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Broder K Reinhardt E Ahern J Lifton R Tamborlane W Pober B 《American journal of medical genetics》1999,83(5):356-360
Previous studies report conflicting frequencies of hypertension in cohorts of patients with Williams syndrome (WS). We studied blood pressure (BP) in WS using 24-hour ambulatory BP monitoring. This technique reliably measures day- and nighttime BP in a subject's natural environment and provides better prognostic information on long-term risks of hypertension than casual BP determinations. Twenty WS subjects evaluated through a multidisciplinary WS clinic and 35 age and gender-matched controls were studied. We found that WS subjects had significantly higher ambulatory BP than controls. After controlling for age, sex, and weight, the diagnosis of WS added approximately 10 mmHg to mean daytime and nighttime BPs. Hypertension, as defined by elevated mean daytime BP, was present in 40% of WS subjects versus 14% of controls (P < 0.05); among the children studied this difference was even more dramatic with 46% of WS children versus 6% of control children classified as hypertensive (P = 0.01). We also demonstrated normal diurnal BP variation but no evidence of a "white coat" effect or increased BP variability. Interestingly, parental reporting of a history of infantile hypercalcemia was strongly associated with the presence of hypertension (P = 0.008). Our data demonstrate that both children and adults with WS have higher mean BP and higher frequency of hypertension than healthy controls. Thus, elevated BP readings in the office setting should not be dismissed but require more thorough assessment. 相似文献
3.
Nelly Mauras Paul Mazaika Bruce Buckingham Stuart Weinzimer Neil H. White Eva Tsalikian Tamara Hershey Allison Cato Peiyao Cheng Craig Kollman Roy W. Beck Katrina Ruedy Tandy Aye Larry Fox Ana Maria Arbelaez Darrell Wilson Michael Tansey William Tamborlane Daniel Peng Matthew Marzelli Karen K. Winer Allan L. Reiss 《Diabetes》2015,64(5):1770-1779
Significant regional differences in gray and white matter volume and subtle cognitive differences between young diabetic and nondiabetic children have been observed. Here, we assessed whether these differences change over time and the relation with dysglycemia. Children ages 4 to <10 years with (n = 144) and without (n = 72) type 1 diabetes (T1D) had high-resolution structural MRI and comprehensive neurocognitive tests at baseline and 18 months and continuous glucose monitoring and HbA1c performed quarterly for 18 months. There were no differences in cognitive and executive function scores between groups at 18 months. However, children with diabetes had slower total gray and white matter growth than control subjects. Gray matter regions (left precuneus, right temporal, frontal, and parietal lobes and right medial-frontal cortex) showed lesser growth in diabetes, as did white matter areas (splenium of the corpus callosum, bilateral superior-parietal lobe, bilateral anterior forceps, and inferior-frontal fasciculus). These changes were associated with higher cumulative hyperglycemia and glucose variability but not with hypoglycemia. Young children with T1D have significant differences in total and regional gray and white matter growth in brain regions involved in complex sensorimotor processing and cognition compared with age-matched control subjects over 18 months, suggesting that chronic hyperglycemia may be detrimental to the developing brain. 相似文献
4.
William T. Cefalu Andrew J.M. Boulton William V. Tamborlane Robert G. Moses Derek LeRoith Eddie L. Greene Frank B. Hu George Bakris Judith Wylie-Rosett Julio Rosenstock Katie Weinger Lawrence Blonde Mary de Groot Matthew C. Riddle Robert Henry Sherita Hill Golden Stephen Rich Lyn Reynolds 《Diabetes care》2015,38(7):1177-1180
5.
