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61.
Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID Registry 总被引:4,自引:4,他引:0
Leiva LE Zelazco M Oleastro M Carneiro-Sampaio M Condino-Neto A Costa-Carvalho BT Grumach AS Quezada A Patiño P Franco JL Porras O Rodríguez FJ Espinosa-Rosales FJ Espinosa-Padilla SE Almillategui D Martínez C Tafur JR Valentín M Benarroch L Barroso R Sorensen RU;Latin American Group for Primary Immunodeficiency Diseases 《Journal of clinical immunology》2007,27(1):101-108
This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with
primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras,
Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered,
the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency
reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined
T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies.
All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency
cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic
granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase
in the reporting of these diseases in all countries. In this report, the estimated minimal incidence of chronic granulomatous
disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence
of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these
diseases are underreported in other participating countries. In addition to a better diagnosis of primary immunodeficiency
diseases, more work on improving the registration of patients by each participating country and by countries that have not
yet joined LAGID is still needed.
Latin American Group for Primary Immunodeficiency Diseases 相似文献
62.
Yukinobu Kodama Hiroki Hanamura Takahiro Muro Hiroo Nakagawa Tomoaki Kurosaki Tadahiro Nakamura 《Journal of drug targeting》2018,26(7):604-609
Fetuin is a biocompatible plasma protein and strongly enhances phagocytosis of bacteria, DNA and apoptotic cells by peripheral blood cells such as monocytes, macrophages and dendritic cells. We developed a novel gene delivery system: ternary complexes constructed with pDNA, polyethylenimine (PEI) and fetuin. Without covalent binding, fetuin was able to coat pDNA–PEI complexes, and stable anionic nanoparticles formed at a weight ratio greater than 30. Optimised pDNA–PEI–fetuin complexes significantly decreased the cytotoxicity of pDNA–PEI complexes in the melanoma cell line B16F10. Furthermore, the pDNA–PEI–fetuin complexes had higher transgene efficiency compared to that of commercial lipofectin previously reported in B16F10 cells despite an anionic surface. The pDNA–PEI–fetuin complexes did not agglutinate with erythrocytes. The pDNA–PEI–fetuin complexes had high gene expression in the spleen after intravenous administration in mice. Thus, the pDNA–PEI–fetuin complexes were a useful in vivo gene delivery system with tropism for the spleen. 相似文献
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64.
CU Menakaya AS Rigby Y Hadland E Barron H Sharma 《Annals of the Royal College of Surgeons of England》2014,96(2):106-110
Introduction
The optimal treatment of high energy tibial fractures remains controversial and a challenging orthopaedic problem. The role of external fixators for all these tibial fractures has been shown to be crucial.Methods
A five-year consecutive series was reviewed retrospectively, identifying two treatment groups: Ilizarov and Taylor Spatial Frame (TSF; Smith & Nephew, Memphis, TN, US). Fracture healing time was the primary outcome measure.Results
A total of 112 patients (85 Ilizarov, 37 TSF) were identified for the review with a mean age of 45 years. This was higher in women (57 years) than in men (41 years). There was no significant difference between frame types (p=0.83). The median healing time was 163 days in both groups. There was no significant difference in healing time between smokers and non-smokers (180 vs 165 days respectively, p=0.07), open or closed fractures (p=0.13) or age and healing time (Spearman''s r=0.12, p=0.18). There was no incidence of non-union or re-fracture following frame removal in either group.Conclusions
Despite the assumption of the rigid construct of the TSF, the median time to union was similar to that of the Ilizarov frame and the TSF therefore can play a significant role in complex tibial fractures. 相似文献65.
