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排序方式: 共有9096条查询结果,搜索用时 31 毫秒
961.
Lilian Lacourpaille MSc François Hug PhD Arnaud Guével PhD Yann Péréon MD PhD Armelle Magot MD Jean‐Yves Hogrel PhD Antoine Nordez PhD 《Muscle & nerve》2015,51(2):284-286
ABSTRACT: Introduction: Assessment of muscle mechanical properties may provide clinically valuable information for follow‐up of patients with Duchenne muscular dystrophy (DMD) through the course of their disease. In this study we aimed to assess the effect of DMD on stiffness of relaxed muscles using elastography (supersonic shear imaging). Methods: Fourteen DMD patients and 13 control subjects were studied. Six muscles were measured at 2 muscle lengths (shortened and stretched): gastrocnemius medialis (GM); tibialis anterior (TA); vastus lateralis (VL); biceps brachii (BB); triceps brachii (TB); and abductor digiti minimi (ADM). Results: Stiffness was significantly higher in DMD patients compared with controls for all the muscles (main effect for population, P < 0.033 in all cases), except for ADM. The effect size was small (d = 0.33 for ADM at both muscle lengths) to large (d = 0.86 for BB/stretched). Conclusions: Supersonic shear imaging is a sensitive non‐invasive technique to assess the increase in muscle stiffness associated with DMD. Muscle Nerve 51 : 284–286, 2015 相似文献
962.
Risk factors for neurodegeneration in idiopathic rapid eye movement sleep behavior disorder: A multicenter study
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Ronald B. Postuma MD MSc Alex Iranzo MD Birgit Hogl MD Isabelle Arnulf MD PhD Luigi Ferini‐Strambi MD PhD Raffaele Manni MD Tomoyuki Miyamoto MD PhD Wolfgang Oertel MD Yves Dauvilliers MD PhD Yo‐El Ju MD Monica Puligheddu MD Karel Sonka MD PhD Amelie Pelletier PhD Juan Santamaria MD Birgit Frauscher MD Smaranda Leu‐Semenescu MD Marco Zucconi MD Michele Terzaghi MD Masayuki Miyamoto MD PhD Marcus M. Unger MD Bertrand Carlander MD Maria‐Livia Fantini MD MSc Jacques Y. Montplaisir MD PhD 《Annals of neurology》2015,77(5):830-839
963.
Genomic studies of multiple myeloma reveal an association between X chromosome alterations and genomic profile complexity
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![点击此处可从《Genes, chromosomes & cancer》网站下载免费的PDF全文](/ch/ext_images/free.gif)
964.
Sarah Issa Nadege Bondurand Emmanuelle Faubert Sylvain Poisson Laure Lecerf Patrick Nitschke Naima Deggouj Natalie Loundon Laurence Jonard Albert David Yves Sznajer Patricia Blanchet Sandrine Marlin Veronique Pingault 《Human mutation》2017,38(5):581-593
Waardenburg syndrome (WS) is a genetic disorder characterized by sensorineural hearing loss and pigmentation anomalies. The clinical definition of four WS types is based on additional features due to defects in structures mostly arising from the neural crest, with type I and type II being the most frequent. While type I is tightly associated to PAX3 mutations, WS type II (WS2) remains partly enigmatic with mutations in known genes (MITF, SOX10) accounting for only 30% of the cases. We performed exome sequencing in a WS2 index case and identified a heterozygous missense variation in EDNRB. Interestingly, homozygous (and very rare heterozygous) EDNRB mutations are already described in type IV WS (i.e., in association with Hirschsprung disease [HD]) and heterozygous mutations in isolated HD. Screening of a WS2 cohort led to the identification of an overall of six heterozygous EDNRB variations. Clinical phenotypes, pedigrees and molecular segregation investigations unraveled a dominant mode of inheritance with incomplete penetrance. In parallel, cellular and functional studies showed that each of the mutations impairs the subcellular localization of the receptor or induces a defective downstream signaling pathway. Based on our results, we now estimate EDNRB mutations to be responsible for 5%–6% of WS2. 相似文献
965.
