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Pantothenate kinase-associated neurodegeneration (PKAN) is a rare autosomal recessive disorder with onset in childhood and rapid progression. There is no causative and insufficient symptomatic drug therapy. Deep brain stimulation (DBS) of the internal pallidum (GPi) has been reported to improve motor function. Most case reports, however, are limited to short observational periods. The impact of DBS on the progression and life expectancy in PKAN is unknown. We present a 5-year outcome and video documentation of bilateral GPi-DBS of an adolescent patient suffering from genetically defined PKAN.  相似文献   
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Interventions in the sense of a surgical session according to SGB V are determined for the most part from the number of general anaesthetics induced for the department. The capacity of an operating theatre can be calculated from experience at 4–6 operations per day, that is to say 1,000 operations per year if 200 full operating days per year are assumed. If we assume that 80% of patients in the trauma surgery department do undergo operative treatment, that the average time each patient is then occupying a bed is 7 days, and that the rate of bed use is 85%, this means that for every 1,000 surgical operations an average of 28.3 beds must be reserved – as a rule of thumb, between 20 and 30 beds for each operating theatre. In Germany, 1000 surgical patients in a trauma surgery department means 200 injured as defined by GUV; on average the injuries are serious in 25% of these cases. Speed in trauma surgery is defined in increments of 1,000 operations performed per year. Thus, double speed means 2,000 operative cases per year, two operating theatres in constant use and the treatment of 500 seriously injured patients needing trauma surgery. The rough figures mentioned can be used as a quantifiable basis for estimation of the performance structure of trauma surgery departments in Germany.  相似文献   
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AIMS: To quantitatively compare the ventricular rate-smoothing (VRS) effects of different ventricular pacing (VP) protocols for atrial fibrillation (AF). METHODS AND RESULTS: Using a recently developed open-source model that can simulate the ventricular response in AF and VP, the performance of fixed-rate pacing and four previously published VRS algorithms were assessed by the mean RR (mRR), the root mean square of successive RR differences (RMSSD), the percentage of ventricular senses (VS%), and the percentage of short RR intervals (sRR%). All pacing protocols cause rate-dependent reduction of RMSSD, VS%, and sRR% with or without shortening of mRR compared to spontaneous AF. Fixed-rate pacing was more sensitive to the intrinsic rate than the VRS algorithms. The performance was generally comparable between different VRS algorithms, although higher mRR and VS% can be achieved at the expense of larger RMSSD and sRR%. CONCLUSION: The effect of VP on ventricular rhythm in AF depends on both intrinsic rate and the aggressiveness of the pacing protocol. Adequate rate control is necessary for effective operation of the VRS algorithm. Choosing VRS algorithm should balance between the beneficial effects of rate regularization and the negative effects of increasing heart rate and percentage of VP.  相似文献   
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BACKGROUND: The isolated perfused canine kidney has been established as a valid model for conducting both renal physiology and transplantation research. This model is of particular importance for developing new strategies to improve graft function after renal transplantation. In the present study, a newly developed method using isolated haemoperfused porcine kidneys was adapted for use in canine kidneys. In contrast to haemoperfusion, synthetic perfusion media can be standardized and can prevent the initiation of blood-mediated reperfusion reactions. Thus, an additional aim was to determine whether blood could be replaced by synthetic cell-free perfusion solutions. METHODS: Canine kidneys (n = 30) were harvested from donors euthanized in veterinary practices for causes unrelated to the present study. The kidneys were isolated and perfused with autologous blood or cell-free synthetic electrolyte buffer (Tyrode solution). During perfusion, we monitored renal perfusate flow (RPF), glomerular filtration rate (GFR), electrolyte and glucose reabsorption, oxygen consumption and urine concentration. RESULTS: Changes in perfusion medium did not affect the RPF. In contrast, GFR, urine concentration and oxygen consumption were significantly higher, whereas fractional excretion of sodium and glucose were significantly lower in blood- than in Tyrode-perfused kidneys. CONCLUSIONS: This system offers a simple model for studying whole-organ functional alterations after acute renal ischaemia. Renal function indicators were below values reported during in vivo physiological conditions. These functions were better conserved when kidneys were perfused with autologous blood than with Tyrode.  相似文献   
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The present study was conducted to test the hypothesis that cholinergic basalforebrain neurons are a major source of cerebrospinal fluid (CSF) cholinesterases. To address thisquestion enzyme activities of acetylcholinesterase (AChE) and butyrylcholinesterase (BChE) inboth CSF and parietal cortex were assayed following selective lesion of basal forebrain cholinergicneurons by a single intracerebroventricular application of the cholinergic immunotoxin192IgG-saporin. Cholinergic immunolesions led to a dramatic decrease in total AChE activity inparietal cortex, which was due to the specific loss of the G4 molecular form while the activity ofthe G1 form was increased as compared to nonlesioned animals. In contrast, the total enzymeactivity of BChE and its molecular forms were not affected by cholinergic lesion in both parietalcortex and CSF. The data suggest, that cholinergic basal forebrain neurons are seemingly not amajor source of cholinesterases in the CSF, and do not provide any evidence for using CSFcholinesterases as a diagnostic marker of basal forebrain cholinergic cell loss in humans.  相似文献   
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The impact of social defeat on lymphocyte subpopulations and T helper subsets was investigated in Long Evans rats. CD4 T helper cell subsets with distinct functional properties and different cytokine profiles can be distinguished by using the mAbs OX-22 (anti-CD45RC) and OX-7 (anti-CD90, Thy1.1). Male intruders were exposed for 2, 6, or 48 h to aggressive resident pairs. All intruders were attacked upon introduction and were defeated as indicated by frequent display of full submissive postures. After 2 and 48 h of confrontation, drastic but differential effects on blood leukocyte numbers, CD4 and CD8a cells, and CD4 subsets were evident. However, after 6 h of confrontation most lymphocyte subset numbers corresponded to baseline levels. Focusing on CD4 subsets after 2 h of confrontation, we demonstrated that only the number of the CD45RCCD90subset declines, whereas neither the number of the CD45RC+CD90subset nor the number of the CD45RCCD90+subset (recent thymic emigrants) was influenced. Con A stimulation of sorted subsets identified the CD45RCCD90as a poor producer of IFN-γ. The data clearly demonstrate that social factors might differentially influence not only T cell subsets but also T helper cell subsets with distinct cytokine profiles in a possibly time-dependent manner. Such a stress-induced shift toward a CD45RC+CD90-dominated milieu may have important consequences in interpreting results obtained from mitogenic stimulation of blood lymphocytes and cytokine production profiles measured after such a stimulation. In addition, a shift toward a CD45RC+CD90dominance may modify the type and magnitude of immune response, at least temporarily.  相似文献   
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