全文获取类型
收费全文 | 213篇 |
免费 | 31篇 |
国内免费 | 2篇 |
专业分类
耳鼻咽喉 | 2篇 |
儿科学 | 7篇 |
妇产科学 | 11篇 |
基础医学 | 2篇 |
口腔科学 | 1篇 |
临床医学 | 32篇 |
内科学 | 73篇 |
皮肤病学 | 19篇 |
神经病学 | 49篇 |
特种医学 | 10篇 |
外科学 | 25篇 |
预防医学 | 9篇 |
眼科学 | 1篇 |
药学 | 2篇 |
肿瘤学 | 3篇 |
出版年
2023年 | 2篇 |
2021年 | 2篇 |
2020年 | 3篇 |
2018年 | 6篇 |
2017年 | 8篇 |
2016年 | 8篇 |
2015年 | 8篇 |
2014年 | 13篇 |
2013年 | 18篇 |
2012年 | 5篇 |
2011年 | 6篇 |
2010年 | 18篇 |
2009年 | 23篇 |
2008年 | 7篇 |
2007年 | 3篇 |
2006年 | 5篇 |
2004年 | 2篇 |
2003年 | 2篇 |
2001年 | 7篇 |
2000年 | 5篇 |
1999年 | 7篇 |
1998年 | 11篇 |
1997年 | 8篇 |
1996年 | 16篇 |
1995年 | 16篇 |
1994年 | 3篇 |
1993年 | 4篇 |
1992年 | 5篇 |
1991年 | 4篇 |
1990年 | 1篇 |
1989年 | 5篇 |
1988年 | 2篇 |
1987年 | 3篇 |
1986年 | 2篇 |
1985年 | 2篇 |
1984年 | 1篇 |
1979年 | 2篇 |
1977年 | 1篇 |
1970年 | 1篇 |
1966年 | 1篇 |
排序方式: 共有246条查询结果,搜索用时 31 毫秒
1.
Edel A. O'Toole MRCPI Patrick Deasy FRCPI † Rosemarie Watson FRCPI FACP 《Pediatric dermatology》1995,12(4):348-350
Abstract: We report a patient with cutis marmorata telangiectatica with the hitherto unreported anomaly of a double aortic arch. The presence of two major vascular anomalies in this patient may be secondary to a developmental defect of the mesoderm during embryogenesis. 相似文献
2.
3.
4.
Luke A. Massey MRCP Caroline Micallef MD FRCR Dominic C. Paviour PhD Sean S. O'Sullivan PhD MRCPI Helen Ling BScMed BMBS MSc David R. Williams PhD Constantinos Kallis PhD Janice L. Holton PhD FRCPath Tamas Revesz MD FRCPath David J. Burn MD FRCP Tarek Yousry Dr med Habil FRCR Andrew J. Lees MD FRCP Nick C. Fox PhD FRCP Hans R. Jäger MD FRCR 《Movement disorders》2012,27(14):1754-1762
Conventional magnetic resonance imaging (cMRI) is often used to aid the diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA), but its ability to predict the histopathological diagnosis has not been systematically studied. cMRI from 48 neuropathologically confirmed cases, including PSP (n = 22), MSA (n = 13), Parkinson's disease (PD) (n = 7), and corticobasal degeneration (n = 6), and controls (n = 9) were assessed blinded to clinical details and systematically rated for reported abnormalities. Clinical diagnosis and macroscopic postmortem findings were retrospectively assessed. Radiological assessment of MRI was correct in 16 of 22 (72.7%) PSP cases and 10 of 13 (76.9%) MSA cases with substantial interrater agreement (Cohen's kappa 0.708; P < .001); no PSP case was misclassified as MSA or vice versa. MRI was less sensitive but more specific than clinical diagnosis in PSP and both more sensitive and specific than clinical diagnosis in MSA. The “hummingbird” and “morning glory” signs were highly specific for PSP, and “the middle cerebellar peduncle sign” and “hot cross bun” for MSA, but sensitivity was lower (up to 68.4%) and characteristic findings may not be present even at autopsy. cMRI, clinical diagnosis, and macroscopic examination at postmortem have similar sensitivity and specificity in predicting a neuropathological diagnosis. We have validated specific radiological signs in pathologically confirmed PSP and MSA. However, the low sensitivity of these and macroscopic findings at autopsy suggest a need for imaging techniques sensitive to microstructural abnormalities without regional atrophy. © 2012 Movement Disorder Society 相似文献
5.
Sinéad M. Murphy MB BCh MRCPI Araya Puwanant MD the Consortium for Clinical Investigations of Neurological Channelopathies Inherited Neuropathies Consortium Consortia of the Rare Disease Clinical Research Network 《Annals of neurology》2012,72(4):481-490
Since the introduction of the Orphan Drug Act in 1983, designed to promote development of treatments for rare diseases, at least 378 orphan drugs have been approved. Incentives include financial support, tax credits, and perhaps most importantly, extended market exclusivity. These incentives have encouraged industry interest and accelerated research on rare diseases, allowing patients with orphan diseases access to treatments. However, extended market exclusivity has been associated with unacceptably high drug costs, both for newly developed drugs and for drugs that were previously widely available. We suggest that a paradoxical effect of orphan product exclusivity can be reduced patient access to existing drugs. In addition, the costs of each new drug are arguably unsustainable for patients and for the American health care system. Of all the specialties, neurology has the third highest number of orphan product designations, and neurological diseases account for at least one‐fifth of rare diseases. Citing the use of tetrabenazine for chorea in Huntington disease, adrenocorticotropic hormone for infantile spasms, and enzyme replacement therapy with alglucosidase alpha for Pompe disease, we highlight these paradoxical effects. ANN NEUROL 2012;72:481–490 相似文献
6.
7.
8.
Geraldine M. McCarthy MB MRCPI Assistant Professor of Medicine Dermot Kenny MB MRCPI Postdoctoral Fellow 《Seminars in arthritis and rheumatism》1992,21(6):368-375
Dietary fish oil supplementation can induce several metabolic changes relevant to rheumatic diseases. Both experimental and clinical evidence show that dietary fish oil supplementation modulates inflammatory and immune responses. Many studies have shown beneficial, albeit modest, effects in the treatment of rheumatoid arthritis. Studies in murine models of systemic lupus erythematosus have been encouraging, but few studies have been performed to assess the effects of dietary fish oil in the human disease or in other systemic rheumatic diseases. Further study on the efficacy of dietary fish oil supplementation in the treatment of specific rheumatic diseases is warranted. 相似文献
9.
10.
Abstract: We describe a 9-year-old boy with an Ro-positive, subacute, cutaneous lupus rash associated with homozygous C2 deficiency. His response to a mild topical steroid and sunscreen was excellent. Hereditary complement deficiency and its association with childhood lupus erythematosus are discussed. 相似文献