全文获取类型
收费全文 | 1253篇 |
免费 | 94篇 |
国内免费 | 9篇 |
专业分类
耳鼻咽喉 | 2篇 |
儿科学 | 29篇 |
妇产科学 | 10篇 |
基础医学 | 193篇 |
口腔科学 | 28篇 |
临床医学 | 88篇 |
内科学 | 293篇 |
皮肤病学 | 48篇 |
神经病学 | 151篇 |
特种医学 | 28篇 |
外科学 | 160篇 |
综合类 | 8篇 |
预防医学 | 66篇 |
眼科学 | 13篇 |
药学 | 90篇 |
中国医学 | 1篇 |
肿瘤学 | 148篇 |
出版年
2024年 | 2篇 |
2023年 | 10篇 |
2022年 | 22篇 |
2021年 | 37篇 |
2020年 | 26篇 |
2019年 | 28篇 |
2018年 | 32篇 |
2017年 | 35篇 |
2016年 | 32篇 |
2015年 | 47篇 |
2014年 | 52篇 |
2013年 | 71篇 |
2012年 | 106篇 |
2011年 | 85篇 |
2010年 | 65篇 |
2009年 | 56篇 |
2008年 | 98篇 |
2007年 | 90篇 |
2006年 | 83篇 |
2005年 | 82篇 |
2004年 | 92篇 |
2003年 | 65篇 |
2002年 | 58篇 |
2001年 | 10篇 |
2000年 | 8篇 |
1999年 | 10篇 |
1998年 | 7篇 |
1997年 | 5篇 |
1996年 | 5篇 |
1995年 | 6篇 |
1994年 | 2篇 |
1993年 | 7篇 |
1992年 | 3篇 |
1990年 | 2篇 |
1989年 | 2篇 |
1988年 | 2篇 |
1986年 | 2篇 |
1985年 | 2篇 |
1984年 | 2篇 |
1980年 | 1篇 |
1979年 | 1篇 |
1978年 | 1篇 |
1976年 | 1篇 |
1975年 | 2篇 |
1973年 | 1篇 |
排序方式: 共有1356条查询结果,搜索用时 11 毫秒
1.
Clinical,immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study 总被引:5,自引:0,他引:5
Plebani A Soresina A Rondelli R Amato GM Azzari C Cardinale F Cazzola G Consolini R De Mattia D Dell'Erba G Duse M Fiorini M Martino S Martire B Masi M Monafo V Moschese V Notarangelo LD Orlandi P Panei P Pession A Pietrogrande MC Pignata C Quinti I Ragno V Rossi P Sciotto A Stabile A;Italian Pediatric Group for XLA-AIEOP 《Clinical immunology (Orlando, Fla.)》2002,104(3):221-230
A questionnaire-based retrospective clinical and immunological survey was conducted in 73 males with a definite diagnosis of X-linked agammaglobulinemia based on BTK sequence analysis. Forty-four were sporadic and 29 familial cases. At December 2000, the patients' ages ranged from 2 to 33 years; mean age at diagnosis and mean duration of follow-up were 3.5 and 10 years respectively. After the mid-1980s all but 2 were on intravenous immunoglobulin (IVIG) substitution therapy, with residual IgG >500 mg/dl in 94% of the patients at the time of enrollment. Respiratory infections were the most frequent manifestation both prior to diagnosis and over follow-up. Chronic lung disease (CLD) was present in 24 patients, in 15 already at diagnosis and in 9 more by 2000. The cumulative risk to present at diagnosis with CLD increased from 0.17 to 0.40 and 0.78 when the diagnosis was made at the ages of 5, 10, and 15 years respectively. For the 9 patients who developed CLD during follow-up, the duration of follow-up, rather than age at diagnosis; previous administration of intramuscular immunoglobulin; and residual IgG levels had a significant effect on the development of CLD. Chronic sinusitis was present in 35 patients (48%), in 15 already at diagnosis and in 20 by 2000. Sistemic infections such as sepsis and meningitis/meningoencephalitis decreased over follow-up, probably due to optimal protection provided by high circulating IgG levels reached with IVIG. 相似文献
2.