Bone Mass and Strength in School‐Age Children Exhibit Sexual Dimorphism Related to Differences in Lean Mass: The Generation R Study 下载免费PDF全文
Carolina Medina‐Gomez Denise HM Heppe Jia‐Lian Yin Katerina Trajanoska André G Uitterlinden Thomas J Beck Vincent WV Jaddoe Fernando Rivadeneira 《Journal of bone and mineral research》2016,31(5):1099-1106
Bone strength, a key determinant of fracture risk, has been shown to display clear sexual dimorphism after puberty. We sought to determine whether sex differences in bone mass and hip bone geometry as an index of strength exist in school‐age prepubertal children and the degree to which the differences are independent of body size and lean mass. We studied 3514 children whose whole‐body and hip scans were measured using the same densitometer (GE‐Lunar iDXA) at a mean age of 6.2 years. Hip dual‐energy X‐ray absorptiometry (DXA) scans underwent hip structural analyses (HSA) with derivation of bone strength indices. Sex differences in these parameters were assessed by regression models adjusted for age, height, ethnicity, weight, and lean mass fraction (LMF). Whole‐body bone mineral density (BMD) and bone mineral content (BMC) levels were 1.3% and 4.3% higher in girls after adjustment by LMF. Independent of LMF, boys had 1.5% shorter femurs, 1.9% and 2.2% narrower shaft and femoral neck with 1.6% to 3.4% thicker cortices than girls. Consequent with this geometry configuration, girls observed 6.6% higher stresses in the medial femoral neck than boys. When considering LMF, the sexual differences on the derived bone strength indices were attenuated, suggesting that differences in muscle loads may reflect an innate disadvantage in bone strength in girls, as consequence of their lower muscular acquisition. In summary, we show that bone sexual dimorphism is already present at 6 years of age, with boys having stronger bones than girls, the relation of which is influenced by body composition and likely attributable to differential adaptation to mechanical loading. Our results support the view that early life interventions (ie, increased physical activity) targeted during the pre‐ and peripubertal stages may be of high importance, particularly in girls, because before puberty onset, muscle mass is strongly associated with bone density and geometry in children. © 2015 American Society for Bone and Mineral Research. 相似文献
6.
R Gruetter E J Novotny S D Boulware D L Rothman G F Mason G I Shulman R G Shulman W V Tamborlane 《Proceedings of the National Academy of Sciences of the United States of America》1992,89(3):1109-1112
Glucose is the main fuel for energy metabolism in the normal human brain. It is generally assumed that glucose transport into the brain is not rate-limiting for metabolism. Since brain glucose concentrations cannot be determined directly by radiotracer techniques, we used 13C NMR spectroscopy after infusing enriched D-[1-13C]glucose to measure brain glucose concentrations at euglycemia and at hyperglycemia (range, 4.5-12.1 mM) in six healthy children (13-16 years old). Brain glucose concentrations averaged 1.0 +/- 0.1 mumol/ml at euglycemia (4.7 +/- 0.3 mM plasma) and 1.8-2.7 mumol/ml at hyperglycemia (7.3-12.1 mM plasma). Michaelis-Menten parameters of transport were calculated to be Kt = 6.2 +/- 1.7 mM and Tmax = 1.2 +/- 0.1 mumol/g.min from the relationship between plasma and brain glucose concentrations. The brain glucose concentrations and transport constants are consistent with transport not being rate-limiting for resting brain metabolism at plasma levels greater than 3 mM. 相似文献
7.
Olivier WV van den Brink Andrew D Cochrane Franklin L Rosenfeldt Daniel J Penny Salvatore Pepe 《Journal of paediatrics and child health》2014,50(10):E63-E67
Background: Cardiac opioid peptides have been identified to exert important adaptive metabolic signalling for cardioprotection against ischaemia or hypoxia‐related injury. Aims: To determine myocardial methionine‐enkephalin content in children with hypoxemic congenital heart defects and to correlate myocardial content of methionine‐enkephalin with the extent of arterial oxygen desaturation. Methods: Children (n= 20, median age of 16 months), undergoing cardiac surgical repair (tetralogy of Fallot, 17/20), were included in this study. Arterial oxygen saturation was measured on admission. Myocardial samples obtained during surgery were assayed via radioimmunochemistry for methionine‐enkephalin content. Results: Greater methionine‐enkephalin content was measured in the right ventricles of the patients suffering from recent cyanotic spells compared with those with no recent spells (cyanotic spells: 2418 ± 844 pg/g wet weight tissue, n= 6; no spells: 1175 ± 189 pg/g wet weight tissue, n= 14, P= 0.04). An inverse correlation was evident between the arterial oxygen saturation and myocardial methionine‐enkephalin content. Conclusion: Myocardial methionine‐enkephalin levels increase with the severity of hypoxic stress in congenital cardiac disease and may play an important adaptive role in countering adrenergic over‐activity and related excess demand on myocardial metabolic capacity. 相似文献
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9.
