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991.
NM Brown RJ K?rner CE Zollman RP Martin MR Millar 《Archives of disease in childhood》1997,76(1):68-69
Two children with cyanotic congenital heart disease and Gram negative bacterial infection of prosthetic material after cardiac surgery were treated successfully with oral ciprofloxacin, initially in combination with netilmicin. The use of oral ciprofloxacin allowed prolonged outpatient treatment to be given, avoiding the need for intravenous access and early repeat surgery. 相似文献
992.
RJ Postlethwaite DM Eminson JM Reynolds AJ Wood S Hollis 《Archives of disease in childhood》1998,78(3):222-229
Growth and psychological functioning were studied in 30 patients with renal failure over a two year period following the offer of growth hormone treatment for significant short stature. Parents' concerns about growth decreased significantly during the study. Twenty eight parents (93%) accepted growth hormone treatment; most (74%) were satisfied with it and would opt for it again (89%). The views of these parents were unrelated to growth outcome in their child. This suggests that the positive responses were related more to the effort to improve growth than to any objective treatment success. In contrast children's reduction in concern about growth, satisfaction with treatment (36%), and decision to opt for growth hormone again (50%) were all significantly related to improvement in growth. Parents' reports of non-compliance increased significantly from 41% at 1 year to 91% at 2 years in the group as a whole. No significant changes were identified in maternal mental distress and no additional costs to the psychological health of the children seem to have resulted from the introduction of growth hormone treatment. A group of children was identified who accepted treatment but had continued poor growth. These appeared to be at particular risk of both physical problems and associated or consequent psychological difficulties. 相似文献
993.
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995.
Ofman R; Hettema EH; Hogenhout EM; Caruso U; Muijsers AO; Wanders RJ 《Human molecular genetics》1998,7(5):847-853
Rhizomelic chondrodysplasia punctata (RCDP) is a genetic disorder which is
clinically characterized by rhizomelic shortening of the upper extremities,
typical dysmorphic facial appearance, congenital contractures and severe
growth and mental retardation. Patients with RCDP can be subdivided into
three subgroups based on biochemical analyses and complementation studies.
The largest subgroup contains patients with mutations in the PEX7 gene
encoding the PTS2 receptor. This results in multiple peroxisomal
abnormalities which includes a deficiency of
acyl-CoA:dihydroxyacetonephosphate acyltransferase (DHAPAT),
alkyl-dihydroxyacetonephosphate synthase (alkyl-DHAP synthase), peroxisomal
3-ketoacyl-CoA thiolase and phytanoyl-CoA hydroxylase, although there are
differences in the extent of the deficiencies observed. Patients in the two
other subgroups have been reported to be either deficient in the activity
of DHAPAT (RCDP type 2) or alkyl-DHAP synthase (RCDP type 3) while no other
abnormalities could be observed. To examine whether the gene encoding
DHAPAT is mutated in patients with RCDP type 2, we determined the
N-terminal amino acid sequence of the enzyme isolated from human placenta.
Using this sequence as a query, we identified a 2040 bp open reading frame
(ORF) in the human database of expressed sequence tags. Expression of this
ORF in the yeast Saccharomyces cerevisiae showed that we have identified
the DHAPAT cDNA. The deduced amino acid sequence revealed no PTS2 consensus
sequence. In contrast DHAPAT appears to contain a putative PTS1 at the
extreme C-terminus. All RCDP type 2 patients analyzed were found to contain
mutations in their DHAPAT cDNA. This demonstrates that RCDP type 2 is the
result of mutations in DHAPAT.
相似文献
996.
Human tubal fluid: production, nutrient composition and response to adrenergic agents 总被引:1,自引:6,他引:1
Tay JI; Rutherford AJ; Killick SR; Maguiness SD; Partridge RJ; Leese HJ 《Human reproduction (Oxford, England)》1997,12(11):2451-2456
Vascularly perfused Fallopian tubes have been used to study the formation
and composition of human tubal fluid and the response to adrenergic agents.
An artery serving the tube was cannulated and perfused with Medium 199
supplemented with bovine serum albumin (BSA) and antibiotics. A second
cannula was attached to the fimbriated end for native tubal fluid
collection. The preparation was viable for up to 2 h. Tubal fluid was only
obtained in tubes removed in the proliferative and early secretory phases
of the ovarian cycle. Isoproterenol (1 mM) added to the perfusate
stimulated fluid production, whereas dibutyryl cyclic AMP (1 mM) reduced
fluid formation by 66%. Glucose, pyruvate and lactate concentrations in
tubal fluid, measured by microfluorescence assays, were 1.11, 0.14 and 5.4
mM respectively. The concentrations of 17 amino acids in tubal fluid were
measured by high performance liquid chromatography following fluorescence
derivatization. Arginine (0.19 mM) > alanine (0.11 mM) > glutamate
(0.09 mM) were present in highest concentration in all phases of the cycle.
All 17 amino acid concentrations in tubal fluid were below those in the
vascular perfusate. These data provides the basis for a culture medium
whose composition mimics the physiological environment to which early human
embryos are exposed.
相似文献
997.