Peyvandi F Tagliabue L Menegatti M Karimi M Komáromi I Katona E Muszbek L Mannucci PM 《Human mutation》2004,23(1):98
Factor XIII (FXIII) deficiency is a very rare severe autosomal bleeding disorder with a frequency of 1:2,000,000 in the general population and only a few patients have been genetically characterized so far. We report a phenotype-genotype characterization of 10 unrelated Iranian patients. Two FXIII (transglutaminase) activity assays showed no FXIII activity, except a conserved residual activity in patients receiving prophylactic substitution treatment. FXIII antigen concentrations measured by two immunoassays were comparable. Genotype characterization identified four novel mutations (2 missense and 2 small deletions) and two previously reported missense mutations in the FXIII A subunit gene (F13A). Molecular modeling was carried out to reveal the structural consequences of the missense mutations, that caused the replacement of an arginine residue involved in the formation of structurally important extensive hydrogen-bonded network. The replacements [c.320G>A (p.Arg77His) in the beta-sandwich, c.868C>T (p.Arg260Cys), c.869G>A (p.Arg260His) and c.1236G>T (p.Arg382Ser) in the core domain] resulted in the loss or impairment of such H-bonded network. Energy decomposition analysis demonstrated that this situation leads to the instability and perhaps to the incorrect folding of the A subunit, that would explain the development of severe FXIII deficiency. 相似文献
3.
4.
Chiara Zuccato Marzia Tartari Donato Goffredo Elena Cattaneo Dorotea Rigamonti 《Pharmacological research》2005,52(3):245-251
Treatment of neurodegenerative diseases represents a major challenge for the pharmaceutical industry. Key to developing novel and efficacious therapeutics is the discovery of new druggable targets. Toward this aim, the current drug discovery process is strongly relying on the improved understanding of disease mechanisms and on a synergistic approach with chemistry, molecular biology and robotics. In this scenario, we present the case of a newly discovered molecular mechanism that may be of interest for drug discovery programmes in Huntington's disease and other neurodegenerative diseases. 相似文献
5.
6.
7.
Janaina Brollo Maximiliano Cassilha Kneubil Edoardo Botteri Nicole Rotmensz Bruno Achutti Duso Luca Fumagalli Marzia Adelia Locatelli Carmen Criscitiello Visnu Lohsiriwat Aron Goldhirsch Maria Cristina Leonardi Roberto Orecchia Giuseppe Curigliano 《Breast (Edinburgh, Scotland)》2013,22(5):856-862
Literature shows that HER2/neu positive breast cancer cells are more sensitive to radiation-induced apoptosis by targeting the epidermal growth factor receptor family tyrosine kinase. We selected 466 patients with pT1-2 HER2/neu positive tumors who received adjuvant trastuzumab for primary invasive breast cancer. Patients were divided into three groups [Quadrantectomy followed by conventional radiotherapy vs Quadrantectomy followed by Intra-operative radiotherapy with electrons vs Mastectomy without radiotherapy]. After a median follow-up of 52 months, the 5-year cumulative incidence of locoregional recurrence (LRR) was 1.9%, 11.5% and 5.0% respectively (p < 0.01). At the multivariate analysis, extensive perivascular invasion, Luminal B HER2/Progesterone Receptor (PgR) negative status and Quadrantectomy followed by Intra-operative radiotherapy with electrons have significantly increased the risk of LRR. Our results suggest that HER2/neu positive breast cancer might have better outcomes when treated simultaneously with external radiotherapy and trastuzumab. Moreover, we underline the importance of PgR and further new stratification of risk among luminal subtypes. 相似文献
8.