Xavier Argemi Philippe Riegel Thierry Lavigne Nicolas Lefebvre Nicolas Grandpré Yves Hansmann Benoit Jaulhac Gilles Prévost Frédéric Schramm 《Journal of clinical microbiology》2015,53(7):2030-2036
The use of matrix-assisted laser desorption ionization–time of flight mass spectrometry (MALDI-TOF MS) for staphylococcal identification is now considered routine in laboratories compared with the conventional phenotypical methods previously used. We verified its microbiological relevance for identifying the main species of coagulase-negative staphylococci (CoNS) by randomly selecting 50 isolates. From 1 January 2007 to 31 August 2008, 12,479 staphylococci were isolated with phenotypic methods, of which 4,594 were identified as Staphylococcus aureus and 7,885 were coagulase negative staphylococci. Using MALDI-TOF MS from 1 January 2011 to 31 August 2012, 14,913 staphylococci were identified, with 5,066 as S. aureus and 9,847 as CoNS. MALDI-TOF MS allowed the identification of approximately 85% of the CoNS strains, whereas only 14% of the CoNS strains were identified to the species level with phenotypic methods because they were often considered contaminants. Furthermore, the use of MALDI-TOF MS revealed the occurrence of recently characterized Staphylococcus species, such as S. pettenkoferi, S. condimenti, and S. piscifermentans. Microbiological relevance analysis further revealed that some species displayed a high rate of microbiological significance, i.e., 40% of the S. lugdunensis strains included in the analysis were associated with infection risk. This retrospective microbiological study confirms the role of MALDI-TOF MS in clinical settings for the identification of staphylococci with clinical consequences. The species distribution reveals the occurrence of the recently identified species S. pettenkoferi and putative virulent species, including S. lugdunensis. 相似文献
966.
Common variable immunodeficiency is associated with defective functions of dendritic cells 总被引:5,自引:1,他引:5
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Bayry J Lacroix-Desmazes S Kazatchkine MD Galicier L Lepelletier Y Webster D Lévy Y Eibl MM Oksenhendler E Hermine O Kaveri SV 《Blood》2004,104(8):2441-2443
Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia and defects in T-cell functions that could be primary or secondary. We addressed whether CVID is associated with impairment in the dendritic cell (DC) compartment, as DCs play a central role in the development of adaptive immunity. We demonstrate that DCs from CVID patients display severely perturbed differentiation, maturation, and function, and express markedly reduced levels of the costimulatory molecules that are critical for T-cell stimulation. Patients' DCs induced weak proliferation of allogeneic T cells and produced significantly low amounts of interleukin-12 (IL-12) upon CD40 signaling. Multiple defects in the immune system, including malfunctioning of DCs, appear to be prominent features of CVID patients. Impairment in both the innate and adaptive compartments of the immune system may thus cumulatively account for the inability of CVID patients to eradicate pathogens through conventional immune pathways, thus resulting in an increased risk for recurrent bacterial infections. 相似文献
967.
Role of MTHFR genetic polymorphisms in the susceptibility to childhood acute lymphoblastic leukemia 总被引:12,自引:1,他引:12
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Krajinovic M Lamothe S Labuda D Lemieux-Blanchard E Theoret Y Moghrabi A Sinnett D 《Blood》2004,103(1):252-257
The central role of methylenetetrahydrofolate reductase (MTHFR) in the folate metabolism renders MTHFR gene polymorphisms (C677T and A1298C) potential modulators of a variety of disorders whose development depends on folate/homocysteine imbalance. Here, we provide additional evidence on the protective role of these polymorphisms in acute lymphoblastic leukemia (ALL), the most common pediatric cancer. A case-control study was conducted in 270 ALL patients and 300 healthy controls of French-Canadian origin. The TT677/AA1298 and CC677/CC1298 individuals were associated with reduced risk of ALL (crude odds ratio [OR] = 0.4; 95% confidence interval [CI], 0.2-0.9; and OR = 0.3; 95% CI, 0.1-0.6; respectively). Further stratification in patients born before and after January 1996 (approximate time of Health Canada recommendation for folic acid supplement in pregnancy) revealed that the protective effect of MTHFR variants is accentuated and present only in children born before 1996. Similar results were obtained when a transmission disequilibrium test was performed on a subset of children (n = 95) in a family-based study. This finding suggests gene-environment interaction and its role in the susceptibility to childhood ALL, which is consistent with previous findings associating either folate deficiency or MTHFR polymorphisms with risk of leukemia. 相似文献
968.
