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1.
In the past 5 years we have discovered 8 boys and 3 girls who suffer from fructose-1,6-bisphosphatase deficiency. Although they all showed the typical symptoms of the deficiency such as frequent vomiting, hypoglycemia, lactic acidosis, and hepatomegaly, the age at diagnosis varied from 2 months to 4 years. All the boys revealed the deficient enzyme activity in leukocytes but none of the girls. The liver biopsy was investigated in six patients to confirm the diagnosis. These results suggest the existence of tissue-specific isoenzymes for fructose-1,6-bisphosphatase possibly with a different gene origin.  相似文献   
2.
OBJECTIVE: To assess how often the aetiology is established in patients with uveitis, what systemic disease are found and what is the contribution of the internist to the diagnostic process. DESIGN: Retrospective study. SETTING: University Hospital Leiden, the Netherlands. METHOD: From January 1987 to April 1992, 342 patients presented with uveitis. All patients underwent a standard ophthalmological examination. Referral to an internist and individualised laboratory screening followed in patients with recurrent, chronic, bilateral or panuveitis. Recorded were: ophthalmological data, results of laboratory screening, results of analysis by the internist, final diagnosis and presence of systemic disease. RESULTS: 149 (44%) patients were examined by the internist, 18 (5.2%) were seen by another specialist. In 169 (49%) patients a specific diagnosis was made. 74 (22%) had a systemic disease, 74 a primary ocular disease. In 28 (8%) a systemic disease was presumed (5% were HLA-B27 positive, 3% had abnormal laboratory results); 5 (1%) patients had endophthalmitis as a complication of a septic process. CONCLUSION: In approximately 1/3 of the patients with uveitis a systemic disease was found. Examination by the internist tailored to the individual patient is essential in the evaluation of uveitis patients.  相似文献   
3.
High strength in combination with low density is the key features for lightweight constructions in automotive and aerospace applications. Tailor-made fiber reinforcements in light-metal matrices could help to achieve this goal. However, the integration of fibers with conventional casting-route manufacturing techniques like squeeze casting or diffusion bonding restricts the component geometry and results in elevated process cost due to long cycle times and the need of additional fiber coatings. In the center of competence for casting and thixoforging Stuttgart (CCT), new processes for manufacturing metal matrix composites are developed. Long-fiber reinforced Al–Si alloys and components are produced by thixoforging of laminates made of alternating metal matrix layers and carbon fiber fabrics. This paper illustrates the manufacturing technology and first experimental results with special focus on fiber penetration and infiltration behavior and also on the formation of fiber-matrix interface to analyze fiber damage by mechanical or chemical attack.  相似文献   
4.
HCI plays an important role in interactive medical image segmentation. The Goals, Operators, Methods, and Selection rules (GOMS) model and the National Aeronautics and Space Administration Task Load Index (NASA-TLX) questionnaire are different methods that are often used to evaluate the HCI process. In this article, we aim at improving the HCI process of interactive segmentation using both the GOMS model and the NASA-TLX questionnaire to: 1) identify the relations between these two methods and 2) propose HCI design suggestions based on the synthesis of the evaluation results using both methods. For this, we conducted an experiment where three physicians used two interactive segmentation approaches to segment different types of organs at risk for radiotherapy planning. Using the GOMS model, we identified 16 operators and 10 methods. Further analysis discovered strong relations between the use of GOMS operators and the results of the NASA-TLX questionnaire. Finally, HCI design issues were identified, and suggestions were proposed based on the evaluation results and the identified relations.  相似文献   
5.
A combination of different electrical measurement techniques is applied to liquid crystal (display) cells; voltage drop, current-voltage, dc stress measurements and impedance spectroscopy. It is found that at small timescale (seconds) conduction is dominated by transport of mobile ions, but at long timescale (hours) by (bulk) generation of extremely low mobility ions. These slow ions accumulate at ionic double layers at the liquid crystal-polyimid interface, giving rise to electric fields over the polyimid alignment layers. These processes of charge transport and accumulation are represented in consistent and insightful electrical network models.  相似文献   
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7.
