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951.
Deepak Kumar Gupta Ashish Suri Ashok Kumar Mahapatra Veer Singh Mehta Ajay Garg Chitra Sarkar Faiz U. Ahmad 《Child's nervous system》2006,22(9):1194-1200
Objectives and importance Rosai–Dorfman disease (RDD) is a rare but distinctive entity of unknown etiology; isolated intracranial RDD is uncommon. Of 37 reported intracranial RDD cases, only three were reported in children.Clinical presentation We report an unusual case of a 15-year-old boy presenting with 4 months history of raised intracranial pressure with visual deterioration. Computed tomography and magnetic resonance imaging revealed bilateral petroclival enhancing lesions with cavernous sinus extension mimicking meningioma. However, histological examination was diagnostic of RDD.Intervention The patient underwent extended right-sided middle fossa approach and near-total tumor removal from petroclival region and cavernous sinus on both sides in two stages 6 weeks apart.Conclusion Ours is the first case of pediatric isolated intracranial RDD presenting with giant bilateral petroclival masses successfully managed with bilateral extended middle fossa approach in two stages. An optimal treatment for RDD is not established, but complete surgical resection alone seems effective. 相似文献
952.
953.
Longitudinal follow-up of bone mineral density in children with nephrotic syndrome and the role of calcium and vitamin D supplements. 总被引:1,自引:0,他引:1
Sanjeev Gulati Raj K Sharma Kiran Gulati Uttam Singh Arvind Srivastava 《Nephrology, dialysis, transplantation》2005,20(8):1598-1603
BACKGROUND: We previously have demonstrated that children with idiopathic nephrotic syndrome (INS) are at risk of metabolic bone disease (MBD). In this study, we report the longitudinal follow-up of these children and the role of calcium and vitamin D supplements. METHODS: We prospectively studied 100 consecutive children with INS. They were treated with prednisone. All were subjected to a baseline clinical, biochemical and radiological evaluation. They were initiated on calcium (500 mg/day) and vitamin D3 (200 IU/day) supplements, followed by a repeat assessment. The primary outcome measure was the Deltaz score (difference between the initial and final z scores) on dual energy X-linked absorptiometry (DEXA). A univariate and multivariate analysis using stepwise linear regression was performed for factors predictive of an improved Deltaz score. RESULTS: Of the 88 children that completed the study, the majority (n = 54) had improved bone mineral density (BMD) at the spine, and another 25 children had stable BMD on calcium and vitamin D3 supplements. The mean spinal BMD values were significantly better on follow-up (0.607+/-0.013 g/cm2) as compared with baseline values (0.561+/-0.010 g/cm2) (P<0.0001). The interval between initial and follow-up assessment was 1.5+/-0.07 years. Children who were on these supplements (n = 73) had a significantly improved z score as compared with those who did not receive them (n = 15) (P = 0.008). On multivariate analysis, the factors predictive of an improved z score were: younger age (P<0.0001), calcium and vitamin D3 supplement (P<0.0001), greater dietary calcium intake (P = 0.022) and lower interval steroid dose (P = 0.001). CONCLUSIONS: Children with greater steroid doses were likely to have low BMD on follow-up. Calcium and vitamin D supplements may help in improving BMD in children with INS. 相似文献
954.
