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11.
The spondylometaphyseal dysplasias are a very heterogeneous group of disorders. Only one has been clearly defined, namely, the spondylometaphyseal dysplasia — Kozlowski type, known also as the common type. This disorder is inherited as an autosomal dominant trait. In this report we describe an Arabic-Moslem kindred with spondylometaphyseal dysplasia, not of the common type, with an autosomal dominant mode of inheritance, indicating further heterogeneity. 相似文献
12.
The time for reproduction in mammals largely depends on the availability of water and food in their habitat. Therefore, in regions where rains are limited to definite seasons of the year, mammals presumably will restrict their breeding correspondingly. But while mammals living in predictable ecosystems would benefit by timing their season to an ultimate predictable cue, such as photoperiod, in unpredictable ecosystems (e.g., deserts) they will need to use a more proximate signal. We suggest a mechanism by which water shortage (low water content in plants) could act as a proximate cue for ending the reproductive season. The golden spiny mouse (Acomys russatus), a diurnal rodent living in extreme deserts, may face an increased dietary salt content as the summer progresses and the vegetation becomes dry. Under laboratory conditions, increased diet salinity lead to reproductive hiatus in females, notable in imperforated vagina, and a significant decrease in the ovaries, uteri, and body masses. In females treated with vasopressin (VP), a hormone expressed during water stress, the uteri and body masses have decreased significantly, and the ovaries exhibited an increased number of atretic follicles. VP has also led to a significant decrease in relative medullary thickness (RMT) of the kidney. It is thus suggested that VP could act as a modulator linking the reproductive system with water economy in desert rodents, possibly through its act on the energetic pathways. 相似文献
13.
Langerhans' cells were studied in the epidermis of two patients with active Behçet's disease and compared with those in two normal controls. Ultrastructural morphology and the percentage of Langerhans' cells found were similar in patients' (1.88%) and the control epidermis (1.79%). The density of Langerhans' cells in adjacent sites of the same epidermis was not homogeneous, being in the range of 0.8-2.8% in Behçet's disease and 0.6-4% in the controls. In the controls, Langerhans' cells were distributed unevenly. Some were located near the basal layer of the epidermis while the rest were in the mid and upper layers. In Behçet's disease most Langerhans' cells were in the mid-epidermis, but some were immediately beneath the stratum granulosum. In the Behçet's disease epidermis the area occupied by Langerhans' cells was increased by about 25% and the number of granules found increased by about 44%. It is suggested that in Behçet's disease the Langerhans' cells are in a more active state. 相似文献
14.
Blumenfeld Zeev; Dirnfeld Martha; Gonen Yael; Abramovici Haim 《Human reproduction (Oxford, England)》1994,9(2):209-213
To investigate the effect of co-treatment with growth hormone(GH) for ovulation induction with human menopausal gonadotrophins(HMG) on conception, we compared the pregnancy rate and responseto co-treatment with GH versus HMG/human chorionic gonadotrophin(HCG) alone in a prospective, randomized, cross-over protocolof volation induction for either in-vivo or in-vitro fertilization(IVF). The main outcome measures were the amount of gonadotrophinused and conception. Co-treatment with GH was associated witha reduction of 30% in gonadotrophin requirement. In 24 clonidinenegative patients 14 pregnancies were achieved (58.3%) eitherin the GH/HMG/HCG cycle or in the succeeding one. GH co-treatmentdid not generate any pregnancy in eight clonidine positive patients.We conclude that growth hormone may increase the pregnancy ratewhen combined with HMG/HCG for ovulation induction, not onlyin the co-treatment cycle but also in the succeeding one. Thebeneficial, synergistic effect of GH co-treatment was detectedin clonidine negative but not in clonidine positive infertilepatients. 相似文献
15.
Behar D Schlesinger M Halle D Ben-Ami H Edoute Y Shahar E Kasis I Shihab S Elstein D Zimran A Mandel H 《American journal of medical genetics》2002,110(1):25-29
Deficiencies of terminal complement components, particularly the latter ones, are often detected because of increased susceptibility to Neisserial infections. Herein we document the first report of C7 deficiency among a highly inbred Arab population living in the lower Galilee region of Israel. Both biochemical and molecular analysis were performed on samples from infected survivors and parents of children who succumbed to Neisserial infections in a 4-year period. Only the index case who suffered recurrent infections and a sibling who had not suffered an infection during the outbreak were found to be C7-deficient. The mutation was found to be the one previously described to be prevalent among Israeli Jews of Moroccan ancestry (mutation G1135C). The implications of this finding are discussed in the context of family pedigree, the protective effect of complement deficiency, and the clinical outcome. 相似文献
16.
Chaim Brautbar Marian Moscovitz Tatyana Livshits Salim Haim Shoshana Hacham-Zadeh Haim A. Cohen Rafi Sharon David Nelken Tirza Cohen 《Tissue antigens》1980,16(3):238-243
Pemphigus vulgaris (PV) is relatively common in Jews. Three HLA antigens were significantly more frequent in 39 Israeli Jewish PV patients than in controls: A26 – 59% vs 20%; Bw38 – 61% vs 20%; and DRw4 – 90% vs 38%. The joint occurrence of A26–Bw38–DRw4 was observed in 46% of PV patients and in 10% of controls. Similar results were recently reported for Jews in the Los Angeles area. Yet, when our patient sample was grouped into Ashkenazi and non-Ashkenazi Jews, it was evident that each of the three antigens had a higher frequency both in Ashkenazi patients and controls as compared to non-Ashkenazim. The relative risk for DRw4 in Ashkenazim was 33.8 as compared to 14.4 in the total sample of Israeli PV patients. The phenotype A26–Bw38–DRw4 was present in 57% of Ashkenazi patients and in 13% of controls. Ashkenazi Jews have the highest prevalence of PV, and HLA associations were strongest with Ashkenazi PV patients. These associations were with three antigens, all of high frequency in that group. 相似文献
17.
