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1.
Béla Nagy Zsolt Bene Zsolt Fejes Sonya L. Heltshe David Reid Nicola J. Ronan Yvonne McCarthy Daniel Smith Attila Nagy Elizabeth Joseloff György Balla János Kappelmayer Milan Macek Scott C. Bell Barry J. Plant Margarida D. Amaral István Balogh 《Journal of cystic fibrosis》2019,18(2):271-277
Background
We have recently shown that human epididymis protein 4 (HE4) levels correlate with the severity of cystic fibrosis (CF) lung disease. However, there are no data on how HE4 levels alter in patients receiving CFTR modulating therapy.Methods
In this retrospective clinical study, 3 independent CF patient cohorts (US-American: 29, Australian: 12 and Irish: 19 cases) were enrolled carrying at least one Class III CFTR CF-causing mutation (p.Gly551Asp) and being treated with CFTR potentiator ivacaftor. Plasma HE4 was measured by immunoassay before treatment (baseline) and 1–6?months after commencement of ivacaftor, and were correlated with FEV1 (% predicted), sweat chloride, C-reactive protein (CRP) and body mass index (BMI).Results
After 1?month of therapy, HE4 levels were significantly lower than at baseline and remained decreased up to 6?months. A significant inverse correlation between absolute and delta values of HE4 and FEV1 (r?=??0.5376; P?<?.001 and r?=??0.3285; P?<?.001), was retrospectively observed in pooled groups, including an independent association of HE4 with FEV1 by multiple regression analysis (β?=??0.57, P?=?.019). Substantial area under the receiver operating characteristic curve (ROC-AUC) value was determined for HE4 when 7% mean change of FEV1 (0.722 [95% CI 0.581–0.863]; P?=?.029) were used as classifier, especially in the first 2?months of treatment (0.806 [95% CI 0.665–0.947]; P?<?.001).Conclusions
This study shows that plasma HE4 levels inversely correlate with lung function improvement in CF patients receiving ivacaftor. Overall, this potential biomarker may be of value for routine clinical and laboratory follow-up of CFTR modulating therapy. 相似文献2.
Renal excretion of endothelin in children 总被引:1,自引:0,他引:1
István Máttyus L. B. Zimmerhackl A. Schwarz M. Brandis M. Miltényi T. Tulassay 《Pediatric nephrology (Berlin, Germany)》1997,11(4):513-521
Endothelin (ET) is a peptide with profound vasoconstrictive potential. First isolated from porcine endothelial cell supernatant,
it is produced also by smooth muscle, epithelial and circulating cells. Besides vasoconstriction, a wide spectrum of biological
activities of ET (via activation of membrane receptors) has been described. These include regulation of other hormones and
neurotransmitters, cellular growth and proliferation, bronchoconstriction, and, in the kidney, natriuresis and water diuresis.
ET exerts its effects mainly in an autocrine and paracrine fashion. A high concentration of ET is found in urine, compared
with plasma originating mainly from the kidney itself. In this review we focus on the role of urinary excretion of ET in children.
ET excretion was determined under different physiological and pathological conditions. In premature infants and newborns,
the daily excretion of ET (corrected for body surface) was higher than in older children; it was constant, and comparable
to the values in healthy adults after the age of 2 years. Renal ET excretion correlated positively with urine flow in both
healthy and sick children. Conditions with tubular and/or collecting duct cell damage, such as severe hypoxia, hemolytic-uremic
syndrome, renal transplantation, diabetes mellitus, chronic renal failure, and contrast media cytotoxicity were characterized
by elevated urinary excretion of ET. In conclusion, the renal excretion of ET is influenced by several factors, probably reflecting
the intrarenal ET production. ET has a low specificity with regard to renal injury.
Received May 7, 1996; received in revised form January 14, 1997; accepted January 17, 1997 相似文献
3.
L Madacsy A Yasar T Tulassay A Körner J Kelemen M Hóbor M Miltényi 《Acta paediatrica (Oslo, Norway : 1992)》1994,83(4):414-417
Twenty-four-hour blood pressure and heart rate measurements were carried out in 14 newly diagnosed diabetics and in 28 diabetics with 5–13 years' duration of the disease; 8 healthy children were used as controls. Mean arterial blood pressure increased at night in 5, decreased slightly (less than 10%) in 5 and decreased markedly (more than 10%) in 18 diabetics with longer duration of the disease. The diurnal-nocturnal differences in heart rates were significantly lower in diabetics with relative "nocturnal hypertension" compared to the control group ( p < 0.05). A significant negative correlation was found between maximal arterial blood pressure during physical exercise and the diurnal-nocturnal differences in mean arterial blood pressure in diabetics ( r =−0.58; p < 0.02). In conclusion, we found elevated nocturnal blood pressure in a subgroup of children with longer duration of diabetes and without increased albumin excretion. However, longitudinal studies of blood pressure profiles are needed to identify the candidates for diabetic vasculopathy among diabetic children. 相似文献
4.