In vivo quantification of serotonin transporters using [(11)C]DASB and positron emission tomography in humans: modeling considerations. 总被引:2,自引:0,他引:2
R Todd Ogden Ashish Ojha Kjell Erlandsson Maria A Oquendo J John Mann Ramin V Parsey 《Journal of cerebral blood flow and metabolism》2007,27(1):205-217
Positron emission tomography (PET) studies of the serotonin transporter (5-HTT) in the human brain are increasingly using the radioligand [(11)C]N, N-dimethyl-2-(2-amino-4-cyanophenylthio) benzylamine. A variety of models have been applied to such data in several published articles; however to date, these models have not been validated with test-retest data. We recruited 11 healthy subjects and conducted two identical scans on each subject on the same day. We considered four different models (one- and two-tissue compartment kinetic models, likelihood estimation in graphical analysis (LEGA; a bias-free alternative to the graphical method), and basis pursuit) along with fast noniterative approximations to the kinetic models. We considered four different outcome measures (total volume of distribution (V(T)), binding potential with (BP) and without (BP(1)), free-fraction adjustment, and specific-to-nonspecific equilibrium partition coefficient (BP(2))). To assess the performance of each model, we compared results using six different metrics (percent difference (PD) and within-subject mean sum of squares for reproducibility, interclass coefficient for reliability, variance across subjects, identifiability based on bootstrap resampling of residuals for each method, and time stability analysis to determine minimal required scanning time). We considered analysis of both at the voxel level and at the region of interest (ROI) level and compared results from these two approaches to assess agreement. We determined that 100 mins of scanning time is adequate and that for ROI-level analysis, LEGA gives best results. Average PD is 5.51 for V(T), 20.7 for BP, 17.2 for BP(1), and 16.5 for BP(2) across all regions. For voxel-level analysis we determined that the one-tissue compartment noniterative model is best. 相似文献
955.
Vaccination with autologous tumor-derived heat-shock protein gp96 after liver resection for metastatic colorectal cancer. 总被引:27,自引:0,他引:27
Vincenzo Mazzaferro Jorgelina Coppa Matteo G Carrabba Licia Rivoltini Marcello Schiavo Enrico Regalia Luigi Mariani Tiziana Camerini Alfonso Marchianò Salvatore Andreola Roberto Camerini Marco Corsi Jonathan J Lewis Pramod K Srivastava Giorgio Parmiani 《Clinical cancer research》2003,9(9):3235-3245
PURPOSE: Heat shock proteins (HSP) from tumor cells contain the gp96 polypeptide associated with cancer-specific antigenic peptides. Mice that are immunized with HSP/peptide-complex (HSPPC) derived from cancer tissue reject tumor from which HSPs are purified. We tested in humans whether vaccination with HSPPC-gp96 (Oncophage) from autologous liver metastases of colorectal carcinoma induces cancer-specific T-cell responses in patients rendered disease free by surgery. Experimental Design: Twenty-nine consecutive patients underwent radical resection of liver metastases [Memorial Sloan-Kettering Cancer Center (MSKCC) score 1-3 (good prognosis), 18 patients; score 4-5 (bad prognosis), 11 patients] and received autologous tumor-derived HSPPC-96. Two vaccine cycles were administered (four weekly injections followed by four biweekly injections after 8 weeks). Class-I HLA-restricted, anti-colon cancer lines T-cell response was measured by ELISPOT assay on peripheral blood mononuclear cells (PBMCs) obtained before and after vaccination. Feasibility, safety, and possible clinical benefits were also evaluated. RESULTS: Either a de novo induced or a significant increase of preexisting class I HLA-restricted T-cell-mediated anti-colon cancer response was observed in 15 (52%) of 29 patients. Frequency of CD3+, CD45RA+, and CCR7- T lymphocytes increased in immune responders. No relevant toxicity was observed. As expected, patients with good prognosis had a significantly better clinical outcome than those with poor prognosis [2-year overall survival (OS), 89 versus 64%, P = 0.001; disease-free survival (DFS), 46 versus 18%, P = 0.001]. Patients with immune response had a statistically significant clinical advantage over nonresponding subjects (2-year OS, 100% versus 50%, P = 0.001; DFS, 51% versus 8%, P = 0.0001). Occurrence of immune response led to better tumor-free survival, whatever the predicted prognosis was (hazard ratio, 0.11-0.12 with/without stratification; P = 0.0012-0.0003). CONCLUSIONS: HSPPC-96 vaccination after resection of colorectal liver metastases is safe and elicits a significant increase in CD8+ T-cell response against colon cancer. In this limited number of patients, two-year OS and DFS were significantly improved in subjects with postvaccination antitumor immune response, independently from other clinical prognostic factors. 相似文献
956.
957.
958.
