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Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long‐term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health‐care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long‐term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short‐term and long‐term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long‐term surveillance.  相似文献   
3.
BACKGROUND: Hemodialysis (HD) patients typically have reduced muscle mass and diminished functional capacity. The role of the muscle insulin-like growth factors (IGFs), a principal anabolic system that is involved in protein synthesis and that has downregulation that is implicated in muscle loss in animal models of uremia, has previously not been assessed in vivo in HD patients. METHODS: Seventeen HD patients were compared cross-sectionally with 17 age-, sex-, and body mass index-matched healthy controls. Body composition was assessed by dual energy x-ray absorptiometry and bioelectrical impedance spectrometry; functional capacity by hand grip strength, quadriceps strength, and 30-second sit-to-stand test; systemic inflammation by tumor necrosis factor-alpha (TNF-alpha) and TNF receptor 1 (TNFR1); serum and muscle IGF-I and IGFBP-3 by radioimmunoassay; and fragmentation of serum IGFBP-3 by Western immunoblotting. RESULTS: Appendicular lean mass was significantly decreased in HD patients compared with controls (17.6 +/- 0.9 versus 21.5 +/- 1.5 kg, P < .05), as were all measures of functional capacity (P < .01 to .001), and highly significant positive correlations between appendicular lean mass and functional capacity were evident (appendicular lean mass and hand-grip strength, quadriceps strength, 30-second sit-to-stand test, all P < .001). TNF-alpha and TNFR1 were elevated in patients (P < .001). Although serum IGF-I and IGFBP-3 levels did not differ between the groups (P = .295 and .379 respectively), fragmented IGFBP-3 levels were increased (53.1 +/- 16.0 versus 29.81 +/- 15.3%, P < .005). In contrast, muscle IGF-I was substantially diminished in the patient group (n = 7) relative to control (n = 5) levels (0.84 +/- 0.06 versus 2.78 +/- 1.80 pg/microg, P < .05). CONCLUSIONS: We provide evidence of reduced IGF-I in HD patients' skeletal muscle that may be a causal factor in the muscle wasting characteristic of this population. Future research should determine the exact consequences and causes of alterations to the muscle IGF system in HD patients.  相似文献   
4.
Hereditary hearing loss (HHL) is a very common disorder. When inherited in an autosomal recessive manner, it typically presents as an isolated finding. Interestingly and unexpectedly, in spite of extreme heterogeneity, mutations in one gene, GJB2, are the most common cause of congenital severe-to-profound deafness in many different populations. In this study, we assessed the contributions made by GJB2 mutations and chromosome 13 g.1777179_2085947del (the deletion more commonly known as del (GJB6-D13S1830) that includes a portion of GJB6 and is hereafter called Delta(GJB6-D13S1830)) to the autosomal recessive non-syndromic deafness (ARNSD) genetic load in Iran. Probands from 664 different nuclear families were investigated. GJB2-related deafness was found in 111 families (16.7%). The carrier frequency of the 35delG mutation showed a geographic variation that is supported by studies in neighboring countries. Delta(GJB6-D13S1830) was not found. Our prevalence data for GJB2-related deafness reveal a geographic pattern that mirrors the south-to-north European gradient and supports a founder effect in southeastern Europe.  相似文献   
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Induction of oxidative stress during the sperm preparation process for assisted reproductive techniques (ART) in men can weaken sperm parameters. Vitamin E (VE) is considered a factor in boosting male fertility. This experimental study (in vitro) aimed to assess the impact of VE supplementation on sperm quality and lipid peroxidation during sperm sampling at different times. For this mention, semen samples were collected from 50 asthenoteratozoospermic men. Samples were divided into control and test groups for 2, 4 and 6 hr that the test group was incubated with VE (2 mM). In two groups, total motility, progressive motility and viability based on the WHO 2010 criteria were assessed. Moreover, malondialdehyde (MDA) levels were evaluated in each group. In the control group, total and progressive motility and sperm viability were decreased significantly after 2 hr; however, MDA levels were increased significantly after 6 hr. Also, in the test group, sperm parameters were increased significantly after 2 hr, and MDA levels were decreased significantly after 6 hr compared to the control group. In outcome, in vitro VE supplementation may protect spermatozoa from the adverse effect of oxidative stress during sperm preparation via preservation antioxidant processes in normal condition.  相似文献   
7.
