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731.
Mohamed A Soliman Hamad Albert HM van Straten Jacques PAM Schönberger Joost F ter Woorst Andre M de Wolf Elisabeth J Martens André AJ van Zundert 《Journal of cardiothoracic surgery》2010,5(1):29
Background
Preoperative left ventricular dysfunction is an established risk factor for early and late mortality after revascularization. This retrospective analysis demonstrates the effects of preoperative ejection fraction on the short-term and long-term survival of patients after coronary artery bypass grafting. 相似文献732.
Solberg BC Dirksen CD Nieman FH van Merode G Poeze M Ramsay G 《Critical care (London, England)》2008,12(3):R68
Introduction
The high cost of critical care resources has resulted in strategies to reduce the costs of ruling out low-risk patients by developing intermediate care units (IMCs). The aim of this study was to compare changes in total hospital costs for intensive care patients before and after the introduction of an IMC at the University Hospital Maastricht. 相似文献733.
The human genome is constantly exposed to various sources of DNA damage. Ineffective protection from this damage leads to genetic instability which can ultimately give rise to somatic disease, causing mutations. Therefore our organism commands a number of highly conserved and effective mechanisms responsible for DNA repair. If these repair mechanisms are defective due to germline mutations in relevant genes, rare diseases with DNA repair deficiencies can arise. Today, a limited number of rare hereditary diseases characterized by genetic defects of DNA repair mechanisms is known, comprising ataxia telangiectasia, Nijmegen breakage syndrome, Werner syndrome, Bloom Syndrome, Fanconi anemia, xeroderma pigmentosum, Cockayne syndrome, trichothiodystrophy. Although heterogeneous in respect to selected symptoms, these rare disorders share many clinical features such as growth retardation, neurological disorders, premature ageing, skin alterations including abnormal pigmentation, telangiectasia, xerosis cutis, pathological wound healing as well as an increased risk of developing different types of cancer. Based on the clinical similarities of symptoms as well as the predominant diagnostic technology available, many of these rare disorders were formerly classified as genodermatoses with cancer predisposition or chromosomal breakage symptoms. These pathological conditions not only severely impair patients with these rare genetic diseases but also represent symptoms affecting large parts of the general population. 相似文献
734.
Hanekamp MN Mazer P van der Cammen-van Zijp MH van Kessel-Feddema BJ Nijhuis-van der Sanden MW Knuijt S Zegers-Verstraeten JL Gischler SJ Tibboel D Kollée LA 《Critical care (London, England)》2006,10(5):R127-11
Introduction
Extracorporeal membrane oxygenation (ECMO) is a supportive cardiopulmonary bypass technique for babies with acute reversible cardiorespiratory failure. We assessed morbidity in ECMO survivors at the age of five years, when they start primary school and major decisions for their school careers must be made. 相似文献735.
Meliha Karsak Konstantin Glebov Marina Scheffold Thomas Bajaj Amit Kawalia Ilker Karaca Sebastian Rading Johannes Kornhuber Oliver Peters Monica Diez‐Fairen Lutz Frlich Michael Hüll Jens Wiltfang Martin Scherer Steffi Riedel‐Heller Anja Schneider Michael T. Heneka Klaus Fliessbach Ahmed Sharaf Holger Thiele Martina Lennarz Frank Jessen Wolfgang Maier Christian Kubisch Zoya Ignatova Peter Nürnberg Pau Pastor Jochen Walter Alfredo Ramirez 《Human mutation》2020,41(1):169-181
Rare coding variants in the triggering receptor expressed on myeloid cells‐2 (TREM2) gene have been associated with Alzheimer disease (AD) and homozygous TREM2 loss‐of‐function variants have been reported in families with monogenic frontotemporal‐like dementia with/without bone abnormalities. In a whole‐exome sequencing study of a family with probable AD‐type dementia without pathogenic variants in known autosomal dominant dementia disease genes and negative for the apolipoprotein E (APOE) ε4 allele, we identified an extremely rare TREM2 coding variant, that is, a glycine‐to‐tryptophan substitution at amino acid position 145 (NM_018965.3:c.433G>T/p.[Gly145Trp]). This alteration is found in only 1 of 251,150 control alleles in gnomAD. It was present in both severely affected as well as in another putatively affected and one 61 years old as yet unaffected family member suggesting incomplete penetrance and/or a variable age of onset. Gly145 maps to an intrinsically disordered region (IDR) of TREM2 between the immunoglobulin‐like and transmembrane domain. Subsequent cellular studies showed that the variant led to IDR shortening and structural changes of the mutant protein resulting in an impairment of cellular responses upon receptor activation. Our results, suggest that a p.(Gly145Trp)‐induced structural disturbance and functional impairment of TREM2 may contribute to the pathogenesis of an AD‐like form of dementia. 相似文献
736.
