全文获取类型
收费全文 | 14268篇 |
免费 | 992篇 |
国内免费 | 32篇 |
专业分类
耳鼻咽喉 | 120篇 |
儿科学 | 431篇 |
妇产科学 | 508篇 |
基础医学 | 2115篇 |
口腔科学 | 242篇 |
临床医学 | 1806篇 |
内科学 | 2500篇 |
皮肤病学 | 409篇 |
神经病学 | 1369篇 |
特种医学 | 272篇 |
外科学 | 1283篇 |
综合类 | 100篇 |
一般理论 | 43篇 |
预防医学 | 1601篇 |
眼科学 | 237篇 |
药学 | 1041篇 |
中国医学 | 24篇 |
肿瘤学 | 1191篇 |
出版年
2024年 | 14篇 |
2023年 | 141篇 |
2022年 | 329篇 |
2021年 | 560篇 |
2020年 | 277篇 |
2019年 | 494篇 |
2018年 | 521篇 |
2017年 | 353篇 |
2016年 | 458篇 |
2015年 | 442篇 |
2014年 | 617篇 |
2013年 | 779篇 |
2012年 | 1116篇 |
2011年 | 1190篇 |
2010年 | 644篇 |
2009年 | 528篇 |
2008年 | 885篇 |
2007年 | 923篇 |
2006年 | 898篇 |
2005年 | 830篇 |
2004年 | 720篇 |
2003年 | 636篇 |
2002年 | 614篇 |
2001年 | 91篇 |
2000年 | 84篇 |
1999年 | 87篇 |
1998年 | 114篇 |
1997年 | 80篇 |
1996年 | 73篇 |
1995年 | 62篇 |
1994年 | 74篇 |
1993年 | 51篇 |
1992年 | 50篇 |
1991年 | 50篇 |
1990年 | 34篇 |
1989年 | 42篇 |
1988年 | 39篇 |
1987年 | 20篇 |
1986年 | 25篇 |
1985年 | 34篇 |
1984年 | 33篇 |
1983年 | 29篇 |
1982年 | 30篇 |
1981年 | 36篇 |
1980年 | 31篇 |
1979年 | 19篇 |
1978年 | 19篇 |
1977年 | 23篇 |
1976年 | 17篇 |
1974年 | 16篇 |
排序方式: 共有10000条查询结果,搜索用时 15 毫秒
161.
162.
163.
Fludarabine,ara-C,novantrone and dexamethasone (FAND) in previously treated chronic lymphocytic leukemia patients 总被引:1,自引:0,他引:1
Mauro FR Foa R Meloni G Gentile M Giammartini E Giannarelli D De Propris MS Rapanotti MC de Fabritiis P Mandelli F 《Haematologica》2002,87(9):926-933
BACKGROUND AND OBJECTIVES: The objective of improving the quality of responses of chronic lymphocytic leukemia (CLL) patients has led to the design of protocols that combine fludarabine (FDR) with synergistic drugs. We evaluated the efficacy and toxicity of a schedule that includes fludarabine, ara-C, novantrone and dexamethasone (FAND) for the management of previously treated CLL patients under 60 years old. DESIGN AND METHODS: Thirty-one patients underwent FAND treatment. Twenty-three patients had active disease (relapsed patients: 9; unresponsive to prior therapy: 14). Eight patients had a partial response (PR) to prior therapy and were treated with the aim of further reducing residual disease. The FAND schedule included fludarabine (25 mg/m(2) i.v. days 1-3), ara-C (1 g/m(2) i.v. day 1: 8 patients; days 1-2: 23 patients), novantrone (10 mg/m(2) i.v. day 1) and dexamethasone (20 mg i.v. days 1-3). Infection prophylaxis consisted of fluconazole, acyclovir, trimethoprim/sulfamethoxasole and granulocyte colony-stimulating factor (G-CSF) in the presence of severe neutropenia. RESULTS: A response was observed in 7/14 refractory patients (complete response-CR: 29%), in all 9 relapsed patients (CR: 78%) and in 7/8 patients (CR: 87.5%) treated in PR. Taken together, 18 CRs were obtained and in 14 (78%) this was associated with a flow cytometric remission (CD5+/CD20(weak+) PB lymphocytes: <10%). Severe granulocytopenia occurred after 86 of the 124 administered courses (69%), but only after 10/86 courses (12%) were major infections recorded. In 10/15 mobilized patients (cyclophosphamide + G-CSF: 6 patients; FAND + G-CSF: 9 patients) after FAND > or = 2 x 10(6)/kg CD34+ cells were collected. Nine patients were autografted in CR and showed a longer response duration than the 9 patients in CR who did not receive further therapy after FAND (53 vs 30% at 41 months; p = 0.05). INTERPRETATION AND CONCLUSIONS: FAND associated with extensive infection prophylaxis and G-CSF support is a highly cytoreductive and well-tolerated treatment for CLL patients and in most cases does not hamper subsequent stem cell mobilization. 相似文献
164.