Purpose: Injuries are one of the leading causes of death and lead to a high social and financial burden. Injury patterns can vary significantly among different age groups and body regions. This study aimed to evaluate the relationship between mechanism of injury, patient comorbidities and severity of injuries. Methods: The study included trauma patients from July 2016 to June 2018, who were admitted to Sina Hospital, Tehran, Iran. The inclusion criteria were all injured patients who had at least one of the following: hospital length of stay more than 24 h, death in hospital, and transfer from the intensive care unit of another hospital. Data collection was performed using the National Trauma Registry of Iran minimum dataset. Results: The most common injury mechanism was road traffic injuries (49.0%), followed by falls (25.5%). The mean age of those who fell was significantly higher in comparison with other mechanisms (p < 0.001). Severe extremity injuries occurred more often in the fall group than in the vehicle collision group (69.0% vs. 43.5%, p < 0.001). Moreover, cases of severe multiple trauma were higher amongst vehicle collisions than injuries caused by falls (27.8% vs. 12.9%, p = 0.003). Conclusion: Comparing falls with motor vehicle collisions, patients who fell were older and sustained more extremity injuries. Patients injured by motor vehicle collision were more likely to have sustained multiple trauma than those presenting with falls. Recognition of the relationship between mechanisms and consequences of injuries may lead to more effective interventions.  相似文献   
8.
Investigations were performed to characterize a recombinant Kunitz protease inhibitory domain of the amyloid beta-protein precursor (rKPI) as anticoagulants. After a single intravenous infusion of wild type rKPI into dogs, its elimination fit a two compartment model with a t1/2alpha and t1/2beta of 5 and 77 min, respectively. Further investigations determined if a variant form of rKPI with 178-fold more potent anti-factor Xa activity (rKPI-DD135, Ki = 0.9 nM) could serve as an anticoagulant in a rabbit model of extracorporeal circulation using a venovenous shunt. A prospective investigation was initiated to compare standard heparin (n = 8) at 400 U/kg with different infusion concentrations of rKPI-DD135. After a single intravenous infusion of 1.89 mg/kg of rKPI-DD135 followed by a constant infusion at 0.003 (n = 3), 0.03 (n = 7), or 0.3 (n = 5) mg/kg/min, the anti-factor Xa activity of the animals' plasma rapidly reaches a steady state for the two lower infusion concentrations of the agent. All infusions of rKPI-DD135 prolong the activated clotting time with less variation than that seen with heparin administration. rKPI-DD135 anticoagulation does not prevent a drop in the platelet counts. Fibrinogen levels decrease only slightly when the circuit is anticoagulated with rKPI-DD135. rKPI-DD135 markedly prolongs the APTT, has little effect on the PT, and reduces plasma prekallikrein and plasminogen activation. The 0.3 mg/kg/min infusion concentration of rKPI-DD135 results in reduced deposition of 111Indium-labeled platelets on the circuit when compared to heparin. Last, after a steady state level is achieved, 60% of the plasma anti-factor Xa activity of rKPI-DD135 is eliminated within 60 min after stopping the infusion. These data show the rKPI-DD135 can provide single agent anticoagulation in a rabbit extracorporeal circuit. Development of short acting factor Xa inhibitors may be useful anticoagulants for cardiopulmonary bypass.  相似文献   
9.
Microbes are found in nearly every habitat and organism on the planet, where they are critical to host health, fitness, and metabolism. In most organisms, few microbes are inherited at birth; instead, acquiring microbiomes generally involves complicated interactions between the environment, hosts, and symbionts. Despite the criticality of microbiome acquisition, we know little about where hosts’ microbes reside when not in or on hosts of interest. Because microbes span a continuum ranging from generalists associating with multiple hosts and habitats to specialists with narrower host ranges, identifying potential sources of microbial diversity that can contribute to the microbiomes of unrelated hosts is a gap in our understanding of microbiome assembly. Microbial dispersal attenuates with distance, so identifying sources and sinks requires data from microbiomes that are contemporary and near enough for potential microbial transmission. Here, we characterize microbiomes across adjacent terrestrial and aquatic hosts and habitats throughout an entire watershed, showing that the most species-poor microbiomes are partial subsets of the most species-rich and that microbiomes of plants and animals are nested within those of their environments. Furthermore, we show that the host and habitat range of a microbe within a single ecosystem predicts its global distribution, a relationship with implications for global microbial assembly processes. Thus, the tendency for microbes to occupy multiple habitats and unrelated hosts enables persistent microbiomes, even when host populations are disjunct. Our whole-watershed census demonstrates how a nested distribution of microbes, following the trophic hierarchies of hosts, can shape microbial acquisition.

