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排序方式: 共有5128条查询结果,搜索用时 31 毫秒
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AIMS: To investigate the serum creatine kinase isoenzyme pattern, specific biochemical markers of bone metabolism, and cytokines in a Chinese family with osteopetrosis, and correlate abnormalities with the pathophysiology of this condition. METHODS: A Chinese female baby was diagnosed with malignant infantile osteopetrosis at the age of 3 weeks by clinical history and biochemical investigations. We studied the laboratory and radiological manifestations of this index case and her family members. RESULTS: Serum CK-BB fraction of our index patient was elevated to 18.0% (normal 1.6-7.6%). Her biochemical markers of bone resorption including serum C-terminal telopeptide concentration and urine N-terminal telopeptide to creatinine ratio were decreased to 0.54 microg/L (normal 0.72-1.56 microg/L) and 159 x 10(-6) (normal 372-900 x 10(-6)), respectively. Serum cytokines including soluble receptor activator of nuclear factor kappa-B ligand (sRANKL) concentration was suppressed to 0.11 pmol/L (normal 0.23-0.82 pmol/L) and osteoprotegerin (OPG) concentration was 4.9 pmol/L (normal 2.8-4.9 pmol/L), resulting in an elevated OPG to sRANKL ratio of 44.5 (normal 3.8-19.4) in favour of bone formation. CONCLUSIONS: If left untreated, this condition is usually fatal within the first year of life. With early diagnosis, management including bone marrow transplantation can be planned ahead and will result in a better survival. 相似文献
33.
Müller AM Hermanns MI Skrzynski C Nesslinger M Müller KM Kirkpatrick CJ 《Experimental and molecular pathology》2002,72(3):221-229
EC culture models are essential to study pathological alterations of endothelial cells (ECs) in pulmonary vascular diseases under standardized conditions. Nevertheless, little is known about the spectrum of alterations of vessel-specific endothelial phenotypes in monolayer cultures. For the comparative study of endothelial markers in vivo and in vitro we investigated immunohistochemically the expression of PECAM-1, vWf, and CD34 by pulmonary ECs in vivo and in stimulated/unstimulated human umbilical vein endothelial cells (HU-VEC) and human pulmonary microvascular endothelial cells (HPMEC). In vivo, vessel type-specific expression patterns were found for vWf and CD34, while PECAM-1 was homogeneously and strongly expressed. While all HUVEC showed a marked vWf staining, about two-thirds of HPMEC exhibited a strong and the rest a moderate vWf staining. In both in vitro models all ECs were clearly PECAM-1-positive. However, only about 20% of the HUVEC and HPMEC were CD34-positive. Our results demonstrate the reduced expression of vessel type-specific endothelial phenotypes by endothelial monolayer cultures, stressing the need to improve culture conditions as well as develop cocultures and three-dimensional culture models. Moreover, the need for endothelial markers specific for single microvascular type ECs becomes obvious in order to establish cultures consisting of only one microvascular ECs subpopulation. 相似文献
34.
Individual differences in cognitive reappraisal: experiential and physiological responses to an anger provocation. 总被引:2,自引:0,他引:2
Iris B Mauss Crystal L Cook Jennifer Y J Cheng James J Gross 《International journal of psychophysiology》2007,66(2):116-124
Effective emotion regulation is widely seen as vital for healthy adaptation. There remains considerable uncertainty, however, as to what constitutes effective emotion regulation. One promising emotion regulation strategy is cognitive reappraisal, which involves reframing emotional events so as to decrease their emotional impact. This strategy is useful because it seems to enable individuals to down-regulate negative feelings without the physiological costs that are associated with other forms of emotion regulation. It remains unknown, however, whether individual differences in the use of reappraisal are associated with experiential and physiological responses to anger-inducing situations. To examine this question, individuals either high or low in reappraisal were made angry in the laboratory while emotion experience and cardiovascular responses were assessed. Results indicated that compared to low reappraisers, high reappraisers had a more adaptive profile of emotion experience and cardiovascular responding. Specifically, across baseline and provocation periods, high reappraisers reported less anger, less negative emotion, and more positive emotion, showed greater cardiac output and ventricular contractility, and lesser total peripheral resistance. These findings suggest that reappraisers are successful at down-regulating negative emotions, even in the context of a potent negative emotion such as anger. 相似文献
35.
