全文获取类型
收费全文 | 2756篇 |
免费 | 167篇 |
国内免费 | 5篇 |
专业分类
耳鼻咽喉 | 13篇 |
儿科学 | 101篇 |
妇产科学 | 42篇 |
基础医学 | 441篇 |
口腔科学 | 25篇 |
临床医学 | 269篇 |
内科学 | 452篇 |
皮肤病学 | 78篇 |
神经病学 | 366篇 |
特种医学 | 100篇 |
外科学 | 196篇 |
综合类 | 6篇 |
预防医学 | 247篇 |
眼科学 | 63篇 |
药学 | 241篇 |
中国医学 | 9篇 |
肿瘤学 | 279篇 |
出版年
2023年 | 15篇 |
2022年 | 35篇 |
2021年 | 63篇 |
2020年 | 55篇 |
2019年 | 46篇 |
2018年 | 72篇 |
2017年 | 50篇 |
2016年 | 65篇 |
2015年 | 74篇 |
2014年 | 116篇 |
2013年 | 128篇 |
2012年 | 193篇 |
2011年 | 208篇 |
2010年 | 164篇 |
2009年 | 129篇 |
2008年 | 175篇 |
2007年 | 188篇 |
2006年 | 216篇 |
2005年 | 167篇 |
2004年 | 142篇 |
2003年 | 151篇 |
2002年 | 150篇 |
2001年 | 22篇 |
2000年 | 22篇 |
1999年 | 19篇 |
1998年 | 29篇 |
1997年 | 36篇 |
1996年 | 20篇 |
1995年 | 22篇 |
1994年 | 14篇 |
1993年 | 11篇 |
1992年 | 9篇 |
1991年 | 12篇 |
1990年 | 9篇 |
1989年 | 8篇 |
1988年 | 5篇 |
1987年 | 6篇 |
1986年 | 5篇 |
1985年 | 4篇 |
1984年 | 9篇 |
1983年 | 4篇 |
1982年 | 8篇 |
1981年 | 4篇 |
1980年 | 8篇 |
1978年 | 5篇 |
1977年 | 6篇 |
1976年 | 3篇 |
1975年 | 3篇 |
1972年 | 3篇 |
1962年 | 2篇 |
排序方式: 共有2928条查询结果,搜索用时 343 毫秒
61.
62.
Pawel Buczkowicz Ute Bartels Eric Bouffet Oren Becher Cynthia Hawkins 《Acta neuropathologica》2014,128(4):573-581
Diffuse intrinsic pontine glioma (DIPG) is the main cause of brain tumour-related death in children. In the majority of cases diagnosis is based on clinical and MRI findings, resulting in the scarcity of pre-treatment specimens available to study. Our group has developed an autopsy-based protocol to investigate the histologic and biologic spectrum of DIPG. This has also allowed us to investigate the terminal pattern of disease and gain a better understanding of what challenges we are facing in treating DIPG. Here, we review 72 DIPG cases with well documented clinical history and molecular data and describe the pathological features of this disease in relation to clinical and genetic features. Fifty-three of the samples were autopsy material (7 pre-treatment) and 19 were pre-treatment biopsy/surgical specimens. Upon histological review, 62 patients had high-grade astrocytomas (18 WHO grade III and 44 WHO grade IV patients), 8 had WHO grade II astrocytomas, and 2 had features of primitive neuroectodermal tumour (PNET). K27M-H3 mutations were exclusively found in tumours with WHO grade II–IV astrocytoma histology. K27M-H3.1 and ACVR1 mutations as well as ALT phenotype were only found in WHO grade III–IV astrocytomas, while PIK3CA mutations and PDGFRA gains/amplifications were found in WHO grade II–IV astrocytomas. Approximately 1/3 of DIPG patients had leptomeningeal spread of their tumour. Further, diffuse invasion of the brainstem, spinal cord and thalamus was common with some cases showing spread as distant as the frontal lobes. These findings suggest that focal radiation may be inadequate for some of these patients. Importantly, we show that clinically classic DIPGs represent a diverse histologic spectrum, including multiple cases which would fit WHO criteria of grade II astrocytoma which nevertheless behave clinically as high-grade astrocytomas and harbour the histone K27M-H3.3 mutation. This suggests that the current WHO astrocytoma grading scheme may not appropriately predict outcome for paediatric brainstem gliomas. 相似文献
63.
64.
65.
