanced power between positive and negative poles in a bipolar DC distribution network (DC-DN) generates an unbalanced current at the neutral line, which enlarges the power losses of the system and the voltage deviation of DC loads. An unbalanced power suppression strategy based on a series-parallel power flow controller (SP-PFC) is proposed in this paper. The SP-PFC is adopted as the interconnection between two different DC-DNs. The topology and operating modes of SP-PFC are analyzed. Subsequently, SP-PFC output voltage and line current expressions under constant power control are derived. The nonlinear relationship between the output voltage and line current is linearized at the operating point. On this basis, the influences of unbalanced load and receiving-end voltage on the SP-PFC are investigated. A small-signal model of bipolar DC-DN containing an SP-PFC is established, and the system stability is analyzed. A simulation model of the bipolar DC distribution network containing an SP-PFC is built up in MATLAB/Simulink, and the effectiveness of the SP-PFC in the suppression of unbalanced power is verified.Green Open Access added to TU Delft Institutional Repository 'You share, we take care!' - Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.DC systems, Energy conversion & Storag

    DC-DC Converters

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    DC-DC converters take a DC voltage as input and generate a different DC voltage (i.e., a DC voltage that is bigger or smaller than input) as output. This chapter studies some of the important DC-DC converter circuits. © 2024, The Author(s), under exclusive license to Springer Nature Switzerland AG
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