Marco Montanaro Roberto Latagliata Michele Cedrone Antonio Spadea Angela Rago Jonny Di Giandomenico Francesca Spirito Raffaele Porrini Marianna De Muro Sabrina Crescenzi Leonetti Nicoletta Villivà Cinzia De Gregoris Massimo Breccia Enrico Montefusco Cristina Santoro Giuseppe Cimino Ignazio Majolino Maria Gabriella Mazzucconi Giuliana Alimena Alesssandro Andriani 《American journal of hematology》2014,89(5):542-546
To identify prognostic factors affecting thrombosis‐free survival (TFS) and overall survival (OS), we report the experience of a Regional cooperative group in a real‐life cohort of 1,144 patients with essential thrombocythemia (ET) diagnosed from January 1979 to December 2010. There were 107 thrombotic events (9.4%) during follow‐up [60 (5.3%) arterial and 47 (4.1%) venous thromboses]. At univariate analysis, risk factors for a shorter TFS were: age >60 years (P < 0.0054, 95% CI 1.18–2.6), previous thrombosis (P < 0.0001, 95% CI 1.58–4.52) and the presence of at least one cardiovascular risk factor (P = 0.036, 95% CI 1.15–3.13). Patients with a previous thrombosis occurred ≥24 months before ET diagnosis had a shorter TFS compared to patients with a previous thrombosis occurred <24 months (P = 0.0029, 95% CI 1.5–6.1); furthermore, patients with previous thrombosis occurred <24 months did not show a shorter TFS compared with patients without previous thrombosis (P = 0.303, 95% CI 0.64–3.21). At multivariate analysis for TFS, only the occurrence of a previous thrombosis maintained its prognostic impact (P = 0.0004, 95% CI 1.48–3.79, RR 2.36). The 10‐year OS was 89.9% (95% CI 87.3–92.5): at multivariate analysis for OS, age >60 years (P < 0.0001), anemia (P < 0.0001), male gender (P = 0.0019), previous thromboses (P = 0.0344), and white blood cell >15 × 109/l (P = 0.0370) were independent risk factors. Previous thrombotic events in ET patients are crucial for TFS but their importance seems related not to the occurrence per se but mainly to the interval between the event and the diagnosis. Am. J. Hematol. 89:542–546, 2014. © 2014 Wiley Periodicals, Inc. 相似文献
66.
Janyaruk Suriyut Satoru Muro Phichaya Baramee Masayo Harada Keiichi Akita 《Anatomical science international / Japanese Association of Anatomists》2020,95(3):305-312
The male pelvic floor is a complex structure formed by several muscles. The levator ani muscle and the perineal muscles are important components of the pel 相似文献
67.
O. Millán L. Rafael-Valdivia D. San Segundo F. Boix M.J. Castro-Panete M. López-Hoyos M. Muro D. Valero-Hervás A. Rimola M. Navasa P. Muñoz M. Miras A. Andrés L. Guirado J. Pascual M. Brunet 《Clinical immunology (Orlando, Fla.)》2014,154(2):141-154
Acute rejection (AR) remains a major challenge in organ transplantation, and there is a need for predictive biomarkers. In the present multicenter study, we prospectively examined a series of biomarkers in liver and kidney recipients. Intracellular expression of IFN-γ, IL-17 and IL-2 and IL-17 soluble production were evaluated both pre-transplantation and post-transplantation (1st and 2nd week, 1st, 2nd and 3rd month). 142 transplant patients (63 liver/79 kidney) were included in the study. Twenty-eight recipients (14 liver/14 kidney) developed AR. Pre- and post-transplantation intracellular expression of %IFN-γ+ in CD4+CD69+ and in CD8+CD69+ and soluble IL17 identified liver and kidney transplant patients at high risk of AR. Pre-transplantation, %IL-2+ in CD8+CD69+ also identified kidney patients at high risk. We constructed pre- and post-transplantation risk prediction models, based on a composite panel of biomarkers, which could provide the basis for future studies and will be a useful tool for the selection and adjustment of immunosuppressive treatments. 相似文献
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69.
R. López‐Hernández M. Valdés J. A. Campillo P. Martínez‐Garcia H. Salama G. Salgado F. Boix M. R. Moya‐Quiles A. Minguela A. Sánchez‐Torres M. Miras A. Garcia F. Carballo M. R. Álvarez‐López M. Muro 《International journal of immunogenetics》2014,41(1):63-68
Tumour necrosis factor alpha (TNF‐α) has an important role in inflammatory response. Alterations in the regulation of TNF‐α have been implicated in a variety of inflammatory disorders, including Inflammatory bowel disease (IBD). Indeed, a common treatment for IBD is the use of TNF‐α inhibitors. Polymorphisms in the TNF‐α promoter region are known to affect the level of gene expression. Our aim was to investigate the influence of these single nucleotide polymorphisms (SNPs) in TNF‐α promoter gene play in the risk of IBD in a Spanish population and their individual response to anti‐TNF‐α treatment. DNA samples from patients with IBD and controls were screened for TNF‐α ?238G/A (rs361525) and ?308G/A (rs1800629) SNPs by PCR‐SSOP using a microbeads luminex assay and compared with response to TNF‐α inhibitors. There were not statistical differences in ?238G/A and ?308G/A allele and genotype frequencies between patients. However, we found an increased frequency of ?308A allele and ?308GA genotype in these nonresponders patients to TNF‐α inhibitors with respect to responders patients (Pc < 0.05). This ?308GA genotype has been classified as high producer of this cytokine. This fact could actually be interesting to explain the different response of patients with IBD with respect to TNF‐α inhibitors. TNF‐α promoter gene polymorphism does not seem to play a role in IBD susceptibility, but particular TNF‐α genotypes may be involved in the different responses to TNF‐α inhibitor treatment in Spanish patients with IBD. 相似文献
70.