全文获取类型
收费全文 | 185篇 |
免费 | 15篇 |
专业分类
儿科学 | 4篇 |
妇产科学 | 1篇 |
基础医学 | 29篇 |
口腔科学 | 3篇 |
临床医学 | 14篇 |
内科学 | 47篇 |
皮肤病学 | 1篇 |
神经病学 | 42篇 |
特种医学 | 9篇 |
外科学 | 25篇 |
预防医学 | 9篇 |
眼科学 | 2篇 |
药学 | 4篇 |
中国医学 | 2篇 |
肿瘤学 | 8篇 |
出版年
2023年 | 1篇 |
2022年 | 6篇 |
2021年 | 8篇 |
2020年 | 6篇 |
2019年 | 7篇 |
2018年 | 10篇 |
2017年 | 5篇 |
2016年 | 4篇 |
2015年 | 14篇 |
2014年 | 9篇 |
2013年 | 5篇 |
2012年 | 20篇 |
2011年 | 16篇 |
2010年 | 11篇 |
2009年 | 6篇 |
2008年 | 12篇 |
2007年 | 6篇 |
2006年 | 10篇 |
2005年 | 8篇 |
2004年 | 3篇 |
2003年 | 4篇 |
2002年 | 3篇 |
2001年 | 1篇 |
2000年 | 2篇 |
1999年 | 2篇 |
1998年 | 1篇 |
1997年 | 1篇 |
1996年 | 5篇 |
1995年 | 4篇 |
1994年 | 1篇 |
1993年 | 1篇 |
1990年 | 1篇 |
1985年 | 1篇 |
1984年 | 2篇 |
1982年 | 1篇 |
1980年 | 1篇 |
1971年 | 1篇 |
1970年 | 1篇 |
排序方式: 共有200条查询结果,搜索用时 31 毫秒
1.
2.
Neuropathological Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases) 总被引:12,自引:0,他引:12
Herbert Budka Adriano Aguzzi Paul Brown Jean-Marie Brucher Orso Bugiani Filippo Gullotta Matti Haltia Jean-Jacques Hauw James W. Ironside Kurt Jellinger Hans A. Kretzschmar Peter L. Lantos Carlo Masullo Wolfgang Schlote Jun Tateishi Roy O. Weller 《Brain pathology (Zurich, Switzerland)》1995,5(4):459-466
Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human transmissible spongiform encephalopathies (prion diseases) are proposed for the following disease entities: CJD - sporadic, iatrogenic (recognised risk) or familial (same disease in 1st degree relative): spongiform encephalopathy in cerebral and/or cerebellar cortex and/or subcortical grey matter; or encephalopathy with prion protein (PrP) immuno-reactivity (plaque and/or diffuse synaptic and/or patchy/perivacuolar types). Gerstmann-Sträussler-Scheinker disease (GSS) (in family with dominantly inherited progressive ataxia and/or dementia): encephalo(myelo)pathy with multicentric PrP plaques. Familial fatal insomnia (FFI) (in member of a family with PRNP178 mutation): thalamic degeneration, variable spongiform change in cerebrum. Kuru (in the Fore population). Without PrP data, the crucial feature is the spongiform change accompanied by neuronal loss and gliosis. This spongiform change is characterised by diffuse or focally clustered small round or oval vacuoles in the neuropil of the deep cortical layers, cerebellar cortex or subcortical grey matter, which might become confluent. Spongiform change should not be confused with non-specific spon-giosis. This includes status spongiosus (“spongiform state”), comprising irregular cavities in gliotic neuropil following extensive neuronal loss (including also lesions of “burnt-out” CJD), “spongy” changes in brain oedema and metabolic encephalopathies, and artefacts such as superficial cortical, perineuronal, or perivascular vacuolation; focal changes indistinguishable from spongiform change may occur in some cases of Alzheimer's and diffuse Lewy body diseases. Very rare cases might not be diagnosed by these criteria. Then confirmation must be sought by additional techniques such as PrP immunoblotting, preparations for electron microscopic examination of scrapie associated fibrils (SAF), molecular biologic studies, or experimental transmission. 相似文献
3.