Christian Staufner Martin Lindner Carlo Dionisi-Vici Peter Freisinger Dries Dobbelaere Claire Douillard Nawal Makhseed Beate K. Straub Kimia Kahrizi Diana Ballhausen Giancarlo la Marca Stefan Kölker Dorothea Haas Georg F. Hoffmann Sarah C. Grünert Henk J. Blom 《Journal of inherited metabolic disease》2016,39(2):273-283
Background
Adenosine kinase deficiency is a recently described defect affecting methionine metabolism with a severe clinical phenotype comprising mainly neurological and hepatic impairment and dysmorphism.Methods
Clinical data of 11 additional patients from eight families with adenosine kinase deficiency were gathered through a retrospective questionnaire. Two liver biopsies of one patient were systematically evaluated.Results
The main clinical symptoms are mild to severe liver dysfunction with neonatal onset, muscular hypotonia, global developmental retardation and dysmorphism (especially frontal bossing). Hepatic involvement is not a constant finding. Most patients have epilepsy and recurrent hypoglycemia due to hyperinsulinism. Major biochemical findings are intermittent hypermethioninemia, increased S-adenosylmethionine and S-adenosylhomocysteine in plasma and increased adenosine in urine. S-adenosylmethionine and S-adenosylhomocysteine are the most reliable biochemical markers. The major histological finding was pronounced microvesicular hepatic steatosis. Therapeutic trials with a methionine restricted diet indicate a potential beneficial effect on biochemical and clinical parameters in four patients and hyperinsulinism was responsive to diazoxide in two patients.Conclusion
Adenosine kinase deficiency is a severe inborn error at the cross-road of methionine and adenosine metabolism that mainly causes dysmorphism, brain and liver symptoms, but also recurrent hypoglycemia. The clinical phenotype varies from an exclusively neurological to a multi-organ manifestation. Methionine-restricted diet should be considered as a therapeutic option.165.
166.
Saed H Zyoud Dala N Daraghmeh Diana O Mezyed Razan L Khdeir Mayas N Sawafta Nora A Ayaseh Ghada H Tabeeb Waleed M Sweileh Rahmat Awang Samah W Al-Jabi 《Lancet》2018
Background
Haemodialysis is a life-saving but burdensome therapy for patients with end-stage renal disease, which can substantially impair health-related quality of life (HRQOL) and outcomes. The aim of this study was to determine the patterns of HRQOL and to identify the risk factors for reduced HRQOL in Palestinian patients receiving treatment by haemodialysis.Methods
This cross-sectional study was done between June 15, 2014, and Jan 15, 2015, using the EuroQOL-5 Dimensions instrument (EQ-5D-5L) for the assessment of HRQOL. We approached patients with end-stage renal disease undergoing haemodialysis at inpatient hospitals from ten different settings at a national level. The study protocol was approved by the Ethics Committee of An-Najah National University. Informed verbal consent was obtained from each participant before the start of the interview. Multiple linear regression was used to estimate which variables were significantly associated with reduced HRQOL.Findings
267 (96%) of 277 eligible patients consented to participate. 139 (52%) participants were men, and the mean age was 53·3 years (SD 16·2). 177 (66%) patients had been treated by haemodialysis for less than 4 years. The reported HRQOL, as measured by mean EQ-5D-5L index value, was 0·37 (SD 0·44). We found a moderate positive correlation between the EuroQol-visual analogue scales and the EQ-5D-5L index value (r=0·44; p<0·0001). The results of a multiple linear regression showed a significant association between HRQOL and age (p=0·0011), female sex (p=0·0167), education level (p=0·0057), number of chronic medications (p=0·0493), and number of comorbid diseases (p=0·0001).Interpretation
Our results provide insight into a number of associations between patient variables such as demographics, clinical factors, and their HRQOL. These findings should help raise health-care providers' awareness and improve the quality of life for patients receiving treatment by haemodialysis, especially those who have no formal education, are elderly, are female, are from refugee camps, or have multiple comorbid diseases or chronic medications.Funding
None. 相似文献167.