Mayan O Pires A Neves P Capela F 《Applied occupational and environmental hygiene》1999,14(11):785-790
Shoe manufacturing is a traditional industry in northern Portugal. There are nearly 1500 factories that employ about 54,000 workers. Among the materials used in the shoe manufacturing process, that could be occupational hazards, are the adhesives, especially adhesives solutions based on organic solvents. The National Institute of Health (Oporto Branch) carried out a study of 100 factories in northern Portugal to study solvent exposure in shoe manufacturing. The surveyed population, (4615 workers) was young (mean age = 33 years; range = 18-45) and predominantly female (68.5%). The results from the air samples analyses show 20 different organic compounds existing in the workplaces. The more common compounds found were n-hexane, toluene, and acetone. The results of the study show that in 53.7 percent of the factories there are operations where the solvent exposure is significant and represents a health hazard to the workers. These operations are mainly gluing, waxing, and polishing stations. Occupational risk of exposure to solvents concerned 44.2 percent of the workers. This study points out the existence of inadequate ventilation in the workplaces and inappropriate personal protective equipment to prevent dermatitis risk from adhesives, wax, and polish. The organic solvents present in workplaces also increase fire risk. 相似文献
18.
Inositol is a simple polyol precursor in a second messenger system important in brain myo-insitol, the natural isomer, which
has been found to be therapeutically effective in depression, panic disorder, and obsessive-compulsive disorder in double-blind
controlled trials. Recently, epiinositol, an unnatural stereoisomer of myo-inositol, was found to have effects similar to
those of myo-inositol to reverse lithium-pilocarpine seizures. We measured the behavior of rats in an elevated plus maze model
of anxiety after chronic treatment of 11 daily intraperitoneal injections of epi-inositol, myo-inositol, or control solution.
Epi-inositol reduced anxiety levels of rats compared with controls, and its effect was stronger than that of myoinositol.
Lithium has been hypothesized to alleviate mania by reducing brain inositol levels. Inositol in brain derives from the second
messenger cycle, from new synthesis, or from diet via transport across the blood brain barrier. Because the first two are
inhibited by lithium, we propose that an inositol-free diet will augment lithium action in mania by enhancing restriction
of inositol. 相似文献
19.
Hodgkin disease: prediction of outcome with 67Ga scintigraphy after one cycle of chemotherapy 总被引:5,自引:0,他引:5
Front D Bar-Shalom R Mor M Haim N Epelbaum R Frenkel A Gaitini D Kolodny GM Israel O 《Radiology》1999,210(2):487-491
PURPOSE: To investigate gallium 67 scintigraphy performed early during treatment as a means to predict outcome and thus to optimize treatment of Hodgkin disease (HD) in the future. MATERIALS AND METHODS: Ninety-eight patients with HD were examined. Thirty-one patients underwent 67Ga scintigraphy after one chemotherapy cycle and 83 patients after a mean 3.5 cycles (range, 2-5 cycles). Sixteen patients underwent 67Ga scintigraphy both after one cycle and at midtreatment. Patients underwent whole-body scintigraphy and single photon emission computed tomography of the torso. Torso computed tomography (CT) was performed after a mean 3.5 cycles (range, 2-6 cycles). Failure-free survival was compared between patients with positive and patients with negative test findings (Kaplan-Meier method), and the significance of the difference was calculated. The association of failure-free survival with various prognostic clinical factors before treatment was compared (log-rank test univariate analysis). RESULTS: Failure-free survival differed significantly (P < .002) between patients with positive and patients with negative 67Ga scintigrams after one chemotherapy cycle but not at midtreatment. Failure-free survival was not significantly different between patients with positive and patients with negative CT scans at midtreatment. Twenty-two (92%) of 24 patients with negative 67Ga scintigrams after one cycle and 64 (82%) of 78 patients with negative scintigrams at midtreatment remained in complete response. In four (57%) of seven patients with positive 67Ga scintigrams after one cycle, treatment failed. CONCLUSION: 67Ga scintigraphy after one cycle of chemotherapy is a good early predictor of outcome of HD. 相似文献
20.
The anticoagulant effect of Vespa orientalis venom sac extract (VSE) was attributed to a proteolytic process, involving mainly coagulation factors VIII and IX [Joshua, H., Ishay, J., 1975. Toxicon 13, 11-20; Korenberg et al., 1988. Toxicon 26, 1169-1176]. Preliminary purification of the proteolytic activity showed the presence of three separate proteases. One of which, protease I, was purified. The purified enzyme migrated as a double band on sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS PAGE). The molecular weights of the bands, under reduced conditions were 42 and 44 kD. Both bands retained activity after the electrophoretic run. The enzyme hydrolyses bovine factor IX (BFIX), factor X (BFX) and prothrombin. The pH optimum for the degradation of BFIX was 7.0 and its isoelectric point is above pH 10. The amino acid composition of the protease was determined. 相似文献