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1.
BACKGROUND: Langerhans' cell histiocytosis (LCH) is an uncommon, poorly understood granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. In 1985, the Philadelphia Work-shop adopted the term "Langerhans' cell histiocytosis" (LCH) to differentiate it from reactive and neoplastic causes of histiocytosis. METHODS: This study includes 73 pediatric patients diagnosed with this condition in Dublin, Ireland, and Nottingham, England, during a 34-year period (1959 to 1993). These patients are reviewed with respect to clinical presentation, difficulty with making a histological diagnosis, their management, and outcome. RESULTS: A total of 49 patients (67%) had head and neck involvement. Bony involvement was the most frequent sign, most frequently located in the skull. There were 11 deaths (15%) in this series, all associated with multisystem disease, and nine of these deaths were in children younger than 2 years of age. CONCLUSIONS: The role of otolaryngologists is important in the early and accurate evaluation, staging, and diagnosis of LCH. It may mimic more common diseases, such as otitis externa, acute mastoiditis, skin rash, gingivitis, or cervical lymphadenopathy. Patients with multisystem disease may be so ill at presentation that the head and neck lesions may be overlooked. The current management of LCH has become increasingly conservative, and in the 1990s, fewer cases are given chemotherapy or radiotherapy. The prognosis is very good for single-system disease and poor for multisystem disseminated disease with early onset.  相似文献   
2.
Thermal analysis has been used to study annealing-induced ordering in segmented elastomers. Twelve segmented elastomers were studied each having approximately 50% by wt hard segment content. Seven general classes of materials were examined including polyether and polyester polyurethanes, polyether polyurethane-urea, and polyether-polyester. Materials were slow cooled (?10°C min?1) from the melt to an annealing temperature (?10°, 20°, 60°, 90° or 120°C) where they were annealed (16, 12, 8, 6 or 4 days, respectively). Annealing was followed by slow cooling (?10°C min?1) to ?120°C after which a d.s.c. experiment was run. In general, annealing resulted in an endothermic peak at a temperature 20°–50°C above that of the temperature of annealing. This phenomenon was observed in both semicrystalline and amorphous materials. The closer the annealing endotherm was to a crystalline endotherm without exceeding it in temperature, the larger its size. Annealing endotherms resulted from hard or soft segment ordering. Only one annealing endotherm was observed for a given annealing history, even though in some materials hard and soft segments could exhibit annealing-induced morphological changes. Hard segment homopolymers were studied yielding results similar to the block polymers containing shorter sequences of the same material. This suggests that annealing-induced ordering is an intradomain phenomenon not associated with the interphase between domains, or necessarily dependent on the chain architecture of segmented elastomers.  相似文献   
3.
Metabolic physiology of aroma-producing Kluyveromyces marxianus   总被引:3,自引:0,他引:3  
Kluyveromyces marxianus has a high potential for industrial production of aroma compounds, such as 2-phenylethanol, which is derived in a bioconversion from L-phenylalanine. In the present work the product yield of K. marxianus in batch cultivation was estimated as 0.65 mol 2-phenylethanol/mol L-phenylalanine and thus significantly below the theoretical optimum of 1 mol/mol. By a comprehensive approach of stoichiometric balancing and GC-MS analysis of various substrates and products of K. marxianus a detailed insight into its metabolism was gained. For this purpose ring-labelled ((13)C(6)) L-phenylalanine and naturally labelled glucose were applied as substrates in tracer studies in batch culture. The produced aroma compounds 2-phenylethanol and 2-phenylethylacetate stem exclusively from the supplied L-phenylalanine, whereas glucose was not converted into these products because of efficient feed-back inhibition of prephenate dehydratase in the L-phenylalanine biosynthetic pathway. It could be further shown that the supplied L-phenylalanine completely covers the anabolic cellular demand for this amino acid. Quantification of (13)CO(2) in the exhaust gas provided clear evidence for catabolic breakdown of L-phenylalanine during cultivation. Metabolic balancing around the pool of free intracellular L-phenylalanine revealed a significant loss of L-phenylalanine into catabolic and anabolic pathways. While 73.3% of L-phenylalanine was converted into 2-phenylethanol or 2-phenylethylacetate, 22.4% was catabolized through the cinnamate pathway and 4.3% was directed towards protein biosynthesis. Catabolic breakdown of L-phenylalanine via hydroxylation to L-tyrosine could be excluded. In addition to an insight into metabolic functioning and regulation of 2-phenylethanol-producing K. marxianus, the approach presented here provides important information on potential targets for genetic optimization of 2-phenylethanol-producing yeasts.  相似文献   
4.