Microbial partners metabolize our food, fight off disease, and run the machinery that sustains the air we breathe, water we drink, and soil under our feet. Despite their importance, most host-associated microbes are generally not present at birth and are instead acquired (1). Because microbial symbionts can influence host health and fitness, the processes that determine how different microbiomes assemble within different hosts is a matter of active and urgent inquiry. Microbial ecologists have made great progress in determining how factors such as abiotic conditions (24), host evolution (5, 6), and microbial traits (79) shape environmental microbiomes, but considerably less is known about how surrounding environments or different guilds of host organisms contribute to host-associated microbiome composition. Longitudinal studies show that microbial richness accumulates and community composition changes over time across a wide diversity of hosts and habitats (1), but we know comparatively little about from where these microbes originate. To better understand microbial transmission and its role in community composition, we propose a framework that relies on theory from foodweb and landscape ecology.The concept of a foodweb has had a place in the ecological lexicon since at least the time of Elton (1927; (10)), and others such as Lindeman (11) and Odum (12) significantly expanded upon this notion to include how macroorganisms interact within their environments, in addition to their feeding relationships. The units of study for foodwebs are ecosystems, which are spatially explicit and include all organisms along with their abiotic environments and their interactions within its bounds (13). This definition was born from the efforts of the founders of the Hubbard Brook Ecosystem Study (HBES; 1963), who recognized that a watershed naturally delineates the boundaries of an ecosystem, an idea that parallels the Hawaiian ahupuaʻa concept. Since then, the HBES and its framework have led to numerous milestones in our understanding of processes such as the effects of long-term changes in acidification (14) and ecosystem impacts of global warming (15). Here, we adopt the notion of the watershed as an entire discrete ecosystem to better understand the landscape ecology of microbes. Landscape ecology is a means to understand how spatial processes affect biodiversity (16). In classic landscape ecology theory, the structure (heterogeneity) and fragmentation of habitats (or patches) within a matrix of otherwise inhospitable areas affect species’ dispersal ability and establishment. This ultimately shapes species’ abundance and distributions across the landscape (17). Contemporary landscape ecology theory extends this idea to include the concept of a landscape continuum, where continuous environmental variables, as opposed to discrete habitat patches surrounded by a matrix, better describe species’ distributions. Connecting these concepts, foodwebs are embedded in landscapes, and watersheds constitute a useful unit of measure to better understand their interactions.To expand concepts from foodweb and landscape ecology to be inclusive of microbes, we must first consider the following: a landscape for microbes can be both structural (e.g., different land covers or hydrology) and biotic (e.g., variation in the distribution of host populations). Also, microbes might better fit a continuous landscape model rather than a patch model if their distributions are not governed merely by the presence of a compatible host or habitat, but rather, if they exist among multiple hosts across a gradient of environmental conditions. This requires microbes to be generalists to some degree and/or a matrix that is at least partially hospitable (18). These considerations are important because while microbial transmission among related hosts is one obvious means of microbiome assembly, this model, in and of itself, is insufficient to sustain microbiomes (defined here as communities of bacteria and archaea) across a dynamic landscape. For example, many plants and animals are either sparse, seasonal, or ephemeral, requiring that their symbiotic microbes be capable of residing, at times, in alternate nearby hosts or environments. This potential for a microbe to persist in, and disperse among, hosts of different kingdoms and guilds, or even between liquid and land, is a trait with the potential to add an additional dimension to microbiome assembly theory (19). Where, then, might a host’s microbes reside when not inside that host? In addition, what factors might predict microbiome distributions among potentially interacting hosts and environments?Variability in matrix suitability and host specialization may result in differing microbial communities reflected in one of three nonmutually exclusive patterns, each of which leaves a diagnostic imprint on microbiome structure. If any host or environment has an equal likelihood of harboring microbes that are present in any other host