他汀类药物是否会增加糖尿病发病风险,一直备受争论。本研究根据已发表和未发表的文献资料,用Meta方法分析该类药物使用与糖尿病发生的相关性。 相似文献
737.
Marjolein Garsen Ramon Sonneveld Angelique LWMM Rops Suzanne Huntink Toin H van Kuppevelt Ton J Rabelink Joost GJ Hoenderop Jo HM Berden Tom Nijenhuis Johan van der Vlag 《The Journal of pathology》2015,237(4):472-481
The glomerular filtration barrier consists of podocytes, the glomerular basement membrane, and endothelial cells covered with a glycocalyx. Heparan sulphate (HS) in the glomerular filtration barrier is reduced in patients with proteinuria, which is associated with increased expression of the HS‐degrading enzyme heparanase. Previously, we showed that heparanase is essential for the development of proteinuria in experimental diabetic nephropathy. Vitamin D supplementation reduces podocyte loss and proteinuria in vitro and in vivo. Therefore, we hypothesize that vitamin D reduces proteinuria by reducing glomerular heparanase. Adriamycin‐exposed rats developed proteinuria and showed increased heparanase expression, which was reduced by 1,25‐dihydroxyvitamin D3 (1,25‐D3) treatment. In vitro, adriamycin increased heparanase mRNA in the podocyte, which could be corrected by 1,25‐D3 treatment. In addition, 1,25‐D3 treatment reduced transendothelial albumin passage after adriamycin stimulation. In line with these results, we showed direct binding of the vitamin D receptor to the heparanase promoter, and 1,25‐D3 dose‐dependently reduced heparanase promoter activity. Finally, 1,25‐D3‐deficient 25‐hydroxy‐1α‐hydroxylase knockout mice developed proteinuria and showed increased heparanase, which was normalized by 1,25‐D3 treatment. Our data suggest that the protective effect of vitamin D on the development of proteinuria is mediated by inhibiting heparanase expression in the podocyte. Copyright © 2015 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. 相似文献
738.
Davor Lessel Katrin Rading Susan E. Campbell Holger Thiele Janine Altmüller Leslie B. Gordon Christian Kubisch 《American journal of medical genetics. Part A》2022,188(1):216-223
Pathogenic biallelic variants in POL3RA have been associated with different disorders characterized by progressive neurological deterioration. These include the 4H leukodystrophy syndrome (hypomyelination, hypogonadotropic hypogonadism, and hypodontia) and adolescent-onset progressive spastic ataxia, as well as Wiedemann–Rautenstrauch syndrome (WRS), a recognizable neonatal progeroid syndrome. The phenotypic differences between these disorders are thought to occur mainly due to different functional effects of underlying POLR3A variants. Here we present the detailed clinical course of a 37-year-old woman in whom we identified a homozygous synonymous POLR3A variant c.3336G>A resulting in leaky splicing r.[3336ins192, =, 3243_3336del94]. She presented at birth with intrauterine growth retardation, lipodystrophy, muscular hypotonia, and several WRS-like facial features, albeit without sparse hair and prominent scalp veins. She had no signs of developmental delay or intellectual disability. Over the years, above characteristic facial features, she showed severe postnatal growth retardation, global lipodystrophy, joint contractures, thoracic hypoplasia, scoliosis, anodontia, spastic quadriplegia, bilateral hearing loss, aphonia, hypogonadotropic hypogonadism, and cerebellar peduncles hyperintensities in brain imaging. These manifestations partially overlap the clinical features of the previously reported POLR3A-associated disorders, mostly mimicking the WRS. Thus, our study expands the POLR3A-mediated phenotypic spectrum and suggests existence of a phenotypic continuum underlying biallelic POLR3A variants. 相似文献