Fabrizio Tagliavini Giorgio Giaccone Orso Bugiani Blas Frangione 《Acta neuropathologica》1993,85(3):267-271
Summary Here ditary cerebral hemorrhage with amyloidosis Dutch type (HCHWA-D) is characterized clinically by recurrent strokes and pathologically by deposition of amyloid (A) in cerebral vessel walls and, to a lesser extent, in the neuropil. Distinct from Alzheimer's disease, amyloid formation in HCHWA-D is not associated with neurofibrillary changes. Since a central issue in the pathophysiology of Alzheimer's disease and related conditions is the role of A in the neurodegencrative process, we investigated HCHWA-D brains for the presence of neuritic abnormalities using antibodies to ubiquitin and to phosphorylated neurofilaments. The study showed that amyloid deposits in the vessel walls and in the neuropil were surrounded by abnormal ubiquitinated neurites, suggesting that A deposition induces neuritic changes.Supported by the Italian Ministry of Health. Department of Social Services, and by N.I.H. Grants AG05891 and AG08721 (to B.F.) 相似文献
4.
Luigi Vetrugno MD Tiziana Bove MD Daniele Orso MD Federico Barbariol MD Flavio Bassi MD Enrico Boero MD Giovanni Ferrari MD Robert Kong MD FRCA EDIC 《Echocardiography (Mount Kisco, N.Y.)》2020,37(4):625-627
Lung ultrasound (LU) has rapidly become a tool for assessment of patients stricken by the novel coronavirus 2019 (COVID-19). Over the past two and a half months (January, February, and first half of March 2020) we have used this modality for identification of lung involvement along with pulmonary severity in patients with suspected or documented COVID-19 infection. Use of LU has helped us in clinical decision making and reduced the use of both chest x-rays and computed tomography (CT). 相似文献
5.
Luigia Di Francesco Melania Dovizio Annalisa Trenti Emanuela Marcantoni Ashleigh Moore Peadar O’Gaora Cathal McCarthy Stefania Tacconelli Annalisa Bruno Sara Alberti Salvatore Gizzo Giovanni Battista Nardelli Genny Orso Orina Belton Lucia Trevisi Dan A Dixon Paola Patrignani 《British journal of pharmacology》2015,172(18):4575-4587
6.
Beatrice Orso PsyD Dario Arnaldi MD Nicola Girtler PsyD Andrea Brugnolo PsyD Elisa Doglione PsyD Pietro Mattioli MD Erica Biassoni MD Roberto Fancellu MD Federico Massa MD Matteo Bauckneht MD Silvia Chiola MD Silvia Morbelli MD Flavio Nobili MD Matteo Pardini MD 《Movement disorders》2021,36(10):2293-2302
7.
Luca Cantarini Donato Rigante Giampaolo Merlini Antonio Vitale Francesco Caso Orso Maria Lucherini Paolo Sfriso Bruno Frediani Leonardo Punzi Mauro Galeazzi Rolando Cimaz Laura Obici 《Seminars in arthritis and rheumatism》2014
Objective
To analyze the clinical manifestations and response to treatment in a cohort of adult patients presenting with recurrent inflammatory attacks and carrying low-penetrance TNFRSF1A variants, as well as to provide data on their long-term follow-up.Methods
We performed a retrospective chart review of 36 patients carrying low-penetrance TNFRSF1A variants. Moreover, 60 genetically negative patients treated for recurrent inflammatory attacks and 13 patients with structural TNFRSF1A mutations were also analyzed. Detailed demographic and clinical data were collected at the time of molecular screening and at each follow-up visit. Treatments and markers of inflammation were also assessed.Results
Individuals with low-penetrance TNFRSF1A variants have a lower family history for inflammatory attacks and present with a later disease onset compared with patients with structural mutations, but do not differ, in this respect, with genetically negative individuals. Moreover, low-penetrance variants are less frequently associated with a chronic disease course, with clinical manifestations such as abdominal pain and myalgia, and with amyloidosis. A distinctive clinical feature is a higher rate of pericarditis. Interestingly, mutation-negative patients were found to present with a significant history of recurrent pharyngitis during childhood. Patients with low-penetrance variants are mostly managed with short courses of steroids or non-steroidal anti-inflammatory drugs on attacks. Although the need for a biological treatment is significantly lower compared with patients with structural mutations, still approximately 20% of individuals with recurrent inflammatory attacks carrying low-penetrance variants ultimately require these therapies.Conclusions
Our study confirms that low-penetrance TNFRSF1A variants can be associated with an autoinflammatory phenotype. Although a chronic disease course is rarely observed, some patients ultimately benefit from a biological treatment. 相似文献8.