BACKGROUND: Microvascular obstruction within an area of myocardial infarction indicates worse functional recovery and a higher risk of postinfarction complications. After prolonged coronary occlusion, contrast-enhanced MRI identifies myocardial infarction as a hyperenhanced region containing a hypoenhanced core. Because the time course of microvascular obstruction after infarction/reperfusion is unknown, we examined whether microvascular obstruction reaches its full extent shortly after reperfusion or shows significant progression over the following 2 days. METHODS AND RESULTS: Seven dogs underwent 90-minute balloon occlusion of the left anterior descending coronary artery (LAD) followed by reflow. Gadolinium-DTPA-enhanced MRI performed at 2, 6, and 48 hours after reperfusion was compared with radioactive microsphere blood flow (MBF) measurements and myocardial staining to define microvascular obstruction (thioflavin S) and infarct size (triphenyltetrazolium chloride, TTC). The MRI hypoenhanced region increased 3-fold during 48 hours after reperfusion (3.2+/-1.8%, 6.7+/-4.4%, and 9.9+/-3.2% of left ventricular mass at 2, 6, and 48 hours, respectively, P<0.03) and correlated well with microvascular obstruction (MBF <50% of remote region, r=0.99 and thioflavin S, r=0.93). MRI hyperenhancement also increased (21.7+/-4.0%, 24.3+/-4.6%, and 28.8+/-5.1% at 2, 6, and 48 hours, P<0.006) and correlated well with infarct size by TTC (r=0.92). The microvascular obstruction/infarct size ratio increased from 13.0+/-4.8% to 22.6+/-8.9% and to 30.4+/-4.2% over 48 hours (P=0.024). CONCLUSION: The extent of microvascular obstruction and the infarct size increase significantly over the first 48 hours after myocardial infarction. These results are consistent with progressive microvascular and myocardial injury well beyond coronary occlusion and reflow.  相似文献   
5.
The reconstruction of helical particles with variable pitch   总被引:2,自引:0,他引:2  
In the reconstruction of helical particles, it is normally assumed that translation along the length of a particle is coupled to rotation about its axis. This assumption is not valid for particles whose pitch varies along the particle length (e.g. actin, HbS fibers), and application of the usual algorithms results in significant errors in both the shape and coordinates of subunits in the reconstructed density map. We have developed an iterative procedure for reconstructing particles with variable pitch. The goal of this procedure is to obtain an accurate estimate of the local pitch of the particle which can then be incorporated into the reconstruction algorithm. This involves synthesis of trial model structures which have constant pitch. The local pitch is derived from a cross-correlation analysis between these trial models and the variable pitch particles. The constant pitch models are constructed using coordinates measured from the reconstructed density maps. Each iteration of the procedure provides an improved estimate of the pitch which is incorporated into the succeeding iteration. The fidelity of the reconstruction is determined from cross-correlation between the original micrograph and a variable pitch model. The iterations are continued until the cross-correlation coefficient between the variable pitch model and the micrograph of the particle is maximized. The implementation of the iterative procedure is described and its behavior is evaluated using model structures which incorporate variations in pitch similar to those actually occurring in sickle hemoglobin fibers. The results indicate that the iterative reconstruction procedure considerably reduces the errors associated with constant pitch reconstructions. These tests provide a basis for applying this procedure in the structural analysis of micrographs of helical particles which display variable pitch. Application to sickle hemoglobin fibers resulted in an improvement in the accuracy with which the hemoglobin S molecules can be located in the density maps.  相似文献   
6.
We investigated the impact of uncertainty on leadership preferences and propose that the conjunction of self-esteem level and stability is an important moderator in this regard. Self-threatening uncertainty is aversive and activates the motivation to regain control. People with high and stable self-esteem should be confident of achieving this goal by self-determined amelioration of the situation and should therefore show a stronger preference for democratic leadership under conditions of uncertainty. By contrast, people with low and unstable self-esteem should place their trust and hope in the abilities of powerful others, resulting in a preference for autocratic leadership. Studies 1a and 1b validate explicit and implicit leadership measures and demonstrate a general prodemocratic default attitude under conditions of certainty. Studies 2 and 3 reveal a democratic reaction for individuals with stable high self-esteem and a submissive reaction for individuals with unstable low self-esteem under conditions of uncertainty. In Study 4, this pattern is cancelled out when individuals evaluate leadership styles from a leader instead of a follower perspective. (PsycINFO Database Record (c) 2011 APA, all rights reserved)  相似文献   
7.
