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991.
992.
Introduction – The relative frequencies of different ataxias vary among different ethnic and geographic groups. The aim of this study was to examine patients with cerebellar ataxia and find the occurrence of autosomal dominant and recessive cerebellar ataxias in the population of the southern and eastern parts of Norway and estimate its prevalence.
Materials and methods – Probands were systematically tested for spinocerebellar ataxia 1, 2, 3, 6 and Friedreich's ataxia. A total of 94 patients with ataxia were assessed.
Results – We registered 60 patients from 39 unrelated families with hereditary ataxias. One family with SCA2 (two patients), one family with Friedreich's ataxia (two patients), two patients heterozygote for Friedreich's ataxia and one metabolic ataxia were identified.
Conclusions – We have few Friedreich's ataxia and SCA 1,2,3 and 6 in our population. Prevalence in Oslo County was estimated at 2.2/100,000 for autosomal recessive and 3.0/100,000 for autosomal dominant ataxia, respectively. 相似文献
Materials and methods – Probands were systematically tested for spinocerebellar ataxia 1, 2, 3, 6 and Friedreich's ataxia. A total of 94 patients with ataxia were assessed.
Results – We registered 60 patients from 39 unrelated families with hereditary ataxias. One family with SCA2 (two patients), one family with Friedreich's ataxia (two patients), two patients heterozygote for Friedreich's ataxia and one metabolic ataxia were identified.
Conclusions – We have few Friedreich's ataxia and SCA 1,2,3 and 6 in our population. Prevalence in Oslo County was estimated at 2.2/100,000 for autosomal recessive and 3.0/100,000 for autosomal dominant ataxia, respectively. 相似文献
993.
994.
A K Burroughs L Marelli E Cholongitas P Manousou K Rolles V Karam V Delvart R Adam C Sabin 《Liver transplantation》2007,13(6):935-6; author reply 937
995.
Contin Educ Anaesth Crit 相似文献
996.
Quantification of Basal and Stimulated ROS Levels as Predictors of Islet Potency and Function 总被引:2,自引:0,他引:2
B. Armann M. S. Hanson E. Hatch A. Steffen L. A. Fernandez 《American journal of transplantation》2007,7(1):38-47
We have developed a luminol-based assay using intact islets, which allows for quantification of reactive oxygen species (ROS). In addition, an index capable of characterizing metabolic and mitochondrial integrity prior to transplantation was created based on the capacity of islets to respond to high glucose and rotenone (mitochondrial respiratory chain complex I inhibitor) by production of ROS. To validate this assay, lipid peroxidation and antioxidative defense capacity were evaluated by detection of malondialdehyde (MDA) levels and glutathione peroxidase activity (GPx), respectively. Also, flow cytometric analyses of ROS (dihydroethidine), apoptosis (Annexin V, active caspases), necrosis (Topro3), and mitochondrial membrane potential (JC-1) were done in parallel to correlate with changes in luminol-measured ROS. ATP/ADP ratios were quantified by HPLC and the predictive value of ROS measurement on islet functional potency was correlated with capacity to reverse diabetes in a streptozotocin-induced diabetic NOD.scid mouse model as well as in human transplant recipients. Our data demonstrate that levels of ROS in islets correlate with the percentage of apoptotic cells and their functional potency in vivo. The ROS indices following glucose and rotenone exposure are indicative of metabolic potency and mitochondrial integrity and can be used as surrogate markers to evaluate the quality of islets prior to transplantation. 相似文献
997.
998.
G. Burastero N. Sessarego G. Grappiolo C. Castellazzo S. Castello A. Pitto G. Cittadini M. Podesta G. Bovio M. Peresi E. Fulcheri F. Frassoni L. Spotorno 《Journal of orthopaedics and traumatology》2007,8(1):49-54
Mesenchymal stem cells (MSC), easily culture-expanded from bone marrow, can significantly enhance bone defect healing. Several
proteins, such as the bone morphogenetic proteins (BMPs) and in particular BMP-7, are involved in bone formation in vitro
and in vivo. In this preclinical study, we evaluated if the association of human MSC (hMSC) with BMP-7 had synergic action
on bone healing. Rat femoral defects (n=12) were treated with: autoclaved bone and mononucleated cells (MNC) as control group
G1; bone and hMSC, group G2; bone with BMP-7, group G3; bone and hMSC plus BMP-7, group G4. Defect regeneration was evaluated
with plain radiographs after 2, 4, 8 and 12 weeks and with histological analysis. We observed organized trabeculae bridging
between the osteotomic ends of the host bone in rats treated with the association of hMSC and rhBMP-7. These trabeculae, formed
by a core of devitalized tissue surrounded by osteoblasts, osteocytes and osteoclasts, were continuous with a cortical-like
structure of bony tissue. Such new bone formation of the group treated with the association of hMSC and rhBMP-7 (G4) was clearly
superior compared to rats treated with rhBMP-7 (G2) or hMSC (G3) alone, as shown by radiographic analysis and histological
study. The present study suggests that the association of hMSC and BMP-7 is more effective than hMSC or BMP-7 alone in the
healing of femoral defects in rats. Further studies with larger samples are required to confirm these results and to evaluate
the best dosage. 相似文献
999.
Philip E Benson C W Ian Douglas Michael V Martin 《American journal of orthodontics and dentofacial orthopedics》2004,126(3):325-330
The objective of this study was to investigate the effect of fluoridated elastomeric ligatures on the microbiology of local dental plaque in vivo. This randomized, prospective, longitudinal, clinical trial had a split-mouth crossover design. The subjects were 30 patients at the beginning of their treatment with fixed orthodontic appliances in the orthodontic departments of the Liverpool and the Sheffield dental hospitals in the United Kingdom. The study consisted of 2 experimental periods of 6 weeks with a washout period between. Fluoridated elastomers were randomly allocated at the first visit to be placed around brackets on tooth numbers 12, 11, 33 or 22, 21, 43. Nonfluoridated elastomers were placed on the contralateral teeth. Standard nonantibacterial fluoridated toothpaste and mouthwash were supplied. After 6 weeks (visit 2), the elastomers were removed, placed in transport media, and plated on agar within 2 hours. Nonfluoridated elastomers were placed on all brackets for 1 visit to allow for a washout period. At visit 3, fluoridated elastomers were placed on the teeth contralateral to those that received them at visit 1. At visit 4, the procedures at visit 2 were repeated. Samples were collected on visits 2 and 4. A logistic regression was performed, with the presence or absence of streptococcal or anaerobic growth as the dependent variable. A mixed-effects analysis of variance was carried out with the percentage of streptococcal or anaerobic bacterial count as the dependent variable. The only significant independent variables were the subject variable (P =<.001) for the percentage of streptococcal and anaerobic bacterial count and the visit variable for the percentage of streptococcal count (P =<.001). The use of fluoridated or nonfluoridated elastomers was not significant for percentage of either streptococcal (P =.288) or anaerobic count (P =.230). Fluoridated elastomers are not effective at reducing local streptococcal or anaerobic bacterial growth after a clinically relevant time in the mouth. 相似文献
1000.
The authors have studied hepatoprotective actions of new derivatives of 3-hydroxipyridine on an experimental model of toxic hepatitis (140 white mice). Mexidol and berlithion are choosed as the preparations of comparison. The method of light microscopy is used for the exploration of morphological changes. The cytolytic contents activity, catalase activity and the level of MDA have been determined in blood serum. The antitoxic effect is valued by the survival of the animals. It is found that all examined bonds is corrected morphological changes in toxic hepatitis and increased the index of animals survival, which is more expressed than the preparations of comparison. 相似文献