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1.
Chelating drugs and chelator metal complexes are used for the prevention, diagnosis and treatment of cancer. Cancer cells and normal cells require essential metal ions such as iron, copper and zinc for growth and proliferation. Chelators can target the metabolic pathways of cancer cells through the control of proteins involved in the regulation of these metals and also of other molecules involved in cell cycle control, angiogenesis and metastatic suppression. Other targets include the inhibition of specific proteins such as ribonucleotide reductase involved in DNA synthesis, the inhibition of free radical damage on DNA caused by iron and copper catalytic centers, the inhibition of microbial growth in immuno compromised cancer patients and the decorporation of radioactive and other toxic metals causing cancer. Chelating drugs and metal ions can affect the metabolism, efficacy and toxicity of anti-cancer drugs such as doxorubicin, mitozantrone, bleiomycin and hydroxyurea (HU). Although many experimental chelators have been shown to be effective as anti-cancer agents, only a few, e.g., dexrazoxane, deferoxamine (DFO) and triapine, have reached the stage of clinical testing or application. In many experimental models, deferiprone (L1) has been shown to be effective in cancer prevention and treatment, and in the inhibition of doxorubicin-induced cardiotoxicity. New anti-cancer drugs could be developed using chelators and chelator complexes with platinum and other metals, and also new protocols of combinations of chelators with known anti-cancer drugs. 相似文献
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Aglaia Vignoli Lucio Giordano Daniela Guerra Francesca La Briola Patrizia Accorsi Miriam Nella Savini Alessandro Iodice Anna Molinaro Maria Paola Canevini 《Epileptic Disord》2014,16(4):433-438
Since the first cases of abnormal paroxystic movements in normal infants were described, the importance of accurate characterization of this medical condition has been increasingly confirmed in the literature. Non‐epileptic attacks mimic epileptic paroxysms in clinical presentation, but they have a typically benign course and are unresponsive to pharmacological treatment. An evident feature of the syndrome is its extreme variability in clinical manifestation. Here, we describe three normal infants with two similar forms of non‐epileptic paroxysms. Electroclinical manifestations and profile of evolution were investigated. Ictal video‐EEG polygraphic recordings were obtained for each patient. The increasing number of such reported clinical cases in the literature may contribute to high quality systematic reviews and the development of useful guidelines in the future. The clinical heterogeneity of non‐epileptic attacks, together with the relative rarity of the condition, may make differential diagnosis with epileptic attacks very challenging. [Published with video sequences] 相似文献
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Suzan?DijkinkEmail author Pieta?Krijnen Aglaia?Hage Gwendolyn?M.?Van der?Wilden George?Kasotakis Dennis?den?Hartog Ali?Salim J.?Carel?Goslings Frank?W.?Bloemers Steven?J.?Rhemrev David?R.?King George?C.?Velmahos Inger?B.?Schipper 《World journal of surgery》2018,42(11):3608-3615
Introduction
The incidence and nature of penetrating injuries differ between countries. The aim of this study was to analyze characteristics and clinical outcomes of patients with penetrating injuries treated at urban Level-1 trauma centers in the USA (USTC) and the Netherlands (NLTC).Methods
In this retrospective cohort study, 1331 adult patients (470 from five NLTC and 861 from three USTC) with truncal penetrating injuries admitted between July 2011 and December 2014 were included. In-hospital mortality was the primary outcome. Outcome comparisons were adjusted for differences in population characteristics in multivariable analyses.Results
In USTC, gunshot wound injuries (36.1 vs. 17.4%, p?<?0.001) and assaults were more frequent (91.2 vs. 77.7%, p?<?0.001). ISS was higher in USTC, but the Revised Trauma Score (RTS) was comparable. In-hospital mortality was similar (5.0 vs. 3.6% in NLTC, p?=?0.25). The adjusted odds ratio for mortality in USTC compared to NLTC was 0.95 (95% confidence interval 0.35–2.54). Hospital stay length of stay was shorter in USTC (difference 0.17 days, 95% CI ?0.29 to ?0.05, p?=?0.005), ICU admission rate was comparable (OR 0.96, 95% CI 0.71–1.31, p?=?0.80), and ICU length of stay was longer in USTC (difference of 0.39 days, 95% CI 0.18–0.60, p?<?0.0001). More USTC patients were discharged to home (86.9 vs. 80.6%, p?<?0.001). Readmission rates were similar (5.6 vs. 3.8%, p?=?0.17).Conclusion
Despite the higher incidence of penetrating trauma, particularly firearm-related injuries, and higher hospital volumes in the USTC compared to the NLTC, the in-hospital mortality was similar. In this study, outcome of care was not significantly influenced by differences in incidence of firearm-related injuries.7.
