Background: Severe hypertriglyceridemia (HTG) is associated with major complications such as acute or relapsing pancreatitis (AP) and atherosclerotic cardiovascular disease (ASCVD). Rapid elimination of triglyceride (TG)-rich lipoproteins (LP) with double filtration plasmapheresis (DFPP) without need for substitution has been found to be effective for the acute, short-term treatment of HTG-induced AP. Data on the long-term use of DFPP to prevent HTG-associated complications are scarce. Objectives: To evaluate the use and efficacy of regular DFPP treatment in clinical practice for preventing recurrence of HTG-associated complications in thera py refractory patients. Methods: Retrospective multicenter study in patients with severe symptomatic drug and diet refractory HTG with regular DFPP treatment. Patients' incidence of HTG-associated pancreatic or cardiovascular complications was compared before treatment and with regular DFPP treatment. Results: Ten patients (3 female) were identified with baseline maximal TG concentrations of 2,587-28,090 mg/dL (median 5,487 mg/dL; interquartile range [IQR] 4,340-12,636). The mean observation period was 3.9 ± 3.4 years before and 3.8 ± 3.0 years after commencement of DFPP. In 5 patients, severe HTG was related to chylomicronemia, 2 patients had familial partial lipodystrophy Dunnigan, and 1 patient had additional LP(a)-hyperlipoproteinemia. The main HTG-associated complication was recurrent AP in 8 patients, including 1 patient treated during pregnancy. Two patients presented severe progressive ASCVD. With long-term DFPP treatment, the annual rate of HTGassocia ted pancreatic or cardiovascular complications declined from median 1.4 (IQR 0.7-2.6) to 0 (IQR 0.0-0.4; p < 0.005). The absolute number of events was reduced by 77%. In 6 patients (60%) episodes of AP did not occur, nor was progression of ASCVD detected clinically or by routine imag-Grupp et al.
The new quality of the superconducting fusion device Wendelstein 7-X (W7-X) is its capability of steady state operation. Additionally the fusion device W7-X is a very complex technical system. The modular and strongly hierarchical control system has been designed to cope with these two requirements unique for fusion devices.To minimize the risks before commissioning the control and data acquisition system at W7-X it will be thoroughly tested in a prototype installation at the WEGA stellarator. WEGA is a classical stellarator which allows steady state plasma pulses at a magnetic field of 0.5T. Despite its lesser complexity WEGA has the same main components, e.g. magnetic coil systems, ECRH, and diagnostics as W7-X and is therefore considered to be a suitable test-bed for the control system. The installation of the new W7-X like control and data acquisition system has been finished in March this year. Individual components of the control system have already been commissioned during the installation phase. In April final commissioning and testing of the complete system took place. First discharges fully controlled by the prototype control system have been realized. The contribution will focus on first discharges controlled by the new system. Furthermore it presents first experiences that will incorporate into the further development of the control system and the tools for planning, preparation, and realization of plasma discharges.
The superconducting fusion device Wendelstein 7-X (W7-X) went into operation in December 2015. One of the most important steps was the commissioning of the superconducting magnet system. It consists of 70 superconducting coils, 14 HTS current leads, and more than 100 superconducting bus bars. The system is divided into seven electrical circuits with ten coils, associated bus bars and two current leads each. The commissioning of the magnet system was performed in two major steps: In a first step the seven coil circuits were separately energized and operated at several current levels in a range between 2 kA and 12.8 kA. In a second step all seven circuits were operated together. The adjustment of the quench detectors, the evaluation of the thermal and mechanical behavior, and the test of the magnet safety system were further work packages. Fast discharges were initiated to check the proper behavior of the magnet safety system. Steady-state operation of up to eight hours was performed to adjust the helium mass flows. The commissioning was successfully completed, the main goal of the magnet system is fully reached, which is also confirmed by magnetic flux measurements and the results of the first plasma operation.
The new quality of the superconducting fusion device Wendelstein 7-X (W7-X) is its potential of steady state operation. W7-X is also a very complex technical system. In these two aspects W7-X compares to other super conducting fusion devices (ITER, KSTAR, EAST, SST-1, Tore Supra, JT-60SA, and LHD). The modular and strongly hierarchical control and data acquisition (CoDaC) system has been designed to cope with these two aspects, unprecedented for the control systems used by most operating fusion devices.The CoDaC system for W7-X will be thoroughly tested in a prototype installation at the WEGA stellarator in order to minimize the risks before commissioning. WEGA is a classical stellarator which allows steady state plasma pulses at a magnetic field of 0.06 T. WEGA can run pulses up to 20s at a magnetic field up to 0.5T. Despite its lesser complexity WEGA has the same main components as W7-X, e.g. magnetic coil systems and ECRH. It is therefore considered to be a suitable test-bed for the control system. This paper focuses on discharges controlled by the new control system and the enhanced surveillance mechanisms introduced during the first year of operation. It shows characteristic curves for the key control parameters of the major components. Taking additionally dependencies and constraints between and within components into account a segmented discharge for each experiment is presented as result.Dear Dr. Volker Schmidt, I revised my contributions and the figures according to your and the reviewer's recommendations.
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