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排序方式: 共有2419条查询结果,搜索用时 15 毫秒
81.
Background: In 2013, the group of Cicero Coimbra, Brazil, reported the clinical efficacy of high doses of vitamin D3 in patients suffering from autoimmune skin disorders (“Coimbra protocol”, CP). However, hypercalcemia and the subsequent impaired renal function may be major concerns raised against this protocol. Methods: We report for the first time for a broad spectrum of autoimmune diseases in 319 patients (mean age (±SD) 43.3 ± 14.6 years, 65.5% female, 34.5% male) safety data for high doses of orally applied vitamin D3 (treatment period: up to 3.5 years) accompanied by a strict low-calcium diet and regular daily fluid intake of at least 2.5 L. Results: Mean vitamin D3 dose was 35,291 ± 21,791 IU per day. The measurement of more than 6100 single relevant laboratory parameters showed all mean values (±SD) within the normal range for total serum calcium (2.4 ± 0.1 mmol/L), serum creatinine (0.8 ± 0.2 mg/dL), serum creatinine associated estimated GFR (92.5 ± 17.3 mL/min), serum cystatin C (0.88 ± 0.19 mg/L), serum TSH (1.8 ± 1 mIU/L), and for 24 h urinary calcium secretion (6.9 ± 3.3 mmol/24 h). We found a very weak relationship between the dosage of oral vitamin D3 and the subsequent calcium levels, both in serum and in urinary excretion over 24 h, respectively. Conclusions: Our data show the reliable safety of the CP in autoimmune patients under appropriate supervision by experienced physicians. 相似文献
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Blepharophimosis,short humeri,developmental delay and hirschsprung disease: Expanding the phenotypic spectrum of MED12 mutations 下载免费PDF全文
Bertrand Isidor Tiphaine Lefebvre Claudine Le Vaillant Gaëlle Caillaud Laurence Faivre Frédéric Jossic Madeleine Joubert Norbert Winer Cédric Le Caignec Guntram Borck Anna Pelet Jeanne Amiel Annick Toutain Nathalie Ronce Martine Raynaud Alain Verloes Albert David 《American journal of medical genetics. Part A》2014,164(7):1821-1825
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Keiji Tanimoto Lars-Gran Hansson Arne Petersson Madeleine Rohlin Carl Christian Johansen 《International journal of oral and maxillofacial surgery》1989,18(6):354-358
The lateral, central and medial parts of 15 right temporomandibular joint autopsy specimens were studied to compare the ability of computed tomography (CT) and single-contrast arthrotomography in diagnosing the disc position and configuration. The radiologic findings were compared with macroscopic findings in a blinded fashion. In the CT examination, the diagnostic accuracy was 40% in determining the disc position and 26.7% in determining the disc configuration. However, in many areas of the joints the disc was not possible to evaluate by CT. With arthrotomography the diagnostic accuracy in determining the disc position was 75.6% and the disc configuration 60%. The sensitivity of CT was 0.45 and of arthrotomography 0.95 for the diagnosis of the disc position. The specificity was 0.87 for CT and 0.76 for arthrotomography. From these results, we conclude that arthrotomography is a reliable aid to diagnosis of TMJ disc changes and that CT cannot replace it. 相似文献
84.