Thrombopoietin Signal Transduction in Purified Murine Megakaryocytes 总被引:4,自引:11,他引:4
168.
Eder H. Cativo Persio D. Lopez Diana P. Cativo Steven A. Atlas Clive Rosendorff 《The American journal of medicine》2021,134(1):104-113.e3
ObjectivesInhibitors of the renin-angiotensin system are recommended for the management of albuminuria in patients with hypertension and diabetes mellitus, but there is little consensus about alternative therapies. Calcium channel blockers are recommended for the management of hypertension, but the data are controversial regarding their role in patients with albuminuria. This review was designed to assess the efficacy of calcium channel blockers compared with inhibitors of the renin-angiotensin system in decreasing albuminuria in diabetic, hypertensive patients with nephropathy.MethodsWe searched MEDLINE, Embase, CENTRAL, and ClinicalTrials.gov for records that compared calcium channel blockers to inhibitors of the renin-angiotensin system and reported pre- and postintervention albuminuria measurements. Two reviewers independently screened abstracts for randomized, controlled trials in adults. We used the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to select 29 trials from 855 records. We synthesized the data through a random-effects model.ResultsWe analyzed data from 2113 trial participants with hypertension and diabetes mellitus who had the equivalent of ≥30 mg/day of urinary albumin excretion. Inhibitors of the renin-angiotensin system were more effective than calcium channel blockers in decreasing albuminuria (standardized difference in means ?0.442; confidence interval, ?0.660 to ?0.225; P < .001). This finding was independent of the blood pressure response to treatment. There was no difference between the 2 drug classes regarding markers of renal function.ConclusionsInhibitors of the renin-angiotensin system are superior to calcium channel blockers for the reduction of albuminuria in nephropathy due to hypertension and diabetes mellitus. The net clinical benefit, however, is small. 相似文献
169.
170.
Martina Pecimonova Jan Radvanszky David Smolak Jaroslav Budis Michal Lichvar Diana Kristinova Ivica Rozova Jan Turna Tomas Szemes 《Medicine》2021,100(22)
Rationale:Periventricular nodular heterotopia-7 (PVNH7) is a neurodevelopmental disorder associated with improper neuronal migration during neurogenesis in cortex development caused by pathogenic variants in the NEDD4L gene.Patient concerns:We report the case of a polystigmatized 2-year-old boy having significant symptomatologic overlap with PVNH7, such as delayed psychomotor and mental development, seizures and infantile spasms, periventricular nodular heterotopia, polymicrogyria, cleft palate, 2 to 3 toe syndactyly, hypotonia, microretrognathia, strabismus, and absent speech and walking. The patient showed also distinct symptoms falling outside PVNH7 symptomatology, also present in the proband''s older brother, such as blue sclerae, hydronephrosis, transversal palmar crease (found also in their father), and bilateral talipes equinovarus. In addition, the patient suffered from many other symptoms.Diagnoses:The boy, his brother and their parents were subjected to whole-exome sequencing. Because of uncertainties in symptomatology and inheritance pattern, the top-down approach was hard to apply. Using the bottom-up approach, we identified a known pathogenic variant, NM_001144967.2(NEDD4L):c.2677G>A:p.Glu893Lys, in the proband''s genome that absented in any other analyzed family member, suggesting its de novo origin.Interventions and outcomes:The patient was treated with Convulex 300 mg/mL for the successful seizure control and Euthyrox 25mg for the treatment of thyroid malfunction. He also took various supplements for the metabolism support and digestion regulation. Moreover, the patient underwent the corrective surgeries of cleft palate and talipes equinovarus.Lessons:We successfully identified the causative mutation NM_001144967.2(NEDD4L):c.2677G>A:p.Glu893Lys explaining symptoms overlapping those reported for PVNH7. Symptoms shared with the brother were not explained by this variant, since he was not a carrier of the pathogenic NEDD4L variant. These are most likely not extended phenotypes of PVNH7, rather an independent clinical entity caused by a yet unidentified genetic factor in the family, highlighting thus the importance of thorough evaluation of symptomatology and genomic findings in affected and unaffected family members, when such data are available. 相似文献