全文获取类型
收费全文 | 806篇 |
免费 | 27篇 |
国内免费 | 4篇 |
专业分类
耳鼻咽喉 | 3篇 |
儿科学 | 50篇 |
妇产科学 | 3篇 |
基础医学 | 62篇 |
口腔科学 | 19篇 |
临床医学 | 35篇 |
内科学 | 82篇 |
皮肤病学 | 37篇 |
神经病学 | 23篇 |
特种医学 | 90篇 |
外科学 | 92篇 |
综合类 | 279篇 |
预防医学 | 23篇 |
眼科学 | 3篇 |
药学 | 11篇 |
1篇 | |
肿瘤学 | 24篇 |
出版年
2022年 | 5篇 |
2021年 | 10篇 |
2020年 | 7篇 |
2019年 | 4篇 |
2018年 | 6篇 |
2017年 | 6篇 |
2016年 | 6篇 |
2015年 | 26篇 |
2014年 | 24篇 |
2013年 | 25篇 |
2012年 | 25篇 |
2011年 | 25篇 |
2010年 | 62篇 |
2009年 | 48篇 |
2008年 | 46篇 |
2007年 | 44篇 |
2006年 | 47篇 |
2005年 | 31篇 |
2004年 | 44篇 |
2003年 | 20篇 |
2002年 | 30篇 |
2001年 | 27篇 |
2000年 | 22篇 |
1999年 | 24篇 |
1998年 | 27篇 |
1997年 | 32篇 |
1996年 | 29篇 |
1995年 | 19篇 |
1994年 | 23篇 |
1993年 | 12篇 |
1992年 | 12篇 |
1991年 | 6篇 |
1989年 | 5篇 |
1988年 | 6篇 |
1987年 | 5篇 |
1986年 | 7篇 |
1985年 | 5篇 |
1984年 | 3篇 |
1982年 | 6篇 |
1981年 | 4篇 |
1980年 | 2篇 |
1979年 | 1篇 |
1978年 | 5篇 |
1977年 | 2篇 |
1976年 | 5篇 |
1974年 | 1篇 |
1973年 | 1篇 |
1972年 | 1篇 |
1955年 | 1篇 |
1953年 | 1篇 |
排序方式: 共有837条查询结果,搜索用时 15 毫秒
11.
12.
13.
14.
Carlos R Ferreira Dillon Kavanagh Ralf Oheim Kristin Zimmerman Julian Stürznickel Xiaofeng Li Paul Stabach R Luke Rettig Logan Calderone Colin MacKichan Aaron Wang Hunter A Hutchinson Tracy Nelson Steven M Tommasini Simon von Kroge Imke AK Fiedler Ethan R Lester Gilbert W Moeckel Björn Busse Thorsten Schinke Thomas O Carpenter Michael A Levine Mark C Horowitz Demetrios T Braddock 《Journal of bone and mineral research》2021,36(5):942-955
Inactivating mutations in human ecto-nucleotide pyrophosphatase/phosphodiesterase-1 (ENPP1) may result in early-onset osteoporosis (EOOP) in haploinsufficiency and autosomal recessive hypophosphatemic rickets (ARHR2) in homozygous deficiency. ARHR2 patients are frequently treated with phosphate supplementation to ameliorate the rachitic phenotype, but elevating plasma phosphorus concentrations in ARHR2 patients may increase the risk of ectopic calcification without increasing bone mass. To assess the risks and efficacy of conventional ARHR2 therapy, we performed comprehensive evaluations of ARHR2 patients at two academic medical centers and compared their skeletal and renal phenotypes with ENPP1-deficient Enpp1asj/asj mice on an acceleration diet containing high phosphate treated with recombinant murine Enpp1-Fc. ARHR2 patients treated with conventional therapy demonstrated improvements in rickets, but all adults and one adolescent analyzed continued to exhibit low bone mineral density (BMD). In addition, conventional therapy was associated with the development of medullary nephrocalcinosis in half of the treated patients. Similar to Enpp1asj/asj mice on normal chow and to patients with mono- and biallelic ENPP1 mutations, 5-week-old Enpp1asj/asj mice on the high-phosphate diet exhibited lower trabecular bone mass, reduced cortical bone mass, and greater bone fragility. Treating the Enpp1asj/asj mice with recombinant Enpp1-Fc protein between weeks 2 and 5 normalized trabecular bone mass, normalized or improved bone biomechanical properties, and prevented the development of nephrocalcinosis and renal failure. The data suggest that conventional ARHR2 therapy does not address low BMD inherent in ENPP1 deficiency, and that ENPP1 enzyme replacement may be effective for correcting low bone mass in ARHR2 patients without increasing the risk of nephrocalcinosis. © 2021 American Society for Bone and Mineral Research (ASBMR). 相似文献
15.
16.
17.
18.
Aneurysm of sinus of Valsalva dissecting into interventricular septum is a rare entity. We report one such case who was incidentally diagnosed by echocardiography to have this abnormality during evaluation of a clinically suspected isolated aortic regurgitation.KEY WORDS: Aneurysm – dissecting – sinus of Valsalva, Echocardiography 相似文献
19.
Introduction
The reflex sympathetic dystrophy (RSD) syndrome usually shows a distally generalized distribution pattern of symptoms. Here we report a case with a distally localized form of RSD.Patient and methods
In a 53-year-old woman, following a local lesion in the nail bed of the left thumb, a neuroma developed at the side of the lesion during the next half year. She was finally operated upon. Following that intervention, a complex and painful clinical syndrome occurred that for the most part affected only the thumb. A clinical neurological examination was carried out, including distal suprasystolic compression of the affected extremity after bandaging it (the so-called ischemia test). For diagnostic and therapeutic reasons, afterwards a conventional blockade of the ipsilateral stellate ganglion was applied.Results
The clinical investigation showed a triad of autonomic (swelling, side difference of skin temperature, hyperhydrosis), motor (reduced movement ability, tremor) and sensory disturbances (spontaneous pain, allodynia), which nearly exclusively affected the entire left thumb. The spontaneous pain showed an orthostatic component (the pain being diminished or exaggerated when the extremity was elevated or lowered, respectively) and was suppressed by the ischemia test. Following the sympathetic block, all symptoms disappeared within one day (follow-up period: 5 months).Conclusion
In contrast to the common clinical picture of RSD, with a distally generalized distribution of symptoms, the present case showed a so-called localized form of RSD, its triad affecting only the thumb with the lesion. Typically, the pain showed an orthostatic component and was suppressed by the ischemia test. The sympathetic block was immediately successful, proving the occurrence of this form of RSD for the first time. In similar clinical cases, this form of RSD should be considered as a differential diagnosis. 相似文献20.