Roberto Della Bruna Iris Bernhard Bernhard Gess Karin Schricker Armin Kurtz 《Pflügers Archiv : European journal of physiology》1995,430(2):265-272
This study aimed to investigate the inter-relation between the angiotensin II (ANG II) AT1 receptor and renin gene expression in rat kidneys. To this end, renin mRNA levels and mRNA levels for AT1a and AT1b were assayed by RNase protection in the kidneys of normal rats, in animals treated with the AT1 antagonist losartan and in rats bearing 0.2-mm left renal artery clips for 2 days. In normal rats, we found a negative correlation between renin mRNA levels and AT1a receptor mRNA levels. Losartan led to a fourfold increase in renin mRNA levels without changing AT1 receptor mRNA levels. Unilateral renal artery clipping increased renin mRNA levels fourfold in the clipped kidney and suppressed renin mRNA levels in the contralateral kidneys. AT1 receptor mRNA levels were not changed in the contralateral intact kidneys, but were significantly decreased by 15–25% in the clipped kidneys. Renin mRNA levels were inversely correlated to AT1a mRNA levels in the clipped, but not in the contralateral, kidneys. Our findings suggest that the systemic activity of the renin angiotensin system has no regulatory influence on renal AT1 receptor gene expression. Renin mRNA levels in normal and in clipped kidneys appear to be negatively determined by the level of AT1a receptor gene expression. Thus modulation of AT1a receptor gene expression could be a pathway for indirect modulation of renin gene expression by ANG II. This conclusion is in agreement with the observation that AT1 receptor antagonists are powerful stimulators of the renin system. 相似文献
36.
Mariappan MR Zehnder J Arber DA Lay M Fadare O Schrijver I 《International journal of surgical pathology》2005,13(3):253-258
Specimen misidentification is a common cause of errors in surgical pathology. We report a case where bone-marrow biopsies from patients of different genders were mislabeled and molecular methods were applied to resolve the identity. A short tandem repeat (STR)-polymerase chain reaction-based assay, commonly used in paternity testing, was employed in an attempt to assign the correct identity to the specimens. However, the specimens had been processed by decalcification and the DNA yield was poor. One of the markers in the assay is the non-STR amelogenin locus that distinguishes the X and Y chromosomes. This amelogenin marker results in a product of low molecular weight, enabling unequivocal resolution of identity despite a poor DNA yield. The prevalence of errors in pathology due to specimen misidentifications is reviewed. 相似文献
37.
Peripheral Tolerance as a Multi-Step Mechanism 总被引:10,自引:0,他引:10
Günter J. Hämmerling Günther Schönrich Iris Ferber Bernd Arnold 《Immunological reviews》1993,133(1):93-104
38.
Tristan Barnes Preston Parry Iris Hart Carol Jones Michele Minet David Patterson 《Somatic Cell and Molecular Genetics》1993,19(4):405-411
De novo UMP synthesis is a critical metabolic pathway for nucleic acid synthesis and for a variety of metabolic pathways. The pathway is a target for many widely used cancer chemotherapy agents, several of which are pyrimidine analogs. Humans and cattle have been described with mutations in UMP synthesis that lead to serious inborn errors of metabolism. Dihydroorotate dehydrogenase (EC 1.3.3.1) (DHODH) carries out the fourth committed step in the pathway and may also be important for mitochondrial electron transport and oxygen radical metabolism. We report here that the gene encoding this enzyme in humans is located in the chromosomal region 16q22. With the mapping of DHODH, the mapping of all the steps of UMP synthesis is complete. All three genes involved map to different human chromosomes. This information is important in consideration of regulation of UMP synthesis in mammals, including humans. 相似文献
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