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101.
The use of a Day Program (DP) format (i.e., intensive daily treatment with no overnight admission) has been shown to be an effective treatment for eating disorders (EDs). The disadvantages, however, include higher cost than outpatient treatment (including costs of meals and staff), greater disruption to patients’ lives, and the use of a highly structured and strict schedule that may interrupt the development of patients’ autonomy in taking responsibility for their recovery. This study investigated whether reducing costs of a DP and the disruption to patients’ lives, and increasing opportunity to develop autonomy, impacted clinical outcomes. Three sequential DP formats were compared in the current study: Format 1 was the most expensive (provision of supported dinners three times/week and extended staff hours); Format 2 included only one dinner/week and provision of a take-home meal. Both formats gave greater support to patients who were not progressing well (i.e., extended admission and extensive support from staff when experiencing feelings of suicidality or self-harm). Format 3 did not provide this greater support but established pre-determined admission lengths and required the patient to step out of the program temporarily when feeling suicidal. Fifty-six patients were included in the analyses: 45% were underweight (body mass index (BMI) < 18.5), 96.4% were female, 63% were given a primary diagnosis of anorexia nervosa (or atypical anorexia nervosa), and mean age was 25.57 years. Clinical outcomes were assessed using self-reported measures of disordered eating, psychosocial impairment, and negative mood, but BMI was recorded by staff. Over admission, 4- and 8-week post-admission, and discharge there were no significant differences between any of the clinical outcomes across the three formats. We can tentatively conclude that decreasing costs and increasing the opportunities for autonomy did not negatively impact patient outcomes, but future research should seek to replicate these results in other and larger populations that allow conclusions to be drawn for different eating disorder diagnostic groups.  相似文献   
102.
Cardiovascular diseases, including atherosclerosis and the dreaded complication myocardial infarction, represent the major cause of death in western countries. It is now generally accepted that chemokines tightly control and modulate all the events which lead to initiation and progression of cardiovascular diseases, making them very attractive therapeutic targets for the pharmaceutical industry. Various studies showed until now the effects of antagonizing/ neutralizing chemokines or blocking chemokine receptors on cardiovascular pathology. The modulation of the CCL2/CCR2, CCL5/CCR1-CCR5, CXCL12/CXCR4 pathways by preventing receptor--ligand interaction, chemokine-glycosaminoglycan interaction, heteromerization, or interfering with the signaling pathways has proven to have high potential in future drug development. However, while trying to understand the effects of individual chemokines, the biologic consequences of multiple and concomitant chemokine expression on leukocyte migration and function should be taken into account as well. Therefore, many aspects should be considered and carefully scrutinized, when devising therapeutic strategies.  相似文献   
103.
The aim of this study was to evaluate the dentoskeletal effects of early treatment in the primary or early mixed dentition with a removable appliance with expansion springs, assessed on postero-anterior (PA) cephalograms, in patients with a unilateral posterior crossbite when compared with untreated subjects. The treatment group consisted of 23 subjects, 8 males, and 15 females treated with a removable appliance for the expansion of the maxillary arch. The mean age at the start of expansion (T1) was 6 years 2 +/- 17 months, and 8 years +/- 18 months at the end of active therapy and after 1 year of retention (T2), with an observation interval of 22 +/- 7 months. The control group comprised 20 subjects (9 males and 11 females) with an untreated unilateral posterior crossbite. Their mean age was 5 years 9 +/- 15 months at the first observation and 7 years and 4 +/- 16 months at the second examination. The interval between the two observations was 18 +/- 7 months. Nine skeletal and two dental measurements on the transverse plane were assessed. The data from the two groups were compared by means of a Student's t-test for independent samples (P < 0.05). Positive dental and skeletal effects induced by the therapy were observed at T2. The width of the upper dental arch and that of the skeletal maxillary transverse dimension were significantly greater (P < 0.001) in the treatment group when compared with the controls.  相似文献   
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Immunotherapies targeting cytotoxic T‐lymphocyte‐associated antigen 4 (CTLA‐4) and the programmed cell death 1 (PD‐1) receptor and its ligand (PD‐L1) have showed significant therapeutic benefit in patients with clinically advanced solid malignancies, including melanoma. However, immune‐related adverse events (irAE) are common, and novel dermatologic toxicities continue to emerge as more patients are treated with immunotherapy. Here we describe a patient treated with combination immunotherapy of ipilimumab and pembrolizumab, who developed asymptomatic erythematous patches on both legs. Histopathologic examination revealed a cutaneous interstitial granulomatous dermatitis. Notably, our patient did not require cessation of immunotherapy for these lesions, which subsequently remained stable, while the patient's melanoma remained controlled. This case expands the dermatologic toxicity profile of immune checkpoint blockade, as recognition of such toxicities is critical to optimal patient management.  相似文献   
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Myoclonus is a possible manifestation of mitochondrial disorders, and its presence is considered, in association with epilepsy and the ragged red fibers, pivotal for the syndromic diagnosis of MERRF (myoclonic epilepsy with ragged red fibers). However, its prevalence in mitochondrial diseases is not known. The aims of this study are the evaluation of the prevalence of myoclonus in a big cohort of mitochondrial patients and the clinical characterization of these subjects. Based on the database of the “Nation‐wide Italian Collaborative Network of Mitochondrial Diseases,” we reviewed the clinical and molecular data of mitochondrial patients with myoclonus among their clinical features. Myoclonus is a rather uncommon clinical feature of mitochondrial diseases (3.6% of 1,086 patients registered in our database). It is not strictly linked to a specific genotype or phenotype, and only 1 of 3 patients with MERRF harbors the 8344A>G mutation (frequently labeled as “the MERRF mutation”). Finally, myoclonus is not inextricably linked to epilepsy in MERRF patients, but more to cerebellar ataxia. In a myoclonic patient, evidences of mitochondrial dysfunction must be investigated, even though myoclonus is not a common sign of mitochondriopathy. Clinical, histological, and biochemical data may predict the finding of a mitochondrial or nuclear DNA mutation. Finally, this study reinforces the notion that myoclonus is not inextricably linked to epilepsy in MERRF patients, and therefore the term “myoclonic epilepsy” seems inadequate and potentially misleading. © 2014 International Parkinson and Movement Disorder Society  相似文献   
109.
The Charcot‐Marie‐Tooth disease Pediatric Scale (CMTPedS) is a Rasch‐built clinical outcome measure of disease severity. It is valid, reliable, and responsive to change for children and adolescents aged 3 to 20 years. The aim of this study was to translate and validate an Italian version of the CMTPedS using a validated framework of transcultural adaptation. The CMTPedS (Italian) was translated and culturally adapted from source into Italian by two experts in CMT with good English language proficiency. The two translations were reviewed by a panel of experts in CMT. The agreed provisional version was back translated into English by a professional translator. The definitive Italian version was developed during a consensus teleconference by the same panel. CMT patients were assessed with the final version of the outcome measure and a subset had a second assessment after 2 weeks to evaluate test‐retest reliability. Seventeen patients with CMT aged 5 to 20 years (eight female) were evaluated with the CMTPedS (Italian), and test‐retest was performed in three patients. The CMTPedS (Italian) showed a high test‐retest reliability. No patient had difficulty in completing the scale. The instructions for the different items were clearly understood by clinicians and therefore the administration of the outcome measure was straight forward and easily understood by the children assessed. The CMTPedS (Italian) will be used for clinical follow‐up and in clinical research studies in the Italian population. The data is fully comparable to that obtained from the English language version.  相似文献   
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