While the plain film and nuclear medicine bone scan are still the traditional imaging modalities used in the evaluation of musculoskeletal infection, the cross-sectional imaging modalities, computed tomography (CT) and magnetic resonance imaging (MRI), have become critical in the delineation of many types of musculoskeletal infection. In particular, the evaluation of soft tissue infections, including cellulitus, myositis, fasciitis, abscess, and septic arthritis are often best evaluated by MRI or CT due to their excellent anatomic resolution and soft tissue contrast. Even in osseous infection, CT and MRI can give better anatomic delineation of the extent of infection. In cases where the plain film and nuclear medicine bone scan findings are complicated due to previous surgery, trauma, or underlying illness, the anatomic resolution and soft tissue contrast provided by MRI and CT are often necessary to determine if underlying infection exists. MRI's visualization of the bone marrow allows for the sensitive detection of osteomyelitis, although specificity for the diagnosis of osteomyelitis is aided by other findings, including cortical destruction. The CT and MRI findings in the spectrum of musculoskeletal infections are discussed and contrasted, and pitfalls in their evaluation of musculoskeletal infection are described.  相似文献   
8.
The authors evaluated a flow-independent magnetic resonance (MR) imaging technique in extremity veins with slow flow that relies on the prolonged T2 of blood to create angiographic contrast. A commercially available heavily T2-weighted fast-spin-echo sequence was optimized for MR venography in volunteer and phantom studies. Good depiction of venous anatomy was routinely obtained with the optimized method. Fast-spin-echo MR venography allowed evaluation of slow-flow systems such as the calf and forearm veins.  相似文献   
9.
PURPOSE: To compare spiral computed tomography (CT) with interscan spacing of 4-5 mm versus 8-10 mm for detection rate and level of confidence in diagnosis of pulmonary nodules. MATERIALS AND METHODS: Four radiologists (two junior and two senior faculty members) retrospectively reviewed 67 spiral CT studies with one to six nodules per study. Every second image was masked, which resulted in 8-mm sections every 8 or 10 mm; then all images reconstructed every 4 or 5 mm were reviewed. Lesions were classified as definite, probable, or possible. RESULTS: Narrow interscan spacing yielded more lesions overall (583 vs 566, P < .025) and more definite lesions and fewer equivocal lesions (482 vs 431 and 101 vs 135, respectively; P < .055). The greatest effects were in the reduction of possible lesions (50 vs 88, P < .001) and in the reduction of false-positive diagnoses made by less experienced radiologists. CONCLUSION: Increased reconstruction frequency of spiral CT volume data sets improves detection of pulmonary nodules and enhances confidence in the diagnosis.  相似文献   
10.
A "one-stop shop" for evaluating cardiac disease with magnetic resonance (MR) imaging is progressing toward clinical reality and promises to have a major effect on the care of patients with cardiac disease. T1-weighted conventional spin-echo imaging gated to the cardiac cycle yields good anatomic detail but requires long imaging times and provides only static images of a single cardiac phase. Fast MR imaging with electrocardiographically (ECG) gated, low-flip-angle, segmented k-space gradient-recalled-echo (GRE) sequences provides excellent image quality with sufficiently high temporal resolution to "freeze" cardiac motion. Segmented k-space sequences improve on standard ECG-gated GRE sequences by allowing many cardiac phases, or frames of a cine sequence, to be imaged in a single breath hold with prospective cardiac gating. As commercial implementations of segmented k-space imaging become more widely available, the applications of this technique are expanding from research protocols to include many clinical applications in the heart and great vessels. Such applications include evaluation of vascular anatomy (coronary angiography, aortic disease, aberrant vessels, vascular access), cardiac anatomy (congenital anomalies, right ventricular dysplasia, constrictive pericarditis, valvular function), myocardial perfusion, and myocardial wall motion.  相似文献   
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