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991.
V. Gouilleux‐Gruart H. Chapel S. Chevret M. Lucas M. Malphettes C. Fieschi S. Patel D. Boutboul M.‐N. Marson L. Gérard M. Lee H. Watier E. Oksenhendler DEFI study group 《Clinical and experimental immunology》2013,171(2):186-194
Treatment of common variable immunodeficiency disorders (CVID) is based on replacement therapy using intravenous (i.v.) or subcutaneous (s.c.) immunoglobulin (Ig)G. Interindividual variation of IgG dose is common. A total of 380 CVID patients on stable IgG replacement from two prospective cohorts were analysed. An ‘efficiency’ index was defined as the ratio of serum IgG trough level minus IgG residual to the average weekly dose of IgG infusion. A reduced efficiency of IgG was associated independently with the i.v. route (P < 0·001) and with the presence of at least one CVID disease‐related phenotype (lymphoproliferation, autoimmune cytopenia or enteropathy) (P < 0·001). High IgG efficiency was noted in patients homozygotes for the variable number tandem repeat (VNTR) 3/3 polymorphism of the neonatal Fc receptor gene [IgG Fc fragment receptor transporter alpha chain (FCGRT)] promoter, and this was particularly significant in patients treated with IVIG (P < 0.01). In a multivariate analysis, FCGRT VNTR 3/3 genotype (P = 0·008) and high serum albumin (P < 0·001) were associated independently with increased efficiency of i.v. Ig. 相似文献
992.
J.‐Q. Yang P. J. Kim R. C. Halder R. R. Singh 《Clinical and experimental immunology》2013,173(1):18-27
Patients with systemic lupus erythematosus (SLE) display reduced numbers and functions of invariant natural killer T (iNK T) cells, which are restored upon treatment with corticosteroids and rituximab. It is unclear whether the iNK T cell insufficiency is a consequence of disease or is a primary abnormality that precedes the onset of disease. To address this, we analysed iNK T cell function at different stages of disease development using the genetically lupus‐susceptible NZB × NZW F1 (BWF1) model. We found that iNK T cell in‐vivo cytokine responses to an iNK T cell ligand α‐galactosylceramide (α‐GalCer) were lower in BWF1 mice than in non‐autoimmune BALB/c and major histocompatibility complex (MHC)‐matched NZB × N/B10.PL F1 mice, although iNK T cell numbers in the periphery were unchanged in BWF1 mice compared to control mice. Such iNK T cell hyporesponsiveness in BWF1 mice was detected at a young age long before the animals exhibited any sign of autoimmunity. In‐vivo activation of iNK T cells is known to transactivate other immune cells. Such transactivated T and B cell activation markers and/or cytokine responses were also lower in BWF1 mice than in BALB/c controls. Finally, we show that iNK T cell responses were markedly deficient in the NZB parent but not in NZW parent of BWF1 mice, suggesting that BWF1 might inherit the iNK T cell defect from NZB mice. Thus, iNK T cells are functionally insufficient in lupus‐prone BWF1 mice. Such iNK T cell insufficiency precedes the onset of disease and may play a pathogenic role during early stages of disease development in SLE. 相似文献
993.
A. S. W. Tjon T. Tha‐In H. J. Metselaar R. van Gent L. J. W. van der Laan Z. M. A. Groothuismink P. A. W. te Boekhorst P. M. van Hagen J. Kwekkeboom 《Clinical and experimental immunology》2013,173(2):259-267
Intravenous immunoglobulin (IVIg) is used to treat autoimmune and systemic inflammatory diseases caused by derailment of humoral and cellular immunity. In this study we investigated whether IVIg treatment can modulate regulatory T cells (Tregs) in humans in vivo. Blood was collected from IVIg-treated patients with immunodeficiency or autoimmune disease who were treated with low-dose (n = 12) or high-dose (n = 15) IVIg before, immediately after and at 7 days after treatment. Percentages and activation status of circulating CD4+CD25+forkhead box protein 3 (FoxP3+) Tregs and of conventional CD4+FoxP3− T-helper cells (Tconv) were measured. The suppressive capacity of Tregs purified from blood collected at the time-points indicated was determined in an ex-vivo assay. High-dose, but not low-dose, IVIg treatment enhanced the activation status of circulating Tregs, as shown by increased FoxP3 and human leucocyte antigen D-related (HLA-DR) expression, while numbers of circulating Tregs remained unchanged. The enhanced activation was sustained for at least 7 days after infusion, and the suppressive capacity of purified Tregs was increased from 41 to 70% at day 7 after IVIg treatment. The activation status of Tconv was not affected by IVIg. We conclude that high-dose IVIg treatment activates Tregs selectively and enhances their suppressive function in humans in vivo. This effect may be one of the mechanisms by which IVIg restores imbalanced immune homeostasis in patients with autoimmune and systemic inflammatory disorders. 相似文献
994.
Y Alade D Tunkel K Schulze J McGready G Jallo M Ain T Yost J Hoover‐Fong 《Clinical genetics》2013,84(3):237-243
Short stature skeletal dysplasia (SD) patients have orthopedic and neurologic complications causing significant pain and physical disability. We conducted a large cross‐sectional online survey in 361 people with short stature SD (>10 years) to describe pain prevalence, characteristics, and the relationship between pain and function. Chronic pain prevalence per Brief Pain Inventory (BPI) was 70.3%. Women reported more pain than men (73% vs 63% p = 0.04). Pain Severity Score (average of current, worst, least and average pain) averaged 3.3 ± 2, while the Pain Interference Score (with daily activities) averaged 3.4 ± 2.7 on a 10‐point scale. Per Bleck scale, 20.5% had little or no functional capacity. Increasing age and decreased ambulation independently predicted chronic pain. Chronic pain is prevalent in short stature SD patients and associated with poor physical function. Further study is required to clarify the temporal relationship among pain, function and treatments. 相似文献
995.