Armand Garot Marie Marquet Alexis Saintamand Sébastien Bender Sandrine Le Noir Pauline Rouaud Claire Carrion Zéliha Oruc Anne-Ga?lle Bébin Jeanne Moreau Kevin Lebrigand Yves Denizot Frederick W. Alt Michel Cogné Eric Pinaud 《Proceedings of the National Academy of Sciences of the United States of America》2016,113(6):1618-1623
969.
Taaffe M Fischer E Baranowski A Majunke N Heinisch C Leetz M Hein R Bayard Y Büscheck F Reschke M Hoffmann I Wunderlich N Wilson N Sievert H 《The American journal of cardiology》2008,101(9):1353-1358
This randomized trial compared procedural complications and 30-day clinical outcomes of 3 patent foramen ovale (PFO) closure devices (Amplatzer, Helex, and CardioSEAL-STARflex). It examined 660 patients (361 men, 299 women, mean age 49.3+/-1.9 years), with 220 patients per group. All patients had a history of paradoxical embolism. All PFO closures were successful technically. Exchange of devices for others was most frequently required for the Helex occluder (7 of 220) and 2 of 220 in either of the other groups. Three device embolizations in the Helex group were retrieved and replaced successfully. One patient with a Helex occluder developed a transient ischemic attack and recovered without treatment. A hemopericardium in that group was punctured without affecting the device. One tamponade in the Amplatzer group required surgical device explantation. In 8 of 660 patients in the CardioSEAL-STARflex group, thrombi resolved after anticoagulation. Sixteen patients (11 in the CardioSEAL-STARflex group, 3 in the Amplatzer group, and 2 in the Helex group) had episodes of atrial fibrillation. PFOs were closed completely in 143 of 220 patients (65%) in the Amplatzer group, 116 of 220 patients (52.7%) in the Helex group, and 137 of 220 patients (62.3%) in the CardioSEAL-STARflex group at 30 days with significant differences between the Helex and Amplatzer occluders (p=0.0005) and the Helex and CardioSEAL-STARflex occluders (p=0.0003). PFO closure can be performed safely with each device. In conclusion, the Helex occluder embolized more frequently. Device thrombus formation and paroxysmal atrial fibrillation were more common with the CardioSEAL-STARflex occluder. 相似文献
970.
Macrophage dynamics are regulated by local macrophage proliferation and monocyte recruitment in injured pancreas
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Gunter Leuckx Yves Heremans Sofie De Groef Ying Cai Yvon Elkrim Conny Gysemans Benoît Stijlemans Mark Van de Casteele Patrick De Baetselier Nico De Leu Jo A. Van Ginderachter 《European journal of immunology》2015,45(5):1482-1493
Pancreas injury by partial duct ligation (PDL) activates a healing response, encompassing β‐cell neogenesis and proliferation. Macrophages (MΦs) were recently shown to promote β‐cell proliferation after PDL, but they remain poorly characterized. We assessed myeloid cell diversity and the factors driving myeloid cell dynamics following acute pancreas injury by PDL. In naive and sham‐operated pancreas, the myeloid cell compartment consisted mainly of two distinct tissue‐resident MΦ types, designated MHC‐IIlo and MHC‐IIhi MΦs, the latter being predominant. MHC‐IIlo and MHC‐IIhi pancreas MΦs differed at the molecular level, with MHC‐IIlo MΦs being more M2‐activated. After PDL, there was an early surge of Ly6Chi monocyte infiltration in the pancreas, followed by a transient MHC‐IIlo MΦ peak and ultimately a restoration of the MHC‐IIhi MΦ‐dominated steady‐state equilibrium. These intricate MΦ dynamics in PDL pancreas depended on monocyte recruitment by C‐C chemokine receptor 2 and macrophage‐colony stimulating factor receptor as well as on macrophage‐colony stimulating factor receptor‐dependent local MΦ proliferation. Functionally, MHC‐IIlo MΦs were more angiogenic. We further demonstrated that, at least in C‐C chemokine receptor 2‐KO mice, tissue MΦs, rather than Ly6Chi monocyte‐derived MΦs, contributed to β‐cell proliferation. Together, our study fully characterizes the MΦ subsets in the pancreas and clarifies the complex dynamics of MΦs after PDL injury. 相似文献