or environment, we might expect host–microbe interaction networks that are randomly structured. Alternatively, if microbes are more likely to co-occur among related hosts or guilds, we might expect these to contain unique and specific consortia of microbes (modules) that are not found elsewhere in the interaction network. Finally, host–microbe interactions might be best characterized as stratified, resulting in a network topology in which microbial diversity is nested such that taxa-poor microbiomes are subsets of those that are taxa-rich. In this scenario, nonhost environmental matrices (e.g., soil, sediment, water) serve as reservoirs of broad microbial diversity that is subsequently, and hierarchically, partitioned into simpler microbiomes. While this concept is fairly intuitive, there are actually few, if any, studies that demonstrate transmission among environmental microbiomes and multiple hosts at ecosystem scales. Instead, many of the insights gleaned into assembly processes of microbiomes are owed to studies of single hosts, tractable model systems, or global syntheses (20). We address this gap by sampling microbiomes from aquatic, marine, and terrestrial foodwebs within a single watershed to examine the dynamics of sources and sinks of microbial diversity.Here, we present a microbial census of a model ecosystem metacommunity in which continental-scale environmental heterogeneity is recapitulated within a comparatively small watershed. Because of this, we can surmise the distribution limits of microbiomes across land, stream, and sea, a feat that would not be plausible in most other landscapes of similar size or environmental variability. From ridge to reef, our compact watershed spans a roughly 3.5 m rainfall differential, ∼27 times that encountered along the Mississippi, the largest watershed in continental North America. Also, our model ecosystem is located on the most isolated archipelago on the planet, making exogenous microbial inputs infrequent, if not unlikely. Furthermore, owing to parallels in environmental heterogeneity and foodweb structure across this compact watershed compared to others, our findings are potentially relevant for highly connected ecosystems that span substantially larger geographic areas.For example, a long-standing question in biogeography is the relationship between organisms’ local distributions and those at larger scales. Many factors influence the distributions of microbes, including their physiology, size, population density, and dispersal abilities (2123). A common assumption is that niche breadth should also predict the range size of an organism, since the ability to survive in broader environments, and to use a greater array of resources, should indicate the ability to occupy more habitats that occur over greater distances (24, 25). This is an important component of source and sink dynamics, because it suggests that local occupancy should predict global distributions. This relationship is seldom tested empirically, however, because small areas rarely contain, or are sampled for, broad climatic variability and host diversity. In the absence of phenotypic, genomic, or even well-resolved taxonomic information about the majority of the earth’s microbial biodiversity, geographic range is one of the few traits that can be directly inferred from short environmental DNA sequence reads. By examining our ecosystem-wide microbiome census within the context of the global survey of the Earth Microbiome Project (26), we assess the relationship between global and local microbial distributions.  相似文献   
10.
Depression, a leading cause of disability worldwide, is also the most prevalent psychiatric problem among Parkinson disease patients. Both depression and Parkinson disease are associated with microstructural anomalies in the brain. Diffusion tensor imaging techniques have been developed to characterize the abnormalities in cerebral tissue. We included 11 studies investigating brain microstructural abnormalities in depressed Parkinson''s disease patients. The included studies found alterations to essential brain structural networks, including impaired network integrity for specific cortical regions, such as the temporal and frontal cortices. Additionally, findings indicate that microstructural changes in specific limbic structures, such as the prefronto‐temporal regions and connecting white matter pathways, are altered in depressed Parkinson''s disease compared to non‐depressed Parkinson''s disease and healthy controls. There remain inconsistencies between studies reporting DTI measures and depression severity in Parkinson disease participants. Additional research evaluating underlying neurobiological relationships between major depression, depressed Parkinson''s disease, and non‐depressed Parkinson''s disease is required to disentangle further mechanisms that underlie depression and related somatic symptoms, in Parkinson disease.  相似文献   
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