Thimm E Richter-Werkle R Kamp G Molke B Herebian D Klee D Mayatepek E Spiekerkoetter U 《Journal of inherited metabolic disease》2012,35(2):263-268
Objective
The implementation of NTBC into treatment of hypertyrosinemia type I (HT I) greatly improved survival by prevention of acute liver failure and hepatocellular carcinoma. However, there are first reports of cognitive impairment in patients with elevated plasma tyrosine concentrations. 相似文献66.
Hoffmann L Haussmann U Mueller M Spiekerkoetter U 《Journal of inherited metabolic disease》2012,35(2):269-277
Tandem mass spectrometry-based newborn screening correctly identifies individuals with very long-chain acyl-CoA dehydrogenase
deficiency (VLCADD). However, a great number of healthy individuals present with identical acylcarnitine profiles during catabolism
in the first three days of life. We routinely perform an enzyme activity assay as confirmation analysis in newborns identified
by screening. Whereas VLCAD residual activities of less than 10% are clearly diagnostic and indicate patients at risk of clinical
disease, the clinical relevance of higher residual activities is unclear. In this study we assess the molecular basis in 34
individuals with residual activities of 10-50%. We identify two pathogenic mutations in patients that result in residual activities
as high as 22%, while individuals with residual activities of 25-50% either present with a heterozygous or no mutation in
the VLCAD gene. In addition, confirmed heterozygous parents present with residual activities as low as 32%. 相似文献
67.
68.
Purpose
Capsule endoscopy (CE) is a very useful tool for the evaluation of the small intestine, but it is time consuming. The aim of this study was to compare evaluation times and detection rates in two different reading modes (single view at a speed of 10 frames per second (fps) and four images simultaneously, i.e., quadview mode at a speed of 20 fps) to find the optimum setting mode for evaluation of CE videos. 相似文献69.
Nevzorova YA Bangen JM Hu W Haas U Weiskirchen R Gassler N Huss S Tacke F Sicinski P Trautwein C Liedtke C 《Hepatology (Baltimore, Md.)》2012,56(3):1140-1149
Liver fibrogenesis is associated with the transition of quiescent hepatocytes and hepatic stellate cells (HSCs) into the cell cycle. Exit from quiescence is controlled by E-type cyclins (cyclin E1 [CcnE1] and cyclin E2 [CcnE2]). Thus, the aim of the current study was to investigate the contribution of E-type cyclins for liver fibrosis in man and mice. Expression of CcnE1, but not of its homolog, CcnE2, was induced in fibrotic and cirrhotic livers from human patients with different etiologies and in murine wild-type (WT) livers after periodical administration of the profibrotic toxin, CCl(4) . To further evaluate the potential function of E-type cyclins for liver fibrogenesis, we repetitively treated constitutive CcnE1(-/-) and CcnE2(-/-) knock-out mice with CCl(4) to induce liver fibrosis. Interestingly, CcnE1(-/-) mice were protected against CCl(4) -mediated liver fibrogenesis, as evidenced by reduced collagen type I α1 expression and the lack of septum formation. In contrast, CcnE2(-/-) mice showed accelerated fibrogenesis after CCl(4) treatment. We isolated primary HSCs from WT, CcnE1(-/-) , and CcnE2(-/-) mice and analyzed their activation, proliferation, and survival in vitro. CcnE1 expression in WT HSCs was maximal when they started to proliferate, but decreased after the cells transdifferentiated into myofibroblasts. CcnE1(-/-) HSCs showed dramatically impaired survival, cell-cycle arrest, and strongly reduced expression of alpha smooth muscle actin, indicating deficient HSC activation. In contrast, CcnE2-deficient HSCs expressed an elevated level of CcnE1 and showed enhanced cell-cycle activity and proliferation, compared to WT cells. Conclusions: CcnE1 and CcnE2 have antagonistic roles in liver fibrosis. CcnE1 is indispensable for the activation, proliferation, and survival of HSCs and thus promotes the synthesis of extracellular matrix and liver fibrogenesis. (HEPATOLOGY 2012;56:1140-1149). 相似文献
70.
Sebastian J. Buss Mostafa Emami Derliz Mereles Grigorios Korosoglou Arnt V. Kristen Andreas Voss Dieter Schellberg Christian Zugck Christian Galuschky Evangelos Giannitsis Ute Hegenbart Anthony D. Ho Hugo A. Katus Stefan O. Schonland Stefan E. Hardt 《Journal of the American College of Cardiology》2012