A novel method is introduced for the generation of landmarks for three-dimensional (3-D) shapes and the construction of the corresponding 3-D statistical shape models. Automatic landmarking of a set of manual segmentations from a class of shapes is achieved by 1) construction of an atlas of the class, 2) automatic extraction of the landmarks from the atlas, and 3) subsequent propagation of these landmarks to each example shape via a volumetric nonrigid registration technique using multiresolution B-spline deformations. This approach presents some advantages over previously published methods: it can treat multiple-part structures and requires less restrictive assumptions on the structure's topology. In this paper, we address the problem of building a 3-D statistical shape model of the left and right ventricle of the heart from 3-D magnetic resonance images. The average accuracy in landmark propagation is shown to be below 2.2 mm. This application demonstrates the robustness and accuracy of the method in the presence of large shape variability and multiple objects.  相似文献   
8.
Orbital soft-tissue motion analysis aids in the localization and diagnosis of orbital disorders. A technique has been developed to objectively quantify and visualize motion in the orbit during gaze. T1-weighted MR volume sequences are acquired during gaze and soft-tissue motion is quantified using optical flow techniques. The flow field is visualized using color-coding: orientation of the flow vector is coded by hue and magnitude by saturation of the pixel. Current clinical circumstances limit MR image acquisition to short sequences and short acquisition times. The effect of these limitations on the performance of optical flow computation has been studied for four representative optical flow algorithms: on short (nine frames) and long (21 frames) simulated sequences of rotation of a magnetic resonance (MR) imaged object, on short measured MR sequences of controlled rotation of the same object and on short MR sequences of motion in the orbit. On the short simulated and motion-controlled sequences, the Lucas and Kanade algorithm showed the best performance with respect to both accuracy and robustness. These motion estimates were accurate to within 20%. Motion in the orbit ranged between 0.05 and 0.25 mm/degree gaze. Color-coding was found to be attractive as a visualization technique, because it shows both magnitude and orientation of all flow vectors without cluttering.  相似文献   
9.
The manifold possibilities of the application of helium-heated steam reformers combined with high temperature nuclear reactors are elucidated in this article. It is shown that the thermodynamic interpretation of the processes does not cause difficulties because of the good heat transfer in helium at high pressure and that helium peak temperatures of 950°C are sufficient for carrying out the process. The mechanical design of the reformer tube does not lead to problems because the helium and process pressures are so chosen as to be approximately equal. The problems of hydrogen and tritium permeation as well as the contamination of the reformer tube with solid fission products seem to be solvable using the knowledge available at present. Furthermore, the various possibilities for the design arrangements of helium-heated reformer tube furnaces are shown. The status of development attained to date is outlined and in conclusion there is a survey regarding the next steps to be taken in steam reformer technology.  相似文献   
10.
Liquid chromatography (LC) was coupled on-line to a continuous-flow enzymatic assay using electrospray ionization mass spectrometry (ESI-MS) as readout for the screening of enzyme inhibitors in complex samples. Inhibitors were detected by changes in the concentration of the enzymatic reaction products, indicating the inhibition of enzymatic activity. The molecular masses of the inhibitors were determined with high certainty by using retention time matching and peak shape comparison. Due to the high matching accuracy, baseline separation of coeluting analytes was not necessary in order to identify the correct masses of the bioactive compounds. The continuous-flow system was successfully applied for the screening of complex samples, such as natural extracts. For a red clover extract, detection limits of 0.3-0.8 micromol/L were obtained. System validation was performed by determining the IC(50) values of four inhibitors in the flow-injection mode. The IC(50) values were in the 0.11-5.6 micromol/L range and correspond closely to data obtained by microtiter plate assays. Detection limits were in the range of 0.018-0.35 micromol/L in the flow-injection mode, and 0.075-0.75 micromol/L in the LC mode. These values are well below the typical compound concentrations (1-10 micromol/L) used in high-throughput screening. Together with an interday precision of 12.6%, these results demonstrate the applicability of the system for bioactivity screening of complex mixtures, generating both chemical and biological information on bioactive compounds in a single run.  相似文献   
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