Angela Peron Aglaia Vignoli Francesca La Briola Emanuela Morenghi Lucia Tansini Rosa Maria Alfano Gaetano Bulfamante Silvia Terraneo Filippo Ghelma Giuseppe Banderali David H. Viskochil John C. Carey Maria Paola Canevini 《European journal of medical genetics》2018,61(7):403-410
Tuberous Sclerosis Complex (TSC) is a multisystemic condition caused by mutations in TSC1 or TSC2, but a pathogenic variant is not identified in up to 10% of the patients. The aim of this study was to delineate the phenotype of pediatric and adult patients with a definite clinical diagnosis of TSC and no mutation identified in TSC1 or TSC2.We collected molecular and clinical data of 240 patients with TSC, assessing over 50 variables. We compared the phenotype of the homogeneous group of individuals with No Mutation Identified (NMI) with that of TSC patients with a TSC1 and TSC2 pathogenic variant.9.17% of individuals were classified as NMI. They were diagnosed at an older age (p?=?0.001), had more frequent normal cognition (p <?0.001) and less frequent epilepsy (p =?0.010), subependymal nodules (p?=?0.022) and giant cell astrocytomas (p?=?0.008) than patients with TSC2 pathogenic variants. NMI individuals showed more frequent bilateral and larger renal angiomyolipomas (p?=?0.001; p?=?0.003) and pulmonary involvement (trend) than patients with TSC1 pathogenic variants. Only one NMI individual had intellectual disability. None presented with a subependymal giant cell astrocytoma. Other medical problems not typical of TSC were found in 42.86%, without a recurrent pattern of abnormalities. Other TSC-associated neuropsychiatric disorders and drug-resistance in epilepsy were equally frequent in the three groups.This study provides a systematic clinical characterization of patients with TSC and facilitates the delineation of a distinctive phenotype indicative of NMI patients, with important implications for surveillance. 相似文献
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Laryngeal motility alteration: A missing link between sleep apnea and vagus nerve stimulation for epilepsy 下载免费PDF全文
Elena Zambrelli Alberto M. Saibene Francesca Furia Valentina Chiesa Aglaia Vignoli Carlotta Pipolo Giovanni Felisati Maria Paola Canevini 《Epilepsia》2016,57(1):e24-e27
This study aimed to evaluate the prevalence and the relationship of sleep breathing disorders (SBDs) and laryngeal motility alterations in patients with drug‐resistant epilepsy after vagus nerve stimulator (VNS) implantation. Twenty‐three consecutive patients with medically refractory epilepsy underwent out‐of‐center sleep testing before and after VNS implantation. Eighteen eligible subjects underwent endoscopic laryngeal examination post‐VNS implantation. Statistical analysis was carried out to assess an association between laryngeal motility alterations and the onset/worsening of SBDs. After VNS implantation, 11 patients showed a new‐onset mild/moderate SBD. Half of the patients already affected by obstructive sleep apnea (OSA) showed worsening of SBD. All of the patients with a new‐onset OSA had a laryngeal pattern with left vocal cord adduction (LVCA) during VNS stimulation. The association between VNS‐induced LVCA and SBD was statistically significant. This study suggests an association between VNS and SBD, hinting to a pivotal role of laryngeal motility alterations. The relationship between SBD and VNS‐induced LVCA supports the need to routinely investigate sleep respiratory and laryngeal motility patterns before and after VNS implantation. 相似文献
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Extrastriate visual cortex in idiopathic occipital epilepsies: The contribution of retinotopic areas to spike generation 下载免费PDF全文
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Symptomatic and presumed symptomatic focal epilepsies in childhood: An observational,prospective multicentre study 下载免费PDF全文
Marilena Vecchi Carmen Barba Debora De Carlo Micol Stivala Renzo Guerrini Emilio Albamonte Domiziana Ranalli Domenica Battaglia Giada Lunardi Clementina Boniver Benedetta Piccolo Francesco Pisani Gaetano Cantalupo Giuliana Nieddu Susanna Casellato Silvia Cappanera Elisabetta Cesaroni Nelia Zamponi Domenico Serino Lucia Fusco Alessandro Iodice Filippo Palestra Lucio Giordano Elena Freri Ilaria De Giorgi Francesca Ragona Tiziana Granata Isabella Fiocchi Stefania Maria Bova Massimo Mastrangelo Alberto Verrotti Sara Matricardi Elena Fontana Davide Caputo Francesca Darra Bernardo Dalla Bernardina Francesca Beccaria Giuseppe Capovilla Maria Pia Baglietto Alessandra Gagliardi Aglaia Vignoli Maria Paola Canevini Egle Perissinotto Stefano Francione 《Epilepsia》2016,57(11):1808-1816