996.
Mohammad Abd Alkhalik Basha Hossam M. Abdelrahman Maha Ibrahime Metwally Nader Ali Alayouty Nesreen Mohey Mohamed M.A. Zaitoun Hosam Nabil Almassry Hala Y. Yousef Ahmed A. El Sammak Sameh Abdelaziz Aly Hesham Youssef Algazzar Mohamed Abd El‐Aziz Mohamed Farag Walid Mosallam Waleed S. Abo Shanab Safaa A. Ibrahim Ekramy A. Mohamed Abd El Motaleb Mohamed Amira Hamed Mohamed Afifi Ola A. Harb Taghreed M. Azmy 《Journal of magnetic resonance imaging : JMRI》2021,53(1):292-304
997.
Michaud Katarzyna Magnin Virginie Faouzi Mohamed Fracasso Tony Aguiar Diego Dedouit Fabrice Grabherr Silke 《International journal of legal medicine》2021,135(5):1829-1836
International Journal of Legal Medicine - Sudden cardiac death (SCD) related to atherosclerotic coronary artery disease (ACAD) resulting in myocardial infarction is the most prevalent cause of... 相似文献
998.
Thomas Helbing Eva‐Maria Herold Alexandra Hornstein Stefanie Wintrich Jennifer Heinke Sebastian Grundmann Cam Patterson Christoph Bode Martin Moser 《The Journal of pathology》2013,231(1):105-116
Epithelial injury is a central finding in pulmonary disease and is accompanied by disruption of epithelial barrier function, leading to pulmonary oedema and inflammation. Injured epithelial cells lose their properties and gain mesenchymal characteristics, a phenotypic switch that contributes to lung remodelling after injury. Here we studied bone morphogenetic protein (BMP) signalling and, in particular, the role of BMP2 and the BMP modulator BMPER in injured lung epithelium. Increased BMP activity, reflected by up‐regulation of the Smad1/5–Id1 axis, is detected after injury of lung epithelium in vitro and in vivo. Two members of the BMP family, BMP2 and BMPER, have opposing effects. BMP2 is up‐regulated after epithelial injury and causes epithelial dysfunction and hyperpermeability, mediated by the Smad1/5–Id1‐dependent down‐regulation of E‐cadherin. In contrast, BMPER expression is decreased following injury, which in turn impairs epithelial integrity, characterized by reduction of E‐cadherin and epithelial leakage in vitro and in vivo. High levels of BMPER antagonized BMP2‐Smad5–Id1 signalling and prevented BMP2‐mediated decrease of E‐cadherin and hyperpermeability, suggesting that BMPER restores epithelial homeostasis. Supporting this notion, pharmacological inhibition of BMP signalling by LDN193189 prevented reduction of E‐cadherin and disruption of epithelial barrier function. Inhibition of excessive BMP activation could be a new approach to restore epithelial integrity and prevent disruption of epithelial barrier function after lung injury. Copyright © 2013 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. 相似文献
999.
Axonal outgrowth is a fundamental process during the development of central (CNS) and peripheral (PNS) nervous system as well as in nerve regeneration and requires accurate axonal navigation and extension to the correct target. These events need proper coordination between membrane trafficking and cytoskeletal rearrangements and are under the control of the small GTPases of the Rho family, among other molecules. Reelin, a relevant protein for CNS development and synaptic function in the adult, is also present in the PNS. Upon sciatic nerve damage, Reelin expression increases and, on the other hand, mice deficient in Reelin exhibit an impaired nerve regeneration. However, the mechanism(s) involved the Reelin‐dependent axonal growth is still poorly understood. In this work, we present evidence showing that Reelin stimulates dorsal root ganglia (DRG) regeneration after axotomy. Moreover, dissociated DRG neurons express the Reelin receptor Apolipoprotein E‐receptor 2 and also require the presence of TC10 to develop their axons. TC10 is a Rho GTPase that promotes neurite outgrowth through the exocytic fusion of vesicles at the growth cone. Here, we demonstrate for the first time that Reelin controls TC10 activation in DRG neurons. Besides, we confirmed that the known CNS Reelin target Cdc42 is also activated in DRG and controls TC10 activity. Finally, in the process of membrane addition, we found that Reelin stimulates the fusion of membrane carriers containing the v‐SNARE protein VAMP7 in vesicles that contain TC10. Altogether, our work shows a new role of Reelin in PNS, opening the option of therapeutic interventions to improve the regeneration process. 相似文献
1000.
Ane Murueta‐Goyena Rocío Del Pino Marta Galds Begoa Arana Marian Acera Mar Carmona‐Abelln Tamara Fernndez‐Valle Beatriz Tijero Olaia Lucas‐Jimnez Natalia Ojeda Naroa Ibarretxe‐Bilbao Javier Pea Jesus Cortes Unai Ayala Maitane Barrenechea Juan Carlos Gmez‐Esteban Iigo Gabilondo 《Annals of neurology》2021,89(1):165-176