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121.
Fabry disease (OMIM 301500) is a rare X-linked recessive disorder caused by mutations in the alpha-galactosidase gene (GLA). Loss of alpha-galactosidase (alpha-Gal) activity leads to the abnormal accumulation of glycosphingolipids in lysosomes predominantly of vascular endothelial cells. Clinically the disorder presents with angiokeratomas, clouding of the cornea, and renal, cardiac, and cerebrovascular complications. In addition, there is an increased incidence of neuropathic pain in Fabry patients. In this study, we investigated the implications of loss of alpha-galactosidase A activity on sensorimotor function and peripheral nervous system. Similar to the described in Fabry disease patients, the sensorimotor assessment of Fabry mice revealed diminished locomotor activity and warm hypoalgesia as assessed in the hot-plate. Moreover Fabry mice displayed alterations both in balance and co-ordination. By histological analysis, the cyto-architecture of Fabry mice sciatic nerves showed an increase in mean cross-sectional area accompanied by a decrease in the density of non-myelinated fibers as well as a trend for a decreased number of small myelinated fibers, a well established feature of Fabry disease. A relative preservation of large myelinated fibers and nerve conduction velocity measurements was observed. Our findings demonstrate for the first time that Fabry knockout mice have Gb3 accumulation in the peripheral nervous system, alterations in sensorimotor function, hypoalgesia and no impairment of motor nerve conduction.  相似文献   
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Pterocaulon polystachyum DC is a native species to southern Brazil, Paraguay, Uruguay and northeastern Argentina. It is utilized to treat animal problems popularly diagnosed as "mycoses". The antifungal and amebicidal activity of its hexane extract has been previously reported, although there are no studies confirming the safety of this plant for therapeutic purposes to date. Hence, this study investigates the toxic effects of a hexane extract of Pterocaulon polystachyum administered as acute and subacute oral treatments. After acute treatment the extract caused alterations in biochemical parameters, morphological alterations in tissues and was genotoxic, according to the comet assay; neither mortality nor visible signs of lethality were seen in mice. Similarly subacute treatment caused important differences in biochemical parameters and tissues, between control and treated groups. The results also revealed genotoxicity in kidney tissue, though no mutagenicity was detected by the micronucleus test. No animal died during the treatment period.  相似文献   
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PURPOSE: This study analyzed the continued nursing education process in three university hospitals in southern Brazil. Its theoretical reference was centered upon the problematization pedagogy of Freire and the levels of practice of Vázquez. METHODS: This was a qualitative case study, based on multiple holistic cases. The data were collected through document analysis, observation of scenarios, and semistructured interviews with 22 nursing employees. RESULTS: The thematic analysis evidenced the continued nursing education process, highlighting initiatives such as the creation of the Center for Nursing Education and Research, the Permanent Nursing Education Center, and the Continued Nursing Education Program. CONCLUSION: This study serves as an important tool for the transformation of the reality of health care subjects and institutions.  相似文献   
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This study investigated the effect of the AMPA receptor antagonist 6,7-dinitroquinoxaline-2,3-dione (DNQX) microinjected into the core and shell sub-regions of the accumbens nucleus (Acb), on the level of fear/anxiety and emotional learning, in female rats submitted to the elevated plus-maze (EPM), an animal model of anxiety. Bilateral microinjections of DNQX (330 and 660 ng) into the Acb shell (AP, +1.08 to +2.16) induced an anxiolytic-like effect in relation to rats microinjected with vehicle, since there was an increased percentage of entries in the open arms of the maze. The 660 ng DNQX microinjection into the Acb shell also increased the percentage of entries into the open arms in relation to 660 ng DNQX microinjection into the Acb core. Prior DNQX microinjections in both core and shell sub-regions of the Acb failed to impair the emotional learning, since the animals exhibited an increase of the open arm avoidance on EPM Trial 2 in relation to EPM trial 1. DNQX microinjections into both sub-regions of the Acb did not change the number of entries into the enclosed arms, either in the EPM Trial 1 or in the EPM Trial 2, which indicates an absence of drug-induced locomotor impairment. Similarly, DNQX microinjections into both sub-regions of the Acb failed to alter the total arm entries, rearing, grooming and head-dipping frequency. The anxiolytic-like effect induced by DNQX suggests that the AMPA receptor in the Acb shell, but not in the Acb core, may underlie anxiety regulation in the EPM.  相似文献   
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This study was aimed to evaluate splenomegaly in patients with the hepatosplenic (HS) form of mansonic schistosomiasis (MS), analyzing the size and weight of the spleen and their relationships with patients' gender and age. Between October, 1993 to July, 1998, 78 patients with the HS form of MS had undergone splenectomy as treatment of choice for bleeding due to portal hypertension, at Hospital das Clínicas, Pernambuco, Brazil. By means of abdominal palpation, the excess spleen felt below the left costal edge was measured, and the weight was obtained after splenectomy along with the histopathological analysis. Liver biopsy was performed intraoperatively in order to confirm MS and to rule out other liver diseases. The mean age of the 78 patients were 45 years and 41 of them (53%) were female. The average spleen weight was 912g and the mean spleen size palpable below the left costal edge was 9.1cm. There was a positive relationship between size and weight (p<0.001). Spleen weight and size were larger in males (p=0.007 and p=0.001, respectively). An inverse correlation between age and spleen weight was observed (p<0.001). A classification based upon spleen weight showed 53% of patients presenting a moderate (501-1000g) and 33% a severe (>1001g) splenomegaly. As for the spleen size, the classification showed 64% of patients presenting moderate (4.1-10cm below the left costal edge) and 21% severe (>10cm) splenomegaly. In conclusion, splenomegaly may be considered a key physical finding in patients with HS form of MS, and we found a good correlation between the spleen sizes clinically evaluated with its weight. The majority of cases presents a moderate to severe splenomegaly and spleen size is larger in men and it seems to decrease with aging.  相似文献   
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The ongoing movement from institutional care to community-orientated care has gradually spread around the developed world. This shift in the philosophy of care has resulted in a reduction in the length of inpatient admissions and contributed to a decline in the number of inpatient beds. This literature review seeks to establish the suitability and relevance of solution-focused brief therapy (SFBT) to mental health nursing practice, with particular emphasis being given to acute inpatient settings. Databases searched for published material in English between 1980 and 2006 were: Cumulative Index of Nursing and Allied Health Literature; MEDLINE; Applied Social Sciences Index and Abstracts; Sociological abstracts; and social service abstracts. Search terms included: SFBT and solution-focused approaches. Only nine papers from the original 203 citations met the inclusion criteria and were thus reviewed. These papers indicated that SFBT constitutes an appropriate set of techniques for use in mental health nursing particularly where staff have relatively brief contact with patients. Preliminary data suggest that SFBT is congruent with the philosophical underpinning of contemporary mental health nursing. However, its clinical utility and effectiveness is not well established and methodologically rigorous studies are urgently needed to determine the appropriateness and effectiveness of such approach to nursing practice.  相似文献   
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