Valerie J. Gillet Glenn Myatt Zsolt Zsoldos A. Peter Johnson 《Perspectives in Drug Discovery and Design》1995,3(1):34-50
Summary Several components of a system for structure generation are now well developed. HIPPO is a program that characterises a receptor binding site for potential target sites within the cavity that can be used in de novo design. The target sites include simple and complex hydrogen bonds, covalent bonds and bonds to metal ions. The SPROUT program for structure generation consists of two main components: the first is skeleton generation, followed by atom substitution to convert the solution skeletons to molecules. A new method of skeleton generation is presented here, where part skeletons are grown outwards from each target site. The part skeletons are then connected together to form solution skeletons. Finally the CAESA program is described, that ranks the output from SPROUT according to ease of synthesis. 相似文献
5.
Authors investigated the concentration changes of arginine vasopressin (AVP) and of atrial natriuretic peptide (ANP) in plasma. The renal characteristics of water- and sodium metabolism in the first 24 hours of the postoperative period in 11 children operated with acute appendicitis have been studied. Crystalloid infusion was applied in 60 ml/kg/24 h dose, in 5 cases however, 1 g/kg albumin vas also administered during the operation. In children getting crystalloid infusion (group "A") the initial AVP value was 29.9 +/- 8.8 pg/ml, which further increased to 58.2 +/- 15.2 pg/ml (p 0.05) by the 6. postoperative hour and even in the 24. hour it remained above the physiological value (19.9 +/- 5.3 pg/ml). During the observation time significant Na- and water retention developed. The albumin administration (group "B") stopped the further increase of AVP values, the Na-household remained in equilibrium and the water retention developed only in smaller degree. The ANP plasma concentration of both groups was in the normal range and did not change during observation time. Our data show that for the postoperative water retention first of all the increased AVP activity is responsible. The more intensive AVP secretion may be induced by the relative intravascular hypovolemia since by enhancing the plasma volume the further increase of postoperative hormone activity could be prevented. 相似文献
6.
Baldauf ZB 《Neuroscience letters》2005,383(1-2):109-114
The distribution pattern of SMI-32-immunoreactivity (SMI-32-ir) of neuronal elements was examined in the visual cortical areas of marmoset monkey. Layer IV of the primary visual cortex (V1) and layers III and V of the extrastriate areas showed the most abundant SMI-32-ir. The different areal and laminar distribution of SMI-32-ir allowed the distinction between various extrastriate areas and determined their exact anatomical boundaries in the New World monkey, Callithrix penicillata. It is shown here that the parcellating nature of SMI-32 described earlier in the visual cortical areas of other mammals - including Old World monkeys - is also present in the marmoset. Furthermore, a comparison became possible between the chemoanatomical organization of New World and Old World primates' visual cortical areas. 相似文献
7.
Effects of chemical mediators of anaphylaxis on ciliary function 总被引:2,自引:0,他引:2
Adam Wanner M.D. David Maurer Ph.D. William M. Abraham Ph.D. Zsolt Szepfalusi Marek Sielczak M.D. 《The Journal of allergy and clinical immunology》1983,72(6):663-667
We assessed the effects of selected chemical mediators of anaphylaxis on CBF in vitro. Ciliated epithelial cells were obtained from the trachea of conscious sheep with a cytology brush and suspended in a perfusion chamber containing KH. Ciliary activity was viewed microscopically and recorded on videotape for subsequent slow-motion analysis of CBF. Prostaglandin E1 (10(-8) M to 10(-6) M), prostaglandin E2 (10(-10) M to 10(-6) M), and leukotriene-C4 (10(-8) M) increased CBF between 7% and 33%. Histamine caused ciliostimulation only at the relatively high concentrations above 10(-5) M (7% increase in CBF), whereas prostaglandin F2 alpha (10(-10) M and 10(-6) M) was without effect. In no preparation was ciliary discoordination observed. These findings indicate that several chemical mediators of anaphylaxis stimulate CBF and that the previously described impairment of mucociliary transport in stable allergic asthma or antigen-induced bronchoconstriction is probably not caused by a primary alteration of ciliary function. 相似文献
8.
9.
Potent inhibition of HIV-1 entry by (s4dU)35 总被引:2,自引:0,他引:2
Horváth A Tokés S Hartman T Watson K Turpin JA Buckheit RW Sebestyén Z Szöllosi J Benko I Bardos TJ Dunn JA Fésüs L Tóth FD Aradi J 《Virology》2005,334(2):214-223
10.