Prifti E Bonacchi M Giunti G Frati G Leacche M Bartolozzi F 《Journal of cardiac surgery》2003,18(5):375-383
OBJECTIVE: The aim of this study is to evaluate in a cohort of patients with impaired left ventricular (LV) function and ischemic mitral valve regurgitation (MVR), the effects of on-pump/beating heart versus conventional surgery in terms of postoperative mortality and morbidity and LV function improvement. MATERIALS AND METHODS: Between January 1993 and February 2001, 91 patients with LVEF between 17% and 35% and chronic ischemic MVR (grade III-IV), underwent MV repair in concomitance with coronary artery bypass grafting (CABG) Sixty-one patients (Group I) underwent cardiac surgery with cardioplegic arrest, and 30 patients (Group II) underwent beating heart combined surgery. Aortic valve insufficiency was considered a contraindication for the on-pump/beating heart procedure. Mean age in Group I was 64.4 +/- 7 years and in Group II, 65 +/- 6 years (p = 0.69). RESULTS: The in-hospital mortality in Group I was 8 (13%) patients versus 2 (7%) patients in Group II (p > 0.1). The cardiopulmonary bypass (CPB) time was significantly higher in Group I (p < 0.001). In Groups I and II, respectively (p > 0.1), 2.5 +/- 1 and 2.7 +/- 0.8 grafts per patient were employed. Perioperative complications were identified in 37 (60.7%) patients in Group I versus 10 (33%) patients in Group II (p = 0.025). Prolonged inotropic support of greater than 24 hours was needed in 48 (78.7%) patients (Group I) versus 15 (50%) patients (Group II) (p = 0.008). Postoperative IABP and low cardiac output incidence were significantly higher in Group I, p = 0.03 and p = 0.027, respectively. Postoperative bleeding greater than 1000 mL was identified in 24 patients (39.4%) in Group I versus 5 (16.7%) in Group II (p = 0.033). Renal dysfunction incidence was 65.6% (40 patients) in Group I versus 36.7% (11 patients) in Group II (p = 0.013). The echocardiographic examination within six postoperative months revealed a significant improvement of MV regurgitation fraction, LV function, and reduced dimensions in both groups. The postoperative RF was significantly lower in Group II patients 12 +/- 6 (%) versus 16 +/- 5.6 (%) in Group I (p = 0.001). The 1, 2, and 3 years actuarial survival including all deaths was 91.3%, 84.2%, and 70% in Group I and 93.3%, 87.1%, and 75% in Group II (p = ns). NYHA FC improved significantly in all patients from both groups. CONCLUSION: We conclude that patients with impaired LV function and ischemic MVR may undergo combined surgery with acceptable mortality and morbidity. The on/pump beating heart MV repair simultaneous to CABG offers an acceptable postoperative outcome in selected patients. 相似文献
9.
Torrente Y Belicchi M Pisati F Pagano SF Fortunato F Sironi M D'Angelo MG Parati EA Scarlato G Bresolin N 《Cell transplantation》2002,11(1):25-34
Stem cell populations have been shown to be extremely versatile: they can generate differentiated cells specific to the tissue in which they reside and descendents that are of different germ layer origin. This raises the possibility of obtaining neuronal cells from new biological source of the same adult human subjects. In this study, we found that epidermal growth factor (EGF) and basic fibroblast growth factor (bFGF) cooperated to induce the proliferation, self-renewal, and expansion of neural stem cell-like population isolated from several newborn and adult mouse tissues: muscle and hematopoietic tissues. This population, in both primary culture and secondary expanded clones, formed spheres of undifferentiated cells that were induced to differentiate into neurons, astrocytes, and oligodendrocytes. Brain engraftment of the somatic-derived neural stem cells generated neuronal phenotypes, demonstrating the great plasticity of these cells with potential clinical application. 相似文献
10.
Massimo Bonacchi Edvin Prifti Massimo Maiani Gabriele Giunti Marzia Leacche 《European journal of cardio-thoracic surgery》2005,28(1):120-126
OBJECTIVE: The aim of this study was to evaluate the feasibility, safety and outcome of skeletonized bilateral internal mammary arteries (BIMA) in patients with unstable angina (UA) undergoing non-elective myocardial revascularization. METHODS: Between January 1997 and December 2003, 758 patients, mean age 62+/-12 years, underwent non-elective coronary artery bypass grafting (CABG) for unstable angina. Two hundred and five (27%) were operated emergently and 503 (73%) urgently. BIMA were employed in 320 (42%) patients (Group B) and isolated left IMA and/or saphenous vein grafts in the remaining 438 (58%) patients (Group M). RESULTS: In-hospital mortality (B = 5.9% and M = 5.3%), and perioperative myocardial infarction (B = 2.2%; M = 1.96%) were similar between the two groups (P = ns). Actuarial survival at 1, 3 and 7 years was 98.7, 97.5 and 96.2% in B and 99, 94.3 and 88.4% in M (P < 0.05 at 7 years follow-up). At 7 years follow-up, the event-free cardiac survival (92 vs. 87%, P = 0.021), angina-free survival (98.6 vs. 94%, P = 0.039), reoperation-free cardiac survival (98 vs. 95%, P = 0.04) and infarct-free cardiac survival (98.7 vs. 96%, P = 0.05) were better in Group B. Multivariate analysis identified age > 65 years (P = 0.02), LVEF < 35% (P = 0.01), > 1 ischemic irreversible area (P = 0.03) as independent predictors for late deaths, while the use of the LIMA (P=0.006) and both mammary arteries (P=0.001) decreased the risk of late deaths. CONCLUSIONS: The use of BIMA in non-elective CABG for UA is safe and effective. Mid-term outcome, however, are superior with improved freedom from cardiac death, from coronary reintervention and from myocardial infarction. 相似文献