Modupeola Diyaolu Jordan Taylor John Austin Michelle Gibson RJ Ramamurthi Ban Tsui Stephanie Chao 《Journal of pediatric surgery》2021,56(6):1190-1195
BackgroundUltrasound-guided (US) transversus abdominis plane (TAP) block is commonly utilized as part of a multi-modal approach for postoperative pain management. This study seeks to determine whether laparoscopic-guided TAP blocks are as effective as US-guided TAP blocks among pediatric patients.MethodIn this prospective, randomized controlled trial, pediatric patients undergoing laparoscopic procedures were randomly assigned to one of two treatment arms: US-guided TAP block (US-arm) or laparoscopic-guided TAP block (LAP-arm). Primary outcome was PACU pain scores. Secondary outcomes included PACU opioid consumption, block completion time and block accuracy.ResultsTwenty-five patients were enrolled in each arm. In the LAP-arm, 59% of blocks were in the transversus abdominis plane compared to 74% of TAP blocks in the US-arm (p = 0.18). Blocks were completed faster in the LAP-arm (2.1 ± 1.9 vs. 7.9 ± 3.4 min, p<0.001). The average highest PACU pain score was 3.4 ± 3.1 for the LAP-arm and 4.3 ± 3.8 for the US-arm (p = 0.37). Overall PACU pain scores and opioid consumption were similar between the groups (1.2 ± 1.3 vs. 1.6 ± 1.6, p = 0.24; 2.2 ± 5.8 vs. 0.9 ± 1.4MME, p = 0.26).ConclusionLaparoscopic TAP blocks have equivalent efficacy in post-operative pain scores, narcotic use, and tissue plane accuracy as compared to US-guided TAP blocks. They are also completed faster and may result in less operating room and general anesthetic time for the pediatric patient. 相似文献
998.
Thispaperdescribesthesurvivalexperiencefrom15selectedsitesofcancersaccordingtodatafromapopulation--basedcancerregistryduringtheperiodof1982--1991forevaluationofcancersurvivalaswellasdifferentcancercontrolmeasures.MATERIALSANDMETHODSDataCollectionCanc... 相似文献
999.
In vitro expansion and analysis of cloned cytotoxic T cells derived from patients with chronic T gamma lymphoproliferative disorders 总被引:3,自引:0,他引:3
Patients with T gamma lymphocytosis are a heterogeneous group, clearly distinguishable from other patients with chronic T lymphoproliferative disorders and usually without proven malignancy. We have attempted in vitro cloning of lymphocytes from three patients with an expansion of phenotypically and functionally different types of T gamma cells. One had T3+ B73.1+ T4 T8+ OKM1+ T gamma cells exerting antibody-dependent cellular cytotoxicity (ADCC) and natural killer (NK) cell cytotoxicity; another had T3+B73.1-T4-T8+OKM1-ADCC+NK- and a third had T3-B73.1+T4-T8- OKM1+ADCC+NK+ cells. On morphological characterization, most of the mononuclear cells of these patients resembled large granular lymphocytes (LGLs). Although lymphocytes of these patients showed almost no proliferative response capacity after stimulation with mitogens, they shared the capacity to proliferate after stimulation with Epstein-Barr virus-transformed lymphoblastoid B (B-LCL) feeder cells. Stable clones were established by this procedure. Clones from patient 3 exerted cytolytic activity against a broad spectrum of tumor cell lines, including fresh biopsy specimens of melanoma tumor target cells. All of these clones (termed activated killer [AK] cells) had the surface phenotype T3-, T4-, T8- or +, HNK1-, OKM1-, Lyt3+, WT1+ and showed ADCC in addition to AK cell cytotoxicity. Most of them were B73.1+ and expressed IgG-Fc receptors. They most likely belong to the T cell lineage, since they express IL2 receptors as recognized by the Tac antibody and did not bind monoclonal antibodies directed against monocytes or granulocytes. Thus lymphocytes with the functional and phenotypical characteristics of T gamma cells can be cloned and expanded in vitro from the peripheral lymphocytes of these patients by using the appropriate stimulus. Our results indicate that, of the heterogeneous population of NK cells, the T3- cells are more rapidly expanded than T3+ subsets. It is discussed whether or not our culture system might selectively induce proliferation in "normal" T cells rather than aberrant ones. 相似文献
1000.
Two frequent missense mutations in Pendred syndrome 总被引:8,自引:3,他引:8
Van Hauwe P; Everett LA; Coucke P; Scott DA; Kraft ML; Ris-Stalpers C; Bolder C; Otten B; de Vijlder JJ; Dietrich NL; Ramesh A; Srisailapathy SC; Parving A; Cremers CW; Willems PJ; Smith RJ; Green ED; Van Camp G 《Human molecular genetics》1998,7(7):1099-1104
Pendred syndrome is an autosomal recessive disorder characterized by early
childhood deafness and goiter. A century after its recognition as a
syndrome by Vaughan Pendred, the disease gene ( PDS ) was mapped to
chromosome 7q22-q31.1 and, recently, found to encode a putative sulfate
transporter. We performed mutation analysis of the PDS gene in patients
from 14 Pendred families originating from seven countries and identified
all mutations. The mutations include three single base deletions, one
splice site mutation and 10 missense mutations. One missense mutation
(L236P) was found in a homozygous state in two consanguineous families and
in a heterozygous state in five additional non-consanguineous families.
Another missense mutation (T416P) was found in a homozygous state in one
family and in a heterozygous state in four families. Pendred patients in
three non-consanguineous families were shown to be compound heterozygotes
for L236P and T416P. In total, one or both of these mutations were found in
nine of the 14 families analyzed. The identification of two frequent PDS
mutations will facilitate the molecular diagnosis of Pendred syndrome.
相似文献