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11.
12.
The GAP-related domain of tuberin, the product of the TSC2 gene, is a target for missense mutations in tuberous sclerosis 总被引:5,自引:0,他引:5
Maheshwar MM; Cheadle JP; Jones AC; Myring J; Fryer AE; Harris PC; Sampson JR 《Human molecular genetics》1997,6(11):1991-1996
Tuberous sclerosis is an autosomal dominant trait in which the
dysregulation of cellular proliferation and differentiation results in the
development of hamartomatous growths in many organs. The TSC2 gene is one
of two genes determining tuberous sclerosis. Inactivating germline
mutations of TSC2 in patients with tuberous sclerosis and somatic loss of
heterozygosity at the TSC2 locus in the associated hamartomas indicate that
TSC2 functions as a tumour suppressor gene and that loss of function is
critical to expression of the tuberous sclerosis phenotype. The TSC2
product, tuberin, has a region of homology with the GTPase activating
protein rap1GAP and stimulates the GTPase activity of rap1a and rab5a in
vitro. Here we show that the region of homology between tuberin and human
rap1GAP and the murine GAP mSpa1 is more extensive than previously reported
and spans approximately 160 amino acid residues encoded within exons 34-38
of the TSC2 gene. Single strand conformation polymorphism analysis of these
exons in 173 unrelated patients with tuberous sclerosis and direct
sequencing of variant conformers together with study of additional family
members enabled characterisation of disease associated mutations in 14
cases. Missense mutations, which occurred in exons 36, 37 and 38 were
identified in eight cases, four of whom shared the same recurrent change
P1675L. Each of the five different missense mutations identified was shown
to occur de novo in at least one sporadic case of tuberous sclerosis. The
high proportion of missense mutations detected in the region of the TSC2
gene encoding the GAP-related domain supports its key role in the
regulation of cellular growth.
相似文献
13.
Elastin point mutations cause an obstructive vascular disease, supravalvular aortic stenosis 总被引:7,自引:2,他引:7
Li DY; Toland AE; Boak BB; Atkinson DL; Ensing GJ; Morris CA; Keating MT 《Human molecular genetics》1997,6(7):1021-1028
Supravalvular aortic stenosis (SVAS) is an inherited obstructive vascular
disease that affects the aorta, carotid, coronary and pulmonary arteries.
Previous molecular genetic data have led to the hypothesis that SVAS
results from mutations in the elastin gene, ELN. In these studies, the
disease phenotype was linked to gross DNA rearrangements (35 and 85 kb
deletions and a translocation) in three SVAS families. However, gross
rearrangements of ELN have not been identified in most cases of autosomal
dominant SVAS. To define the spectrum of ELN mutations responsible for this
disorder, we refined the genomic structure of human ELN and used this
information in mutational analyses. ELN point mutations co-segregate with
the disease in four familial cases and are associated with SVAS in three
sporadic cases. Two of the mutations are nonsense, one is a single base
pair deletion and four are splice site mutations. In one sporadic case, the
mutation arose de novo. These data demonstrate that point mutations of ELN
cause autosomal dominant SVAS.
相似文献
14.
15.
Short-term therapy for recurrent abortion using intravenous immunoglobulins: results of a double-blind placebo-controlled Italian study 总被引:1,自引:3,他引:1
Perino A; Vassiliadis A; Vucetich A; Colacurci N; Menato G; Cignitti M; Semprini AE 《Human reproduction (Oxford, England)》1997,12(11):2388-2392
It is still unclear whether i.v. immunoglobulins (Ig) can facilitate the
reproductive prognosis of women who have suffered recurrent pregnancy loss.
We report the results of a multicentre placebo- controlled study on the
effect of Ig administration on pregnancy outcome in 46 women who had
suffered at least three recurrent miscarriages. All were screened to
exclude chromosomal or Mullerian abnormalities, the presence of antinuclear
antibodies, lupus anticoagulant (LA) or elevated titres of anticardiolipin
antibodies which may have revealed an underlying autoimmune problem. To
avoid a selection bias towards ongoing pregnancies, i.v. Ig or placebo were
administered between weeks 5 and 7 of gestation for 2 consecutive days as
soon as each woman knew she was pregnant and before embryonic heart
activity could be detected. A further infusion was administered at week 8
when ultrasonography confirmed an ongoing embryonic development. In all,
68% of the women who received Ig went to term versus 79% of those who
received a placebo (not significant), with no significant differences in
the pregnancy course or the perinatal outcome. These results suggest either
that women with recurrent miscarriages who have no recognized cause of
pregnancy loss have a good reproductive prognosis without any treatment or
that the emotional care associated with the administration of a placebo can
indirectly facilitate the progression of pregnancy.
相似文献
16.
John A. Wolfe Bruce E. Stuck Steven T. Schuschereba Leslie P. Fox 《Documenta ophthalmologica. Advances in ophthalmology》1985,59(3):277-299
A moderately severe thermal injury of the central cornea of 48 Dutch-belted rabbit eyes was produced with a carbon (CO2) laser. The lesions were photographed with a slit lamp (SL) camera immediately following the injury and at 1, 2, 4, 7, 14, 21, 30 and 60 days after the exposure. Lesion size, opaqueness, and depth were graded clinically by SL biomicroscopy at the same intervals. No significant differences were found (p 0.05) between groups of eyes treated with flurbiprofen (0.03%), prednisolone acetate (1%), and vehicle control four-times-a-day for three weeks following injury. Additionally, eyes were studied histopathologically at 3 and 60 days following injury by light and transmission electron microscopy, and clinically at 30 and 60 days by endothelial specular microscopy. Important clinical and histopathological findings included coagulative necrosis of the corneal epithelium, epithelial sloughing, fusion of stromal collagen, stromal edema and inflammatory cell infiltration, stromal scar formation, corneal thinning, endothelial hyperplasia and metaplasia, fibrinous anterior chamber reaction with hypopyon, and retrocorneal fibrous membrane formation. 相似文献
17.
