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Wir berichten über den ersten Fall von polyzystischer lipomembran?ser Osteodysplasie oder “brain, bone and fat disease” in Deutschland. Die nach dem Erstbeschreibern auch als Morbus J?rvi-Hakola-Nasu bezeichnete Erkrankung ist bisher vor allem in Japan und in Finnland beschrieben worden. Einzelne F?lle wurden aus Schweden, Norwegen, Italien, Südafrika, Belgien und den USA berichtet. Im deutschsprachigen Raum ist bisher ein Fall aus ? ver?ffentlicht worden.  相似文献   
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Oxygen withdrawal from myocardial cells leads to changes of the transmembrane action potential (mainly action potential shortening), to cellular uncoupling, and to changes of vascular permeability. This study was aimed at the simultaneous measurement of electrical activity and passive electrical properties (extracellular and intracellular longitudinal resistance) in arterially perfused rabbit papillary muscles under different conditions of changed oxygen supply. These included 1) complete anoxia (erythrocyte-free perfusate), 2) hypoxia (PO2 between 23-28 mm Hg, erythrocytes present) in the presence and absence of glucose, and 3) normoxia with erythrocyte-free perfusate. Similarly to myocardial ischemia, rapid cellular uncoupling occurred only after an initial stable period of approximately 17 minutes, and it required complete anoxia. The marked shortening of the action potential developed before cellular uncoupling. In six out of eight experiments, the fibers were inexcitable when uncoupling started. In severe hypoxia, no significant change of internal longitudinal resistance was observed over 35-40 minutes. The time course of the extracellular longitudinal resistance was different from the change in intracellular resistance: A marked decrease occurred almost immediately after the onset of oxygen withdrawal. This decrease was followed by a small increase in conduction velocity, which was most likely due to a change in the interstitial compartment (edema). It was observed during anoxic as well as during hypoxic perfusion. We conclude that 1) cellular uncoupling in arterially perfused tissue requires almost complete oxygen lack and occurs with a delay of more than 10 minutes, 2) marked action potential shortening precedes uncoupling, and therefore can not simply be attributed to an increase in free, intracellular calcium, and 3) vascular endothelial function is more sensitive to oxygen withdrawal than the myocyte.  相似文献   
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Abstract Background and Aim: The prediction of soft tissue esthetics is important for achieving an optimal esthetic outcome in orthodontic treatment planning. Applicable procedures have so far been restricted to two-dimensional profile predictions that have not proven to be very reliable. The goal of this investigation was therefore to develop a novel finite element-based procedure that allows a three-dimensional, easily visualized, quantitative analysis and prediction of soft tissue behavior for the clinician. The procedure to be developed should be easy to handle and not entail any additional radiation exposure for the patient. Material and Methods: Using a three-dimensional scanner, the facial surfaces of 20 probands were digitalized and individual FEM models were generated. Results: After reduction of data redundancy via several conversion steps, a patient-specific simulation model was prepared consisting of 20,000 to 40,000 individual elements to which specific physical properties could be assigned. The average time required for generating a virtual model was 50 minutes. Problems occurring during model generation were rare (mainly shadowing phenomena and movement artifacts). Conclusion: The procedure outlined herein makes the reliable generation of patient-specific simulation models possible for facial soft tissue prediction in orthodontics.  相似文献   
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BACKGROUND AND AIM: The goal of this study was to analyze the validity and prediction accuracy of a newly-developed procedure for three-dimensional soft tissue prediction based on Finite Element Method, and to compare the results with prediction produced using an existing two-dimensional prediction program (Dentofacial Planner Plus). PATIENTS AND METHODS: In twelve patients who underwent combined surgical-orthodontic treatment, profile prediction was generated using both procedures preoperatively and then compared at predefined measurement points with the patient's actual postoperative soft tissue status. RESULTS: The deviations observed depended on the facial region, whereby the prediction errors for both procedures were much greater in the lower facial third than in the midfacial third. Calculating in all the measurement points, the mean horizontal prediction error was 0.32 mm for the Finite Element Method and 0.75 mm for the Dentofacial Planner Plus. Overall, we were able to demonstrate the new procedure's superior validity and quality of visualization. In addition to profile prediction, the procedure allows a differentiated three-dimensional assessment of esthetically important regions such as the cheeks, nasolabial folds and the nasal wings. Additional X-radiation is not necessary in this risk-free and stress-free procedure. CONCLUSION: Three-dimensional soft tissue prediction employing finite element modeling is a useful aid for implementing esthetically-optimized treatment planning.  相似文献   
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Pars plana vitrectomy in chronic uveitis   总被引:1,自引:0,他引:1  
Statistical analysis of 54 eyes with chronical endogenous uveitis, mostly juvenile, treated with simple or combined pars plana vitrectomy. Most striking is the high frequency of localized or combined cyclitis with good functional results, although complications of the disease (retinal detachment, vasculitis, PVR, macular damage) are extremely frequent in cyclitis, and this group also includes, three cases of postoperative phthisis. The best argument in favor of an chronic uveitis is the fact that an overwhelming majority of the patients were able to switch from rigorous systemic preoperative medication to simple eye drops or no treatment at all.  相似文献   
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This study aimed at investigating the direct effects of two low osmolality radiographic contrast media, iohexol (non-ionic) and ioxaglate (ionic), on transmembrane action potentials, contractile force and refractoriness of isolated rat atrial preparations. Superfused with 10% solution, ioxaglate induced a biphasic mechanical response, initially decreasing and thereafter increasing contractile force. The same concentration of iohexol only increased contractile force. The two contrast media increased the resting membrane potential, action potential amplitude and rate of depolarization to the same extent. Iohexol prolonged repolarization. Both contrast media decreased the effective refractory period. The direct effects of contrast media on myocardial contractility in this study can explain the different haemodynamic effects of iohexol and ioxaglate in vivo. The observed effects on the action potentials in vitro can explain commonly observed ECG changes during coronary arteriography. The changes in depolarization, repolarization and refractoriness are probably related to the arrhythmogenic properties of these contrast media.  相似文献   
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