Luca Cantarini Orso Maria Lucherini Rolando Cimaz Donato Rigante Cosima Tatiana Baldari Franco Laghi Pasini Mauro Galeazzi 《Rheumatology international》2012,32(12):4015-4018
Tumor necrosis factor receptor-1?Cassociated periodic syndrome (TRAPS) is the most common autosomal dominant autoinflammatory disorder and is caused by mutations in the TNFRSF1A gene encoding the 55-kDa receptor for tumor necrosis factor (TNF)-??. TRAPS is characterized by recurrent attacks of fever, typically lasting from 1 to 3?weeks. In addition to fever, common clinical features include periorbital edema, a migratory erythematous plaque simulating erysipela with underlying myalgia, and arthralgia or arthritis. Serosal membrane inflammation is also a common feature, usually in the form of polyserositis. To date, at least 40 different TNFRSF1A mutations have been identified, but few patients with symptoms highly suggestive of TRAPS with no mutations in the TNFRSF1A gene have recently been described, thus suggesting that not all mutations are yet known or that alternative mechanisms might be involved in the pathogenesis of the disease. We report on three such patients here. 相似文献
9.
Luca?CantariniEmail author Orso?Maria?Lucherini Gabriele?Simonini Mauro?Galeazzi Cosima?Tatiana?Baldari Rolando?Cimaz 《Rheumatology international》2012,32(2):465-467
Systemic juvenile idiopathic arthritis (SJIA) is a disorder characterized by arthritis in children starting before 16 years
of age associated with daily high fever, persisting for more than 2 weeks, and at least one of the following clinical features:
evanescent cutaneous rash, lymphadenopathy, serositis or hepatosplenomegaly. SJIA patients carry a significantly higher frequency
of MEFV mutations, the gene responsible for familial Mediterranean fever, and may be characterized by a more aggressive disease.
In this line, we describe a 9-year-old girl affected with SJIA who carried a heterozygous G196W mutation in MEFV. Our patient was characterized by an aggressive disease course, resistance to conventional immunosuppressive agents and developed
renal amyloidosis just 2 years after the disease onset. 相似文献
10.
Baldasseroni S Urso R Maggioni AP Orso F Fabbri G Marchionni N Tavazzi L;IN-CHF Investigators 《Nutrition, metabolism, and cardiovascular diseases : NMCD》2012,22(5):442-448
Background and aimsIn the field of cardiovascular diseases, elevated levels of serum uric acid (UA) reflect a marked activation of the xanthine oxidase pathway with increase in free radicals production; it is often associated with an inflammatory state, oxygen consumption and endothelial dysfunction. All these associations have been also confirmed in heart failure (HF) but the pathophysiological role of UA in this setting is not well understood. The aim of this study was to evaluate the prognostic role of UA in outpatients enrolled in the Italian Registry of Congestive Heart Failure (IN-CHF).Methods and resultsAll patients met the European Society of Cardiology (ESC) criteria for diagnosis of HF. We considered patients with complete clinical data and UA level available at the baseline and at 1-year follow-up. The study population was composed of 877 patients aged 63 ± 12 years. One-year mortality was 10.8% and dead patients had a higher level of UA than survivors (7.1 mg dl?1 vs 6.6 mg dl?1, p < 0.0207). In multivariable full model of analysis, UA did not result in an independent predictor of death in overall population, but only in patients with low body mass index (BMI) (≤22 kg m?2) (hazard ratio (HR): 2.38, 95% confidence interval (CI) 1.36–4.18). In this subgroup, a statistically significant gradual relationship between UA and survival was detected starting from values higher than 8 mg dl?1.ConclusionElevated level of UA is not an independent predictor of mortality in chronic HF, but it markedly worsens outcome if associated with low level of BMI. This association is likely an indicator of chronic inflammatory and catabolic state. 相似文献