【目的】 探讨儿童牙龈炎与恒牙龋的相关关系,为儿童牙龈炎防治工作提供理论依据。 【方法】 采用多阶段分层整群随机抽样的方法共调查广州市12~13岁儿童1 115人。检查方法和标准参考1997年WHO推荐的《口腔健康调查基本方法》,牙龈炎检查六颗指数牙,龋病检查全口恒牙,分别采用牙龈指数(GI)和DMFT记录检查结果。Logistic回归分析儿童牙龈炎与恒牙龋病的关系。 【结果】 广州市12~13岁儿童牙龈炎患病率72.65%,中、重度牙龈炎患病率分别为28.16%和5.83%。恒牙龋患病率为25.83%,其中未充填龋占龋齿的73.73%。Logistic回归分析显示未充填龋是广州市12~13岁儿童牙龈炎患病的危险因素。 【结论】 广州市12~13岁儿童牙龈炎患病率较高,有未充填龋存在的儿童是广州市儿童牙龈炎重点防治人群。 相似文献
10.
Collagen induced MMP-2 activation in human breast cancer 总被引:6,自引:0,他引:6
Erik W. Thompson Ming Yu Jorge Bueno Liang Jin Sourindra N. Maiti Fernando L. Palao-Marco Helena Pulyaeva Jeffrey W. Tamborlane Reza Tirgari Irene Wapnir Hala Azzam 《Breast cancer research and treatment》1994,31(2-3):357-370
Summary Matrix metalloproteinase-2 (MMP-2), a zymogen requiring proteolytic activation for catalytic activity, has been implicated broadly in the invasion and metastasis of many cancer model systems, including human breast cancer (HBC). MMP-2 has been immunolocalized to carcinomatous human breast, where the degree of activation of MMP-2 correlates well with tumor grade and patient prognosis. Using Matrigel assays, we have stratified HBC cell lines for invasivenessin vitro, and compared this to their potential for metastatic spread in nude mice. HBC cell lines expressing the mesenchymal marker protein vimentin were found to be highly invasivein vitro, and tended to form metastases in nude mice. We have further discovered that culture on collagen-I gels (VitrogenTM; Vg) induces MMP-2-activator in highly invasive but not poorly invasive HBC cell lines. As seen for other MMP-2-activator inducing regimens, this induction requires protein synthesis and an intact MMP-2 hemopexin-like domain, appears to be mediated by a cell surface activity, and can be inhibited by metalloproteinase inhibitors. The induction is highly specific to collagen I, and is not seen with thin coatings of collagen I, collagen IV, laminin, or fibronectin, or with 3-dimensional gels of laminin, Matrigel, or gelatin. This review focuses on collagen I and MMP-2, their localization and source in HBC, and their relationship(s) to MMP-2 activation and HBC metastasis. The relevance of collagen I in activation of MMP-2in vivo is discussed in terms of stromal cell: tumor cell interaction for collagen I deposition, MMP-2 production, and MMP-2-activation. Such cooperativity may existin vivo for MMP-2 participation in HBC dissemination. A more complete understanding of the regulation of MMP-2-activator by type I collagen may provide new avenues for improved diagnosis and prognosis of human breast cancer. 相似文献