Madeleine Rohlin Rob M.H. Schaub † Peter Holbrook ‡ Edvitar Leibur ‡ Gérard Lévy ‡ Lenka Roubalikova ‡ Maria Nilner ‡ Valerie Roger-Leroi ‡ Gunter Danner ‡ Haluk Iseri ‡ Cecile Feldman ‡ 《European journal of dental education》2002,6(S3):67-77
Continuous quality improvement (CQI) can be envisaged as a circular process of goal-setting, followed by external and internal evaluations resulting in improvements that can serve as goals for a next cycle. The need for CQI is apparent, because of public accountability, maintaining European standards and the improvement of dental education. Many examples are known where recommendations from both external and internal evaluation are used for the improvement of dental education. Unfortunately, the implementation of the recommendations is inconsistent, rarely systematic and usually not transparent. This section agreed that it is essential to apply CQI in a structured, systematic and transparent way if we are to improve and maintain the quality of dental education. A model is proposed which includes three aspects: a) the process of CQI; b) the subjects to which CQI should be applied; and c) the management tools to govern CQI. It is stressed, that CQI is a process that can be applied in any dental school irrespective of curriculum or educational approach within the relevant context of the country or the region. The approach needs to recognize the complexity and the need to balance a quality improvement with accountability. A CQI system is also constrained in any organization by the attitudes and values of the staff. Inevitably there has to be a wide range in the application of CQI. Nevertheless, an agreed model on CQI might enhance convergence towards higher standards of dental education. The process of CQI can be supported by developments in information and communication technology (ICT): collection of data, identifying the steps in CQI, formats of reports, etc. The section was set, as one of its tasks, to advise on the development of a network based on a number of case studies on the application of CQI in dental education. 相似文献
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Johanna C. Badcock Madeleine E. Graham Georgie Paulik 《Clinical psychology & psychotherapy》2020,27(1):79-86
Determining reliable and clinically significant change is central to evidence‐based practice yet rarely used in routine clinical settings. This paper illustrates these methods in the context of an evaluation of cognitive behaviour therapy for distressing auditory hallucinations (“voices”). We used data from a clinical sample attending Perth Voices Clinic, a transdiagnostic outpatient service for distressing voices, and a previously published reference sample of healthy voice hearers. Our outcomes on the primary measure of voice distress, derived from a previous factor analysis of the Psychotic Symptom Rating Scale‐Auditory Hallucinations subscale, showed that 62.9% of clients were classified as Recovered/Improved, 35.5% were classified as Unchanged, and 0.02% were classified as Deteriorated. Partial support for the validity of these classifications was obtained from the scores on the Depression, Anxiety, Stress Scales (Lovibond & Lovibond, 1995) but not on the Social and Occupational Functional Assessment Scale (Goldman et al., 1992). Clients classified as Recovered showed better emotional functioning on the Depression, Anxiety, Stress Scales compared with those who did not make a clinically significant change in voice distress. A tool is provided to assist practitioners to evaluate whether individual clients have benefited from therapy for distressing voices or not, which can be used to guide future treatment decisions ( https://osf.io/gd9e5/ ). 相似文献
89.
Paola Bianchi Elisa Fermo Kimberly Lezon-Geyda Eduard J. van Beers Holmes D. Morton Wilma Barcellini Bertil Glader Satheesh Chonat Yaddanapudi Ravindranath Peter E. Newburger Nina Kollmar Jenny M. Despotovic Madeleine Verhovsek Mukta Sharma Janet L. Kwiatkowski Kevin H. M. Kuo Marcin W. Wlodarski Hassan M. Yaish Susanne Holzhauer Heng Wang Joachim Kunz Kathryn Addonizio Hasan Al-Sayegh Wendy B. London Oliver Andres Richard van Wijk Patrick G. Gallagher Rachael F. F. Grace 《American journal of hematology》2020,95(5):472-482
Pyruvate kinase (PK) deficiency is a rare recessive congenital hemolytic anemia caused by mutations in the PKLR gene. This study reports the molecular features of 257 patients enrolled in the PKD Natural History Study. Of the 127 different pathogenic variants detected, 84 were missense and 43 non-missense, including 20 stop-gain, 11 affecting splicing, five large deletions, four in-frame indels, and three promoter variants. Within the 177 unrelated patients, 35 were homozygous and 142 compound heterozygous (77 for two missense, 48 for one missense and one non-missense, and 17 for two non-missense variants); the two most frequent mutations were p.R510Q in 23% and p.R486W in 9% of mutated alleles. Fifty-five (21%) patients were found to have at least one previously unreported variant with 45 newly described mutations. Patients with two non-missense mutations had lower hemoglobin levels, higher numbers of lifetime transfusions, and higher rates of complications including iron overload, extramedullary hematopoiesis, and pulmonary hypertension. Rare severe complications, including lower extremity ulcerations and hepatic failure, were seen more frequently in patients with non-missense mutations or with missense mutations characterized by severe protein instability. The PKLR genotype did not correlate with the frequency of complications in utero or in the newborn period. With ICCs ranging from 0.4 to 0.61, about the same degree of clinical similarity exists within siblings as it does between siblings, in terms of hemoglobin, total bilirubin, splenectomy status, and cholecystectomy status. Pregnancy outcomes were similar across genotypes in PK deficient women. This report confirms the wide genetic heterogeneity of PK deficiency. 相似文献
90.