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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.
Prof. Dr. Doris Balogh 《European Surgery》1997,29(6):309-311
3.
G Balogh 《Orvosi hetilap》1991,132(38):2075-2080
Experiences obtained with 400 cases of thoracal empyema are described. The fact that the rate of lethality was 18.7% until present points out to the severity of the disease. The semi-conservative therapeutical methods used by the author (drainage, drainage and pleura lavage, pleura lavage and thoracoscopy, balance drainage) are presented. With their sensibly more frequent use the rate of operation could be reduced by 8% (from 36.5% to 28.5%) and the lethality rate by 7.6% (from 18.7% to 11.1). Further improvement of the results may be expected of the increased number of early detection (basic care), concentration of empyemic patients in special institutes (departments, wards) and due to it of a more efficient special treatment of uniform contemplation. 相似文献
4.
5.
R Balogh S Bond K Parker 《Nursing standard (Royal College of Nursing (Great Britain) : 1987)》1992,7(1):35-38
Increasing interest in audit in the health service has focused attention on the tools and methods used to achieve it. These can be extremely expensive, so a tendency to opt for off-the-shelf packages has developed. This article reviews how one such instrument, The Central Nottinghamshire Psychiatric Nursing Audit, has been used in two settings over the past three years, with particular emphasis on its cost-effectiveness. 相似文献
6.
The prolonged effect of nickel chloride and cadmium chloride on the rat fetal myocardium was studied experimentally administered to the pregnant mother through a gastric tube in doses of 12.5 mg/b. wt. It could be demonstrated that, due to nickel administration, changes simulating cardiomyopathy and severe mitochondrial lesions developed and the number of collagenous fibres and glycogen granules accumulated, while as a result of cadmium chloride administration, changes were apparent mainly in the endothelial cells, but with simultaneous mitochondrial impairments, too. Nickel and cadmium are contained by cigarette smoke. Based on experimental studies, authors propose new arguments on the damaging effect of smoking of pregnant women. 相似文献
7.
Attila Patocs Peter Gergics Katalin Balogh Miklos Toth Ferenc Fazakas Istvan Liko Karoly Racz 《BMC medical genetics》2008,9(1):29
Von Hippel-Lindau disease (VHL) is a rare autosomal dominant disease characterized by development of cystic and tumorous lesions
at multiple sites, including the brain, spinal cord, kidneys, adrenals, pancreas, epididymis and eyes. The clinical phenotype
results from molecular abnormalities of the VHL tumor suppressor gene, mapped to human chromosome 3p25-26. The VHL gene encodes two functionally active VHL proteins due to the presence of two translational initiation sites separated by
53 codons. The majority of disease-causing mutations have been detected downstream of the second translational initiation
site, but there are conflicting data as to whether few mutations located in the first 53 codons, such as the Pro25Leu could
have a pathogenic role. In this paper we report a large Hungarian VHL type 2 family consisting of 32 members in whom a disease-causing
AGT80AAT (Ser80Ile) c.239G>A, p.Ser80Ile mutation, but not the concurrent CCT25CTT (Pro25Leu) c.74C>T, p.Pro25Leu variant
co-segregated with the disease. To our knowledge, the Ser80Ile mutation has not been previously described in VHL type 2 patients
with high risk of pheochromocytoma and renal cell cancer. Therefore, this finding represents a novel genotype-phenotype association
and VHL kindreds with Ser80Ile mutation will require careful surveillance for pheochromocytoma. We concluded that the Pro25Leu
variant is a rare, neutral variant, but the presence such a rare gene variant may make genetic counseling difficult. 相似文献
8.
M E Sherman M L Silverman K Balogh S S Tan 《Archives of pathology & laboratory medicine》1987,111(8):732-736
Multilocular renal cyst is a pathologically distinctive lesion of uncertain pathogenesis that usually presents as a large abdominal mass in male infants or middle-aged women. We performed detailed light and electron microscopic studies of three nephrectomy specimens that contained multilocular cysts and attempted to correlate their morphology with various theories of pathogenesis. In one of our cases, a microscopic focus of clear cells that resembled renal cell adenocarcinoma was observed in the wall of one locule. Although such a finding has been reported previously, the lack of well-documented cases with metastases suggests that the prognosis for patients with this lesion remains uniformly excellent. 相似文献
9.
Mikls Miltnyi Andrew E. Czeizel Lidia Balogh Zoltn Detre 《American journal of medical genetics. Part A》1992,43(5):789-790
We describe two sibs with tetraectrodactyly and oligomeganephronic renal hypoplasia. The parents were unaffected. This syndrome of apparently autosomal recessive origin appears to be the first Mendelian form of the acrorenal developmental field defect identified so far. © 1992 Wiley-Liss, Inc. 相似文献
10.