The ileoanal J pouch: radiographic evaluation 总被引:1,自引:0,他引:1
Endorectal ileoanal pull-through offers an attractive alternative to proctocolectomy and ileostomy for patients with ulcerative colitis, Gardner syndrome, and familial polyposis. To our knowledge, a careful radiographic analysis of the ileum, ileal pouch, and ileoanal anastomosis after ileoanal pull-through has not been reported. Thirty-two patients with ulcerative colitis, Gardner syndrome, and familial polyposis underwent colectomy, mucosal proctectomy, and endorectal ileoanal pull-through of a 15-cm ileal "J" pouch and loop ileostomy. Twenty-five (78%) of 32 of all the pouches radiographically demonstrated spiral folds extending from the middle of the pouch to the pectinate line. Other radiographic features included a mesenteric mass effect, pseudopolyps, and a central lucency that indicated intrapouch sutures. Radiographs provide useful information in the postoperative management of the ileal pull-through. 相似文献
18.
Wieland D Stuck AE Siu AL Adams J Rubenstein LZ 《Evaluation & the health professions》1995,18(3):252-282
The authors recently published a meta-analysis of controlled trials of comprehensive geriatric assessment (CGA). The results supported the view that efficacy of CGA is strongly related to the patients, objectives, and basic design of CGA programs, and that particular program models and design features are associated with important health outcome improvements (e.g., survival, living at home, and functional improvement at follow-up). Present objectives include the outline of methods and how they were developed given the condition of the trial database and scientific context. Aspects of the approach, such as (a) survey of primary trialists to recover unpublished information and standardize data, (b) development of a program typology to guide the principal analysis, and (c) incorporation of program design features as covariates where statistical heterogeneity was detected, proved extremely useful, and have implications for other systematic reviews of similarly complex primary trials of new health care technologies, health services, and organizational interventions. 相似文献
19.
Barton JK Hammer DX Pfefer TJ Lund DJ Stuck BE Welch AJ 《Lasers in surgery and medicine》1999,24(3):236-243
BACKGROUND AND OBJECTIVE: Simultaneous irradiation and viewing of 10-120 microm cutaneous blood vessels were performed to investigate the effects of 2-micros 577-nm dye laser pulses. STUDY DESIGN/MATERIALS AND METHODS: A modified scanning laser confocal microscope recorded vessel response to different radiant exposures (J/cm2). Probit analysis determined the 50% probability ("threshold") radiant exposure necessary to cause embolized or partly occluding coagula, coagula causing complete blood flow stoppage, and hemorrhage. RESULTS: A statistically significant difference in the threshold radiant exposure existed for each damage category for blood vessels 10-30 microm in diameter, but not for larger vessels. For vessels over 60 microm, complete flow stoppage was unattainable; increasing laser pulse energy produced hemorrhage. In larger vessels, coagula often were attached to the superficial vessel wall while blood flowed underneath. Monte Carlo optical and finite difference thermal modeling confirmed experimental results. CONCLUSION: These results provide insight into the role of pulse duration and vessel diameter in the outcome of pulsed dye laser irradiation. 相似文献
20.
Black tea and mammary gland carcinogenesis by 7,12- dimethylbenz[a]anthracene in rats fed control or high fat diets 总被引:3,自引:1,他引:3
Epidemiological studies suggest that tea may reduce cancer risk, and in
laboratory rodents, chemopreventive effects of tea or purified extracts of
tea have been demonstrated in lung, gastrointestinal tract and skin. There
is some evidence of chemoprevention by tea in the mammary gland, but the
data are not conclusive. In order to evaluate more fully the possible
influence of black tea on 7,12-dimethylbenz[a]anthracene (DMBA)-induced
mammary gland tumors in the female S-D (Sprague-Dawley) rat, three large
studies were performed: experiment 1, tumorigenesis in rats fed AIN-76A
diet and given 25 mg/kg DMBA and 1.25 or 2.5% whole tea extract or water to
drink; experiment 2, tumorigenesis in rats given 15 mg/kg DMBA and the same
diet and fluids as in experiment 1; experiment 3, tumorigenesis in rats fed
control or HF (high fat, corn oil) diet and given 15 mg/kg DMBA and 2% tea
or water to drink. Tea was given throughout the experiment; DMBA was given
by gastric gavage at 8 weeks of age. There was no consistent effect of tea
on tumorigenesis in rats fed AIN-76A diet; there was, however, evidence in
experiment 3 of a reduction of tumorigenesis by tea in rats fed the HF
diet. In experiment 3, rats fed the HF diet and given water showed the
expected increase in tumor burden (number and weight) compared with rats
fed control diet. However, rats fed the HF diet and given 2% tea showed no
increase in tumor burden; their tumor burden was significantly lower than
in rats fed the HF diet and given water (P < 0.01) and was not different
from rats fed control diet and given water or tea. In addition, in
experiment 3, the number of malignant tumors per tumor- bearing rat was
increased by the HF diet in water-drinking rats (P < 0.01) but not in
tea-drinking rats. Therefore, it appears that tea partially blocked the
promotion of DMBA-induced mammary tumorigenesis by the HF diet.
相似文献