Cerebral palsy—An etiological study 总被引:1,自引:0,他引:1
Five hundred and forty four cases of cerebral palsy were studied to find the etiology. Male to female ratio was 1.9∶1. Prenatal,
natal and postnatal factors were found in 42 (7.72%), 238 (43.75%) and 142 (26.1%) cases respectively. Only 79 (14.52%) cases
were found to have more than one factor which could have contributed to brain darnage. In 43 (7.9%) cases the prenatal, natal
and postnatal history were normal and the cause was not known. Toxemia (1.29%) cases and microcephaly (1.84%) cases were the
most common etiological factors in the prenatal category. Among the natal causes, birth anoxia was the most common etiological
factor and was observed in 24.45% cases. Infections of the central nervous system comprised the major etiopathogenetic factors
of the postnatal causes −11.95% cases had encephalitis, while 5.15% cases had meningitis. In cases where more than one etiology
was present, the most frequent causes were a combination of prematurity or birth anoxia in association with toxemia, antepartum
hemorrhage, prolonged labour, twins, forceps or caesarian delivery. Anoxia was consistently the most common etiological factor
in those cases of monoplegia paraplegia, quadriplegia, diplegia, and ataxia, i.e. in 0.55%, 1.29%, 11.76%, 6.07% and 0.55%
cases respectively. The present study reveals that majority of the cases were found to have natal or post natal etiology. 相似文献
959.
The design, synthesis, and biological evaluation of several unsaturated acyclonucleosides related to augustmycin A are described. The (propargyloxy)methyl acyclonucleoside analogues of 6-chloropurine, adenine, 6-methoxypurine, hypoxanthine, 6-mercaptopurine, and azathioprine have been prepared. The 9-[(propargyloxy)methyl]adenine (5) and 9-[(propargyloxy)methyl]hypoxanthine (12) analogues were converted to the corresponding 5'-tributylstannyl intermediates (9 and 13), respectively, which gave 9-[[[(Z)-5-iodo-5-propenyl]oxy]methyl]adenine (10) and 9-[[[(Z)-5-iodo-5-propenyl]oxy]methyl]hypoxanthine (14), respectively, after iododestannylation. The [125I]-radiolabeled congeners of 10 and 14 were prepared as potential metabolic markers. Among the unsaturated acyclonucleosides tested, 9-[(propargyloxy)methyl]-6-chloropurine (3), 9-[(propargyloxy)methyl]-6-mercaptopurine (15), 9-[(propargyloxy)methyl]azathioprine (17), and angustmycin A analogue 10 showed inhibition of cancer cell growth, but only at a minimal level, and 17 also showed 14% cancer cell death in vitro. Compound 10 provided approximately 50% protection against HIV at 10(-4) M concentrations. Biodistribution results of [125I]-10 in mice indicate that compound 10 is readily metabolized via deiodination in vivo, possibly by serving as a substrate for the enzyme S-adenosyl-L-homocysteine hydrolase. 相似文献
960.
Incubation of human placental aldose reductase (EC 1.1.1.21) with menadione (0.5-3.0 mM) resulted in time-dependent loss of the catalytic activity of the enzyme. Kinetic analysis of the data suggests that the inactivation process follows a single apparent rate constant that displays hyperbolic dependence on menadione concentration, indicating that menadione forms a kinetically significant, dissociable complex with the enzyme before the formation of an inactive enzyme-menadione complex. The inactivation of the enzyme with menadione was reversed upon dialysis of the inactivated enzyme against buffer containing 10 mM dithiothreitol suggesting that menadione reacts with enzyme sulfhydryl residue(s). Inactivation of the enzyme was significantly prevented by dithiothreitol (5 mM), NADPH (0.1 mM), and DL-glyceraldehyde (10 mM). Correlation of the fractional remaining activity with the extent of modification indicates that loss of catalytic activity corresponds to the modification of a single amino acid residue of the enzyme protein. Recombinant human aldose reductase, obtained by overexpression in Escherichia coli, and aldose reductase in which Cys-80 or Cys-303 was replaced by serine were also inactivated by menadione. However, enzyme in which Cys-298 was replaced by serine was insensitive to menadione. On the basis of these observations, it is suggested that menadione forms a thiodione-like adduct with Cys-298, leading to inactivation of the enzyme. 相似文献